Relationship between Semmes-Weinstein Monofilaments perception Test and sensory nerve conduction studies in Carpal Tunnel Syndrome.

PubMed

Raji, Parvin; Ansari, Noureddin Nakhostin; Naghdi, Soofia; Forogh, Bijan; Hasson, Scott

2014-01-01

The Semmes-Weinstein Monofilament Test (SWMT) is a clinical widely used test to quantify the sensibility in patients with Carpal Tunnel Syndrome (CTS). No study has investigated the relationship between the SWMT and sensory nerve conduction studies (SNCS) in patients with CTS. To assess the relationship between the SWMT and SNCS findings in patients with CTS. This cross-sectional clinical measurement study included 35 patients with CTS (55 hands) with a mean age of 45 ± 12 years. The outcome measures were the SWMT and SNCS measures of distal latency (DLs), amplitude (AMPs), and nerve conduction velocity (NCV). The median innervated fingers were tested using SWMT and electrodiagnostic tests. The primary outcome was the correlations between the SWMTs and NCS measures. All of the patients/hands had abnormal NCS findings. When looking at the three digits of interest (thumb, index and middle), the thumb SWMTs had the highest number of abnormal findings (58.2%), with the middle digit having the lowest (45.5%). All NCS findings were statistically different between abnormal and normal thumb SWMTs and abnormal and normal total summed SWMTs. There were significant moderate correlations between thumb SWMT scores and all NCS outcomes. Although only approximately 50% of the CTS diagnosed through NCS are corroborated through SWMT; the significant associations between SWMT and NCS measures suggest that SWMT is a valid test for assessing sensations in patients with CTS.

Low Peripheral Nerve Conduction Velocities and Amplitudes Are Strongly Related to Diabetic Microvascular Complications in Type 1 Diabetes

PubMed Central

Charles, Morten; Soedamah-Muthu, Sabita S.; Tesfaye, Solomon; Fuller, John H.; Arezzo, Joseph C.; Chaturvedi, Nishi; Witte, Daniel R.

2010-01-01

OBJECTIVE Slow nerve conduction velocity and reduction in response amplitude are objective hallmarks of diabetic sensorimotor polyneuropathy. Because subjective or clinical indicators of neuropathy do not always match well with the presence of abnormal nerve physiology tests, we evaluated associations to nerve conduction in patients with type 1 diabetes. RESEARCH DESIGN AND METHODS Nerve conduction studies were performed in the distal sural and ulnar sensory nerves and the peroneal motor nerve in 456 individuals with type 1 diabetes who participated in the follow-up visit of the EURODIAB Prospective Complications Study (EPCS). We used multivariate regression models to describe associations to decreased nerve conduction measures. RESULTS In addition to an effect of duration of diabetes and A1C, which were both associated with low nerve conduction velocity and response amplitude, we found that the presence of nephropathy, retinopathy, or a clinical diagnosis of neuropathy was associated with low nerve conduction velocity and amplitude. In the case of nonproliferative retinopathy, the odds ratio (OR) for being in lowest tertile was 2.30 (95% CI 1.13–4.67) for nerve conduction velocity. A similar OR was found for each 2% difference in A1C (2.39 [1.68–3.41]). CONCLUSIONS We show that the presence of other microvascular diabetes complications, together with diabetes duration and A1C, are associated with low nerve conduction velocity and amplitude response and that cardiovascular disease or risk factors do not seem to be associated with these measures. PMID:20823346

Evaluative procedures to detect, characterize, and assess the severity of diabetic neuropathy.

PubMed

Dyck, P J

1991-01-01

Minimal criteria for diabetic neuropathy need to be defined and universally applied. Standardized evaluative procedures need to be agreed and normal ranges determined from healthy volunteers. Types and stages of neuropathy should be established and assessments performed on representative populations of both Type 1 and Type 2 diabetic patients. Potential minimal criteria include absent ankle reflexes and vibratory sensation, and abnormalities of nerve conduction. However, the preferred criterion is the identification of more than two statistically defined abnormalities among symptoms and deficits, nerve conduction, quantitative sensory examination or quantitative autonomic examination. Various evaluative procedures are available. Symptoms should be assessed and scores can be assigned to neurological deficits. However, assessments of nerve conduction provide the most specific, objective, sensitive, and repeatable procedures, although these may be the least meaningful. Many techniques are available for quantitative sensory examination, but are poorly standardized and normal values are not available. For quantitative autonomic examination, tests are available for the adequacy of cardiovascular and peripheral vascular reflexes and increasingly for other autonomic functions. In any assessment of nerve function the conditions should be optimized and standardized, and stimuli defined. Specific instructions should be given and normal ranges established in healthy volunteers.

N-hexane neuropathy with vertigo and cold allodynia in a silk screen printer: A case study.

PubMed

Pradhan, Sunil; Tandon, Ruchika

2015-01-01

N-hexane neuropathy is an occupational disease caused by exposure to n-hexane, which is used as a solvent in silk screen printing. Here, we describe a 35-year-old man, a silk screen printer by profession, who presented with dizziness, distal swelling of both lower limbs for 10 months and tingling and burning sensation in both feet for 9.5 months along with cold allodynia. The patient had normal results of a motor and sensory system examination, apart from an impaired temperature sense. Nerve conduction tests showed a conduction block in bilateral common peroneal nerves and absence of conduction in bilateral sural nerves. These symptoms resolved when further exposure to n-hexane was ceased but cold allodynia remained. Thus, cold allodynia and impaired temperature sense can be a manifestation of n-hexane neuropathy. Hence, abnormalities on nerve conduction studies can be detected in n-hexane neuropathy patients, even before clinical examination detects any such abnormalities. In the case of the patients presenting with sensory motor neuropathy, history of occupational exposure to n-hexane becomes important, as the sooner the disease is detected, the better the chances of recovery. This work is available in Open Access model and licensed under a CC BY-NC 3.0 PL license.

A Novel Technique for Clinical Assessment of Laryngeal Nerve Conduction: Normal and Abnormal Results

PubMed Central

Sulica, Lucian; Carey, Bridget; Branski, Ryan C.

2014-01-01

Objectives/Hypothesis To describe a novel conduction study of the laryngeal nerves, including normal values and abnormal findings. Study Design Prospective nonrandomized. Methods Seventeen healthy adult volunteers, as well as three patients with clinically identified laryngeal neuropathy, underwent low-level brief electrical stimulation of the laryngeal mucosa by means of a wire inserted via a transnasal flexible laryngoscope. Bilateral hookwire electrodes recorded the result in the laryngeal adductor muscles. Results This study yields an early response ipsilateral to the side of stimulation (LR1), which is uniform and consistent (right 5 13.2 6 0.80 msec; left 5 15.2 6 1.20 msec), and late bilateral responses (ipsilateral LR2 [LR2i] and contralateral LR2 [LR2c]), which exhibit greater variation in latency and morphology (right LR2i 5 50.5 6 3.38 msec; left LR2i 5 52.2 msec; right LR2c 5 50.7 6 4.26; left LR2c 5 50.6 6 4.07). Findings in abnormal patients differ significantly from normal, consistent with the distribution of neuropathy. Conclusions We describe a novel, clinically applicable conduction study of laryngeal nerves. Normative electrodiagnostic values and variations of the reflex responses of the laryngeal adductor muscles in response to irritative stimulation of the laryngeal mucosa (Laryngeal Closure Reflex) are proposed. By enabling the determination of electrophysiological parameters of the superior laryngeal and recurrent laryngeal branches of cranial nerve X (CN X), this procedure, which is used as an adjunct to laryngeal electromyography, may provide earlier and more accurate information regarding the extent and grade of nerve injury. Because injury grade relates directly to prognosis, the information derived from this test may have clinical relevance in determining optimal treatment. Level of Evidence 4. PMID:23835889

Clinical and Electrodiagnostic Abnormalities of the Median Nerve in US Army Dental Assistants at the Onset of Training

DTIC Science & Technology

2012-01-01

EMG studies). Data Management and Analysis Descriptive statistics for subject demographics and nerve conduction study variables were calculated using...military N/A Family history of CTS; previous work history as electrician, guitar player 49 (R) None N/A Dental assistant; waiter NCS indicates

Median and ulnar neuropathies in U.S. Army Medical Command Band members.

PubMed

Shaffer, Scott W; Koreerat, Nicholas R; Gordon, Lindsay B; Santillo, Douglas R; Moore, Josef H; Greathouse, David G

2013-12-01

Musicians have been reported as having a high prevalence of upper-extremity musculoskeletal disorders, including carpal tunnel syndrome. The purpose of this study was to determine the presence of median and ulnar neuropathies in U.S. Army Medical Command (MEDCOM) Band members at Fort Sam Houston, Texas. Thirty-five MEDCOM Band members (30 males, 5 females) volunteered to participate. There were 33 right-handed musicians, and the mean length of time in the MEDCOM Band was 12.2 yrs (range, 1-30 yrs). Subjects completed a history form, were interviewed, and underwent a physical examination of the cervical spine and bilateral upper extremities. Nerve conduction studies of the bilateral median and ulnar nerves were performed. Electrophysiological variables served as the reference standard for median and ulnar neuropathy and included distal sensory latencies, distal motor latencies, amplitudes, conduction velocities, and comparison study latencies. Ten of the 35 subjects (29%) presented with abnormal electrophysiologic values suggestive of an upper extremity mononeuropathy. Nine of the subjects had abnormal median nerve electrophysiologic values at or distal to the wrist; 2 had bilateral abnormal values. One had an abnormal ulnar nerve electrophysiologic assessment at the elbow. Nine of these 10 subjects had clinical examination findings consistent with the electrophysiological findings. The prevalence of mononeuropathies in this sample of band members is similar to that found in previous research involving civilian musicians (20-36%) and far exceeds that reported in the general population. Prospective research investigating screening, examination items, and injury prevention measures in musicians appears to be warranted.

Neuromuscular ultrasound in patients with carpal tunnel syndrome and normal nerve conduction studies.

PubMed

Aseem, Fazila; Williams, Jessica W; Walker, Francis O; Cartwright, Michael S

2017-06-01

Nerve conduction studies (NCS) are sensitive for carpal tunnel syndrome (CTS), but a small proportion of patients with clinical CTS have normal NCS. This retrospective study was designed to assess the neuromuscular ultrasound findings in a group of CTS patients. The electronic medical record was reviewed by a neurologist to identify patients who had a diagnosis of CTS with normal NCS, including either mixed median-ulnar comparison or transcarpal sensory studies, and complete neuromuscular ultrasound evaluation for CTS. Fourteen individuals (22 wrists) met all criteria. A total of 92.3% had median nerve cross-sectional area enlargement at the wrist (mean 16.3 mm 2 ), 100% had increased wrist-to-forearm median nerve area ratio (mean 2.4), 82.4% had decreased median nerve echogenicity, 75.0% had decreased median nerve mobility, and 7.1% had increased median nerve vascularity. A large proportion of patients with clinical CTS but normal NCS have abnormal neuromuscular ultrasound findings. Muscle Nerve 55: 913-915, 2017. © 2016 Wiley Periodicals, Inc.

Segmental somatosensory-evoked potentials as a diagnostic tool in chronic inflammatory demyelinating polyneuropathies, and other sensory neuropathies.

PubMed

Koutlidis, R M; Ayrignac, X; Pradat, P-F; Le Forestier, N; Léger, J-M; Salachas, F; Maisonobe, T; Fournier, E; Viala, K

2014-09-01

Somatosensory-evoked potentials with segmental recordings were performed with the aim of distinguishing chronic inflammatory demyelinating polyneuropathy from other sensory neuropathies. Four groups of 20 subjects each corresponded to patients with (1) possible sensory chronic inflammatory demyelinating polyneuropathy, (2) patients with sensory polyneuropathy of unknown origin, (3) patients with amyotrophic lateral sclerosis and (4) normal subjects. The patients selected for this study had preserved sensory potentials on electroneuromyogram and all waves were recordable in evoked potentials. Somatosensory-evoked potentials evaluations were carried out by stimulation of the posterior tibial nerve at the ankle, recording peripheral nerve potential in the popliteal fossa, radicular potential and spinal potential at the L4-L5 and T12 levels, and cortical at C'z, with determination of distal conduction time, proximal and radicular conduction time and central conduction time. In the group of chronic inflammatory demyelinating polyneuropathy, 80% of patients had abnormal conduction in the N8-N22 segment and 95% had abnormal N18-N22 conduction time. In the group of neuropathies, distal conduction was abnormal in most cases, whereas 60% of patients had no proximal abnormality. None of the patients in the group of amyotrophic lateral sclerosis had an abnormal N18-N22 conduction time. Somatosensory-evoked potentials with segmental recording can be used to distinguish between atypical sensory chronic inflammatory demyelinating polyneuropathy and other sensory neuropathies, at the early stage of the disease. Graphical representation of segmental conduction times provides a rapid and accurate visualization of the profile of each patient. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Electrodiagnosis of reversible conduction failure in Guillain-Barré syndrome.

PubMed

Chan, Yee-Cheun; Punzalan-Sotelo, Aubrey M; Kannan, Therimadasamy A; Shahrizaila, Nortina; Umapathi, Thirugnanam; Goh, Eunice J H; Fukami, Yuki; Wilder-Smith, Einar; Yuki, Nobuhiro

2017-11-01

In this study we propose electrodiagnostic criteria for early reversible conduction failure (ERCF) in axonal Guillain-Barré syndrome (GBS) and apply them to a cohort of GBS patients. Serial nerve conduction studies (NCS) were retrospectively analyzed in 82 GBS patients from 3 centers. The criteria for the presence of ERCF in a nerve were: (i) a 50% increase in amplitude of distal compound muscle action potentials or sensory nerve action potentials; or (ii) resolution of proximal motor conduction block with an accompanying decrease in distal latencies or compound muscle action potential duration or increase in conduction velocities. Of 82 patients from 3 centers, 37 (45%) had ERCF, 21 (26%) had a contrasting evolution pattern, and 8 (10%) had both. Sixteen patients did not show an amplitude increase of at least 50%. Our proposed criteria identified a group of patients with a characteristic evolution of NCS abnormality that is consistent with ERCF. Muscle Nerve 56: 919-924, 2017. © 2017 Wiley Periodicals, Inc.

Screening for Electrophysiological Abnormalities in Chronic Hepatitis C Infection: Peripheral Neuropathy and Optic Neuropathy.

PubMed

Köşkderelioğlu, Aslı; Ortan, Pınar; Ari, Alpay; Gedizlioğlu, Muhteşem

2016-03-01

To investigate the existence of peripheral and optic neuropathies in asymptomatic individuals with hepatitis C infection. Thirty consecutive patients who were followed in a hepatitis C outpatient clinic were recruited for electrophysiological evaluation together with 30 age- and gender-compatible healthy controls. All patients had a detailed neurological examination. The information regarding the disease duration and management with interferons were collected. Nerve conduction studies and visual evoked potentials (VEP) were recorded in all subjects. The results of the patient and control groups were statistically compared. Of the patients with hepatitis C infection, 16 were females and 14 males. The mean age was 57.5 years, and the average disease duration was 6.43 years. The P100 latencies in the patient group were within normal limits, while the amplitudes were meaningfully small by comparison with the controls. There were some abnormalities in the nerve conduction studies of 15 patients. Sensorial neuropathy was detected in two patients, sensorimotor polyneuropathy in four, carpal tunnel syndrome in seven, and carpal tunnel syndrome and sensorimotor polyneuropathy as comorbid states in another two patients. The nerve conduction studies and VEP parameters were entirely normal in the control group. Hepatitis C-related neurological abnormalities may occur both in the central and peripheral nervous system. Mononeuritis multiplex, sensorial axonal neuropathy, and multiple mononeuropathies are some of the presentations of the peripheral nervous system involvement. The mode of infection is considered to be via vasculitic mechanisms. In addition, optic neuropathy is a known complication of interferon treatment. Autoantibodies, cytokines, chemokines, and cryoglobulins are accused to play roles in the pathogenesis. In this study, we investigated the involvement of the peripheral nervous system and optic nerves in a group of patients with hepatitis C. The results were in favor of peripheral nerve injury of various types and optic neuropathy of the axonal type.

Screening for Electrophysiological Abnormalities in Chronic Hepatitis C Infection: Peripheral Neuropathy and Optic Neuropathy

PubMed Central

KÖŞKDERELİOĞLU, Aslı; ORTAN, Pınar; ARI, Alpay; GEDİZLİOĞLU, Muhteşem

2016-01-01

Introduction To investigate the existence of peripheral and optic neuropathies in asymptomatic individuals with hepatitis C infection. Methods Thirty consecutive patients who were followed in a hepatitis C outpatient clinic were recruited for electrophysiological evaluation together with 30 age- and gender-compatible healthy controls. All patients had a detailed neurological examination. The information regarding the disease duration and management with interferons were collected. Nerve conduction studies and visual evoked potentials (VEP) were recorded in all subjects. The results of the patient and control groups were statistically compared. Results Of the patients with hepatitis C infection, 16 were females and 14 males. The mean age was 57.5 years, and the average disease duration was 6.43 years. The P100 latencies in the patient group were within normal limits, while the amplitudes were meaningfully small by comparison with the controls. There were some abnormalities in the nerve conduction studies of 15 patients. Sensorial neuropathy was detected in two patients, sensorimotor polyneuropathy in four, carpal tunnel syndrome in seven, and carpal tunnel syndrome and sensorimotor polyneuropathy as comorbid states in another two patients. The nerve conduction studies and VEP parameters were entirely normal in the control group. Conclusion Hepatitis C-related neurological abnormalities may occur both in the central and peripheral nervous system. Mononeuritis multiplex, sensorial axonal neuropathy, and multiple mononeuropathies are some of the presentations of the peripheral nervous system involvement. The mode of infection is considered to be via vasculitic mechanisms. In addition, optic neuropathy is a known complication of interferon treatment. Autoantibodies, cytokines, chemokines, and cryoglobulins are accused to play roles in the pathogenesis. In this study, we investigated the involvement of the peripheral nervous system and optic nerves in a group of patients with hepatitis C. The results were in favor of peripheral nerve injury of various types and optic neuropathy of the axonal type. PMID:28360761

The prevalence of early subclinical somatic neuropathy in children and adolescents with Type 1 diabetes mellitus and its association with the persistence of autoantibodies to glutamic acid decarboxylase (GAD) and islet antigen-2 (IA-2).

PubMed

Louraki, Maria; Katsalouli, Marina; Kanaka-Gantenbein, Christina; Kafassi, Nikolitsa; Critselis, Eleni; Kallinikou, Dimitra; Tsentidis, Charalampos; Karavanaki, Kyriaki

2016-07-01

To evaluate the prevalence of early somatic neuropathy in children and adolescents with Type 1 diabetes mellitus (Type 1 DM) and its association with the presence of glutamic acid decarboxylase and islet antigen-2 autoantibodies (GADA and IA-2A). A cross-sectional study was conducted in a hospital-based cohort of pediatric Type 1 DM patients (n=85, mean(±SD) age: 13.5±3.4years, mean(±SD) disease duration 5.5±3.4years). Peripheral neuropathy was assessed with nerve conduction studies (NCS). GADA and IA-2A titers were measured with radioligand assays. Among the study population, 34.1% had at least one abnormal electrophysiological parameter, although predominantly asymptomatic. The highest rates of abnormality were detected in sensory peroneal nerve (25.9%) followed by sural nerve (15.3%). Affected patients were not different in terms of age, diabetes duration or glycaemic control. Among the participants, 62.4% had positive GADA, 58.8% positive IA-2A and 42.4% double antibody positivity. Abnormal NCS correlated neither with GADA nor with IA-2A levels or positivity. However lower sensory nerve action potential in the peroneal nerve, indicative of early axonal dysfunction, was observed in patients with GADA or IA-2A positivity. Absence of both antibodies was associated with better action potentials in all the examined nerves of the lower limbs. Impaired indices of subclinical peripheral primarily sensory neuropathy were present among one third of Type 1 DM children and adolescents, with no impact of diabetes duration or glycaemic control. GADA and IA-2A seem to be involved in the development of axonal degeneration, in a pathway which remains to be identified. Copyright © 2016. Published by Elsevier Ireland Ltd.

[MRI in Duane retraction syndrome: Preliminary results].

PubMed

Denis, D; Cousin, M; Zanin, E; Toesca, E; Girard, N

2011-09-01

Duane retraction syndrome (DRS) is a congenital ocular motility disorder with innervational dysgenesis. MRI improves our understanding of this disease by providing in vivo access to nerves and oculomotor muscles. The goal of this prospective study (2000-2008) was to analyze DRS clinically and neuroradiologically. Twenty-four patients (27 eyes) received a complete ophthalmologic evaluation and a brain-orbital MRI. The average age was 6.1 years. MRI was performed with 3D T2 CISS-weighted images through the brainstem to visualize the cisternal segments of the cranial nerves and the orbit (lateral and medial recti muscles). MRI anomalies were classified according to type I, II, and III and depending on their condition in the posterior fossa (absence, hypoplasia) and in the orbit (muscle anomalies). Of 27 eyes, 70% were type I, 19% type II, and 11% type III. MRI showed abducens nerve abnormalities in 93% of the cases (78% absence) and muscle abnormalities in 57.5% of the cases. A detailed description showed 100% abducens nerve abnormalities and 58% abnormal lateral rectus muscle in type I, 60% abducens nerve abnormalities and 60% abnormal lateral rectus muscle in type II, and 100% abducens nerve abnormalities and 66% abnormal lateral rectus in type III. This study presents two major findings: detection of abducens nerve abnormalities in most cases of DRS whatever the type, associated with muscle abnormalities, and the confirmation that this absence may exist in type II (2/5). Thus MRI proved to be a valuable tool for investigating these patients, improving the comprehension of the physiopathogenics of this disease. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Which nerve conduction parameters can predict spontaneous electromyographic activity in carpal tunnel syndrome?

PubMed

Chang, Chia-Wei; Lee, Wei-Ju; Liao, Yi-Chu; Chang, Ming-Hong

2013-11-01

We investigate electrodiagnostic markers to determine which parameters are the best predictors of spontaneous electromyographic (EMG) activity in carpal tunnel syndrome (CTS). We enrolled 229 patients with clinically proven and nerve conduction study (NCS)-proven CTS, as well as 100 normal control subjects. All subjects were evaluated using electrodiagnostic techniques, including median distal sensory latencies (DSLs), sensory nerve action potentials (SNAPs), distal motor latencies (DMLs), compound muscle action potentials (CMAPs), forearm median nerve conduction velocities (FMCVs) and wrist-palm motor conduction velocities (W-P MCVs). All CTS patients underwent EMG examination of the abductor pollicis brevis (APB) muscle, and the presence or absence of spontaneous EMG activities was recorded. Normal limits were determined by calculating the means ± 2 standard deviations from the control data. Associations between parameters from the NCS and EMG findings were investigated. In patients with clinically diagnosed CTS, abnormal median CMAP amplitudes were the best predictors of spontaneous activity during EMG examination (p<0.001; OR 36.58; 95% CI 15.85-84.43). If the median CMAP amplitude was ≤ 2.1 mV, the rate of occurrence of spontaneous EMG activity was >95% (positive predictive rate >95%). If the median CMAP amplitude was higher than the normal limit (>4.9 mV), the rate of no spontaneous EMG activity was >94% (negative predictive rate >94%). An abnormal SNAP amplitude was the second best predictor of spontaneous EMG activity (p<0.001; OR 4.13; 95% CI 2.16-7.90), and an abnormal FMCV was the third best predictor (p=0.01; OR 2.10; 95% CI 1.20-3.67). No other nerve conduction parameters had significant power to predict spontaneous activity upon EMG examination. The CMAP amplitudes of the APB are the most powerful predictors of the occurrence of spontaneous EMG activity. Low CMAP amplitudes are strongly associated with spontaneous activity, whereas high CMAP amplitude are less associated with spontaneous activity, implying that needle EMG examination should be recommended for the detection of spontaneous activity in those CTS patients whose NCS reveals CMAP amplitudes between 2.1 mV and the lower normal limit (4.9mV in the present study). Using NCS, electromyographers can predict the presence of spontaneous EMG activity in CTS patients. Copyright © 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

Electrophysiologic changes following treatment with organophosphorus-induced delayed neuropathy-producing agents in the adult hen.

PubMed

Robertson, D G; Schwab, B W; Sills, R D; Richardson, R J; Anderson, R J

1987-03-15

Although clinical, pathological, and biochemical effects of organophosphorus-induced delayed neuropathy (OPIDN) have been intensively investigated in the adult hen, detailed electrophysiological studies are lacking. Adult white leghorn hens were treated with a single oral dose of either 30 mg/kg tri-2-cresyl phosphate (TOCP), 750 mg/kg TOCP, 4 mg/kg di-n-butyl-2,2-dichlorovinyl phosphate (DBCV), or 30 mg/kg di-n-butyl-2,2-dichlorovinyl phosphinate (DBCV-P). The 750 mg/kg TOCP and DBCV, but not the 30 mg/kg TOCP and DBCV-P, treatments resulted in clinical signs of OPIDN and mild to marked damage of the tibial nerve 21 days after dose. Twenty-four hr lymphocyte neurotoxic esterase (NTE) inhibition was used as an index of brain NTE inhibition for the various organophosphorus compound (OP) treatment. Twenty-four hr lymphocyte NTE inhibition for 30 mg/kg TOCP, 750 mg/kg TOCP, DBCV, and DBCV-P was 54.1, 87.1, 84.8, and 68.3%, respectively. Twenty-one days after dose, the TOCP-treated hens exhibited some abnormalities in conduction velocity and action potential duration in the tibial or sciatic nerves. No abnormalities were observed in action potential parameters of either the DBCV or DBCV-P treatments. Neurotoxic OP (TOCP and DBCV) treatment resulted in decreased refractoriness in the tibial nerve, increased refractoriness in the sciatic nerve, and elevated strength duration threshold for both nerves. These changes were not present in nerves from DBCV-P (a non-neurotoxic NTE inhibitor)-treated hens. These results suggest that refractory period and strength duration abnormalities in peripheral nerve correlate well with the production of OPIDN and are evident without coincident clinical signs or histopathology.

The Role of Cervical and Ocular Vestibular Evoked Myogenic Potentials in the Assessment of Patients with Vestibular Schwannomas

PubMed Central

Chiarovano, Elodie; Darlington, Cynthia; Vidal, Pierre-Paul; Lamas, Georges; de Waele, Catherine

2014-01-01

Objectives To investigate the clinical utility of VEMPs in patients suffering from unilateral vestibular schwannoma (VS) and to determine the optimal stimulation parameter (air conducted sound, bone conducted vibration) for evaluating the function of the vestibular nerve. Methods Data were obtained in 63 patients with non-operated VS, and 20 patients operated on VS. Vestibular function was assessed by caloric, cervical and ocular VEMP testing. 37/63 patients with conclusive ACS ocular VEMPs responses were studied separately. Results In the 63 non-operated VS patients, cVEMPs were abnormal in 65.1% of patients in response to AC STB and in 49.2% of patients to AC clicks. In the 37/63 patients with positive responses from the unaffected side, oVEMPs were abnormal in 75.7% of patients with ACS, in 67.6% with AFz and in 56.8% with mastoid BCV stimulation. In 16% of the patients, VEMPs were the only abnormal test (normal caloric and normal hearing). Among the 26 patients who did not show oVEMP responses on either side with ACS, oVEMPs responses could be obtained with AFz (50%) and with mastoid stimulation (89%). Conclusions The VEMP test demonstrated significant clinical value as it yielded the only abnormal test results in some patients suffering from a unilateral vestibular schwannoma. For oVEMPs, we suggest that ACS stimulation should be the initial test. In patients who responded to ACS and who had normal responses, BCV was not required. In patients with abnormal responses on the affected side using ACS, BCV at AFz should be used to confirm abnormal function of the superior vestibular nerve. In patients who exhibited no responses on either side to ACS, BCV was the only approach allowing assessment of the function of the superior vestibular nerve. We favor using AFz stimulation first because it is easier to perform in clinical practice than mastoid stimulation. PMID:25137289

Neural autoantibodies and neurophysiologic abnormalities in patients exposed to molds in water-damaged buildings.

PubMed

Campbell, Andrew W; Thrasher, Jack D; Madison, Roberta A; Vojdani, Aristo; Gray, Michael R; Johnson, Al

2003-08-01

Adverse health effects of fungal bioaerosols on occupants of water-damaged homes and other buildings have been reported. Recently, it has been suggested that mold exposure causes neurological injury. The authors investigated neurological antibodies and neurophysiological abnormalities in patients exposed to molds at home who developed symptoms of peripheral neuropathy (i.e., numbness, tingling, tremors, and muscle weakness in the extremities). Serum samples were collected and analyzed with the enzyme-linked immunosorbent assay (ELISA) technique for antibodies to myelin basic protein, myelin-associated glycoprotein, ganglioside GM1, sulfatide, myelin oligodendrocyte glycoprotein, alpha-B-crystallin, chondroitin sulfate, tubulin, and neurofilament. Antibodies to molds and mycotoxins were also determined with ELISA, as reported previously. Neurophysiologic evaluations for latency, amplitude, and velocity were performed on 4 motor nerves (median, ulnar, peroneal, and tibial), and for latency and amplitude on 3 sensory nerves (median, ulnar, and sural). Patients with documented, measured exposure to molds had elevated titers of antibodies (immunoglobulin [Ig]A, IgM, and IgG) to neural-specific antigens. Nerve conduction studies revealed 4 patient groupings: (1) mixed sensory-motor polyneuropathy (n = 55, abnormal), (2) motor neuropathy (n = 17, abnormal), (3) sensory neuropathy (n = 27, abnormal), and (4) those with symptoms but no neurophysiological abnormalities (n = 20, normal controls). All groups showed significantly increased autoantibody titers for all isotypes (IgA, IgM, and IgG) of antibodies to neural antigens when compared with 500 healthy controls. Groups 1 through 3 also exhibited abnormal neurophysiologic findings. The authors concluded that exposure to molds in water-damaged buildings increased the risk for development of neural autoantibodies, peripheral neuropathy, and neurophysiologic abnormalities in exposed individuals.

A Pilot Study of a Novel Automated Somatosensory Evoked Potential (SSEP) Monitoring Device for Detection and Prevention of Intraoperative Peripheral Nerve Injury in Total Shoulder Arthroplasty Surgery.

PubMed

Chui, Jason; Murkin, John M; Drosdowech, Darren

2018-05-21

Peripheral nerve injury is a potentially devastating complication after total shoulder arthroplasty (TSA) surgery. This pilot study aimed to assess the feasibility of using an automated somatosensory evoked potential (SSEP) device to provide a timely alert/intervention to minimize intraoperative nerve insults during TSA surgery. A prospective, single-arm, observational study was conducted in a single university hospital. The attending anesthesiologist monitored the study participants using the EPAD automated SSEP device and an intervention was made if there was an alert during TSA surgery. The median, radial, and ulnar nerve SSEP on the operative arm, as well as the median nerve SSEP of the nonoperative arm were monitored for each patient. All patients were evaluated for postoperative neurological deficits 6 weeks postoperatively. In total, 21 patients were consented and were successfully monitored. In total, 4 (19%) patients developed intraoperative abnormal SSEP signal changes in the operative arm, in which 3 were reversible and 1 was irreversible till the end of surgery. Median and radial nerves were mostly involved (3/4 patients). The mean cumulative duration of nerve insult (abnormal SSEP) was 21.7±26.2 minutes. Univariate analysis did not identify predictor of intraoperative nerve insults. No patients demonstrated postoperative peripheral neuropathy at 6 weeks. A high incidence (19%) of intraoperative nerve insult was observed in this study demonstrating the feasibility of using an automated SSEP device to provide a timely alert and enable an intervention in order to minimize peripheral nerve injury during TSA. Further randomized studies are warranted.

[Role of short-latency somatosensory evoked potential in the diagnosis of chronic inflammatory demyelinating polyneuropathy].

PubMed

Sun, Rui-Di; Fu, Bing; Jiang, Jun

2017-05-01

To investigate the role of short-latency somatosensory evoked potential (SSEP) in the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). A total of 48 children with a confirmed or suspected CIDP and 40 healthy children were enrolled. Nerve electrophysiological examination and/or SSEP examination was performed (the children in the healthy control group only underwent SSEP examination). Four-lead electromyography was used for nerve electrophysiological examination, including at least 4 motor nerves and 2 sensory nerves. N6 (elbow potential), N13 (cervical cord potential), and N20 (cortex potential) of the median nerve and N8 (popliteal fossa potential), N22 (lumbar cord potential), and P39 (cortex potential) of the tibial nerve were observed by SSEP examination. Among the 48 children with CIDP, 35 had demyelination in both motor and sensory nerves, 8 had demyelination in sensory nerves, and 5 had axonal degeneration. SSEP examination showed that 7 had conduction abnormality in the trunk of the brachial plexus and/or the posterior root and 33 had damage in the lumbosacral plexus and/or the posterior root. The 40 children with abnormal findings of SSEP examination included 8 children with affected sensory nerves and 5 children with secondary axonal degeneration who did not meet the electrophysiological diagnostic criteria for CIDP. Compared with the healthy control group, the CIDP group had significantly prolonged latency periods of N13 and N22 (P<0.05). SSEP can be used for the auxiliary diagnosis of CIDP, especially in CIDP children with affected sensory nerves or secondary axonal degeneration.

Sonographic and electrodiagnostic features of hereditary neuropathy with liability to pressure palsies.

PubMed

Ginanneschi, Federica; Filippou, Georgios; Giannini, Fabio; Carluccio, Maria A; Adinolfi, Antonella; Frediani, Bruno; Dotti, Maria T; Rossi, Alessandro

2012-12-01

In hereditary neuropathy with liability to pressure palsies (HNPP), the increase in distal motor latencies (DMLs) is often out of proportion to the slowing of conduction velocities, but the pathophysiological mechanism is still unclear. We used a combined electrophysiological and ultrasonographic (US) approach to provide insight into this issue. Twelve HNPP subjects underwent extensive electrophysiological studies and US measurements of the cross-sectional area (CSA) of several peripheral nerves. US nerve enlargement was only observed in the carpal tunnel, Guyon's canal, the elbow and the fibular head. We did not observe US abnormalities at sites where nerve entrapment is uncommon. An increase in DMLs was observed regardless of US nerve enlargement. The increased nerve CSA only in common sites of entrapment likely reflected the well-documented nerve vulnerability to mechanical stress in HNPP. No morphometric changes were seen in the distal nerve segments where compression/entrapment is unlikely, despite the fact that the DMLs were increased. These data suggest that factors other than mechanical stress are responsible for the distal slowing of action potential propagation. We speculate that a mixture of mechanical insults and an axon-initiated process in the distal nerves underlies the distal slowing and/or conduction failure in HNPP. © 2012 Peripheral Nerve Society.

Clinical and electrodiagnostic characteristics of nitrous oxide-induced neuropathy in Taiwan.

PubMed

Li, Han-Tao; Chu, Chun-Che; Chang, Kuo-Hsuan; Liao, Ming-Feng; Chang, Hong-Shiu; Kuo, Hung-Chou; Lyu, Rong-Kuo

2016-10-01

Nitrous oxide-induced neuropathy is toxic neuropathy occasionally encountered in Taiwanese neurological clinics. Only several case reports described their electrodiagnostic features. We used a case-control design to investigate the detailed electrodiagnostic characteristics and possible factors relating to severe nerve injury. We retrospectively reviewed 33 patients with nitrous oxide-induced neuropathy over a 10-year period and reported their demographic data, spinal cord MRI, laboratory examinations and nerve conduction studies. 56 healthy controls' nerve conduction studies were collected for comparison analysis. We noted significant motor and sensory amplitudes reduction, conduction velocities slowing, and latencies prolongation in most tested nerves compared to the controls. Similar nerve conduction study characteristics with prominent lower limbs' motor and sensory amplitudes reduction was observed in patient groups with or without abnormal vitamin B12 and/or homocysteine levels. Among those with lower limbs' motor or sensory amplitudes reduction <20% of the lower limit of normal, higher homocysteine levels were detected. Severe impairments of the lower limbs' sensory and motor amplitudes were frequently noted in patients with nitrous oxide exposure. Nitrous oxide exposure itself is an important factor for the development of neuropathy. Our study contributes to the understanding of electrodiagnostic features underlying the nitrous oxide-induced neuropathy. Copyright © 2016 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

Neurological Assessment and Nerve Conduction Study Findings in 22 Patients with Alkaptonuria from Jordan.

PubMed

Alrawashdeh, Omar; Alsbou, Mohammad; Alzoubi, Hamed; Al-Shagahin, Hani

2016-11-02

Alkaptonuria is a rare metabolic disease characterised by accumulative deposition of homogentisic acid in the connective tissue of the body. This results in early degeneration of tendons, cartilages, heart valves, and other tissues. The main objective of the study is to examine the possibility of the nervous system involvement in patients with alkaptonuria The sample consists of two groups; 22 patients with AKU and 20 controls. A neurological assessment has been carried out including detailed medical history, neurological examination, and a nerve conduction study of the nerves of the dominant hand. The prevalence of any abnormality was compared between the two groups using chi square test. The mean values of the nerve conduction study were compared between the two groups using student t-test. There was a higher prevalence of low back pain, hearing problems and tinnitus, numbness and neuropathic pain in alkaptonuria patients. There was no significant difference between the two groups in other conditions such as seizures, headache, and syncope. The values of the nerve conduction study did not show significant difference between the two groups. Neurologically related symptoms in alkaptonuria mostly represent complications of the connective tissue degeneration rather than direct involvement of the nervous system. This has been supported further by the normal findings of the neurophysiology study in patients with alkaptonuria.

Niceritrol prevents the decrease in red blood cell 2,3-diphosphoglycerate and neuropathy in streptozotocin-induced diabetic rats.

PubMed

Hotta, N; Nakamura, J; Kakuta, H; Fukasawa, H; Koh, N; Sakakibara, F; Mori, K; Sakamoto, N

1995-01-01

Nerve ischemia/hypoxia has been linked to the pathogenesis of diabetic complications. Red blood cell 2,3-diphosphoglycerate is an important regulator of peripheral tissue oxygenation; however, the relationship between 2,3-diphosphoglycerate concentration and diabetic complications has not been studied in detail. This investigation focused on the relationship between red blood cell 2,3-diphosphoglycerate and diabetic neuropathy, by measuring motor nerve conduction velocity and sciatic nerve blood flow in streptozotocin-induced diabetic rats. The effect of treatment with niceritrol, a nicotinic acid derivative that acts as a vasodilator and reduces serum lipid concentrations, on 2,3-diphosphoglycerate concentration and diabetic neuropathy was also examined. Untreated diabetic rats had significantly lower concentrations of red blood cell 2,3-diphosphoglycerate, higher concentrations of serum total cholesterol and triglyceride, as well as reduced motor nerve conduction velocity and sciatic nerve blood flow, compared to untreated normal rats. Niceritrol prevented these abnormalities without correcting hyperglycemia in diabetic rats, but had no effect on these parameters in normal rats. Red blood cell 2,3-diphosphoglycerate concentration and motor nerve conduction velocity showed a positive correlation with sciatic nerve blood flow and 2,3-diphosphoglycerate, respectively. These observations suggest that ischemia/hypoxia plays an important role in the development of diabetic neuropathy, and that niceritrol has a therapeutic effect on this condition by improving endoneurial ischemia/hypoxia.

Chronic inflammatory pure sensory polyradiculoneuropathy: a rare CIDP variant with unusual electrophysiology.

PubMed

Rajabally, Yusuf A; Wong, Siew L

2012-03-01

We describe a patient presenting with progressive upper limb numbness and sensory ataxia of the 4 limbs. Motor nerve conduction studies were completely normal. Sensory electrophysiology showed reduced/absent upper limb sensory action potentials (SAPs). In the lower limbs, SAPs were mostly normal. Sensory conduction velocities were normal. Forearm sensory conduction blocks were present for both median nerves on antidromic testing. The maximal recordable sural SAP was preserved in comparison to maximal recordable radial SAP, consistent with an "abnormal radial normal sural" pattern. Somatosensory evoked potentials were unrecordable for tibial and median nerves. Cerebrospinal fluid protein was raised (0.99 g/L). The patient worsened on oral corticosteroids but subsequently made substantial functional recovery on intravenous immunoglobulins. This case is different to those previously reported of sensory chronic inflammatory demyelinating polyradiculoneuropathy, given its exclusive sensory electrophysiologic presentation, presence of predominant upper limb reduced sensory amplitudes, and detection of sensory conduction blocks. These electrophysiologic features were of paramount importance in establishing diagnosis and effective therapy.

MRI abnormalities of peripheral nerve and muscle are common in amyotrophic lateral sclerosis and share features with multifocal motor neuropathy

PubMed Central

Staff, Nathan P.; Amrami, Kimberly K.; Howe, Benjamin M.

2015-01-01

Introduction MRI of peripheral nerve and muscle in patients with ALS may be performed to investigate alternative diagnoses including multifocal motor neuropathy (MMN). MRI findings of peripheral nerve and muscle are not well described in these conditions, making interpretation of results difficult. Methods We examined systematically the peripheral nerve and muscle MRI findings in patients with ALS (n=60) and MMN (n=8). Results In patients with ALS and MMN, abnormal MRIs were common (85% and 75%, respectively) but did not correlate with disease severity. Peripheral nerve MRI abnormalities were similar in frequency (ALS: 58% vs. MMN: 63%) with most changes being of mild-to-moderate severity. Muscle MRI changes were more common in ALS (57% vs. 33%), and no muscle atrophy was seen in patients with MMN. Discussion MRI abnormalities of peripheral nerve and muscle in ALS and MMN are common and share some features. PMID:25736373

Quantitative thermal sensory testing -- value of testing for both cold and warm sensation detection in evaluation of small fiber neuropathy.

PubMed

Shukla, Garima; Bhatia, Manvir; Behari, Madhuri

2005-10-01

Small fiber neuropathy is a common neurological disorder, often missed or ignored by physicians, since examination and routine nerve conduction studies are usually normal in this condition. Many methods including quantitative thermal sensory testing are currently being used for early detection of this condition, so as to enable timely investigation and treatment. This study was conducted to assess the yield of quantitative thermal sensory testing in diagnosis of small fiber neuropathy. We included patients presenting with history suggestive of positive and/or negative sensory symptoms, with normal examination findings, clinically suggestive of small fiber neuropathy, with normal or minimally abnormal routine nerve conduction studies. These patients were subjected to quantitative thermal sensory testing using a Medoc TSA-II Neurosensory analyser at two sites and for two modalities. QST data were compared with those in 120 normal healthy controls. Twenty-five patients (16 males, 9 females) with mean age 46.8+/-16.6 years (range: 21-75 years) were included in the study. The mean duration of symptoms was 1.6+/-1.6 years (range: 3 months-6 years). Eighteen patients (72%) had abnormal thresholds in at least one modality. Thermal thresholds were normal in 7 out of the 25 patients. This study demonstrates that quantitative thermal sensory testing is a fairly sensitive method for detection of small fiber neuropathy especially in patients with normal routine nerve conduction studies.

Peripheral neuropathy in Tangier disease: A literature review and assessment.

PubMed

Mercan, Metin; Yayla, Vildan; Altinay, Serdar; Seyhan, Serhat

2018-06-01

Tangier disease (TD) (OMIM#205400) is a rare cause of inherited metabolic neuropathies characterized by marked deficiency of high-density lipoproteins and accumulation of cholesterol esters in various tissue resulting from reverse cholesterol transport deficiency. We report a case of a patient with TD with multifocal demyelinating neuropathy with conduction block who presents with winging scapula, tongue, and asymmetric extremity weakness. We also present a review of all studies published from 1960 to 2017 regarding peripheral neuropathy in TD. Our search identified 54 patients with TD with peripheral neuropathy. Syringomyelia-like neuropathy subtype (52.4%) was more frequent than multifocal sensorial and motor neuropathy subtype (26.2%), focal neuropathy subtype (19.1%), and distal symmetric polyneuropathy subtype (2.4%). Splenomegaly was the most common (40.7%) clinical manifestation in these patients. The pattern of electrodiagnostic abnormalities are: (1) demyelinating abnormalities were more predominant in the upper extremities than in the lower extremities and (2) slowing of motor nerve conduction was more prominent in the intermediate segment than in distal nerve segments. The sural-sparing pattern was present in 34.6% and conduction block was present in 11.5% of the patients. Our literature review and our case showed the clinical spectrum of TD neuropathy is quite wide and that it should be considered in the differential diagnosis of non-uniform demyelinating neuropathies. © 2018 Peripheral Nerve Society.

Nerve Entrapment Syndromes at the Wrist and Elbow by Sonography.

PubMed

Klauser, Andrea S; Buzzegoli, Tommaso; Taljanovic, Mihra S; Strobl, Sylvia; Rauch, Stefan; Teh, James; Wanschitz, Julia; Löscher, Wolfgang; Martinoli, Carlo

2018-07-01

Nerve entrapment syndromes of the upper extremity are associated with structural abnormalities or by an intrinsic abnormality of the nerve. Nerve entrapment syndromes generally have a typical clinical presentation, and findings on physical examination and in conjunction with electrodiagnostic studies imaging is used to evaluate the cause, severity, and etiology of the entrapment. With the development of high-frequency linear array transducers (12-24 MHz), ultrasound (US) is incomparable in terms of spatial resolution to depict morphological aspects and changes in nerves. US can identify the abnormalities causing entrapment, such as fibrous bands, ganglia, anomalous muscles, and osseous deformities, with the advantage of dynamic assessment under active and passive examination. US is a unique diagnostic modality that allows superb visualization of both large and small peripheral terminal nerve branches of the upper extremity and enables the correct diagnosis of various nerve entrapment syndromes. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Assessing sudomotor impairment in patients with peripheral neuropathy: Comparison between electrochemical skin conductance and skin biopsy.

PubMed

Duchesne, Mathilde; Richard, Laurence; Vallat, Jean-Michel; Magy, Laurent

2018-04-27

Sudoscan provides a rapid assessment of sudomotor function based on the measurement of electrochemical skin conductance (ESC), which is thought to be proportional to small nerve fibres innervating the sweat glands. However, the relationship between ESC and small nerve fibre density on skin biopsy remains unclear. In a retrospective single-centre study, we compared ESC measurements with autonomic sweat gland nerve fibre density (SGNFD) and somatic intraepidermal nerve fibre density (IENFD) in patients with polyneuropathy. 63 patients were included (mean age: 60.6 ± 13.3 years). ESC was more strongly correlated with SGNFD (r = 0.49; p = 0.0005) than with IENFD (r = 0.42; p = 0.0005). Foot ESC was lower in patients with abnormal SGNFD (1.0 ± 0.3 µS/kg versus 0.7 ± 0.4 µS/kg; p = 0.0419) or abnormal IENFD (1.1 ± 0.3 µS/kg versus 0.8 ± 0.3 µS/kg; p = 0.0425). ESC measurement is a novel method of potential value for assessing sudomotor function. More studies are required to define its place beside ancient well-established techniques. The weak correlation of ESC with skin biopsy results suggests that mechanisms other than the loss of innervating fibres may be responsible for sweat gland dysfunction in polyneuropathies. Copyright © 2018. Published by Elsevier B.V.

The Expanded Bead Size of Corneal C-Nerve Fibers Visualized by Corneal Confocal Microscopy Is Associated with Slow Conduction Velocity of the Peripheral Nerves in Patients with Type 2 Diabetes Mellitus.

PubMed

Ishibashi, Fukashi; Kojima, Rie; Taniguchi, Miki; Kosaka, Aiko; Uetake, Harumi; Tavakoli, Mitra

2016-01-01

This study aims to establish the corneal nerve fiber (CNF) morphological alterations in a large cohort of type 2 diabetic patients and to investigate the association between the bead size, a novel parameter representing composite of accumulated mitochondria, glycogen particles, and vesicles in CNF, and the neurophysiological dysfunctions of the peripheral nerves. 162 type 2 diabetic patients and 45 healthy control subjects were studied in detail with a battery of clinical and neurological examinations and corneal confocal microscopy. Compared with controls, patients had abnormal CNF parameters. In particular the patients had reduced density and length of CNF and beading frequency and increased bead size. Alterations in CNF parameters were significant even in patients without neuropathy. The HbA1c levels were tightly associated with the bead size, which was inversely related to the motor and sensory nerve conduction velocity (NCV) and to the distal latency period of the median nerve positively. The CNF density and length positively correlated with the NCV and amplitude. The hyperglycemia-induced expansion of beads in CNF might be a predictor of slow NCV in peripheral nerves in type 2 diabetic patients.

A nationwide survey of combined central and peripheral demyelination in Japan.

PubMed

Ogata, Hidenori; Matsuse, Dai; Yamasaki, Ryo; Kawamura, Nobutoshi; Matsushita, Takuya; Yonekawa, Tomomi; Hirotani, Makoto; Murai, Hiroyuki; Kira, Jun-ichi

2016-01-01

To clarify the clinical features of combined central and peripheral demyelination (CCPD) via a nationwide survey. The following characteristics were used to define CCPD: T2 high-signal intensity lesions in the brain, optic nerves or spinal cord on MRI, or abnormalities on visual-evoked potentials; conduction delay, conduction block, temporal dispersion or F-wave abnormalities suggesting demyelinating neuropathy based on nerve conduction studies; exclusion of secondary demyelination. We conducted a nationwide survey in 2012, sending questionnaires to 1332 adult and paediatric neurology institutions in Japan. We collated 40 CCPD cases, including 29 women. Age at onset was 31.7±14.1 years (mean±SD). Sensory disturbance (94.9%), motor weakness (92.5%) and gait disturbance (79.5%) were common. Although cerebrospinal fluid protein levels were increased in 82.5%, oligoclonal IgG bands and elevated IgG indices were detected in 7.4% and 18.5% of cases, respectively. Fifteen of 21 patients (71.4%) had abnormal visual-evoked potentials. Antineurofascin 155 antibodies were positive in 5/11 (45.5%). Corticosteroids, intravenous immunoglobulins and plasmapheresis resulted in an 83.3%, 66.7% and 87.5% improvement, respectively, whereas interferon-β was effective in only 10% of cases. CCPD cases with simultaneous onset of central nervous system (CNS) and peripheral nervous system (PNS) involvement exhibited greater disability, but less recurrence and more frequent extensive cerebral and spinal cord MRI lesions compared to those with temporarily separated onset, whereas optic nerve involvement was more common in the latter. CCPD shows different characteristics from classical demyelinating diseases, and distinctive features exist between cases with simultaneous and temporarily separated onset of CNS and PNS involvement. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Neurological Assessment and Nerve Conduction Study Findings in 22 Patients with Alkaptonuria from Jordan

PubMed Central

Alrawashdeh, Omar; Alsbou, Mohammad; Alzoubi, Hamed; Al-shagahin, Hani

2017-01-01

Alkaptonuria is a rare metabolic disease characterised by accumulative deposition of homogentisic acid in the connective tissue of the body. This results in early degeneration of tendons, cartilages, heart valves, and other tissues. The main objective of the study is to examine the possibility of the nervous system involvement in patients with alkaptonuria The sample consists of two groups; 22 patients with AKU and 20 controls. A neurological assessment has been carried out including detailed medical history, neurological examination, and a nerve conduction study of the nerves of the dominant hand. The prevalence of any abnormality was compared between the two groups using chi square test. The mean values of the nerve conduction study were compared between the two groups using student t-test. There was a higher prevalence of low back pain, hearing problems and tinnitus, numbness and neuropathic pain in alkaptonuria patients. There was no significant difference between the two groups in other conditions such as seizures, headache, and syncope. The values of the nerve conduction study did not show significant difference between the two groups. Neurologically related symptoms in alkaptonuria mostly represent complications of the connective tissue degeneration rather than direct involvement of the nervous system. This has been supported further by the normal findings of the neurophysiology study in patients with alkaptonuria. PMID:28217270

Skin denervation and its clinical significance in late-stage chronic kidney disease.

PubMed

Chao, Chi-Chao; Wu, Vin-Cent; Tan, Chun-Hsiang; Wang, Yi-Mei; Tseng, Ming-Tsung; Wu, Pei-Chen; Lin, Yea-Huey; Lin, Whei-Min; Wu, Kwan-Dun; Hsieh, Sung-Tsang

2011-02-01

To investigate the skin innervation and its clinical significance in late-stage chronic kidney disease (CKD). Case series. National Taiwan University Hospital, Taipei, Taiwan. Forty consecutive nondiabetic patients with late-stage CKD (14 female and 26 male; mean [SD] age, 60.7 [12.3] years), including 2 cases with stage 3 CKD, 6 with stage 4 CKD, and 32 with stage 5 CKD, ie, end-stage kidney disease. Clinical evaluation of neurological deficits, nerve conduction study, autonomic function tests, and a 3-mm-diameter skin biopsy specimen taken from the distal leg. Quantitation of epidermal innervation, parameters of nerve conduction study, R-R interval variability, and sympathetic skin response. Clinically, 21 patients (52.5%) were symptomatic with paresthesia over the limbs or autonomic symptoms. The intraepidermal nerve fiber (IENF) density was markedly reduced in patients with CKD compared with age- and sex-matched controls (mean [SD], 2.8 [2.0] vs 8.6 [2.8] fibers/mm; P < .001). Skin denervation was observed in 27 patients (67.5%). Fifteen patients (37.5%) had abnormalities on nerve conduction studies, and 29 patients (72.5%) had abnormal results on autonomic function tests. By analysis with multiple regression models, the IENF density was negatively correlated with the duration of renal disease (P = .02). Additionally, the R-R interval variability at rest was linearly correlated with the IENF density (P = .02) and the absence of sympathetic skin responses at the soles was associated with reduced IENF density (P = .03). Small-fiber sensory and autonomic neuropathies constitute the major form of neuropathy in late-stage CKD. Furthermore, skin denervation was associated with the duration of renal disease.

Morphological abnormalities of embryonic cranial nerves after in utero exposure to valproic acid: implications for the pathogenesis of autism with multiple developmental anomalies.

PubMed

Tashiro, Yasura; Oyabu, Akiko; Imura, Yoshio; Uchida, Atsuko; Narita, Naoko; Narita, Masaaki

2011-06-01

Autism is often associated with multiple developmental anomalies including asymmetric facial palsy. In order to establish the etiology of autism with facial palsy, research into developmental abnormalities of the peripheral facial nerves is necessary. In the present study, to investigate the development of peripheral cranial nerves for use in an animal model of autism, rat embryos were treated with valproic acid (VPA) in utero and their cranial nerves were visualized by immunostaining. Treatment with VPA after embryonic day 9 had a significant effect on the peripheral fibers of several cranial nerves. Following VPA treatment, immunoreactivity within the trigeminal, facial, glossopharyngeal and vagus nerves was significantly reduced. Additionally, abnormal axonal pathways were observed in the peripheral facial nerves. Thus, the morphology of several cranial nerves, including the facial nerve, can be affected by prenatal VPA exposure as early as E13. Our findings indicate that disruption of early facial nerve development is involved in the etiology of asymmetric facial palsy, and may suggest a link to the etiology of autism. Copyright © 2011 ISDN. Published by Elsevier Ltd. All rights reserved.

Middle ear abnormalities in Van Maldergem syndrome.

PubMed

Verheij, Emmy; Thomeer, Henricus G X M; Pameijer, Frank A; Topsakal, Vedat

2017-01-01

Van Maldergem syndrome (VMS) is a very rare syndrome that was first described in 1992. The main features of this syndrome comprise intellectual disability, blepharo-naso-facial malformation, and hand anomalies. Almost all nine described patients have been shown to be affected by conductive hearing impairment attributed to microtia, and atresia of the outer ear canal. Here, we present a VMS patient with congenital malformations of the middle ear as the main reason for severe conductive bilateral hearing impairment. To our knowledge, this is the first report to describe middle ear abnormalities in VMS. These malformations were seen on high resolution Computed Tomography scanning and during an exploratory tympanotomy. Due to the severity of the middle ear abnormalities and the risk for facial nerve damage, the patient was not offered an ossicular chain reconstruction but a bone conduction device after this exploratory tympanotomy. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Conduction velocity and refractory period of single motor nerve fibres in antecedent poliomyelitis.

PubMed Central

Borg, K; Borg, J

1987-01-01

The axonal conduction velocity and the axonal refractory period were determined using electromyographic techniques for 60 single motor units in 11 patients with antecedent poliomyelitis. The results were compared with previous observations in healthy subjects as well as subjects with motor neuron disease. In antecedent poliomyelitis there was a reduced proportion of nerve fibres with low conduction velocity as compared with the findings in healthy subjects. The mean value of the axonal conduction velocities in antecedent poliomyelitis (42.4 +/- 3.7 m/s, M +/- SD) was significantly higher (p less than 0.01) while in motor neuron disease the corresponding value (35.2 +/- 7.8 m/s) was significantly lower (p less than 0.005) than in healthy subjects (39.8 +/- 4.9 m/s). In antecedent poliomyelitis the conduction velocity and refractory period were related as in healthy subjects while abnormally long refractory periods were observed in motor neuron disease. Images PMID:3585356

Anomalous Bilateral Communication between the Inferior Alveolar Nerve and the Auriculotemporal Nerve: A Rare Variation

PubMed Central

BHARDWAJ, Nikha; SAHNI, Priya; SINGHVI, Abhishek; NAYAK, Meghanand; TIWARI, Vineeta

2014-01-01

Branches of the posterior division of the mandibular nerve show various anomalous communications in the infratemporal region. Understanding such communication has relevance in the management of neuropathies and surgical procedures in this region. This study was conducted to explore such communicating branches, anticipating that they might provide information of clinical significance. A total of 15 human cadavers (30 infratemporal regions) were studied to explore such communicating branches in infratemporal region. The branches of the posterior division of the mandibular nerve were carefully dissected, and these branches were studied and analysed for any abnormal course. In one case, a rare type of bilateral communication between the auriculotemporal nerve and the inferior alveolar nerve, forming a loop with no association with any structure, was observed. It is possible that such communicating branches may be associated with delayed regression of the first arch vessels. The clinical implications of these anomalous communications require further detailed study for improved management of neuropathies and surgical procedures. PMID:25977637

High-frequency ultrasound as an adjunct to neural electrophysiology: Evaluation and prognosis of Bell's palsy.

PubMed

Li, Shuo; Guo, Rui-Jun; Liang, Xiao-Ning; Wu, Yue; Cao, Wen; Zhang, Zhen-Ping; Zhao, Wei; Liang, Hai-Dong

2016-01-01

Bell's palsy is a form of temporary facial nerve paralysis that occurs primarily in young adults. Previously, various methods were used to assess outcomes in facial nerve disease. The aim of the present study was to characterize the main branches of the normal and abnormal facial nerve using high-frequency ultrasonography (HFUS). A total of 104 healthy volunteers, 40 patients with acute onset of Bell's palsy and 30 patients who underwent 3-month routine therapy for Bell's palsy disease were included in the study. The healthy volunteers and patients were selected for HFUS examination and VII nerve conduction. The results showed significant differences in nerve diameter, echogenicity, delitescence and amplitude in different groups. Statistically significant correlations were identified for severity grading in one of the experimental groups during HFUS examinations. In conclusion, HFUS as a complementary technique paired with neural electrophysiology may establish the normal values of facial nerve. Additionally, HFUS was beneficial in the process of evaluation and prognosis of Bell's palsy disease.

CONGENITAL ABNORMALITIES OF CRANIAL NERVE DEVELOPMENT: OVERVIEW, MOLECULAR MECHANISMS, AND FURTHER EVIDENCE OF HETEROGENEITY AND COMPLEXITY OF SYNDROMES WITH CONGENITAL LIMITATION OF EYE MOVEMENTS

PubMed Central

Traboulsi, Elias I

2004-01-01

ABSTRACT Purpose The clinical and molecular genetic classification of syndromes with congenital limitation of eye movements and evidence of cranial nerve dysgenesis continues to evolve. This monograph details clinical and molecular genetic data on a number of families and isolated patients with congenital fibrosis of the extraocular muscles (CFEOM) and related disorders, and presents an overview of the mechanisms of abnormal patterns of motor and sensory cranial nerve development in these rare syndromes. Methods Clinical examination of one patient with CFEOM1, one family with clinical features of CFEOM2, one family with recessive CFEOM3, one family with horizontal gaze palsy and progressive scoliosis (HGPPS), and four patients with various combinations of congenital cranial nerve abnormalities. Genotyping of families with CFEOM and HGPPS for polymorphic markers in the regions of the three known CFEOM loci and in the HGPPS region, and mutation analysis of the ARIX and KIF21A genes in patients with CFEOM were performed according to standard published protocols. Results The patient with CFEOM1 had the second most common mutation in KIF21A, a 2861 G>A mutation that resulted in an R954Q substitution. The family with CFEOM2 phenotype did not map to the CFEOM2 locus. The family with recessive CFEOM3 did not map to any of the known loci. The HGPPS family mapped to 11q23–q25. One patient had optic nerve hypoplasia and fifth nerve dysfunction. Two patients had the rare combination of Möbius syndrome and CFEOM. One patient had Möbius syndrome and fifth nerve dysfunction. Conclusions There is genetic heterogeneity in CFEOM2 and CFEOM3. Abnormalities in sensory nerves can also accompany abnormalities of motor nerves, further substantiating the effect of individual mutations on developing motor as well as sensory cranial nerve nuclei. PMID:15747768

Non-recurrent laryngeal nerve with a coexisting contralateral nerve demonstrating extralaryngeal branching.

PubMed

Constable, James D; Bathala, Srinivasalu; Ahmed, Jacob J; McGlashan, Julian A

2017-03-17

Non-recurrence and extralaryngeal branching are 2 of the more frequently encountered anomalies of the recurrent laryngeal nerve. If not anticipated intraoperatively, these abnormalities can put the nerve at risk, with subsequent vocal cord palsy. It is therefore important to report on and understand these abnormalities. We present a unique case of a non-recurrent laryngeal nerve with a coexisting contralateral nerve demonstrating extralaryngeal branching. This case allows us to demonstrate the importance of arteria lusoria in head and neck surgery, and to conclude that non-recurrence and extralaryngeal branching can occur separately within individual nerves in the same patient. The case also highlights the importance of a systematic intraoperative approach to the identification of every recurrent laryngeal nerve, especially in bilateral procedures having already exposed an anomalous nerve on one side. 2017 BMJ Publishing Group Ltd.

BMI, HOMA-IR, and Fasting Blood Glucose Are Significant Predictors of Peripheral Nerve Dysfunction in Adult Overweight and Obese Nondiabetic Nepalese Individuals: A Study from Central Nepal.

PubMed

Thapa, Lekhjung; Rana, P V S

2016-01-01

Objective. Nondiabetic obese individuals have subclinical involvement of peripheral nerves. We report the factors predicting peripheral nerve function in overweight and obese nondiabetic Nepalese individuals. Methodology. In this cross-sectional study, we included 50 adult overweight and obese nondiabetic volunteers without features of peripheral neuropathy and 50 healthy volunteers to determine the normative nerve conduction data. In cases of abnormal function, the study population was classified on the basis of the number of nerves involved, namely, "<2" or "≥2." Multivariable logistic regression analysis was carried out to predict outcomes. Results. Fasting blood glucose (FBG) was the significant predictor of motor nerve dysfunction (P = 0.039, 95% confidence interval (CI) = 1.003-1.127). Homeostatic model assessment of insulin resistance (HOMA-IR) was the significant predictor (P = 0.019, 96% CI = 1.420-49.322) of sensory nerve dysfunction. Body mass index (BMI) was the significant predictor (P = 0.034, 95% CI = 1.018-1.577) in case of ≥2 mixed nerves' involvement. Conclusion. FBG, HOMA-IR, and BMI were significant predictors of peripheral nerve dysfunction in overweight and obese Nepalese individuals.

Long-term neuromuscular outcomes of west nile virus infection: A clinical and electromyographic evaluation of patients with a history of infection.

PubMed

Athar, Parveen; Hasbun, Rodrigo; Nolan, Melissa S; Salazar, Lucrecia; Woods, Steven P; Sheikh, Kazim; Murray, Kristy O

2018-01-01

Neuromuscular clinical manifestations during acute West Nile virus (WNV) infection are well documented; however, long-term neurologic outcomes still require investigation. We conducted a long-term follow-up study in patients with history of WNV infection. Of the 117 patients who participated in neurologic and neurocognitive evaluations, 30 were referred for neuromuscular and electrodiagnostic evaluation based on abnormal findings. We found that 33% of these patients (10 of 30) showed abnormalities on nerve conduction and/or needle electromyography due to primary or secondary outcomes of WNV infection. Most common electrodiagnostic findings and causes of long-term disability were related to anterior horn cell poliomyelitis (WNV poliomyelitis). Electrical data on these patient populations were similar to those observed in chronic poliomyelitis. With more than 16,000 cases of WNV neuroinvasive disease reported across the USA since 1999, understanding clinical outcomes from infection will provide a resource for physicians managing long-term care of these patients. Muscle Nerve 57: 77-82, 2018. © 2017 Wiley Periodicals, Inc.

Facial nerve mapping and monitoring in lymphatic malformation surgery.

PubMed

Chiara, Jospeh; Kinney, Greg; Slimp, Jefferson; Lee, Gi Soo; Oliaei, Sepehr; Perkins, Jonathan A

2009-10-01

Establish the efficacy of preoperative facial nerve mapping and continuous intraoperative EMG monitoring in protecting the facial nerve during resection of cervicofacial lymphatic malformations. Retrospective study in which patients were clinically followed for at least 6 months postoperatively, and long-term outcome was evaluated. Patient demographics, lesion characteristics (i.e., size, stage, location) were recorded. Operative notes revealed surgical techniques, findings, and complications. Preoperative, short-/long-term postoperative facial nerve function was standardized using the House-Brackmann Classification. Mapping was done prior to incision by percutaneously stimulating the facial nerve and its branches and recording the motor responses. Intraoperative monitoring and mapping were accomplished using a four-channel, free-running EMG. Neurophysiologists continuously monitored EMG responses and blindly analyzed intraoperative findings and final EMG interpretations for abnormalities. Seven patients collectively underwent 8 lymphatic malformation surgeries. Median age was 30 months (2-105 months). Lymphatic malformation diagnosis was recorded in 6/8 surgeries. Facial nerve function was House-Brackmann grade I in 8/8 cases preoperatively. Facial nerve was abnormally elongated in 1/8 cases. EMG monitoring recorded abnormal activity in 4/8 cases--two suggesting facial nerve irritation, and two with possible facial nerve damage. Transient or long-term facial nerve paresis occurred in 1/8 cases (House-Brackmann grade II). Preoperative facial nerve mapping combined with continuous intraoperative EMG and mapping is a successful method of identifying the facial nerve course and protecting it from injury during resection of cervicofacial lymphatic malformations involving the facial nerve.

Less common upper limb mononeuropathies.

PubMed

Williams, Faren H; Kumiga, Bryan

2013-05-01

This article will focus on the less commonly injured nerves of the upper extremity. These nerves may be involved when trauma results in fractures, dislocations, or swelling with resultant nerve compression. Tumors and ganglions can also compress nerves, causing pain and, over time, demyelination or axon degeneration with weakness. Other mechanisms for upper limb nerve injury include participation in high-level sports, that is, those that generate torque about the arm and shoulder, abnormal stresses about the joints and muscles, or muscle hypertrophy, which may result in nerve injury. The goals of this review are to discuss the clinical presentation and possible causes of upper extremity nerve entrapments and to formulate an electrodiagnostic plan for evaluation. Descriptions of the appropriate nerve conduction studies or needle electromyographic protocols are included for specific nerves. The purpose of the electrodiagnostic examination is to evaluate the degree of nerve injury, axon loss over time, and later, evidence for reinnervation to assist with prognostication. The latter has implications for management of the neuropathy, including the type of exercises and therapy that may be indicated to help maintain the stability and motion of the involved joint(s) and promote strengthening over time as the nerve regenerates. Copyright © 2013 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.

Role of blink reflex in diagnosis of subclinical cranial neuropathy in diabetic mellitus type II.

PubMed

Kazem, Shakouri S; Behzad, Davoudi

2006-05-01

Peripheral neuropathy (PN) is one of the late complications of diabetes mellitus. Cranial nerves III, VII, and V are among the most commonly affected in diabetic patients. Traditional electrodiagnosis (Edx) studies are a useful method for diagnosis of PN and symptomatic cranial neuropathy, and may not be useful for detecting subclinical involvement of cranial nerves. The main objective of this study is to evaluate the role of blink reflex (BR) for early diagnosis of cranial neuropathy in diabetic patients with PN. A prospective study was performed on NIDDM patients with PN. One hundred eighty-eight subjects were included in our study in which 142 acted as healthy subjects and 46 as diabetic patients. Patients were excluded with prior history of cranial nerve lesions, stroke, or any other disease with polyneuropathy or drug-induced neuropathy. Routine nerve conduction studies were performed, and only patients with PN were included in this study. Abnormalities were found in 54.4% of patients. R1, IR2, and CR2 were prolonged relative to the healthy group. Statistically there was no significant difference in R/D ratio of patients (P=0.201). Also, there was a positive correlation between R1, IR2, and CR2 latencies with duration of diabetes and severity of polyneuropathy, but not for R/D. The greatest correlation was shown in R1 latency (69.9% abnormality). BR is a noninvasive and very useful method for the evaluation and diagnosis of subclinical cranial nerve involvement in diabetic patients.

Role of Blink Reflex in diagnosis of subclinical cranial neuropathy in Diabetic Mellitus type II.

PubMed

Kazem, S S; Behzad, D

2005-01-01

Peripheral Neuropathy (PN) is one of the late complications of Diabetes Mellitus. Cranial nerves III, VII and V are among the most commonly affected in diabetic patients. Traditional Electrodiagnosis (Edx) studies are useful method for diagnosis of PN and symptomatic cranial neuropathy, and may not be useful for detecting subclinical involvement of cranial nerves. The main objective of this study is to evaluate the role of Blink Reflex (BR) for early diagnosis of cranial neuropathy in diabetic patients with PN. A prospective study was performed on NIDDM patients with a PN. 188 subjects were included in our study in which 142 acted as healthy subjects and 46 as diabetic patients. Patients were excluded with prior history of cranial nerve lesions, stroke, and other disease with polyneuropathy or drug-induced neuropathy. Routine nerve conduction studies were performed and only patients with PN were included in this study. Abnormalities were found in 54.4% of patients. R1, IR2 and CR2 were prolonged relative to healthy group. Statistically there was no significant difference in R/D ratio of patients (P = 0.201). Also there was a positive correlation between R1, IR2 and CR2 latencies with duration of diabetes and severity of polyneuropathy, but not for R/D. The greatest correlation was shown in R1 latency (69.9% abnormality). BR is a non-invasive and very useful method for evaluation and diagnosis of subclinical cranial nerve involvement in diabetic patients.

Nerve and muscle involvement in mitochondrial disorders: an electrophysiological study.

PubMed

Mancuso, Michelangelo; Piazza, Selina; Volpi, Leda; Orsucci, Daniele; Calsolaro, Valeria; Caldarazzo Ienco, Elena; Carlesi, Cecilia; Rocchi, Anna; Petrozzi, Lucia; Calabrese, Rosanna; Siciliano, Gabriele

2012-04-01

Involvement of the peripheral nervous system in mitochondrial disorders (MD) has been previously reported. However, the exact prevalence of peripheral neuropathy and/or myopathy in MD is still unclear. In order to evaluate the prevalence of neuropathy and myopathy in MD, we performed sensory and motor nerve conduction studies (NCS) and concentric needle electromyography (EMG) in 44 unselected MD patients. NCS were abnormal in 36.4% of cases, and were consistent with a sensori-motor axonal multineuropathy (multifocal neuropathy), mainly affecting the lower limbs. EMG evidence of myopathy was present in 54.5% of patients, again mainly affecting the lower limbs. Nerve and muscle involvement was frequently subclinical. Peripheral nerve and muscle involvement is common in MD patients. Our study supports the variability of the clinical expression of MD. Further studies are needed to better understand the molecular basis underlying the phenotypic variability among MD patients.

Repeater F-waves are signs of motor unit pathology in polio survivors.

PubMed

Hachisuka, Akiko; Komori, Tetsuo; Abe, Tatsuya; Hachisuka, Kenji

2015-05-01

The purpose of this study was to determine whether F-waves reveal electrophysiological features of anterior horn cells in polio survivors. Forty-three polio survivors and 20 healthy controls underwent motor nerve conduction studies of the median and tibial nerves bilaterally, including sampling of F-waves elicited by 100 stimuli and the determination of motor unit number estimation (MUNE). A significant increase in abnormally stereotyped ("repeater") F-waves and a reduction of F-wave persistence were observed in both nerves in the polio group as compared with the control group. Repeater F-waves had a negative correlation with MUNE. These trends in F-wave persistence and repeater F-waves after motor unit loss are characteristic findings in polio survivors. Repeater F-waves are a sign of motor unit pathology. © 2014 The Authors. Muscle & Nerve Published by Wiley Periodicals, Inc.

Electrophysiological abnormalities associated with extensive myelinated retinal nerve fibers.

PubMed

Tay, Su Ann; Sanjay, Srinivasan

2012-07-01

An observational case report of electrophysiological abnormalities in a patient with anisomyopic amblyopia as a result of unilateral extensive myelinated retinal nerve fibers (MNFs) is illustrated. The electrophysiological readings revealed an abnormal pattern electroretinogram (PERG) but normal full-field electroretinogram readings in the affected eye. The visual-evoked potential was also undetectable in that eye. Our findings suggest that extensive MNFs can be associated with electrophysiological abnormalities, in particular the PERG, which can aid in diagnosing the cause of impaired vision when associated with amblyopia.

Variations in the branching pattern of posterior division of mandibular nerve: a case report.

PubMed

Muraleedharan, Aparna; Veeramani, Raveendranath; Chand, Parkash

2014-11-01

Abnormal communications among the branches of mandibular nerve especially the posterior division are significant due to various procedures undertaken in this region. These variations are worth reporting as they pose serious implications in several interventions in this region, and may even lead to false diagnosis. During routine dissection, the mandibular nerve and its branches were dissected in the infratemporal fossa. The branches from the posterior division of the mandibular nerve namely the inferior alveolar and auriculotemporal nerves were carefully dissected, and their abnormal branching pattern was noted. There was a communicating branch between left inferior alveolar and auriculotemporal nerve. There was also a variant recurrent branch from the left inferior alveolar nerve that supplied the lateral pterygoid muscle. Such variant branches and communications between the branches of mandibular nerve as seen in this case have an embryological basis and are clinically important in this region especially for dental surgeries and anesthesia.

Extraneural findings during peripheral nerve ultrasound: Prevalence and further assessment.

PubMed

Bignotti, Bianca; Zaottini, Federico; Airaldi, Sonia; Martinoli, Carlo; Tagliafico, Alberto

2018-01-01

In this study we evaluated the frequency and further assessment of extraneural findings encountered during peripheral nerve ultrasound (US). Our retrospective review identified 278 peripheral nerve US examinations of 229 patients performed between December 2014 and December 2015. Reports were reviewed to assess the number of studies without peripheral nerve abnormalities and the frequency and further assessment of extraneural findings. A total of 107 peripheral nerve US examinations of 90 patients (49 men and 41 women, mean age 55 ± 16 years) did not report peripheral nerve abnormalities. Extraneural findings were observed in 24 of 107 (22.4%) studies. Fifteen of the 278 [5.4% (95% confidence interval 2.7%-8.1%)] studies led to a recommendation for additional imaging or clinical evaluation of an extraneural finding. At least 5.4% (15 of 278) of peripheral nerve US studies led to additional clinical or imaging assessment. Muscle Nerve 57: 65-69, 2018. © 2017 Wiley Periodicals, Inc.

Axonal neuropathy in female carriers of the fragile X premutation with fragile x-associated tremor ataxia syndrome.

PubMed

Ram, Suresh; Devapriya, Inoka A; Fenton, Grace; Mcvay, Lindsey; Nguyen, Danh V; Tassone, Flora; Maselli, Ricardo A; Hagerman, Randi J

2015-08-01

In this study we examined whether females with the fragile X-associated tremor ataxia syndrome (FXTAS) and non-FXTAS premutation carriers have electrophysiological signs of underlying peripheral neuropathy. Nerve conduction studies (NCS) were performed on 19 women with FXTAS, 20 non-FXTAS carriers, and 26 age-matched controls. The results were compared with existing data on corresponding male carriers. Women with FXTAS and non-FXTAS carriers had reduced sensory nerve action potential amplitudes. Also, there was a strong trend for reduced compound muscle action potential amplitudes in women with FXTAS, but not in non-FXTAS carriers. No significant slowing of nerve conduction velocities, prolongation of F-wave latencies, or associations with molecular measures was observed. This study suggests an underlying axonal neuropathy in women with FXTAS. However, in comparison to men with FXTAS, the NCS abnormalities in women were less severe, possibly due to the effect of a normal X chromosome. © 2014 Wiley Periodicals, Inc.

Physical Examination Has a Low Yield in Screening for Carpal Tunnel Syndrome

PubMed Central

Dale, Ann Marie; Descatha, Alexis; Coomes, Justin; Franzblau, Alfred; Evanoff, Bradley

2013-01-01

Background Physical examination is often used to screen workers for carpal tunnel syndrome (CTS). In a population of newly-hired workers, we evaluated the yield of such screening. Methods Our study population included 1108 newly-hired workers in diverse industries. Baseline data included a symptom questionnaire, physical exam, and bilateral nerve conduction testing of the median and ulnar nerves; individual results were not shared with the employer. We tested three outcomes: symptoms of CTS, abnormal median nerve conduction, and a case definition of CTS that required both symptoms and median neuropathy. Results Of the exam measures used, only Semmes-Weinstein sensory testing had a sensitivity value above 31%. Positive predictive values were low, and likelihood ratios were all under 5.0 for positive testing and over 0.2 for negative testing. Conclusion Physical examination maneuvers have a low yield for the diagnosis of CTS in workplace surveillance programs and in post-offer, pre-placement screening programs. PMID:21154516

A study of axonal degeneration in the optic nerves of aging mice

NASA Technical Reports Server (NTRS)

Johnson, J. E., Jr.; Philpott, D. E.; Miquel, J.

1978-01-01

The optic nerves of C57BL/6J mice ranging from 3 to 30 months were examined by electron microscopy. At all ages investigated, optic nerve axons contained enlarged mitochondria with abnormal cristae. With increasing age, a large number of necrotic axons were observed and were in the process of being phagocytized. The abnormal mitochondria may represent preliminary changes that eventually lead to necrosis of the axon.

[Clinical and electrophysiological findings in carpal tunnel syndrome].

PubMed

Kohara, Nobuo

2007-11-01

Carpal tunnel syndrome (CTS) is the most common nerve entrapment disorder. The clinical features of CTS are variable, but usually include pain and paresthesia in the thumb, first two fingers, and the radial-half of the ring finger. Paresthesia and sensory deficits might involve the entire palm area in some cases. Pain frequently radiate proximally into the forearm, and occasionally to the shoulder. Many patients experience pain at night and are awakened by abnormal sensations. Shaking hand relief the symptom. The two classic tests for nerve compression at the wrist are the Tinel test and the Phalen maneuver, which diagnostic value is limited. Golden standard for the diagnosis is the combination of the clinical findings and the electrophysiological study. Routine median nerve conduction study is valuable. Prolonged terminal latency of motor or sensory nerve would be found in most CTS hands. If the routine study showed equivocal, more sensitive methods are needed. Those include segmental sensory conduction study across the carpal tunnel by median stimulation at midpalm, a comparison of median and ulnar sensory nerve latencies at ring finger and a comparison of median and radial sensory nerve latencies at thumb. A difference between the median motor latency to the second lumbrical and the ulnar motor latency to the interossei muscles has also diagnostic value in some cases. In addition, inching method can localized the compression site. Using these techniques, the diagnosis of CTS would become more reliable.

Longitudinal studies of time-dependent changes in both bladder and erectile function after streptozotocin-induced diabetes in Fischer 344 male rats.

PubMed

Melman, Arnold; Zotova, Elena; Kim, Mimi; Arezzo, Joseph; Davies, Kelvin; DiSanto, Michael; Tar, Moses

2009-11-01

To provide sensitive physiological endpoints for the onset and long-term progression of deficits induced by diabetes mellitus (DM) in bladder and erectile function in male rats, and to evaluate parallel changes in urogenital and nerve function induced by hyperglycaemia over a protracted period as a model for chronic deficits in patients with diabetes. The study comprised in 877 male, 3-month-old, Fischer 344 rats; 666 were injected intraperitoneally with 35 mg/kg streptozotocin (STZ) and divided into insulin-treated and untreated diabetic groups. The rats were studied over 8 months and measurements made of both erectile and bladder function, as well as nerve conduction studies over the duration of the study. There was an early (first month) abnormality of both erectile and bladder function that persisted through the 8 months of the study. The erectile dysfunction was manifest as reduced intracavernous pressure/blood pressure ratio, and the bladder dysfunction as a persistent increase in detrusor overactivity with no detrusor decompensation. Insulin treatment prevented or modified the abnormality in each organ. Hyperglycaemia caused a progressive decrease in caudal nerve conduction velocity. The mean digital sensory and tibial motor nerve conduction velocity did not deteriorate over time. Correlation measurements of nerve and organ function were not consistent. The results of this extensive long-term study show early and profound effects of hyperglycaemia on the smooth muscle of the penis and bladder, that were persistent and stable in surviving rats over the 8 months. The physiological changes did not correlate well with neurological measurements of those organs. Significantly, diverse smooth-muscle cellular and subcellular events antedated the measured neurological manifestations of the hyperglycaemia by several months. Although autonomic diabetic neuropathy is a primary life-threatening complication of long-term diabetes in humans, this rat model of STZ-induced diabetes showed that the rapid onset of physiological manifestations was based on many molecular changes in the smooth muscle cells in this model of type 1 DM.

Retrospective study of a TTR FAP cohort to modify NIS+7 for therapeutic trials.

PubMed

Suanprasert, N; Berk, J L; Benson, M D; Dyck, P J B; Klein, C J; Gollob, J A; Bettencourt, B R; Karsten, V; Dyck, P J

2014-09-15

Protein stabilization and oligonucleotide therapies are being tested in transthyretin amyloid polyneuropathy (TTR FAP) trials. From retrospective analysis of 97 untreated TTR FAP patients, we test the adequacy of Neuropathy Impairment Score+7 tests (NIS+7) and modifications to comprehensively score impairments for use in such therapeutic trials. Our data confirms that TTR FAP usually is a sensorimotor polyneuropathy with autonomic features which usually is symmetric, length dependent, lower limb predominant and progressive. NIS+7 adequately assesses weakness and muscle stretch reflexes without ceiling effects but not sensation loss, autonomic dysfunction or nerve conduction abnormalities. Three modifications of NIS+7 are suggested: 1) use of Smart Somatotopic Quantitative Sensation Testing (S ST QSTing); 2) choice of new autonomic assessments, e.g., sudomotor testing of distributed anatomical sites; and 3) use of only compound muscle action potential amplitudes (of ulnar, peroneal and tibial nerves) and sensory nerve action potentials of ulnar and sural nerve - than the previously recommended attributes suggested for the sensitive detection of diabetic sensorimotor polyneuropathy. These modifications of NIS+7 if used in therapeutic trials should improve characterization and quantification of sensation and autonomic impairment in TTR FAP and provide better nerve conduction tests. Copyright © 2014 Elsevier B.V. All rights reserved.

Congenital sensory neuropathy

PubMed Central

Barry, J. E.; Hopkins, I. J.; Neal, B. W.

1974-01-01

Two infants with sporadic congenital sensory neuropathy are described. The criteria of generalized lack of superficial sensory appreciation, hypotonia, areflexia, together with histological evidence of abnormalities of sensory neural structures in skin and peripheral nerves have been met. No abnormality of motor or autonomic nerves was shown. ImagesFIG. PMID:4131674

[Features of peripheral nerve injuries in workers exposed to vibration: an analysis of 197 cases].

PubMed

Situ, J; Lin, C M; Qin, Z H; Zhu, D X; Lin, H; Zhang, F F; Zhang, J J

2016-12-20

Objective: To investigate the features of peripheral nerve injuries in workers exposed to vibration. Methods: A total of 197 male workers [median age: 34 years (21 - 50 years) ; median working years of vibration exposure: 7.3 years (1 - 20 years) ] engaged in grinding in an enterprise were enrolled. Their clinical data and electromyography results were analyzed to investigate the features of peripheral nerve impairment. Results: Of all workers, 96 (48.73%) had abnormal electromyography results. Of all workers, 88 (44.7%) had simple mild median nerve injury in the wrist, who accounted for 91.7% (88/96) of all workers with abnormal electromy-ography results. Six workers had ulnar nerve injury, superficial radial nerve injury, or/and superficial peroneal nerve injury and accounted for 6.3% of all workers with abnormal electromyography results. Of all workers, 88 had a reduced amplitude of median nerve sensory transduction, and 28 had slowed median nerve sensory transduction. A total of 46 workers were diagnosed with occupational hand-arm vibration disease and hospitalized for treatment. They were followed up for more than 4 months after leaving their jobs, and most of them showed improvements in neural electromyography results and returned to a normal state. Conclusion: Workers exposed to vibration have a high incidence rate of nerve injury in the hand, mainly sensory function impairment at the distal end of the median nerve, and all injuries are mild peripheral nerve injuries. After leaving the vibration job and being treated, most workers can achieve improvements and return to a normal state.

Beneficial effects of gamma linolenic acid supplementation on nerve conduction velocity, Na+, K+ ATPase activity, and membrane fatty acid composition in sciatic nerve of diabetic rats.

PubMed

Coste, T; Pierlovisi, M; Leonardi, J; Dufayet, D; Gerbi, A; Lafont, H; Vague, P; Raccah, D

1999-07-01

Metabolic and vascular abnormalities are implicated in the pathogenesis of diabetic neuropathy. Two principal metabolic defects are altered lipid metabolism resulting from the impairment of delta-6-desaturase, which converts linoleic acid (LA) into gamma linolenic acid (GLA), and reduced nerve Na+, K+ ATPase activity. This reduction may be caused by a lack of incorporation of (n-6) fatty acids in membrane phospholipids. Because this ubiquitous enzyme maintains the membrane electrical potential and allows repolarization, disturbances in its activity can alter the process of nerve conduction velocity (NCV). We studied the effects of supplementation with GLA (260 mg per day) on NCV, fatty acid phospholipid composition, and Na+, K+ ATPase activity in streptozotocin-diabetic rats. Six groups of 10 rats were studied. Two groups served as controls supplemented with GLA or sunflower oil (GLA free). Two groups with different durations of diabetes were studied: 6 weeks with no supplementation and 12 weeks supplemented with sunflower oil. To test the ability of GLA to prevent or reverse the effects of diabetes, two groups of diabetic rats were supplemented with GLA, one group for 12 weeks and one group for 6 weeks, starting 6 weeks after diabetes induction. Diabetes resulted in a 25% decrease in NCV (P < 0.0001), a 45% decrease in Na+, K+ ATPase activity (P < 0.0001), and an abnormal phospholipid fatty acid composition. GLA restored NCV both in the prevention and reversal studies and partially restored Na+, K+ ATPase activity in the preventive treatment group (P < 0.0001). These effects were accompanied by a modification of phospholipid fatty acid composition in nerve membranes. Overall, the results suggest that membrane fatty acid composition plays a direct role in NCV and confirm the beneficial effect of GLA supplementation in diabetic neuropathy.

Brainstem abnormalities and vestibular nerve enhancement in acute neuroborreliosis.

PubMed

Farshad-Amacker, Nadja A; Scheffel, Hans; Frauenfelder, Thomas; Alkadhi, Hatem

2013-12-21

Borreliosis is a widely distributed disease. Neuroborreliosis may present with unspecific symptoms and signs and often remains difficult to diagnose in patients with central nervous system symptoms, particularly if the pathognomonic erythema chronica migrans does not develop or is missed. Thus, vigilance is mandatory in cases with atypical presentation of the disease and with potentially severe consequences if not recognized early. We present a patient with neuroborreliosis demonstrating brain stem and vestibular nerve abnormalities on magnetic resonance imaging. A 28-year-old Caucasian female presented with headaches, neck stiffness, weight loss, nausea, tremor, and gait disturbance. Magnetic resonance imaging showed T2-weighted hyperintense signal alterations in the pons and in the vestibular nerves as well as bilateral post-contrast enhancement of the vestibular nerves. Serologic testing of the cerebrospinal fluid revealed the diagnosis of neuroborreliosis. Patients infected with neuroborreliosis may present with unspecific neurologic symptoms and magnetic resonance imaging as a noninvasive imaging tool showing signal abnormalities in the brain stem and nerve root enhancement may help in establishing the diagnosis.

Early electrophysiological findings in Fisher-Bickerstaff syndrome.

PubMed

Alberti, M A; Povedano, M; Montero, J; Casasnovas, C

2017-09-06

The term Fisher-Bickerstaff syndrome (FBS) has been proposed to describe the clinical spectrum encompassing Miller-Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis. The pathophysiology of FBS and the nature of the underlying neuropathy (demyelinating or axonal) are still subject to debate. This study describes the main findings of an early neurophysiological study on 12 patients diagnosed with FBS. Retrospective evaluation of clinical characteristics and electrophysiological findings of 12 patients with FBS seen in our neurology department within 10 days of disease onset. Follow-up electrophysiological studies were also evaluated, where available. The most frequent electrophysiological finding, present in 5 (42%) patients, was reduced sensory nerve action potential (SNAP) amplitude in one or more nerves. Abnormalities were rarely found in motor neurography, with no signs of demyelination. The cranial nerve exam revealed abnormalities in 3 patients (facial neurography and/or blink reflex test). Three patients showed resolution of SNAP amplitude reduction in serial neurophysiological studies, suggesting the presence of reversible sensory nerve conduction block. Results from cranial MRI scans were normal in all patients. An electrophysiological pattern of sensory axonal neuropathy, with no associated signs of demyelination, is an early finding of FBS. Early neurophysiological evaluation and follow-up are essential for diagnosing patients with FBS. Copyright © 2017 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

Alterations of Na,K-ATPase isoenzymes in the rat diabetic neuropathy: protective effect of dietary supplementation with n-3 fatty acids.

PubMed

Gerbi, A; Maixent, J M; Barbey, O; Jamme, I; Pierlovisi, M; Coste, T; Pieroni, G; Nouvelot, A; Vague, P; Raccah, D

1998-08-01

Diabetic neuropathy is a degenerative complication of diabetes accompanied by an alteration of nerve conduction velocity (NCV) and Na,K-ATPase activity. The present study in rats was designed first to measure diabetes-induced abnormalities in Na,K-ATPase activity, isoenzyme expression, fatty acid content in sciatic nerve membranes, and NCV and second to assess the preventive ability of a fish oil-rich diet (rich in n-3 fatty acids) on these abnormalities. Diabetes was induced by intravenous streptozotocin injection. Diabetic animals (D) and nondiabetic control animals (C) were fed the standard rat chow either without supplementation or supplemented with either fish oil (DM, CM) or olive oil (DO, CO) at a daily dose of 0.5 g/kg by gavage during 8 weeks. Analysis of the fatty acid composition of purified sciatic nerve membranes from diabetic animals showed a decreased incorporation of C16:1(n-7) fatty acids and arachidonic acids. Fish oil supplementation changed the fatty acid content of sciatic nerve membranes, decreasing C18:2(n-6) fatty acids and preventing the decreases of arachidonic acids and C18:1(n-9) fatty acids. Protein expression of Na,K-ATPase alpha subunits, Na,K-ATPase activity, and ouabain affinity were assayed in purified sciatic nerve membranes from CO, DO, and DM. Na,K-ATPase activity was significantly lower in sciatic nerve membranes of diabetic rats and significantly restored in diabetic animals that received fish oil supplementation. Diabetes induced a specific decrease of alpha1- and alpha3-isoform activity and protein expression in sciatic nerve membranes. Fish oil supplementation restored partial activity and expression to varying degrees depending on the isoenzyme. These effects were associated with a significant beneficial effect on NCV. This study indicates that fish oil has beneficial effects on diabetes-induced alterations in sciatic nerve Na,K-ATPase activity and function.

Lewis-Sumner syndrome and Tangier disease.

PubMed

Théaudin, Marie; Couvert, Philippe; Fournier, Emmanuel; Bouige, Daniel; Bruckert, Eric; Perrotte, Paul; Vaschalde, Yvan; Maisonobe, Thierry; Bonnefont-Rousselot, Dominique; Carrié, Alain; Le Forestier, Nadine

2008-07-01

To report unusual electrophysiologic data in a patient with Tangier disease in an effort to better understand the pathophysiologic features of the peripheral nerve lesions in this disease. Case report. A 15-year-old girl had subacute onset of asymmetric neuropathy with persistent conduction block, resembling Lewis-Sumner syndrome. Electrophysiologic data in Tangier disease. After initially unsuccessful treatment with intravenously administered immunoglobulins, the finding of an abnormal lipid profile led to the diagnosis of Tangier disease due to the R587W mutation in the adenotriphosphate-binding cassette transporter-1 gene (ABCA1) (OMIM 9q22-q31). Conduction block, which is the electrophysiologic hallmark of focal demyelination, can be present in Tangier disease. It could be induced by focal nerve ischemia or by preferential lipid deposition in the paranodal regions of myelinated Schwann cells. The presence of a conduction block in Tangier disease may lead to a misdiagnosis of dysimmune neuropathy.

What is the Best Strategy on Detection of Cornea Neuropathy in People with Diabetes? Recent Advances in Potential Measurements.

PubMed

Lv, Ying; Zhao, Shaozhen

2018-03-26

There are well-acknowledged clinical or pre-clinical measurements concerning diabetic peripheral neuropathy(DPN). The current gold standard for diagnosis of diabetic peripheral neuropathy is nerve conduction suitable for detecting large nerve fiber function[1] and intraepidermal nerve fiber density assessment for small fiber damage evaluation[2]. The lack of a sensitive, non-invasive, and repeatable endpoint to measure changes in small nerve fibers is a major factor holding back clinical trials for the treatment of diabetic peripheral neuropathy. As cornea is the most densely innerved tissue, assessing corneal nerves' structure and function will be promising to predict and assess the degree of DPN [3]. In the diabetic micro-environment, damaged corneal nerves lead to decreased corneal sensitivity, both of which resulting in abnormal tear function. According to this theory, the measurements of nerve structure, corneal sensitivity, tear secretion and tear components, to some extent, can reveal and assess the state of corneal neuropathy. This review focuses on summarizing the knowledge of the latest detective methods of diabetic corneal neuropathy, popular in use or possible to further in study and be applied into clinical practice. Copyright © 2018 Elsevier B.V. All rights reserved.

Electrophysiological abnormalities associated with extensive myelinated retinal nerve fibers

PubMed Central

Tay, Su Ann; Sanjay, Srinivasan

2012-01-01

An observational case report of electrophysiological abnormalities in a patient with anisomyopic amblyopia as a result of unilateral extensive myelinated retinal nerve fibers (MNFs) is illustrated. The electrophysiological readings revealed an abnormal pattern electroretinogram (PERG) but normal full-field electroretinogram readings in the affected eye. The visual-evoked potential was also undetectable in that eye. Our findings suggest that extensive MNFs can be associated with electrophysiological abnormalities, in particular the PERG, which can aid in diagnosing the cause of impaired vision when associated with amblyopia. PMID:22824610

High-resolution imaging of retinal nerve fiber bundles in glaucoma using adaptive optics scanning laser ophthalmoscopy.

PubMed

Takayama, Kohei; Ooto, Sotaro; Hangai, Masanori; Ueda-Arakawa, Naoko; Yoshida, Sachiko; Akagi, Tadamichi; Ikeda, Hanako Ohashi; Nonaka, Atsushi; Hanebuchi, Masaaki; Inoue, Takashi; Yoshimura, Nagahisa

2013-05-01

To detect pathologic changes in retinal nerve fiber bundles in glaucomatous eyes seen on images obtained by adaptive optics (AO) scanning laser ophthalmoscopy (AO SLO). Prospective cross-sectional study. Twenty-eight eyes of 28 patients with open-angle glaucoma and 21 normal eyes of 21 volunteer subjects underwent a full ophthalmologic examination, visual field testing using a Humphrey Field Analyzer, fundus photography, red-free SLO imaging, spectral-domain optical coherence tomography, and imaging with an original prototype AO SLO system. The AO SLO images showed many hyperreflective bundles suggesting nerve fiber bundles. In glaucomatous eyes, the nerve fiber bundles were narrower than in normal eyes, and the nerve fiber layer thickness was correlated with the nerve fiber bundle widths on AO SLO (P < .001). In the nerve fiber layer defect area on fundus photography, the nerve fiber bundles on AO SLO were narrower compared with those in normal eyes (P < .001). At 60 degrees on the inferior temporal side of the optic disc, the nerve fiber bundle width was significantly lower, even in areas without nerve fiber layer defect, in eyes with glaucomatous eyes compared with normal eyes (P = .026). The mean deviations of each cluster in visual field testing were correlated with the corresponding nerve fiber bundle widths (P = .017). AO SLO images showed reduced nerve fiber bundle widths both in clinically normal and abnormal areas of glaucomatous eyes, and these abnormalities were associated with visual field defects, suggesting that AO SLO may be useful for detecting early nerve fiber bundle abnormalities associated with loss of visual function. Copyright © 2013 Elsevier Inc. All rights reserved.

Peripheral neuropathy in patients with myotonic dystrophy type 2.

PubMed

Leonardis, L

2017-05-01

Myotonic dystrophy type 2 (dystrophia myotonica type 2-DM2) is an autosomal dominant multi-organ disorder. The involvement of the peripheral nervous system was found in 25%-45% of patients with myotonic dystrophy type 1, although limited data are available concerning polyneuropathy in patients with DM2, which was the aim of this study with a thorough presentation of the cases with peripheral neuropathy. Patients with genetically confirmed DM2 underwent motor nerve conduction studies of the median, ulnar, tibial and fibular nerves and sensory nerve conduction studies of the median (second finger), ulnar (fifth finger), radial (forearm) and sural nerves. Seventeen adult patients with DM2 participated in the study. Fifty-three percent (9/17) of our patients had abnormality of one or more attributes (latency, amplitude or conduction velocity) in two or more separate nerves. Four types of neuropathies were found: (i) predominantly axonal motor and sensory polyneuropathy, (ii) motor polyneuropathy, (iii) predominantly demyelinating motor and sensory polyneuropathy and (iv) mutilating polyneuropathy with ulcers. The most common forms are axonal motor and sensory polyneuropathy (29%) and motor neuropathy (18% of all examined patients). No correlations were found between the presence of neuropathy and age, CCTG repeats, blood glucose or HbA1C. Peripheral neuropathy is common in patients with DM2 and presents one of the multisystemic manifestations of DM2. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

The Characteristics of Chronic Inflammatory Demyelinating Polyneuropathy in Patients with and without Diabetes – An Observational Study

PubMed Central

Dunnigan, Samantha K.; Ebadi, Hamid; Breiner, Ari; Katzberg, Hans D.; Barnett, Carolina; Perkins, Bruce A.; Bril, Vera

2014-01-01

Introduction We aimed to determine whether the clinical characteristics and electrodiagnostic classification of nerve injury, and response to treatment differed in patients diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) with and without diabetes. Methods CIDP patients with diabetes (CIDP+DM) (n = 67) and without diabetes (CIDP-DM) (n = 67) underwent clinical examination and nerve conduction studies (NCS). CIDP-DM patients were selected using age and gender matching with the existing CIDP+DM cohort. Patients treated with immunotherapies were classified as responders (R) (n = 46) or non-responders (NR) (n = 54) based on clinical response to treatment. The groups were compared using analysis of variance, contingency tables and Kruskal-Wallis analyses. Results CIDP+DM subjects had more severe neuropathy based on higher lower limb vibration potential thresholds (VPT)(p = 0.004), higher Toronto Clinical Neuropathy Score (TCNS) (p = 0.0009), more proximal weakness (p = 0.03), more gait abnormality (p = 0.03) and more abnormal NCS. CIDP+DM subjects had more abnormal sural NCS with lower sural sensory nerve action potential amplitudes (2.4±3.0 µV, 6.6±6.0 µV, p<0.0001) and slower sural nerve conduction velocities (38.6±5.4 m/s, 41.0±5.3 m/s, p = 0.04). CIDP-DM subjects were more likely to receive immune therapies (93% vs 57%, p = <0.0001), despite no significant differences in treatment responder rates (p = 0.71). Patients who responded to therapy had shorter duration of CIDP than non-responders (8.0±6.0 y vs 11.9±7.6 y, p = 0.004). Discussion The clinical phenotype and electrophysiological profile of CIDP patients differs according to the presence or absence of diabetes. Despite CIDP+DM patients having more severe clinical and electrophysiological neuropathy, they are less likely to receive disease-modifying/specific therapy, yet have similar response rates to treatment as those without diabetes. Specifically, the duration of neuropathy - not diabetes status - was associated with treatment response. PMID:24586703

[F-waves in brachial plexus palsy correlated to the prognosis of intrinsic paralysis].

PubMed

Nobuta, S

1995-04-01

F-waves were examined in 80 nerves of 40 brachial plexus palsies in 37 cases. The electrical responses were evoked by 30 consecutive supramaximal electric stimuli to the median and ulnar nerves at the wrist and elbow, and recorded from the abductor pollicis brevis and abductor digiti minimi muscles. Three parameters in the F-waves were analyzed--conduction velocity, the difference between the maximal and minimal latencies, and the amplitude. In all cases, examinations were done repeatedly to detect changes in these parameters, and the results were compared with the clinical course of the intrinsic muscle function. Twenty-seven cases were investigated before and after explorative surgery. The findings were divided into four groups. The 1st group consisted of 12 nerves in which F-waves were not recorded. The intrinsic muscle power in this group was zero, and did not show any restoration. The 2nd group consisted of 10 nerves in which the conduction velocity was delayed. The muscle power in this group was fair, poor or trace, and there was no change in conduction velocity and muscle function. The 3rd group consisted of 18 nerves in which parameters other than the conduction velocity were abnormal, and the intrinsic muscle power in this group was fair, good or normal. In 7 of these nerves, the large latency difference decreased to normal at the 2nd, 3rd or 4th test with functional recovery in the intrinsic muscle. The high amplitude also changed to normal at the 2nd test with functional recovery. The 4th group consisted of 40 nerves in which all the parameters were normal and had full intrinsic muscle power. In conclusion, an examination of the F-waves was valuable to indicate the prognosis of the intrinsic muscle in the hand in brachial plexus palsy.

Assessing Decreased Sensation and Increased Sensory Phenomena in Diabetic Polyneuropathies

PubMed Central

Herrmann, David N.; Staff, Nathan P.; Dyck, P. James B.

2013-01-01

Loss of sensation and increased sensory phenomena are major expressions of varieties of diabetic polyneuropathies needing improved assessments for clinical and research purposes. We provide a neurobiological explanation for the apparent paradox between decreased sensation and increased sensory phenomena. Strongly endorsed is the use of the 10-g monofilaments for screening of feet to detect sensation loss, with the goal of improving diabetic management and prevention of foot ulcers and neurogenic arthropathy. We describe improved methods to assess for the kind, severity, and distribution of both large- and small-fiber sensory loss and which approaches and techniques may be useful for conducting therapeutic trials. The abnormality of attributes of nerve conduction may be used to validate the dysfunction of large sensory fibers. The abnormality of epidermal nerve fibers/1 mm may be used as a surrogate measure of small-fiber sensory loss but appear not to correlate closely with severity of pain. Increased sensory phenomena are recognized by the characteristic words patients use to describe them and by the severity and persistence of these symptoms. Tests of tactile and thermal hyperalgesia are additional markers of neural hyperactivity that are useful for diagnosis and disease management. PMID:24158999

Brain stem and inner ear abnormalities in children with auditory neuropathy spectrum disorder and cochlear nerve deficiency.

PubMed

Huang, B Y; Roche, J P; Buchman, C A; Castillo, M

2010-11-01

Cranial abnormalities, including CND, are common in children with ANSD. The purpose of this study was to assess whether CND is associated with brain or inner ear abnormalities in a cohort of children with ANSD. Two neuroradiologists retrospectively reviewed cranial MR imaging examinations in 103 children with ANSD. Brain, cochlear nerve, and temporal bone abnormalities were described and tabulated. Findings were stratified on the basis of the presence and laterality of CND, and differences in the presence of associated inner ear or intracranial abnormalities were assessed by using 2-tailed Fisher exact tests. CND was identified in 33.0% of children and 26.9% of ears with ANSD. Significantly more patients with bilateral CND had intracranial abnormalities than those with unilateral CND (60.0% versus 15.8%; P = .012). Forty percent of patients with bilateral CND, 0% of patients with unilateral CND, and 10.1% of those without CND demonstrated hindbrain malformations. Patients with bilateral CND were more likely to demonstrate hindbrain malformations than patients with normal nerves (P = .01) or unilateral CND (P = .004). Labyrinthine abnormalities were significantly more common in patients with bilateral CND than in those without CND (P ≤ .001). Cochlear anomalies were more common in patients with bilateral versus unilateral CND (P = .01). IAC and cochlear aperture stenosis were more common in those with unilateral and bilateral CND than those without CND (both P < .001). Cochlear and hindbrain abnormalities are significantly more common among patients with ANSD with bilateral CND compared with those with at least 1 intact cochlear nerve.

The vagus and recurrent laryngeal nerves in the rodent experimental model of esophageal atresia.

PubMed

Qi, B Q; Merei, J; Farmer, P; Hasthorpe, S; Myers, N A; Beasley, S W; Hutson, J M

1997-11-01

After surgical correction of their esophageal atresia and tracheoesophageal fistula (EA-TEF), many patients exhibit evidence of esophageal dysmotility. Controversy exists as to whether the esophageal motility disorders result from denervation caused by surgery or from an inherent abnormal innervation of the esophagus. The present study used an Adriamycin-induced EA-TEF fetal rat model to trace the course and branching of both the vagus and recurrent laryngeal nerves. Abnormalities observed in EA-TEF rat fetuses include: (1) fewer branches from both recurrent laryngeal nerves; (2) deviation of the left vagus from its normal course below the aorta, passing behind the fistula to approach and join with the right vagus to form a single nerve trunk on the right side of the esophagus; (3) relatively few branches from the single vagal nerve trunk (composed of fibers of the left and the right vagus) on the surface of the lower esophagus. Fetuses affected by EA-TEF have inherent abnormalities in the course and branching pattern of the vagus nerves as they descend through the thorax, culminating in a deficient extrinsic nerve fiber plexus in the lower esophagus. These observations may account for the esophageal motility disorders seen in patients who have EA-TEF even before surgical intervention.

Motor root conduction block in the Lewis-Sumner syndrome.

PubMed

Lo, Yew Long; Dan, Yang-Fang; Tan, Yam-Eng; Leoh, Teng-Hee

2011-03-01

The Lewis-Sumner syndrome (LSS) is a rare immune-mediated peripheral nerve disorder presenting with asymmetric upper limb sensory complaints and motor weakness. Asian patients with LSS have not been reported in the English literature. Three Asian patients with features of LSS were prospectively studied. Our patients tended to older, female, and have involvement of the upper limbs exclusively than those in the West. They have a markedly longer disease duration before a diagnosis was made, which could also be the result of difficulty in eliciting motor root conduction block as a sign of proximal demyelination as observed in every patient. Pain is a universal feature as is sensory nerve conduction abnormality. None responded to immunotherapy, but disease stabilization was observed over the chronic course. Although rare, these unique observations in Asian patients with LSS differ from those reported in Western literature. The presence of motor root conduction block demonstrated for the first time is instrumental in establishing a diagnosis.

Abnormal flexor carpi radialis H-reflex as a specific indicator of C7 as compared with C6 radiculopathy.

PubMed

Zheng, Chaojun; Zhu, Yu; Lv, Feizhou; Ma, Xiaosheng; Xia, Xinlei; Wang, Lixun; Jin, Xiang; Weber, Robert; Jiang, Jianyuan; Anuvat, Kevin

2014-12-01

The H-reflex of the flexor carpi radialis (FCR H-reflex) has not been commonly used for the diagnosis of cervical radiculopathy when compared with the routinely tested soleus H-reflex. Although both S1 and S2 roots innervate the soleus, the H-reflex is selectively related to S1 nerve root function clinically. Flexor carpi radialis is also innervated by two nerve roots which are C6 and C7. Although they are among the most common roots involved in cervical radiculopathy, few studies reported if the attenuation of the FCR H-reflex is caused by lesions affecting C7 or C6 nerve roots, or both. We aimed to identify whether an abnormal FCR H-reflex was attributed to the C7 or C6 nerve root lesion, or both. The sensitivities of needle electromyography, FCR H-reflex, and provocative tests in unilateral C7 or C6 radiculopathy were also compared in this study. A concentric needle electrode recorded bilateral FCR H-reflexes in 41 normal subjects (control group), 51 patients with C7 radiculopathy, and 54 patients with C6 radiculopathy. Clinical, radiological, and surgical approaches identified the precise single cervical nerve root involved in all patient groups. The H-reflex and M-wave latencies were measured and compared bilaterally. Abnormal FCR H-reflex was defined as the absence of the H-reflex or a side-to-side difference over 1.5 milliseconds which was based on the normal side-to-side difference of the H-reflex latency of 16.9 milliseconds (SD = 1.7 milliseconds) from the control group. We also determined standard median and ulnar conduction and needle electromyography. The provocative tests included bilateral determination of the Shoulder Abduction and Spurling's tests in all radiculopathy group patients. Abnormal FCR H-reflexes were recorded in 45 (88.2%) of C7 radiculopathy group patients, and 2 (3.7%) of C6 radiculopathy group patients (P < 0.05). Needle electromyography was abnormal in 41 (80.4%) of C7 radiculopathy patients and 43 (79.6%) of C6 radiculopathy patients. Provocative tests were positive in 15 (29.4%) of C7 radiculopathy patients and 25 (46.3%) of C6 radiculopathy patients. Flexor carpi radialis H-Reflex provides a sensitive assessment of evaluating the C7 spinal reflex pathway. Clinically, a combination of the FCR H-reflex with needle electromyography may yield the highest level of diagnostic information for evaluating clinical cases of C7 radiculopathy.

Multifocal visual evoked potentials reveal normal optic nerve projections in human carriers of oculocutaneous albinism type 1a.

PubMed

Hoffmann, Michael B; Wolynski, Barbara; Meltendorf, Synke; Behrens-Baumann, Wolfgang; Käsmann-Kellner, Barbara

2008-06-01

In albinism, part of the temporal retina projects abnormally to the contralateral hemisphere. A residual misprojection is also evident in feline carriers that are heterozygous for tyrosinase-related albinism. This study was conducted to test whether such residual abnormalities can also be identified in human carriers of oculocutaneous tyrosinase-related albinism (OCA1a). In eight carriers heterozygous for OCA1a and in eight age- and sex-matched control subjects, monocular pattern-reversal and -onset multifocal visual evoked potentials (mfVEPs) were recorded at 60 locations comprising a visual field of 44 degrees diameter (VERIS 5.01; EDI, San Mateo, CA). For each eye and each stimulus location, interhemispheric difference potentials were calculated and correlated with each other, to assess the lateralization of the responses: positive and negative correlations indicate lateralizations on the same or opposite hemispheres, respectively. Misrouted optic nerves are expected to yield negative interocular correlations. The analysis also allowed for the assessment of the sensitivity and specificity of the detection of projection abnormalities. No significant differences were obtained for the distributions of the interocular correlation coefficients of controls and carriers. Consequently, no local representation abnormalities were observed in the group of OCA1a carriers. For pattern-reversal and -onset stimulation, an assessment of the control data yielded similar specificity (97.9% and 94.6%) and sensitivity (74.4% and 74.8%) estimates for the detection of projection abnormalities. The absence of evidence for projection abnormalities in human OCA1a carriers contrasts with the previously reported evidence for abnormalities in cat-carriers of tyrosinase-related albinism. This discrepancy suggests that animal models of albinism may not provide a match to human albinism.

Lipid-lowering drugs (statins) and peripheral neuropathy.

PubMed

Emad, Mohammadreza; Arjmand, Hosein; Farpour, Hamid Reza; Kardeh, Bahareh

2018-03-01

Peripheral neuropathy is a disorder with often unknown causes. Some drugs, including statins, are proposed to be among the causes of peripheral neuropathy. This study aimed at evaluating this condition by electrodiagnostic study among patients who had received statins. This case-control study was conducted in Shiraz, Iran in 2015, and included 39 patients aged 35-55 who had received statins for at least 6 months, and 39 healthy matched controls. Using electrodiagnosis, the sensory and motor wave features (amplitude, latency and nerve conduction velocity) of the peripheral nerves (Median, Ulnar, Tibial, Sural, and Peroneal) were evaluated among the subjects. Data were analyzed using SPSS software and p<0.05 was considered statistically significant. Regarding the occurrence of neuropathy, there were no significant differences in any of the definitions presented for peripheral neuropathy. However, the difference was close to significance for one definition [2 abnormalities in 2 nerves (p=0.055)]. Regarding mean values of the features, significant differences were observed in two features: amplitude of the peroneal motor nerve (p=0.048) and amplitude of the sural sensory nerve (p=0.036). Since statins are widely used, awareness regarding their side-effects would lead to better treatment. Even though no significant differences were found between the groups regarding the occurrence of peripheral neuropathy, there were significant differences in amplitudes of the sural sensory response and the peroneal motor response. This indicates the involvement of peripheral nerves. Therefore, we recommend that patients and physicians should be informed about the possible symptoms of this condition.

Inhibition of development of peripheral neuropathy in streptozotocin-induced diabetic rats with N-acetylcysteine.

PubMed

Sagara, M; Satoh, J; Wada, R; Yagihashi, S; Takahashi, K; Fukuzawa, M; Muto, G; Muto, Y; Toyota, T

1996-03-01

N-acetylcysteine (NAC) is a precursor of glutathione (GSH) synthesis, a free radical scavenger and an inhibitor of tumour necrosis factor alpha (TNF). Because these functions might be beneficial in diabetic complications, in this study we examined whether NAC inhibits peripheral neuropathy. Motor nerve conduction velocity (MNCV) was significantly decreased in streptozotocin-induced-diabetic Wistar rats compared to control rats. Oral administration of NAC reduced the decline of MNCV in diabetic rats. Structural analysis of the sural nerve disclosed significant reduction of fibres undergoing myelin wrinkling and inhibition of myelinated fibre atrophy in NAC-treated diabetic rats. NAC treatment had no effect on blood glucose levels or on the nerve glucose, sorbitol and cAMP contents, whereas it corrected the decreased GSH levels in erythrocytes, the increased lipid peroxide levels in plasma and the increased lipopolysaccharide-induced TNF activity in sera of diabetic rats. Thus, NAC inhibited the development of functional and structural abnormalities of the peripheral nerve in streptozotocin-induced diabetic rats.

Vestibular-evoked myogenic potential in patients with unilateral vestibular neuritis: abnormal VEMP and its recovery.

PubMed

Ochi, Kentaro; Ohashi, Toru; Watanabe, Shoji

2003-02-01

The incidence of inferior vestibular nerve disorders in patients suffering from unilateral vestibular neuritis and the recovery of these disorders were evaluated by monitoring the vestibular-evoked myogenic potential (VEMP). Eight patients ranged from 21 to 73 years that suffered from unilateral vestibular neuritis underwent VEMP and caloric testing. Abnormal VEMP was observed in two of the eight patients with unilateral vestibular neuritis. Two patients were diagnosed as having an inferior vestibular nerve disorder. One of these patients showed recovery of the inferior vestibular nerve function as assessed by the VEMP. Disorders of the inferior vestibular nerve function and their recovery was confirmed by our current results. The time course of recoveries of the superior and inferior vestibular nerve systems were similar in the two patients.

Focal neurological deficits

MedlinePlus

... include: Abnormal blood vessels (vascular malformation) Brain tumor Cerebral palsy Degenerative nerve illness (such as multiple sclerosis) Disorders of a single nerve or nerve group (for example, carpal tunnel syndrome ) Infection of the brain (such as meningitis or encephalitis) Injury Stroke Home ...

Congenital malformations of the inner ear and the vestibulocochlear nerve in children with sensorineural hearing loss: evaluation with CT and MRI.

PubMed

Westerhof, J P; Rademaker, J; Weber, B P; Becker, H

2001-01-01

The purpose of this work was to study the diagnostic value of CT and MRI in children with sensorineural hearing loss and to analyze anatomic abnormalities of the inner ear and the vestibulocochlear nerve in this patient group. We evaluated 42 inner ears in 21 children with congenital deafness who had congenital inner ear malformations and who were candidates for cochlear implants. All patients were studied with high resolution MR and helical CT examinations. The MR study included a T2-weighted 3D fast SE sequence. We describe and tabulate the anatomic abnormalities. Special attention was given to abnormalities of the vestibulocochlear nerve. The field of view in the plane according to the length axis of the internal auditory canal (IAC) was 4 cm. Additional continuous parasagittal reformations perpendicular to the length axis of the IAC were studied with a field of view of 3 cm. CT and MRI allowed accurate identification of malformations of the inner ear in children with congenital deafness. We identified 99 malformations, with a majority of patients demonstrating multiple abnormalities. Common imaging findings were Mondini abnormality and Mondini variants (12/42) and fusion of the lateral or superior semicircular canal with the vestibule (12/42). MRI demonstrated in 9 of 21 patients a rudimentary or absent vestibulocochlear nerve in the auditory canal. CT and MRI are important modalities to analyze the inner ear in children who are candidates for cochlear implants. MRI with an extremely small field of view should be used to study possible abnormalities of the vestibulocochlear nerves. This may alter clinical care and allow cochlear implant placement in patients whose electrodiagnostic studies suggest that the implant should not be performed. The detailed analysis of abnormalities of the inner ear might establish prognostic factors.

The impact of extended electrodiagnostic studies in Ulnar Neuropathy at the elbow

PubMed Central

Todnem, Kari; Michler, Ralf Peter; Wader, Tony Eugen; Engstrøm, Morten; Sand, Trond

2009-01-01

Background This study aimed to explore the value of extended motor nerve conduction studies in patients with ulnar nerve entrapment at the elbow (UNE) in order to find the most sensitive and least time-consuming method. We wanted to evaluate the utility of examining both the sensory branch from the fifth finger and the dorsal branch of the ulnar nerve. Further we intended to study the clinical symptoms and findings, and a possible correlation between the neurophysiological findings and pain. Methods The study was prospective, and 127 UNE patients who were selected consecutively from the list of patients, had a clinical and electrodiagnostic examination. Data from the most symptomatic arm were analysed and compared to the department's reference limits. Student's t - test, chi-square tests and multiple regression models were used. Two-side p-values < 0.05 were considered as significant. Results Ulnar paresthesias (96%) were more common than pain (60%). Reduced ulnar sensitivity (86%) and muscle strength (48%) were the most common clinical findings. Adding a third stimulation site in the elbow mid-sulcus for motor conduction velocity (MCV) to abductor digiti minimi (ADM) increased the electrodiagnostic sensitivity from 80% to 96%. Additional recording of ulnar MCV to the first dorsal interosseus muscle (FDI) increased the sensitivity from 96% to 98%. The ulnar fifth finger and dorsal branch sensory studies were abnormal in 39% and 30% of patients, respectively. Abnormal electromyography in FDI was found in 49% of the patients. Patients with and without pain had generally similar conduction velocity parameter means. Conclusion We recommend three stimulation sites at the elbow for MCV to ADM. Recording from FDI is not routinely indicated. Sensory studies and electromyography do not contribute much to the sensitivity of the electrodiagnostic evaluation, but they are useful to document axonal degeneration. Most conduction parameters are unrelated to the presence of pain. PMID:19814833

Cardiac Abnormalities in Primary Hyperoxaluria

PubMed Central

Mookadam, Farouk; Smith, Travis; Jiamsripong, Panupong; Moustafa, Sherif E; Monico, Carla G.; Lieske, John C.; Milliner, Dawn S.

2018-01-01

Background In patients with primary hyperoxaluria (PH), oxalate overproduction can result in recurrent urolithiasis and nephrocalcinosis, which in some cases results in a progressive decline in renal function, oxalate retention, and systemic oxalosis involving bone, retina, arterial media, peripheral nerves, skin, and heart. Oxalosis involving the myocardium or conduction system can potentially lead to heart failure and fatal arrhythmias. Methods and Results A retrospective review of our institution’s database was conducted for all patients with a confirmed diagnosis of PH between 1/1948 and 1/2006 (n=103). Electrocardiogram (ECG) and echocardiography were used to identify cardiac abnormalities. Ninety-three patients fulfilled the inclusion criteria, 58% were male. Mean follow-up was 11.9 (median 8.8) years. In 38 patients who received an ECG or echocardiography, 31 were found to have any cardiac abnormalities. Cardiac findings correlated with decline in renal function. Conclusions Our data suggests that physicians caring for patients with PH should pay close attention to cardiac status, especially if renal function is impaired. PMID:20921818

Ametropia, retinal anatomy, and OCT abnormality patterns in glaucoma. 2. Impacts of optic nerve head parameters

NASA Astrophysics Data System (ADS)

Baniasadi, Neda; Wang, Mengyu; Wang, Hui; Jin, Qingying; Elze, Tobias

2017-12-01

Clinicians use retinal nerve fiber layer thickness (RNFLT) measured by optical coherence tomography (OCT) as an adjunct to glaucoma diagnosis. Ametropia is accompanied by changes to the optic nerve head (ONH), which may affect how OCT machines mark RNFLT measurements as abnormal. These changes in abnormality patterns may bias glaucoma diagnosis. Here, we investigate the relationship between OCT abnormality patterns and the following ONH-related and ametropia-associated parameters on 421 eyes of glaucoma patients: optic disc tilt and torsion, central retinal vessel trunk location (CRVTL), and nasal and temporal retinal curvature adjacent to ONH, quantified as nasal/temporal slopes of the inner limiting membrane. We applied multivariate logistic regression with abnormality marks as regressands to 40,401 locations of the peripapillary region and generated spatial maps of locations of false positive/negative abnormality marks independent of glaucoma severity. Effects of torsion and temporal slope were negligible. The effect of tilt could be explained by covariation with ametropia. For CRVTL/nasal slope, abnormality pattern shifts at 7.2%/23.5% of the peripapillary region were detected, respectively, independent of glaucoma severity and ametropia. Therefore, CRVTL and nasal curvature should be included in OCT RNFLT norms. Our spatial location maps may aid clinicians to improve diagnostic accuracy.

[Magnetic resonance imaging features in two Chinese family with congenital fibrosis of extraocular muscles].

PubMed

Wu, Li; Zhou, Lian-Hong; Liu, Chang-Sheng; Cha, Yun-Fei; Wang, Jiong; Xing, Yi-Qiao

2009-11-01

The aim of this article was to investigate the structural basis of ocular motility and visual abnormalities in humans with congenital fibrosis of the extraocular muscles (CFEOM). 17 volunteers from 2 CFEOM pedigrees Clinical ophthalmic and motility examed and 18 normal control subjects were correlated with thin-sectioned magnetic resonance imaging (MRI) across the orbit and the brain-stem level. Subjects with CFEOM had severe bilateral blepharoptosis, limited supraduction, and variable ophthalmoplegia. In affected subjects, MRI demonstrated atrophy of the levator palpebrae superioris, all EOMs, and the optic nerves, and small or absent orbital motor nerves. The oculomotor nerve was most severely hypoplastic, but the abducens was also affected. Subjects with CFEOM exhibited subclinical but highly significant reduction from normal in mean optic nerve size (P < 0.05). There are also some difference between the two CFEOM pedigrees. These findings suggest that neuronal disease is primary in CFEOM, with myopathy arising secondary to abnormal innervation and the oculomotor nucleus and trochlear nucleus of the abnormalities defects.

Viral neurotropism, peripheral neuropathy and other morphological abnormalities in bovine ephemeral fever virus-infected downer cattle.

PubMed

Barigye, R; Davis, S; Hunt, R; Hunt, N; Walsh, S; Elliott, N; Burnup, C; Aumann, S; Day, C; Dyrting, K; Weir, R; Melville, L F

2016-10-01

This study assessed the neurotropism of bovine ephemeral fever (BEF) virus (BEFV) and described histomorphological abnormalities of the brain, spinal cord and peripheral nerves that may causally contribute to paresis or paralysis in BEF. Four paralysed and six asymptomatic but virus-infected cattle were monitored, and blood and serum samples screened by qRT-PCR, virus isolation and neutralisation tests. Fresh brain, spinal cord, peripheral nerve and other tissues were qRT-PCR-tested for viral RNA, while formalin-fixed specimens were processed routinely and immunohistochemically evaluated for histomorphological abnormalities and viral antigen distribution, respectively. The neurotropism of BEFV was immunohistochemically confirmed in the brain and peripheral nerves and peripheral neuropathy was demonstrated in three paralysed but not the six aneurological but virus-infected animals. Wallerian degeneration (WD) was present in the ventral funicular white matter of the lumbar spinal cord of a paralysed steer and in cervical and thoracic spinal cord segments of three paralysed animals. Although no spinal cord lesions were seen in the steer euthanased within 7 days of illness, peripheral neuropathy was present and more severe in nerves of the brachial plexuses than in the gluteal or fibular nerves. The only steer with WD in the lumbar spinal cord also showed intrahistiocytic cell viral antigen that was spatially distributed within areas of moderate brain stem encephalitis. The data confirmed neurotropism of BEFV in cattle and documented histomorphological abnormalities in peripheral nerves and brain which, together with spinal cord lesions, may contribute to chronic paralysis in BEFV-infected downer cattle. © 2016 Australian Veterinary Association.

Wernicke-Korsakoff syndrome

MedlinePlus

... cause leg tremor Vision changes such as abnormal eye movements (back and forth movements called nystagmus), double vision , ... may show damage to many nerve systems: Abnormal eye movement Decreased or abnormal reflexes Fast pulse (heart rate) ...

Chronic inflammatory demyelinating polyneuropathy associated with primary biliary cirrhosis.

PubMed

Murata, Ken-ya; Ishiguchi, Hiroshi; Ando, Ryuki; Miwa, Hideto; Kondo, Tomoyoshi

2013-12-01

We report a patient with chronic inflammatory demyelinating polyneuropathy associated with primary biliary cirrhosis (PBC). Except for minimal biochemical abnormalities, clinical symptoms of PBC were not observed, and we diagnosed our patient with asymptomatic PBC from the results of a liver biopsy. Although the patient noticed little muscle weakness, an electrophysiological study demonstrated slow conduction velocities and prolonged distal latencies, with definite conduction blocks in the median, ulnar, and tibial nerves. The disturbed sensory pattern was asymmetrical, and sensory nerve action potentials were not evoked. From these observations, we diagnosed this patient with chronic inflammatory demyelinating polyneuropathy. Neuropathy associated with PBC is very rare. We must differentiate demyelinating neuropathy with PBC in patients with asymmetrical sensory dominant neuropathy with high immunoglobulin M titers, and investigate for the presence of anti-mitochondrial antibodies to rule out a complication of asymptomatic PBC. Copyright © 2013 Elsevier Ltd. All rights reserved.

Optic nerve head component responses of the multifocal electroretinogram in MS.

PubMed

Frohman, Teresa C; Beh, Shin Chien; Saidha, Shiv; Schnurman, Zane; Conger, Darrel; Conger, Amy; Ratchford, John N; Lopez, Carmen; Galetta, Steven L; Calabresi, Peter A; Balcer, Laura J; Green, Ari J; Frohman, Elliot M

2013-08-06

To employ a novel stimulation paradigm in order to elicit multifocal electroretinography (mfERG)-induced optic nerve head component (ONHC) responses, believed to be contingent upon the transformation in electrical transmission properties of retinal ganglion cell axons from membrane to saltatory conduction mechanisms, as they traverse the lamina cribrosa and obtain oligodendrocyte myelin. We further sought to characterize abnormalities in ONHC responses in eyes from patients with multiple sclerosis (MS). In 10 normal subjects and 7 patients with MS (including eyes with and without a history of acute optic neuritis), we utilized a novel mfERG stimulation paradigm that included interleaved global flashes in order to elicit the ONHC responses from 103 retinal patches of pattern-reversal stimulation. The number of abnormal or absent ONHC responses was significantly increased in MS patient eyes compared to normal subject eyes (p < 0.001, by general estimating equation modeling, and accounting for age and within-subject, intereye correlations). Studying the relationship between ONHC abnormalities and alterations in validated structural and functional measures of the visual system may facilitate the ability to dissect and characterize the pathobiological mechanisms that contribute to tissue damage in MS, and may have utility to detect and monitor neuroprotective or restorative effects of novel therapies.

Long- and Short-Term Health Effects of Pesticide Exposure: A Cohort Study from China

PubMed Central

Li, Yifan; Zhang, Chao; Yin, Yanhong; Chen, Zhaohui; Jin, Yanhong; Cai, Jinyang; Cui, Fang

2015-01-01

Pesticides are extensively used by farmers in China. However, the effects of pesticides on farmers’ health have not yet been systematically studied. This study evaluated the effects of pesticides exposure on hematological and neurological indicators over 3 years and 10 days respectively. A cohort of 246 farmers was randomly selected from 3 provinces (Guangdong, Jiangxi, and Hebei) in China. Two rounds of health investigations, including blood tests and neurological examinations, were conducted by medical doctors before and after the crop season in 2012. The data on pesticide use in 2009–2011 were collected retrospectively via face-to-face interviews and the 2012 data were collected from personal records maintained by participants prospectively. Ordinary least square (OLS), Probit, and fixed effect models were used to evaluate the relationship between pesticides exposure frequency and the health indicators. Long-term pesticide exposure was found to be associated with increased abnormality of nerve conductions, especially in sensory nerves. It also affected a wide spectrum of health indicators based on blood tests and decreased the tibial nerve compound muscle action potential amplitudes. Short-term health effects included alterations in complete blood count, hepatic and renal functions, and nerve conduction velocities and amplitudes. However, these effects could not be detected after 3 days following pesticide exposure. Overall, our results demonstrate that pesticide exposure adversely affects blood cells, the liver, and the peripheral nervous system. Future studies are needed to elucidate the specific effects of each pesticide and the mechanisms of these effects. PMID:26042669

Congenital oval or round window anomaly with or without abnormal facial nerve course: surgical results for 15 ears.

PubMed

Thomeer, Henricus; Kunst, Henricus; Verbist, Berit; Cremers, Cor

2012-07-01

To describe the audiometric results in a consecutive series of patients with congenital ossicular aplasia (Class 4a) or dysplasia of the oval and/or round window (Class 4b), which might include a possible anomalous course of the facial nerve. Retrospective chart study. Tertiary referral center. A tertiary referral center study with a total of 14 patients with congenital minor ear anomalies as part of a consecutive series (n = 89) who underwent exploratory tympanotomies (15 ears). Audiometric results. In 8 of 15 ears, ossicular reconstruction was attempted. In the short term (1 mo), there was a serviceable hearing outcome (air-bone gap closure to within 25 dB) in 4 ears. However, the long-term results showed deterioration because of an increased air-bone gap in all but 1 ear. No facial nerve lesion was observed postoperatively. Congenital dysplasia or aplasia of the oval and/or round window is an uncommon congenital minor ear anomaly. Classical microsurgical opportunities are rare in this group of anomalies. Newer options for hearing rehabilitation, such as the osseointegrated passive bone conduction devices, have become viable alternatives for conventional air conduction hearing devices. In the near future, upcoming active bone conduction devices might become the most preferred surgical option. In cases in which the facial nerve is only partially overlying the oval window, a type of malleostapedotomy procedure might result in a serviceable postoperative hearing level.

Behavioural, morphological and electrophysiological assessment of the effects of type 2 diabetes mellitus on large and small nerve fibres in Zucker diabetic fatty, Zucker lean and Wistar rats.

PubMed

Garcia-Perez, E; Schönberger, T; Sumalla, M; Stierstorfer, B; Solà, R; Doods, H; Serra, J; Gorodetskaya, N

2018-04-20

Peripheral neuropathy is a common complication in type 2 diabetes mellitus (T2DM). The most common presentation is in the form of a distal axonal sensory-motor polyneuropathy that involves large and small nerve fibres in variable proportion. Zucker Diabetic Fatty (ZDF), Zucker Lean (ZL) and Wistar Han (WH) rats were used to assess the behavioural, morphological and electrophysiological effects that T2DM have on peripheral large and small nerve fibres of 6- to 40-week-old rats. ZDF rats presented mechanical hypersensitivity that initially worsened in parallel to the progression of diabetes and eventually reverted at later stages of the disease. The reversal from hypersensitivity to hyposensitivity paralleled a reduction in the number of intraepithelial skin nerve terminals and in the nerve fibre lengths. However, no increased levels of degeneration of dorsal root ganglion neurons were observed. Nerve conduction studies showed a reduction in sensory and motor nerve conduction velocity (CV) in hyperglycaemic ZDF rats. Microneurography showed significant alterations in several parameters of activity-dependent slowing (ADS) of mechano-insensitive C-nociceptors in ZDF rats. Surprisingly, some of these changes were also observed in ZL rats. Moreover, we found spontaneous activity in all three strains implying that C-nociceptors become hyperexcitable and spontaneously active not only in ageing hyperglycaemic ZDF rats but also in age-matched and apparently normoglycaemic ZL and WH rats fed with the same diet. ZDF rats presented a diabetic neuropathy involving large and small nerve fibres; additionally, ZL and WH rats also showed early small abnormalities in C-fibres, clearly detected by microneurography SIGNIFICANCE: This study provides a functional description of large and small nerve fibre function in a diabetic model that recapitulates many of the findings observed in patients suffering from type 2 diabetes mellitus. © 2018 European Pain Federation - EFIC®.

Complex regional pain syndrome type I (RSD): pathology of skeletal muscle and peripheral nerve.

PubMed

van der Laan, L; ter Laak, H J; Gabreëls-Festen, A; Gabreëls, F; Goris, R J

1998-07-01

Reflex sympathetic dystrophy (RSD) (recently reclassified as complex regional pain syndrome type I) is a syndrome occurring in extremities and, when chronic, results in severe disability and untractable pain. RSD may be accompanied by neurologic symptoms even when there is no previous neurologic lesion. There is no consensus as to the pathogenic mechanism involved in RSD. To gain insight into the pathophysiology of RSD, we studied histopathology of skeletal muscle and peripheral nerve from patients with chronic RSD in a lower extremity. In eight patients with chronic RSD, an above-the-knee amputation was performed because of a nonfunctional limb. Specimens of sural nerves, tibial nerves, common peroneal nerves, gastrocnemius muscles, and soleus muscles were obtained from the amputated legs and analyzed by light and electron microscopy. In all patients, the affected leg showed similar neurologic symptoms such as spontaneous pain, hyperpathy, allodynia, paresis, and anesthesia dolorosa. The nerves showed no consistent abnormalities of myelinated fibers. In four patients, the C-fibers showed electron microscopic pathology. In all patients, the gastrocnemius and soleus muscle specimens showed a decrease of type I fibers, an increase of lipofuscin pigment, atrophic fibers, and severely thickened basal membrane layers of the capillaries. In chronic RSD, efferent nerve fibers were histologically unaffected; from afferent fibers, only C-fibers showed histopathologic abnormalities. Skeletal muscle showed a variety of histopathologic findings, which are similar to the histologic abnormalities found in muscles of patients with diabetes.

Diagnostic strategies using physical examination are minimally useful in defining carpal tunnel syndrome in population-based research studies.

PubMed

Descatha, A; Dale, A-M; Franzblau, A; Coomes, J; Evanoff, B

2010-02-01

We evaluated the utility of physical examination manoeuvres in the prediction of carpal tunnel syndrome (CTS) in a population-based research study. We studied a cohort of 1108 newly employed workers in several industries. Each worker completed a symptom questionnaire, a structured physical examination and nerve conduction study. For each hand, our CTS case definition required both median nerve conduction abnormality and symptoms classified as "classic" or "probable" on a hand diagram. We calculated the positive predictive values and likelihood ratios for physical examination manoeuvres in subjects with and without symptoms. The prevalence of CTS in our cohort was 1.2% for the right hand and 1.0% for the left hand. The likelihood ratios of a positive test for physical provocative tests ranged from 2.0 to 3.3, and those of a negative test from 0.3 to 0.9. The post-test probability of positive testing was <50% for all strategies tested. Our study found that physical examination, alone or in combination with symptoms, was not predictive of CTS in a working population. We suggest using specific symptoms as a first-level screening tool, and nerve conduction study as a confirmatory test, as a case definition strategy in research settings.

A neurophysiological study of facial numbness in multiple sclerosis: Integration with clinical data and imaging findings.

PubMed

Koutsis, Georgios; Kokotis, Panagiotis; Papagianni, Aikaterini E; Evangelopoulos, Maria-Eleftheria; Kilidireas, Constantinos; Karandreas, Nikolaos

2016-09-01

To integrate neurophysiological findings with clinical and imaging data in a consecutive series of multiple sclerosis (MS) patients developing facial numbness during the course of an MS attack. Nine consecutive patients with MS and recent-onset facial numbness were studied clinically, imaged with routine MRI, and assessed neurophysiologically with trigeminal somatosensory evoked potential (TSEP), blink reflex (BR), masseter reflex (MR), facial nerve conduction, facial muscle and masseter EMG studies. All patients had unilateral facial hypoesthesia on examination and lesions in the ipsilateral pontine tegmentum on MRI. All patients had abnormal TSEPs upon stimulation of the affected side, excepting one that was tested following remission of numbness. BR was the second most sensitive neurophysiological method with 6/9 examinations exhibiting an abnormal R1 component. The MR was abnormal in 3/6 patients, always on the affected side. Facial conduction and EMG studies were normal in all patients but one. Facial numbness was always related to abnormal TSEPs. A concomitant R1 abnormality on BR allowed localization of the responsible pontine lesion, which closely corresponded with MRI findings. We conclude that neurophysiological assessment of MS patients with facial numbness is a sensitive tool, which complements MRI, and can improve lesion localization. Copyright © 2016 Elsevier B.V. All rights reserved.

Vibration upshot of operating mechanical sewing machine: an insight into common peroneal nerve conduction study.

PubMed

Yadav, Prakash Kumar; Yadav, Ram Lochan; Sharma, Deepak; Shah, Dev Kumar; Sapkota, Niraj Khatri; Thakur, Dilip; Limbu, Nirmala; Islam, Md Nazrul

2017-01-01

Most of the people associated with tailoring occupation in Nepal are still using mechanical sewing machine as an alternative of new technology for tailoring. Common peroneal nerves of both right and left legs are exposed to strenuous and chronic stress exerted by vibration and paddling of mechanical sewing machine. The study included 30 healthy male tailors and 30 healthy male individuals. Anthropometric variables as well as cardio respiratory variables were determined for each subject. Standard Nerve Conduction Techniques using constant measured distances were applied to evaluate common peroneal nerve (motor) in both legs of each individual. Data were analyzed and compared between study and control groups using Man Whitney U test setting the significance level p  ≤ 0.05. Anthropometric and cardio respiratory variables were not significantly altered between the study and control groups. The Compound muscle action potential (CMAP) latency of common peroneal nerves of both right [(11.29 ± 1.25 vs. 10.03 ± 1.37), P  < 0.001] and left [(11.28 ± 1.38 vs. 10.05 ± 1.37), P  < 0.01] legs was found to be significantly prolonged in study group as compared to control group. The Amp-CMAP of common peroneal nerves of both right [(4.57 ± 1.21 vs. 6.22 ± 1.72), P  < 0.001] and left [(4.31 ± 1.55 vs. 6.25 ± 1.70), P  < 0.001] legs was found significantly reduced in study group as compared to control group. Similarly, the motor nerve conduction velocity (MNCV) of common peroneal nerves of both right [(43.72 ± 3.25 vs. 47.49 ± 4.17), P  < 0.001] and left [(42.51 ± 3.82 vs. 46.76 ± 4.51), P  < 0.001] legs was also found to be significantly reduced in study group in comparison to control group. Operating mechanical sewing machine by paddling chronically and arduously could have attributed to abnormal nerve conduction study parameters due to vibration effect of the machine on right and left common peroneal nerves. The results of present study follow the trend towards presymptomatic or asymptomatic neuropathy similar to subclinical neuropathy.

Electrophysiological studies of shoemakers exposed to sub-TLV levels of n-hexane.

PubMed

Neghab, Masoud; Soleimani, Esmaeel; Khamoushian, Kambiz

2012-01-01

Exposure to n-hexane, a neurotoxic solvent, has been associated with sensorimotor polyneuropathy, both in occupationally exposed workers and in glue-sniffing addicts. The present study was carried out to ascertain whether exposure to sub-TLV levels of n-hexane was associated with electrophysiological abnormalities and to determine if these possible abnormalities have any correlations with parameters such as the biological exposure index (BEI) of this neurotoxic chemical, workers' TWA exposure to n-hexane and/or duration of employment. Twenty-seven asymptomatic male workers from 6 shoemaking workshops were studied and compared with a group of 20 age- and sex-matched normal controls with no history of exposure to any neurotoxic agent. They underwent physical examinations as well as conventional needle electromyographic examinations and sensory and motor nerve conduction studies of upper and lower extremities. The TWA exposure to n-hexane and urinary concentration of free 2,5-hexanedione were also determined. Data were analyzed using version 16.0 of the SPSS/PC statistical package. The TWA exposure to n-hexane was estimated to be 83.2 mg/m(3). Electrophysiological studies showed that the amplitudes of sensory nerve action potential (SAP) for median and sural nerves were significantly lower in exposed subjects than in unexposed normal controls. Additionally, a significant correlation was found between these decreases and the urinary concentration of free 2,5-hexanedione. The significant decrements in SAP amplitudes for the median and sural nerves may be considered as appropriate indicators for early detection of n-hexane-induced peripheral neuropathy in asymptomatic workers with current exposure to sub-TLV levels of n-hexane.

Peripheral neuropathy in children with type 1 diabetes.

PubMed

Louraki, M; Karayianni, C; Kanaka-Gantenbein, C; Katsalouli, M; Karavanaki, K

2012-10-01

Diabetic neuropathy (DN) is a major complication of type 1 diabetes mellitus (T1DM) with significant morbidity and mortality in adulthood. Clinical neuropathy is rarely seen in paediatric populations, whereas subclinical neuropathy is commonly seen, especially in adolescents. Peripheral DN involves impairment of the large and/or small nerve fibres, and can be diagnosed by various methods. Nerve conduction studies (NCS) are the gold-standard method for the detection of subclinical DN; however, it is invasive, difficult to perform and selectively detects large-fibre abnormalities. Vibration sensation thresholds (VSTs) and thermal discrimination thresholds (TDTs) are quicker and easier and, therefore, more suitable as screening tools. Poor glycaemic control is the most important risk factor for the development of DN. Maintaining near-normoglycaemia is the only way to prevent or reverse neural impairment, as the currently available treatments can only relieve the symptoms of DN. Early detection of children and adolescents with nervous system abnormalities is crucial to allow all appropriate measures to be taken to prevent the development of DN. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

Ocular Manifestations of Noonan Syndrome: A Prospective Clinical and Genetic Study of 25 Patients.

PubMed

van Trier, Dorothée C; Vos, Anna M C; Draaijer, Renske W; van der Burgt, Ineke; Draaisma, Jos M Th; Cruysberg, Johannes R M

2016-10-01

To determine the full spectrum of ocular manifestations in patients with Noonan syndrome (NS). Prospective cross-sectional clinical and genetic study in a tertiary referral center. Twenty-five patients with NS (mean age, 14 years; range, 8 months-25 years) clinically diagnosed by validated criteria. All patients were examined by the same team following a detailed study protocol. Genetic analyses were performed in 23 patients. Ocular abnormalities of vision and refraction, external ocular features, ocular position and motility, anterior segment, posterior segment, and intraocular pressure. Ocular features of vision and refraction were amblyopia (32%), myopia (40%), and astigmatism (52%). External ocular features were epicanthic folds (84%), hypertelorism (68%), ptosis (56%), high upper eyelid crease (64%), lower eyelid retraction (60%), abnormal upward slanting palpebral fissures (36%), downward slanting palpebral fissures (32%), and lagophthalmos (28%). Orthoptic abnormalities included strabismus (40%), abnormal stereopsis (44%), and limited ocular motility (40%). Anterior segment abnormalities included prominent corneal nerves (72%) and posterior embryotoxon (32%). Additional ocular features were found, including nonglaucomatous optic disc excavation (20%), relatively low (<10 mmHg) intraocular pressure (22%), and optic nerve hypoplasia (4%). Mutations were established in 22 patients: 19 PTPN11 mutations (76%), 1 SOS1 mutation, 1 BRAF mutation, and 1 KRAS mutation. The patient with the highest number of prominent corneal nerves had an SOS1 mutation. The patient with the lowest visual acuity, associated with bilateral optic nerve hypoplasia, had a BRAF mutation. Patients with severe ptosis and nearly total absence of levator muscle function had PTPN11 mutations. All patients showed at least 3 ocular features (range, 3-13; mean, 7), including at least 1 external ocular feature in more than 95% of the patients. Noonan syndrome is a clinical diagnosis with multiple genetic bases associated with an extensive variety of congenital ocular abnormalities. Ocular features of NS are characterized by 1 or more developmental anomalies of the eyelids (involving the position, opening, and closure) associated with various other ocular abnormalities in childhood, including amblyopia, myopia, astigmatism, strabismus, limited ocular motility, prominent corneal nerves, and posterior embryotoxon. Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

Nerve Conduction Study on Sural Nerve among Nepalese Tailors Using Mechanical Sewing Machine.

PubMed

Yadav, Prakash Kumar; Yadav, Ram Lochan; Sharma, Deepak; Shah, Dev Kumar; Thakur, Dilip; Limbu, Nirmala; Islam, Md Nazrul

2017-03-01

The use of new technologies and innovations are out of access for people living in a developing country like Nepal. The mechanical sewing machine is still in existence at a large scale and dominant all over the country. Tailoring is one of the major occupations adopted by skilled people with lower socioeconomic status and education level. Sural nerves of both right and left legs are exposed to strenuous and chronic stress exerted by chronic paddling of mechanical sewing machine with legs. To evaluate the influence of chronic and strenuous paddling on right and left sural nerves. The study recruited 30 healthy male tailors with median age {34(31-37.25)} years (study group), and, 30 healthy male volunteers with age {34(32-36.25)} years (control group). Anthropometric measurements (age, height, weight, BMI and length of both right and left legs) as well as cardio respiratory measurements [Systolic Blood Pressure (SBP), Dystolic Blood Pressure (DBP), Pules Rate (PR) and Respiratory Rate (RR)] were recorded for each subject. Standard nerve conduction techniques using constant measured distances were applied to evaluate sural nerve (sensory) in both legs of each individual. The differences in variables between the study and control groups were tested using Student's t-test for parametric variables and Mann-Whitney U test for nonparametric variables. A p-value of ≤ 0.05 was considered significant. Age, height, weight, body mass index and leg length were not significantly different between tailors and control groups. Cardio respiratory measurements (SBP, DBP, PR and RR) were also not significantly altered between both the groups. The sensory nerve conduction velocities (m/s) of the right {44.23(42.72-47.83) vs 50(46- 54)} and left sural nerves {45.97±5.86 vs 50.67±6.59} m/s were found significantly reduced in tailors in comparison to control group. Similarly amplitudes (μv) of right sural (20.75±5.42 vs 24.10±5.45) and left sural nerves {18.2(12.43-21.8) vs 32.0(26.5-35.25)} were significantly less in tailors in comparison to control group whereas, latencies (ms) of right sural {2.6(2.2-2.7) vs 2.0(2.0-2.250} and left sural nerve {2.4(2.07-2.72) vs 2.0(2.0-2.0)} were found significantly high in tailors as compared with control group. Operating mechanical sewing machine by paddling chronically and arduously could have attributed to abnormal nerve conduction study parameters due to vibration effect of the machine on right and left sural nerves. The results of present study follow the trend towards presymptomatic or asymptomatic neuropathy similar to subclinical neuropathy.

Nerve Conduction Study on Sural Nerve among Nepalese Tailors Using Mechanical Sewing Machine

PubMed Central

Yadav, Ram Lochan; Sharma, Deepak; Shah, Dev Kumar; Thakur, Dilip; Limbu, Nirmala; Islam, Md. Nazrul

2017-01-01

Introduction The use of new technologies and innovations are out of access for people living in a developing country like Nepal. The mechanical sewing machine is still in existence at a large scale and dominant all over the country. Tailoring is one of the major occupations adopted by skilled people with lower socioeconomic status and education level. Sural nerves of both right and left legs are exposed to strenuous and chronic stress exerted by chronic paddling of mechanical sewing machine with legs. Aim To evaluate the influence of chronic and strenuous paddling on right and left sural nerves. Materials and Methods The study recruited 30 healthy male tailors with median age {34(31-37.25)} years (study group), and, 30 healthy male volunteers with age {34(32-36.25)} years (control group). Anthropometric measurements (age, height, weight, BMI and length of both right and left legs) as well as cardio respiratory measurements [Systolic Blood Pressure (SBP), Dystolic Blood Pressure (DBP), Pules Rate (PR) and Respiratory Rate (RR)] were recorded for each subject. Standard nerve conduction techniques using constant measured distances were applied to evaluate sural nerve (sensory) in both legs of each individual. The differences in variables between the study and control groups were tested using Student’s t-test for parametric variables and Mann-Whitney U test for nonparametric variables. A p-value of ≤ 0.05 was considered significant. Results Age, height, weight, body mass index and leg length were not significantly different between tailors and control groups. Cardio respiratory measurements (SBP, DBP, PR and RR) were also not significantly altered between both the groups. The sensory nerve conduction velocities (m/s) of the right {44.23(42.72-47.83) vs 50(46- 54)} and left sural nerves {45.97±5.86 vs 50.67±6.59} m/s were found significantly reduced in tailors in comparison to control group. Similarly amplitudes (μv) of right sural (20.75±5.42 vs 24.10±5.45) and left sural nerves {18.2(12.43-21.8) vs 32.0(26.5-35.25)} were significantly less in tailors in comparison to control group whereas, latencies (ms) of right sural {2.6(2.2-2.7) vs 2.0(2.0-2.250} and left sural nerve {2.4(2.07-2.72) vs 2.0(2.0-2.0)} were found significantly high in tailors as compared with control group. Conclusion Operating mechanical sewing machine by paddling chronically and arduously could have attributed to abnormal nerve conduction study parameters due to vibration effect of the machine on right and left sural nerves. The results of present study follow the trend towards presymptomatic or asymptomatic neuropathy similar to subclinical neuropathy. PMID:28511376

Retinal architecture and mfERG: Optic nerve head component response characteristics in MS.

PubMed

Schnurman, Zane S; Frohman, Teresa C; Beh, Shin C; Conger, Darrel; Conger, Amy; Saidha, Shiv; Galetta, Steven; Calabresi, Peter A; Green, Ari J; Balcer, Laura J; Frohman, Elliot M

2014-05-27

To describe a novel neurophysiologic signature of the retinal ganglion cell and to elucidate its relationship to abnormalities in validated structural and functional measures of the visual system. We used multifocal electroretinogram-generated optic nerve head component (ONHC) responses from normal subjects (n = 18), patients with multiple sclerosis (MS) (n = 18), and those with glaucoma (n = 3). We then characterized the relationship between ONHC response abnormalities and performance on low-contrast visual acuity, multifocal visual-evoked potential-induced cortical responses, and average and quadrant retinal nerve fiber layer (RNFL) thicknesses, as measured by spectral-domain optical coherence tomography. Compared with the eyes of normal subjects, the eyes of patients with MS exhibited an increased number of abnormal or absent ONHC responses (p < 0.0001). For every 7-letter reduction in low-contrast letter acuity, there were corresponding 4.6 abnormal ONHC responses at 2.5% contrast (p < 0.0001) and 6.6 abnormalities at the 1.25% contrast level (p < 0.0001). Regarding average RNFL thickness, for each 10-μm thickness reduction, we correspondingly observed 6.8 abnormal ONHC responses (p = 0.0002). The most robust association was between RNFL thinning in the temporal quadrant and ONHC response abnormalities (p < 0.0001). Further characterization of ONHC abnormalities (those that are reversible and irreversible) may contribute to the development of novel neurotherapeutic strategies aimed at achieving neuroprotective, and perhaps even neurorestorative, effects in disorders that target the CNS in general, and MS in particular. © 2014 American Academy of Neurology.

Demyelination of vestibular nerve axons in unilateral Ménière's disease.

PubMed

Spencer, Robert F; Sismanis, Aristides; Kilpatrick, Jefferson K; Shaia, Wayne T

2002-11-01

We conducted a study to determine whether vestibular nerves in patients with unilateral Ménière's disease whose symptoms are refractory to medical management exhibit neuropathologic changes. We also endeavored to determine whether retrocochlear abnormalities are primary or secondary factors in the disease process. To these ends, we obtained vestibular nerve segments from five patients during retrosigmoid (posterior fossa) neurectomy, immediately fixed them, and processed them for light and electron microscopy. We found that all five segments exhibited moderate to severe demyelination with axonal sparing. Moreover, we noted that reactive astrocytes produced an extensive proliferation of fibrous processes and that the microglia assumed a phagocytic role. We conclude that the possible etiologies of demyelination include viral and/or immune-mediated factors similar to those seen in other demyelinating diseases, such as multiple sclerosis and Guillain-Barré syndrome. Our findings suggest that some forms of Ménière's disease that are refractory to traditional medical management might be the result of retrocochlear pathology that affects the neuroglial portion of the vestibular nerve.

Choristoma of the middle ear: a component of a new syndrome?

PubMed

Buckmiller, L M; Brodie, H A; Doyle, K J; Nemzek, W

2001-05-01

Salivary choristoma of the middle ear is a rare entity. The authors report the 26th known case, which is unique in several respects: the patient had abnormalities of the first and second branchial arches, as well as the otic capsule and facial nerve in ways not yet reported. Our patient presented with bilateral preauricular pits, conchal bands, an ipsilateral facial palsy, and bilateral Mondini-type deformities. A review of the literature revealed salivary choristomas of the middle ear to be frequently associated with branchial arch abnormalities, most commonly the second, as well as abnormalities of the facial nerve. All 25 cases were reviewed and the results reported with respect to clinical presentation, associated abnormalities, operative findings, and hearing results. It has been proposed that choristoma of the middle ear may represent a component of a syndrome along with unilateral hearing loss, abnormalities of the incus and/or stapes, and anomalies of the facial nerve. Eighty-six percent of the reported patients with choristoma have three or four of the four criteria listed to designate middle ear salivary choristoma as part of a syndrome. In the remaining four patients, all of the structures were not assessed.

Nervous system (image)

MedlinePlus

Peripheral Neuropathy is not a distinct disease, but the manifestation of many conditions that damage the peripheral nerves ( ... abnormal. Damaged motor nerves impair movement or function. Peripheral neuropathy may be caused by direct or indirect injury, ...

The sensitivity of clinical diagnostic methods in the diagnosis of diabetic neuropathy.

PubMed

Onde, M E; Ozge, A; Senol, M G; Togrol, E; Ozdag, F; Saracoglu, M; Misirli, H

2008-01-01

This study assessed the sensitivity of various methods for the clinical diagnosis of diabetic peripheral neuropathy. A total of 147 randomly selected patients with diabetes mellitus and 65 age- and sex-matched healthy controls were evaluated by various clinical (the neuropathy symptom score [NSS], the neuropathy disability score [NDS], vibration perception thresholds [VPTs], Tinel's sign and Phalen's sign), laboratory (fasting plasma glucose and glycosylated haemoglobin levels) and electro-physiological (nerve conduction studies, H-reflex and F-wave measurements) methods. In the patient group, 8.2% had an abnormal NSS, 28.5% had a positive Phalen's sign, 32.6% had a positive Tinel's sign, 42.8% had an abnormal VPT and 57.1% had an abnormal NDS. Significant correlations were found between electro-physiologically confirmed neuropathy and the two provocation tests and abnormal VPTs. In conclusion, assessment with a complete neurological examination and standard electrophysiological tests is very important for the diagnosis of diabetic peripheral neuropathy and the prevention of morbidity in patients with or without symptoms.

F-18 FDG PET/CT findings of a case of sacral nerve root neurolymphomatosis that occurred during chemotherapy.

PubMed

Suga, Kazuyoshi; Yasuhiko, Kawakami; Matsunaga, Naofumi; Yujiri, Toshiaki; Nakazora, Tatsuki; Ariyoshi, Kouichi

2011-01-01

Neurolymphomatosis (NL) is a rare, unique subtype of lymphomatous infiltration of peripheral nerves. Clinical/radiologic diagnosis of NL is challenging. We report F-18 FDG PET/CT findings of a case of breast diffuse large B-cell lymphoma, in which NL developed regardless of regression of systemic lesions during induction chemotherapy. FDG PET/CT showed characteristic findings of well-demarcated, linear abnormal FDG uptake along a sacral vertebral foramen, leading to diagnosis of NL, with the finding of thickened nerve roots on magnetic resonance imaging. Altered chemotherapeutic regimen resulted in disappearance of these abnormal FDG uptake, with recovery of neurologic symptoms. Peripheral nerve NL may occur during chemotherapy, and FDG PET/CT can be a useful imaging modality in diagnosis and monitoring of therapeutic response of this disease.

Polyol pathway, 2,3-diphosphoglycerate in erythrocytes and diabetic neuropathy in rats.

PubMed

Nakamura, J; Koh, N; Sakakibara, F; Hamada, Y; Wakao, T; Hara, T; Mori, K; Nakashima, E; Naruse, K; Hotta, N

1995-12-27

The relationship between the 2,3-diphosphoglycerate concentration in red blood cells as a biological indicator of tissue hypoxia and diabetic neuropathy, and the effect of a potent aldose reductase inhibitor, (2S,4S)-6-fluoro-2'5'-dioxospiro [chroman-4,4'-imidazolidine]-2-carboxamide (SNK-860), on both were investigated in streptozotocin-induced diabetic rats. Diabetic rats demonstrated significantly delayed motor nerve conduction velocity and reduced sciatic nerve blood flow. Altered biochemical features in the sciatic nerves, including a marked accumulation of sorbitol and fructose, myo-inositol depletion and decreased Na+/K(+)-ATPase activity were also detected in diabetic rats. These defects were accompanied by a decrease in the red blood cell 2,3-diphosphoglycerate concentration. Treatment with SNK-860 partially or completely ameliorated these abnormalities. These observations suggest that a decrease in the red blood cell 2,3-diphosphoglycerate concentration is one of the factors contributing to tissue hypoxia, which results in diabetic neuropathy, and that this decrease is mediated through an aldose reductase inhibitor-sensitive pathway.

Premature aging-related peripheral neuropathy in a mouse model of progeria.

PubMed

Goss, James R; Stolz, Donna Beer; Robinson, Andria Rasile; Zhang, Mingdi; Arbujas, Norma; Robbins, Paul D; Glorioso, Joseph C; Niedernhofer, Laura J

2011-08-01

Peripheral neuropathy is a common aging-related degenerative disorder that interferes with daily activities and leads to increased risk of falls and injury in the elderly. The etiology of most aging-related peripheral neuropathy is unknown. Inherited defects in several genome maintenance mechanisms cause tissue-specific accelerated aging, including neurodegeneration. We tested the hypothesis that a murine model of XFE progeroid syndrome, caused by reduced expression of ERCC1-XPF DNA repair endonuclease, develops peripheral neuropathy. Nerve conduction studies revealed normal nerve function in young adult (8 week) Ercc1(-/Δ) mice, but significant abnormalities in 20 week-old animals. Morphologic and ultrastructural analysis of the sciatic nerve from mutant mice revealed significant alterations at 20 but not 8 weeks of age. We conclude that Ercc1(-/Δ) mice have accelerated spontaneous peripheral neurodegeneration that mimics aging-related disease. This provides strong evidence that DNA damage can drive peripheral neuropathy and offers a rapid and novel model to test therapies. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Acute bilateral painless radiculitis with abnormal Borrelia burgdorferi immunoblot.

PubMed

Almodovar, Jorge L; Hehir, Michael K; Nicholson, Katharine A; Stommel, Elijah W

2012-12-01

A 57-year-old woman with a history of hypertension and hypothyroidism presented with painless left arm weakness and numbness 2 weeks before evaluation. Nerve conduction studies of the left arm revealed normal motor and sensory responses. Needle examination revealed acute denervation changes in all myotomes of the affected extremity, including cervical paraspinals on the left, and several myotomes on the contralateral side. The laboratory evaluation revealed normal anti-GM1 antibodies and 3 IgM/5 IgG bands on Lyme Western Blot. The patient began treatment with 28 days of intravenous ceftriaxone. On follow-up, patient had regained full strength of her extremities with no sensory deficits. Inflammatory borrelia radiculitis usually presents with pain in the distribution of the affected nerves and nerve roots. The novelty of this case report rests on (1) the absence of primary borreliosis symptomatology preceding the radiculitis and (2) the painless and bilateral clinical presentation in a patient with suspected Lyme radiculitis.

[Evaluation of motor and sensory neuroconduction of the median nerve in patients with carpal tunnel syndrome treated with non-coherent light emitted by gallium arsenic diodes].

PubMed

Viera Alemán, C; Purón, E; Hamilton, M L; Santos Anzorandia, C; Navarro, A; Pineda Ortiz, I

The treatment selection in the carpal tunnel syndrome according to the damage of the median nerve is important and all of these have adverse effects. A good alternative without undesired reactions is irradiation of the carpal tunnel with not coherent light between 920 and 940 nm emitted by gallium arsenide diodes, resembling the physic and therapeutic laser effects. Twenty-six female patients with idiopathic middle carpal tunnel syndrome were irradiated 15 minutes daily during three weeks. The median nerve motor and sensitive neuroconduction was studied before and immediately after the treatment. The abnormal neuroconduction variables (latency, amplitude and velocity conduction) did not modify when treatment concluded, in spite of all the patients reported disappearance of pain and numbness in damaged hands. Not coherent light does not change the fibers functional state explored by conventional neuroconductions techniques. It remains to know if this light produces fine fibers improvement.

Pathogenesis of cranial neuropathies in Moebius syndrome: Electrodiagnostic orofacial studies.

PubMed

Renault, Francis; Flores-Guevara, Roberto; Sergent, Bernard; Baudon, Jean Jacques; Aouizerate, Jessie; Vazquez, Marie-Paule; Gitiaux, Cyril

2018-02-09

We designed a retrospective study of 59 patients with congenital sporadic nonprogressive bilateral facial and abducens palsies. Examinations included needle electromyography (EMG) of facial and oral muscles, facial nerve motor latency and conduction velocity (FNCV), and blink responses (BR). Neurogenic EMG changes were found in 1 or more muscles in 55 of 59 patients, with no abnormal spontaneous activity. EMG changes were homogeneously neurogenic in 17 patients, homogeneously myopathic in 1 patient, and heterogeneous in 41 of 59 patients. Motor latency was increased according to recordings from 52 of 137 facial muscles. An increase of motor latency was not associated with neurogenic EMG (Fischer's test: right, P = 1; left, P = 0.76). FNCV was slowed in 19 of 36 patients. BR was absent bilaterally in 35 of 58 patients; when present, R1 and R2 latencies were normal. Our results support the hypothesis of an early developmental defect localized in motor cranial nerves with spared V-VII internuclear pathways. Muscle Nerve, 2018. © 2018 Wiley Periodicals, Inc.

Neurological abnormalities associated with CDMA exposure.

PubMed

Hocking, B; Westerman, R

2001-09-01

Dysaesthesiae of the scalp and neurological abnormality after mobile phone use have been reported previously, but the roles of the phone per se or the radiations in causing these findings have been questioned. We report finding a neurological abnormality in a patient after accidental exposure of the left side of the face to mobile phone radiation [code division multiple access (CDMA)] from a down-powered mobile phone base station antenna. He had headaches, unilateral left blurred vision and pupil constriction, unilateral altered sensation on the forehead, and abnormalities of current perception thresholds on testing the left trigeminal ophthalmic nerve. His nerve function recovered during 6 months follow-up. His exposure was 0.015-0.06 mW/cm(2) over 1-2 h. The implications regarding health effects of radiofrequency radiation are discussed.

Electromyographic studies of motor control in humans.

PubMed

Shahani, B T; Wierzbicka, M M

1987-11-01

Electromyography and electroneurography have proved to be useful in investigation and understanding of a variety of neurologic disorders. In most laboratories, however, these electrodiagnostic techniques have been used to help in the diagnosis of diseases that affect the peripheral nerves, neuromuscular junctions, or skeletal muscle fibers. Although major advances in electronic and computer technology have made it possible to study, quantitate, and document reflex activity in intact human subjects, most neurologists still rely on gross clinical observations and most electromyographers continue to use conventional techniques of EMG and nerve conduction studies to differentiate "myopathy" from "neuropathy." This article is a review of some of the electromyographic techniques that have been used in the authors' laboratory for the study of normal and abnormal motor control in man and the treatment of patients with disorders of motor control.

Visualization of the diaphragm muscle with ultrasound improves diagnostic accuracy of phrenic nerve conduction studies.

PubMed

Johnson, Nicholas E; Utz, Michael; Patrick, Erica; Rheinwald, Nicole; Downs, Marlene; Dilek, Nuran; Dogra, Vikram; Logigian, Eric L

2014-05-01

Evaluation of phrenic neuropathy (PN) with phrenic nerve conduction studies (PNCS) is associated with false negatives. Visualization of diaphragmatic muscle twitch with diaphragm ultrasound (DUS) when performing PNCS may help to solve this problem. We performed bilateral, simultaneous DUS-PNCS in 10 healthy adults and 12 patients with PN. The amplitude of the diaphragm compound muscle action potential (CMAP) (on PNCS) and twitch (on DUS) was calculated. Control subjects had <38% side-to-side asymmetry in twitch amplitude (on DUS) and 53% asymmetry in phrenic CMAP (on PCNS). In the 12 patients with PN, 12 phrenic neuropathies were detected. Three of these patients had either significant side-to-side asymmetry or absolute reduction in diaphragm movement that was not detected with PNCS. There were no cases in which the PNCS showed an abnormality but the DUS did not. The addition of DUS to PNCS enhances diagnostic accuracy in PN. Copyright © 2013 Wiley Periodicals, Inc.

[Early diagnosis and prognosis evaluation of Bell palsy with blink reflex ].

PubMed

Xie, Dan-dan; Li, Xiao-song; Liu, Yuan-yuan

2014-11-01

To determine the value of blink reflex in early diagnosis and prognosis evaluation of Bell palsy. Blink reflex and facial nerve conduction were examined in 58 patients with Bell palsy within one week after symptom onset. The patients without response of R1 , R2 and R2 ' waves were classified as complete efferent retardarce (Group A, 30 cases), and those with response of R1 , R2 and R2 ' waves were classified as incomplete efferent anomalies (Group B, 28 cases). The clinical outcomes after three months of systemic therapy were evaluated using the House-Blackmann (H-B) scale. Efferent anomalies of blink reflex occurred in ail of the 58 patients. Abnormal results of facial nerve conduction appeared in 23 (39. 7%) patients. The three months therapy was effective in 93% patients in Group B and 70% patients in Group A (P<0. 05). Blink reflex can play a significant role in early diagnosis and prognosis evaluation of Bell palsy.

Score Reliability and Construct Validity of the Flinn Performance Screening Tool for Adults With Symptoms of Carpal Tunnel Syndrome

PubMed Central

Flinn, Sharon R.; Pease, William S.; Freimer, Miriam L.

2013-01-01

OBJECTIVE We investigated the psychometric properties of the Flinn Performance Screening Tool (FPST) for people referred with symptoms of carpal tunnel syndrome (CTS). METHOD An occupational therapist collected data from 46 participants who completed the Functional Status Scale (FSS) and FPST after the participants’ nerve conduction velocity study to test convergent and contrasted-group validity. RESULTS Seventy-four percent of the participants had abnormal nerve conduction studies. Cronbach’s α coefficients for subscale and total scores of the FPST ranged from .96 to .98. Intrarater reliability for six shared items of the FSS and the FPST was supported by high agreement (71%) and a fair κ statistic (.36). Strong to moderate positive relationships were found between the FSS and FPST scores. Functional status differed significantly among severe, mild, and negative CTS severity groups. CONCLUSION The FPST shows adequate psychometric properties as a client-centered screening tool for occupational performance of people referred for symptoms of CTS. PMID:22549598

Sensory chronic inflammatory demyelinating polyneuropathy: an under-recognized entity?

PubMed

Ayrignac, Xavier; Viala, Karine; Koutlidis, Régine Morizot; Taïeb, Guillaume; Stojkovic, Tanya; Musset, Lucille; Léger, Jean-Marc; Fournier, Emmanuel; Maisonobe, Thierry; Bouche, Pierre

2013-11-01

Sensory chronic inflammatory demyelinating polyneuropathy (CIDP) can be difficult to diagnose. We report 22 patients with chronic sensory polyneuropathy with ≥1 clinical sign atypical for chronic idiopathic axonal polyneuropathy (CIAP) but no electrodiagnostic criteria for CIDP. Clinical signs atypical for CIAP were: sensory ataxia (59%), generalized areflexia (36%), cranial nerve involvement (32%), rapid upper limb involvement (40%), and age at onset ≤55 years (50%). Additional features were: normal sensory nerve action potentials (36%), abnormal radial/normal sural pattern (23%), abnormal somatosensory evoked potentials (SSEPs) (100%), elevated cerebrospinal fluid (CSF) protein (73%), and demyelinating features in 5/7 nerve biopsies. Over 90% of patients responded to immunotherapy. We conclude that all patients had sensory CIDP. Sensory CIDP patients can be misdiagnosed as having CIAP. If atypical clinical/electrophysiologic features are present, we recommend performing SSEPs and CSF examination. Nerve biopsy should be restricted to disabled patients if other examinations are inconclusive. Copyright © 2013 Wiley Periodicals, Inc.

Computed tomography of cystic nerve root sleeve dilatation.

PubMed

Neave, V C; Wycoff, R R

1983-10-01

A case of cystic nerve root sleeve dilatation in the lumbar area associated with a chronic back pain syndrome is presented. Prominent computed tomography (CT) findings include: (a) rounded masses in the region of the foramina isodense with cerebrospinal fluid in the subarachnoid space; (b) associated asymmetry of epidural fat distribution; (c) enlargement of the neural foramina in axial sections with scalloped erosion of the adjacent posteriolateral vertebral body, pedicle, and pedicular-laminar junction with preservation of cortex and without bony sclerosis or infiltrative appearance; (d) prominent or ectatic dural sac with lack of usual epidural landmarks between the sac and vertebral body; and (e) multilevel abnormalities throughout the entire lumbar region. Myelographic and CT correlations are demonstrated with a review of the literature. A discussion of the various cystic abnormalities involving nerve root sheaths is undertaken in an attempt to clarify the confusing nomenclature applied to nerve root sleeve pathology.

Cranial mononeuropathy III

MedlinePlus

... is one of the cranial nerves that control eye movement. Causes may include: Brain aneurysm Infections Abnormal blood ... show: Enlarged (dilated) pupil of the affected eye Eye movement abnormalities Eyes that are not aligned Your health ...

Hemilingual spasm: defining a new entity, its electrophysiological correlates and surgical treatment through microvascular decompression.

PubMed

Osburn, Leisha L; Møller, Aage R; Bhatt, Jay R; Cohen-Gadol, Aaron A

2010-07-01

We report on vascular compression syndrome of the 12th cranial nerve (hypoglossal), an occurrence not previously reported, and demonstrate, through corresponding objective electrophysiological evidence, that microvascular decompression of the hypoglossal nerve root can cure hemilingual spasm. A 52-year-old man had lower face muscle twitching and tongue spasms, which worsened with talking, chewing, or emotional stress. Carbamazepine offered only temporary relief, and relief from injections of botulinum toxin was insignificant. He was referred for surgical treatment. High-resolution magnetic resonance imaging of his posterior fossa contents revealed no obvious evidence of any compressive vessel along the facial nerve, but a compressive vessel along the hypoglossal nerve was apparent. The presence of preoperative tongue spasms encouraged interoperative monitoring of tongue motor responses. The facial nerve exit zone was explored, but microsurgical inspection of the seventh/eighth cranial nerve complex did not reveal any compressive vessel. However, at the anterolateral aspect of the medulla oblongata, the hypoglossal nerve was clearly compressed and distorted laterally by a large tortuous vertebral artery. When the artery was mobilized away from the nerve, the abnormal late electromyographic response to transcranial electrical stimulation disappeared; immediately after shredded Teflon was interpositioned between the artery and the nerve, the abnormal spontaneous tongue fasciculation also disappeared. The patient has remained spasm free 6 months after surgery. Hemilingual spasm may be caused by vascular contact/compression along cranial nerve XII at the lower brainstem and belong to the same family of cranial nerve hyperactivity disorders as hemifacial spasm.

Detecting acute neurotoxicity during platinum chemotherapy by neurophysiological assessment of motor nerve hyperexcitability

PubMed Central

2010-01-01

Background Platinum-based drugs, such as cisplatin and oxaliplatin, are well-known for inducing chronic sensory neuropathies but their acute and motor neurotoxicities are less well characterised. Use was made of nerve conduction studies and needle electromyography (EMG) to assess motor nerve excitability in cancer patients during their first treatment cycle with platinum-based chemotherapy in this study. Methods Twenty-nine adult cancer patients had a neurophysiological assessment either before oxaliplatin plus capecitabine, on days 2 to 4 or 14 to 20 after oxaliplatin plus capecitabine, or on days 2 to 4 after carboplatin plus paclitaxel or cisplatin, undertaken by a neurophysiologist who was blinded to patient and treatment details. Patients completed a symptom questionnaire at the end of the treatment cycle. Results Abnormal spontaneous high frequency motor fibre action potentials were detected in 100% of patients (n = 6) and 72% of muscles (n = 22) on days 2 to 4 post-oxaliplatin, and in 25% of patients (n = 8) and 13% of muscles (n = 32) on days 14 to 20 post-oxaliplatin, but in none of the patients (n = 14) or muscles (n = 56) tested prior to oxaliplatin or on days 2 to 4 after carboplatin plus paclitaxel or cisplatin. Repetitive compound motor action potentials were less sensitive and less specific than spontaneous high frequency motor fibre action potentials for detection of acute oxaliplatin-induced motor nerve hyperexcitability but were present in 71% of patients (n = 7) and 32% of muscles (n = 32) on days 2 to 4 after oxaliplatin treatment. Acute neurotoxicity symptoms, most commonly cold-induced paraesthesiae and jaw or throat tightness, were reported by all patients treated with oxaliplatin (n = 22) and none of those treated with carboplatin plus paclitaxel or cisplatin (n = 6). Conclusions Abnormal spontaneous high frequency motor fibre activity is a sensitive and specific endpoint of acute oxaliplatin-induced motor nerve hyperexcitability, detectable on EMG on days 2 to 4 post-treatment. Objective EMG assessment of motor nerve excitability could compliment patient-reported symptomatic endpoints of acute oxaliplatin-induced neurotoxicity in future studies. PMID:20731872

Nerve fibre layer analysis with GDx with a variable corneal compensator in patients with multiple sclerosis.

PubMed

Della Mea, Giovanni; Bacchetti, Sonia; Zeppieri, Marco; Brusini, Paolo; Cutuli, Daniela; Gigli, Gian Luigi

2007-01-01

To evaluate the ability of GDx with variable corneal compensator (VCC) compared to visual-evoked potentials (VEPs) and standard automated perimetry (SAP) in the detection of early optic nerve damage in patients with multiple sclerosis (MS). 46 eyes of 23 MS patients were included. Ten of them had a history of acute retrobulbar optic neuritis. A control group of 20 normal subjects was also included. All subjects underwent a complete ophthalmological examination and testing with SAP, GDx VCC and VEPs. 19 eyes (41.3%) were abnormal with GDx VCC compared to 38 eyes (82.6%) with SAP and 31 (64.4%) with VEPs. In the optic neuritis group, 9 eyes (69.2%) had optic nerve pallor; SAP was abnormal in 8 of these eyes (61.5%) while VEPs and GDx VCC were abnormal in 6 eyes (46.1%). 2/20 eyes (10.0%) in the control group gave a false-positive abnormal result with SAP. GDx VCC and VEP were normal for all the eyes in the control group. GDx VCC is less able to detect early defects in MS patients compared to the currently used standard techniques of SAP and VEPs. Copyright (c) 2007 S. Karger AG, Basel.

Prognostic significance of electrophysiological tests for facial nerve outcome in vestibular schwannoma surgery.

PubMed

van Dinther, J J S; Van Rompaey, V; Somers, T; Zarowski, A; Offeciers, F E

2011-01-01

To assess the prognostic significance of pre-operative electrophysiological tests for facial nerve outcome in vestibular schwannoma surgery. Retrospective study design in a tertiary referral neurology unit. We studied a total of 123 patients with unilateral vestibular schwannoma who underwent microsurgical removal of the lesion. Nine patients were excluded because they had clinically abnormal pre-operative facial function. Pre-operative electrophysiological facial nerve function testing (EPhT) was performed. Short-term (1 month) and long-term (1 year) post-operative clinical facial nerve function were assessed. When pre-operative facial nerve function, evaluated by EPhT, was normal, the outcome from clinical follow-up at 1-month post-operatively was excellent in 78% (i.e. HB I-II) of patients, moderate in 11% (i.e. HB III-IV), and bad in 11% (i.e. HB V-VI). After 1 year, 86% had excellent outcomes, 13% had moderate outcomes, and 1% had bad outcomes. Of all patients with normal clinical facial nerve function, 22% had an abnormal EPhT result and 78% had a normal result. No statistically significant differences could be observed in short-term and long-term post-operative facial function between the groups. In this study, electrophysiological tests were not able to predict facial nerve outcome after vestibular schwannoma surgery. Tumour size remains the best pre-operative prognostic indicator of facial nerve function outcome, i.e. a better outcome in smaller lesions.

Guillain-Barré syndrome in Bangladesh: validation of Brighton criteria.

PubMed

Islam, Mohammad B; Islam, Zhahirul; Farzana, Kaniz S; Sarker, Sumit K; Endtz, Hubert P; Mohammad, Quazi D; Jacobs, Bart C

2016-12-01

Guillain-Barré syndrome has a diverse clinical phenotype related to geographical origin. To date, the majority of large-scale studies on Guillain-Barré syndrome (GBS) have been conducted in developed countries. We aimed to evaluate the key diagnostic features and assess the suitability of the Brighton criteria in 344 adult GBS patients from Bangladesh. All patients fulfilled the National Institute of Neurological Diseases and Stroke (NINDS) diagnostic criteria. Standardized data on demographic characteristics and clinical features, cerebrospinal fluid (CSF) analysis, and nerve conduction study (NCS) results were elaborated to measure the sensitivity of Brighton criteria. Most patients (88%) were admitted to hospital after the nadir weakness. Symmetrical weakness and reduced reflexes were found in 98% of patients. CSF albuminocytologic dissociation was detected in 238/269 (89%) cases and abnormal nerve physiology in 258/259 (>99%) cases. Only 27 (8%) patients received either intravenous immunoglobulin (IVIg) or plasmapheresis. In total, 200 (58%) patients met level 1 of the Brighton criteria; 97 (28%) patients met level 2; 42 (12%) patients met level 3; and 5 (2%) patients met level 4. This analysis showed that despite the heterogeneity of GBS in Bangladesh, the Brighton criteria showed a high sensitivity in the diagnosis of GBS. © 2016 Peripheral Nerve Society.

The WISTAH hand study: A prospective cohort study of distal upper extremity musculoskeletal disorders

PubMed Central

2012-01-01

Background Few prospective cohort studies of distal upper extremity musculoskeletal disorders have been performed. Past studies have provided somewhat conflicting evidence for occupational risk factors and have largely reported data without adjustments for many personal and psychosocial factors. Methods/design A multi-center prospective cohort study was incepted to quantify risk factors for distal upper extremity musculoskeletal disorders and potentially develop improved methods for analyzing jobs. Disorders to analyze included carpal tunnel syndrome, lateral epicondylalgia, medial epicondylalgia, trigger digit, deQuervain’s stenosing tenosynovitis and other tendinoses. Workers have thus far been enrolled from 17 different employment settings in 3 diverse US states and performed widely varying work. At baseline, workers undergo laptop administered questionnaires, structured interviews, two standardized physical examinations and nerve conduction studies to ascertain demographic, medical history, psychosocial factors and current musculoskeletal disorders. All workers’ jobs are individually measured for physical factors and are videotaped. Workers are followed monthly for the development of musculoskeletal disorders. Repeat nerve conduction studies are performed for those with symptoms of tingling and numbness in the prior six months. Changes in jobs necessitate re-measure and re-videotaping of job physical factors. Case definitions have been established. Point prevalence of carpal tunnel syndrome is a combination of paraesthesias in at least two median nerve-served digits plus an abnormal nerve conduction study at baseline. The lifetime cumulative incidence of carpal tunnel syndrome will also include those with a past history of carpal tunnel syndrome. Incident cases will exclude those with either a past history or prevalent cases at baseline. Statistical methods planned include survival analyses and logistic regression. Discussion A prospective cohort study of distal upper extremity musculoskeletal disorders is underway and has successfully enrolled over 1,000 workers to date. PMID:22672216

Neurophysiological profile of peripheral neuropathy associated with childhood mitochondrial disease.

PubMed

Menezes, Manoj P; Rahman, Shamima; Bhattacharya, Kaustuv; Clark, Damian; Christodoulou, John; Ellaway, Carolyn; Farrar, Michelle; Pitt, Matthew; Sampaio, Hugo; Ware, Tyson L; Wedatilake, Yehani; Thorburn, David R; Ryan, Monique M; Ouvrier, Robert

2016-09-01

Peripheral nerve involvement is common in mitochondrial disease but often unrecognised due to the prominent central nervous system features. Identification of the underlying neuropathy may assist syndrome classification, targeted genetic testing and rehabilitative interventions. Clinical data and the results of nerve conduction studies were obtained retrospectively from the records of four tertiary children's hospital metabolic disease, neuromuscular or neurophysiology services. Nerve conductions studies were also performed prospectively on children attending a tertiary metabolic disease service. Results were classified and analysed according to the underlying genetic cause. Nerve conduction studies from 27 children with mitochondrial disease were included in the study (mitochondrial DNA (mtDNA) - 7, POLG - 7, SURF1 - 10, PDHc deficiency - 3). Four children with mtDNA mutations had a normal study while three had mild abnormalities in the form of an axonal sensorimotor neuropathy when not acutely unwell. One child with MELAS had a severe acute axonal motor neuropathy during an acute stroke-like episode that resolved over 12months. Five children with POLG mutations and disease onset beyond infancy had a sensory ataxic neuropathy with an onset in the second decade of life, while the two infants with POLG mutations had a demyelinating neuropathy. Seven of the 10 children with SURF1 mutations had a demyelinating neuropathy. All three children with PDHc deficiency had an axonal sensorimotor neuropathy. Unlike CMT, the neuropathy associated with mitochondrial disease was not length-dependent. This is the largest study to date of peripheral neuropathy in genetically- classified childhood mitochondrial disease. Characterising the underlying neuropathy may assist with the diagnosis of the mitochondrial syndrome and should be an integral part of the assessment of children with suspected mitochondrial disease. Copyright © 2016 Elsevier B.V. and Mitochondria Research Society. All rights reserved.

[Effects of 1-bromopropane on neurological and hematological changes of female exposed workers].

PubMed

Li, Wei-Hua; Zhou, Zhi-Jun; Wang, Qiang-Yi; Ichihara, Gaku; Takeuchi, Yasuhiro; Ding, Xun-Cheng

2010-05-01

To investigate the health effects of 1-bromopropane (1-BP) on female exposed workers. Four 1-BP manufacturing plants were investigated. Workers were interviewed with questionnaire and examined with neurobehavioral core test battery, nerve conduction velocity tests of nervus tibialis and nervus suralis, vibration sensation test, hematological and biochemical tests. Ambient 1-BP concentration was measured with detection tube, and time-weighed average levels of individual workers were estimated with passive samplers. 1-BP concentration in the plants ranged from 0 to 402.40 mg/m3 (Geomean 32.19 mg/m3). Time-weighted average exposure levels (TWA-8 h) ranged from 0.35 to 535.19 mg/m3 (Geomean 14.08 mg/m3). Compared with the control group, 1-BP exposed workers showed reduced motor nerve conduction velocity [(44.8 +/- 8.7) m/s] and sensory nerve conduction velocity [(45.5 +/- 4.9) m/s], prolonged distal latency [(7.5 +/- 2.1) ms], reduced toe vibration perception, and altered neurobehavior parameters(POMS vigor, tension, anxiety, confusion) significantly (P < 0.05). As to hematological and biochemical indicators, the exposed workers showed decreased white blood cell count [(5.6 +/- 2.17) x 10(3)/microl], red blood cell count [(3.9 +/- 0.4) x 10(6)/microl], hemoglobin [(121.1 +/- 14.5) g/L] and creatine kinase [(82.0 +/- 27.5) IU/L] (P < 0.05), and increased serum total protein (8.0 +/- 0.5 g/dl), lactate dehydrogenase [(335.2 +/- 356.6) IU/L], thyroid-stimulating hormone [(3.6 +/- 2.3) microIU/ml] and follicle-stimulating hormone levels (18.7 +/- 24.4 mIU/ml) (P < 0.05). 1-BP exposure may affect peripheral nerves and central nervous system, and lead to abnormal hematological and biomedical indicators.

The WISTAH hand study: a prospective cohort study of distal upper extremity musculoskeletal disorders.

PubMed

Garg, Arun; Hegmann, Kurt T; Wertsch, Jacqueline J; Kapellusch, Jay; Thiese, Matthew S; Bloswick, Donald; Merryweather, Andrew; Sesek, Richard; Deckow-Schaefer, Gwen; Foster, James; Wood, Eric; Kendall, Richard; Sheng, Xiaoming; Holubkov, Richard

2012-06-06

Few prospective cohort studies of distal upper extremity musculoskeletal disorders have been performed. Past studies have provided somewhat conflicting evidence for occupational risk factors and have largely reported data without adjustments for many personal and psychosocial factors. A multi-center prospective cohort study was incepted to quantify risk factors for distal upper extremity musculoskeletal disorders and potentially develop improved methods for analyzing jobs. Disorders to analyze included carpal tunnel syndrome, lateral epicondylalgia, medial epicondylalgia, trigger digit, deQuervain's stenosing tenosynovitis and other tendinoses. Workers have thus far been enrolled from 17 different employment settings in 3 diverse US states and performed widely varying work. At baseline, workers undergo laptop administered questionnaires, structured interviews, two standardized physical examinations and nerve conduction studies to ascertain demographic, medical history, psychosocial factors and current musculoskeletal disorders. All workers' jobs are individually measured for physical factors and are videotaped. Workers are followed monthly for the development of musculoskeletal disorders. Repeat nerve conduction studies are performed for those with symptoms of tingling and numbness in the prior six months. Changes in jobs necessitate re-measure and re-videotaping of job physical factors. Case definitions have been established. Point prevalence of carpal tunnel syndrome is a combination of paraesthesias in at least two median nerve-served digits plus an abnormal nerve conduction study at baseline. The lifetime cumulative incidence of carpal tunnel syndrome will also include those with a past history of carpal tunnel syndrome. Incident cases will exclude those with either a past history or prevalent cases at baseline. Statistical methods planned include survival analyses and logistic regression. A prospective cohort study of distal upper extremity musculoskeletal disorders is underway and has successfully enrolled over 1,000 workers to date.

Imaging the Facial Nerve: A Contemporary Review

PubMed Central

Gupta, Sachin; Mends, Francine; Hagiwara, Mari; Fatterpekar, Girish; Roehm, Pamela C.

2013-01-01

Imaging plays a critical role in the evaluation of a number of facial nerve disorders. The facial nerve has a complex anatomical course; thus, a thorough understanding of the course of the facial nerve is essential to localize the sites of pathology. Facial nerve dysfunction can occur from a variety of causes, which can often be identified on imaging. Computed tomography and magnetic resonance imaging are helpful for identifying bony facial canal and soft tissue abnormalities, respectively. Ultrasound of the facial nerve has been used to predict functional outcomes in patients with Bell's palsy. More recently, diffusion tensor tractography has appeared as a new modality which allows three-dimensional display of facial nerve fibers. PMID:23766904

Abnormal Origin and Course of the Accessory Phrenic Nerve: Case Report.

PubMed

Paraskevas, George; Koutsouflianiotis, Konstantinos; Kitsoulis, Panagiotis; Spyridakis, Ioannis

In the current cadaveric study an unusual sizeable accessory phrenic nerve (APN) was encountered emerging from the trunk of the supraclavicular nerves and forming a triangular loop that was anastomosing with the phrenic nerve. That neural loop surrounded the superficial cervical artery which displayed a spiral course. The form of a triangular loop of APN involving the aforementioned artery and originating from the supraclavicular nerve to the best of our knowledge has not been documented previously in the literature. The variable morphological features of the APN along with its clinical applications are briefly discussed.

Enhancement of multiple cranial and spinal nerves in vanishing white matter: expanding the differential diagnosis.

PubMed

Eluvathingal Muttikkal, Thomas Jose; Montealegre, Denia Ramirez; Matsumoto, Julie Ann

2018-03-01

Abnormal cranial or spinal nerve contrast enhancement on MRI in cases of suspected pediatric leukodystrophy is recognized as an important clue to the diagnosis of either metachromatic leukodystrophy or globoid cell leukodystrophy (Krabbe disease). We report a case of genetically confirmed childhood vanishing white matter with enhancement of multiple cranial and spinal nerves in addition to the more typical intracranial findings. This case expands the limited differential diagnosis of cranial nerve or spinal nerve enhancement in cases of suspected leukodystrophy and may aid in more efficient work-up and earlier diagnosis of vanishing white matter.

Retinal nerve fibre thickness measured with optical coherence tomography accurately detects confirmed glaucomatous damage.

PubMed

Hood, D C; Harizman, N; Kanadani, F N; Grippo, T M; Baharestani, S; Greenstein, V C; Liebmann, J M; Ritch, R

2007-07-01

To assess the accuracy of optical coherence tomography (OCT) in detecting damage to a hemifield, patients with hemifield defects confirmed on both static automated perimetry (SAP) and multifocal visual evoked potentials (mfVEP) were studied. Eyes of 40 patients with concomitant SAP and mfVEP glaucomatous loss and 25 controls underwent OCT retinal nerve fibre layer (RNFL), mfVEP and 24-2 SAP tests. For the mfVEP and 24-2 SAP, a hemifield was defined as abnormal based upon cluster criteria. On OCT, a hemifield was considered abnormal if one of the five clock hour sectors (3 and 9 o'clock excluded) was at <1% (red) or two were at <5% (yellow). Seventy seven (43%) of the hemifields were abnormal on both mfVEP and SAP tests. The OCT was abnormal for 73 (95%) of these. Only 1 (1%) of the 100 hemifields of the controls was abnormal on OCT. Sensitivity/specificity (one eye per person) was 95/98%. The OCT RNFL test accurately detects abnormal hemifields confirmed on both subjective and objective functional tests. Identifying abnormal hemifields with a criterion of 1 red (1%) or 2 yellow (5%) clock hours may prove useful in clinical practice.

Restudy of malformations of the internal auditory meatus, cochlear nerve canal and cochlear nerve.

PubMed

Li, Youjin; Yang, Jun; Liu, Jinfen; Wu, Hao

2015-07-01

The present study aims to restudy the correlation between the internal auditory meatus (IAM), the cochlear nerve canal (CNC), the cochlear nerve (CN) and inner ear malformations. In this retrospective study design, the abnormal diameter of the IAM, CNC and CN in patients with any kind of inner ear malformations was evaluated using multi-slice spiral computed tomography (MSCT) (37 patients) and magnetic resonance imaging (MRI) (18 patients). Of 37 MSCT-diagnosed patients, 2 had IAM atresia, 11 IAM stenosis, 22 enlarged IAM, and 2 normal IAM with an abnormal CN. MRI diagnoses of 18 patients revealed 8 cases of aplastic CN, 6 hypoplastic CN, and 4 normal CN. CNC stenosis was associated with CN hypoplasia (P < 0.001). Patients with absent or stenotic IAM had less CN development than those with normal or enlarged IAM (P = 0.001). We propose a modification of the existing classification systems with a view to distinguishing malformations of the IAM, CNC and CN.

The Use of Quantitative SPECT/CT Imaging to Assess Residual Limb Health

DTIC Science & Technology

2017-10-01

loss in 2020.4 BACKGROUND The integrity of the vasculature, nerves, and soft tissue within the extremities is of high importance, as an impairment or...formation, and vascular abnormalities .6 Therefore, effective diagnosis can be critical in directing the medical treatment of patients. Standard noninva...and identify damage to their nor- mal fasicular pattern, nerve swelling or thickening, loss of nerve bundle integrity , and development of neuromas

Different nerve ultrasound patterns in charcot-marie-tooth types and hereditary neuropathy with liability to pressure palsies.

PubMed

Padua, Luca; Coraci, Daniele; Lucchetta, Marta; Paolasso, Ilaria; Pazzaglia, Costanza; Granata, Giuseppe; Cacciavillani, Mario; Luigetti, Marco; Manganelli, Fiore; Pisciotta, Chiara; Piscosquito, Giuseppe; Pareyson, Davide; Briani, Chiara

2018-01-01

Nerve ultrasound in Charcot-Marie-Tooth (CMT) disease has focused mostly on the upper limbs. We performed an evaluation of a large cohort of CMT patients in which we sonographically characterized nerve abnormalities in different disease types, ages, and nerves. Seventy patients affected by different CMT types and hereditary neuropathy with liability to pressure palsies (HNPP) were evaluated, assessing median, ulnar, fibular, tibial, and sural nerves bilaterally. Data were correlated with age. Nerve dimensions were correlated with CMT type, age, and nerve site. Nerves were larger in demyelinating than in axonal neuropathies. Nerve involvement was symmetric. CMT1 patients had larger nerves than did patients with other CMT types. Patients with HNPP showed enlargement at entrapment sites. Our study confirms the general symmetry of ultrasound nerve patterns in CMT. When compared with ultrasound studies of nerves of the upper limbs, evaluation of the lower limbs did not provide additional information. Muscle Nerve 57: E18-E23, 2018. © 2017 Wiley Periodicals, Inc.

Gait abnormalities caused by selective anesthesia of the suprascapular nerve in horses.

PubMed

Devine, Dustin V; Jann, Henry W; Payton, Mark E

2006-05-01

To assess gait abnormalities associated with selective anesthesia of the suprascapular nerve (SSN) achieved by use of perineural catheterization and thereby determine the function of that nerve as it relates to gait in horses. 3 adult horses with no preexisting clinically apparent lameness at a walk. Each horse was anesthetized; the right SSN was exposed surgically for placement of a perineural catheter to permit delivery of 1 mL of 2% mepivacaine hydrochloride. Six hours after recovery from anesthesia, each horse was videotaped while walking (50-step data acquisition period) before and after administration of mepivacaine. Videotapes were reviewed and the proportion of abnormal steps before and after selective SSN anesthesia was assessed. A step was considered abnormal if a marked amount of scapulohumeral joint instability (ie, lateral luxation of the proximal portion of the humerus) was observed during the weight-bearing phase of the stride. Clinically apparent gait dysfunction was detected in all 3 horses following perineural administration of the local anesthetic agent. Anesthesia of the SSN resulted in scapulohumeral joint instability as evidenced by consistent lateral excursion of the shoulder region during the weight-bearing phase of gait at a walk. The proportion of abnormal steps before and after SSN anesthesia was significantly different in all 3 horses. These data support the role of the SSN in shoulder joint stability in horses and define SSN dysfunction as 1 mechanism by which the syndrome and gait dysfunction clinically referred to as sweeny may develop.

Exacerbation of Charcot-Marie-Tooth type 2E neuropathy following traumatic nerve injury

PubMed Central

Villalon, Eric; Dale, Jeffrey M.; Jones, Maria; Shen, Hailian; Garcia, Michael L.

2018-01-01

Charcot-Marie-Tooth disease (CMT) is the most commonly inherited peripheral neuropathy. CMT disease signs include distal limb neuropathy, abnormal gait, sensory defects, and deafness. We generated a novel line of CMT2E mice expressing hNF-LE397K, which displayed muscle atrophy of the lower limbs without denervation, proximal reduction in large caliber axons, and decreased nerve conduction velocity. In this study, we challenged wild type, hNF-L, and hNF-LE397K mice with crush injury to the sciatic nerve. We analyzed functional recovery by measuring toe spread and analyzed gaitusing the Catwalk system. hNF-LE397K mice demonstrated reduced recovery from nerve injury consistent with increased susceptibility to neuropathy observed in CMT patients. In addition, hNF-LE397K developed a permanent reduction in their ability to weight bear, increased mechanical allodynia, and premature gait shift in the injured limb, which led to increasingly disrupted interlimb coordination in hNF-LE397K. Exacerbation of neuropathy after injury and identification of gait alterations in combination with previously described pathology suggests that hNF-LE397K mice recapitulate many of clinical signs associated with CMT2. Therefore, hNF-LE397K mice provide a model for determining the efficacy of novel therapies. PMID:26423936

Sacral neuromodulation for women with Fowler's syndrome.

PubMed

Swinn, M J; Kitchen, N D; Goodwin, R J; Fowler, C J

2000-10-01

Neuromodulation of the sacral nerves has been found to be an effective therapy for a variety of lower urinary tract dysfunctions. The reported success rate for the period of trial stimulation (peripheral nerve evaluation test) prior to permanent implantation of a sacral nerve stimulator is variable, but generally reported to be in the region of 30-50%. We present here the results of the peripheral nerve evaluation test in 38 patients with urinary retention. 34 of the 38 had been found to have an abnormality of their striated urethral sphincter on electromyography using a concentric needle electrode, i.e., they had the disorder which was described by Fowler and coworkers in 1988. The overall success rate in this group was 68%. We believe that our relatively high success rate is due to sacral neuromodulation working via a mechanism which involves the urethral sphincter, an abnormality which had been demonstrated in 89% of these patients. Twelve of the patients subsequently underwent permanent implantation of a sacral nerve stimulator, and all of them have experienced a return of voiding. However, in 2 patients, there is a persisting need for self-catheterization. There is, however, a high reoperation rate.

Recurrent largngeal nerve paralysis: a laryngographic and computed tomographic study

DOE Office of Scientific and Technical Information (OSTI.GOV)

Agha, F.P.

Vocal cord paralysis is a relatively common entity, usually resulting from a pathologic process of the vagus nerve or its recurrent larynegeal branch. It is rarely caused by intralargngeal lesions. Four teen patients with recurrent laryngeal nerve paralysis (RLNP) were evaluated by laryngography, computed tomography (CT), or both. In the evaluation of the paramedian cord, CT was limited in its ability to differentiate between tumor or RLNP as the cause of the fixed cord, but it yielded more information than laryngography on the structural abnormalities of the larynx and pre-epiglottic and paralaryngeal spaces. Laryngography revealed distinct features of RLNP andmore » is the procedure of choice for evaluation of functional abnormalities of the larynx until further experience with faster CT scanners and dynamic scanning of the larynx is gained.« less

Diagnosis of Lumbar Foraminal Stenosis using Diffusion Tensor Imaging.

PubMed

Eguchi, Yawara; Ohtori, Seiji; Suzuki, Munetaka; Oikawa, Yasuhiro; Yamanaka, Hajime; Tamai, Hiroshi; Kobayashi, Tatsuya; Orita, Sumihisa; Yamauchi, Kazuyo; Suzuki, Miyako; Aoki, Yasuchika; Watanabe, Atsuya; Kanamoto, Hirohito; Takahashi, Kazuhisa

2016-02-01

Diagnosis of lumbar foraminal stenosis remains difficult. Here, we report on a case in which bilateral lumbar foraminal stenosis was difficult to diagnose, and in which diffusion tensor imaging (DTI) was useful. The patient was a 52-year-old woman with low back pain and pain in both legs that was dominant on the right. Right lumbosacral nerve compression due to a massive uterine myoma was apparent, but the leg pain continued after a myomectomy was performed. No abnormalities were observed during nerve conduction studies. Computed tomography and magnetic resonance imaging indicated bilateral L5 lumbar foraminal stenosis. DTI imaging was done. The extraforaminal values were decreased and tractography was interrupted in the foraminal region. Bilateral L5 vertebral foraminal stenosis was treated by transforaminal lumbar interbody fusion and the pain in both legs disappeared. The case indicates the value of DTI for diagnosing vertebral foraminal stenosis.

Neuromuscular Highlights-AAN 2005.

PubMed

Cheema, Zahid; Saperstein, David; Jackson, Carolyn; Newman, Daniel

2006-06-01

Summary of Neuromuscular Presentations at the 57 Annual AAN 2005 meeting in Miami Florida on topics of Facioscapulohumeral muscular dystrophy (FSHD), Duchenne muscular dystrophy (DMD), Diabetic Neuropathy, Charco Marie Tooth disease (CMT), Comparison of injected steroids versus Surgery for carpal tunnel syndrome, Rituximab in Anti-MAG associated polyneuropathy, Cannabis based medicine (CBM) in the treatment of neuropathic pain, utility of skin biopsy with intraepidermal nerve fiber density (IENFD) in sensory complaints, comparing sympathetic skin responses (SSRs) and skin biopsy in diagnosing small fiber sensory neuropathy, Chronic inflammatory demyelinating polyneuropathy (CIDP) clinical and electrophysiologic predictors, affect of limb warming in mild ulnar nerve conduction study (NCS) abnormalities, Tamoxifen affect in ALS, open label study of 3,4 DAP, Pyridostigmine and Ephedrine in fast channel syndrome, Mexilitine as an antimyotonia treatment in myotonic dystrophy (DM1), frontal lobe impairment evaluation in DM1 and DM2 patients and phenotype-genotype correlation in patients with dysferlinopathy.

Imaging of Cranial Nerves III, IV, VI in Congenital Cranial Dysinnervation Disorders

PubMed Central

Kim, Jae Hyoung

2017-01-01

Congenital cranial dysinnervation disorders are a group of diseases caused by abnormal development of cranial nerve nuclei or their axonal connections, resulting in aberrant innervation of the ocular and facial musculature. Its diagnosis could be facilitated by the development of high resolution thin-section magnetic resonance imaging. The purpose of this review is to describe the method to visualize cranial nerves III, IV, and VI and to present the imaging findings of congenital cranial dysinnervation disorders including congenital oculomotor nerve palsy, congenital trochlear nerve palsy, Duane retraction syndrome, Möbius syndrome, congenital fibrosis of the extraocular muscles, synergistic divergence, and synergistic convergence. PMID:28534340

Quantitative muscle ultrasound is useful for evaluating secondary axonal degeneration in chronic inflammatory demyelinating polyneuropathy.

PubMed

Hokkoku, Keiichi; Matsukura, Kiyoshi; Uchida, Yudai; Kuwabara, Midori; Furukawa, Yuichi; Tsukamoto, Hiroshi; Hatanaka, Yuki; Sonoo, Masahiro

2017-10-01

In chronic inflammatory demyelinating polyneuropathy (CIDP), exclusion of secondary axonal degeneration is challenging with conventional methods such as nerve conduction study (NCS), needle electromyography, and nerve biopsy. Increased echo intensity (EI) and decreased muscle thickness (MT) identified on muscle ultrasound (MUS) examination represent muscle denervation due to axonal degeneration in neurogenic disorders, suggesting MUS as a new tool to detect secondary axonal degeneration in patients with CIDP. EI and MT of abductor pollicis brevis, abductor digiti minimi, and first dorsal interosseous muscles were measured in 16 CIDP patients. Raw values were converted into z -scores using data from 60 normal controls (NCs). Six of 45 muscles showed abnormally high EI and low MT, suggesting denervation following secondary axonal degeneration. These six muscles belonged to two patients with long disease history, unresponsiveness to treatment, and long interval from onset to initial therapy. There were no significant differences in EI and MT ( p  = .23 and .67, respectively) between the CIDP and NC groups, although NCS results revealed obvious demyelinating abnormalities in all CIDP patients, suggesting the fact that muscle structures will be preserved, and EI and MT will not change unless secondary axonal degeneration occurs in CIDP. MUS is a promising tool for evaluating secondary axonal degeneration in patients with CIDP.

Brainstem auditory and somatosensory evoked potentials in relation to clinical and neuroimaging findings in Chiari type 1 malformation.

PubMed

Moncho, Dulce; Poca, Maria-Antonia; Minoves, Teresa; Ferré, Alejandro; Rahnama, Kimia; Sahuquillo, Juan

2015-04-01

The aim of this study was to describe the abnormalities found in the recordings of evoked potentials (EPs), in particular those of brainstem auditory evoked potentials and somatosensory evoked potentials, in a homogeneous series of patients with Chiari type 1 malformation (CM-1) and study their relationship with clinical symptoms and malformation severity. CM-1 is characterized by cerebellar tonsils that descend below the foramen magnum and may be associated with EP alterations. However, only a small number of authors have described these tests in CM-1, and the patient groups studied to date have been small and heterogeneous. The clinical findings, neuroimages, and EP findings were retrospectively studied in a cohort of 50 patients with CM-1. Seventy percent of patients had EP abnormalities (brainstem auditory evoked potential: 52%, posterior tibial nerve somatosensory evoked potential: 42%, and median nerve somatosensory evoked potential: 34%). The most frequent alteration was an increased central conduction time. Morphometric measurements differed between the normal and pathological groups, although no statistical significance was found when comparing these groups. A high percentage of patients with CM-1 show EP alterations regardless of their clinical or radiological findings, thus highlighting the necessity of performing these tests, especially in patients with few or no symptoms.

A novel NF1 mutation in a Chinese patient with giant café-au-lait macule in neurofibromatosis type 1 associated with a malignant peripheral nerve sheath tumor and bone abnormality.

PubMed

Tong, H-X; Li, M; Zhang, Y; Zhu, J; Lu, W-Q

2012-08-29

Neurofibromatosis type 1 (NF1; OMIM#162200) is a common neurocutaneous disorder that is characterized by multiple café-au-lait, skinfold freckling, Lisch nodules, and neurofibromas. Mutations in the NF1 gene, which encodes the neurofibromin protein, have been identified as the pathogenic gene of NF1. In this study, we present a clinical and molecular study of a Chinese patient with giant café-au-lait in NF1. The patient showed >6 café-au-lait spots on the body, axillary freckling, and multiple subcutaneous neurofibromas. He also had a malignant peripheral nerve sheath tumor and bone abnormalities. The germline mutational analysis of the NF1 gene revealed a novel missense mutation in exon 13. It is a novel heterozygous nucleotide G>A transition at position 2241 of the NF1 gene. We found no mutation in malignant peripheral nerve sheath tumor DNA from this patient. This expands the database for NF1 gene mutations in NF1. Its absence in the normal chromosomes suggests that it is responsible for the NF1 phenotype. To our knowledge, this is the first case of giant café-au-lait macule in NF1 associated with a malignant peripheral nerve sheath tumor and bone abnormality.

Positioning patients for spine surgery: Avoiding uncommon position-related complications

PubMed Central

Kamel, Ihab; Barnette, Rodger

2014-01-01

Positioning patients for spine surgery is pivotal for optimal operating conditions and operative-site exposure. During spine surgery, patients are placed in positions that are not physiologic and may lead to complications. Perioperative peripheral nerve injury (PPNI) and postoperative visual loss (POVL) are rare complications related to patient positioning during spine surgery that result in significant patient disability and functional loss. PPNI is usually due to stretch or compression of the peripheral nerve. PPNI may present as a brachial plexus injury or as an isolated injury of single nerve, most commonly the ulnar nerve. Understanding the etiology, mechanism and pattern of injury with each type of nerve injury is important for the prevention of PPNI. Intraoperative neuromonitoring has been used to detect peripheral nerve conduction abnormalities indicating peripheral nerve stress under general anesthesia and to guide modification of the upper extremity position to prevent PPNI. POVL usually results in permanent visual loss. Most cases are associated with prolonged spine procedures in the prone position under general anesthesia. The most common causes of POVL after spine surgery are ischemic optic neuropathy and central retinal artery occlusion. Posterior ischemic optic neuropathy is the most common cause of POVL after spine surgery. It is important for spine surgeons to be aware of POVL and to participate in safe, collaborative perioperative care of spine patients. Proper education of perioperative staff, combined with clear communication and collaboration while positioning patients in the operating room is the best and safest approach. The prevention of uncommon complications of spine surgery depends primarily on identifying high-risk patients, proper positioning and optimal intraoperative management of physiological parameters. Modification of risk factors extrinsic to the patient may help reduce the incidence of PPNI and POVL. PMID:25232519

Vincristine-induced neuropathy in pediatric patients with acute lymphoblastic leukemia in Oman: Frequent autonomic and more severe cranial nerve involvement.

PubMed

Nazir, Hanan F; AlFutaisi, Amna; Zacharia, Mathew; Elshinawy, Mohamed; Mevada, Surekha T; Alrawas, Abdulhakim; Khater, Doaa; Jaju, Deepali; Wali, Yasser

2017-12-01

Vincristine (VCR) induced peripheral neuropathy is a common complication in children with acute lymphoblastic leukemia (ALL). A retrospective data analysis over an interval of 10 years (2006-2016) of all children with ALL seen at Sultan Qaboos University Hospital was carried out. Electronic medical records of eligible patients were reviewed. Patients with clinical evidence of neuropathy and abnormal nerve conduction studies (NCSs) were included in the study. Nineteen (nine females and 10 males) out of 103 pediatric patients developed VCR-related neuropathy, and their age ranged between 2.5 and 14 years. Symptoms started after 2-11 doses of VCR. All 19 patients had documented peripheral neuropathy on NCSs. The autonomic nervous system and cranial nerves affection was relatively common in our patients; two presented with bradycardia, two patients with unexplained tachycardia, and five had abdominal pain and constipation, complicated by typhlitis in two patients. One patient developed unilateral hearing loss. Two patients developed severe life-threatening cranial nerve involvement with bilateral ptosis and recurrent laryngeal nerve involvement presented as vocal cord paralysis, hoarseness of voice, frequent chocking, and aspiration episodes. Peripheral neuropathy was the commonest form of VCR-related neuropathy. Autonomic neuropathy was relatively common in our patients. Cranial neuropathy is a serious side effect of VCR that can be severe, involving multiple cranial nerves and needs prompt recognition and management. Concomitant administration of pyridoxine and pyridostigmine does not seem to protect against further neurological damage in some patients. © 2017 Wiley Periodicals, Inc.

The vascular and neurogenic factors associated with erectile dysfunction in patients after pelvic fractures.

PubMed

Guan, Yong; Wendong, Sun; Zhao, Shengtian; Liu, Tongyan; Liu, Yuqiang; Zhang, Xiulin; Yuan, Mingzhen

2015-01-01

Erectile dysfunction (ED) is a common complication of pelvic fractures. To identify the vascular and neurogenic factors associated with ED, 120 patients admitted with ED after traumatic pelvic fracture between January 2009 and June 2013 were enrolled in this study. All patients answered the International Index of Erectile Function (IIEF-5) questionnaire. Nocturnal penile tumescence (NPT) testing confirmed the occurrence of ED in 96 (80%) patients on whom penile duplex ultrasound and neurophysiological testing were further performed. Of these ED patients 29 (30%) were demonstrated only with vascular abnormality, 41 (42.7%) were detected only with neural abnormality, 26 (27.1%) revealed mixed abnormalities. Of the 55 patients (29+26) with vascular problems, 7 patients (12.7%) with abnormal arterial response to intracavernous injection of Bimix (15mg papaverine and 1mg phentolamine), 31 (56.4%) with corporal veno-occlusive dysfunction and 17 (30.9%) had both problems. Of the 67 (41+26) patients with abnormal neurophysiological outcomes, 51 (76.1%) with abnormal bulbocavernosus re?ex (BCR), 20 (29.9%) with pathological pudendal nerve evoked potentials (PDEPs) and 25 (37.3%) with abnormal posterior tibial somatosensory nerve evoked potentials (PTSSEPs). Our observation indicated that neurogenic factors are important for the generation of ED in patients with pelvic fracture; venous impotence is more common than arteriogenic ED.

Retinal nerve fibre thickness measured with optical coherence tomography accurately detects confirmed glaucomatous damage

PubMed Central

Hood, D C; Harizman, N; Kanadani, F N; Grippo, T M; Baharestani, S; Greenstein, V C; Liebmann, J M; Ritch, R

2007-01-01

Aim To assess the accuracy of optical coherence tomography (OCT) in detecting damage to a hemifield, patients with hemifield defects confirmed on both static automated perimetry (SAP) and multifocal visual evoked potentials (mfVEP) were studied. Methods Eyes of 40 patients with concomitant SAP and mfVEP glaucomatous loss and 25 controls underwent OCT retinal nerve fibre layer (RNFL), mfVEP and 24‐2 SAP tests. For the mfVEP and 24‐2 SAP, a hemifield was defined as abnormal based upon cluster criteria. On OCT, a hemifield was considered abnormal if one of the five clock hour sectors (3 and 9 o'clock excluded) was at <1% (red) or two were at <5% (yellow). Results Seventy seven (43%) of the hemifields were abnormal on both mfVEP and SAP tests. The OCT was abnormal for 73 (95%) of these. Only 1 (1%) of the 100 hemifields of the controls was abnormal on OCT. Sensitivity/specificity (one eye per person) was 95/98%. Conclusions The OCT RNFL test accurately detects abnormal hemifields confirmed on both subjective and objective functional tests. Identifying abnormal hemifields with a criterion of 1 red (1%) or 2 yellow (5%) clock hours may prove useful in clinical practice. PMID:17301118

Losartan corrects abnormal frequency response of renal vasculature in congestive heart failure.

PubMed

DiBona, Gerald F; Sawin, Linda L

2003-11-01

In congestive heart failure, renal blood flow is decreased and renal vascular resistance is increased in a setting of increased activity of both the sympathetic nervous and renin-angiotensin systems. The renal vasoconstrictor response to renal nerve stimulation is enhanced. This is associated with an abnormality in the low-pass filter function of the renal vasculature wherein higher frequencies (> or =0.01 Hz) within renal sympathetic nerve activity are not normally attenuated and are passed into the renal blood flow signal. This study tested the hypothesis that excess angiotensin II action mediates the abnormal frequency response characteristics of the renal vasculature in congestive heart failure. In anesthetized rats, the renal vasoconstrictor response to graded frequency renal nerve stimulation was significantly greater in congestive heart failure than in control rats. Losartan attenuated the renal vasoconstrictor response to a significantly greater degree in congestive heart failure than in control rats. In control rats, the frequency response of the renal vasculature was that of a first order (-20 dB/frequency decade) low-pass filter with a corner frequency (-3 dB, 30% attenuation) of 0.002 Hz and 97% attenuation (-30 dB) at > or =0.1 Hz. In congestive heart failure rats, attenuation did not exceed 45% (-5 dB) over the frequency range of 0.001-0.6 Hz. The frequency response of the renal vasculature was not affected by losartan treatment in control rats but was completely restored to normal by losartan treatment in congestive heart failure rats. The enhanced renal vasoconstrictor response to renal nerve stimulation and the associated abnormality in the frequency response characteristics of the renal vasculature seen in congestive heart failure are mediated by the action of angiotensin II on renal angiotensin II AT1 receptors.

Ultrasonographic identification of nerve pathology in neuralgic amyotrophy: Enlargement, constriction, fascicular entwinement, and torsion.

PubMed

Arányi, Zsuzsanna; Csillik, Anita; Dévay, Katalin; Rosero, Maja; Barsi, Péter; Böhm, Josef; Schelle, Thomas

2015-10-01

The aim of this study was to characterize the ultrasonographic findings on nerves in neuralgic amyotrophy. Fourteen patients with neuralgic amyotrophy were examined using high-resolution ultrasound. Four types of abnormalities were found: (1) focal or diffuse nerve/fascicle enlargement (57%); (2) incomplete nerve constriction (36%); (3) complete nerve constriction with torsion (50%; hourglass-like appearance); and (4) fascicular entwinement (28%). Torsions were confirmed intraoperatively and were seen on the radial nerve in 85% of patients. A significant correlation was found between no spontaneous recovery of nerve function and constriction/torsion/fascicular entwinement (P = 0.007). Ultrasonographic nerve pathology in neuralgic amyotrophy varies in order of severity from nerve enlargement to constriction to nerve torsion, with treatment ranging from conservative to surgical. We postulate that the constriction caused by inflammation is the precursor of torsion and that development of nerve torsion is facilitated by the rotational movements of limbs. © 2015 Wiley Periodicals, Inc.

Peripheral neuropathy in genetically characterized patients with mitochondrial disorders: A study from south India.

PubMed

Bindu, Parayil Sankaran; Govindaraju, Chikanna; Sonam, Kothari; Nagappa, Madhu; Chiplunkar, Shwetha; Kumar, Rakesh; Gayathri, Narayanappa; Bharath, M M Srinivas; Arvinda, Hanumanthapura R; Sinha, Sanjib; Khan, Nahid Akthar; Govindaraj, Periyasamy; Nunia, Vandana; Paramasivam, Arumugam; Thangaraj, Kumarasamy; Taly, Arun B

2016-03-01

There are relatively few studies, which focus on peripheral neuropathy in large cohorts of genetically characterized patients with mitochondrial disorders. This study sought to analyze the pattern of peripheral neuropathy in a cohort of patients with mitochondrial disorders. The study subjects were derived from a cohort of 52 patients with a genetic diagnosis of mitochondrial disorders seen over a period of 8 years (2006-2013). All patients underwent nerve conduction studies and those patients with abnormalities suggestive of peripheral neuropathy were included in the study. Their phenotypic features, genotype, pattern of peripheral neuropathy and nerve conduction abnormalities were analyzed retrospectively. The study cohort included 18 patients (age range: 18 months-50 years, M:F- 1.2:1).The genotype included mitochondrial DNA point mutations (n=11), SURF1 mutations (n=4) and POLG1(n=3). Axonal neuropathy was noted in 12 patients (sensori-motor:n=4; sensory:n=4; motor:n=4) and demyelinating neuropathy in 6. Phenotype-genotype correlations revealed predominant axonal neuropathy in mtDNA point mutations and demyelinating neuropathy in SURF1. Patients with POLG related disorders had both sensory ataxic neuropathy and axonal neuropathy. A careful analysis of the family history, clinical presentation, biochemical, histochemical and structural analysis may help to bring out the mitochondrial etiology in patients with peripheral neuropathy and may facilitate targeted gene testing. Presence of demyelinating neuropathy in Leigh's syndrome may suggest underlying SURF1 mutations. Sensory ataxic neuropathy with other mitochondrial signatures should raise the possibility of POLG related disorder. Copyright © 2015. Published by Elsevier B.V.

Acute corneal epithelial debridement unmasks the corneal stromal nerve responses to ocular stimulation in rats: implications for abnormal sensations of the eye.

PubMed

Hirata, Harumitsu; Mizerska, Kamila; Dallacasagrande, Valentina; Guaiquil, Victor H; Rosenblatt, Mark I

2017-05-01

It is widely accepted that the mechanisms for transducing sensory information reside in the nerve terminals. Occasionally, however, studies have appeared demonstrating that similar mechanisms may exist in the axon to which these terminals are connected. We examined this issue in the cornea, where nerve terminals in the epithelial cell layers are easily accessible for debridement, leaving the underlying stromal (axonal) nerves undisturbed. In isoflurane-anesthetized rats, we recorded extracellularly from single trigeminal ganglion neurons innervating the cornea that are excited by ocular dryness and cooling: low-threshold (<2°C cooling) and high-threshold (>2°C) cold-sensitive plus dry-sensitive neurons playing possible roles in tearing and ocular pain. We found that the responses in both types of neurons to dryness, wetness, and menthol stimuli were effectively abolished by the debridement, indicating that their transduction mechanisms lie in the nerve terminals. However, some responses to the cold, heat, and hyperosmolar stimuli in low-threshold cold-sensitive plus dry-sensitive neurons still remained. Surprisingly, the responses to heat in approximately half of the neurons were augmented after the debridement. We were also able to evoke these residual responses and follow the trajectory of the stromal nerves, which we subsequently confirmed histologically. The residual responses always disappeared when the stromal nerves were cut at the limbus, suggesting that the additional transduction mechanisms for these sensory modalities originated most likely in stromal nerves. The functional significance of these residual and enhanced responses from stromal nerves may be related to the abnormal sensations observed in ocular disease. NEW & NOTEWORTHY In addition to the traditional view that the sensory transduction mechanisms exist in the nerve terminals, we report here that the proximal axons (stromal nerves in the cornea from which these nerve terminals originate) may also be capable of transducing sensory information. We arrived at this conclusion by removing the epithelial cell layers of the cornea in which the nerve terminals reside but leaving the underlying stromal nerves undisturbed. Copyright © 2017 the American Physiological Society.

Acute corneal epithelial debridement unmasks the corneal stromal nerve responses to ocular stimulation in rats: implications for abnormal sensations of the eye

PubMed Central

Mizerska, Kamila; Dallacasagrande, Valentina; Guaiquil, Victor H.; Rosenblatt, Mark I.

2017-01-01

It is widely accepted that the mechanisms for transducing sensory information reside in the nerve terminals. Occasionally, however, studies have appeared demonstrating that similar mechanisms may exist in the axon to which these terminals are connected. We examined this issue in the cornea, where nerve terminals in the epithelial cell layers are easily accessible for debridement, leaving the underlying stromal (axonal) nerves undisturbed. In isoflurane-anesthetized rats, we recorded extracellularly from single trigeminal ganglion neurons innervating the cornea that are excited by ocular dryness and cooling: low-threshold (<2°C cooling) and high-threshold (>2°C) cold-sensitive plus dry-sensitive neurons playing possible roles in tearing and ocular pain. We found that the responses in both types of neurons to dryness, wetness, and menthol stimuli were effectively abolished by the debridement, indicating that their transduction mechanisms lie in the nerve terminals. However, some responses to the cold, heat, and hyperosmolar stimuli in low-threshold cold-sensitive plus dry-sensitive neurons still remained. Surprisingly, the responses to heat in approximately half of the neurons were augmented after the debridement. We were also able to evoke these residual responses and follow the trajectory of the stromal nerves, which we subsequently confirmed histologically. The residual responses always disappeared when the stromal nerves were cut at the limbus, suggesting that the additional transduction mechanisms for these sensory modalities originated most likely in stromal nerves. The functional significance of these residual and enhanced responses from stromal nerves may be related to the abnormal sensations observed in ocular disease. NEW & NOTEWORTHY In addition to the traditional view that the sensory transduction mechanisms exist in the nerve terminals, we report here that the proximal axons (stromal nerves in the cornea from which these nerve terminals originate) may also be capable of transducing sensory information. We arrived at this conclusion by removing the epithelial cell layers of the cornea in which the nerve terminals reside but leaving the underlying stromal nerves undisturbed. PMID:28250152

Sympathetically maintained pain presenting first as temporomandibular disorder, then as parotid dysfunction.

PubMed

Giri, Subha; Nixdorf, Donald

2007-08-01

Complex regional pain syndrome (CRPS) is a chronic condition that usually affects extremities, such as the arms or legs. It is characterized by intense pain, swelling, redness, hypersensitivity in a region not defined by a single peripheral nerve and additional sudomotor effects, such as excessive sweating. The clinical criteria for the diagnosis of sympathetically maintained pain as outlined by the International Association for the Study of Pain include: Onset following an initiating noxious event (CRPS-type I) or nerve injury (CRPS-type II). Spontaneous allodynia that is not limited to peripheral nerve distribution and is not proportionate to the inciting event; abnormal sudomotor activity, skin blood flow abnormality, edema, other autonomic symptoms; and exclusion of other conditions that may otherwise contribute to the extent of the symptoms. Only 13 cases of CRPS involving sympathetically maintained pain in the head and neck region have been described, and all reported trauma as the identifiable etiologic factor. The case presented here is another occurrence of sympathetically maintained pain in the head and neck region, but without nerve injury as a clear initiating factor.

Cross sectional study to evaluate the effect of duration of type 2 diabetes mellitus on the nerve conduction velocity in diabetic peripheral neuropathy.

PubMed

Hussain, Gauhar; Rizvi, S Aijaz Abbas; Singhal, Sangeeta; Zubair, Mohammad; Ahmad, Jamal

2014-01-01

To study the nerve conduction velocity in clinically undetectable and detectable peripheral neuropathy in type 2 diabetes mellitus with variable duration. This cross sectional study was conducted in diagnosed type 2 diabetes mellitus patients. They were divided in groups: Group I (n=37) with clinically detectable diabetic peripheral neuropathy of shorter duration and Group II (n=27) with clinically detectable diabetic peripheral neuropathy of longer duration. They were compared with T2DM patients (n=22) without clinical neuropathy. Clinical diagnosis was based on neuropathy symptom score (NSS) and neuropathy disability score (NDS) for signs. Nerve conduction velocity was measured in both upper and lower limbs. Median, ulnar, common peroneal and posterior tibial nerves were selected for motor nerve conduction study and median and sural nerves were selected for sensory nerve conduction study. The comparisons were done between nerve conduction velocities of motor and sensory nerves in patients of clinically detectable neuropathy and patients without neuropathy in type 2 diabetes mellitus population. This study showed significant electrophysiological changes with duration of disease. Nerve conduction velocities in lower limbs were significantly reduced even in patients of shorter duration with normal upper limb nerve conduction velocities. Diabetic neuropathy symptom score (NSS) and neuropathy disability score (NDS) can help in evaluation of diabetic sensorimotor polyneuropathy though nerve conduction study is more powerful test and can help in diagnosing cases of neuropathy. Copyright © 2013 Diabetes India. Published by Elsevier Ltd. All rights reserved.

Imaging of Cranial Nerves III, IV, VI in Congenital Cranial Dysinnervation Disorders.

PubMed

Kim, Jae Hyoung; Hwang, Jeong Min

2017-06-01

Congenital cranial dysinnervation disorders are a group of diseases caused by abnormal development of cranial nerve nuclei or their axonal connections, resulting in aberrant innervation of the ocular and facial musculature. Its diagnosis could be facilitated by the development of high resolution thin-section magnetic resonance imaging. The purpose of this review is to describe the method to visualize cranial nerves III, IV, and VI and to present the imaging findings of congenital cranial dysinnervation disorders including congenital oculomotor nerve palsy, congenital trochlear nerve palsy, Duane retraction syndrome, Möbius syndrome, congenital fibrosis of the extraocular muscles, synergistic divergence, and synergistic convergence. © 2017 The Korean Ophthalmological Society.

Reduced ventral cingulum integrity and increased behavioral problems in children with isolated optic nerve hypoplasia and mild to moderate or no visual impairment.

PubMed

Webb, Emma A; O'Reilly, Michelle A; Clayden, Jonathan D; Seunarine, Kiran K; Dale, Naomi; Salt, Alison; Clark, Chris A; Dattani, Mehul T

2013-01-01

To assess the prevalence of behavioral problems in children with isolated optic nerve hypoplasia, mild to moderate or no visual impairment, and no developmental delay. To identify white matter abnormalities that may provide neural correlates for any behavioral abnormalities identified. Eleven children with isolated optic nerve hypoplasia (mean age 5.9 years) underwent behavioral assessment and brain diffusion tensor imaging, Twenty four controls with isolated short stature (mean age 6.4 years) underwent MRI, 11 of whom also completed behavioral assessments. Fractional anisotropy images were processed using tract-based spatial statistics. Partial correlation between ventral cingulum, corpus callosum and optic radiation fractional anisotropy, and child behavioral checklist scores (controlled for age at scan and sex) was performed. Children with optic nerve hypoplasia had significantly higher scores on the child behavioral checklist (p<0.05) than controls (4 had scores in the clinically significant range). Ventral cingulum, corpus callosum and optic radiation fractional anisotropy were significantly reduced in children with optic nerve hypoplasia. Right ventral cingulum fractional anisotropy correlated with total and externalising child behavioral checklist scores (r = -0.52, p<0.02, r = -0.46, p<0.049 respectively). There were no significant correlations between left ventral cingulum, corpus callosum or optic radiation fractional anisotropy and behavioral scores. Our findings suggest that children with optic nerve hypoplasia and mild to moderate or no visual impairment require behavioral assessment to determine the presence of clinically significant behavioral problems. Reduced structural integrity of the ventral cingulum correlated with behavioral scores, suggesting that these white matter abnormalities may be clinically significant. The presence of reduced fractional anisotropy in the optic radiations of children with mild to moderate or no visual impairment raises questions as to the pathogenesis of these changes which will need to be addressed by future studies.

Lower cranial nerves.

PubMed

Soldatos, Theodoros; Batra, Kiran; Blitz, Ari M; Chhabra, Avneesh

2014-02-01

Imaging evaluation of cranial neuropathies requires thorough knowledge of the anatomic, physiologic, and pathologic features of the cranial nerves, as well as detailed clinical information, which is necessary for tailoring the examinations, locating the abnormalities, and interpreting the imaging findings. This article provides clinical, anatomic, and radiological information on lower (7th to 12th) cranial nerves, along with high-resolution magnetic resonance images as a guide for optimal imaging technique, so as to improve the diagnosis of cranial neuropathy. Copyright © 2014 Elsevier Inc. All rights reserved.

Predicting pain relief: Use of pre-surgical trigeminal nerve diffusion metrics in trigeminal neuralgia.

PubMed

Hung, Peter S-P; Chen, David Q; Davis, Karen D; Zhong, Jidan; Hodaie, Mojgan

2017-01-01

Trigeminal neuralgia (TN) is a chronic neuropathic facial pain disorder that commonly responds to surgery. A proportion of patients, however, do not benefit and suffer ongoing pain. There are currently no imaging tools that permit the prediction of treatment response. To address this paucity, we used diffusion tensor imaging (DTI) to determine whether pre-surgical trigeminal nerve microstructural diffusivities can prognosticate response to TN treatment. In 31 TN patients and 16 healthy controls, multi-tensor tractography was used to extract DTI-derived metrics-axial (AD), radial (RD), mean diffusivity (MD), and fractional anisotropy (FA)-from the cisternal segment, root entry zone and pontine segment of trigeminal nerves for false discovery rate-corrected Student's t -tests. Ipsilateral diffusivities were bootstrap resampled to visualize group-level diffusivity thresholds of long-term response. To obtain an individual-level statistical classifier of surgical response, we conducted discriminant function analysis (DFA) with the type of surgery chosen alongside ipsilateral measurements and ipsilateral/contralateral ratios of AD and RD from all regions of interest as prediction variables. Abnormal diffusivity in the trigeminal pontine fibers, demonstrated by increased AD, highlighted non-responders (n = 14) compared to controls. Bootstrap resampling revealed three ipsilateral diffusivity thresholds of response-pontine AD, MD, cisternal FA-separating 85% of non-responders from responders. DFA produced an 83.9% (71.0% using leave-one-out-cross-validation) accurate prognosticator of response that successfully identified 12/14 non-responders. Our study demonstrates that pre-surgical DTI metrics can serve as a highly predictive, individualized tool to prognosticate surgical response. We further highlight abnormal pontine segment diffusivities as key features of treatment non-response and confirm the axiom that central pain does not commonly benefit from peripheral treatments.

The pattern and diagnostic criteria of sensory neuronopathy: a case–control study

PubMed Central

Camdessanché, Jean-Philippe; Jousserand, Guillemette; Ferraud, Karine; Vial, Christophe; Petiot, Philippe; Honnorat, Jérôme

2009-01-01

Acquired sensory neuronopathies encompass a group of paraneoplastic, dysimmune, toxic or idiopathic disorders characterized by degeneration of peripheral sensory neurons in dorsal root ganglia. As dorsal root ganglia cannot easily be explored, the clinical diagnosis of these disorders may be difficult. The question as to whether there exists a common clinical pattern of sensory neuronopathies, allowing the establishment of validated and easy-to-use diagnostic criteria, has not yet been addressed. In this study, logistic regression was used to construct diagnostic criteria on a retrospective study population of 78 patients with sensory neuronopathies and 56 with other sensory neuropathies. For this, sensory neuronopathy was provisionally considered as unambiguous in 44 patients with paraneoplastic disorder or cisplatin treatment and likely in 34 with a dysimmune or idiopathic setting who may theoretically have another form of neuropathy. To test the homogeneity of the sensory neuronopathy population, likely candidates were compared with unambiguous cases and then the whole population was compared with the other sensory neuropathies population. Criteria accuracy was checked on 37 prospective patients referred for diagnosis of sensory neuropathy. In the study population, sensory neuronopathy showed a common clinical and electrophysiological pattern that was independent of the underlying cause, including unusual forms with only patchy sensory loss, mild electrical motor nerve abnormalities and predominant small fibre or isolated lower limb involvement. Logistic regression allowed the construction of a set of criteria that gave fair results with the following combination: ataxia in the lower or upper limbs + asymmetrical distribution + sensory loss not restricted to the lower limbs + at least one sensory action potential absent or three sensory action potentials <30% of the lower limit of normal in the upper limbs + less than two nerves with abnormal motor nerve conduction study in the lower limbs. PMID:19506068

Morphological differences in skeletal muscle atrophy of rats with motor nerve and/or sensory nerve injury★

PubMed Central

Zhao, Lei; Lv, Guangming; Jiang, Shengyang; Yan, Zhiqiang; Sun, Junming; Wang, Ling; Jiang, Donglin

2012-01-01

Skeletal muscle atrophy occurs after denervation. The present study dissected the rat left ventral root and dorsal root at L4-6 or the sciatic nerve to establish a model of simple motor nerve injury, sensory nerve injury or mixed nerve injury. Results showed that with prolonged denervation time, rats with simple motor nerve injury, sensory nerve injury or mixed nerve injury exhibited abnormal behavior, reduced wet weight of the left gastrocnemius muscle, decreased diameter and cross-sectional area and altered ultrastructure of muscle cells, as well as decreased cross-sectional area and increased gray scale of the gastrocnemius muscle motor end plate. Moreover, at the same time point, the pathological changes were most severe in mixed nerve injury, followed by simple motor nerve injury, and the changes in simple sensory nerve injury were the mildest. These findings indicate that normal skeletal muscle morphology is maintained by intact innervation. Motor nerve injury resulted in larger damage to skeletal muscle and more severe atrophy than sensory nerve injury. Thus, reconstruction of motor nerves should be considered first in the clinical treatment of skeletal muscle atrophy caused by denervation. PMID:25337102

Retinopathy of Prematurity

ERIC Educational Resources Information Center

Steinweg, Sue Byrd; Griffin, Harold C.; Griffin, Linda W.; Gingras, Happy

2005-01-01

The eyes of premature infants are especially vulnerable to injury after birth. A serious complication is called retinopathy of prematurity (ROP), which is abnormal growth of the blood vessels in an infant's eye. Retinopathy of prematurity develops when abnormal blood vessels grow and spread throughout the retina, which is the nerve tissue at the…

Visual abnormalities associated with enhanced optic nerve myelination.

PubMed

Yu, Minzhong; Narayanan, S Priyadarshini; Wang, Feng; Morse, Emily; Macklin, Wendy B; Peachey, Neal S

2011-02-16

Expression of the constitutively active serine/threonine kinase Akt in oligodendrocytes results in enhanced myelination in the CNS. Here, we have examined the effects of this Akt overexpression on optic nerve structure and on optic nerve function, assessed using the visual evoked potential (VEP). Transgenic mice have been generated with the Plp promoter driving expression of a modified form of Akt, in which aspartic acids are substituted for Thr308 and Ser473. These Plp-Akt-DD (Akt-DD) mice, and littermate controls, were studied at different ages. Optic nerves were examined anatomically at 2 and 6 months of age. At 2 months of age, optic nerves were substantially thicker in Akt-DD mice, reflecting an increase in myelination of optic nerve axons. By electron microscopy, myelin thickness was increased in Akt-DD optic nerve, with extended paranodal domains having excess paranodal loops, and the density of nodes of Ranvier was reduced, relative to control mice. We recorded VEPs in response to strobe flash ganzfeld stimuli presented after overnight dark- and light-adapted conditions at ages ranging from 1 to 10 months. It was possible to record a clear VEP from Akt-DD mice at all ages examined. At 1 month of age, VEP implicit times were somewhat shorter in Akt-DD transgenic mice than in control animals. Beyond 6months of age, VEP latencies were consistently delayed in Akt-DD transgenic mice. These abnormalities did not reflect an alteration in retinal function as there were no significant differences between ERGs obtained from control or Akt-DD transgenic mice. In young mice, the somewhat faster responses may reflect improved transmission due to increased myelination of optic nerve axons. In older mice, where the Akt-DD optic nerve is markedly thicker than control, it is remarkable that optic nerves continue to function. Copyright © 2010 Elsevier B.V. All rights reserved.

Equine protozoal myeloencephalitis due to Neospora hughesi and equine motor neuron disease in a mule.

PubMed

Finno, Carrie J; Eaton, Joshua Seth; Aleman, Monica; Hollingsworth, Steven R

2010-07-01

A 23-year-old female mule was presented for bilateral ocular abnormalities and an abnormal pelvic limb gait. Anisocoria, unilateral enophthalmos, medial strabismus, ptosis, pupillary light reflex deficits, and bilateral reticulated pigmentary retinopathy were observed on ophthalmic examination. Neurologic abnormalities included right-sided facial nerve paralysis, extensive symmetric muscle atrophy, and asymmetric pelvic limb ataxia with an abnormal pelvic limb gait. A positive titer (1:40) for equine protozoal myeloencephalitis (EPM) associated with Neospora hughesi was obtained from cerebrospinal fluid with minimal (<1 red blood cell/microL) blood contamination. Muscle biopsies of the sacrocaudalis dorsalis medialis muscle revealed predominantly type I neurogenic muscle atrophy, consistent with a diagnosis of equine motor neuron disease (EMND). Treatment included a 2-month course of ponazuril (5 mg/kg PO q24 h), vitamin E (8000 IU PO q24 h), and selenium (2 mg PO q24 h). Clinical improvement was not observed after 2 months although the mule remained stable. Clinical deterioration was reported upon discontinuation of the ponazuril after a 2-month course. Concurrent disease with EPM associated with N. hughesi and EMND should be considered in cases demonstrating cranial nerve abnormalities, pronounced symmetric muscle atrophy, unusual asymmetric gait abnormalities, and reticulated pigmentary retinopathy.

Exacerbation of Charcot-Marie-Tooth type 2E neuropathy following traumatic nerve injury.

PubMed

Villalón, Eric; Dale, Jeffrey M; Jones, Maria; Shen, Hailian; Garcia, Michael L

2015-11-19

Charcot-Marie-Tooth disease (CMT) is the most commonly inherited peripheral neuropathy. CMT disease signs include distal limb neuropathy, abnormal gait, sensory defects, and deafness. We generated a novel line of CMT2E mice expressing hNF-L(E397K), which displayed muscle atrophy of the lower limbs without denervation, proximal reduction in large caliber axons, and decreased nerve conduction velocity. In this study, we challenged wild type, hNF-L and hNF-L(E397K) mice with crush injury to the sciatic nerve. We analyzed functional recovery by measuring toe spread and analyzed gait using the Catwalk system. hNF-L(E397K) mice demonstrated reduced recovery from nerve injury consistent with increased susceptibility to neuropathy observed in CMT patients. In addition, hNF-L(E397K) developed a permanent reduction in their ability to weight bear, increased mechanical allodynia, and premature gait shift in the injured limb, which led to increasingly disrupted interlimb coordination in hNF-L(E397K). Exacerbation of neuropathy after injury and identification of gait alterations in combination with previously described pathology suggests that hNF-L(E397K) mice recapitulate many of clinical signs associated with CMT2. Therefore, hNF-L(E397K) mice provide a model for determining the efficacy of novel therapies. Copyright © 2015 Elsevier B.V. All rights reserved.

Diffusion tensor imaging with quantitative evaluation and fiber tractography of lumbar nerve roots in sciatica.

PubMed

Shi, Yin; Zong, Min; Xu, Xiaoquan; Zou, Yuefen; Feng, Yang; Liu, Wei; Wang, Chuanbing; Wang, Dehang

2015-04-01

To quantitatively evaluate nerve roots by measuring fractional anisotropy (FA) values in healthy volunteers and sciatica patients, visualize nerve roots by tractography, and compare the diagnostic efficacy between conventional magnetic resonance imaging (MRI) and DTI. Seventy-five sciatica patients and thirty-six healthy volunteers underwent MR imaging using DTI. FA values for L5-S1 lumbar nerve roots were calculated at three levels from DTI images. Tractography was performed on L3-S1 nerve roots. ROC analysis was performed for FA values. The lumbar nerve roots were visualized and FA values were calculated in all subjects. FA values decreased in compressed nerve roots and declined from proximal to distal along the compressed nerve tracts. Mean FA values were more sensitive and specific than MR imaging for differentiating compressed nerve roots, especially in the far lateral zone at distal nerves. DTI can quantitatively evaluate compressed nerve roots, and DTT enables visualization of abnormal nerve tracts, providing vivid anatomic information and localization of probable nerve compression. DTI has great potential utility for evaluating lumbar nerve compression in sciatica. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

The vascular and neurogenic factors associated with erectile dysfunction in patients after pelvic fractures

PubMed Central

Guan, Yong; Wendong, Sun; Zhao, Shengtian; Liu, Tongyan; Liu, Yuqiang; Zhang, Xiulin; Yuan, Mingzhen

2015-01-01

ABSTRACT Erectile dysfunction (ED) is a common complication of pelvic fractures. To identify the vascular and neurogenic factors associated with ED, 120 patients admitted with ED after traumatic pelvic fracture between January 2009 and June 2013 were enrolled in this study. All patients answered the International Index of Erectile Function (IIEF-5) questionnaire. Nocturnal penile tumescence (NPT) testing confirmed the occurrence of ED in 96 (80%) patients on whom penile duplex ultrasound and neurophysiological testing were further performed. Of these ED patients 29 (30%) were demonstrated only with vascular abnormality, 41 (42.7%) were detected only with neural abnormality, 26 (27.1%) revealed mixed abnormalities. Of the 55 patients (29+26) with vascular problems, 7 patients (12.7%) with abnormal arterial response to intracavernous injection of Bimix (15mg papaverine and 1mg phentolamine), 31 (56.4%) with corporal veno-occlusive dysfunction and 17 (30.9%) had both problems. Of the 67 (41+26) patients with abnormal neurophysiological outcomes, 51 (76.1%) with abnormal bulbocavernosus reflex (BCR), 20 (29.9%) with pathological pudendal nerve evoked potentials (PDEPs) and 25 (37.3%) with abnormal posterior tibial somatosensory nerve evoked potentials (PTSSEPs). Our observation indicated that neurogenic factors are important for the generation of ED in patients with pelvic fracture; venous impotence is more common than arteriogenic ED. PMID:26689522

Mechanism of gastrointestinal abnormal motor activity induced by cisplatin in conscious dogs.

PubMed

Ando, Hiroyuki; Mochiki, Erito; Ohno, Tetsuro; Yanai, Mitsuhiro; Toyomasu, Yoshitaka; Ogata, Kyoichi; Tabe, Yuichi; Aihara, Ryuusuke; Nakabayashi, Toshihiro; Asao, Takayuki; Kuwano, Hiroyuki

2014-11-14

To investigate whether 5-hydroxytryptamine (serotonin; 5-HT) is involved in mediating abnormal motor activity in dogs after cisplatin administration. After the dogs had been given a 2-wk recovery period, all of them were administered cisplatin, and the motor activity was recorded using strain gauge force transducers. Blood and intestinal fluid samples were collected to measure 5-HT for 24 h. To determine whether 5-HT in plasma or that in intestinal fluids is more closely related to abnormal motor activity we injected 5-HT into the bloodstream and the intestinal tract of the dogs. Cisplatin given intravenously produced abnormal motor activity that lasted up to 5 h. From 3 to 4 h after cisplatin administration, normal intact dogs exhibited retropropagation of motor activity accompanied by emesis. The concentration of 5-HT in plasma reached the peak at 4 h, and that in intestinal fluids reached the peak at 3 h. In normal intact dogs with resection of the vagus nerve that were administered kytril, cisplatin given intravenously did not produce abnormal motor activity. Intestinal serotonin administration did not produce abnormal motor activity, but intravenous serotonin administration did. After the intravenous administration of cisplatin, abnormal motor activity was produced in the involved vagus nerve and in the involved serotonergic neurons via another pathway. This study was the first to determine the relationship between 5-HT and emesis-induced motor activity.

LepVax, a defined subunit vaccine that provides effective pre-exposure and post-exposure prophylaxis of M. leprae infection.

PubMed

Duthie, Malcolm S; Pena, Maria T; Ebenezer, Gigi J; Gillis, Thomas P; Sharma, Rahul; Cunningham, Kelly; Polydefkis, Michael; Maeda, Yumi; Makino, Masahiko; Truman, Richard W; Reed, Steven G

2018-01-01

Sustained elimination of leprosy as a global health concern likely requires a vaccine. The current standard, BCG, confers only partial protection and precipitates paucibacillary (PB) disease in some instances. When injected into mice with the T helper 1 (Th1)-biasing adjuvant formulation Glucopyranosyl Lipid Adjuvant in stable emulsion (GLA-SE), a cocktail of three prioritized antigens (ML2055, ML2380 and ML2028) reduced M. leprae infection levels. Recognition and protective efficacy of a single chimeric fusion protein incorporating these antigens, LEP-F1, was confirmed in similar experiments. The impact of post-exposure immunization was then assessed in nine-banded armadillos that demonstrate a functional recapitulation of leprosy. Armadillos were infected with M. leprae 1 month before the initiation of post-exposure prophylaxis. While BCG precipitated motor nerve conduction abnormalities more rapidly and severely than observed for control infected armadillos, motor nerve injury in armadillos treated three times, at monthly intervals with LepVax was appreciably delayed. Biopsy of cutaneous nerves indicated that epidermal nerve fiber density was not significantly altered in M. leprae -infected animals although Remak Schwann cells of the cutaneous nerves in the distal leg were denser in the infected armadillos. Importantly, LepVax immunization did not exacerbate cutaneous nerve involvement due to M. leprae infection, indicating its safe use. There was no intraneural inflammation but a reduction of intra axonal edema suggested that LepVax treatment might restore some early sensory axonal function. These data indicate that post-exposure prophylaxis with LepVax not only appears safe but, unlike BCG, alleviates and delays the neurologic disruptions caused by M. leprae infection.

Ultrasonography in neuralgic amyotrophy: Sensitivity, spectrum of findings, and clinical correlations.

PubMed

ArÁnyi, Zsuzsanna; Csillik, Anita; DéVay, Katalin; Rosero, Maja; Barsi, PéTer; BÖhm, Josef; Schelle, Thomas

2017-12-01

The aim of this study was to assess the value of ultrasonography in neuralgic amyotrophy. Fifty-three patients with 70 affected nerves were examined with high-resolution ultrasound. The most commonly affected nerve was the anterior interosseous (23%). Ultrasonographic abnormalities in the affected nerves, rather than in the brachial plexus, were observed, with an overall sensitivity of 74%. Findings included the swelling of the nerve/fascicle with or without incomplete/complete constriction and rotational phenomena (nerve torsion and fascicular entwinement). A significant difference was found among the categories of ultrasonographic findings with respect to clinical outcome (P = 0.01). In nerves with complete constriction and rotational phenomena, reinnervation was absent or negligible, indicating surgery was warranted. Ultrasonography may be used as a diagnostic aid in neuralgic amyotrophy, which was hitherto a clinical and electrophysiological diagnosis, and may also help in identifying potential surgical candidates. Muscle Nerve 56: 1054-1062, 2017. © 2017 Wiley Periodicals, Inc.

Neuropsychological study in patients with ALL. Two different CNS prevention therapies--cranial irradiation plus IT methotrexate vs. IT methotrexate alone

DOE Office of Scientific and Technical Information (OSTI.GOV)

Pavlovsky, S.; Fisman, N.; Arizaga, R.

Neurological, psychopedagogic, and psychologic long-term sequelae were evaluated in two groups of ALL patients in continuous CR for more than 2 years treated with two different CNS prophylaxis schemes. Group A, 19 patients received cranial irradiation 2400 rads plus IT MTX-DMT, and group B, 23 patients IT MTX-DMT only during induction and maintenance. All the patients were evaluated by performing neurological examination, EEG, EMG with nerve conduction velocity, CT scans, CSF studies, psychometric and psychologic studies, and neuropsychological evaluation. The most important findings were: 11 patients from group A (58%) showed abnormal CT and only one patient from group Bmore » showed CT abnormalities. The neuropsychologic evaluation (performed by L. Bender technique and Picq-Vayer scale) showed more severe impairment (grade 3-4) in eight patients from group A (42%) and none in group B (p less than 0.001). Higher incidence of abnormalities in group A suggests the existence of more severe sequelae in the patients treated with cranial irradiation plus IT MTX-DMT than with IT MTX-DMT alone.« less

Imaging Characteristics of Children with Auditory Neuropathy Spectrum Disorder

PubMed Central

Roche, Joseph P.; Huang, Benjamin Y.; Castillo, Mauricio; Bassim, Marc K.; Adunka, Oliver F.; Buchman, Craig A.

2013-01-01

Objective To identify and define the imaging characteristics of children with auditory neuropathy spectrum disorder (ANSD). Design Retrospective medical records review and analysis of both temporal bone computed tomography (CT) and magnetic resonance images (MRI) in from children with the diagnosis of ANSD. Setting Tertiary referral center. Patients 118 children with the electrophysiological characteristics of ANSD with available imaging studies for review. Interventions Two neuroradiologists and a neurotologist reviewed each study and consensus descriptions were established. Main outcome measures The type and number of imaging findings were tabulated. Results Sixty-eight (64%) MRIs revealed at least one imaging abnormality while selective use of CT identified 23 (55%) with anomalies. The most prevalent MRI findings included cochlear nerve deficiency (n=51; 28% of 183 nerves), brain abnormalities (n=42; 40% of 106 brains) and prominent temporal horns (n=33, 16% of 212 temporal lobes). The most prevalent CT finding from selective use of CT was cochlear dysplasia (n=13; 31%). Conclusions MRI will identify many abnormalities in children with ANSD that are not readily discernable on CT. Specifically, both developmental and acquired abnormalities of the brain, posterior cranial fossa, and cochlear nerves are not uncommonly seen in this patient population. Inner ear anomalies are well delineated using either imaging modality. Since many of the central nervous system findings identified in this study using MRI can alter the treatment and prognosis for these children, we believe that MRI should be the initial imaging study of choice for children with ANSD. PMID:20593543

Facial Paralysis in Patients With Hemifacial Microsomia: Frequency, Distribution, and Association With Other OMENS Abnormalities.

PubMed

Li, Qiang; Zhou, Xu; Wang, Yue; Qian, Jin; Zhang, Qingguo

2018-05-15

Although facial paralysis is a fundamental feature of hemifacial microsomia, the frequency and distribution of nerve abnormalities in patients with hemifacial microsomia remain unclear. In this study, the authors classified 1125 cases with microtia (including 339 patients with hemifacial microsomia and 786 with isolated microtia) according to Orbital Distortion Mandibular Hypoplasia Ear Anomaly Nerve Involvement Soft Tissue Dependency (OMENS) scheme. Then, the authors performed an independent analysis to describe the distribution feature of nerve abnormalities and reveal the possible relationships between facial paralysis and the other 4 fundamental features in the OMENS system. Results revealed that facial paralysis is present 23.9% of patients with hemifacial microsomia. The frontal-temporal branch is the most vulnerable branch in the total 1125 cases with microtia. The occurrence of facial paralysis is positively correlated with mandibular hypoplasia and soft tissue deficiency both in the total 1125 cases and the hemifacial microsomia patients. Orbital asymmetry is related to facial paralysis only in the total microtia cases, and ear deformity is related to facial paralysis only in hemifacial microsomia patients. No significant association was found between the severity of facial paralysis and any of the other 4 OMENS anomalies. These data suggest that the occurrence of facial paralysis may be associated with other OMENS abnormalities. The presence of serious mandibular hypoplasia or soft tissue deficiency should alert the clinician to a high possibility but not a high severity of facial paralysis.

Use of a multimodal conservative management protocol for the treatment of a patient with cervical radiculopathy

PubMed Central

Radpasand, Mohsen

2011-01-01

Objective The purpose of this study is to describe and discuss the treatment of a cervical disk herniation using a sequential multimodal conservative management approach. Clinical Features A 40-year-old man had complaints of headache and severe sharp neck pain radiating to his left shoulder down to his arm, forearm, and hand. Results of electromyography/nerve conduction studies were abnormal. Magnetic resonance imaging revealed a large disk protrusion at C5-C6 with indentation of the thecal sac and a spur at the posterior margin. Moderate left neural foraminal narrowing was present at C5-C6 with narrowed intervertebral disk space at C5-C6 and C6-C7. Intervention and Outcome High-velocity, low-amplitude chiropractic manipulation; electrotherapy; ice; and exercise were used for treatment. The Neck Disability Index was used as a primary and electromyography/nerve conduction studies as a secondary outcome measurement. Based on the Neck Disability Index, there was an overall 89.65% symptoms improvement from the baseline. Conclusions This case study demonstrated possible beneficial effects of the multimodal treatment approach in a patient with cervical radiculopathy. PMID:22027207

[Association between neuropathy and peripheral vascular insufficiency in patients with diabetes mellitus type 2].

PubMed

Millán-Guerrero, Rebeca O; Vásquez, Clemente; Isaís-Millán, Sara; Trujillo-Hernández, Benjamín; Caballero-Hoyos, Ramiro

2011-01-01

Diabetes mellitus (DM) can present complications of neuropathy and peripheral arterial disease with high risk for developing foot ulcers and consequent amputations. To identify the association between peripheral vascular disease, and neuropathy in type 2 Diabetes mellitus patients from the Hospital General de Zona No. 1 IMSS in Colima, Mexico. Cross-sectional study of 80 patients with diabetes mellitus evaluated by means of the Edinburgh Claudication Questionnaire, Michigan Neuropathy Screening Instrument, ankle-arm index, Motor Nerve Conduction Velocity and H-reflex. 51 women and 29 men were studied. Mean age was 53.9 +/- 9.6 years, mean diabetes mellitus progression was 8 +/- 6.6 years and mean glucose level was 283 +/- 110 mg/mL. Neuropathy presented in 65 patients (81.2%). Ankle/arm index revealed 19% of patients presented with moderate peripheral vascular insufficiency. Motor Nerve Conduction Velocity was abnormal in 40% of patients and H-reflex was absent in 70%. Grade 2 motor-sensitive polyneuropathy was found in 70-80% of patients and moderate peripheral vascular insufficiency in 19%. It can thus be inferred that the complication of diabetic neuropathy appears before that of peripheral vessel damage.

Transcutaneous stimulation of the posterior tibial nerve for treating refractory urge incontinence of idiopathic and neurogenic origin.

PubMed

Valles-Antuña, C; Pérez-Haro, M L; González-Ruiz de L, C; Quintás-Blanco, A; Tamargo-Diaz, E M; García-Rodríguez, J; San Martín-Blanco, A; Fernandez-Gomez, J M

2017-09-01

To assess the efficacy of treatment with transcutaneous posterior tibial nerve stimulation (TPTNS) in patients with urge urinary incontinence, of neurogenic or nonneurogenic origin, refractory to first-line therapeutic options. We included 65 patients with urge urinary incontinence refractory to medical treatment. A case history review, a urodynamic study and a somatosensory evoked potentials (SEP) study were conducted before the TPTNS, studying the functional urological condition by means of a voiding diary. The treatment consisted of 10 weekly sessions of TPTNS lasting 30minutes. Some 57.7% of the patients showed abnormal tibial SEPs, and 42% showed abnormal pudendal SEPs. A statistically significant symptomatic improvement was observed in all clinical parameters after treatment with TPTNS, and 66% of the patients showed an overall improvement, regardless of sex, the presence of underlying neurological disorders, detrusor hyperactivity in the urodynamic study or SEP disorders. There were no adverse effects during the treatment. TPTNS is an effective and well tolerated treatment in patients with urge incontinence refractory to first-line therapies and should be offered early in the treatment strategy. New studies are needed to identify the optimal parameters of stimulation, the most effective treatment protocols and long-term efficacy, as well as its applicability to patients with a neurogenic substrate. Copyright © 2017 AEU. Publicado por Elsevier España, S.L.U. All rights reserved.

Unilateral blindness with third cranial nerve palsy and abnormal enhancement of extraocular muscles on magnetic resonance imaging of orbit after the ingestion of methanol.

PubMed

Chung, Tae Nyoung; Kim, Sun Wook; Park, Yoo Seok; Park, Incheol

2010-05-01

Methanol is generally known to cause visual impairment and various systemic manifestations. There are a few reported specific findings for methanol intoxication on magnetic resonance imaging (MRI) of the brain. A case is reported of unilateral blindness with third cranial nerve palsy oculus sinister (OS) after the ingestion of methanol. Unilateral damage of the retina and optic nerve were confirmed by fundoscopy, flourescein angiography, visual evoked potential and electroretinogram. The optic nerve and extraocular muscles (superior rectus, medial rectus, inferior rectus and inferior oblique muscle) were enhanced by gadolinium-DTPA on MRI of the orbit. This is the first case report of permanent monocular blindness with confirmed unilateral damage of the retina and optic nerve, combined with third cranial nerve palsy after methanol ingestion.

Vestibular evoked myogenic potentials and health-related quality of life in patients with vestibular neuritis.

PubMed

Viciana, David; Lopez-Escamez, Jose A

2010-08-01

To evaluate the usefulness of vestibular evoked myogenic potentials (VEMPs) in subjects with vestibular neuritis (VN) and to determine the impact of the disease in health-related quality of life (HRQoL). Case series. Tertiary referral center. Fifty patients with VN (episode of sudden onset of prolonged vertigo [>24 h] associated with peripheral vestibular hypofunction, imbalance in absence of hearing loss, or other neurologic symptoms). VEMPs were measured in 41 patients by using an air-conducted 500 Hz tone burst. HRQoL was evaluated in all cases by the Medical Outcomes Study 36-Item Short Form Health Survey (SF-36) and Dizziness Handicap Inventory Short Form (DHI-S) instruments, after the acute episode was resolved. Latencies P1 or N1 peaks, corrected amplitude or the absence of response, for VEMPs; scores obtained in SF-36 and DHI-S instruments. VEMPs showed abnormal results in 21 (51%) of 41 cases, with an increase in ipsilateral latencies for P1 and N1 peaks being the most common finding. Three patients (7%) had ipsilateral abnormal VEMP response with normal caloric response, indicating isolated involvement of inferior vestibular nerve. The total score obtained for the DHI-S was 14.76 +/- 11.07 (range, 0-34/40), suggesting a variable impact among patients with VN. For the SF-36, scores in men with VN were worse than their age-matched controls for all dimensions, except for mental health. However, women only showed lower scores for general health and social function. Abnormal VEMP responses demonstrate the involvement of the inferior vestibular nerve in half of the patients with VN. Moreover, VN has a moderate impact in HRQoL, and it is perceived more disabling by men than women.

The prevalence of tarsal tunnel syndrome in patients with lumbosacral radiculopathy.

PubMed

Zheng, Chaojun; Zhu, Yu; Jiang, Jianyuan; Ma, Xiaosheng; Lu, Feizhou; Jin, Xiang; Weber, Robert

2016-03-01

Tarsal tunnel syndrome (TTS) is a painful foot condition. Lumbosacral radiculopathy (LR) may also present with symptoms occurring in TTS. However, no studies have been reported to determine the possible coexistence of these two conditions. The aim of our study was to identify the prevalence of TTS in patients with confirmed LR and to analyze the clinical and electrodiagnostic features of patients with both TTS and LR. Medial and lateral plantar nerve mixed studies, peroneal motor studies and deep peroneal sensory studies were performed in 81 normal subjects and 561 patients with LR. The Tinel's test and other provocative tests were performed in the LR patient group, and the clinical symptoms of TTS were also analyzed. The frequency of TTS was investigated in all radiculopathy group patients with different nerve root lesions. Concomitant TTS was found in 27 (4.8%) patients with LR. Abnormal results of sensory/mixed conduction tests were observed in 25/27 (92.6%) patients, and 11/27 (40.7%) patients had abnormal results of motor conduction tests. Positivity for the Tinel's test and special provocative tests was found in 15/27 (55.6%) and 17/27 (63.0%) patients, respectively. Overall, 9/27 (33.3%) patients had typical symptoms, and suspicious clinical symptoms were found in the other 14/27 (51.9%) patients. The frequency of coexisting TTS was not statistically different among the single-level L4, L5 or S1 radiculopathy, or between the single-level and multi-level radiculopathies (P > 0.05). The findings suggest that the prevalence of TTS is significant in patients with LR. Thus, more caution should be paid when diagnosing and managing patients with LR due to the possible existence of TTS, as their management strategies are quite different.

Reduction in Retinal Nerve Fiber Layer Thickness in Young Adults with Autism Spectrum Disorders

ERIC Educational Resources Information Center

Emberti Gialloreti, Leonardo; Pardini, Matteo; Benassi, Francesca; Marciano, Sara; Amore, Mario; Mutolo, Maria Giulia; Porfirio, Maria Cristina; Curatolo, Paolo

2014-01-01

Recent years have seen an increase in the use of retinal nerve fiber layer (RNFL) evaluation as an easy-to-use, reproducible, proxy-measure of brain structural abnormalities. Here, we evaluated RNFL thickness in a group of subjects with high functioning autism (HFA) or with Asperger Syndrome (AS) to its potential as a tool to study autism…

Clinical-pathologic correlations in voltage-gated Kv1 potassium channel complex-subtyped autoimmune painful polyneuropathy.

PubMed

Lahoria, Rajat; Pittock, Sean J; Gadoth, Avi; Engelstad, Janean K; Lennon, Vanda A; Klein, Christopher J

2017-04-01

Voltage-gated Kv1 potassium channel complex (VGKC) autoantibodies subtyped for leucine-rich glioma-inactivated 1 (LGI1), contactin-associated-proteinlike 2 (CASPR2), and Kv IgGs have a spectrum of neurological presentations. Painful polyneuropathy is seen in some patients, but nerve pathology descriptions are lacking. Clinicopathologic features were studied in subtyped VGKC-autoantibody-seropositive patients who had undergone nerve biopsies. Five patients were identified, 1 LGI1 IgG positive and 1 CASPR2 IgG positive, but all negative for Kv1.1-, 1.2-, 1.6-subtyped IgG autoantibodies. Median symptom duration was 17 months. Pain was the predominant symptom; 3 had mild sensory loss and/or weakness. Histopathological abnormalities were limited to axonal loss in 3. None had mononuclear cellular infiltrates. Electron micrographs revealed no interstitial abnormalities. Three patients reported marked improvement in pain with immunotherapy. The nerve biopsy histopathology of patients subtyped for LGI1 and CASPR2 IgGs within the VGKC-complex spectrum disorders shows either normal density or axonal fiber loss without inflammatory infiltrates. A reversible neural hyperexcitable mechanism is considered to be the cause of this painful polyneuropathy. Muscle Nerve 55: 520-525, 2017. © 2016 Wiley Periodicals, Inc.

A cross-sectional electromyography assessment in linear scleroderma patients

PubMed Central

2014-01-01

Background Muscle atrophy and asymmetric extremity growth is a common feature of linear scleroderma (LS). Extra-cutaneous features are also common and primary neurologic involvement, with sympathetic dysfunction, may have a pathogenic role in subcutaneous and muscle atrophy. The aim was investigate nerve conduction and muscle involvement by electromyography in pediatric patients with LS. Methods We conducted a retrospective review of LS pediatric patients who had regular follow up at a single pediatric center from 1997–2013. We selected participants if they had consistently good follow up and enrolled consecutive patients in the study. We examined LS photos as well as clinical, serological and imaging findings. Electromyograms (EMG) were performed with bilateral symmetric technique, using surface and needle electrodes, comparing the affected side with the contralateral side. Abnormal muscle activity was categorized as a myopathic or neurogenic pattern. Results Nine LS subjects were selected for EMG, 2 with Parry-Romberg/Hemifacial Atrophy Syndrome, 7 linear scleroderma of an extremity and 2 with mixed forms (linear and morphea). Electromyogram analysis indicated that all but one had asymmetric myopathic pattern in muscles underlying the linear streaks. Motor and sensory nerve conduction was also evaluated in upper and lower limbs and one presented a neurogenic pattern. Masticatory muscle testing showed a myopathic pattern in the atrophic face of 2 cases with head and face involvement. Conclusion In our small series of LS patients, we found a surprising amount of muscle dysfunction by EMG. The muscle involvement may be possibly related to a secondary peripheral nerve involvement due to LS inflammation and fibrosis. Further collaborative studies to confirm these findings are needed. PMID:25053924

Dysfunction of the neuromuscular junction in spinal muscular atrophy types 2 and 3.

PubMed

Wadman, Renske I; Vrancken, Alexander F J E; van den Berg, Leonard H; van der Pol, W Ludo

2012-11-13

Spinal muscular atrophy (SMA) is pathologically characterized by degeneration of anterior horn cells. Recent observations in animal models of SMA and muscle tissue from patients with SMA suggest additional abnormalities in the development and maturation of the neuromuscular junction. We therefore evaluated neuromuscular junction function in SMA with repetitive nerve stimulation. In this case-control study, repetitive nerve stimulation was performed in 35 patients with SMA types 2, 3, and 4, 20 healthy controls, and 5 controls with motor neuron disease. Pathologic decremental responses (>10%) during 3-Hz repetitive nerve stimulation were observed in 17 of 35 patients (49%) with SMA types 2 and 3, but not in healthy controls or controls with motor neuron disease. None of the patients or controls had an abnormal incremental response of >60%. The presence of an abnormal decremental response was not specific for the type of SMA, nor was it associated with compound muscle action potential amplitude, clinical scores, or disease duration. Two of 4 patients with SMA type 3 who tried pyridostigmine reported increased stamina. These data suggest dysfunction of the neuromuscular junction in patients with SMA types 2 and 3. Therefore, drugs that facilitate neuromuscular transmission are candidate drugs for evaluation in carefully designed, placebo-controlled, clinical trials.

Peripheral Motor and Sensory Nerve Conduction following Transplantation of Undifferentiated Autologous Adipose Tissue-Derived Stem Cells in a Biodegradable U.S. Food and Drug Administration-Approved Nerve Conduit.

PubMed

Klein, Silvan M; Vykoukal, Jody; Li, De-Pei; Pan, Hui-Lin; Zeitler, Katharina; Alt, Eckhard; Geis, Sebastian; Felthaus, Oliver; Prantl, Lukas

2016-07-01

Conduits preseeded with either Schwann cells or stem cells differentiated into Schwann cells demonstrated promising results for the outcome of nerve regeneration in nerve defects. The concept of this trial combines nerve repair by means of a commercially available nerve guidance conduit and preseeding with autologous, undifferentiated, adipose tissue-derived stem cells. Adipose tissue-derived stem cells were harvested from rats and subsequently seeded onto a U.S. Food and Drug Administration-approved type I collagen conduit. Sciatic nerve gaps 10 mm in length were created, and nerve repair was performed by the transplantation of either conduits preseeded with autologous adipose tissue-derived stem cells or acellular (control group) conduits. After 6 months, the motor and sensory nerve conduction velocity were assessed. Nerves were removed and examined by hematoxylin and eosin, van Gieson, and immunohistochemistry (S100 protein) staining for the quality of axonal regeneration. Nerve gaps treated with adipose tissue-derived stem cells showed superior nerve regeneration, reflected by higher motor and sensory nerve conduction velocity values. The motor and sensory nerve conduction velocity were significantly greater in nerves treated with conduits preseeded with adipose tissue-derived stem cells than in nerves treated with conduits alone (p < 0.05). Increased S100 immunoreactivity was detected for the adipose tissue-derived stem cell group. In this group, axon arrangement inside the conduits was more organized. Transplantation of adipose tissue-derived stem cells significantly improves motor and sensory nerve conduction velocity in peripheral nerve gaps. Preseeded conduits showed a more organized axon arrangement inside the conduit in comparison with nerve conduits alone. The approach used here could readily be translated into a clinical therapy. Therapeutic, V.

Diplopia after inferior alveolar nerve block: case report and related physiology.

PubMed

You, Tae Min

2015-06-01

Although inferior alveolar nerve block is one of the most common procedures performed at dental clinics, complications or adverse effects can still occur. On rare occasions, ocular disturbances, such as diplopia, blurred vision, amaurosis, mydriasis, abnormal pupillary light reflex, retrobulbar pain, miosis, and enophthalmos, have also been reported after maxillary and mandibular anesthesia. Generally, these symptoms are temporary but they can be rather distressing to both patients and dental practitioners. Herein, we describe a case of diplopia caused by routine inferior alveolar nerve anesthesia, its related physiology, and management.

Diplopia after inferior alveolar nerve block: case report and related physiology

PubMed Central

2015-01-01

Although inferior alveolar nerve block is one of the most common procedures performed at dental clinics, complications or adverse effects can still occur. On rare occasions, ocular disturbances, such as diplopia, blurred vision, amaurosis, mydriasis, abnormal pupillary light reflex, retrobulbar pain, miosis, and enophthalmos, have also been reported after maxillary and mandibular anesthesia. Generally, these symptoms are temporary but they can be rather distressing to both patients and dental practitioners. Herein, we describe a case of diplopia caused by routine inferior alveolar nerve anesthesia, its related physiology, and management. PMID:28879264

[Pay attention to the secondary optic neuropathy and the safe appropriate applications of optic neuroprotection].

PubMed

Zhong, Y

2016-12-11

Secondary optic neuropathy of optic nerve abnormalities is the leading cause of persistent visual impairment. Previous ocular neuroprotection studies have proved that the nerve growth factor and other agents are of significant in the preservation of optic nerve axon and retinal ganglion cells. However, finding novel safe and effective approach as well as the appropriate applications of optic neuroprotection should be highly emphasized and would be very helpful in the treatment of optic neuropathy. (Chin J Ophthalmol, 2016, 52: 881 - 884) .

Terminal changes in hereditary sensory and autonomic neuropathy: a long-term follow-up of a sporadic case.

PubMed

Lee, Sang-Soo; Lee, Sung-Hyun; Han, Seol-Heui

2003-07-01

We describe terminal changes in a long-term follow-up of a 51-year-old man with sporadic hereditary sensory and autonomic neuropathy (HSAN). From the age of 15 years onwards, he suffered from multiple painless ulcers of his feet and fingers, necessitating amputation. Neurological studies revealed almost complete sensory loss affecting all modalities in the upper and lower limbs, minimal involvement of motor fibers, and areflexia. A neurophysiological abnormality involved an absence of sensory action potentials with relatively normal motor nerve conduction velocities. Biopsy of the sural nerve showed almost total loss of myelinated fibers with a mild decrease in unmyelinated fibers. Despite the late onset of the disease, the progressive course, and the lancinating pain, the terminal features of this patient, which involved a selective loss of myelinated fibers and widespread sensory loss, seem to be symptomatic of HSAN II, the progressive form of autosomal recessive sensory neuropathy, and emphasize the clinical heterogeneity of HSAN.

Fiber type-specific afferent nerve activity induced by transient contractions of rat bladder smooth muscle in pathological states

PubMed Central

Kuga, Nahoko; Tanioka, Asao; Hagihara, Koichiro; Kawai, Tomoyuki

2017-01-01

Bladder smooth muscle shows spontaneous phasic contractions, which undergo a variety of abnormal changes depending on pathological conditions. How abnormal contractions affect the activity of bladder afferent nerves remains to be fully tested. In this study, we examined the relationship between transient increases in bladder pressure, representing transient contraction of bladder smooth muscle, and spiking patterns of bladder afferent fibers of the L6 dorsal root, in rat pathological models. All recordings were performed at a bladder pressure of approximately 10 cmH2O by maintaining the degree of bladder filling. In the cyclophosphamide-induced model, both Aδ and C fibers showed increased sensitivity to transient bladder pressure increases. In the prostaglandin E2-induced model, Aδ fibers, but not C fibers, specifically showed overexcitation that was time-locked with transient bladder pressure increases. These fiber type-specific changes in nerve spike patterns may underlie the symptoms of urinary bladder diseases. PMID:29267380

Abnormal cardiovascular response to exercise in hypertension: contribution of neural factors.

PubMed

Mitchell, Jere H

2017-06-01

During both dynamic (e.g., endurance) and static (e.g., strength) exercise there are exaggerated cardiovascular responses in hypertension. This includes greater increases in blood pressure, heart rate, and efferent sympathetic nerve activity than in normal controls. Two of the known neural factors that contribute to this abnormal cardiovascular response are the exercise pressor reflex (EPR) and functional sympatholysis. The EPR originates in contracting skeletal muscle and reflexly increases sympathetic efferent nerve activity to the heart and blood vessels as well as decreases parasympathetic efferent nerve activity to the heart. These changes in autonomic nerve activity cause an increase in blood pressure, heart rate, left ventricular contractility, and vasoconstriction in the arterial tree. However, arterial vessels in the contracting skeletal muscle have a markedly diminished vasoconstrictor response. The markedly diminished vasoconstriction in contracting skeletal muscle has been termed functional sympatholysis. It has been shown in hypertension that there is an enhanced EPR, including both its mechanoreflex and metaboreflex components, and an impaired functional sympatholysis. These conditions set up a positive feedback or vicious cycle situation that causes a progressively greater decrease in the blood flow to the exercising muscle. Thus these two neural mechanisms contribute significantly to the abnormal cardiovascular response to exercise in hypertension. In addition, exercise training in hypertension decreases the enhanced EPR, including both mechanoreflex and metaboreflex function, and improves the impaired functional sympatholysis. These two changes, caused by exercise training, improve the muscle blood flow to exercising muscle and cause a more normal cardiovascular response to exercise in hypertension. Copyright © 2017 the American Physiological Society.

Mitochondrial alterations with mitochondrial DNA depletion in the nerves of AIDS patients with peripheral neuropathy induced by 2'3'-dideoxycytidine (ddC).

PubMed

Dalakas, M C; Semino-Mora, C; Leon-Monzon, M

2001-11-01

The 2'3'-dideoxycytidine (ddC), a nonazylated dideoxynucleoside analog used for the treatment of AIDS, causes a dose-dependent, painful, sensorimotor axonal peripheral neuropathy in up to 30% of the patients. To investigate the cause of the neuropathy, we performed morphological and molecular studies on nerve biopsy specimens from well-selected patients with ddC-neuropathy and from control subjects with disease, including patients with AIDS-related neuropathy never treated with ddC. Because ddC, in vitro, inhibits the replication of mitochondrial DNA (mtDNA), we counted the number of normal and abnormal mitochondria in a 0.04 mm(2) cross-sectional area of the nerves and quantified the copy numbers of mtDNA by competitive PCR in all specimens. A varying degree of axonal degeneration was present in all nerves. Abnormal mitochondria with enlarged size, excessive vacuolization, electron-dense concentric inclusions and degenerative myelin structures were prominent in the ddC-neuropathy and accounted for 55% +/- 2.5% of all counted mitochondria in the axon and Schwann cells, compared with 9% +/- 0.7% of the controls (p < 0.001). Significantly (p < 0.005) reduced copy numbers, with as high as 80% depletion, of the mtDNA was demonstrated in the nerves of the ddC-treated patients compared with the controls. We conclude that ddC induces a mitochondrial neuropathy with depletion of the nerve's mtDNA. The findings are consistent with the ability of ddC to selectively inhibit the gamma-DNA polymerase in neuronal cell lines. Toxicity to mitochondria of the peripheral nerve is a new cause of acquired neuropathy induced by exogenous toxins and may be the cause of neuropathy associated with the other neurotoxic antiretroviral drugs or toxic-metabolic conditions.

Diagnostic value of the near-nerve needle sensory nerve conduction in sensory inflammatory demyelinating polyneuropathy.

PubMed

Odabasi, Zeki; Oh, Shin J

2018-03-01

In this study we report the diagnostic value of the near-nerve needle sensory nerve conduction study (NNN-SNCS) in sensory inflammatory demyelinating polyneuropathy (IDP) in which the routine nerve conduction study was normal or non-diagnostic. The NNN-SNCS was performed to identify demyelination in the plantar nerves in 14 patients and in the median or ulnar nerve in 2 patients with sensory IDP. In 16 patients with sensory IDP, routine NCSs were either normal or non-diagnostic for demyelination. Demyelination was identified by NNN-SNCS by dispersion and/or slow nerve conduction velocity (NCV) below the demyelination marker. Immunotherapy was initiated in 11 patients, 10 of whom improved or remained stable. NNN-SNCS played an essential role in identifying demyelinaton in 16 patients with sensory IDP, leading to proper treatment. Muscle Nerve 57: 414-418, 2018. © 2017 Wiley Periodicals, Inc.

Mechanism of gastrointestinal abnormal motor activity induced by cisplatin in conscious dogs

PubMed Central

Ando, Hiroyuki; Mochiki, Erito; Ohno, Tetsuro; Yanai, Mitsuhiro; Toyomasu, Yoshitaka; Ogata, Kyoichi; Tabe, Yuichi; Aihara, Ryuusuke; Nakabayashi, Toshihiro; Asao, Takayuki; Kuwano, Hiroyuki

2014-01-01

AIM: To investigate whether 5-hydroxytryptamine (serotonin; 5-HT) is involved in mediating abnormal motor activity in dogs after cisplatin administration. METHODS: After the dogs had been given a 2-wk recovery period, all of them were administered cisplatin, and the motor activity was recorded using strain gauge force transducers. Blood and intestinal fluid samples were collected to measure 5-HT for 24 h. To determine whether 5-HT in plasma or that in intestinal fluids is more closely related to abnormal motor activity we injected 5-HT into the bloodstream and the intestinal tract of the dogs. RESULTS: Cisplatin given intravenously produced abnormal motor activity that lasted up to 5 h. From 3 to 4 h after cisplatin administration, normal intact dogs exhibited retropropagation of motor activity accompanied by emesis. The concentration of 5-HT in plasma reached the peak at 4 h, and that in intestinal fluids reached the peak at 3 h. In normal intact dogs with resection of the vagus nerve that were administered kytril, cisplatin given intravenously did not produce abnormal motor activity. Intestinal serotonin administration did not produce abnormal motor activity, but intravenous serotonin administration did. CONCLUSION: After the intravenous administration of cisplatin, abnormal motor activity was produced in the involved vagus nerve and in the involved serotonergic neurons via another pathway. This study was the first to determine the relationship between 5-HT and emesis-induced motor activity. PMID:25400453

Differential aging of median and ulnar sensory nerve parameters.

PubMed

Werner, Robert A; Franzblau, Alfred; D'Arcy, Hannah J S; Evanoff, Bradley A; Tong, Henry C

2012-01-01

Nerve conduction velocity slows and amplitude declines with aging. Median and ulnar sensory nerves were tested at the annual meetings of the American Dental Association. Seven hundred four subjects had at least two observations. The rate of change in the nerve parameters was estimated while controlling for gender, age, change in hand temperature, baseline body mass index (BMI), and change in BMI. Amplitudes of the median sensory nerve action potentials decreased by 0.58 μV per year, whereas conduction velocity decreased at a rate of 0.41 m/s per year. Corresponding values for the ulnar nerve were 0.89 μV and 0.29 m/s per year. The rates of change in amplitudes did not differ, but the median nerve demonstrated a more rapid loss of conduction velocity. The rate of change for the median conduction velocity was higher than previously reported. The rate of change of median conduction velocity was significantly greater than for the ulnar nerve. Copyright © 2011 Wiley Periodicals, Inc.

Hypoglossal-facial nerve reconstruction using a Y-tube-conduit reduces aberrant synkinetic movements of the orbicularis oculi and vibrissal muscles in rats.

PubMed

Kaya, Yasemin; Ozsoy, Umut; Turhan, Murat; Angelov, Doychin N; Sarikcioglu, Levent

2014-01-01

The facial nerve is the most frequently damaged nerve in head and neck trauma. Patients undergoing facial nerve reconstruction often complain about disturbing abnormal synkinetic movements of the facial muscles (mass movements, synkinesis) which are thought to result from misguided collateral branching of regenerating motor axons and reinnervation of inappropriate muscles. Here, we examined whether use of an aorta Y-tube conduit during reconstructive surgery after facial nerve injury reduces synkinesis of orbicularis oris (blink reflex) and vibrissal (whisking) musculature. The abdominal aorta plus its bifurcation was harvested (N = 12) for Y-tube conduits. Animal groups comprised intact animals (Group 1), those receiving hypoglossal-facial nerve end-to-end coaptation alone (HFA; Group 2), and those receiving hypoglossal-facial nerve reconstruction using a Y-tube (HFA-Y-tube, Group 3). Videotape motion analysis at 4 months showed that HFA-Y-tube group showed a reduced synkinesis of eyelid and whisker movements compared to HFA alone.

Hypoglossal-Facial Nerve Reconstruction Using a Y-Tube-Conduit Reduces Aberrant Synkinetic Movements of the Orbicularis Oculi and Vibrissal Muscles in Rats

PubMed Central

Kaya, Yasemin; Ozsoy, Umut; Turhan, Murat; Angelov, Doychin N.; Sarikcioglu, Levent

2014-01-01

The facial nerve is the most frequently damaged nerve in head and neck trauma. Patients undergoing facial nerve reconstruction often complain about disturbing abnormal synkinetic movements of the facial muscles (mass movements, synkinesis) which are thought to result from misguided collateral branching of regenerating motor axons and reinnervation of inappropriate muscles. Here, we examined whether use of an aorta Y-tube conduit during reconstructive surgery after facial nerve injury reduces synkinesis of orbicularis oris (blink reflex) and vibrissal (whisking) musculature. The abdominal aorta plus its bifurcation was harvested (N = 12) for Y-tube conduits. Animal groups comprised intact animals (Group 1), those receiving hypoglossal-facial nerve end-to-end coaptation alone (HFA; Group 2), and those receiving hypoglossal-facial nerve reconstruction using a Y-tube (HFA-Y-tube, Group 3). Videotape motion analysis at 4 months showed that HFA-Y-tube group showed a reduced synkinesis of eyelid and whisker movements compared to HFA alone. PMID:25574468

Reversible acute axonal polyneuropathy associated with Wernicke-Korsakoff syndrome: impaired physiological nerve conduction due to thiamine deficiency?

PubMed

Ishibashi, S; Yokota, T; Shiojiri, T; Matunaga, T; Tanaka, H; Nishina, K; Hirota, H; Inaba, A; Yamada, M; Kanda, T; Mizusawa, H

2003-05-01

Acute axonal polyneuropathy and Wernicke-Korsakoff encephalopathy developed simultaneously in three patients. Nerve conduction studies (NCS) detected markedly decreased compound muscle action potentials (CMAPs) and sensory nerve action potentials (SNAPs) with minimal conduction slowing; sympathetic skin responses (SSRs) were also notably decreased. Sural nerve biopsies showed only mild axonal degeneration with scattered myelin ovoid formation. The symptoms of neuropathy lessened within two weeks after an intravenous thiamine infusion. CMAPs, SNAPs, and SSRs also increased considerably. We suggest that this is a new type of peripheral nerve impairment: physiological conduction failure with minimal conduction delay due to thiamine deficiency.

Optic nerve axons and acquired alterations in the appearance of the optic disc.

PubMed Central

Wirtschafter, J D

1983-01-01

The pathophysiologic events in optic nerve axons have recently been recognized as crucial to an understanding of clinically significant acquired alterations in the ophthalmoscopic appearance of the optic disc. Stasis and related abnormalities of axonal transport appear to explain most aspects of optic nerve head swelling, including optic disc drusen and retinal cottonwool spots. Loss of axoplasm and axonal death can be invoked to interpret optic disc pallor, thinning and narrowing of rim tissue, changes in the size and outline of the optic cup, laminar dots, atrophy of the retinal nerve fiber layer, and acquired demyelination and myelination of the retinal nerve fiber layer. It is speculated that the axons may also play a role in the mechanical support of the lamina cribrosa in resisting the pressure gradient across the pars scleralis of the optic nerve head. Axons and their associated glial cells may be involved in those cases where "reversibility" of cupping of the optic disc has been reported. The structure, physiology, and experimental pathologic findings of the optic nerve head have been reviewed. Many aspects concerning the final anatomic appearance of the optic nerve head have been explained. However, many questions remain concerning the intermediate mechanisms by which increased intracranial pressure retards the various components of axonal transport in papilledema and by which increased IOP causes axonal loss in glaucoma. Investigation of the molecular biology of axonal constituents and their responses to abnormalities in their physical and chemical milieu could extend our understanding of the events that result from mechanical compression and local ischemia. Moreover, we have identified a need to further explore the role of axons in the pathophysiology of optic disc cupping. Images FIGURE 2 FIGURE 3 FIGURE 4 FIGURE 5 FIGURE 6 FIGURE 7 FIGURE 8 FIGURE 11 FIGURE 12 FIGURE 13 PMID:6203209

A combined third and fourth branchial arch anomaly: clinical and embryological implications.

PubMed

Mehrzad, H; Georgalas, C; Huins, C; Tolley, N S

2007-08-01

Embryological abnormalities of the branchial apparatus present an interesting diagnostic and surgical challenge. Thymic cysts are a rare form of branchial apparatus anomaly, resulting from abnormal development of the third pharyngeal pouch. We present two cases of a thymic cyst coexisting with a non recurrent inferior laryngeal nerve (NRILN), two anomalies that to our knowledge have not been associated previously. A possible embryological explication for this double abnormality is discussed, while the clinical implications of this association are presented.

Restoration of Trigeminal Cutaneous Sensation with Cross-Face Sural Nerve Grafts: A Novel Approach to Facial Sensory Rehabilitation.

PubMed

Catapano, Joseph; Scholl, David; Ho, Emily; Zuker, Ronald M; Borschel, Gregory H

2015-09-01

Although treating facial palsy is considered debilitating for patients, trigeminal nerve palsy and sensory deficits of the face are overlooked components of disability. Complete anesthesia leaves patients susceptible to occult injury, and facial sensation is an important component of interaction and activities of daily living. Sensory reconstruction is well established in the restoration of hand sensation; however, only one previous report proposed a surgical strategy for sensory nerve reconstruction of the face with use of nerve transfers. Nerve transfers, when used alone, have limited application because of their restricted arc of rotation in the face; extending their arc by adding nerve grafts greatly expands their utility. The following cases demonstrate the early results after V2 and V3 reconstruction with cross-face nerve grafts in three patients with acquired trigeminal nerve palsy. Cross-face nerve grafts using the sural nerve permit more proximal reconstruction of the infraorbital and mental nerves, which allows reinnervation of their entire cutaneous distribution. All patients demonstrated improved sensation in the reconstructed dermatomes, and no patients reported donor-site abnormalities. Cross-face nerve grafts result in minimal donor-site morbidity and are promising as a surgical strategy to address sensory deficits of the face. Therapeutic, V.

Comparison of Nerve Excitability Testing, Nerve Conduction Velocity, and Behavioral Observations for Acrylamide Induced Peripheral Neuropathy

EPA Science Inventory

Nerve excitability (NE) testing is a sensitive method to test for peripheral neurotoxicity in humans,and may be more sensitive than compound nerve action potential (CNAP) or nerve conduction velocity (NCV).We used acrylamide to compare the NE and CNAP/NCV methods. Behavioral test...

Clinical findings and electrodiagnostic testing in 108 consecutive cases of lumbosacral radiculopathy due to herniated disc.

PubMed

Mondelli, M; Aretini, A; Arrigucci, U; Ginanneschi, F; Greco, G; Sicurelli, F

2013-10-01

This prospective study aim to examine whether clinical findings and electrodiagnostic testing (EDX) in patients with lumbosacral monoradiculopathy due to herniated disc (HD) differ as a function of root involvement level (L5 vs. S1) and HD zone (paramedian vs. intraforaminal). All patients with L4, L5 or S1 monoradiculopathy were prospectively enrolled at four electromyography (EMG) labs over a 2-year period. The diagnosis was based on a congruence between patient history and MRI evidence of HD. We compared the sensitivities of clinical findings and EDX with respect to both root involvement level and HD zone. Multivariate logistic regression was performed in order to verify the association between abnormal EMG, clinical, and neuroradiological findings. One hundred and eight patients (mean age 47.7 years, 55% men) were consecutively enrolled. Sensory loss in the painful dermatome was the most frequent finding at physical examination (56% of cases). EMG was abnormal in at least one muscle supplied by femoral and sciatic nerves in 45 cases (42%). Inclusion of paraspinal muscles increased sensitivity to only 49% and that of proximal muscles was useless. Motor and sensory neurography was seldom abnormal. The most frequent motor neurographic abnormalities were a delay of F-wave minimum latency and decrease in the compound muscle action potential amplitude from extensor digitorum brevis and abductor hallucis in L5 and S1 radiculopathies, respectively. Sensory neurography was usually normal, the amplitude of sensory nerve action potential was seldom reduced when HD injured dorsal root ganglion or postganglionic root fibres. Multivariate logistic regression analysis showed that EMG abnormalities could be predicted by myotomal muscular weakness, abnormal deep reflexes, and paraesthesiae. The only clinical and electrophysiological differences with respect to root involvement level concerned deep reflexes and motor neurography of deep peroneal and tibial nerves. Only some EDX parameters are helpful for the diagnosis of lumbosacral radiculopathy. EMG was abnormal in less than 50% of cases and its abnormalities could be predicted by some clinical findings. However, neurography is useful as a tool for differential diagnosis between radiculopathy and more diffuse disorders of the peripheral nervous system (polyneuropathy, plexopathy). Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Electro-mechanical response of a 3D nerve bundle model to mechanical loads leading to axonal injury.

PubMed

Cinelli, I; Destrade, M; Duffy, M; McHugh, P

2017-07-01

Axonal damage is one of the most common pathological features of traumatic brain injury, leading to abnormalities in signal propagation for nervous systems. We present a 3D fully coupled electro-mechanical model of a nerve bundle, made with the finite element software Abaqus 6.13-3. The model includes a real-time coupling, modulated threshold for spiking activation and independent alteration of the electrical properties for each 3-layer fibre within the bundle. Compression and tension are simulated to induce damage at the nerve membrane. Changes in strain, stress distribution and neural activity are investigated for myelinated and unmyelinated nerve fibres, by considering the cases of an intact and of a traumatized nerve membrane. Results show greater changes in transmitting action potential in the myelinated fibre.

Acute Blindness.

PubMed

Meekins, Jessica M

2015-09-01

Sudden loss of vision is an ophthalmic emergency with numerous possible causes. Abnormalities may occur at any point within the complex vision pathway, from retina to optic nerve to the visual center in the occipital lobe. This article reviews specific prechiasm (retina and optic nerve) and cerebral cortical diseases that lead to acute blindness. Information regarding specific etiologies, pathophysiology, diagnosis, treatment, and prognosis for vision is discussed. Copyright © 2015 Elsevier Inc. All rights reserved.

Long-term clinical effects of epalrestat, an aldose reductase inhibitor, on diabetic peripheral neuropathy: the 3-year, multicenter, comparative Aldose Reductase Inhibitor-Diabetes Complications Trial.

PubMed

Hotta, Nigishi; Akanuma, Yasuo; Kawamori, Ryuzo; Matsuoka, Kempei; Oka, Yoshitomo; Shichiri, Motoaki; Toyota, Takayoshi; Nakashima, Mitsuyoshi; Yoshimura, Isao; Sakamoto, Nobuo; Shigeta, Yukio

2006-07-01

We sought to evaluate the long-term efficacy and safety of epalrestat, an aldose reductase inhibitor, on diabetic peripheral neuropathy. Subjects with diabetic neuropathy, median motor nerve conduction velocity (MNCV) >or=40 m/s, and HbA(1c)

Deficient "sensory" beta synchronization in Parkinson's disease.

PubMed

Degardin, A; Houdayer, E; Bourriez, J-L; Destée, A; Defebvre, L; Derambure, P; Devos, D

2009-03-01

Beta rhythm movement-related synchronization (beta synchronization) reflects motor cortex deactivation and sensory afference processing. In Parkinson's disease (PD), decreased beta synchronization after active movement reflects abnormal motor cortex idling and may be involved in the pathophysiology of akinesia. The objectives of the present study were to (i) compare event-related synchronization after active and passive movement and electrical nerve stimulation in PD patients and healthy, age-matched volunteers and (ii) evaluate the effect of levodopa. Using a 128-electrode EEG system, we studied beta synchronization after active and passive index finger movement and electrical median nerve stimulation in 13 patients and 12 control subjects. Patients were recorded before and after 150% of their usual morning dose of levodopa. The peak beta synchronization magnitude in the contralateral primary sensorimotor (PSM) cortex was significantly lower in PD patients after active movement, passive movement and electrical median nerve stimulation, compared with controls. Levodopa partially reversed the drop in beta synchronization after active movement but not after passive movement or electrical median nerve stimulation. If one considers that beta synchronization reflects sensory processing, our results suggest that integration of somaesthetic afferences in the PSM cortex is abnormal in PD during active and passive movement execution and after simple electrical median nerve stimulation. Better understanding of the mechanisms involved in the deficient beta synchronization observed here could prompt the development of new therapeutic approaches aimed at strengthening defective processes. The lack of full beta synchronization restoration by levodopa might be related to the involvement of non-dopaminergic pathways.

Anterior Ethmoidal Nerve Block in Prevention of Postoperative Agitation

ClinicalTrials.gov

2018-02-08

Postoperative Agitation: Impaired Awareness, Abnormal Cognitive Function, Confusion, and Verbal and Physical Agitation During Recovery From General Anesthesia; Deviated Nasal Septum; Chronic Rhinosinusitis (Diagnosis); Nasal Polyps

Electrophysiology of Cranial Nerve Testing: Spinal Accessory and Hypoglossal Nerves.

PubMed

Stino, Amro M; Smith, Benn E

2018-01-01

Multiple techniques have been developed for the electrodiagnostic evaluation of cranial nerves XI and XII. Each of these carries both benefits and limitations, with more techniques and data being available in the literature for spinal accessory than hypoglossal nerve evaluation. Spinal accessory and hypoglossal neuropathy are relatively uncommon cranial mononeuropathies that may be evaluated in the outpatient electrodiagnostic laboratory setting. A review of available literature using PubMed was conducted regarding electrodiagnostic technique in the evaluation of spinal accessory and hypoglossal nerves searching for both routine nerve conduction studies and repetitive nerve conduction studies. The review provided herein provides a resource by which clinical neurophysiologists may develop and implement clinical and research protocols for the evaluation of both of these lower cranial nerves in the outpatient setting.

Regenerative peripheral nerve interface viability and signal transduction with an implanted electrode.

PubMed

Kung, Theodore A; Langhals, Nicholas B; Martin, David C; Johnson, Philip J; Cederna, Paul S; Urbanchek, Melanie G

2014-06-01

The regenerative peripheral nerve interface is an internal interface for signal transduction with external electronics of prosthetic limbs; it consists of an electrode and a unit of free muscle that is neurotized by a transected residual peripheral nerve. Adding a conductive polymer coating on electrodes improves electrode conductivity. This study examines regenerative peripheral nerve interface tissue viability and signal fidelity in the presence of an implanted electrode coated or uncoated with a conductive polymer. In a rat model, the extensor digitorum longus muscle was moved as a nonvascularized free tissue transfer and neurotized by the divided peroneal nerve. Either a stainless steel pad electrode (n = 8) or a pad electrode coated with poly(3,4-ethylenedioxythiophene) conductive polymer (PEDOT) (n = 8) was implanted on the muscle transfer and secured with an encircling acellular extracellular matrix. The contralateral muscle served as the control. The free muscle transfers were successfully revascularized and over time reinnervated as evidenced by serial insertional needle electromyography. Compound muscle action potentials were successfully transduced through the regenerative peripheral nerve interface. The conductive polymer coating on the implanted electrode resulted in increased recorded signal amplitude that was observed throughout the course of the study. Histologic examination confirmed axonal sprouting, elongation, and synaptogenesis within regenerative peripheral nerve interface regardless of electrode type. The regenerative peripheral nerve interface remains viable over seven months in the presence of an implanted electrode. Electrodes with and without conductive polymer reliably transduced signals from the regenerative peripheral nerve interface. Electrodes with a conductive polymer coating resulted in recording more of the regenerative peripheral nerve interface signal.

Distributed abnormalities of brain white matter architecture in patients with dominant optic atrophy and OPA1 mutations.

PubMed

Rocca, Maria A; Bianchi-Marzoli, Stefania; Messina, Roberta; Cascavilla, Maria Lucia; Zeviani, Massimo; Lamperti, Costanza; Milesi, Jacopo; Carta, Arturo; Cammarata, Gabriella; Leocani, Letizia; Lamantea, Eleonora; Bandello, Francesco; Comi, Giancarlo; Falini, Andrea; Filippi, Massimo

2015-05-01

Using advanced MRI techniques, we investigated the presence and topographical distribution of brain grey matter (GM) and white matter (WM) alterations in dominant optic atrophy (DOA) patients with genetically proven OPA1 mutation as well as their correlation with clinical and neuro-ophthalmologic findings. Nineteen DOA patients underwent neurological, neuro-ophthalmologic and brainstem auditory evoked potentials (BAEP) evaluations. Voxel-wise methods were applied to assess regional GM and WM abnormalities in patients compared to 20 healthy controls. Visual acuity was reduced in 16 patients. Six DOA patients (4 with missense mutations) had an abnormal I peripheral component (auditory nerve) at BAEP. Compared to controls, DOA patients had significant atrophy of the optic nerves (p < 0.0001). Voxel-based morphometry (VBM) analysis showed that, compared to controls, DOA patients had significant WM atrophy of the chiasm and optic tracts; whereas no areas of GM atrophy were found. Tract-based spatial statistics (TBSS) analysis showed that compared to controls, DOA patients had significantly lower mean diffusivity, axial and radial diffusivity in the WM of the cerebellum, brainstem, thalamus, fronto-occipital-temporal lobes, including the cingulum, corpus callosum, corticospinal tract and optic radiation bilaterally. No abnormalities of fractional anisotropy were detected. No correlations were found between volumetric and diffusivity abnormalities quantified with MRI and clinical and neuro-ophthalmologic measures of disease severity. Consistently with pathological studies, tissue loss in DOA patients is limited to anterior optic pathways reflecting retinal ganglion cell degeneration. Distributed abnormalities of diffusivity indexes might reflect abnormal intracellular mitochondrial morphology as well as alteration of protein levels due to OPA1 mutations.

Parkinson disease affects peripheral sensory nerves in the pharynx.

PubMed

Mu, Liancai; Sobotka, Stanislaw; Chen, Jingming; Su, Hungxi; Sanders, Ira; Nyirenda, Themba; Adler, Charles H; Shill, Holly A; Caviness, John N; Samanta, Johan E; Sue, Lucia I; Beach, Thomas G

2013-07-01

Dysphagia is very common in patients with Parkinson disease (PD) and often leads to aspiration pneumonia, the most common cause of death in PD. Current therapies are largely ineffective for dysphagia. Because pharyngeal sensation normally triggers the swallowing reflex, we examined pharyngeal sensory nerves in PD patients for Lewy pathology.Sensory nerves supplying the pharynx were excised from autopsied pharynges obtained from patients with clinically diagnosed and neuropathologically confirmed PD (n = 10) and healthy age-matched controls (n = 4). We examined the glossopharyngeal nerve (cranial nerve IX), the pharyngeal sensory branch of the vagus nerve (PSB-X), and the internal superior laryngeal nerve (ISLN) innervating the laryngopharynx. Immunohistochemistry for phosphorylated α-synuclein was used to detect Lewy pathology. Axonal α-synuclein aggregates in the pharyngeal sensory nerves were identified in all of the PD subjects but not in the controls. The density of α-synuclein-positive lesions was greater in PD patients with dysphagia versus those without dysphagia. In addition, α-synuclein-immunoreactive nerve fibers in the ISLN were much more abundant than those in cranial nerve IX and PSB-X. These findings suggest that pharyngeal sensory nerves are directly affected by pathologic processes in PD. These abnormalities may decrease pharyngeal sensation, thereby impairing swallowing and airway protective reflexes and contributing to dysphagia and aspiration.

Development of a nerve conduction technique for the recurrent laryngeal nerve.

PubMed

J Kim, Sang; G Lee, Dae; Kwon, Jeong-Yi

2014-12-01

To develop a reliable and safe laryngeal nerve conduction technique and to obtain consistent parameters as normal reference values. A prospective single-arm study. A nerve conduction test was performed on the contralateral normal side in 42 patients with unilateral vocal fold palsy. The recording was performed in the intact thyroarytenoid muscle using a monopolar needle. The electrical stimulation using a 37-mm monopolar needle was applied 3 cm below the lower margin of the cricoid cartilage, just lateral to the trachea and medial to the carotid artery, and its intensity was gradually increased until the amplitude of the electrical response reached the maximum level. The latency of the evoked muscle response was acquired at the first evoked waveform deflection from the baseline. The average latency of the recurrent laryngeal nerves was 1.98 ± 0.26 ms. The latencies showed normal distribution according to the quantile-quantile plot and Kolmogorov-Smirnov test (P = .098). There was no significant difference in latencies between the right and left recurrent laryngeal nerves. Anthropometric factors including height and weight did not show any correlation with the latencies. We developed a reliable and safe laryngeal nerve conduction technique and obtained normal reference values for the recurrent laryngeal nerve conduction study. This laryngeal nerve conduction study can be an additional tool for detecting recurrent laryngeal nerve injury if it is performed in combination with the conventional laryngeal electromyography. 4. © 2014 The American Laryngological, Rhinological and Otological Society, Inc.

Neural control of renal function in health and disease.

PubMed

DiBona, G F

1994-04-01

The renal sympathetic innervation of the kidney exerts significant effects on multiple aspects of renal function, including renal haemodynamics, tubular sodium and water reabsorption and renin secretion. These effects constitute an important control system which is important in the physiological regulation of arterial pressure and total body fluid and sodium homeostasis. Abnormalities in this regulatory mechanism have pathophysiological consequences and are manifest in clinically relevant human disease states. Decreased renal sympathetic nerve activity results in impaired renin secretion, the inability to conserve sodium normally and an attenuated ability to dispose of both acute and chronic sodium loads. Increased renal sympathetic nerve activity contributes significantly to the excess renal sodium retention and related renal abnormalities observed in both hypertension and oedema forming conditions, such as cardiac failure, cirrhosis and nephrotic syndrome.

Effect of hip and knee position on nerve conduction in the common fibular nerve.

PubMed

Broadhurst, Peter Kaas; Robinson, Lawrence R

2017-09-01

The aim of this study was to measure the influence that hip and knee position have on routine fibular motor nerve conduction studies. Healthy subjects under age 40 were recruited (n = 24) to have fibular nerve conduction studies completed in various positions, using hip extension-knee extension as a control. A mean increase in conduction velocity of 2.5 m/s across the knee (P = 0.020) was seen during hip flexion compared with hip extension. A mean decrease in velocity of 1.6 m/s through the leg segment (P = 0.016) was seen during knee flexion compared with knee extension. This study shows that the optimal position of the leg during fibular nerve studies is with the hip in flexion and knee in extension, to more accurately reflect nerve length for velocity calculations. This may have implications for other peripheral nerves with respect to proximal joint position affecting calculated velocity. Muscle Nerve 56: 519-521, 2017. © 2017 Wiley Periodicals, Inc.

Sensory conduction of the sural nerve in polyneuropathy.

PubMed

Burke, D; Skuse, N F; Lethlean, A K

1974-06-01

Using surface electrodes, sensory nerve action potentials (SAP) have been recorded in the proximal segment (mid-calf to lateral malleolus) and the distal segment (lateral malleolus to toe 5) of the sural nerve and in the median nerve in 79 control subjects. The values obtained for the distal segment of the sural nerve varied widely and in seven apparently normal subjects no SAP could be distinguished. In the proximal segment conduction velocities were over 40 m/s and there was no significant change with age, unlike the median nerve in which a highly significant slowing occurred with age. Comparison of the results of sural and median sensory conduction studies in 300 consecutive patients screened for sensory polyneuropathy confirms the value of sural nerve sensory studies as a routine screening test, and confirms the belief that the changes in polyneuropathy are usually more prominent in lower limb nerves. It is therefore suggested that studies of sural sensory conduction form the single most useful test in the diagnosis of sensory polyneuropathy.

Ultrasound-guided therapeutic injections for neural pathology about the foot and ankle: a 4 year retrospective review.

PubMed

Walter, William R; Burke, Christopher J; Adler, Ronald S

2017-06-01

To describe a 4-year clinical experience with ultrasound-guided therapeutic perineural injections of peripheral nerves about the foot and ankle. Retrospective analysis of foot and ankle perineural injections performed between January 2012 and August 2016. Demographics, clinical indications, presence of structural pathology, immediate and interval pain relief, as well as complications were recorded. Fifty-nine therapeutic injections were performed among 46 patients, accounting for multiple injections in a single visit or multiple visits [mean age = 43 years (range 18-75), 31 female (67%) and 15 male (33%)]. Most commonly, perineural injections involved the hallux branch of the medial plantar nerve (n = 17, 22%). Least commonly, perineural injections involved the saphenous nerve (n = 3, 4%). Other injections in our series include sural (10), superficial (11) and deep (7) peroneal, medial (5) and lateral (3) plantar nerves, and the posterior tibial nerve (3). Ultrasound evaluation revealed structural abnormality associated with the nerve in 30 cases (51%)-most commonly thickening with perineural scarring (n = 14). Of 45 injections with complete documentation, immediate relief of symptoms was reported in 43 (96%) cases. Interval symptom relief was achieved in 23 injections [short term (n = 12), intermediate (n = 6), and long term (n = 5)] out of 38 for which follow-up was available (61%). Complications are rare, occurring in only one case. Ultrasound-guided perineural injections about the foot and ankle are safe and provide lasting symptomatic relief for many indications. Concomitant sonographic evaluation identifies structural abnormalities that may contribute to neuropathic symptoms, allowing targeting of injection or clinical therapy.

Analysis of vestibular testing in patients with vestibular schwannoma based on the nerve of origin, the localization, and the size of the tumor.

PubMed

Suzuki, Mitsuya; Yamada, Chikako; Inoue, Rika; Kashio, Akinori; Saito, Yuki; Nakanishi, Wakako

2008-10-01

We aimed to analyze the factors influencing caloric response and vestibular evoked myogenic potential (VEMP) in vestibular schwannoma. The subjects comprised 130 patients with unilateral vestibular schwannoma pathologically diagnosed by surgery. Caloric response and the amplitude and latency of VEMP were measured and analyzed based on the nerve of origin, localization, and size of the tumor. The tumors were classified into 3 types based on localization: intracanalicular, intermediate, and medial; and into 4 grades based on size: 9 mm or less, 10 to 19 mm, 20 to 29 mm, and 30 mm or greater. : Abnormal rates of caloric response and VEMP in patients with tumors arising from the superior vestibular nerve were not significantly different from those in patients with tumors of the inferior vestibular nerve. In the intermediate and medial type-but not in the intracanalicular type-a significant difference in tumor size was observed between patients with normal caloric response and those with canal paresis as also between patients with normal VEMP and those with abnormal VEMP. In patients with tumors that maximally measured 10 to 19 mm or of the intermediate type, the p- and n-wave latencies of VEMP were significantly prolonged compared with those in the normal opposite ear. 1) The nerve of origin of tumors cannot be predicted based on caloric response and VEMP. 2) In the intermediate and medial types, caloric response and the VEMP amplitude are significantly diminished in association with an increase in tumor size. 3) Prolonged VEMP latencies seem to be not only caused by tumor compression to the brainstem or vestibular spinal tract but also by tumor compression isolated to the inferior vestibular nerve.

Effect of Limb Lengthening on Internodal Length and Conduction Velocity of Peripheral Nerve

PubMed Central

Gillingwater, Thomas H.; Anderson, Heather; Cottrell, David; Sherman, Diane L.; Ribchester, Richard R.; Brophy, Peter J.

2013-01-01

The influences of axon diameter, myelin thickness, and internodal length on the velocity of conduction of peripheral nerve action potentials are unclear. Previous studies have demonstrated a strong dependence of conduction velocity on internodal length. However, a theoretical analysis has suggested that this relationship may be lost above a nodal separation of ∼0.6 mm. Here we measured nerve conduction velocities in a rabbit model of limb lengthening that produced compensatory increases in peripheral nerve growth. Divided tibial bones in one hindlimb were gradually lengthened at 0.7 mm per day using an external frame attached to the bone. This was associated with a significant increase (33%) of internodal length (0.95–1.3 mm) in axons of the tibial nerve that varied in proportion to the mechanical strain in the nerve of the lengthened limb. Axonal diameter, myelin thickness, and g-ratios were not significantly altered by limb lengthening. Despite the substantial increase in internodal length, no significant change was detected in conduction velocity (∼43 m/s) measured either in vivo or in isolated tibial nerves. The results demonstrate that the internode remains plastic in the adult but that increases in internodal length of myelinated adult nerve axons do not result in either deficiency or proportionate increases in their conduction velocity and support the view that the internodal lengths of nerves reach a plateau beyond which their conduction velocities are no longer sensitive to increases in internodal length. PMID:23467369

The significance of nerve sugar levels for the peripheral nerve impairment of spontaneously diabetic GK (Goto-Kakizaki) rats.

PubMed

Suzuki, K; Yen-Chung, H; Toyota, T; Goto, Y; Hirata, Y; Okada, K

1990-05-01

This study was carried out to clarify the relationship between the slowing of motor nerve conduction velocity and nerve levels of sorbitol, fructose, glucose and myoinositol in spontaneously diabetic GK (Goto-Kakizaki) rats. The motor nerve conduction velocity in GK rats was constantly lower than in normal controls at three and nine months of age. This constant decrease in motor nerve conduction velocity in GK rats was closely related to glucose intolerance in GK rats soon after birth. Nerve levels of sorbitol, glucose and fructose in GK rats were significantly increased as compared to normal controls at nine months old, but not (except glucose) at three months old. The increase in nerve concentrations of sugars in GK rats was progressive with age. However, levels of glucose, sorbitol and fructose in normal Wistar rats remain unchanged with age. Although nerve myo-inositol levels in GK rats were lower at three and nine months than those of normal controls, a significant difference in myo-inositol levels was observed only at nine months. On the contrary, nerve myo-inositol level in normal Wistar rats did not show age-related change. These findings suggested that both enhanced polyol pathway activity and myo-inositol depletion play important roles in the reduction of motor nerve conduction velocity.

Folliculosebaceous cystic hamartoma with a neural component: an immunohistochemical study.

PubMed

Toyoda, M; Morohashi, M

1997-07-01

We present a case of follicular cystic hamartoma, a distinctive cutaneous malformation characterized by marked overgrowth of folliculosebaceous units accompanied by appreciable mesenchymal alterations, including fibroplasia, increased vascular components, and numerous adipocytes. A conspicuous feature of our case is an aggregation of thick trespassing nerve bundles in the deep portion of the neoplasm. An immunohistochemical study revealed the nerve bundles were immunoreactive for the general neuronal marker protein gene product 9.5. The nerves, however, stained negatively with antibodies against neuropeptides such as calcitonin gene-related peptide, substance P, vasoactive intestinal polypeptide, and neuropeptide Y, all of which are known to be contained in normal cutaneous nerves. The presence of these aberrant nerve bundles devoid of the normal expression of neuropeptides supports the concept that folliculosebaceous cystic hamartoma is a true, tumor-like malformation characterized by abnormal overgrowth of normal components of the skin.

Polyarteritis Nodosa

MedlinePlus

... may also include skin abnormalities (rash, ulcers) and peripheral neuropathy (pain, the sensations of burning, tingling, or numbness, ... Skin Kidney Gastrointestinal tract Heart Eye Genitals Nerve Peripheral neuropathies are very common (50 to 70%). This includes ...

Oculoauriculovertebral spectrum with radial anomaly in child.

PubMed

Taksande, Amar; Vilhekar, Krishna

2013-01-01

Oculoauriculovertebral spectrum (OAVS) or Goldenhar syndrome is a wide spectrum of congenital anomalies that involves structures arising from the first and second branchial arches. It is characterized by a wide spectrum of symptoms and physical features. These abnormalities mainly involve the cheekbones, jaws, mouth, ears, eyes, or vertebrae. Other conditions with ear and/or radial involvement, such as, the Nager syndrome, Holt-Oram syndrome, Radial-renal syndrome, facioauriculoradial dysplasia, Fanconi anemia, and Vertebral, Anal atresia, Cardiac, Trachea, Esophageal, Renal, and Limb (VACTERL) association should be considered for differential diagnosis. Here we report a child who had facial asymmetry, microsomia, microtia, congenital facial nerve palsy, conductive hearing loss, skin tags, iris coloboma, and preaxial polydactyly.

Indication of peripheral nerve hyperexcitability in adult-onset subacute sclerosing panencephalitis (SSPE).

PubMed

Schreurs, Annabel; Stålberg, Erik V; Punga, Anna Rostedt

2008-04-01

Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection of immune-resistant measles virus. Diagnostic hallmarks include widespread cortical dysfunction on EEG, myoclonus, white matter abnormalities on neuroradiological examination and the presence of IgG anti-measles antibodies in the cerebrospinal fluid. We present the first case of SSPE with signs of peripheral nerve hyperexcitability, observed as extra discharges following the compound motor action potential at motor nerve stimulation. In addition we demonstrate the importance of SSPE in the differential diagnosis of adult patients with psychiatric and neurological symptoms.

Structure and stability of internodal myelin in mouse models of hereditary neuropathy.

PubMed

Avila, Robin L; Inouye, Hideyo; Baek, Rena C; Yin, Xinghua; Trapp, Bruce D; Feltri, M Laura; Wrabetz, Lawrence; Kirschner, Daniel A

2005-11-01

Peripheral neuropathies often result in abnormalities in the structure of internodal myelin, including changes in period and membrane packing, as observed by electron microscopy (EM). Mutations in the gene that encodes the major adhesive structural protein of internodal myelin in the peripheral nervous system of humans and mice--P0 glycoprotein--correlate with these defects. The mechanisms by which P0 mutations interfere with myelin packing and stability are not well understood and cannot be provided by EM studies that give static and qualitative information on fixed material. To gain insights into the pathogenesis of mutant P0, we used x-ray diffraction, which can detect more subtle and dynamic changes in native myelin, to investigate myelin structure in sciatic nerves from murine models of hereditary neuropathies. We used mice with disruption of one or both copies of the P0 gene (models of Charcot-Marie-Tooth-like neuropathy [CMT1B] or Dejerine-Sottas-like neuropathy) and mice with a CMT1B resulting from a transgene encoding P0 with an amino terminal myc-tag. To directly test the structural role of P0, we also examined a mouse that expresses P0 instead of proteolipid protein in central nervous system myelin. To link our findings on unfixed nerves with EM results, we analyzed x-ray patterns from unembedded, aldehyde-fixed nerves and from plastic-embedded nerves. From the x-ray patterns recorded from whole nerves, we assessed the amount of myelin and its quality (i.e. relative thickness and regularity). Among sciatic nerves having different levels of P0, we found that unfixed nerves and, to a lesser extent, fixed but unembedded nerves gave diffraction patterns of sufficient quality to distinguish periods, sometimes differing by a few Angstroms. Certain packing abnormalities were preserved qualitatively by aldehyde fixation, and the relative amount and structural integrity of myelin among nerves could be distinguished. Measurements from the same nerve over time showed that the amount of P0 affected myelin's stability against swelling, thus directly supporting the hypothesis that packing defects underlie instability in "live" or intact myelin. Our findings demonstrate that diffraction can provide a quantitative basis for understanding, at a molecular level, the membrane packing defects that occur in internodal myelin in demyelinating peripheral neuropathies.

An Analysis of the Symptomatic Domains Most Relevant to Charcot Marie Tooth Neuropathy (CMT) Patients

ClinicalTrials.gov

2017-06-09

Charcot Marie Tooth Disease (CMT); Hereditary Sensory and Motor Neuropathy; Nerve Compression Syndromes; Tooth Diseases; Congenital Abnormalities; Genetic Diseases, Inborn; Heredodegenerative Disorders, Nervous System

Genetics Home Reference: Huntington disease-like syndrome

MedlinePlus

... abnormal protein can build up in nerve cells (neurons) and disrupt the normal functions of these cells. The dysfunction and eventual death of neurons in certain areas of the brain underlie the ...

Changes in nerve conduction and Pi/PCr ratio during denervation-reinnervation of the gastrocsoleus muscles of rats

NASA Technical Reports Server (NTRS)

Lai, K. S.; Jaweed, M. M.; Seestead, R.; Herbison, G. J.; Ditunno, J. F. Jr; McCully, K.; Chance, B.

1992-01-01

The purpose of this investigation was to study the changes in nerve conduction and phosphate metabolites of the gastrocsoleus muscles of rats during denervation-reinnervation. Sixteen male Sprague-Dawley rats underwent unilateral crush-denervation of the left sciatic nerves at the sciatic notch. Six rats were used for measurement of motor conduction latency and action potential amplitude of the gastrocsoleus muscle by stimulating the sciatic nerve at one, two and eight weeks after nerve crush. The other ten rats were designated for evaluation of the ratio of inorganic phosphorous (Pi) to phosphocreatine (PCr) by a 31P-phosphoenergetic spectrometer at two weeks and eight weeks after nerve crush. None of the sciatic nerves showed conduction to the gastrocsoleus at one or two weeks after nerve crush. At eight weeks postcrush, the motor conduction latency returned to within normal limits, whereas the action potential amplitude was only 55% of the normal. For the eight-week period of study, the Pi/PCr ratio of the normal control muscles ranged between 0.09 +/- 0.02 and 0.11 +/- 0.02 (mean +/- SD). The denervated muscles showed an increase of Pi/PCr ratio by 54% at two weeks postcrush, compared to the respective contralateral control sides. The ratios returned to the normal value by eight weeks postcrush. In summary, these data suggested that the metabolic recovery of the crush-denervated muscle followed the same pattern as the parameters of nerve conduction.

Severe sensory neuropathy in patients with adult-onset multiple acyl-CoA dehydrogenase deficiency.

PubMed

Wang, Zhaoxia; Hong, Daojun; Zhang, Wei; Li, Wurong; Shi, Xin; Zhao, Danhua; Yang, Xu; Lv, He; Yuan, Yun

2016-02-01

Multiple Acyl-CoA dehydrogenase deficiency (MADD) is an autosomal recessive disorder of fatty acid oxidation. Most patients with late-onset MADD are clinically characterized by lipid storage myopathy with dramatic responsiveness to riboflavin treatment. Abnormalities of peripheral neuropathy have rarely been reported in patients with late-onset MADD. We describe six patients who presented with proximal limb weakness and loss of sensation in the distal limbs. Muscle biopsy revealed typical myopathological patterns of lipid storage myopathy and blood acylcarnitine profiles showed a combined elevation of multiple acylcarnitines supporting the diagnosis of MADD. However, nerve conduction investigations and sural nerve biopsies in these patients indicated severe axonal sensory neuropathy. Causative ETFDH gene mutations were found in all six cases. No other causative gene mutations were identified in mitochondrial DNA and genes associated with hereditary neuropathies through next-generation-sequencing panel. Late-onset patients with ETFDH mutations can present with proximal muscle weakness and distal sensory neuropathy, which might be a new phenotypic variation, but the precise underlying pathogenesis remains to be elucidated. Copyright © 2015. Published by Elsevier B.V.

[A young woman with central facial nerve palsy].

PubMed

Broere, Christiaan M; de Witte, B R René; Claes, J F H M Franka

2014-01-01

The distinction between central and peripheral facial nerve palsy can be difficult but is very important for the workup and treatment. A tumefactive demyelinating lesion (TDL) is a rare condition that can sometimes cause diagnostic difficulties due to its similarity to a brain tumour. We present a 20-year-old female patient who visited her GP with a discrete right-sided drooping corner of her mouth. The GP started treatment with oral glucorticoids because of presumed Bell's palsy and referred her to the neurology outpatient clinic. Repeated neurological examination showed central facial palsy on the right side of the face. An MRI study of the brain revealed a single large contrast-enhanced abnormality in the left hemisphere that was diagnosed as TDL after exclusion of other causes. In view of the limited number of clinical symptoms, an expectative policy was conducted. The patient recovered spontaneously and repeated MRI studies showed partial regression of TDL. TDL is often considered to be a first presentation of multiple sclerosis. Accurate analysis with MRI can help in making a diagnosis without the need for a biopsy.

Electrodiagnostic features of acute paralytic poliomyelitis associated with West Nile virus infection.

PubMed

Al-Shekhlee, Amer; Katirji, Bashar

2004-03-01

West Nile virus (WNV) infection is a potentially fatal disease, with meningoencephalitis being its most common neurological manifestation. Guillain-Barré syndrome (GBS) has also been described, but acute paralytic poliomyelitis has only recently been recognized. We report the clinical and electrodiagnostic findings of five patients with WNV infection, who presented with acute paralytic poliomyelitis. Three patients manifested focal asymmetrical weakness, and two had rapid ascending quadriplegia mimicking GBS. Electrodiagnostic studies during the acute illness showed normal sensory nerve action potentials, compound motor action potentials of normal or reduced amplitude, and no slowing of nerve conduction velocities. Depending on the timing of the examination, fibrillation potentials were widespread, including in those with focal weakness. Cervical magnetic resonance imaging in one patient showed abnormal T2-weighted signals in the spinal cord gray matter. On follow-up, signs of clinical improvement were seen in one patient, whereas two remained quadriplegic and ventilator-dependent 5 months after the onset. This report highlights the value of the electrodiagnostic studies in the diagnosis and prognosis of focal or generalized weakness due to acute paralytic poliomyelitis associated with WNV infection.

Association between wrist ratio and carpal tunnel syndrome: Effect modification by body mass index.

PubMed

Thiese, Matthew S; Merryweather, Andrew; Koric, Alzina; Ott, Ulrike; Wood, Eric M; Kapellusch, Jay; Foster, James; Garg, Arun; Deckow-Schaefer, Gwen; Tomich, Suzanna; Kendall, Richard; Drury, David L; Wertsch, Jacqueline; Hegmann, Kurt T

2017-12-01

Previous studies have reported higher wrist ratios (WR) related to carpal tunnel syndrome (CTS) but have not assessed effect modification by obesity and may have inadequately controlled for confounders. Baseline data of a multicenter prospective cohort study were analyzed. CTS was defined by nerve conduction study (NCS) criteria and symptoms. Among the 1,206 participants, a square-shaped wrist was associated with CTS after controlling for confounders (prevalence ratio = 2.27; 95% confidence interval [95% CI], 1.33-3.86). Body mass index (BMI) was a strong effect modifier on the relationship between WR and both CTS and abnormal NCS results, with normal weight strata of rectangular versus square wrists = 8.18 (95% CI, 1.63-49.96) and 7.12 (95% CI, 2.19-23.16), respectively. A square wrist is significantly associated with CTS after controlling for confounders. Effect modification by high BMI masked the eightfold magnitude adjusted relationship seen between WR and CTS among normal weight participants. Muscle Nerve 56: 1047-1053, 2017. © 2017 Wiley Periodicals, Inc.

Results of intraoperative neuromonitoring in thyroid surgery and preoperative vocal cord paralysis.

PubMed

Lorenz, Kerstin; Abuazab, Mohammed; Sekulla, Carsten; Schneider, Rick; Nguyen Thanh, Phuong; Dralle, Henning

2014-03-01

Systematic studies of intermittent intraoperative neuromonitoring (IONM) have shown that IONM enhances recurrent laryngeal nerve (RLN) identification via functional assessment, but does not significantly reduce rates of vocal cord (VC) paralysis (VCP). The reliability of functional nerve assessment depends on the preoperative integrity of VC mobility. The present study was therefore performed to analyze the validity of IONM in patients with pre-existing VC paralysis. Of 8,128 patients, 285 (3.5 %) with preoperative VCP underwent thyroid surgery using standardized IONM of the RLN and vagus nerves (VNs). VC function was assessed by pre- and postoperative direct videolaryngoscopy. Quantitative parameters of IONM in patients with VCP were compared with IONM in patients with intact VC function. Clinical symptoms and surgical outcomes of patients with pre-existing VCP were analyzed. A total of 244 patients revealed negative, and 41 revealed positive IONM on the side of the VCP. VCP with positive IONM revealed significantly lower amplitudes of VN and RLN than intact VN (p = 0.010) and RLN (p = 0.011). Symptoms of patients with VCP included hoarseness (25 %), dyspnea (29 %), stridor (13 %), and dysphagia (13 %); 13 % were asymptomatic. New VCP occurred in five patients, ten needed tracheostomy for various reasons, and one patient died. Patients with pre-existing VCP revealed significantly reduced amplitude of ipsilateral VN and RLN, indicating retained nerve conductivity despite VC immobility. Preoperative laryngoscopy is therefore indispensable for reliable IONM and risk assessment, even in patients without voice abnormalities.

Awareness of dysgeusia and gustatory tests in patients undergoing chemotherapy for breast cancer.

PubMed

Kuba, Sayaka; Fujiyama, Rie; Yamanouchi, Kosho; Morita, Michi; Sakimura, Chika; Hatachi, Toshiko; Matsumoto, Megumi; Yano, Hiroshi; Takatsuki, Mitsuhisa; Hayashida, Naomi; Nagayasu, Takeshi; Eguchi, Susumu

2018-05-12

We analyzed the prevalence of gustatory test abnormalities in breast cancer (BC) patients undergoing chemotherapy. We enrolled 43 BC patients undergoing chemotherapy and 38 BC patients who had never undergone chemotherapy (control group). Two gustatory tests were conducted: an instillation method examining the threshold for four basic taste stimuli and an electrogustometry method measuring the threshold for perception with electric stimulation at the front two-thirds of the tongue (cranial nerve VII) and at the back third of the tongue (cranial nerve IX). The results of the two gustatory tests and clinicopathological factors were compared between the chemotherapy and control groups and between patients with and without awareness of dysgeusia in the chemotherapy group. In the chemotherapy group, 19 (44%) patients were aware of dysgeusia and 8 (19%) had hypogeusia using the instillation method. Although more patients had parageusia in the chemotherapy than control group, no significant differences in the results of the two gustatory tests were observed. Patients with dysgeusia awareness had a higher threshold at cranial nerve IX using the electrogustometry method than those without dysgeusia awareness; no significant differences in hypogeusia were observed using the instillation method. In fact, 74% (14/19) of patients with dysgeusia awareness could identify the four tastes accurately using the instillation method. Similar results were observed for the instillation and electrogustometry methods at cranial nerve VII. While approximately half of the chemotherapy patients were aware of dysgeusia, 81% (35/43) of them could accurately identify the four basic tastes using the instillation method.

Diagnostic Value of Facial Nerve Antidromic Evoked Potential in Patients With Bell's Palsy: A Preliminary Study

PubMed Central

Lee, Ji Hoon; Kim, Sun Mi; Yang, Hea Eun; Lee, Jang Woo

2014-01-01

Objective To assess the practical diagnostic value of facial nerve antidromic evoked potential (FNAEP), we compared it with the diagnostic value of the electroneurography (ENoG) test in Bell's palsy. Methods In total, 20 patients with unilateral Bell's palsy were recruited. Between the 1st and 17th days after the onset of facial palsy, FNAEP and ENoG tests were conducted. The degeneration ratio and FNAEP latency difference between the affected and unaffected sides were calculated in all subjects. Results In all patients, FNAEP showed prolonged latencies on the affected side versus the unaffected side. The difference was statistically significant. In contrast, there was no significant difference between sides in the normal control group. In 8 of 20 patients, ENoG revealed a degeneration ratio less than 50%, but FNAEP show a difference of more than 0.295±0.599 ms, the average value of normal control group. This shows FNAEP could be a more sensitive test for Bell's palsy diagnosis than ENoG. In particular, in 10 patients tested within 7 days after onset, an abnormal ENoG finding was noted in only four of them, but FNAEP showed a significant latency difference in all patients at this early stage. Thus, FANEP was more sensitive in detecting facial nerve injury than the ENoG test (p=0.031). Conclusion FNAEP has some clinical value in the diagnosis of facial nerve degeneration. It is important that FNAEP be considered in patients with facial palsy at an early stage and integrated with other relevant tests. PMID:25024963

Vocal cord collapse during phrenic nerve-paced respiration in congenital central hypoventilation syndrome.

PubMed

Domanski, Mark C; Preciado, Diego A

2012-01-01

Phrenic nerve pacing can be used to treat congenital central hypoventilation syndrome (CCHS). We report how the lack of normal vocal cord tone during phrenic paced respiration can result in passive vocal cord collapse and produce obstructive symptoms. We describe a case of passive vocal cord collapse during phrenic nerve paced respiration in a patient with CCHS. As far as we know, this is the first report of this etiology of airway obstruction. The patient, a 7-year-old with CCHS and normal waking vocal cord movement, continued to require nightly continuous positive airway pressure (CPAP) despite successful utilization of phrenic nerve pacers. On direct laryngoscopy, the patient's larynx was observed while the diaphragmatic pacers were sequentially engaged. No abnormal vocal cord stimulation was witnessed during engaging of either phrenic nerve stimulator. However, the lack of normal inspiratory vocal cord abduction during phrenic nerve-paced respiration resulted in vocal cord collapse and partial obstruction due to passive adduction of the vocal cords through the Bernoulli effect. Bilateral phrenic nerve stimulation resulted in more vocal cord collapse than unilateral stimulation. The lack of vocal cord abduction on inspiration presents a limit to phrenic nerve pacers.

Ultrasonographic findings in hereditary neuropathy with liability to pressure palsies.

PubMed

Bayrak, Ayse O; Bayrak, Ilkay Koray; Battaloglu, Esra; Ozes, Burcak; Yildiz, Onur; Onar, Musa Kazim

2015-02-01

The aims of this study were to evaluate the sonographic findings of patients with hereditary neuropathy with liability to pressure palsies (HNPP) and to examine the correlation between sonographic and electrophysiological findings. Nine patients whose electrophysiological findings indicated HNPP and whose diagnosis was confirmed by genetic analysis were enrolled in the study. The median, ulnar, peroneal, and tibial nerves were evaluated by ultrasonography. We ultrasonographically evaluated 18 median, ulnar, peroneal, and tibial nerves. Nerve enlargement was identified in the median, ulnar, and peroneal nerves at the typical sites of compression. None of the patients had nerve enlargement at a site of noncompression. None of the tibial nerves had increased cross-sectional area (CSA) values. There were no significant differences in median, ulnar, and peroneal nerve distal motor latencies (DMLs) between the patients with an increased CSA and those with a normal CSA. In most cases, there was no correlation between electrophysiological abnormalities and clinical or sonographic findings. Although multiple nerve enlargements at typical entrapment sites on sonographic evaluation can suggest HNPP, ultrasonography cannot be used as a diagnostic tool for HNPP. Ultrasonography may contribute to the differential diagnosis of HNPP and other demyelinating polyneuropathies or compression neuropathies; however, further studies are required.

Use of a Novel High-Resolution Magnetic Resonance Neurography Protocol to Detect Abnormal Dorsal Root Ganglia in Sjögren Patients With Neuropathic Pain

PubMed Central

Birnbaum, Julius; Duncan, Trisha; Owoyemi, Kristie; Wang, Kenneth C.; Carrino, John; Chhabra, Avneesh

2014-01-01

Abstract The diagnosis and treatment of patients with Sjögren syndrome (SS) with neuropathic pain pose several challenges. Patients with SS may experience unorthodox patterns of burning pain not conforming to a traditional “stocking-and-glove” distribution, which can affect the face, torso, and proximal extremities. This distribution of neuropathic pain may reflect mechanisms targeting the proximal-most element of the peripheral nervous system—the dorsal root ganglia (DRG). Skin biopsy can diagnose such a small-fiber neuropathy and is a surrogate marker of DRG neuronal cell loss. However, SS patients have been reported who have similar patterns of proximal neuropathic pain, despite having normal skin biopsy studies. In such cases, DRGs may be targeted by mechanisms not associated with neuronal cell loss. Therefore, alternative approaches are warranted to help characterize abnormal DRGs in SS patients with proximal neuropathic pain. We performed a systematic review of the literature to define the frequency and spectrum of SS peripheral neuropathies, and to better understand the attribution of SS neuropathic pain to peripheral neuropathies. We found that the frequency of SS neuropathic pain exceeded the prevalence of peripheral neuropathies, and that painful peripheral neuropathies occurred less frequently than neuropathies not always associated with pain. We developed a novel magnetic resonance neurography (MRN) protocol to evaluate DRG abnormalities. Ten SS patients with proximal neuropathic pain were evaluated by this MRN protocol, as well as by punch skin biopsies evaluating for intraepidermal nerve fiber density (IENFD) of unmyelinated nerves. Five patients had radiographic evidence of DRG abnormalities. Patients with MRN DRG abnormalities had increased IENFD of unmyelinated nerves compared to patients without MRN DRG abnormalities (30.2 [interquartile range, 4.4] fibers/mm vs. 11.0 [4.1] fibers/mm, respectively; p = 0.03). Two of these 5 SS patients whose neuropathic pain resolved with intravenous immunoglobulin (IVIg) therapy had improvement of MRN DRG abnormalities. We have developed a novel MRN protocol that can detect DRG abnormalities in SS patients with neuropathic pain who do not have markers of peripheral neuropathy. We found that SS patients with MRN DRG abnormalities had statistically significant, increased IENFD on skin biopsy studies, which may suggest a relationship between trophic mediators and neuropathic pain. Given that our literature review has demonstrated that many SS neuropathic pain patients do not have a neuropathy, our findings suggest an important niche for this MRN DRG technique in the evaluation of broader subsets of SS neuropathic pain patients who may not have underlying neuropathies. The improvement of MRN DRG abnormalities in patients with IVIg-induced remission of neuropathic pain suggests that our MRN protocol may be capturing reversible, immune-mediated mechanisms targeting the DRG. PMID:24797167

Optimal learning in a virtual patient simulation of cranial nerve palsies: the interaction between social learning context and student aptitude.

PubMed

Johnson, Teresa R; Lyons, Rebecca; Chuah, Joon Hao; Kopper, Regis; Lok, Benjamin C; Cendan, Juan C

2013-01-01

Simulation in medical education provides students with opportunities to practice interviews, examinations, and diagnosis formulation related to complex conditions without risks to patients. To examine differences between individual and team participation on learning outcomes and student perspectives through use of virtual patients (VPs) for teaching cranial nerve (CN) evaluation. Fifty-seven medical students were randomly assigned to complete simulation exercises either as individuals or as members of three-person teams. Students interviewed, examined, and diagnosed VPs with possible CN damage in the neurological exam rehearsal virtual environment (NERVE). Knowledge of CN abnormalities was assessed pre- and post-simulation. Student perspectives of system usability were evaluated post-simulation. An aptitude-treatment interaction (ATI) effect was detected; at pre-test scores ≤ 50%, students in teams scored higher (83%) at post-test than did students as individuals (62%, p = 0.02). Post-simulation, students in teams reported greater confidence in their ability to diagnose CN abnormalities than did students as individuals (p = 0.02; mean rating = 4.0/5.0 and 3.4/5.0, respectively). The ATI effect allows us to begin defining best practices for the integration of VP simulators into the medical curriculum. We are persuaded to implement future NERVE exercises with small teams of medical students.

Neck ultrasonography for detection of non-recurrent laryngeal nerve

PubMed Central

Citton, Marilisa; Viel, Giovanni

2016-01-01

Background Non-recurrent laryngeal nerve (NRLN) is a rare anatomical variant (0.3–6%) that is associated with some arterial abnormalities (absence of the brachiocephalic trunk and presence of a right aberrant subclavian lusorian artery). The availability of a preoperative diagnosis of NRLN may reduce the risk of nerve injuries. Preoperative ultrasonography (US) has been suggested as a reliable diagnostic tool to detect the arterial abnormalities associated with NRLN, but the literature is relatively scarce. This paper was aimed to review the literature, in order to offer an up to-date on this technique and its results. Methods A web search, focusing on humans, was performed by PubMed database, including papers published up to August 2016, using the key words “ultrasonography” AND “non-recurrent laryngeal nerve” or “nonrecurrent laryngeal nerve”. Results Eight papers, including 3,740 patients who underwent neck US for the detection of NRLN were selected. Only five studies focused on the preoperative use of US. The incidence of NRLN varied between 0.4% and 1.94%. The sensitivity and specificity varied between 99–100% and 41–100%, respectively. Conclusions US is a simple, non-invasive and cost-effective method to detect NRLN, also if its accuracy is not absolute. It may be used preoperatively and to prevent the intraoperative nerve damage, since the risk of NRLN palsies is significantly reduced when a preoperative diagnosis is available. PMID:28149804

Optimal learning in a virtual patient simulation of cranial nerve palsies: The interaction between social learning context and student aptitude

PubMed Central

JOHNSON, TERESA R.; LYONS, REBECCA; CHUAH, JOON HAO; KOPPER, REGIS; LOK, BENJAMIN C.; CENDAN, JUAN C.

2013-01-01

Background Simulation in medical education provides students with opportunities to practice interviews, examinations, and diagnosis formulation related to complex conditions without risks to patients. Aim To examine differences between individual and team participation on learning outcomes and student perspectives through use of virtual patients (VPs) for teaching cranial nerve (CN) evaluation. Methods Fifty-seven medical students were randomly assigned to complete simulation exercises either as individuals or as members of three-person teams. Students interviewed, examined, and diagnosed VPs with possible CN damage in the Neurological Exam Rehearsal Virtual Environment (NERVE). Knowledge of CN abnormalities was assessed pre- and post-simulation. Student perspectives of system usability were evaluated post-simulation. Results An aptitude-treatment interaction (ATI) effect was detected; at pre-test scores ≤50%, students in teams scored higher (83%) at post-test than did students as individuals (62%, p = 0.02). Post-simulation, students in teams reported greater confidence in their ability to diagnose CN abnormalities than did students as individuals (p = 0.02; mean rating = 4.0/5.0 and 3.4/5.0, respectively). Conclusion The ATI effect allows us to begin defining best practices for the integration of VP simulators into the medical curriculum. We are persuaded to implement future NERVE exercises with small teams of medical students. PMID:22938679

Genetics Home Reference: Simpson-Golabi-Behmel syndrome

MedlinePlus

... Wilms tumor and a cancerous tumor called a neuroblastoma that arises from developing nerve cells. Related Information ... Heart Defects Health Topic: Craniofacial Abnormalities Health Topic: Neuroblastoma Health Topic: Wilms Tumor Genetic and Rare Diseases ...

Genetics Home Reference: Alport syndrome

MedlinePlus

... particularly the organ of Corti, that transform sound waves into nerve impulses for the brain. Alterations in type IV collagen often result in abnormal inner ear function, which can lead to hearing loss. In the ...

21 CFR 882.5830 - Implanted diaphragmatic/phrenic nerve stimulator.

Code of Federal Regulations, 2012 CFR

2012-04-01

... which an abnormally low amount of air enters the lungs) caused by brain stem disease, high cervical spinal cord injury, or chronic lung disease. The stimulator consists of an implanted receiver with...

21 CFR 882.5830 - Implanted diaphragmatic/phrenic nerve stimulator.

Code of Federal Regulations, 2013 CFR

2013-04-01

... which an abnormally low amount of air enters the lungs) caused by brain stem disease, high cervical spinal cord injury, or chronic lung disease. The stimulator consists of an implanted receiver with...

21 CFR 882.5830 - Implanted diaphragmatic/phrenic nerve stimulator.

Code of Federal Regulations, 2014 CFR

2014-04-01

... which an abnormally low amount of air enters the lungs) caused by brain stem disease, high cervical spinal cord injury, or chronic lung disease. The stimulator consists of an implanted receiver with...

Sciatic Nerve Conductivity is Impaired by Hamstring Strain Injuries.

PubMed

Kouzaki, Karina; Nakazato, Koichi; Mizuno, Masuhiko; Yonechi, Tooru; Higo, Yusuke; Kubo, Yoshiaki; Kono, Tokuyoshi; Hiranuma, Kenji

2017-10-01

The aim of this study was to assess sciatic nerve conductivity in athletes with a history of hamstring strain injuries. Twenty-seven athletes with a history of hamstring strain injuries were included in the injured group. The control group consisted of 16 uninjured participants. We measured the proximal and distal latencies and calculated the sciatic nerve conduction velocity to evaluate neuronal conductivity. The results were expressed as median values and interquartile ranges. Both proximal latency and distal latency of the injured limb in the injured group were significantly longer than those of the uninjured limb (p<0.05). The nerve conduction velocity of the injured limb in the injured group was significantly lower than that of the uninjured limb (p<0.05). There were no significant side-to-side differences in the control group. Sciatic nerve conductivity impairments may exist in athletes with a history of hamstring strain injuries. © Georg Thieme Verlag KG Stuttgart · New York.

An Evaluation of Peripapillary Retinal Nerve Fiber Layer Thickness in Children With Epilepsy Receiving Treatment of Valproic Acid.

PubMed

Dereci, Selim; Koca, Tuğba; Akçam, Mustafa; Türkyilmaz, Kemal

2015-07-01

We investigated the peripapillary retinal nerve fiber layer thickness with optical coherence tomography in epileptic children receiving valproic acid monotherapy. The study was conducted on children aged 8-16 years who were undergoing valproic acid monotherapy for epilepsy. The study group comprised a total of 40 children who met the inclusion criteria and 40 healthy age- and sex-matched children as a control group. Children with at least a 1-year history of epilepsy and taking 10-40 mg/kg/day treatment were included in the study. Peripapillary retinal nerve fiber layer thickness measurements were performed using Cirrus HD optical coherence tomography. All children and parents were informed about the study and informed consent was obtained from the parents of all the participants. The study group included 21 girls and 19 boys with a mean age of 10.6 ± 2.3 years. According to the results of optical coherence tomography measurements, the mean peripapillary retinal nerve fiber layer thickness was 91.6 ± 9.7 in the patient group and 95.5 ± 7.4 μm in the control group (P < 0.05). The superior peripapillary retinal nerve fiber layer thickness was 112.0 ± 13.2 in the patient group and 120.0 ± 14.7 μm in the control group (P < 0.02). According to the results of both measurements, the peripapillary retinal nerve fiber layer thickness was significantly lower in the patient group. Neither color vision loss nor visual field examination abnormality could be documented. According to the optical coherence tomography measurements, the average and superior peripapillary retinal nerve fiber layer thicknesses were thinner in patients with epilepsy who were receiving valproic acid monotherapy compared with healthy children. This situation can lead to undesirable results in terms of eye health. New studies are needed to investigate whether these findings are the result of epilepsy or can be attributed to valproic acid and whether there are adverse effects of valproic acid later in life. Copyright © 2015 Elsevier Inc. All rights reserved.

The Snapping Elbow Syndrome as a Reason for Chronic Elbow Neuralgia in a Tennis Player - MR, US and Sonoelastography Evaluation.

PubMed

Łasecki, Mateusz; Olchowy, Cyprian; Pawluś, Aleksander; Zaleska-Dorobisz, Urszula

2014-01-01

Ulnar neuropathy is the second most common peripheral nerve neuropathy after median neuropathy, with an incidence of 25 cases per 100 000 men and 19 cases per 100 000 women each year. Skipping (snapping) elbow syndrome is an uncommon cause of pain in the posterior-medial elbow area, sometimes complicated by injury of the ulnar nerve. One of the reason is the dislocation of the abnormal insertion of the medial triceps head over the medial epicondyle during flexion and extension movements. Others are: lack of the Osboune fascia leading to ulnar nerve instability and focal soft tissue tumors (fibromas, lipomas, etc). Recurrent subluxation of the nerve at the elbow results in a tractional and frictional neuritis with classical symptoms of peripheral neuralgia. As far as we know snapping triceps syndrome had never been evaluated in sonoelastography. A 28yo semi-professional left handed tennis player was complaining about pain in posterior-medial elbow area. Initial US examination suggest golfers elbow syndrome which occurs quite commonly and has a prevalence of 0.3-0.6% in males and 0-3-1.1% in women and may be associated (approx. 50% of cases) with ulnar neuropathy. However subsequently made MRI revealed unusual distal triceps anatomy, moderate ulnar nerve swelling and lack of medial epicondylitis symptoms. Followed (second) US examination and sonoelastography have detected slipping of the both ulnar nerve and the additional band of the medial triceps head. Snapping elbow syndrome is a poorly known medical condition, sometimes misdiagnosed as the medial epicondylitis. It describes a broad range of pathologies and anatomical abnormalities. One of the most often reasons is the slipping of the ulnar nerve as the result of the Osborne fascia/anconeus epitrochlearis muscle absence. Simultaneously presence of two or more "snapping reasons" is rare but should be always taken under consideration. There are no sonoelastography studies describing golfers elbow syndrome, additional triceps band and ulnar neuritis. Our data suggest that the sonoelastography signs are similar to those seen in well described lateral epicondylitis syndrome, Achilles tendinitis and medial nerve neuralgia.

Impact of anatomical parameters on optical coherence tomography retinal nerve fiber layer thickness abnormality patterns

NASA Astrophysics Data System (ADS)

Baniasadi, Neda; Wang, Mengyu; Wang, Hui; Jin, Qingying; Mahd, Mufeed; Elze, Tobias

2017-02-01

Purpose: To evaluate the effects of four anatomical parameters (angle between superior and inferior temporal retinal arteries [inter-artery angle, IAA], optic disc [OD] rotation, retinal curvature, and central retinal vessel trunk entry point location [CRVTL]) on retinal nerve fiber layer thickness (RNFLT) abnormality marks by OCT machines. Methods: Cirrus OCT circumpapillary RNFLT measurements and Humphrey visual fields (HVF 24-2) of 421 patients from a large glaucoma clinic were included. Ellipses were fitted to the OD borders. Ellipse rotation relative to the vertical axis defined OD rotation. CRVTL was manually marked on the horizontal axis of the ellipse on the OCT fundus image. IAA was calculated between manually marked retinal artery locations at the 1.73mm radius around OD. Retinal curvature was determined by the inner limiting membrane on the horizontal B-scan closest to the OD center. For each location on the circumpapillary scanning area, logistic regression was used to determine if each of the four parameters had a significant impact on RNFLT abnormality marks independent of disease severity. The results are presented on spatial maps of the entire scanning area. Results: Variations in IAA significantly influenced abnormality marks on 38.8% of the total scanning area, followed by CRVTL (19.2%) and retinal curvature (18.7%). The effect of OD rotation was negligible (<1%). Conclusions: A natural variation in IAA, retinal curvature, and CRVTL can affect OCT abnormality ratings, which may bias clinical diagnosis. Our spatial maps may help OCT manufacturers to introduce location specific norms to ensure that abnormality marks indicate ocular disease instead of variations in eye anatomy.

Effect of diabetic retinopathy and panretinal photocoagulation on retinal nerve fiber layer and optic nerve appearance.

PubMed

Lim, Michele C; Tanimoto, Suzana A; Furlani, Bruno A; Lum, Brent; Pinto, Luciano M; Eliason, David; Prata, Tiago S; Brandt, James D; Morse, Lawrence S; Park, Susanna S; Melo, Luiz A S

2009-07-01

To determine if panretinal photocoagulation (PRP) alters retinal nerve fiber layer (RNFL) thickness and optic nerve appearance. Patients with diabetes who did and did not undergo PRP and nondiabetic control subjects were enrolled in a prospective study. Participants underwent optical coherence tomography of the peripapillary retina and optic nerve. Stereoscopic optic nerve photographs were graded in a masked fashion. Ninety-four eyes of 48 healthy individuals, 89 eyes of 55 diabetic patients who did not undergo PRP, and 37 eyes of 24 subjects with diabetes who underwent PRP were included in this study. Eyes that had been treated with PRP had thinner peripapillary RNFL compared with the other groups; this was statistically significantly different in the inferior (P = .004) and nasal (P = .003) regions. Optic nerve cupping did not increase with severity of disease classification, but the proportion of optic nerves graded as suspicious for glaucoma or as having nonglaucomatous optic neuropathy did (P = .008). These grading categories were associated with thinner RNFL measurements. Diabetic eyes that have been treated with PRP have thinner RNFL than nondiabetic eyes. Optic nerves in eyes treated with PRP are more likely to be graded as abnormal, but their appearance is not necessarily glaucomatous and may be related to thinning of the RNFL.

Reversible median nerve impairment after three weeks of repetitive work.

PubMed

Tabatabaeifar, Sorosh; Svendsen, Susanne Wulff; Johnsen, Birger; Hansson, Gert-Åke; Fuglsang-Frederiksen, Anders; Frost, Poul

2017-03-01

Objectives The aim of this study was to evaluate the development of impaired median nerve function in relation to hand-intensive seasonal work. We hypothesized that at end-season, median nerve conduction would be impaired and then recover within weeks. Methods Using nerve conduction studies (NCS), we examined median nerve conduction before, during, and after engaging in 22 days of mink skinning. For a subgroup, we used goniometry and surface electromyography to characterize occupational mechanical exposures. Questionnaire information on symptoms, disability, and lifestyle factors was obtained. Results The study comprised 11 male mink skinners with normal median nerve conduction at pre-season (mean age 35.7 years, mean number of seasons with skinning 8.9 years). Mink skinning was characterized by a median angle of wrist flexion/extension of 16º extension, a median velocity of wrist flexion/extension of 22 °/s, and force exertions of 11% of maximal voluntary electrical activity. At end-season, mean distal motor latency (DML) had increased 0.41 ms (P<0.001), mean sensory nerve conduction velocity (SNCV) digit 2 had decreased 6.3 m/s (P=0.004), and mean SNCV digit 3 had decreased 6.2 m/s (P=0.01); 9 mink skinners had decreases in nerve conduction, 5 fulfilled electrodiagnostic criteria and 4 fulfilled electrodiagnostic and clinical criteria (a positive Katz hand diagram) for carpal tunnel syndrome (CTS). Three to six weeks post-season, the changes had reverted to normal. Symptom and disability scores showed corresponding changes. Conclusions In this natural experiment, impaired median nerve conduction developed during 22 days of repetitive industrial work with moderate wrist postures and limited force exertion. Recovery occurred within 3-6 weeks post-season.

Technology-enabled examinations of cardiac rhythm, optic nerve, oral health, tympanic membrane, gait and coordination evaluated jointly with routine health screenings: an observational study at the 2015 Kumbh Mela in India

PubMed Central

Gupta, Otkrist; Patalano II, Vincent; Mohit, Mrinal; Merchant, Rikin; Subramanian, S V

2018-01-01

Objectives Technology-enabled non-invasive diagnostic screening (TES) using smartphones and other point-of-care medical devices was evaluated in conjunction with conventional routine health screenings for the primary care screening of patients. Design Dental conditions, cardiac ECG arrhythmias, tympanic membrane disorders, blood oxygenation levels, optic nerve disorders and neurological fitness were evaluated using FDA-approved advanced smartphone powered technologies. Routine health screenings were also conducted. A novel remote web platform was developed to allow expert physicians to examine TES data and compare efficacy with routine health screenings. Setting The study was conducted at a primary care centre during the 2015 Kumbh Mela in Maharashtra, India. Participants 494 consenting 18–90 years old adults attending the 2015 Kumbh Mela were tested. Results TES and routine health screenings identified unique clinical conditions in distinct patients. Intraoral fluorescent imaging classified 63.3% of the population with dental caries and periodontal diseases. An association between poor oral health and cardiovascular illnesses was also identified. Tympanic membrane imaging detected eardrum abnormalities in 13.0% of the population, several with a medical history of hearing difficulties. Gait and coordination issues were discovered in eight subjects and one subject had arrhythmia. Cross-correlations were observed between low oxygen saturation and low body mass index (BMI) with smokers (p=0.0087 and p=0.0122, respectively), and high BMI was associated with elevated blood pressure in middle-aged subjects. Conclusions TES synergistically identified clinically significant abnormalities in several subjects who otherwise presented as normal in routine health screenings. Physicians validated TES findings and used routine health screening data and medical history responses for comprehensive diagnoses for at-risk patients. TES identified high prevalence of oral diseases, hypertension, obesity and ophthalmic conditions among the middle-aged and elderly Indian population, calling for public health interventions. PMID:29678964

Histomorphometric and Ultrastructural Evaluation of Long-Term Alpha Lipoic Acid and Vitamin B12 Use After Experimental Sciatic Nerve Injury in Rats.

PubMed

Arikan, Murat; Togral, Guray; Hasturk, Askin Esen; Horasanli, Bahriye; Helvacioglu, Fatma; Dagdeviren, Atilla; Tekindal, Mustafa Agah; Parpucu, Murat

2016-01-01

To analyze the therapeutic effects of long-term alpha lipoic acid (A-LA) and vitamin B12 use via histomorphometric methods and electron microscopy in the transected sciatic nerves of rats. Forty rats were randomized into five groups (n=8/group). In group I, 1 cm segment of sciatic nerve was resected without any other intervention. In group II (sham), following right sciatic nerve transection, primary epineurial anastomosis was performed by placing the edges of the nerve end-to-end. In group III (saline), after right sciatic nerve transection, the ends of the nerves were brought together and closed after application of intraperitoneal physiologic saline. In group IV, 2 mg/kg of alpha lipoic acid and in group V, 2 mg/kg of vitamin B12 was administered intraperitoneally before surgical intervention. Histomorphometric and electron microscopic analyses revealed that vitamin B12 did not prevent structural changes, abnormal myelination and g-ratio deviations regarding the functional aspects of the sciatic nerve. Alpha lipoic acid was more effective in restructuring the histomorphometric and structural aspects of the nerve with more myelinated fibers with optimal values (0.55-0.68) than vitamin B12 groups, in which the number of myelinated nerve fibers significantly decreased at optimal intervals (0.55-0.68). A-LA administration following peripheral nerve transection injury is more effective in promoting nerve healing regarding the structural aspects of the sciatic nerve compared to vitamin B12 and also myelination of nerve fibers by increasing g-values.

Nerve ultrasound shows subclinical peripheral nerve involvement in neurofibromatosis type 2.

PubMed

Telleman, Johan A; Stellingwerff, Menno D; Brekelmans, Geert J; Visser, Leo H

2018-02-01

Neurofibromatosis type 2 (NF2) is mainly associated with central nervous system (CNS) tumors. Peripheral nerve involvement is described in symptomatic patients, but evidence of subclinical peripheral nerve involvement is scarce. We conducted a cross-sectional pilot study in 2 asymptomatic and 3 minimally symptomatic patients with NF2 to detect subclinical peripheral nerve involvement. Patients underwent clinical examination, nerve conduction studies (NCS), and high-resolution ultrasonography (HRUS). A total of 30 schwannomas were found, divided over 20 nerve segments (33.9% of all investigated nerve segments). All patients had at least 1 schwannoma. Schwannomas were identified with HRUS in 37% of clinically unaffected nerve segments and 50% of nerve segments with normal NCS findings. HRUS shows frequent subclinical peripheral nerve involvement in NF2. Clinicians should consider peripheral nerve involvement as a cause of weakness and sensory loss in the extremities in patients with this disease. Muscle Nerve 57: 312-316, 2018. © 2017 Wiley Periodicals, Inc.

Recovery from distal ulnar motor conduction block injury: serial EMG studies.

PubMed

Montoya, Liliana; Felice, Kevin J

2002-07-01

Acute conduction block injuries often result from nerve compression or trauma. The temporal pattern of clinical, electrophysiologic, and histopathologic changes following these injuries has been extensively studied in experimental animal models but not in humans. Our recent evaluation of a young man with an injury to the deep motor branch of the ulnar nerve following nerve compression from weightlifting exercises provided the opportunity to follow the course and recovery of a severe conduction block injury with sequential nerve conduction studies. The conduction block slowly and completely resolved, as did the clinical deficit, over a 14-week period. The reduction in conduction block occurred at a linear rate of -6.1% per week. Copyright 2002 Wiley Periodicals, Inc.

Congenital anomalies of the optic nerve

PubMed Central

Amador-Patarroyo, Manuel J.; Pérez-Rueda, Mario A.; Tellez, Carlos H.

2014-01-01

Congenital optic nerve head anomalies are a group of structural malformations of the optic nerve head and surrounding tissues, which may cause congenital visual impairment and blindness. Each entity in this group of optic nerve anomalies has individually become more prevalent as our ability to differentiate between them has improved due to better characterization of cases. Access to better medical technology (e.g., neuroimaging and genetic analysis advances in recent years) has helped to expand our knowledge of these abnormalities. However, visual impairment may not be the only problem in these patients, some of these entities will be related to ophthalmologic, neurologic and systemic features that will help the physician to identify and predict possible outcomes in these patients, which sometimes may be life-threatening. Herein we present helpful hints, associations and management (when plausible) for them. PMID:25859137

Excitability properties of motor axons in adults with cerebral palsy

PubMed Central

Klein, Cliff S.; Zhou, Ping; Marciniak, Christina

2015-01-01

Cerebral palsy (CP) is a permanent disorder caused by a lesion to the developing brain that significantly impairs motor function. The neurophysiological mechanisms underlying motor impairment are not well understood. Specifically, few have addressed whether motoneuron or peripheral axon properties are altered in CP, even though disruption of descending inputs to the spinal cord may cause them to change. In the present study, we have compared nerve excitability properties in seven adults with CP and fourteen healthy controls using threshold tracking techniques by stimulating the median nerve at the wrist and recording the compound muscle action potential over the abductor pollicis brevis. The excitability properties in the CP subjects were found to be abnormal. Early and late depolarizing and hyperpolarizing threshold electrotonus was significantly larger (i.e., fanning out), and resting current–threshold (I/V) slope was smaller, in CP compared to control. In addition resting threshold and rheobase tended to be larger in CP. According to a modeling analysis of the data, an increase in leakage current under or through the myelin sheath, i.e., the Barrett–Barrett conductance, combined with a slight hyperpolarization of the resting membrane potential, best explained the group differences in excitability properties. There was a trend for those with greater impairment in gross motor function to have more abnormal axon properties. The findings indicate plasticity of motor axon properties far removed from the site of the lesion. We suspect that this plasticity is caused by disruption of descending inputs to the motoneurons at an early age around the time of their injury. PMID:26089791

Abnormalities of gaze in cerebrovascular disease.

PubMed

Pedersen, R A; Troost, B T

1981-01-01

Disorders of ocular motility may occur after injury at several levels of the neuraxis. Unilateral supranuclear disorders of gaze tend to be transient; bilateral disorders more enduring. Nuclear disorders of gaze also tend to be enduring and are frequently present in association with long tract signs and cranial nerve palsies on opposite sides of the body. Nystagmus is a reliable sign of posterior fossa or peripheral eight nerve pathology. Familiarity with these concepts may help the clinician answer questions regarding localization and prognosis.

Sonography-guided recording for superficial peroneal sensory nerve conduction study.

PubMed

Kim, Ki Hoon; Park, Byung Kyu; Kim, Dong Hwee; Kim, Yuntae

2018-04-01

We sought to establish the optimal recording position for antidromic conduction of the superficial peroneal nerve (SPN) by using ultrasonography (USG). The sensory nerve action potentials (SNAPs) of the intermediate dorsal cutaneous nerve (IDCN) and medial dorsal cutaneous nerve (MDCN) in 64 limbs of 32 healthy participants were recorded (nerve conduction study [NCS]-1). Both nerves were identified by using USG, and the SNAPs were obtained from the USG-guided repositioned electrodes (NCS-2). The IDCN and MDCN were located at 29.3% ± 5.1% and 43.9% ± 4.9% of the intermalleolar distance from the lateral malleolus, respectively. Significantly greater amplitude was shown for SNAPs of both nerves in NCS-2 versus NCS-1. The optimal recording position is likely to be lateral, one-third from the lateral malleolus for the IDCN, and just lateral to the midpoint of the intermalleolar line for the MDCN. When the SPN response is unexpectedly attenuated, USG-guided repositioning of the electrodes should be considered. Muscle Nerve 57: 628-633, 2018. © 2017 Wiley Periodicals, Inc.

Classification and Current Management of Inner Ear Malformations.

PubMed

Sennaroğlu, Levent; Bajin, Münir Demir

2017-09-29

Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous malformations. Here, the pathology involves inner ear hair cells. There is no gross bony abnormality and, therefore, in these cases high-resolution computerized tomography and magnetic resonance imaging of the temporal bone reveal normal findings. The remaining 20% have various malformations involving the bony labyrinth and, therefore, can be radiologically demonstrated by computerized tomography and magnetic resonance imaging. The latter group involves surgical challenges as well as problems in decision-making. Some cases may be managed by a hearing aid, others need cochlear implantation, and some cases are candidates for an auditory brainstem implantation (ABI). During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During surgery for inner ear malformations, the surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article, inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcomes after various implantation methods are closely related to the status of the cochlear nerve, and a practical classification of the cochlear nerve deficiency is also provided.

[Myelinated nerve fibers coexisted with epiretinal membrane in macula--case report].

PubMed

Swiech-Zubilewicz, Anna; Bieliński, Paweł; Dolar-Szczasny, Joanna; Zarnowski, Tomasz

2012-01-01

We describe a case of peripapillary myelinated retinal nerve fibers complicated by epiretinal membrane in region of macula. 72 years old man was refered to our Clinic with suspicion of retinal detachment of right eye. Visual acuity of right eye was based to 0.05, in left eye was 0.5. IOP was normal in both eyes. In biomcroscopic evaluation the slight cortical cataract was observed in both eyes. Stereoscopic evaluation of right eye revealed the presence of massive peripapillary myelinated retinal nerve fibers and epiretinal membrane in the macula. In the left eye the less intense peripapillary myelinated retinal nerve fibers were noticed as well and the macular region was unchanged. With the use of OCT examination of the retina the presence of epiretinal membrane in the right eye was confirmed. OCT in the fellow eye presented an undisturbed foveal profile without any epiretinal abnormalities. Myelinated retinal nerve fibers can be complicated by epiretinal membrane. Probably the presence of macular pathologies depends on the extensions of nerve fibers. OCT examination is very helpful to give a proper diagnosis.

Bedside Optic Nerve Sheath Diameter Assessment in the Identification of Increased Intracranial Pressure in Suspected Idiopathic Intracranial Hypertension.

PubMed

Irazuzta, Jose E; Brown, Martha E; Akhtar, Javed

2016-01-01

We determined whether the bedside assessment of the optic nerve sheath diameter could identify elevated intracranial pressure in individuals with suspected idiopathic intracranial hypertension. This was a single-center, prospective, rater-blinded study performed in a freestanding pediatric teaching hospital. Patients aged 12 to 18 years scheduled for an elective lumbar puncture with the suspicion of idiopathic intracranial hypertension were eligible to participate. Optic nerve sheath diameter was measured via ultrasonography before performing a sedated lumbar puncture for measuring cerebrospinal fluid opening pressure. Abnormal measurements were predefined as optic nerve sheath diameter ≥4.5 mm and a cerebrospinal fluid opening pressure greater than 20 cmH2O. Thirteen patients participated in the study, 10 of whom had elevated intracranial pressure. Optic nerve sheath diameter was able to predict or rule out elevated intracranial pressure in all patients. Noninvasive assessment of the optic nerve sheath diameter could help to identify patients with elevated intracranial pressure when idiopathic intracranial hypertension is suspected. Copyright © 2016 Elsevier Inc. All rights reserved.

Occipital Neuralgia Diagnosis and Treatment: The Role of Ultrasound.

PubMed

Narouze, Samer

2016-04-01

Occipital neuralgia is a form of neuropathic type of pain in the distribution of the greater, lesser, or third occipital nerves. Patients with intractable occipital neuralgia do not respond well to conservative treatment modalities. This group of patients represents a significant therapeutic challenge and may require interventional or invasive therapeutic approaches. Occipital neuralgia frequently occurs as a result of nerve entrapment or irritation by a tight muscle or vascular structure, or nerve trauma during whiplash injury. Although the entrapment theory is most commonly accepted, it lacks strong clinical evidence to support it. Accordingly, the available interventional approaches have been targeting the accessible part of the occipital nerve rather than the entrapped part. Bedside sonography is an excellent imaging modality for soft tissue structures. Ultrasound not only allows distinguishing normal from abnormal entrapped occipital nerves, it can identify the level and the cause of entrapment as well. Ultrasound guidance allows precise occipital nerve blocks and interventions at the level of the "specific" entrapment location rather than into the site of "presumed" entrapment. © 2016 American Headache Society.

Shah-Waardenburg syndrome and PCWH associated with SOX10 mutations: a case report and review of the literature.

PubMed

Verheij, Johanna B G M; Sival, Deborah A; van der Hoeven, Johannes H; Vos, Yvonne J; Meiners, Linda C; Brouwer, Oebele F; van Essen, Anthonie J

2006-01-01

Shah-Waardenburg syndrome is a rare congenital disorder with variable clinical expression, characterised by aganglionosis of the rectosigmoïd (Hirschsprung disease), and abnormal melanocyte migration, resulting in pigmentary abnormalities and sensorineural deafness (Waardenburg syndrome). Mutations in the EDN, EDNRB and SOX10 genes can be found in patients with this syndrome. SOX10 mutations are specifically associated with a more severe phenotype called PCWH: peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and Hirschsprung disease. Neuronal expression of SOX10 occurs in neural crest cells during early embryonic development and in glial cells of the peripheral and central nervous systems during late embryonic development and in adults. We present a 4-year-old girl with the PCWH phenotype associated with a de novo nonsense mutation (S384X) in SOX10. Main clinical features were mental retardation, peripheral neuropathy, deafness, Hirschsprung disease, distal arthrogryposis, white hairlock, and growth retardation. She presented with hypotonia, developmental delay, reduced peripheral nerve conduction velocities, and radiologically assessed central hypomyelination. Subsequently, the formation of abnormal myelin within the central and peripheral nervous system was functionally and radiologically assessed. Children presenting with features of Waardenburg syndrome and neurological dysfunction should be tested for mutations in the SOX10 gene to enable diagnosis and counselling.

Combined KHFAC+DC nerve block without onset or reduced nerve conductivity after block

PubMed Central

Franke, Manfred; Vrabec, Tina; Wainright, Jesse; Bhadra, Niloy; Bhadra, Narendra; Kilgore, Kevin

2017-01-01

Background Kilohertz Frequency Alternating Current waveforms (KHFAC) have been shown to provide peripheral nerve conductivity block in many acute and chronic animal models. KHFAC nerve block could be used to address multiple disorders caused by neural over-activity, including blocking pain and spasticity. However, one drawback of KHFAC block is a transient activation of nerve fibers during the initiation of the nerve block, called the onset response. The objective of this study is to evaluate the feasibility of using charge balanced direct current (CBDC) waveforms to temporarily block motor nerve conductivity distally to the KHFAC electrodes to mitigate the block onset-response. Methods A total of eight animals were used in this study. A set of four animals were used to assess feasibility and reproducibility of a combined KHFAC+CBDC block. A following randomized study, conducted on a second set of four animals, compared the onset response resulting from KHFAC alone and combined KHFAC+CBDC waveforms. To quantify the onset, peak forces and the force-time integral were measured during KHFAC block initiation. Nerve conductivity was monitored throughout the study by comparing muscle twitch forces evoked by supra-maximal stimulation proximal and distal to the block electrodes. Each animal of the randomized study received at least 300 seconds (range: 318 to 1563s) of cumulative DC to investigate the impact of combined KHFAC+CBDC on nerve viability. Results The peak onset force was reduced significantly from 20.73 N (range: 18.6–26.5 N) with KHFAC alone to 0.45 N (range: 0.2–0.7 N) with the combined CBDC and KHFAC block waveform (p<0.001). The area under the force curve was reduced from 6.8 Ns (range: 3.5–21.9 Ns) to 0.54 Ns (range: 0.18–0.86Ns) (p<0.01). No change in nerve conductivity was observed after application of the combined KHFAC+CBDC block relative to KHFAC waveforms. Conclusion The distal application of CBDC can significantly reduce or even completely prevent the KHFAC onset response without a change in nerve conductivity. PMID:25115572

Combined KHFAC + DC nerve block without onset or reduced nerve conductivity after block

NASA Astrophysics Data System (ADS)

Franke, Manfred; Vrabec, Tina; Wainright, Jesse; Bhadra, Niloy; Bhadra, Narendra; Kilgore, Kevin

2014-10-01

Objective. Kilohertz frequency alternating current (KHFAC) waveforms have been shown to provide peripheral nerve conductivity block in many acute and chronic animal models. KHFAC nerve block could be used to address multiple disorders caused by neural over-activity, including blocking pain and spasticity. However, one drawback of KHFAC block is a transient activation of nerve fibers during the initiation of the nerve block, called the onset response. The objective of this study is to evaluate the feasibility of using charge balanced direct current (CBDC) waveforms to temporarily block motor nerve conductivity distally to the KHFAC electrodes to mitigate the block onset-response. Approach. A total of eight animals were used in this study. A set of four animals were used to assess feasibility and reproducibility of a combined KHFAC + CBDC block. A following randomized study, conducted on a second set of four animals, compared the onset response resulting from KHFAC alone and combined KHFAC + CBDC waveforms. To quantify the onset, peak forces and the force-time integral were measured during KHFAC block initiation. Nerve conductivity was monitored throughout the study by comparing muscle twitch forces evoked by supra-maximal stimulation proximal and distal to the block electrodes. Each animal of the randomized study received at least 300 s (range: 318-1563 s) of cumulative dc to investigate the impact of combined KHFAC + CBDC on nerve viability. Main results. The peak onset force was reduced significantly from 20.73 N (range: 18.6-26.5 N) with KHFAC alone to 0.45 N (range: 0.2-0.7 N) with the combined CBDC and KHFAC block waveform (p < 0.001). The area under the force curve was reduced from 6.8 Ns (range: 3.5-21.9 Ns) to 0.54 Ns (range: 0.18-0.86 Ns) (p < 0.01). No change in nerve conductivity was observed after application of the combined KHFAC + CBDC block relative to KHFAC waveforms. Significance. The distal application of CBDC can significantly reduce or even completely prevent the KHFAC onset response without a change in nerve conductivity.

Exploring vocal recovery after cranial nerve injury in Bengalese finches.

PubMed

Urbano, Catherine M; Peterson, Jennifer R; Cooper, Brenton G

2013-02-08

Songbirds and humans use auditory feedback to acquire and maintain their vocalizations. The Bengalese finch (Lonchura striata domestica) is a songbird species that rapidly modifies its vocal output to adhere to an internal song memory. In this species, the left side of the bipartite vocal organ is specialized for producing louder, higher frequencies (≥2.2kHz) and denervation of the left vocal muscles eliminates these notes. Thus, the return of higher frequency notes after cranial nerve injury can be used as a measure of vocal recovery. Either the left or right side of the syrinx was denervated by resection of the tracheosyringeal portion of the hypoglossal nerve. Histologic analyses of syringeal muscle tissue showed significant muscle atrophy in the denervated side. After left nerve resection, songs were mainly composed of lower frequency syllables, but three out of five birds recovered higher frequency syllables. Right nerve resection minimally affected phonology, but it did change song syntax; syllable sequence became abnormally stereotyped after right nerve resection. Therefore, damage to the neuromuscular control of sound production resulted in reduced motor variability, and Bengalese finches are a potential model for functional vocal recovery following cranial nerve injury. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Congenital oculomotor nerve synkinesis associated with fetal retinoid syndrome.

PubMed

Morrison, David G; Elsas, Frederick J; Descartes, Maria

2005-04-01

Isotretinoin (RA), used for the treatment of cystic acne, is a powerful teratogen, causing craniofacial dysmorphisms and neural tube defects. We present two patients with RA embryopathy and oculomotor nerve synkinesis. Retrospective review of patient records. Two patients presented with third nerve synkinesis and fetal RA exposure. Both had marked elevation of the upper eyelids on adduction such that the lid fissures alternately opened and closed on gaze from side to side. Both patients showed typical dysmorphisms of RA embryopathy. The first patient had complete agenesis of the cerebellar vermix and died at 2 years. The second patient had restricted extraocular muscles in one eye and was exotropic and hypotropic. Both patients demonstrated simultaneous innervation of the medial rectus and levator palpebrae muscles causing coincident lid elevation in adduction. This evidence of oculomotor nerve synkinesis is consistent with animal studies showing abnormalities in the formation of cranial nerve ganglia following fetal RA exposure. RA is a powerful teratogen. These patients provide additional clinical evidence of its influence on neural migration during early development.

Motor Neuron Diseases

MedlinePlus

... and can detect abnormalities in sensory nerves. Laboratory tests of blood, urine, or other substances can rule out muscle diseases and other disorders that may have symptoms similar to those of MND. For example, analysis of the fluid that surrounds the brain ...

Genetics Home Reference: hereditary sensory neuropathy type IA

MedlinePlus

... by nerve abnormalities in the legs and feet (peripheral neuropathy). Many people with this condition experience prickling or ... Research Network: Inherited Neuropathies Consortium The Foundation for Peripheral Neuropathy: Symptoms General Information from MedlinePlus (5 links) Diagnostic ...

Rapidly Progressive Quadriplegia and Encephalopathy.

PubMed

Wynn, DonRaphael; McCorquodale, Donald; Peters, Angela; Juster-Switlyk, Kelsey; Smith, Gordon; Ansari, Safdar

2016-11-01

A woman aged 77 years was transferred to our neurocritical care unit for evaluation and treatment of rapidly progressive motor weakness and encephalopathy. Examination revealed an ability to follow simple commands only and abnormal movements, including myoclonus, tongue and orofacial dyskinesias, and opsoclonus. Imaging study findings were initially unremarkable, but when repeated, they demonstrated enhancement of the cauda equina nerve roots, trigeminal nerve, and pachymeninges. Cerebrospinal fluid examination revealed mildly elevated white blood cell count and protein levels. Serial electrodiagnostic testing demonstrated a rapidly progressive diffuse sensory motor axonopathy, and electroencephalogram findings progressed from generalized slowing to bilateral periodic lateralized epileptiform discharges. Critical details of her recent history prompted a diagnostic biopsy. Over time, the patient became completely unresponsive with no further abnormal movements and ultimately died. The differential diagnosis, pathological findings, and diagnosis are discussed with a brief review of a well-known yet rare diagnosis.

Long term ocular and neurological involvement in severe congenital toxoplasmosis.

PubMed

Meenken, C; Assies, J; van Nieuwenhuizen, O; Holwerda-van der Maat, W G; van Schooneveld, M J; Delleman, W J; Kinds, G; Rothova, A

1995-06-01

This study was set up to determine the long term ocular and systemic sequelae in patients with severe congenital toxoplasmosis. Cross sectional and retrospective study of 17 patients with severe congenital toxoplasmosis. In addition to chorioretinitis (100%), the most common abnormal ocular features were optic nerve atrophy (83%), visual acuity of less than 0.1 (85%), strabismus, and microphthalmos. In 50% of cases we observed iridic abnormalities and about 40% developed a cataract. Overt endocrinological disease, diagnosed in five of 15 patients, included panhypopituitarism (n = 2), gonadal failure with dwarfism (n = 1), precocious puberty with dwarfism and thyroid deficiency (n = 1), and diabetes mellitus and thyroid deficiency (n = 1). The observed endocrinological involvement was associated in all cases with obstructive hydrocephalus with a dilated third ventricle and optic nerve atrophy. The recognition of long term ocular, neurological, and endocrinological sequelae of congenital toxoplasmosis is important for medical management of these severely handicapped patients.

Temporary persistence of conduction block after prolonged kilohertz frequency alternating current on rat sciatic nerve

NASA Astrophysics Data System (ADS)

Bhadra, Narendra; Foldes, Emily; Vrabec, Tina; Kilgore, Kevin; Bhadra, Niloy

2018-02-01

Objective. Application of kilohertz frequency alternating current (KHFAC) waveforms can result in nerve conduction block that is induced in less than a second. Conduction recovers within seconds when KHFAC is applied for about 5-10 min. This study investigated the effect of repeated and prolonged application of KHFAC on rat sciatic nerve with bipolar platinum electrodes. Approach. Varying durations of KHFAC at signal amplitudes for conduction block with intervals of no stimulus were studied. Nerve conduction was monitored by recording peak Gastrocnemius muscle force utilizing stimulation electrodes proximal (PS) and distal (DS) to a blocking electrode. The PS signal traveled through the block zone on the nerve, while the DS went directly to the motor end-plate junction. The PS/DS force ratio provided a measure of conduction patency of the nerve in the block zone. Main results. Conduction recovery times were found to be significantly affected by the cumulative duration of KHFAC application. Peak stimulated muscle force returned to pre-block levels immediately after cessation of KHFAC delivery when it was applied for less than about 15 min. They fell significantly but recovered to near pre-block levels for cumulative stimulus of 50  ±  20 min, for the tested On/Off times and frequencies. Conduction recovered in two phases, an initial fast one (60-80% recovery), followed by a slower phase. No permanent conduction block was seen at the end of the observation period during any experiment. Significance. This carry-over block effect may be exploited to provide continuous conduction block in peripheral nerves without continuous application of KHFAC.

Abnormal afferent nerve endings in the soft palatal mucosa of sleep apnoics and habitual snorers.

PubMed

Friberg, D; Gazelius, B; Hökfelt, T; Nordlander, B

1997-07-23

Habitual snoring precedes obstructive sleep apnea (OSA), but the pathophysiological mechanisms behind progression are still unclear. The patency of upper airways depends on a reflexogen mechanism reacting on negative intrapharyngeal pressure at inspiration, probably mediated by mucosal receptors, i.e., via afferent nerve endings. Such nerves contain a specific nerve protein, protein-gene product 9.5 (PGP 9.5) and in some cases substance P (SP) and calcitonin gene-related (CGRP). Biopsies of the soft palatial mucosa were obtained from non-smoking men ten OSA patients, 11 habitual snorers and 11 non-snoring controls. The specimens were immunohistochemically analyzed for PGP 9.5, SP and CGRP. As compared to controls, an increased number of PGP-, SP- and CGRP-immunoreactive nerves were demonstrated in the mucosa in 9/10 OSA patients and 4/11 snorers, in addition to varicose nerve endings in the papillae and epithelium. Using double staining methodology, it could be shown that SP- and CGRP-like immunoreactivities (LIs) often coexisted in these fibres, as did CGRP- and PGP 9.5-LIs. The increased density in sensory nerve terminals are interpreted to indicate an afferent nerve lesion. Our results support the hypothesis of a progressive neurogenic lesion as a contributory factor to the collapse of upper airways during sleep in OSA patients.

Health effects among refrigeration repair workers exposed to fluorocarbons.

PubMed Central

Campbell, D D; Lockey, J E; Petajan, J; Gunter, B J; Rom, W N

1986-01-01

Refrigeration repair workers may be intermittently exposed to fluorocarbons and their thermal decomposition products. A case of peripheral neuropathy (distal axonopathy) in a commercial refrigeration repairman prompted an epidemiological investigation of the health of refrigeration repair workers. No additional cases of peripheral neuropathy were identified among the 27 refrigeration repair workers studied. A reference group of 14 non-refrigeration repair workers was also studied. No differences were noted between groups for the ulnar (motor and sensory), median (motor and sensory), peroneal, sural, or tibial nerve conduction velocities. Refrigeration repair workers reported palpitations and lightheadedness significantly more often than workers in the reference group. No clinical neurological or electroneurophysiological abnormalities were detected in eight refrigeration repair workers followed up for three years during continuous employment. PMID:3004555

The influence of unilateral saccular impairment on functional balance performance and self-report dizziness.

PubMed

McCaslin, Devin L; Jacobson, Gary P; Grantham, Sarah L; Piker, Erin G; Verghese, Susha

2011-09-01

Postural stability in humans is largely maintained by vestibular, visual, and somatosensory inputs to the central nervous system. Recent clinical advances in the assessment of otolith function (e.g., cervical and ocular vestibular evoked myogenic potentials [cVEMPs and oVEMPs], subjective visual vertical [SVV] during eccentric rotation) have enabled investigators to identify patients with unilateral otolith impairments. This research has suggested that patients with unilateral otolith impairments perform worse than normal healthy controls on measures of postural stability. It is not yet known if patients with unilateral impairments of the saccule and/or inferior vestibular nerve (i.e., unilaterally abnormal cVEMP) perform differently on measures of postural stability than patients with unilateral impairments of the horizontal SCC (semicircular canal) and/or superior vestibular nerve (i.e., unilateral caloric weakness). Further, it is not known what relationship exists, if any, between otolith system impairment and self-report dizziness handicap. The purpose of this investigation was to determine the extent to which saccular impairments (defined by a unilaterally absent cVEMP) and impairments of the horizontal semicircular canal (as measured by the results of caloric testing) affect vestibulospinal function as measured through the Sensory Organization Test (SOT) of the computerized dynamic posturography (CDP). A secondary objective of this investigation was to measure the effects, if any, that saccular impairment has on a modality-specific measure of health-related quality of life. A retrospective cohort study. Subjects were assigned to one of four groups based on results from balance function testing: Group 1 (abnormal cVEMP response only), Group 2 (abnormal caloric response only), Group 3 (abnormal cVEMP and abnormal caloric response), and Group 4 (normal control group). Subjects were 92 adult patients: 62 were seen for balance function testing due to complaints of dizziness, vertigo, or unsteadiness, and 30 served as controls. All subjects underwent videonystagmography or electronystagmography (VNG/ENG), vestibular evoked myogenic potentials (VEMPs), self-report measures of self-perceived dizziness disability/handicap (Dizziness Handicap Inventory), and tests of postural control (Neurocom Equitest). Subjects were categorized into one of four groups based on balance function test results. All variables were subjected to a multifactor analysis of variance (ANOVA). The Dizziness Handicap Inventory (DHI) total scores and equilibrium scores served as the dependent variables. Results showed that patients with abnormal unilateral saccular or inferior vestibular nerve function (i.e., abnormal cVEMP) demonstrated significantly impaired postural control when compared to normal participants. However, this group demonstrated significantly better postural stability when compared to the group with abnormal caloric responses alone and the group with abnormal caloric responses and abnormal cVEMP results. Patients with an abnormal cVEMP did not differ significantly on the DHI compared to the other two impaired groups. We interpret these findings as evidence that a significantly asymmetrical cVEMP in isolation negatively impacts performance on measures of postural control compared to normal subjects but not compared to patients with significant caloric weaknesses. However, patients with a unilaterally abnormal cVEMP do not differ from patients with significant caloric weaknesses in regard to self-perceived dizziness handicap. American Academy of Audiology.

Biomechanical and functional variation in rat sciatic nerve following cuff electrode implantation

PubMed Central

2014-01-01

Background Nerve cuff electrodes are commonly and successfully used for stimulating peripheral nerves. On the other hand, they occasionally induce functional and morphological changes following chronic implantation, for reasons not always clear. We hypothesize that restriction of nerve mobility due to cuff implantation may alter nerve conduction. Methods We quantified acute changes in nerve-muscle electrophysiology, using electromyography, and nerve kinematics in anesthetized Sprague Dawley rat sciatic nerves during controlled hindlimb joint movement. We compared electrophysiological and biomechanical response in uncuffed nerves and those secured within a cuff electrode using analysis of variance (ANOVA) and regression analysis. Results Tethering resulting from cuff implantation resulted in altered nerve strain and a complex biomechanical environment during joint movement. Coincident with biomechanical changes, electromyography revealed significantly increased variability in the response of conduction latency and amplitude in cuffed, but not free, nerves following joint movement. Conclusion Our findings emphasize the importance of the mechanical interface between peripheral nerves and their devices on neurophysiological performance. This work has implications for nerve device design, implantation, and prediction of long-term efficacy. PMID:24758405

A PILOT STUDY OF DIAGNOSTIC NEUROMUSCULAR ULTRASOUND IN BELL'S PALSY

PubMed Central

TAWFIK, EMAN A.; WALKER, FRANCIS O.; CARTWRIGHT, MICHAEL S.

2015-01-01

Background and purpose Neuromuscular ultrasound of the cranial nerves is an emerging field which may help in the assessment of cranial neuropathies. The aim of this study was to evaluate the role of neuromuscular ultrasound in Bell's palsy. A second objective was to assess the possibility of any associated vagus nerve abnormality. Methods Twenty healthy controls and 12 Bell's palsy patients were recruited. The bilateral facial nerves, vagus nerves, and frontalis muscles were scanned using an 18 MHz linear array transducer. Facial nerve diameter, vagus nerve cross-sectional area, and frontalis thickness were measured. Results Mean facial nerve diameter was 0.8 ± 0.2 mm in controls and 1.1 ± 0.3 mm in patients group. The facial nerve diameter was significantly larger in patients than controls (p = 0.006, 95% CI for the difference between groups of 0.12-0.48), with a significant side-to-side difference in patients as well (p = 0.004, 95% CI for side-to-side difference of 0.08-0.52). ROC curve analysis of the absolute facial nerve diameter revealed a sensitivity of 75 % and a specificity of 70 %. No significant differences in vagus nerve cross-sectional area or frontalis thickness were detected between patients and controls. Conclusions Ultrasound can detect facial nerve enlargement in Bell's palsy and may have a role in assessment, or follow-up, of Bell's palsy and other facial nerve disorders. The low sensitivity of the current technique precludes its routine use for diagnosis, however, this study demonstrates its validity and potential for future research. PMID:26076910

Inherited focal, episodic neuropathies: hereditary neuropathy with liability to pressure palsies and hereditary neuralgic amyotrophy.

PubMed

Chance, Phillip F

2006-01-01

Hereditary neuropathy with liability to pressure palsies (HNPP; also called tomaculous neuropathy) is an autosomal-dominant disorder that produces a painless episodic, recurrent, focal demyelinating neuropathy. HNPP generally develops during adolescence, and may cause attacks of numbness, muscular weakness, and atrophy. Peroneal palsies, carpal tunnel syndrome, and other entrapment neuropathies may be frequent manifestations of HNPP. Motor and sensory nerve conduction velocities may be reduced in clinically affected patients, as well as in asymptomatic gene carriers. The histopathological changes observed in peripheral nerves of HNPP patients include segmental demyelination and tomaculous or "sausage-like" formations. Mild overlap of clinical features with Charcot-Marie-Tooth (CMT) disease type 1 (CMT1) may lead patients with HNPP to be misdiagnosed as having CMT1. HNPP and CMT1 are both demyelinating neuropathies, however, their clinical, pathological, and electrophysiological features are quite distinct. HNPP is most frequently associated with a 1.4-Mb pair deletion on chromosome 17p12. A duplication of the identical region leads to CMT1A. Both HNPP and CMT1A result from a dosage effect of the PMP22 gene, which is contained within the deleted/duplicated region. This is reflected in reduced mRNA and protein levels in sural nerve biopsy samples from HNPP patients. Treatment for HNPP consists of preventative and symptom-easing measures. Hereditary neuralgic amyotrophy (HNA; also called familial brachial plexus neuropathy) is an autosomal-dominant disorder causing episodes of paralysis and muscle weakness initiated by severe pain. Individuals with HNA may suffer repeated episodes of intense pain, paralysis, and sensory disturbances in an affected limb. The onset of HNA is at birth or later in childhood with prognosis for recovery usually favorable; however, persons with HNA may have permanent residual neurological dysfunction following attack(s). Episodes are often triggered by infections, immunizations, the puerperium, and stress. Electrophysiological studies show normal or mildly prolonged motor nerve conduction velocities distal to the affected brachial plexus. Pathological studies have found axonal degeneration in nerves examined distal to the plexus abnormality. In some HNA pedigrees there are characteristic facial features, including hypotelorism. The prognosis for recovery of normal function of affected limbs in HNA is good, although recurrent episodes may cause residual deficits. HNA is genetically linked to chromosome 17q25, where mutations in the septin-9 (SEPT9) gene have been found.

Spatial analysis improves the detection of early corneal nerve fiber loss in patients with recently diagnosed type 2 diabetes

PubMed Central

Winter, Karsten; Strom, Alexander; Zhivov, Andrey; Allgeier, Stephan; Papanas, Nikolaos; Ziegler, Iris; Brüggemann, Jutta; Ringel, Bernd; Peschel, Sabine; Köhler, Bernd; Stachs, Oliver; Guthoff, Rudolf F.; Roden, Michael

2017-01-01

Corneal confocal microscopy (CCM) has revealed reduced corneal nerve fiber (CNF) length and density (CNFL, CNFD) in patients with diabetes, but the spatial pattern of CNF loss has not been studied. We aimed to determine whether spatial analysis of the distribution of corneal nerve branching points (CNBPs) may contribute to improving the detection of early CNF loss. We hypothesized that early CNF decline follows a clustered rather than random distribution pattern of CNBPs. CCM, nerve conduction studies (NCS), and quantitative sensory testing (QST) were performed in a cross-sectional study including 86 patients recently diagnosed with type 2 diabetes and 47 control subjects. In addition to CNFL, CNFD, and branch density (CNBD), CNBPs were analyzed using spatial point pattern analysis (SPPA) including 10 indices and functional statistics. Compared to controls, patients with diabetes showed lower CNBP density and higher nearest neighbor distances, and all SPPA parameters indicated increased clustering of CNBPs (all P<0.05). SPPA parameters were abnormally increased >97.5th percentile of controls in up to 23.5% of patients. When combining an individual SPPA parameter with CNFL, ≥1 of 2 indices were >99th or <1st percentile of controls in 28.6% of patients compared to 2.1% of controls, while for the conventional CNFL/CNFD/CNBD combination the corresponding rates were 16.3% vs 2.1%. SPPA parameters correlated with CNFL and several NCS and QST indices in the controls (all P<0.001), whereas in patients with diabetes these correlations were markedly weaker or lost. In conclusion, SPPA reveals increased clustering of early CNF loss and substantially improves its detection when combined with a conventional CCM measure in patients with recently diagnosed type 2 diabetes. PMID:28296936

Deficiency of a membrane skeletal protein, 4.1G, results in myelin abnormalities in the peripheral nervous system.

PubMed

Saitoh, Yurika; Ohno, Nobuhiko; Yamauchi, Junji; Sakamoto, Takeharu; Terada, Nobuo

2017-12-01

We previously demonstrated that a membrane skeletal molecular complex, 4.1G-membrane palmitoylated protein 6 (MPP6)-cell adhesion molecule 4, is incorporated in Schwann cells in the peripheral nervous system (PNS). In this study, we evaluated motor activity and myelin ultrastructures in 4.1G-deficient (-/-) mice. When suspended by the tail, aged 4.1G -/- mice displayed spastic leg extension, especially after overwork. Motor-conduction velocity in 4.1G -/- mice was slower than that in wild-type mice. Using electron microscopy, 4.1G -/- mice exhibited myelin abnormalities: myelin was thicker in internodes, and attachment of myelin tips was distorted in some paranodes. In addition, we found a novel function of 4.1G for sorting a scaffold protein, Lin7, due to disappearance of the immunolocalization and reduction of the production of Lin7c and Lin7a in 4.1G -/- sciatic nerves, as well as the interaction of MPP6 and Lin7 with immunoprecipitation. Thus, we herein propose 4.1G functions as a signal for proper formation of myelin in PNS.

Normal-tension glaucoma (Low-tension glaucoma)

PubMed Central

Anderson, Douglas R

2011-01-01

Glaucoma is now considered an abnormal physiology in the optic nerve head that interacts with the level of intraocular pressure (IOP), with the degree and rate of damage depending on the IOP and presumably the degree of abnormal physiology. Diagnosis of normal-tension glaucoma (NTG), defined as glaucoma without a clearly abnormal IOP, depends on recognizing symptoms and signs associated with optic nerve vulnerability, in addition to absence of other explanations for disc abnormality and visual field loss. Among the findings are a halo or crescent of absence of retinal pigment epithelium around the disc, bilateral pre-chiasmal visual field defects, splinter hemorrhages at the disc margin, vascular dysregulation (low blood pressure, cold hands and feet, migraine headache with aura, and the like), or a family history of glaucoma. Possibly relevant, is a history of hemodynamic crisis, arterial obstructive disease, or sleep apnea. Neurological evaluation with imaging is needed only for atypical cases or ones that progress unexpectedly. Management follows the same principle of other chronic glaucomas, to lower the IOP by a substantial amount, enough to prevent disabling visual loss. However, many NTG cases are non-progressive. Therefore, it may often be wisein mild cases to determine whether the case is progressive and the rate of progression before deciding on how aggressivene to be with therapy. Efforts at neuroprotection and improvement in blood flow have not yet been shown effective. PMID:21150042

Time course and predictors of median nerve conduction after carpal tunnel release.

PubMed

Rotman, Mitchell B; Enkvetchakul, Bobby V; Megerian, J Thomas; Gozani, Shai N

2004-05-01

To identify predictors of outcome and of electrophysiologic recovery in patients with carpal tunnel syndrome (CTS) treated by endoscopic carpal tunnel release using a nerve conduction testing system (NC-Stat; NEUROMetrix, Inc, Waltham, MA). Validity of the automated nerve conduction testing system was shown by comparing presurgical distal motor latencies (DMLs) against a reference obtained by referral to an electromyography laboratory. The DML was evaluated in 48 patients with CTS. Measurements were obtained within 1 hour of surgery and at 2 weeks, 6 weeks, 3 months, and 6 months after carpal tunnel release. Presurgical and postsurgical DMLs were then compared and correlated with variables and possible predictors of outcome including age, body mass index, gender, and presurgical DMLs. The automated nerve conduction testing system DMLs matched those of reference electromyography/nerve conduction study values with high correlation. Sensitivity of the automated nerve conduction testing system when compared with a standardized CTS case definition was 89%, with a specificity of 95%. A significant correlation was found between the DML before release and the DML 1 hour after release. Moreover, maximal postsurgical DML improvement was highly dependent on the presurgical DML, with no improvement shown for the <4-ms group, mild improvement for the 4-to-6-ms group, and maximal improvement in the >6-ms group. Among the clinical variables of age, gender, and body mass index only age was mildly predictive of postrelease DML changes at 6 months. No other correlations between clinical variables and postsurgical DMLs were significant. In addition the predictive value of age was lost when combined with the presurgical DML in a multivariate analysis. Postsurgical changes in the median nerve DML were highly dependent on the prerelease latency. The sensitivity and specificity of a nerve conduction monitoring system in detecting and aiding in the diagnosis of CTS is useful in the long-term management of patients with CTS and can aid in determining the level of improvement in median nerve function after endoscopic carpal tunnel release.

Hepatocellular carcinoma metastasizing to the skull base involving multiple cranial nerves.

PubMed

Kim, Soo Ryang; Kanda, Fumio; Kobessho, Hiroshi; Sugimoto, Koji; Matsuoka, Toshiyuki; Kudo, Masatoshi; Hayashi, Yoshitake

2006-11-07

We describe a rare case of HCV-related recurrent multiple hepatocellular carcinoma (HCC) metastasizing to the skull base involving multiple cranial nerves in a 50-year-old woman. The patient presented with symptoms of ptosis, fixation of the right eyeball, and left abducens palsy, indicating disturbances of the right oculomotor and trochlear nerves and bilateral abducens nerves. Brain contrast-enhanced computed tomography (CT) revealed an ill-defined mass with abnormal enhancement around the sella turcica. Brain magnetic resonance imaging (MRI) disclosed that the mass involved the clivus, cavernous sinus, and petrous apex. On contrast-enhanced MRI with gadolinium-chelated contrast medium, the mass showed inhomogeneous intermediate enhancement. The diagnosis of metastatic HCC to the skull base was made on the basis of neurological findings and imaging studies including CT and MRI, without histological examinations. Further studies may provide insights into various methods for diagnosing HCC metastasizing to the craniospinal area.

Reliability of magnetic resonance imaging for the detection of hypopituitarism in children with optic nerve hypoplasia.

PubMed

Ramakrishnaiah, Raghu H; Shelton, Julie B; Glasier, Charles M; Phillips, Paul H

2014-01-01

It is essential to identify hypopituitarism in children with optic nerve hypoplasia (ONH) because they are at risk for developmental delay, seizures, or death. The purpose of this study is to determine the reliability of neurohypophyseal abnormalities on magnetic resonance imaging (MRI) for the detection of hypopituitarism in children with ONH. Cross-sectional study. One hundred one children with clinical ONH who underwent MRI of the brain and orbits and a detailed pediatric endocrinologic evaluation. Magnetic resonance imaging studies were performed on 1.5-Tesla scanners. The imaging protocol included sagittal T1-weighted images, axial fast fluid-attenuated inversion-recovery/T2-weighted images, and diffusion-weighted images of the brain. Orbital imaging included fat-saturated axial and coronal images and high-resolution axial T2-weighted images. The MRI studies were reviewed by 2 pediatric neuroradiologists for optic nerve hypoplasia, absent or ectopic posterior pituitary, absent pituitary infundibulum, absent septum pellucidum, migration anomalies, and hemispheric injury. Medical records were reviewed for clinical examination findings and endocrinologic status. All patients underwent a clinical evaluation by a pediatric endocrinologist and a standardized panel of serologic testing that included serum insulin-like growth factor-1, insulin-like growth factor binding protein-3, prolactin, cortisol, adrenocorticotropic hormone, thyroid-stimulating hormone, and free thyroxine levels. Radiologists were masked to patients' endocrinologic status and funduscopic findings. Sensitivity and specificity of MRI findings for the detection of hypopituitarism. Neurohypophyseal abnormalities, including absent pituitary infundibulum, ectopic posterior pituitary bright spot, and absent posterior pituitary bright spot, occurred in 33 children. Magnetic resonance imaging disclosed neurohypophyseal abnormalities in 27 of the 28 children with hypopituitarism (sensitivity, 96%). A normal neurohypophysis occurred in 67 of 73 children with normal endocrinologic function (specificity, 92%). Neurohypophyseal abnormalities on MRI are sensitive and specific indicators of hypopituitarism in children with ONH. Copyright © 2014 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

Meis2 is essential for cranial and cardiac neural crest development.

PubMed

Machon, Ondrej; Masek, Jan; Machonova, Olga; Krauss, Stefan; Kozmik, Zbynek

2015-11-06

TALE-class homeodomain transcription factors Meis and Pbx play important roles in formation of the embryonic brain, eye, heart, cartilage or hematopoiesis. Loss-of-function studies of Pbx1, 2 and 3 and Meis1 documented specific functions in embryogenesis, however, functional studies of Meis2 in mouse are still missing. We have generated a conditional allele of Meis2 in mice and shown that systemic inactivation of the Meis2 gene results in lethality by the embryonic day 14 that is accompanied with hemorrhaging. We show that neural crest cells express Meis2 and Meis2-defficient embryos display defects in tissues that are derived from the neural crest, such as an abnormal heart outflow tract with the persistent truncus arteriosus and abnormal cranial nerves. The importance of Meis2 for neural crest cells is further confirmed by means of conditional inactivation of Meis2 using crest-specific AP2α-IRES-Cre mouse. Conditional mutants display perturbed development of the craniofacial skeleton with severe anomalies in cranial bones and cartilages, heart and cranial nerve abnormalities. Meis2-null mice are embryonic lethal. Our results reveal a critical role of Meis2 during cranial and cardiac neural crest cells development in mouse.

Sensory and motor neuropathy in a Border Collie.

PubMed

Harkin, Kenneth R; Cash, Walter C; Shelton, G Diane

2005-10-15

A 5-month-old female Border Collie was evaluated because of progressive hind limb ataxia. The predominant clinical findings suggested a sensory neuropathy. Sensory nerve conduction velocity was absent in the tibial, common peroneal, and radial nerves and was decreased in the ulnar nerve; motor nerve conduction velocity was decreased in the tibial, common peroneal, and ulnar nerves. Histologic examination of nerve biopsy specimens revealed considerable nerve fiber depletion; some tissue sections had myelin ovoids, foamy macrophages, and axonal degeneration in remaining fibers. Marked depletion of most myelinated fibers within the peroneal nerve (a mixed sensory and motor nerve) supported the electrodiagnostic findings indicative of sensorimotor neuropathy. Progressive deterioration in motor function occurred over the following 19 months until the dog was euthanatized. A hereditary link was not established, but a littermate was similarly affected. The hereditary characteristic of this disease requires further investigation.

21 CFR 882.1550 - Nerve conduction velocity measurement device.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Nerve conduction velocity measurement device. 882.1550 Section 882.1550 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES NEUROLOGICAL DEVICES Neurological Diagnostic Devices § 882.1550 Nerve...

21 CFR 882.1550 - Nerve conduction velocity measurement device.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Nerve conduction velocity measurement device. 882.1550 Section 882.1550 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES NEUROLOGICAL DEVICES Neurological Diagnostic Devices § 882.1550 Nerve...

Effects of clinical infrared laser on superficial radial nerve conduction

DOE Office of Scientific and Technical Information (OSTI.GOV)

Greathouse, D.G.; Currier, D.P.; Gilmore, R.L.

The purposes of this study were to demonstrate the effects of infrared laser radiation on the sensory nerve conduction of a specified peripheral nerve in man and determine temperature changes in the tissue surrounding the treated nerve. Twenty healthy adults were divided into three groups: control (n = 5); experimental (n = 10), infrared laser radiation at 20 sec/cm2; and experimental (n = 5), infrared laser radiation treatment at 120 sec/cm2. Antidromic sensory nerve conduction studies were performed on the superficial radial nerve of each subject's right forearm. The infrared laser radiation was applied at a fixed intensity for fivemore » 1-cm2 segments. Latency, amplitude, and temperature measurements were recorded pretest; posttest; and posttest intervals of 1, 3, 5, 10, and 15 minutes. An analysis of variance with repeated measures was used to examine the data. No significant change was noted in the distal sensory latency or amplitude of the evoked sensory potential in either experimental or control groups as a result of the applications of the infrared laser radiation treatment. This study demonstrates that infrared laser used at clinically applied intensities does not alter conduction of sensory nerves nor does it elevate the subcutaneous temperature.« less

Comparison of electrophysiological findings in axonal and demyelinating Guillain-Barre syndrome

PubMed Central

Yadegari, Samira; Nafissi, Shahriar; Kazemi, Neda

2014-01-01

Background: Incidence and predominant subtype of Guillain-Barre syndrome (GBS) differs geographically. Electrophysiology has an important role in early diagnosis and prediction of prognosis. This study is conducted to determine the frequent subtype of GBS in a large group of patients in Iran and compare nerve conduction studies in axonal and demyelinating forms of GBS. Methods: We retrospectively evaluated the medical records and electrodiagnostic study (EDS) of 121 GBS patients who were managed in our hospital during 11 years. After regarding the exclusion criteria, patients classified as three groups: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). The most frequent subtype and then electrophysiological characteristic based on the time of EDS and their cerebrospinal fluid (CSF) profile were assessed. Results: Among 70 patients finally included in the study, 67% were men. About 63%, 23%, and 14% had AIDP, AMAN, and AMSAN, respectively. AIDP patients represented a wider range of ages compared with other groups. Higher levels of CSF protein, abnormal late responses and sural sparing were more frequent in AIDP subtype. Five AMSAN patients also revealed sural sparing. Conduction block (CB) was observed in one AMAN patient. Prolonged F-wave latency was observed only in AIDP cases. CB and inexcitable sensory nerves were more frequent after 2 weeks, but reduced F-wave persistency was more prominent in the early phase. Conclusion: AIDP was the most frequent subtype. Although the electrophysiology and CSF are important diagnostic tools, classification should not be made based on a distinct finding. PMID:25422732

Whole plantar nerve conduction study with disposable strip electrodes.

PubMed

Hemmi, Shoji; Kurokawa, Katsumi; Nagai, Taiji; Okamoto, Toshio; Murakami, Tatsufumi; Sunada, Yoshihide

2016-02-01

A new method to evaluate whole plantar nerve conduction with disposable strip electrodes (DSEs) is described. Whole plantar compound nerve action potentials (CNAPs) were recorded at the ankle. DSEs were attached to the sole for simultaneous stimulation of medial and lateral plantar nerves. We also conducted medial plantar nerve conduction studies using an established method and compared the findings. Whole plantar CNAPs were recorded bilaterally from 32 healthy volunteers. Mean baseline to peak amplitude for CNAPs was 26.9 ± 11.8 μV, and mean maximum conduction velocity was 65.8 ± 8.3 m/s. The mean amplitude of CNAPs obtained by our method was 58.2% higher than that of CNAPs obtained by the Saeed method (26.9 μV vs. 17.0 μV; P < 0.0001). The higher mean amplitude of whole plantar CNAPs obtained by our method suggests that it enables CNAPs to be obtained easily, even in elderly people. © 2015 Wiley Periodicals, Inc.

Phrenic nerve conduction studies: normative data and technical aspects.

PubMed

Maranhão, Analucia Abreu; Carvalho, Sonia Regina da Silva; Caetano, Marcelo Ribeiro; Alamy, Alexandre Hofke; Peixoto, Eduardo Mesquita; Filgueiras, Pedro Del Esporte Peçanha

2017-12-01

The aim of the present study was to define normative data of phrenic nerve conduction parameters of a healthy population. Phrenic nerve conduction studies were performed in 27 healthy volunteers. The normative limits for expiratory phrenic nerve compound muscle action potential were: amplitude (0.47 mv - 0.83 mv), latency (5.74 ms - 7.10 ms), area (6.20 ms/mv - 7.20 ms/mv) and duration (18.30 ms - 20.96 ms). Inspiratory normative limits were: amplitude (0.67 mv - 1.11 mv), latency (5.90 ms - 6.34 ms), area (5.62 ms/mv - 6.72 ms/mv) and duration (13.77 ms - 15.37 ms). The best point of phrenic nerve stimulus in the neck varies among individuals between the medial and lateral border of the clavicular head of the sternocleidomastoid muscle and stimulation of both sites, then choosing the best phrenic nerve response, seems to be the appropriate procedure.

Color blindness among multiple sclerosis patients in Isfahan.

PubMed

Shaygannejad, Vahid; Golabchi, Khodayar; Dehghani, Alireza; Ashtari, Fereshteh; Haghighi, Sepehr; Mirzendehdel, Mahsa; Ghasemi, Majid

2012-03-01

Multiple sclerosis (MS) is a disease of young and middle aged individuals with a demyelinative axonal damage nature in central nervous system that causes various signs and symptoms. As color vision needs normal function of optic nerve and macula, it is proposed that MS can alter it via influencing optic nerve. In this survey, we evaluated color vision abnormalities and its relationship with history of optic neuritis and abnormal visual evoked potentials (VEPs) among MS patients. The case group was included of clinically definitive MS patients and the same number of normal population was enrolled as the control group. Color vision of all the participants was evaluated by Ishihara test and then visual evoked potential (VEPs) and history of optic neuritis (ON) was assessed among them. Then, frequency of color blindness was compared between the case and the control group. Finally, color blinded patients were compared to those with the history of ON and abnormal VEPs. 63 MS patients and the same number of normal populations were enrolled in this study. 12 patients had color blindness based on the Ishihara test; only 3 of them were among the control group, which showed a significant different between the two groups (P = 0.013). There was a significant relationship between the color blindness and abnormal VEP (R = 0.53, P = 0.023) but not for the color blindness and ON (P = 0.67). This study demonstrates a significant correlation between color blindness and multiple sclerosis including ones with abnormal prolonged VEP latencies. Therefore, in individuals with acquired color vision impairment, an evaluation for potentially serious underlying diseases like MS is essential.

New Theoretical Model of Nerve Conduction in Unmyelinated Nerves

PubMed Central

Akaishi, Tetsuya

2017-01-01

Nerve conduction in unmyelinated fibers has long been described based on the equivalent circuit model and cable theory. However, without the change in ionic concentration gradient across the membrane, there would be no generation or propagation of the action potential. Based on this concept, we employ a new conductive model focusing on the distribution of voltage-gated sodium ion channels and Coulomb force between electrolytes. Based on this new model, the propagation of the nerve conduction was suggested to take place far before the generation of action potential at each channel. We theoretically showed that propagation of action potential, which is enabled by the increasing Coulomb force produced by inflowing sodium ions, from one sodium ion channel to the next sodium channel would be inversely proportionate to the density of sodium channels on the axon membrane. Because the longitudinal number of sodium ion channel would be proportionate to the square root of channel density, the conduction velocity of unmyelinated nerves is theoretically shown to be proportionate to the square root of channel density. Also, from a viewpoint of equilibrium state of channel importation and degeneration, channel density was suggested to be proportionate to axonal diameter. Based on these simple basis, conduction velocity in unmyelinated nerves was theoretically shown to be proportionate to the square root of axonal diameter. This new model would also enable us to acquire more accurate and understandable vision on the phenomena in unmyelinated nerves in addition to the conventional electric circuit model and cable theory. PMID:29081751

Ascending aortic aneurysm causing hoarse voice: a variant of Ortner's syndrome

PubMed Central

Eccles, Sinan Robert; Banks, John; Kumar, Pankaj

2012-01-01

A 68-year-old man with a persistent hoarse voice was found to have a left vocal cord paralysis. Clinical examination revealed signs consistent with aortic regurgitation. Subsequent investigation revealed an ascending aortic aneurysm. He underwent aortic root and ascending aorta replacement and his hoarseness improved. Ortner's syndrome refers to hoarseness due to recurrent laryngeal nerve palsy secondary to a cardiovascular abnormality. Recurrent laryngeal nerve palsy due to aneurysmal dilation of the ascending aorta is extremely rare, with aneurysms of the aortic arch being a more common cause. PMID:23060380

Diagnostic utility of F waves in clinically diagnosed patients of carpal tunnel syndrome.

PubMed

Joshi, Anand G; Gargate, Ashwini R

2013-01-01

Sensory nerve conduction velocity (SNCV) of median nerve measured across the carpal tunnel, difference between distal sensory latencies (DSLs) of median and ulnar nerves and difference between distal motor latencies (DMLs) of median and ulnar nerves are commonly used nerve conduction parameters for diagnosis of carpal tunnel syndrome (CTS). These are having high degree of sensitivity and specificity. Study of median nerve F-wave minimal latency (FWML) and difference between F-wave minimal latencies (FWMLs) of median and ulnar nerves have also been reported to be useful parameters for diagnosis of CTS. However, there is controversy regarding superiority of F-wave study for diagnosis of CTS. So the aim of present study was to compare sensitivity and specificity of median FWML and difference between FWMLs of median and ulnar nerves with that of above mentioned electrophysiological parameters and to find out which parameters are having more sensitivity and specificity, for early diagnosis of CTS. Median and ulnar nerves sensory and motor conduction, median and ulnar nerves F-wave studies were carried out bilaterally in 125 clinically diagnosed patients of carpal tunnel syndrome. These parameters were also studied in 45 age matched controls. Difference between DSLs of median and ulnar nerves, median SNCV and difference between DMLs of median and ulnar nerves were having highest sensitivity and specificity while median FWML and difference between FWMLs of median and ulnar nerves was having lowest sensitivity and specificity for diagnosis of CTS. So in conclusion F-wave study is not superior parameter for diagnosis of CTS.

Occupational neurology.

PubMed Central

Feldman, R. G.

1987-01-01

The nervous system is vulnerable to the effects of certain chemicals and physical conditions found in the work environment. The activities of an occupational neurologist focus on the evaluation of patients with neurological disorders caused by occupational or environmental conditions. When one is making a differential diagnosis in patients with neurological disorders, the possibility of toxic exposure or encounters with physical factors in the workplace must not be overlooked. Central to an accurate clinical diagnosis is the patient's history. A diagnosis of an occupational or environmental neurological problem requires a careful assessment of the clinical abnormalities and confirmation of these disabilities by objective tests such as nerve conduction velocity, evoked potentials, electroencephalogram, neuropsychological batteries, or nerve biopsy. On the basis of information about hazards in the workplace, safety standards and environmental and biological monitoring can be implemented in the workplace to reduce the risks of undue injury. Clinical manifestations of headache, memory disturbance, and peripheral neuropathy are commonly encountered presentations of the effects of occupational hazards. Physicians in everyday clinical practice must be aware of the signs and symptoms associated with exposure to possible neurotoxins and work methods. Occupational and environmental circumstances must be explored when evaluating patients with neurologic disorders. PMID:3577214

Successful Treatment with Microvascular Decompression Surgery of a Patient with Hemiparesis Caused by Vertebral Artery Compression of the Medulla Oblongata: Case Report and Review of the Literature.

PubMed

Ren, Jibin; Sun, Hongtao; Diao, Yunfeng; Niu, Xuegang; Wang, Hang; Wei, Zhengjun; Yuan, Fei

2017-12-01

There are few reports on hemiparesis caused by vascular medullary compression, which can occur because of dolichoectasia of the vertebrobasilar arterial system. In this article, we report a case of vertebral artery compression of the medulla oblongata in a 67-year-old woman. The patient was hypertensive, and she developed hemiparesis and intermittent spasms over 5 years. These spasms had worsened during the last year. Cranial nerve magnetic resonance imaging showed compression of the medulla oblongata by the left vertebral artery. A motor evoked potential (MEP) examination showed abnormal conduction of MEPs of bilateral toe abductors. The patient underwent microvascular decompression surgery under general anesthesia through a retrosigmoid keyhole approach. This operation led to relief of vascular compression and symptomatic improvement. Our case suggests that detailed history, imaging studies, and electrophysiologic studies help lead to a correct and early diagnosis of hemiparesis caused by vascular compression of the rostral ventrolateral medulla. Microvascular decompression surgery improves patient symptoms, and intraoperative electrophysiologic monitoring helps to avoid injury to important adjacent nerves. Copyright © 2017 Elsevier Inc. All rights reserved.

Brainstem encephalitis and acute polyneuropathy associated with hepatitis E infection.

PubMed

Salim, Omar Jabbar; Davidson, Amy; Li, Kathy; Leach, John Paul; Heath, Craig

2017-09-11

A 59-year-old man presented with feverish illness. His Glasgow Coma Scale was 15, had reduced visual acuity in the left eye with partial left ptosis and mild left hemiparesis with an extensor left plantar. Over 48 hours, he accrued multiple cranial nerves palsies and progressed to a flaccid paralysis necessitating admission to an intensive care unit.Cerebrospinal fluid (CSF) study showed 20 lymphocytes and raised protein. Viral and bacterial PCRs were negative. Samples for Lyme, blood-borne viruses, syphilis and autoantibodies were also negative. MRI brain showed T2 abnormalities within the brainstem. Nerve conduction studies revealed an acute motor and sensory axonal neuropathy pattern of Guillian Barre Syndrome (GBS). The patient was treated for both infective and inflammatory causes of brainstem encephalitis and GBS.Retrospective studies confirmed the presence of hepatitis E virus (HEV) RNA in CSF and serum studies showed positive HEV IgG and IgM prior to intravenous infusion. After 3 months of intensive rehabilitation, the patient was discharged home walking with a frame. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Median and ulnar neuropathies in university guitarists.

PubMed

Kennedy, Rachel H; Hutcherson, Kimberly J; Kain, Jennifer B; Phillips, Alicia L; Halle, John S; Greathouse, David G

2006-02-01

Descriptive study. To determine the presence of median and ulnar neuropathies in both upper extremities of university guitarists. Peripheral nerve entrapment syndromes of the upper extremities are well documented in musicians. Guitarists and plucked-string musicians are at risk for entrapment neuropathies in the upper extremities and are prone to mild neurologic deficits. Twenty-four volunteer male and female guitarists (age range, 18-26 years) were recruited from the Belmont University School of Music and the Vanderbilt University Blair School of Music. Individuals were excluded if they were pregnant or had a history of recent upper extremity or neck injury. Subjects completed a history form, were interviewed, and underwent a physical examination. Nerve conduction status of the median and ulnar nerves of both upper extremities was obtained by performing motor, sensory, and F-wave (central) nerve conduction studies. Descriptive statistics of the nerve conduction study variables were computed using Microsoft Excel. Six subjects had positive findings on provocative testing of the median and ulnar nerves. Otherwise, these guitarists had normal upper extremity neural and musculoskeletal function based on the history and physical examinations. When comparing the subjects' nerve conduction study values with a chart of normal nerve conduction studies values, 2 subjects had prolonged distal motor latencies (DMLs) of the left median nerve of 4.3 and 4.7 milliseconds (normal, < 4.2 milliseconds). Prolonged DMLs are compatible with median neuropathy at or distal to the wrist. Otherwise, all electrophysiological variables were within normal limits for motor, sensory, and F-wave (central) values. However, comparison studies of median and ulnar motor latencies in the same hand demonstrated prolonged differences of greater than 1.0 milliseconds that affected the median nerve in 2 additional subjects, and identified contralateral limb involvement in a subject with a prolonged distal latency. The other 20 subjects demonstrated normal comparison studies of the median and ulnar nerves in both upper extremities. In this descriptive study of a population of 24 university guitarists, 4 musicians (17%) were found to have electrophysiologic evidence of median neuropathy at or distal to the wrist or carpal tunnel syndrome. Ulnar nerve electrophysiological function was within normal limits for all subjects examined.

NON-INVASIVE EVALUATION OF NERVE CONDUCTION IN SMALL DIAMETER FIBERS IN THE RAT.

PubMed

Zotova, Elena G; Arezzo, Joseph C

2013-01-01

A novel non-invasive technique was applied to measure velocity within slow conducting axons in the distal extreme of the sciatic nerve (i.e., digital nerve) in a rat model. The technique is based on the extraction of rectified multiple unit activity (MUA) from in vivo whole nerve compound responses. This method reliably identifies compound action potentials in thinly myelinated fibers conducting at a range of 9-18 m/s (Aδ axons), as well as in a subgroup of unmylinated C fibers conducting at approximately 1-2 m/s. The sensitivity of the method to C-fiber conduction was confirmed by the progressive decrement of the responses in the 1-2 m/s range over a 20-day period following the topical application of capsaicin (ANOVA p <0.03). Increasing the frequency of applied repetitive stimulation over a range of 0.75 Hz to 6.0 Hz produced slowing of conduction and a significant decrease in the magnitude of the compound C-fiber response (ANOVA p <0.01). This technique offers a unique opportunity for the non-invasive, repeatable, and quantitative assessment of velocity in the subsets of Aδ and C fibers in parallel with evaluation of fast nerve conduction.

Phrenic nerve transfer to the musculocutaneous nerve for the repair of brachial plexus injury: electrophysiological characteristics

PubMed Central

Liu, Ying; Xu, Xun-cheng; Zou, Yi; Li, Su-rong; Zhang, Bin; Wang, Yue

2015-01-01

Phrenic nerve transfer is a major dynamic treatment used to repair brachial plexus root avulsion. We analyzed 72 relevant articles on phrenic nerve transfer to repair injured brachial plexus that were indexed by Science Citation Index. The keywords searched were brachial plexus injury, phrenic nerve, repair, surgery, protection, nerve transfer, and nerve graft. In addition, we performed neurophysiological analysis of the preoperative condition and prognosis of 10 patients undergoing ipsilateral phrenic nerve transfer to the musculocutaneous nerve in our hospital from 2008 to 201 3 and observed the electromyograms of the biceps brachii and motor conduction function of the musculocutaneous nerve. Clinically, approximately 28% of patients had brachial plexus injury combined with phrenic nerve injury, and injured phrenic nerve cannot be used as a nerve graft. After phrenic nerve transfer to the musculocutaneous nerve, the regenerated potentials first appeared at 3 months. Recovery of motor unit action potential occurred 6 months later and became more apparent at 12 months. The percent of patients recovering ‘excellent’ and ‘good’ muscle strength in the biceps brachii was 80% after 18 months. At 12 months after surgery, motor nerve conduction potential appeared in the musculocutaneous nerve in seven cases. These data suggest that preoperative evaluation of phrenic nerve function may help identify the most appropriate nerve graft in patients with an injured brachial plexus. The functional recovery of a transplanted nerve can be dynamically observed after the surgery. PMID:25883637

Phrenic nerve transfer to the musculocutaneous nerve for the repair of brachial plexus injury: electrophysiological characteristics.

PubMed

Liu, Ying; Xu, Xun-Cheng; Zou, Yi; Li, Su-Rong; Zhang, Bin; Wang, Yue

2015-02-01

Phrenic nerve transfer is a major dynamic treatment used to repair brachial plexus root avulsion. We analyzed 72 relevant articles on phrenic nerve transfer to repair injured brachial plexus that were indexed by Science Citation Index. The keywords searched were brachial plexus injury, phrenic nerve, repair, surgery, protection, nerve transfer, and nerve graft. In addition, we performed neurophysiological analysis of the preoperative condition and prognosis of 10 patients undergoing ipsilateral phrenic nerve transfer to the musculocutaneous nerve in our hospital from 2008 to 201 3 and observed the electromyograms of the biceps brachii and motor conduction function of the musculocutaneous nerve. Clinically, approximately 28% of patients had brachial plexus injury combined with phrenic nerve injury, and injured phrenic nerve cannot be used as a nerve graft. After phrenic nerve transfer to the musculocutaneous nerve, the regenerated potentials first appeared at 3 months. Recovery of motor unit action potential occurred 6 months later and became more apparent at 12 months. The percent of patients recovering 'excellent' and 'good' muscle strength in the biceps brachii was 80% after 18 months. At 12 months after surgery, motor nerve conduction potential appeared in the musculocutaneous nerve in seven cases. These data suggest that preoperative evaluation of phrenic nerve function may help identify the most appropriate nerve graft in patients with an injured brachial plexus. The functional recovery of a transplanted nerve can be dynamically observed after the surgery.

Efficacy and safety of nerve growth factor for the treatment of neurological diseases: a meta-analysis of 64 randomized controlled trials involving 6,297 patients

PubMed Central

Zhao, Meng; Li, Xiao-yan; Xu, Chun-ying; Zou, Li-ping

2015-01-01

OBJECTIVE: China is the only country where nerve growth factor is approved for large-scale use as a clinical medicine. More than 10 years ago, in 2003, nerve growth factor injection was listed as a national drug. The goal of this article is to evaluate comprehensively the efficacy and safety of nerve growth factor for the treatment of neurological diseases. DATA RETRIEVAL: A computer-based retrieval was performed from six databases, including the Cochrane Library, PubMed, EMBASE, Sino Med, CNKI, and the VIP database, searching from the clinical establishment of nerve growth factor for treatment until December 31, 2013. The key words for the searches were “nerve growth factor, randomized controlled trials” in Chinese and in English. DATA SELECTION: Inclusion criteria: any study published in English or Chinese referring to randomized controlled trials of nerve growth factor; patients with neurological diseases such as peripheral nerve injury, central nerve injury, cranial neuropathy, and nervous system infections; patients older than 7 years; similar research methods and outcomes assessing symptoms; and measurement of nerve conduction velocities. The meta-analysis was conducted using Review Manager 5.2.3 software. MAIN OUTCOME MEASURES: The total effective rate, the incidence of adverse effects, and the nerve conduction velocity were recorded for each study. RESULTS: Sixty-four studies involving 6,297 patients with neurological diseases were included. The total effective rate in the group treated with nerve growth factor was significantly higher than that in the control group (P < 0.0001, RR: 1.35, 95%CI: 1.30–1.40). The average nerve conduction velocity in the nerve growth factor group was significantly higher than that in the control group (P < 0.00001, MD: 4.59 m/s, 95%CI: 4.12–5.06). The incidence of pain or scleroma at the injection site in the nerve growth factor group was also higher than that in the control group (P < 0.00001, RR: 6.30, 95%CI: 3.53–11.27), but such adverse effects were mild. CONCLUSION: Nerve growth factor can significantly improve nerve function in patients with nervous system disease and is safe and effective. PMID:26109961

The phrenic nerve with accompanying vessels: a silent cause of cardiovascular border obliteration on chest radiography.

PubMed

Farhana, Shiri; Ashizawa, Kazuto; Hayashi, Hideyuki; Ogihara, Yukihiro; Aso, Nobuya; Hayashi, Kuniaki; Uetani, Masataka

2015-12-01

Our aim was to clarify the frequency of cardiovascular border obliteration on frontal chest radiography and to prove that the phrenic nerve with accompanying vessels can be considered as a cause of obliteration of cardiovascular border on an otherwise normal chest radiography. Two radiologists reviewed chest radiographs and computed tomography (CT) images of 100 individuals. CT confirmed the absence of intrapulmonary or extrapulmonary abnormalities in all of them. We examined the frequency of cardiovascular border obliteration on frontal chest radiography and summarized the causes of obliteration as pericardial fat pad, phrenic nerve, intrafissure fat, pulmonary vessels, and others, comparing them with CT in each case. Cardiovascular border was obliterated on frontal chest radiography in 46 cases on the right and in 61 on the left. The phrenic nerve with accompanying vessels was found to be a cause of obliteration in 34 of 46 cases (74%) on the right and 29 of 61 (48%) cases on the left. The phrenic nerve was the most frequent cause of cardiovascular border obliteration on both sides. The phrenic nerve with accompanying vessels, forming a prominent fold of parietal pleura, can be attributed as a cause of cardiovascular border obliteration on frontal chest radiography.

Identification of the effects of peripheral nerves injury on the muscle control - A review

NASA Astrophysics Data System (ADS)

Cabaj, Anna; Zmyslowski, Wojciech

2011-01-01

Impairment of motor function following peripheral nerve injury is a serious clinical problem. Generally nerve injury leads to erroneous control of muscle activity that results in gait and voluntary movement abnormalities followed by muscle atrophy. This article presents a review of studies on the effects of peripheral nerve injury on the motor system performed on animal models. We focused our attention on the results that are fundamental for better understanding of the degenerative and regenerative processes induced by nerve injury as well as of the mechanisms of structural changes in neuronal networks controlling movement. Quoted results are also important for clinical applications because they allow to develop new diagnostic and therapeutic techniques that can be used after nerve injury inducing motor deficits. However, till now no efficient therapy inducing satisfactory recovery was found. There is still a need to continue an advanced basic research directed to develop effective therapies. Thus the aim of this review is to compare the results of recent studies performed on various animal models in order to propose new methods for identification of mechanisms responsible for muscle deficits and propose targets for new pharmacological therapies.

Interplay between mast cells, enterochromaffin cells, and sensory signaling in the aging human bowel.

PubMed

Yu, Y; Daly, D M; Adam, I J; Kitsanta, P; Hill, C J; Wild, J; Shorthouse, A; Grundy, D; Jiang, W

2016-10-01

Advanced age is associated with a reduction in clinical visceral pain perception. However, the underlying mechanisms remain largely unknown. Previous studies have suggested that an abnormal interplay between mast cells, enterochromaffin (EC) cells, and afferent nerves contribute to nociception in gastrointestinal disorders. The aim of this study was to investigate how aging affects afferent sensitivity and neuro-immune association in the human bowel. Mechanical and chemical sensitivity of human bowel afferents were examined by ex vivo afferent nerve recordings. Age-related changes in the density of mast cells, EC cells, sensory nerve terminals, and mast cell-nerve micro-anatomical association were investigated by histological and immune staining. Human afferents could be broadly classified into subpopulations displaying mechanical and chemical sensitivity, adaptation, chemo-sensitization, and recruitment. Interestingly human bowel afferent nerve sensitivity was attenuated with age. The density of substance P-immunoreactive (SP-IR) nerve varicosities was also reduced with age. In contrast, the density of ileal and colonic mucosal mast cells was increased with age, as was ileal EC cell number. An increased proportion of mast cells was found in close apposition to SP-IR nerves. Afferent sensitivity in human bowel was reduced with advancing age. Augmentation of mast cells and EC cell numbers and the mast cell-nerve association suggest a compensatory mechanism for sensory neurodegeneration. © 2016 The Authors. Neurogastroenterology & Motility Published by John Wiley & Sons Ltd.

Macrophage-Mediated Glial Cell Elimination in the Postnatal Mouse Cochlea

PubMed Central

Brown, LaShardai N.; Xing, Yazhi; Noble, Kenyaria V.; Barth, Jeremy L.; Panganiban, Clarisse H.; Smythe, Nancy M.; Bridges, Mary C.; Zhu, Juhong; Lang, Hainan

2017-01-01

Hearing relies on the transmission of auditory information from sensory hair cells (HCs) to the brain through the auditory nerve. This relay of information requires HCs to be innervated by spiral ganglion neurons (SGNs) in an exclusive manner and SGNs to be ensheathed by myelinating and non-myelinating glial cells. In the developing auditory nerve, mistargeted SGN axons are retracted or pruned and excessive cells are cleared in a process referred to as nerve refinement. Whether auditory glial cells are eliminated during auditory nerve refinement is unknown. Using early postnatal mice of either sex, we show that glial cell numbers decrease after the first postnatal week, corresponding temporally with nerve refinement in the developing auditory nerve. Additionally, expression of immune-related genes was upregulated and macrophage numbers increase in a manner coinciding with the reduction of glial cell numbers. Transient depletion of macrophages during early auditory nerve development, using transgenic CD11bDTR/EGFP mice, resulted in the appearance of excessive glial cells. Macrophage depletion caused abnormalities in myelin formation and transient edema of the stria vascularis. Macrophage-depleted mice also showed auditory function impairment that partially recovered in adulthood. These findings demonstrate that macrophages contribute to the regulation of glial cell number during postnatal development of the cochlea and that glial cells play a critical role in hearing onset and auditory nerve maturation. PMID:29375297

Clinical and electrophysiological characteristics of symmetric polyneuropathy in a cohort of systemic lupus erythematosus patients.

PubMed

Jasmin, R; Sockalingam, S; Ramanaidu, L P; Goh, K J

2015-03-01

Peripheral neuropathy in systemic lupus erythematosus (SLE) is heterogeneous and its commonest pattern is symmetrical polyneuropathy. The aim of this study was to describe the prevalence, clinical and electrophysiological features, disease associations and effects on function and quality of life of polyneuropathy in SLE patients, defined using combined clinical and electrophysiological diagnostic criteria. Consecutive SLE patients seen at the University of Malaya Medical Centre were included. Patients with medication and other disorders known to cause neuropathy were excluded. Demographic, clinical and laboratory data were obtained using a pre-defined questionnaire. Function and health-related quality of life was assessed using the modified Rankin scale and the SF-36 scores. Nerve conduction studies (NCS) were carried out in both upper and lower limbs. Polyneuropathy was defined as the presence of bilateral clinical symptoms and/or signs and bilateral abnormal NCS parameters. Of 150 patients, 23 (15.3%) had polyneuropathy. SLE-related polyneuropathy was mainly characterized by sensory symptoms of numbness/tingling and pain with mild signs of absent ankle reflexes and reduced pain sensation. Function was minimally affected and there were no differences in quality of life scores. NCS abnormalities suggested mild length-dependent axonal neuropathy, primarily in the distal lower limbs. Compared to those without polyneuropathy, SLE-related polyneuropathy patients were significantly older but had no other significant demographic or disease associations. SLE-related polyneuropathy is a chronic, axonal and predominantly sensory neuropathy, associated with older age. Its underlying pathogenetic mechanisms are unknown, although a possibility could be an increased susceptibility of peripheral nerves in SLE patients to effects of aging. © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

Fibrolipomatous hamartoma: pathognomonic on MR imaging.

PubMed

Marom, E M; Helms, C A

1999-05-01

To assess the MR imaging characteristics, presenting symptoms, age and nerve distribution of fibrolipomatous hamartoma. A computer search was performed of the term fibrolipomatous hamartoma through the musculoskeletal section MR imaging results at our institution from June 7, 1996 to January 21, 1998 followed by a search of the terms lipomatous hamartoma, median nerve, surrounding fat, increased fatty signal, coaxial, and neuroma. MR images and medical files were retrospectively reviewed by two experienced musculoskeletal radiologists for imaging characteristics, nerve and age distribution as well as for history of trauma. In addition three consultation cases from outside institutions were added for determination of image characteristics. Ten fibrolipomatous hamartomas were identified: eight in the median nerve, one in the ulnar nerve and one in the sciatic nerve. Mean age was 32.3 years (range 4-75 years, SD 21 years). Imaging characteristics were serpiginous low-intensity structures representing thickened nerve fascicles, surrounded by evenly distributed fat, high signal intensity on T1-weighted sequences and low signal intensity on T2-weighted sequences. The amount of fat varied; however, distribution in eight cases (80%) was predominantly between nerve fibers rather than surrounding them peripherally. All had a coaxial-cable-like appearance on axial planes and a spaghetti-like appearance on coronal planes that was not seen in any other type of median nerve abnormality imaged during the study period. The MR imaging characteristics of fibrolipomatous hamartoma are pathognomonic, obviating the need for biopsy for diagnosis.

Evaluation of retinal nerve fiber layer thickness in the area of apparently normal hemifield in glaucomatous eyes with optical coherence tomography.

PubMed

Kee, Changwon; Cho, Changhwan

2003-06-01

The authors investigated the correlation between visual field defects detected by automated perimetry and the thickness of the retinal nerve fiber layer measured with optical coherence tomography, and examined whether there is a decrease in retinal nerve fiber layer thickness in the apparently normal hemifield of glaucomatous eyes. Forty-one patients with glaucoma and 41 normal control subjects were included in this study. Statistical correlations between the sum of the total deviation of 37 stimuli of each hemifield and the ratio of decrease in retinal nerve fiber layer thickness were evaluated. The statistical difference between the retinal nerve fiber layer thickness of the apparently normal hemifield in glaucomatous eyes and that of the corresponding hemifield in normal subjects was also evaluated. There was a statistically significant correlation in the sum of the total deviation and retinal nerve fiber layer thickness decrease ratio (superior hemifield, P = 0.001; inferior hemifield, P = 0.003). There was no significant decrease in retinal nerve fiber layer thickness in the area that corresponded to the normal visual field in the hemifield defect with respect to the horizontal meridian in glaucomatous eyes (superior side, P = 0.148; inferior side, P = 0.341). Optical coherence tomography was capable of demonstrating and measuring retinal nerve fiber layer abnormalities. No changes in the retinal nerve fiber layer thickness of the apparently normal hemifield were observed in glaucomatous eyes.

Facial nerve conduction after sclerotherapy in children with facial lymphatic malformations: report of two cases.

PubMed

Lin, Pei-Jung; Guo, Yuh-Cherng; Lin, Jan-You; Chang, Yu-Tang

2007-04-01

Surgical excision is thought to be the standard treatment of choice for lymphatic malformations. However, when the lesions are limited to the face only, surgical scar and facial nerve injury may impair cosmetics and facial expression. Sclerotherapy, an injection of a sclerosing agent directly through the skin into a lesion, is an alternative method. By evaluating facial nerve conduction, we observed the long-term effect of facial lymphatic malformations after intralesional injection of OK-432 and correlated the findings with anatomic outcomes. One 12-year-old boy with a lesion over the right-side preauricular area adjacent to the main trunk of facial nerve and the other 5-year-old boy with a lesion in the left-sided cheek involving the buccinator muscle were enrolled. The follow-up data of more than one year, including clinical appearance, computed tomography (CT) scan and facial nerve evaluation were collected. The facial nerve conduction study was normal in both cases. Blink reflex in both children revealed normal results as well. Complete resolution was noted on outward appearance and CT scan. The neurophysiologic data were compatible with good anatomic and functional outcomes. Our report suggests that the inflammatory reaction of OK-432 did not interfere with adjacent facial nerve conduction.

Fish oil supplementation prevents diabetes-induced nerve conduction velocity and neuroanatomical changes in rats.

PubMed

Gerbi, A; Maixent, J M; Ansaldi, J L; Pierlovisi, M; Coste, T; Pelissier, J F; Vague, P; Raccah, D

1999-01-01

Diabetic neuropathy has been associated with a decrease in nerve conduction velocity, Na,K-ATPase activity and characteristic histological damage of the sciatic nerve. The aim of this study was to evaluate the potential effect of a dietary supplementation with fish oil [(n-3) fatty acids] on the sciatic nerve of diabetic rats. Diabetes was induced by intravenous streptozotocin injection. Diabetic animals (n = 20) were fed a nonpurified diet supplemented with either olive oil (DO) or fish oil (DM), and control animals (n = 10) were fed a nonpurified diet supplemented with olive oil at a daily dose of 0.5 g/kg by gavage for 8 wk. Nerves were characterized by their conduction velocity, morphometric analysis and membrane Na, K-ATPase activity. Nerve conduction velocity, as well as Na,K-ATPase activity, was improved by fish oil treatment. A correlation was found between these two variables (R = 0.999, P < 0.05). Moreover, a preventive effect of fish oil was observed on nerve histological damage [endoneurial edema, axonal degeneration (by 10-15%) with demyelination]. Moreover, the normal bimodal distribution of the internal diameter of myelinated fibers was absent in the DO group and was restored in the DM group. These data suggest that fish oil therapy may be effective in the prevention of diabetic neuropathy.

Potential Involvement of Draxin in the Axonal Projection of Cranial Nerves, Especially Cranial Nerve X, in the Chick Hindbrain

PubMed Central

Zhang, Sanbing; Cui, Huixian; Wang, Lei; Kang, Lin; Huang, Guannan; Du, Juan; Li, Sha; Tanaka, Hideaki; Su, Yuhong

2016-01-01

The appropriate projection of axons within the nervous system is a crucial component of the establishment of neural circuitry. Draxin is a repulsive axon guidance protein. Draxin has important functions in the guidance of three commissures in the central nervous system and in the migration of neural crest cells and dI3 interneurons in the chick spinal cord. Here, we report that the distribution of the draxin protein and the location of 23C10-positive areas have a strong temporal and spatial correlation. The overexpression of draxin, especially transmembrane draxin, caused 23C10-positive axon bundles to misproject in the dorsal hindbrain. In addition, the overexpression of transmembrane draxin caused abnormal formation of the ganglion crest of the IX and X cranial nerves, misprojection of some anti-human natural killer-1 (HNK-1)-stained structures in the dorsal roof of the hindbrain, and a simultaneous reduction in the efferent nerves of some motoneuron axons inside the hindbrain. Our data reveal that draxin might be involved in the fascicular projection of cranial nerves in the hindbrain. PMID:27199282

Slower nerve conduction velocity in individuals with functional ankle instability.

PubMed

Simon, J; Docherty, C

2014-08-01

The purpose of this study is to quantify nerve conduction velocity differences in individuals with functional ankle instability compared to a "healthy" population. 38 participants ages 18-30 were recruited from a large university with approximately 43,000 students. 19 subjects (9 men and 10 women; age=21.0±1.4 years; height=172.0±9.3 cm; mass=74.4±1 2.4 kg) with symptoms of functional ankle instability were in the functional ankle instability group. 19 subjects (10 men, 9 women; age=22.0±2.6 years; height=169.8±9.1 cm; mass=69.0±14.8 kg) with "healthy" ankles were in the control group. Nerve conduction velocity was conducted using one trial at 2 different sites: posterior to the fibular head (fibular), and 10 cm superior/posterior of the first site (popliteal). Nerve conduction velocity (m/sec) was assessed using a SierraWave II system (Cadwell Laboratories; Kennewick, WA). A MANCOVA was performed on the two dependent variables (fibular and popliteal). Covariates included surface temperature of the leg, body mass index, and age. The independent variable was group (functional ankle instability and control). The effect of group was significantly related to nerve conduction velocity at the fibular site (F(1, 27) =16.49, p=0.01) and popliteal site (F(1, 27)=4.51, p=0.01), with responses significantly faster for individuals in the control group than the functional ankle instability group. These results indicate that patients with functional ankle instability might have damage to the peroneal nerve which results in slower peroneal nerve conduction velocity. © Georg Thieme Verlag KG Stuttgart · New York.

Effectiveness of fibrin adhesive in facial nerve anastomosis in dogs compared with standard microsuturing technique.

PubMed

Attar, Bijan Movahedian; Zalzali, Haidar; Razavi, Mohammad; Ghoreishian, Mehdi; Rezaei, Majid

2012-10-01

Epineural suturing is the most common technique used for peripheral nerve anastomosis. In addition to the foreign body reaction to the suture material, the surgical duration and difficulty of suturing in confined anatomic locations are major problems. We evaluated the effectiveness of fibrin glue as an acceptable alternative for nerve anastomosis in dogs. Eight adult female dogs weighing 18 to 24 kg were used in the present study. The facial nerve was transected bilaterally. On the right side, the facial nerve was subjected to epineural suturing; and on the left side, the nerve was anastomosed using fibrin adhesive. After 16 weeks, the nerve conduction velocity and proportion of the nerve fibers that crossed the anastomosis site were evaluated and compared for the epineural suture (right side) and fibrin glue (left side). The data were analyzed using the paired t test and univariate analysis of variance. The mean postoperative nerve conduction velocity was 29.87 ± 7.65 m/s and 26.75 ± 3.97 m/s on the right and left side, respectively. No statistically significant difference was found in the postoperative nerve conduction velocity between the 2 techniques (P = .444). The proportion of nerve fibers that crossed the anastomotic site was 71.25% ± 7.59% and 72.25% ± 8.31% on the right and left side, respectively. The histologic evaluation showed no statistically significant difference in the proportion of the nerve fibers that crossed the anastomotic site between the 2 techniques (P = .598). The results suggest that the efficacies of epineural suturing and fibrin gluing in peripheral nerve anastomosis are similar. Copyright © 2012 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

Perimetric measurements with flicker-defined form stimulation in comparison with conventional perimetry and retinal nerve fiber measurements.

PubMed

Horn, Folkert K; Tornow, Ralf P; Jünemann, Anselm G; Laemmer, Robert; Kremers, Jan

2014-04-11

We compared the results of flicker-defined form (FDF) perimetry with standard automated perimetry (SAP) and retinal nerve fiber layer (RNFL) thickness measurements using spectral domain optical coherence tomography (OCT). A total of 64 healthy subjects, 45 ocular hypertensive patients, and 97 "early" open-angle glaucoma (OAG) patients participated in this study. Definition of glaucoma was based exclusively on glaucomatous optic disc appearance. All subjects underwent FDF perimetry, SAP, and peripapillary measurements of the RNFL thickness. The FDF perimetry and SAP were performed at identical test locations (G1 protocol). Exclusion criteria were subjects younger than 34 years, SAP mean defect (SAP MD) > 5 dB, eye diseases other than glaucoma, or nonreliable FDF measurements. The correlations between the perimetric data on one hand and RNFL thicknesses on the other hand were analyzed statistically. The age-corrected sensitivity values and the local results from the controls were used to determine FDF mean defect (FDF MD). The FDF perimetry and SAP showed high concordance in this cohort of experienced patients (MD values, R = -0.69, P < 0.001). Of a total of 42 OAG patients with abnormal SAP MD, 38 also displayed abnormal FDF MD. However, FDF MD was abnormal in 28 of 55 OAG patients with normal SAP MD. The FDF MD was significantly (R = -0.61, P < 0.001) correlated with RNFL thickness with a (nonsignificantly) larger correlation coefficient than conventional SAP MD (R = -0.48, P < 0.001). The FDF perimetry is able to uncover functional changes concurrent with the changes in RNFL thickness. The FDF perimetry may be an efficient functional test to detect early glaucomatous nerve atrophy. (ClinicalTrials.gov number, NCT00494923.).

Hibernating myocardium results in partial sympathetic denervation and nerve sprouting.

PubMed

Fernandez, Stanley F; Ovchinnikov, Vladislav; Canty, John M; Fallavollita, James A

2013-01-15

Hibernating myocardium due to chronic repetitive ischemia is associated with regional sympathetic nerve dysfunction and spontaneous arrhythmic death in the absence of infarction. Although inhomogeneity in regional sympathetic innervation is an acknowledged substrate for sudden death, the mechanism(s) responsible for these abnormalities in viable, dysfunctional myocardium (i.e., neural stunning vs. sympathetic denervation) and their association with nerve sprouting are unknown. Accordingly, markers of sympathetic nerve function and nerve sprouting were assessed in subendocardial tissue collected from chronically instrumented pigs with hibernating myocardium (n = 18) as well as sham-instrumented controls (n = 7). Hibernating myocardium exhibited evidence of partial sympathetic denervation compared with the normally perfused region and sham controls, with corresponding regional reductions in tyrosine hydroxylase protein (-32%, P < 0.001), norepinephrine uptake transport protein (-25%, P = 0.01), and tissue norepinephrine content (-45%, P < 0.001). Partial denervation induced nerve sprouting with regional increases in nerve growth factor precursor protein (31%, P = 0.01) and growth associated protein-43 (38%, P < 0.05). All of the changes in sympathetic nerve markers were similar in animals that developed sudden death (n = 9) compared with electively terminated pigs with hibernating myocardium (n = 9). In conclusion, sympathetic nerve dysfunction in hibernating myocardium is most consistent with partial sympathetic denervation and is associated with regional nerve sprouting. The extent of sympathetic remodeling is similar in animals that develop sudden death compared with survivors; this suggests that sympathetic remodeling in hibernating myocardium is not an independent trigger for sudden death. Nevertheless, sympathetic remodeling likely contributes to electrical instability in combination with other factors.

Hibernating myocardium results in partial sympathetic denervation and nerve sprouting

PubMed Central

Fernandez, Stanley F.; Ovchinnikov, Vladislav; Canty, John M.

2013-01-01

Hibernating myocardium due to chronic repetitive ischemia is associated with regional sympathetic nerve dysfunction and spontaneous arrhythmic death in the absence of infarction. Although inhomogeneity in regional sympathetic innervation is an acknowledged substrate for sudden death, the mechanism(s) responsible for these abnormalities in viable, dysfunctional myocardium (i.e., neural stunning vs. sympathetic denervation) and their association with nerve sprouting are unknown. Accordingly, markers of sympathetic nerve function and nerve sprouting were assessed in subendocardial tissue collected from chronically instrumented pigs with hibernating myocardium (n = 18) as well as sham-instrumented controls (n = 7). Hibernating myocardium exhibited evidence of partial sympathetic denervation compared with the normally perfused region and sham controls, with corresponding regional reductions in tyrosine hydroxylase protein (−32%, P < 0.001), norepinephrine uptake transport protein (−25%, P = 0.01), and tissue norepinephrine content (−45%, P < 0.001). Partial denervation induced nerve sprouting with regional increases in nerve growth factor precursor protein (31%, P = 0.01) and growth associated protein-43 (38%, P < 0.05). All of the changes in sympathetic nerve markers were similar in animals that developed sudden death (n = 9) compared with electively terminated pigs with hibernating myocardium (n = 9). In conclusion, sympathetic nerve dysfunction in hibernating myocardium is most consistent with partial sympathetic denervation and is associated with regional nerve sprouting. The extent of sympathetic remodeling is similar in animals that develop sudden death compared with survivors; this suggests that sympathetic remodeling in hibernating myocardium is not an independent trigger for sudden death. Nevertheless, sympathetic remodeling likely contributes to electrical instability in combination with other factors. PMID:23125211

Endoscopic carpal tunnel release: a prospective analysis of factors associated with unsatisfactory results.

PubMed

Straub, T A

1999-04-01

The first 100 consecutive cases of endoscopic carpal tunnel release (ECTR) performed by the author were studied prospectively during 6 to 24 months follow-up. Various preoperative and postoperative factors were subjected to statistical analysis to determine possible associations with unsatisfactory results. Overall, 92% of hands had a satisfactory result from ECTR, although not all were rendered symptom-free. There were no significant complications. Preoperative factors associated with an increased likelihood of unsatisfactory results included hands with preoperative weakness, widened two-point discrimination, myofascial pain syndrome or fibromyalgia, involvement in litigation, multiple compressive neuropathies, or the presence of abnormal psychological factors. A trend to less satisfactory results was present in Workers' Compensation cases and patients with normal motor latencies on nerve conduction studies. Multiple postoperative factors correlated with unsatisfactory results.

Waardenburg syndrome: a rare cause of inherited neuropathy due to SOX10 mutation.

PubMed

Bogdanova-Mihaylova, Petya; Alexander, Michael D; Murphy, Raymond P J; Murphy, Sinéad M

2017-09-01

Waardenburg syndrome (WS) is a rare disorder comprising sensorineural deafness and pigmentation abnormalities. Four distinct subtypes are defined based on the presence or absence of additional symptoms. Mutations in six genes have been described in WS. SOX10 mutations are usually associated with a more severe phenotype of WS with peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, and Hirschsprung disease. Here we report a 32-year-old man with a novel heterozygous missense variant in SOX10 gene, who presented with congenital deafness, Hirschsprung disease, iris heterochromia, foot deformity, and intermediate conduction velocity length-dependent sensorimotor neuropathy. This case highlights that the presence of other non-neuropathic features in a patient with presumed hereditary neuropathy should alert the clinician to possible atypical rare causes. © 2017 Peripheral Nerve Society.

Electroactive biodegradable polyurethane significantly enhanced Schwann cells myelin gene expression and neurotrophin secretion for peripheral nerve tissue engineering.

PubMed

Wu, Yaobin; Wang, Ling; Guo, Baolin; Shao, Yongpin; Ma, Peter X

2016-05-01

Myelination of Schwann cells (SCs) is critical for the success of peripheral nerve regeneration, and biomaterials that can promote SCs' neurotrophin secretion as scaffolds are beneficial for nerve repair. Here we present a biomaterials-approach, specifically, a highly tunable conductive biodegradable flexible polyurethane by polycondensation of poly(glycerol sebacate) and aniline pentamer, to significantly enhance SCs' myelin gene expression and neurotrophin secretion for peripheral nerve tissue engineering. SCs are cultured on these conductive polymer films, and the biocompatibility of these films and their ability to enhance myelin gene expressions and sustained neurotrophin secretion are successfully demonstrated. The mechanism of SCs' neurotrophin secretion on conductive films is demonstrated by investigating the relationship between intracellular Ca(2+) level and SCs' myelination. Furthermore, the neurite growth and elongation of PC12 cells are induced by adding the neurotrophin medium suspension produced from SCs-laden conductive films. These data suggest that these conductive degradable polyurethanes that enhance SCs' myelin gene expressions and sustained neurotrophin secretion perform great potential for nerve regeneration applications. Copyright © 2016 Elsevier Ltd. All rights reserved.

Suprascapular nerve entrapment in newsreel cameramen.

PubMed

Karataş, Gülçin Kaymak; Göğüş, Feride

2003-03-01

To determine presence of suprascapular nerve entrapment in a group of newsreel cameramen. Thirty-six men working as newsreel cameramen participated in the study. In addition to musculoskeletal and neurologic examinations, bilateral suprascapular nerve conduction studies and needle electromyography were performed. A group of 19 healthy, male volunteers were included in the study as normal controls for suprascapular nerve conduction studies. In newsreel cameramen, mean suprascapular nerve latency was 3.20 +/- 0.56 msec and 2.84 +/- 0.36 msec for right and left shoulders, respectively (P = 0.001). The mean latency difference between right and left suprascapular nerves was -0.05 +/- 0.19 msec in the control group and 0.36 +/- 0.58 msec in the cameramen group (P < 0.001). Six subjects' right suprascapular nerve motor latencies were 2 SD above the normal mean values. There was no relationship between suprascapular nerve latencies and the age, professional life, and number of hours worked daily by the subjects. Carrying a heavy, mobile camera on the shoulder might cause suprascapular nerve entrapment in newsreel cameramen. This could be considered an occupational disorder of the suprascapular nerve, like meat-packer's neuropathy.

Performance standards of the nerve conduction study technologist.

PubMed

Neal, Peggy J; Katirji, Bashar

2008-06-01

The primary performance standard of the nerve conduction study technologist is to perform nerve conduction studies (NCS) on patients with trauma or diseases of the peripheral nervous system. However, to be in compliance with various healthcare/medical accreditation agencies, such as Occupational Safety and Health Administration (OSHA), Centers for Disease Control and Prevention (CDC), or The Joint Commission [formerly known as the Joint Commission on Accreditation of Healthcare Organizations (JCAHO)], there are many other important performance objectives that must be met as well. It is the responsibility of the NCS technologist to meet these standards on a daily basis. Once these standards become an expected responsibility of the NCS technologist's position, the patient undergoing a nerve conduction study should be assured of a high quality test performed in an optimal safety environment.

Correlation between Clinical Features and Magnetic Resonance Imaging Findings in Lumbar Disc Prolapse.

PubMed

Thapa, S S; Lakhey, R B; Sharma, P; Pokhrel, R K

2016-05-01

Magnetic resonance imaging is routinely done for diagnosis of lumbar disc prolapse. Many abnormalities of disc are observed even in asymptomatic patient.This study was conducted tocorrelate these abnormalities observed on Magnetic resonance imaging and clinical features of lumbar disc prolapse. A This prospective analytical study includes 57 cases of lumbar disc prolapse presenting to Department of Orthopedics, Tribhuvan University Teaching Hospital from March 2011 to August 2012. All patientshad Magnetic resonance imaging of lumbar spine and the findings regarding type, level and position of lumbar disc prolapse, any neural canal or foraminal compromise was recorded. These imaging findings were then correlated with clinical signs and symptoms. Chi-square test was used to find out p-value for correlation between clinical features and Magnetic resonance imaging findings using SPSS 17.0. This study included 57 patients, with mean age 36.8 years. Of them 41(71.9%) patients had radicular leg pain along specific dermatome. Magnetic resonance imaging showed 104 lumbar disc prolapselevel. Disc prolapse at L4-L5 and L5-S1 level constituted 85.5%.Magnetic resonance imaging findings of neural foramina compromise and nerve root compression were fairly correlated withclinical findings of radicular pain and neurological deficit. Clinical features and Magnetic resonance imaging findings of lumbar discprolasehad faircorrelation, but all imaging abnormalities do not have a clinical significance.

The comparison of multiple F-wave variable studies and magnetic resonance imaging examinations in the assessment of cervical radiculopathy.

PubMed

Lin, Chu-Hsu; Tsai, Yuan-Hsiung; Chang, Chia-Hao; Chen, Chien-Min; Hsu, Hung-Chih; Wu, Chun-Yen; Hong, Chang-Zern

2013-09-01

The aims of this study were to investigate the correlation of the findings of multiple median and ulnar F-wave variables and magnetic resonance imaging examinations in the prediction of cervical radiculopathy. The data of 68 patients who underwent both nerve conduction studies of the upper extremities and cervical spine magnetic resonance imaging within 3 mos of the nerve conduction studies were retrospectively reviewed and reinterpreted. The associations between multiple median and ulnar F-wave variables (including persistence, chronodispersion, and minimal, maximal, and mean latencies) and magnetic resonance imaging evidence of lower cervical spondylotic radiculopathy (i.e., C7, C8, and T1 radiculopathy) were investigated. Patients with lower cervical radiculopathy exhibited reduced right median F-wave persistence (P = 0.011), increased right ulnar F-wave chronodispersion (P = 0.041), and a trend toward increased left ulnar F-wave chronodispersion (P = 0.059); however, there were no other consistent significant differences in the F-wave variables between patients with and patients without magnetic resonance imaging evidence of lower cervical radiculopathy. In comparison with normal reference values established previously, the sensitivity and positive predictive value of F-wave variable abnormalities for predicting lower cervical radiculopathy were low. There was a low correlation between F-wave studies and magnetic resonance imaging examinations. The diagnostic utility of multiple F-wave variables in the prediction of cervical radiculopathy was not supported by this study.

Neuropathology of leukoencephalopathy with brainstem and spinal cord involvement and high lactate caused by a homozygous mutation of DARS2.

PubMed

Yamashita, Sumimasa; Miyake, Noriko; Matsumoto, Naomichi; Osaka, Hitoshi; Iai, Mizue; Aida, Noriko; Tanaka, Yukichi

2013-04-01

We diagnosed three siblings from consanguineous east Asian parents with leukoencephalopathy with brainstem and spinal cord involvement and high lactate (LBSL) from characteristic MRI, MRS findings and a homozygous mutation in the DARS2 gene. The neurological symptoms of the three patients consisted of psychomotor developmental delay, cerebellar ataxia since infancy, spasticity in the initial phase and peripheral neuropathy in later stages. Their mental development was delayed, but did not deteriorate. MRI signal abnormalities included the same abnormalities reported previously but tended to be more extensive. Signal abnormalities in the cerebral and cerebellar white matter were homogeneous and confluent from early stages. In addition, other tract such as the central tegmental tract was involved. Furthermore, an atrophic change in the cerebral white matter was observed on follow-up in one case. Two of the patients were autopsied and neuropathological findings revealed characteristic vacuolar changes in the white matter of the cerebrum, cerebellum and the nerve tracts of the brain stem and spinal cord. The central myelin sheath showed intralamellar splitting by electron microscopy. These findings were consistent to a spongy degeneration in the diffuse white matter of the brain, or spongiform leukoencephalopathy. In addition, peripheral nerves showed both axonal degeneration and abnormal myelin structures. We discussed the relationship between deficits in mitochondrial aspartyl-tRNA synthetase activity and the neuropathology observed. Copyright © 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Adult polyglucosan body disease presenting as a unilateral progressive plexopathy.

PubMed

Naddaf, Elie; Kassardjian, Charles D; Kurt, Yasemin Gulcan; Akman, Hasan Orhan; Windebank, Anthony J

2016-06-01

Adult polyglucosan body disease (APBD) usually presents with progressive spastic paraparesis, neurogenic bladder, and distal lower limb sensory abnormalities. It is caused by mutations in the glycogen branching enzyme gene (GBE1). We describe a woman with an unusual phenotype manifesting as progressive left brachial more than lumbosacral plexopathies, with central sensory and corticospinal tract involvement. Magnetic resonance imaging of the brain and cervical spine showed abnormal T2 signal within the ventral pons and medulla bilaterally, involving the pyramidal tracts and the medial leminisci. There was also medullary and cervical spine atrophy. On nerve biopsy, large polyglucosan bodies were noted in the endoneurium. The patient was found to be compound heterozygous for 2 novel mutations in GBE1. Peripheral blood leukocyte GBE activity was markedly reduced to 7% of normal, confirming the diagnosis of APBD. In this report we describe a new phenotype of APBD associated with 2 novel mutations. Muscle Nerve 53: 976-981, 2016. © 2016 Wiley Periodicals, Inc.

Not all cases of nyctalopia are benign: Unusual and serendipitous presentation of Arnold–Chiari Type 1 malformation at a Pediatric Tertiary Care Center

PubMed Central

Patra, Kailash Chandra; Kirtane, Abhijeet Prakash

2016-01-01

The Arnold–Chiari Type 1 malformation (CM1) is a rare congenital abnormality characterized by ectopia or caudal herniation of the cerebellar tonsils through the foramen magnum into the cervical spine, resulting in crowding at the craniocervical junction. It seldom presents in childhood with symptoms and a normal neurological examination. More typically, CM1 presents in young adult women with neurological symptoms including a headache, cervical pain, cranial nerve palsies, neurosensory deficit, and ataxia. Ocular manifestations associated with Chiari I include third and sixth cranial nerve paresis and convergence/divergence abnormalities. Papilledema is a rare manifestation of Chiari I with a clinical presentation often similar to that of idiopathic intracranial hypertension. To underscore this noteworthy complication, the authors report a case of an 8-year-old boy who presented with nyctalopia and suboccipital headaches, but was diagnosed serendipitously as a case of papilledema due to Chiari I malformation. PMID:27857798

Not all cases of nyctalopia are benign: Unusual and serendipitous presentation of Arnold-Chiari Type 1 malformation at a Pediatric Tertiary Care Center.

PubMed

Patra, Kailash Chandra; Kirtane, Abhijeet Prakash

2016-01-01

The Arnold-Chiari Type 1 malformation (CM1) is a rare congenital abnormality characterized by ectopia or caudal herniation of the cerebellar tonsils through the foramen magnum into the cervical spine, resulting in crowding at the craniocervical junction. It seldom presents in childhood with symptoms and a normal neurological examination. More typically, CM1 presents in young adult women with neurological symptoms including a headache, cervical pain, cranial nerve palsies, neurosensory deficit, and ataxia. Ocular manifestations associated with Chiari I include third and sixth cranial nerve paresis and convergence/divergence abnormalities. Papilledema is a rare manifestation of Chiari I with a clinical presentation often similar to that of idiopathic intracranial hypertension. To underscore this noteworthy complication, the authors report a case of an 8-year-old boy who presented with nyctalopia and suboccipital headaches, but was diagnosed serendipitously as a case of papilledema due to Chiari I malformation.

Cone dysfunctions in retinitis pigmentosa with retinal nerve fiber layer thickening

PubMed Central

Sobacı, Güngör; Özge, Gökhan; Gündoğan, Fatih Ç

2012-01-01

Purpose To investigate whether or not thicker retinal nerve fiber layer (RNFL) in retinitis pigmentosa (RP) patients relates to functional abnormalities of the photoreceptors. Methods Optical coherence tomography-based RNFL thickness was measured by Stratus-3™ (Zeiss, Basel, Switzerland) optical coherence tomography and electroretinogram (ERG) recordings made using the RETI-port® system (Roland, Wiesbaden, Germany) in 27 patients with retinitis pigmentosa and in 30 healthy subjects. Results Photopic ERG b-wave amplitude, cone ERG b-wave latency, 30 Hz flicker amplitude, and 30 Hz flicker latency had significant correlations to the RNFL-temporal (r = −0.55, P = 0.004, r = 0.68, P = 0.001, r = −0.65, P = 0.001, and r = −0.52, P = 0.007, respectively). Eyes with thicker RNFL (ten eyes) differed significantly from those with thinner RNFL (eight eyes) regarding cone ERG b-wave latency values only (P = 0.001). Conclusion Thicker RNFL in patients with retinitis pigmentosa may be associated with functional abnormality of the cone system. PMID:22536039

Cone dysfunctions in retinitis pigmentosa with retinal nerve fiber layer thickening.

PubMed

Sobacı, Güngör; Ozge, Gökhan; Gündoğan, Fatih Ç

2012-01-01

To investigate whether or not thicker retinal nerve fiber layer (RNFL) in retinitis pigmentosa (RP) patients relates to functional abnormalities of the photoreceptors. Optical coherence tomography-based RNFL thickness was measured by Stratus-3™ (Zeiss, Basel, Switzerland) optical coherence tomography and electroretinogram (ERG) recordings made using the RETI-port(®) system (Roland, Wiesbaden, Germany) in 27 patients with retinitis pigmentosa and in 30 healthy subjects. Photopic ERG b-wave amplitude, cone ERG b-wave latency, 30 Hz flicker amplitude, and 30 Hz flicker latency had significant correlations to the RNFL-temporal (r = -0.55, P = 0.004, r = 0.68, P = 0.001, r = -0.65, P = 0.001, and r = -0.52, P = 0.007, respectively). Eyes with thicker RNFL (ten eyes) differed significantly from those with thinner RNFL (eight eyes) regarding cone ERG b-wave latency values only (P = 0.001). Thicker RNFL in patients with retinitis pigmentosa may be associated with functional abnormality of the cone system.

Long term ocular and neurological involvement in severe congenital toxoplasmosis.

PubMed Central

Meenken, C; Assies, J; van Nieuwenhuizen, O; Holwerda-van der Maat, W G; van Schooneveld, M J; Delleman, W J; Kinds, G; Rothova, A

1995-01-01

AIMS--This study was set up to determine the long term ocular and systemic sequelae in patients with severe congenital toxoplasmosis. METHODS--Cross sectional and retrospective study of 17 patients with severe congenital toxoplasmosis. RESULTS--In addition to chorioretinitis (100%), the most common abnormal ocular features were optic nerve atrophy (83%), visual acuity of less than 0.1 (85%), strabismus, and microphthalmos. In 50% of cases we observed iridic abnormalities and about 40% developed a cataract. Overt endocrinological disease, diagnosed in five of 15 patients, included panhypopituitarism (n = 2), gonadal failure with dwarfism (n = 1), precocious puberty with dwarfism and thyroid deficiency (n = 1), and diabetes mellitus and thyroid deficiency (n = 1). The observed endocrinological involvement was associated in all cases with obstructive hydrocephalus with a dilated third ventricle and optic nerve atrophy. CONCLUSION--The recognition of long term ocular, neurological, and endocrinological sequelae of congenital toxoplasmosis is important for medical management of these severely handicapped patients. PMID:7626575

Morphological changes in the sciatic nerve, skeletal muscle, heart and brain of rabbits receiving continuous sciatic nerve block with 0.2% ropivacaine.

PubMed

Zhou, Yangning; He, Miao; Zou, Tianxiao; Yu, Bin

2015-01-01

To investigate the morphological changes in various tissues of rabbits receiving sciatic nerve block with 0.2% ropivacaine for 48 h. Twenty healthy were randomly assigned to normal saline group (N group) and ropivacaine group (R group). The right sciatic nerve was exposed, and a nerve-blocking trocar cannula embedded. Animals received an injection of 0.5% ropivacaine hydrochloride at a dose of 0.75 ml/kg. Rabbit was then connected to an infusion pump containing 50 ml of normal saline in N group, or to a infusion pump containing 0.2% ropivacaine hydrochloride in R group at 0.25 ml/kg•h-1. In both R group and N group, a small number of nerve cells exhibited pyknotic degeneration. More nerve cells with pyknotic degeneration were found in R group than in N group (P<0.001). At 48 h after surgery, there was a significant correlation between the abnormality of right hind limb and the degree of edema in sciatic nerve (P<0.01). Pyknotic degeneration of sciatic nerve increased after an infusion of 0.2% ropivacaine hydrochloride for 48 h, suggesting the neurotoxicity of ropivacaine. An infusion of 0.2% ropivacaine hydrochloride for 48 h may cause necrosis of skeletal muscle cells. The sciatic nerve edema would greatly affect the hindlimb motor while both pyknotic degeneration of sciatic nerve and skeletal muscle have little influence on the hindlimb movement. After an infusion of 0.2% ropivacaine hydrochloride for 48 h, the morphology of right atrium and brain tissues around the ventriculus tertius and medulla oblongata remained unchanged.

Relationship Between Optic Nerve Appearance and Retinal Nerve Fiber Layer Thickness as Explored with Spectral Domain Optical Coherence Tomography

PubMed Central

Aleman, Tomas S.; Huang, Jiayan; Garrity, Sean T.; Carter, Stuart B.; Aleman, Wendy D.; Ying, Gui-shuang; Tamhankar, Madhura A.

2014-01-01

Purpose To study the relationship between the appearance of the optic nerve and the retinal nerve fiber layer (RNFL) thickness determined by spectral domain optical coherence tomography (OCT). Methods Records from patients with spectral domain-OCT imaging in a neuro-ophthalmology practice were reviewed. Eyes with glaucoma/glaucoma suspicion, macular/optic nerve edema, pseudophakia, and with refractive errors > 6D were excluded. Optic nerve appearance by slit lamp biomicroscopy was related to the RNFL thickness by spectral domain-OCT and to visual field results. Results Ninety-one patients (176 eyes; mean age: 49 ± 15 years) were included. Eighty-three eyes (47%) showed optic nerve pallor; 89 eyes (50.6%) showed RNFL thinning (sectoral or average peripapillary). Average peripapillary RNFL thickness in eyes with pallor (mean ± SD = 76 ± 17 μm) was thinner compared to eyes without pallor (91 ± 14 μm, P < 0.001). Optic nerve pallor predicted RNFL thinning with a sensitivity of 69% and a specificity of 75%. Optic nerve appearance predicted RNFL thinning (with a sensitivity and specificity of 81%) when RNFL had thinned by ∼ 40%. Most patients with pallor had RNFL thinning with (66%) or without (25%) visual field loss; the remainder had normal RNFL and fields (5%) or with visual field abnormalities (4%). Conclusions Optic nerve pallor as a predictor of RNFL thinning showed fair sensitivity and specificity, although it is optimally sensitive/specific only when substantial RNFL loss has occurred. Translational Relevance Finding an acceptable relationship between the optic nerve appearance by ophthalmoscopy and spectral domain-OCT RNFL measures will help the clinician's interpretation of the information provided by this technology, which is gaining momentum in neuro-ophthalmic research. PMID:25374773

Early identification of ‘acute-onset’ chronic inflammatory demyelinating polyneuropathy

PubMed Central

Sung, Jia-Ying; Tani, Jowy; Park, Susanna B.; Kiernan, Matthew C.

2014-01-01

Distinguishing patients with acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy prior to relapse is often challenging at the onset of their clinical presentation. In the present study, nerve excitability tests were used in conjunction with the clinical phenotype and disease staging, to differentiate between patients with acute-onset chronic inflammatory demyelinating polyneuropathy and patients with acute inflammatory demyelinating polyneuropathy at an early stage, with the aim to better guide treatment. Clinical assessment, staging and nerve excitability tests were undertaken on patients initially fulfilling the diagnostic criteria of acute inflammatory demyelinating polyneuropathy soon after symptom onset and their initial presentation. Patients were subsequently followed up for minimum of 12 months to determine if their clinical presentations were more consistent with acute-onset chronic inflammatory demyelinating polyneuropathy. Clinical severity as evaluated by Medical Research Council sum score and Hughes functional grading scale were not significantly different between the two cohorts. There was no difference between the time of onset of initial symptoms and nerve excitability test assessment between the two cohorts nor were there significant differences in conventional nerve conduction study parameters. However, nerve excitability test profiles obtained from patients with acute inflammatory demyelinating polyneuropathy demonstrated abnormalities in the recovery cycle of excitability, including significantly reduced superexcitability (P < 0.001) and prolonged relative refractory period (P < 0.01), without changes in threshold electrotonus. In contrast, in patients with acute-onset chronic inflammatory demyelinating polyneuropathy, a different pattern occurred with the recovery cycle shifted downward (increased superexcitability, P < 0.05; decreased subexcitability, P < 0.05) and increased threshold change in threshold electrotonus in both hyperpolarizing and depolarizing directions [depolarizing threshold electrotonus (90–100 ms) P < 0.005, hyperpolarizing threshold electrotonus (10–20 ms), P < 0.01, hyperpolarizing threshold electrotonus (90–100 ms), P < 0.05], perhaps suggesting early hyperpolarization. In addition, using excitability parameters superexcitability, subexcitability and hyperpolarizing threshold electrotonus (10–20 ms), the patients with acute inflammatory demyelinating polyneuropathy and acute-onset chronic inflammatory demyelinating polyneuropathy could be clearly separated into two non-overlapping groups. Studies of nerve excitability may be able to differentiate acute from acute-onset chronic inflammatory demyelinating polyneuropathy at an early stage. Characteristic nerve excitability parameter changes occur in early acute-onset chronic inflammatory demyelinating polyneuropathy, to match the clinical phenotype. Importantly, this pattern of change was strikingly different to that shown by patients with acute inflammatory demyelinating polyneuropathy, suggesting that nerve excitability techniques may be useful in distinguishing acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy at the initial stage. PMID:24983276

Application of conductive polymers, scaffolds and electrical stimulation for nerve tissue engineering.

PubMed

Ghasemi-Mobarakeh, Laleh; Prabhakaran, Molamma P; Morshed, Mohammad; Nasr-Esfahani, Mohammad Hossein; Baharvand, Hossein; Kiani, Sahar; Al-Deyab, Salem S; Ramakrishna, Seeram

2011-04-01

Among the numerous attempts to integrate tissue engineering concepts into strategies to repair nearly all parts of the body, neuronal repair stands out. This is partially due to the complexity of the nervous anatomical system, its functioning and the inefficiency of conventional repair approaches, which are based on single components of either biomaterials or cells alone. Electrical stimulation has been shown to enhance the nerve regeneration process and this consequently makes the use of electrically conductive polymers very attractive for the construction of scaffolds for nerve tissue engineering. In this review, by taking into consideration the electrical properties of nerve cells and the effect of electrical stimulation on nerve cells, we discuss the most commonly utilized conductive polymers, polypyrrole (PPy) and polyaniline (PANI), along with their design and modifications, thus making them suitable scaffolds for nerve tissue engineering. Other electrospun, composite, conductive scaffolds, such as PANI/gelatin and PPy/poly(ε-caprolactone), with or without electrical stimulation, are also discussed. Different procedures of electrical stimulation which have been used in tissue engineering, with examples on their specific applications in tissue engineering, are also discussed. Copyright © 2011 John Wiley & Sons, Ltd.

Preparation and characterization of electrical conductive PVA based materials for peripheral nerve tube-guides.

PubMed

Gonçalves, C; Ribeiro, J; Pereira, T; Luís, A L; Mauricio, A C; Santos, J D; Lopes, M A

2016-08-01

Peripheral nerve regeneration is a serious clinical problem. Presently, there are several nerve tube-guides available in the market, however with some limitations. The goal of this work was the development of a biomaterial with high electrical conductivity to produce tube-guides for nerve regeneration after neurotmesis injuries whenrver an end-to-end suture without tension is not possible. A matrix of poly(vinyl alcohol) (PVA) was used loaded with the following electrical conductive materials: COOH-functionalized multiwall carbon nanotubes (MWCNTs), poly(pyrrole) (PPy), magnesium chloride (MgCl2 ), and silver nitrate (AgNO3 ). The tube-guide production was carried out by a freezing/thawing process (physical crosslinking) with a final annealing treatment. After producing the tube-guide for nerve regeneration, the physicochemical characterization was performed. The most interesting results were achieved by loading PVA with 0.05% of PPy or COOH- functionalized CNTs. These tubes combined the electrical conductivity of carbon nanotubes (CNTs) and PPy with the biocompatibility of PVA matrix, with potential clinical application for nerve regeneration. © 2016 Wiley Periodicals, Inc. J Biomed Mater Res Part A: 104A: 1981-1987, 2016. © 2016 Wiley Periodicals, Inc.

Flaccid quadriplegia due to thyrotoxic myopathy.

PubMed

Couillard, Philippe; Wijdicks, Eelco F M

2014-04-01

Acute flaccid paralysis is an important clinical problem in neurological critical care. After implementing life-supporting measures, it is imperative to identify the correct diagnosis to provide timely appropriate care. Thyrotoxicosis is a recognized cause of myopathy, but rarely of quadriplegia. Here, we report a case of hyperthyroidism with severe weakness. Case report and video demonstration of clinical examination. We describe a case of a 59-year-old woman with Grave's disease who presented to the hospital with progressive shortness of breath secondary to atrial fibrillation with rapid ventricular response. Following contrast administration, she had a pulseless electrical activity arrest from which she recovered without cognitive sequelae, but with flaccid quadriplegia, facial diplegia, and hypophonia. CK was mildly elevated and electrolytes were essentially normal. Nerve conduction studies and electromyography demonstrated features supporting an acute myopathy without evidence of neuromuscular junction conduction abnormality. Normalization of thyroid hormones resulted in slow, but steady improvement over months after which she regained ambulation. Acute flaccid quadriplegia can result from thyrotoxicosis. With normalization of thyroid function, recovery can be expected.

Changes in Ionic Conductance Signature of Nociceptive Neurons Underlying Fabry Disease Phenotype

PubMed Central

Namer, Barbara; Ørstavik, Kirstin; Schmidt, Roland; Mair, Norbert; Kleggetveit, Inge Petter; Zeidler, Maximillian; Martha, Theresa; Jorum, Ellen; Schmelz, Martin; Kalpachidou, Theodora; Kress, Michaela; Langeslag, Michiel

2017-01-01

The first symptom arising in many Fabry patients is neuropathic pain due to changes in small myelinated and unmyelinated fibers in the periphery, which is subsequently followed by a loss of sensory perception. Here we studied changes in the peripheral nervous system of Fabry patients and a Fabry mouse model induced by deletion of α-galactosidase A (Gla−/0). The skin innervation of Gla−/0 mice resembles that of the human Fabry patients. In Fabry diseased humans and Gla−/0 mice, we observed similar sensory abnormalities, which were also observed in nerve fiber recordings in both patients and mice. Electrophysiological recordings of cultured Gla−/0 nociceptors revealed that the conductance of voltage-gated Na+ and Ca2+ currents was decreased in Gla−/0 nociceptors, whereas the activation of voltage-gated K+ currents was at more depolarized potentials. Conclusively, we have observed that reduced sensory perception due to small-fiber degeneration coincides with altered electrophysiological properties of sensory neurons. PMID:28769867

Color blindness among multiple sclerosis patients in Isfahan

PubMed Central

Shaygannejad, Vahid; Golabchi, Khodayar; Dehghani, Alireza; Ashtari, Fereshteh; Haghighi, Sepehr; Mirzendehdel, Mahsa; Ghasemi, Majid

2012-01-01

Background: Multiple sclerosis (MS) is a disease of young and middle aged individuals with a demyelinative axonal damage nature in central nervous system that causes various signs and symptoms. As color vision needs normal function of optic nerve and macula, it is proposed that MS can alter it via influencing optic nerve. In this survey, we evaluated color vision abnormalities and its relationship with history of optic neuritis and abnormal visual evoked potentials (VEPs) among MS patients. Materials and Methods: The case group was included of clinically definitive MS patients and the same number of normal population was enrolled as the control group. Color vision of all the participants was evaluated by Ishihara test and then visual evoked potential (VEPs) and history of optic neuritis (ON) was assessed among them. Then, frequency of color blindness was compared between the case and the control group. Finally, color blinded patients were compared to those with the history of ON and abnormal VEPs. Results: 63 MS patients and the same number of normal populations were enrolled in this study. 12 patients had color blindness based on the Ishihara test; only 3 of them were among the control group, which showed a significant different between the two groups (P = 0.013). There was a significant relationship between the color blindness and abnormal VEP (R = 0.53, P = 0.023) but not for the color blindness and ON (P = 0.67). Conclusions: This study demonstrates a significant correlation between color blindness and multiple sclerosis including ones with abnormal prolonged VEP latencies. Therefore, in individuals with acquired color vision impairment, an evaluation for potentially serious underlying diseases like MS is essential. PMID:23267377

Uncompacted Myelin Lamellae and Nodal Ion Channel Disruption in POEMS Syndrome.

PubMed

Hashimoto, Rina; Koike, Haruki; Takahashi, Mie; Ohyama, Ken; Kawagashira, Yuichi; Iijima, Masahiro; Sobue, Gen

2015-12-01

To elucidate the significance of uncompacted myelin lamellae (UML) and ion channel disruption at the nodes of Ranvier in the polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, we evaluated sural nerve biopsy specimens from 33 patients with POEMS syndrome and from 7 control patients. Uncompacted myelin lamellae distribution was assessed by electron microscopy and immunofluorescence microscopy. In the POEMS patient biopsies, UML were seen more frequently in small versus large myelinated fibers. Paranodes and Schmidt-Lanterman incisures, where normal physiologic UM is located, were frequently associated with UM. Widening of the nodes of Ranvier (i.e. segmental demyelination) was not associated with UML. There was axonal hollowing with neurofilament condensation at Schmidt-Lanterman incisures with abnormal UML, suggesting axonal damage at those sites in the POEMS patient biopsies. Myelin sheath irregularity was conspicuous in large myelinated fibers and was associated with abnormally widened bizarrely shaped Schmidt-Lanterman incisures. Indirect immunofluorescent studies revealed abnormalities of sodium (pan sodium) and potassium (KCNQ2) channels, even at nonwidened nodes of Ranvier. Thus, UML was not apparently associated with segmental demyelination but seemed to be associated with axonal damage. These observations suggest that nodal ion channel disruption may be associated with functional deficits in POEMS syndrome patient nerves.

Electrical Stimulation of the Ear, Head, Cranial Nerve, or Cortex for the Treatment of Tinnitus: A Scoping Review

PubMed Central

Adjamian, Peyman

2016-01-01

Tinnitus is defined as the perception of sound in the absence of an external source. It is often associated with hearing loss and is thought to result from abnormal neural activity at some point or points in the auditory pathway, which is incorrectly interpreted by the brain as an actual sound. Neurostimulation therapies therefore, which interfere on some level with that abnormal activity, are a logical approach to treatment. For tinnitus, where the pathological neuronal activity might be associated with auditory and other areas of the brain, interventions using electromagnetic, electrical, or acoustic stimuli separately, or paired electrical and acoustic stimuli, have been proposed as treatments. Neurostimulation therapies should modulate neural activity to deliver a permanent reduction in tinnitus percept by driving the neuroplastic changes necessary to interrupt abnormal levels of oscillatory cortical activity and restore typical levels of activity. This change in activity should alter or interrupt the tinnitus percept (reduction or extinction) making it less bothersome. Here we review developments in therapies involving electrical stimulation of the ear, head, cranial nerve, or cortex in the treatment of tinnitus which demonstrably, or are hypothesised to, interrupt pathological neuronal activity in the cortex associated with tinnitus. PMID:27403346

The role of laryngeal electromyography in vagus nerve stimulation-related vocal fold dysmotility.

PubMed

Saibene, Alberto M; Zambrelli, Elena; Pipolo, Carlotta; Maccari, Alberto; Felisati, Giovanni; Felisati, Elena; Furia, Francesca; Vignoli, Aglaia; Canevini, Maria Paola; Alfonsi, Enrico

2017-03-01

Vagus nerve stimulation (VNS) is a useful tool for drug-resistant epilepsy, but it induces known laryngeal side effects, with a significant role on patients' quality of life. VNS patients may show persistent left vocal fold (LVF) palsy at rest and/or recurrent LVF adduction during stimulation. This study aims at electromyographically evaluating laryngeal muscles abnormalities in VNS patients. We compared endoscopic laryngeal evaluation data in six VNS patients with laryngeal muscle electromyography (LMEMG) carried out on the thyroarytenoid, cricothyroid, posterior cricoarytenoid, and cricopharyngeal muscles. Endoscopy showed LVF palsy at rest in 3/6 patients in whom LMEMG documented a tonic spastic activity with reduced phasic modulation. In four out of six patients with recurrent LVF adduction during VNS activation, LMEMG showed a compound muscle action potential persisting for the whole stimulation. This is the first LMEMG report of VNS-induced motor unit activation via recurrent laryngeal nerve and upper laryngeal nerve stimulation. LMEMG data were could, therefore, be considered consistent with the endoscopic laryngeal examination in all patient.

Traction injury of the recurrent laryngeal nerve: Results of continuous intraoperative neuromonitoring in a swine model.

PubMed

Lee, Hye Yoon; Cho, Young Geon; You, Ji Young; Choi, Byoung Ho; Kim, Joon Yub; Wu, Che-Wei; Chiang, Feng-Yu; Kim, Hoon Yub

2016-04-01

Recurrent laryngeal nerve (RLN) palsy is the most serious complication after thyroidectomy. However, little is known about the degree of traction injury that causes loss of signal. The purpose of this study was to evaluate traction injuries in the swine RLN using continuous intraoperative neuromonitoring (IONM) and determine the traction power that results in loss of signal. Thirteen swine underwent traction injury to the RLNs with continuous IONM, and stress-strain curves were determined for 8 nerves using the universal material testing machine in an ex vivo model. Traction injury at a mean power of 2.83 MPa caused loss of signal. The mean physiologic limit strain and tensile strength of the swine RLNs were found to be 15.0% and 4.9 MPa, respectively. Histological analysis showed no abnormal structural findings. Traction injury of swine RLNs causes loss of signal at a power of 2.83 MPa. However, all injured nerves recovered within 7 days with no observed structural damage. © 2015 Wiley Periodicals, Inc.

Neural regulation of renal tubular sodium reabsorption and renin secretion: integrative aspects.

PubMed

DiBona, G F

1987-01-01

Efferent renal sympathetic nerve activity plays an important role in the regulation of renal function. Via its direct influence on renal tubular sodium reabsorption throughout the entire mammalian nephron, alterations in efferent renal sympathetic nerve activity represent an important physiological contribution to the overall role of the kidney in the regulation of external sodium balance and the defense against sodium deficit and surfeit. Abnormalities of this mechanism can lead to inappropriate renal sodium retention and augmentation of renin secretion, two factors which are capable of contributing to the development and maintenance of hypertension.

Kv1.1 knock-in ataxic mice exhibit spontaneous myokymic activity exacerbated by fatigue, ischemia and low temperature.

PubMed

Brunetti, Orazio; Imbrici, Paola; Botti, Fabio Massimo; Pettorossi, Vito Enrico; D'Adamo, Maria Cristina; Valentino, Mario; Zammit, Christian; Mora, Marina; Gibertini, Sara; Di Giovanni, Giuseppe; Muscat, Richard; Pessia, Mauro

2012-09-01

Episodic ataxia type 1 (EA1) is an autosomal dominant neurological disorder characterized by myokymia and attacks of ataxic gait often precipitated by stress. Several genetic mutations have been identified in the Shaker-like K(+) channel Kv1.1 (KCNA1) of EA1 individuals, including V408A, which result in remarkable channel dysfunction. By inserting the heterozygous V408A, mutation in one Kv1.1 allele, a mouse model of EA1 has been generated (Kv1.1(V408A/+)). Here, we investigated the neuromuscular transmission of Kv1.1(V408A/+) ataxic mice and their susceptibility to physiologically relevant stressors. By using in vivo preparations of lateral gastrocnemius (LG) nerve-muscle from Kv1.1(+/+) and Kv1.1(V408A/+) mice, we show that the mutant animals exhibit spontaneous myokymic discharges consisting of repeated singlets, duplets or multiplets, despite motor nerve axotomy. Two-photon laser scanning microscopy from the motor nerve, ex vivo, revealed spontaneous Ca(2+) signals that occurred abnormally only in preparations dissected from Kv1.1(V408A/+) mice. Spontaneous bursting activity, as well as that evoked by sciatic nerve stimulation, was exacerbated by muscle fatigue, ischemia and low temperatures. These stressors also increased the amplitude of compound muscle action potential. Such abnormal neuromuscular transmission did not alter fiber type composition, neuromuscular junction and vascularization of LG muscle, analyzed by light and electron microscopy. Taken together these findings provide direct evidence that identifies the motor nerve as an important generator of myokymic activity, that dysfunction of Kv1.1 channels alters Ca(2+) homeostasis in motor axons, and also strongly suggest that muscle fatigue contributes more than PNS fatigue to exacerbate the myokymia/neuromyotonia phenotype. More broadly, this study points out that juxtaparanodal K(+) channels composed of Kv1.1 subunits exert an important role in dampening the excitability of motor nerve axons during fatigue or ischemic insult. Copyright © 2012 Elsevier Inc. All rights reserved.

Nanofiber Nerve Guide for Peripheral Nerve Repair and Regeneration

DTIC Science & Technology

2016-04-01

faster regeneration and functional recovery. Peripheral nerve injury is a common complication of complex tissue trauma and often results in significant...having poor regeneration overall, the areas of regenerating nerve tissue could often be found in sections of the nerve guide where luminal spaces of...conducted in this Aim also provided important insight into the NGC design parameters necessary to allow for maximum nerve tissue ingrowth and regeneration

Carpal tunnel syndrome and keyboard use at work: a population-based study.

PubMed

Atroshi, Isam; Gummesson, Christina; Ornstein, Ewald; Johnsson, Ragnar; Ranstam, Jonas

2007-11-01

To investigate the relationship between carpal tunnel syndrome (CTS) and keyboard use at work in a general population. A health status questionnaire was mailed to 2,465 persons of working age (25-65 years) who were randomly selected from the general population of a representative region of Sweden. The questionnaire required the subjects to provide information about the presence and severity of pain, numbness and tingling in each body region, employment history, and work activities, including average time spent using a keyboard during a usual working day. Those reporting recurrent hand numbness or tingling in the median nerve distribution were asked to undergo a physical examination and nerve conduction testing. The prevalence of CTS, defined as symptoms plus abnormal results on nerve conduction tests, was compared between groups of subjects that differed in their intensity of keyboard use, adjusting for age, sex, body mass index, and smoking status. Eighty-two percent responded to the questionnaire, and 80% of all symptomatic persons attended the examinations. Persons who had reported intensive keyboard use on the questionnaire were significantly less likely to be diagnosed as having CTS than were those who had reported little keyboard use, with a prevalence that increased from 2.6% in the highest keyboard use group (> or = 4 hours/day), to 2.9% in the moderate use group (1 to <4 hours/day), 4.9% in the low use group (<1 hour/day), and 5.2% in the no keyboard use at work group (P for trend = 0.032). Using > or = 1 hour/day to designate high keyboard use and <1 hour/day to designate low keyboard use, the prevalence ratio of CTS in the groups with high to low keyboard use was 0.55 (95% confidence interval 0.32, 0.96). Intensive keyboard use appears to be associated with a lower risk of CTS.

Conduction block of mammalian myelinated nerve by local cooling to 15–30°C after a brief heating

PubMed Central

Zhang, Zhaocun; Lyon, Timothy D.; Kadow, Brian T.; Shen, Bing; Wang, Jicheng; Lee, Andy; Kang, Audry; Roppolo, James R.; de Groat, William C.

2016-01-01

This study aimed at understanding thermal effects on nerve conduction and developing new methods to produce a reversible thermal block of axonal conduction in mammalian myelinated nerves. In 13 cats under α-chloralose anesthesia, conduction block of pudendal nerves (n = 20) by cooling (5–30°C) or heating (42–54°C) a small segment (9 mm) of the nerve was monitored by the urethral striated muscle contractions and increases in intraurethral pressure induced by intermittent (5 s on and 20 s off) electrical stimulation (50 Hz, 0.2 ms) of the nerve. Cold block was observed at 5–15°C while heat block occurred at 50–54°C. A complete cold block up to 10 min was fully reversible, but a complete heat block was only reversible when the heating duration was less than 1.3 ± 0.1 min. A brief (<1 min) reversible complete heat block at 50–54°C or 15 min of nonblock mild heating at 46–48°C significantly increased the cold block temperature to 15–30°C. The effect of heating on cold block fully reversed within ∼40 min. This study discovered a novel method to block mammalian myelinated nerves at 15–30°C, providing the possibility to develop an implantable device to block axonal conduction and treat many chronic disorders. The effect of heating on cold block is of considerable interest because it raises many basic scientific questions that may help reveal the mechanisms underlying cold or heat block of axonal conduction. PMID:26740534

Kv1.1 knock-in ataxic mice exhibit spontaneous myokymic activity exacerbated by fatigue, ischemia and low temperature

PubMed Central

Brunetti, Orazio; Imbrici, Paola; Botti, Fabio Massimo; Pettorossi, Vito Enrico; D'Adamo, Maria Cristina; Valentino, Mario; Zammit, Christian; Mora, Marina; Gibertini, Sara; Di Giovanni, Giuseppe; Muscat, Richard; Pessia, Mauro

2012-01-01

Episodic ataxia type 1 (EA1) is an autosomal dominant neurological disorder characterized by myokymia and attacks of ataxic gait often precipitated by stress. Several genetic mutations have been identified in the Shaker-like K+ channel Kv1.1 (KCNA1) of EA1 individuals, including V408A, which result in remarkable channel dysfunction. By inserting the heterozygous V408A, mutation in one Kv1.1 allele, a mouse model of EA1 has been generated (Kv1.1V408A/+). Here, we investigated the neuromuscular transmission of Kv1.1V408A/+ ataxic mice and their susceptibility to physiologically relevant stressors. By using in vivo preparations of lateral gastrocnemius (LG) nerve–muscle from Kv1.1+/+ and Kv1.1V408A/+ mice, we show that the mutant animals exhibit spontaneous myokymic discharges consisting of repeated singlets, duplets or multiplets, despite motor nerve axotomy. Two-photon laser scanning microscopy from the motor nerve, ex vivo, revealed spontaneous Ca2 + signals that occurred abnormally only in preparations dissected from Kv1.1V408A/+ mice. Spontaneous bursting activity, as well as that evoked by sciatic nerve stimulation, was exacerbated by muscle fatigue, ischemia and low temperatures. These stressors also increased the amplitude of compound muscle action potential. Such abnormal neuromuscular transmission did not alter fiber type composition, neuromuscular junction and vascularization of LG muscle, analyzed by light and electron microscopy. Taken together these findings provide direct evidence that identifies the motor nerve as an important generator of myokymic activity, that dysfunction of Kv1.1 channels alters Ca2 + homeostasis in motor axons, and also strongly suggest that muscle fatigue contributes more than PNS fatigue to exacerbate the myokymia/neuromyotonia phenotype. More broadly, this study points out that juxtaparanodal K+ channels composed of Kv1.1 subunits exert an important role in dampening the excitability of motor nerve axons during fatigue or ischemic insult. PMID:22609489

Essential Function of Protein 4.1G in Targeting of Membrane Protein Palmitoylated 6 into Schmidt-Lanterman Incisures in Myelinated Nerves

PubMed Central

Saitoh, Yurika; Ohno, Nobuhiko; Komada, Masayuki; Saitoh, Sei; Peles, Elior; Ohno, Shinichi

2012-01-01

Protein 4.1G is a membrane skeletal protein found in specific subcellular structures in myelinated Schwann cells and seminiferous tubules. Here, we show that in the mouse sciatic nerve, protein 4.1G colocalized at Schmidt-Lanterman incisures (SLI) and the paranodes with a member of the membrane-associated guanylate kinase (MAGUK) family, membrane protein palmitoylated 6 (MPP6). Coimmunoprecipitation experiments revealed that MPP6 was interacting with protein 4.1G. In contrast to wild-type nerves, in 4.1G knockout mice, MPP6 was found largely in the cytoplasm near Schwann cell nuclei, indicating an abnormal protein transport. Although the SLI remained in the 4.1G knockout sciatic nerves, as confirmed by E-cadherin immunostaining, their shape was altered in aged 4.1G knockout nerves compared to their shape in wild-type nerves. In the seminiferous tubules, MPP6 was localized similarly to protein 4.1G along cell membranes of the spermatogonium and early spermatocytes. However, in contrast to myelinated peripheral nerves, the specific localization of MPP6 in the seminiferous tubules was unaltered in the absence of protein 4.1G. These results indicate that 4.1G has a specific role in the targeting of MPP6 to the SLI and the assembly of these subcellular structures. PMID:22025680

Substance P-immunoreactive nerves in endobronchial biopsies in cough-variant asthma and classic asthma.

PubMed

Lee, Sang Yeub; Kim, Min Kyung; Shin, Chol; Shim, Jae Jeong; Kim, Han Kyeom; Kang, Kyung Ho; Yoo, Se Hwa; In, Kwang Ho

2003-01-01

Unlike classic asthma, cough-variant asthma does not show any evidence of airway obstruction. The main symptom is a dry cough with little known pathophysiology. Hypersensitivity of the cough receptors in cough-variant asthma and an increase in the sensory nerve density of the airway epithelium in persistent dry cough patients have been reported. Therefore, it is possible that there is a higher sensory nerve density in cough-variant asthma patients than in classic asthma patients. This study was undertaken to compare the substance P (SP)-immunoreactive nerve density in mucosal biopsies of cough-variant asthma patients, classic asthma patients, and in control subjects. Bronchoscopic biopsies were performed in 6 cough-variant asthma patients, 14 classic asthma patients, and 5 normal controls. The tissues obtained were stained immunohistochemically. The SP-immunoreactive nerve density was measured in the bronchial epithelium using a light microscope at 400 x magnification. SP- immunoreactive nerve density for the cough-variant asthma group was significantly higher than that of the classic asthma group (p = 0.001), and of the normal control group (p = 0.006). It is possible that a sensory nerve abnormality within the airway may be related to hypersensitivity of the cough receptor, and that this may be one of the pathophysiologies of cough-variant asthma. Copyright 2003 S. Karger AG, Basel

Otosclerosis surgery: approaches, profits and complications.

PubMed

Japaridze, Sh; Lomidze, L; Jashi, M; Kekelidze, I; Gegenava, Kh

2009-05-01

A systematic analysis of stapedoplasty output in otosclerosis cases was carried out. The operations were done during the period of 2005-2008 years at the Department of Otorhinolaryngology of the Tbilisi State Medical University. From the overall number of 107 patients, 78 were females and 29 males, 72.9% and 27.1%, respectively. The ages ranged from 16 to 57 years. The mean age was 35 years. The conductive and mixed forms of hearing losses were diagnosed in 70 and 37 cases, 65.4% and 34.6%, respectively. Right ear was operated in 46 patients, left ear in 57, and both ears in four, 43.3%, 53.0%, and 3.7%, respectively. 111 ears have been cured thus in sum. Intumescences of external ear tube were observed during the operation in 22 patients, 20.6%. In 7, 6.5%, the facial nerve was located downward. Particularly abnormal placement of the facial nerve was the case in two patients, 1.9%. The endaural approach has been proved to own advantages over the transmeatal one. The data generally confirmed that stapedotomy, as compared to stapedectomy, is a better choice for the surgery output. In beneficial cases the air/bone gaps after the operation closed totally or nearly totally. Such a proper outcome was reached in 93 out of 111 ears operated, 83.8%. In most of remainder ears the gaps after the operation narrowed significantly but far not completely. The definite surgery failure happened in one case only, 0.9%. To balance the preserved middle-ear problems in non-perfect surgery cases, the hearing aids of bone-conduction types have been recommended. In mixed otosclerosis cases, conversely, the aids of air-conduction types were advised to overcome the coexisted inner-ear pathologies.

Anoxia increases potassium conductance in hippocampal nerve cells.

PubMed

Hansen, A J; Hounsgaard, J; Jahnsen, H

1982-07-01

The effect of anoxia on nerve cell function was studied by intra- and extracellular microelectrode recordings from the CA1 and CA3 region in guinea pig hippocampal slices. Hyperpolarization and concomitant reduction of the nerve cell input resistance was observed early during anoxia. During this period the spontaneous activity first disappeared, then the evoked activity gradually disappeared. The hyperpolarization was followed by depolarization and an absence of a measurable input resistance. All the induced changes were reversed when the slice was reoxygenated. Reversal of the electro-chemical gradient for Cl- across the nerve cell membrane did not affect the course of events during anoxia. Aminopyridines blocked the anoxic hyperpolarization and attenuated the decrease of membrane resistance, but had no effect on the later depolarization. Blockers of synaptic transmission. Mn++, Mg++ and of Na+-channels (TTX) were without effect on the nerve cell changes during anoxia. It is suggested that the reduction of nerve cell excitability in anoxia is primarily due to increased K+-conductance. Thus, the nerve cells are hyperpolarized and the input resistance reduced, causing higher threshold and reduction of synaptic potentials. The mechanism of the K+-conductance activation is unknown at present.

Interleukin-1β overproduction is a common cause for neuropathic pain, memory deficit, and depression following peripheral nerve injury in rodents.

PubMed

Gui, Wen-Shan; Wei, Xiao; Mai, Chun-Lin; Murugan, Madhuvika; Wu, Long-Jun; Xin, Wen-Jun; Zhou, Li-Jun; Liu, Xian-Guo

2016-01-01

Chronic pain is often accompanied by short-term memory deficit and depression. Currently, it is believed that short-term memory deficit and depression are consequences of chronic pain. Here, we test the hypothesis that the symptoms might be caused by overproduction of interleukin-1beta (IL-1β) in the injured nerve independent of neuropathic pain following spared nerve injury in rats and mice. Mechanical allodynia, a behavioral sign of neuropathic pain, was not correlated with short-term memory deficit and depressive behavior in spared nerve injury rats. Spared nerve injury upregulated IL-1β in the injured sciatic nerve, plasma, and the regions in central nervous system closely associated with pain, memory and emotion, including spinal dorsal horn, hippocampus, prefrontal cortex, nucleus accumbens, and amygdala. Importantly, the spared nerve injury-induced memory deficits, depressive, and pain behaviors were substantially prevented by peri-sciatic administration of IL-1β neutralizing antibody in rats or deletion of IL-1 receptor type 1 in mice. Furthermore, the behavioral abnormalities induced by spared nerve injury were mimicked in naïve rats by repetitive intravenous injection of re combinant rat IL-1β (rrIL-1β) at a pathological concentration as determined from spared nerve injury rats. In addition, microglia were activated by both spared nerve injury and intravenous injection of rrIL-1β and the effect of spared nerve injury was substantially reversed by peri-sciatic administration of anti-IL-1β. Neuropathic pain was not necessary for the development of cognitive and emotional disorders, while the overproduction of IL-1β in the injured sciatic nerve following peripheral nerve injury may be a common mechanism underlying the generation of neuropathic pain, memory deficit, and depression. © The Author(s) 2016.

Preoperative computed tomography diagnosis of non-recurrent laryngeal nerve in patients with esophageal carcinoma.

PubMed

Niu, Zhong-Xi; Zhang, Hang; Chen, Long-Qi; Shi, Hui; Peng, Jun; Su, Li-Wei; Li, Wei; Xiao, Bo; He, Shu; Yue, Hong-Xu

2017-01-01

The non-recurrent laryngeal nerve (NRLN) is a rare but potentially serious anomaly that is commonly associated with the aberrant right subclavian artery (ARSA). It is easy to damage during surgical resection of esophageal cancer, leading to severe complications. Preoperative enhanced thoracic computed tomography (CT) scans of 2697 patients with esophageal carcinoma treated in our hospital between January 2010 and December 2013 were examined. We classified the positional relationship between the right subclavian artery and the membranous wall of the trachea into two types and used this method to predicate NRLN by identifying ARSA. Twenty-six patients (0.96%) were identified with ARSA, all of which were cases of NRLN by CT. NRLN was identified during surgery in the 26 patients, and a normal right recurrent laryngeal nerve was observed in 2671 patients. The ARSA was detected on the dorsal side of the membranous wall of the trachea in all 26 NRLN cases, while it was detected on the ventral side in all 2671 recurrent laryngeal nerve cases. Enhanced CT scanning is a reliable method for predicting NRLN by identifying ARSA. Preoperative recognition of this nerve anomaly allows surgeons to avoid damaging the nerve and abnormal vessels during esophagectomy. © 2016 The Authors. Thoracic Cancer published by China Lung Oncology Group and John Wiley & Sons Australia, Ltd.

Effects of Elevated Pax6 Expression and Genetic Background on Mouse Eye Development

PubMed Central

Chanas, Simon A.; Collinson, J. Martin; Ramaesh, Thaya; Dorà, Natalie; Kleinjan, Dirk A.; Hill, Robert E.; West, John D.

2009-01-01

Purpose To analyze the effects of Pax6 overexpression and its interaction with genetic background on eye development. Methods Histologic features of eyes from hemizygous PAX77+/− transgenic (high Pax6 gene dose) and wild-type mice were compared on different genetic backgrounds. Experimental PAX77+/−↔wild-type and control wild-type↔wild-type chimeras were analyzed to investigate the causes of abnormal eye development in PAX77+/− mice. Results PAX77+/− mice showed an overlapping but distinct spectrum of eye abnormalities to Pax6+/− heterozygotes (low Pax6 dose). Some previously reported PAX77+/− eye abnormalities did not occur on all three genetic backgrounds examined. Several types of eye abnormalities occurred in the experimental PAX77+/−↔wild-type chimeras, and they occurred more frequently in chimeras with higher contributions of PAX77+/− cells. Groups of RPE cells intruded into the optic nerve sheath, indicating that the boundary between the retina and optic nerve may be displaced. Both PAX77+/− and wild-type cells were involved in this ingression and in retinal folds, suggesting that neither effect was cell-autonomous. Cell-autonomous effects included failure of PAX77+/− and wild-type cells to mix normally and overrepresentation of PAX77+/− in the lens epithelium and RPE. Conclusions The extent of PAX77+/− eye abnormalities depended on PAX77+/− genotype, genetic background, and stochastic variation. Chimera analysis identified two types of cell-autonomous effects of the PAX77+/− genotype. Abnormal cell mixing between PAX77+/− and wild-type cells suggests altered expression of cell surface adhesion molecules. Some phenotypic differences between PAX77+/−↔wild-type and Pax6+/−↔wild-type chimeras may reflect differences in the levels of PAX77+/− and Pax6+/− contributions to chimeric lenses. PMID:19387074

End-to-side neurorraphy: a long-term study of neural regeneration in a rat model.

PubMed

Tarasidis, G; Watanabe, O; Mackinnon, S E; Strasberg, S R; Haughey, B H; Hunter, D A

1998-10-01

This study evaluated long-term reinnervation of an end-to-side neurorraphy and the resultant functional recovery in a rat model. The divided distal posterior tibial nerve was repaired to the side of an intact peroneal nerve. Control groups included a cut-and-repair of the posterior tibial nerve and an end-to-end repair of the peroneal nerve to the posterior tibial nerve. Evaluations included walking-track analysis, nerve conduction studies, muscle mass measurements, retrograde nerve tracing, and histologic evaluation. Walking tracks indicated poor recovery of posterior tibial nerve function in the experimental group. No significant difference in nerve conduction velocities was seen between the experimental and control groups. Gastrocnemius muscle mass measurements revealed no functional recovery in the experimental group. Similarly, retrograde nerve tracing revealed minimal motor neuron staining in the experimental group. However, some sensory staining was seen within the dorsal root ganglia of the end-to-side group. Histologic study revealed minimal myelinated axonal regeneration in the experimental group as compared with findings in the other groups. These results suggest that predominantly sensory regeneration occurs in an end-to-side neurorraphy at an end point of 6 months.

[Regional anatomy of the dorsal penile nerve and its clinical significance].

PubMed

Zhang, Chun-Ying; Li, Xing-Hua; Yuan, Tan; Zhang, Hai-Feng; Liu, Ji-Hong; Ye, Zhang-Qun

2009-02-01

To investigate the number, course and distribution of normal dorsal penile nerves and their clinical significance for selective neurectomy of the dorsal penile nerve in the treatment of primary premature ejaculation. We dissected 38 cadaveric adult penises and recorded the number, course and distribution of the dorsal penile nerves. A total of 314 cases of primary premature ejaculation underwent selective neurectomy of the dorsal penile nerve. The patients ranged between 20 and 45 years in age and from 1 to 22 years in disease course. The dorsal penile nerves were distributed in parallel bilaterally in all the cadaveric penises and branched into the ventral side in 4 of them. The total number of dorsal penile nerves was (3.6 +/- 1.2) in the 38 cadaveric penises, 7 in 1 case, 6 in 1 case, 5 in 6 cases, 4 in 9 cases, 3 in 14 cases and 2 in 7 cases, while that of the 314 patients with primary premature ejaculation was (7.0 +/- 1.9), 5 in 64 cases, 6 in 56 cases, 7 in 52 cases, 8 in 40 cases, 9 in 33 cases, 10 in 28 cases, 11 in 25 cases, 12 in 11 cases and 13 in 5 cases. Selective neurectomy of the dorsal penile nerve achieved an intravaginal ejaculation latency of (4.31 +/- 1.87) minutes and sexual satisfaction rate of (61 +/- 17) %, significantly different from those before the operation ([1.24 +/- 0.32] min, [23 +/- 6] %; all P < 0.01). The abnormal increase of dorsal penile nerves possibly lies at the bottom of the pathogenesis of primary premature ejaculation. Selective neurectomy of the dorsal penile nerve is safe and effective for the treatment of primary premature ejaculation.

ATG5 overexpression is neuroprotective and attenuates cytoskeletal and vesicle-trafficking alterations in axotomized motoneurons.

PubMed

Leiva-Rodríguez, Tatiana; Romeo-Guitart, David; Marmolejo-Martínez-Artesero, Sara; Herrando-Grabulosa, Mireia; Bosch, Assumpció; Forés, Joaquim; Casas, Caty

2018-05-24

Injured neurons should engage endogenous mechanisms of self-protection to limit neurodegeneration. Enhancing efficacy of these mechanisms or correcting dysfunctional pathways may be a successful strategy for inducing neuroprotection. Spinal motoneurons retrogradely degenerate after proximal axotomy due to mechanical detachment (avulsion) of the nerve roots, and this limits recovery of nervous system function in patients after this type of trauma. In a previously reported proteomic analysis, we demonstrated that autophagy is a key endogenous mechanism that may allow motoneuron survival and regeneration after distal axotomy and suture of the nerve. Herein, we show that autophagy flux is dysfunctional or blocked in degenerated motoneurons after root avulsion. We also found that there were abnormalities in anterograde/retrograde motor proteins, key secretory pathway factors, and lysosome function. Further, LAMP1 protein was missorted and underglycosylated as well as the proton pump v-ATPase. In vitro modeling revealed how sequential disruptions in these systems likely lead to neurodegeneration. In vivo, we observed that cytoskeletal alterations, induced by a single injection of nocodazole, were sufficient to promote neurodegeneration of avulsed motoneurons. Besides, only pre-treatment with rapamycin, but not post-treatment, neuroprotected after nerve root avulsion. In agreement, overexpressing ATG5 in injured motoneurons led to neuroprotection and attenuation of cytoskeletal and trafficking-related abnormalities. These discoveries serve as proof of concept for autophagy-target therapy to halting the progression of neurodegenerative processes.

Optic nerve size evaluated by magnetic resonance imaging in children with optic nerve hypoplasia, multiple pituitary hormone deficiency, isolated growth hormone deficiency, and idiopathic short stature.

PubMed

Birkebaek, Niels Holtum; Patel, Leena; Wright, Neville Bryce; Grigg, John Russell; Sinha, Smeeta; Hall, Catherine Margaret; Price, David Anthony; Lloyd, Ian Christopher; Clayton, Peter Ellis

2004-10-01

To objectively define criteria for intracranial optic nerve (ON) size in ON hypoplasia (ONH) on magnetic resonance imaging (MRI) scans. Intracranial ON sizes from MRI were compared between 46 children with ONH diagnosed by ophthalmoscopy (group 1, isolated ONH, 8 children; and group 2, ONH associated with abnormalities of the hypothalamic-pituitary axis and septum pellucidum, 38 children) and children with multiple pituitary hormone deficiency (group 3, multiple pituitary hormone deficiency, 14 children), isolated growth hormone deficiency (group 4, isolated growth hormone deficiency, 15 children), and idiopathic short stature (group 5, idiopathic short stature, 10 children). Intracranial ON size was determined by the cross-sectional area, calculated as [pi x (1/2) height x (1/2) width]. Groups 1 and 2 had lower intracranial ON size than did groups 3, 4, and 5 (P < .001). No patients in groups 3 through 5 who had MRI after 12 months of age (when 95% adult size of ONs is attained) had ONs <2.9 mm 2 . Visual acuity correlated significantly with ON size (P < .01). Magnetic resonance imaging of the ONs with cross-sectional area <2.9 mm 2 in a short child more than 12 months of age, with or without hypothalamic-pituitary axis abnormalities, confirms the clinical diagnosis of ONH.

Diffusion tensor imaging of the optic tracts in multiple sclerosis: association with retinal thinning and visual disability.

PubMed

Dasenbrock, Hormuzdiyar H; Smith, Seth A; Ozturk, Arzu; Farrell, Sheena K; Calabresi, Peter A; Reich, Daniel S

2011-04-01

Visual disability is common in multiple sclerosis, but its relationship to abnormalities of the optic tracts remains unknown. Because they are only rarely affected by lesions, the optic tracts may represent a good model for assessing the imaging properties of normal-appearing white matter in multiple sclerosis. Whole-brain diffusion tensor imaging was performed on 34 individuals with multiple sclerosis and 26 healthy volunteers. The optic tracts were reconstructed by tractography, and tract-specific diffusion indices were quantified. In the multiple-sclerosis group, peripapillary retinal nerve-fiber-layer thickness and total macular volume were measured by optical coherence tomography, and visual acuity at 100%, 2.5%, and 1.25% contrast was examined. After adjusting for age and sex, optic-tract mean and perpendicular diffusivity were higher (P=.002) in multiple sclerosis. Lower optic-tract fractional anisotropy was correlated with retinal nerve-fiber-layer thinning (r=.51, P=.003) and total-macular-volume reduction (r=.59, P=.002). However, optic-tract diffusion indices were not specifically correlated with visual acuity or with their counterparts in the optic radiation. Optic-tract diffusion abnormalities are associated with retinal damage, suggesting that both may be related to optic-nerve injury, but do not appear to contribute strongly to visual disability in multiple sclerosis. Copyright © 2010 by the American Society of Neuroimaging.

Diffusion Tensor Imaging of the Optic Tracts in Multiple Sclerosis: Association with Retinal Thinning and Visual Disability

PubMed Central

Dasenbrock, Hormuzdiyar H.; Smith, Seth A.; Ozturk, Arzu; Farrell, Sheena K.; Calabresi, Peter A.; Reich, Daniel S.

2009-01-01

Background and purpose Visual disability is common in multiple sclerosis, but its relationship to abnormalities of the optic tracts remains unknown. Because they are only rarely affected by lesions, the optic tracts may represent a good model for assessing the imaging properties of normal-appearing white matter in multiple sclerosis. Methods Whole-brain diffusion tensor imaging was performed on 34 individuals with multiple sclerosis and 26 healthy volunteers. The optic tracts were reconstructed by tractography, and tract-specific diffusion indices were quantified. In the multiple-sclerosis group, peripapillary retinal nerve-fiber-layer thickness and total macular volume were measured by optical coherence tomography, and visual acuity at 100%, 2.5%, and 1.25% contrast was examined. Results After adjusting for age and sex, optic-tract mean and perpendicular diffusivity were higher (p=0.002) in multiple sclerosis. Lower optic-tract fractional anisotropy was correlated with retinal nerve-fiber-layer thinning (r=0.51, p=0.003) and total-macular-volume reduction (r=0.59, p=0.002). However, optic-tract diffusion indices were not specifically correlated with visual acuity or with their counterparts in the optic radiation. Conclusions Optic-tract diffusion abnormalities are associated with retinal damage, suggesting that both may be related to optic-nerve injury, but do not appear to contribute strongly to visual disability in multiple sclerosis. PMID:20331501

Distal polyneuropathy in an adult Birman cat with toxoplasmosis.

PubMed

Mari, Lorenzo; Shelton, G Diane; De Risio, Luisa

2016-01-01

A 6-year-old female spayed Birman cat presented with a history of weight loss, stiff and short-strided gait in the pelvic limbs and reluctance to jump, progressing to non-ambulatory tetraparesis over 6 weeks. Poor body condition, dehydration and generalised muscle wastage were evident on general examination. Neurological examination revealed mildly depressed mental status, non-ambulatory flaccid tetraparesis and severely decreased proprioception and spinal reflexes in all four limbs. The neuroanatomical localisation was to the peripheral nervous system. Haematology, feline immunodeficiency virus/feline leukaemia virus serology, serum biochemistry, including creatine kinase and thyroxine, thoracic radiographs and abdominal ultrasound did not reveal significant abnormalities. Electromyography revealed fibrillation potentials and positive sharp waves in axial and appendicular muscles. Decreased motor conduction velocities and compound muscle action potential amplitudes were detected in ulnar and sciatic-tibial nerves. Residual latency was increased in the sciatic-tibial nerve. Histologically, several intramuscular nerve branches were depleted of myelinated fibres and a few showed mononuclear infiltrations. Toxoplasma gondii serology titres were compatible with active toxoplasmosis. Four days after treatment initiation with oral clindamycin the cat recovered the ability to walk. T gondii serology titres and neurological examination were normal after 11 and 16 weeks, respectively. Clindamycin was discontinued after 16 weeks. One year after presentation the cat showed mild relapse of clinical signs and seroconversion, which again resolved following treatment with clindamycin. To our knowledge, this is the first report of distal polyneuropathy associated with toxoplasmosis in a cat. This case suggests the inclusion of toxoplasmosis as a possible differential diagnosis for acquired polyneuropathies in cats.

The rat caudal nerves: a model for experimental neuropathies.

PubMed

Schaumburg, Herbert H; Zotova, Elena; Raine, Cedric S; Tar, Moses; Arezzo, Joseph

2010-06-01

This study provides a detailed investigation of the anatomy of the rat caudal nerve along its entire length, as well as correlated nerve conduction measures in both large and small diameter axons. It determines that rodent caudal nerves provide a simple, sensitive experimental model for evaluation of the pathophysiology of degeneration, recovery, and prevention of length-dependent distal axonopathy. After first defining the normal anatomy and electrophysiology of the rat caudal nerves, acrylamide monomer, a reliable axonal toxin, was administered at different doses for escalating time periods. Serial electrophysiological recordings were obtained, during intoxication, from multiple sites along caudal and distal sciatic nerves. Multiple sections of the caudal and sciatic nerves were examined with light and electron microscopy. The normal distribution of conduction velocities was determined and acrylamide-induced time- and dose-related slowing of velocities at the vulnerable ultraterminal region was documented. Degenerative morphological changes in the distal regions of the caudal nerves appeared well before changes in the distal sciatic nerves. Our study has shown that (1) rat caudal nerves have a complex neural structure that varies along a distal-to-proximal gradient and (2) correlative assessment of both morphology and electrophysiology of rat caudal nerves is easily achieved and provides a highly sensitive index of the onset and progression of the length-dependent distal axonopathy.

Use of Nerve Conduction Velocity to Assess Peripheral Nerve Health in Aging Mice

PubMed Central

Walsh, Michael E.; Sloane, Lauren B.; Fischer, Kathleen E.; Austad, Steven N.; Richardson, Arlan

2015-01-01

Nerve conduction velocity (NCV), the speed at which electrical signals propagate along peripheral nerves, is used in the clinic to evaluate nerve function in humans. A decline in peripheral nerve function is associated with a number of age-related pathologies. While several studies have shown that NCV declines with age in humans, there is little information on the effect of age on NCV in peripheral nerves in mice. In this study, we evaluated NCV in male and female C57Bl/6 mice ranging from 4 to 32 months of age. We observed a decline in NCV in both male and female mice after 20 months of age. Sex differences were detected in sensory NCV as well as the rate of decline during aging in motor nerves; female mice had slower sensory NCV and a slower age-related decline in motor nerves compared with male mice. We also tested the effect of dietary restriction on NCV in 30-month-old female mice. Dietary restriction prevented the age-related decline in sciatic NCV but not other nerves. Because NCV is clinically relevant to the assessment of nerve function, we recommend that NCV be used to evaluate healthspan in assessing genetic and pharmacological interventions that increase the life span of mice. PMID:25477428

Different role of TTX-sensitive voltage-gated sodium channel (NaV 1) subtypes in action potential initiation and conduction in vagal airway nociceptors.

PubMed

Kollarik, M; Sun, H; Herbstsomer, R A; Ru, F; Kocmalova, M; Meeker, S N; Undem, B J

2018-04-15

The action potential initiation in the nerve terminals and its subsequent conduction along the axons of afferent nerves are not necessarily dependent on the same voltage-gated sodium channel (Na V 1) subunits. The action potential initiation in jugular C-fibres within airway tissues is not blocked by TTX; nonetheless, conduction of action potentials along the vagal axons of these nerves is often dependent on TTX-sensitive channels. This is not the case for nodose airway Aδ-fibres and C-fibres, where both action potential initiation and conduction is abolished by TTX or selective Na V 1.7 blockers. The difference between the initiation of action potentials within the airways vs. conduction along the axons should be considered when developing Na V 1 blocking drugs for topical application to the respiratory tract. The action potential (AP) initiation in the nerve terminals and its subsequent AP conduction along the axons do not necessarily depend on the same subtypes of voltage-gated sodium channels (Na V 1s). We evaluated the role of TTX-sensitive and TTX-resistant Na V 1s in vagal afferent nociceptor nerves derived from jugular and nodose ganglia innervating the respiratory system. Single cell RT-PCR was performed on vagal afferent neurons retrogradely labelled from the guinea pig trachea. Almost all of the jugular neurons expressed the TTX-sensitive channel Na V 1.7 along with TTX-resistant Na V 1.8 and Na V 1.9. Tracheal nodose neurons also expressed Na V 1.7 but, less frequently, Na V 1.8 and Na V 1.9. Na V 1.6 were expressed in ∼40% of the jugular and 25% of nodose tracheal neurons. Other Na V 1 α subunits were only rarely expressed. Single fibre recordings were made from the vagal nodose and jugular nerve fibres innervating the trachea or lung in the isolated perfused vagally-innervated preparations that allowed for selective drug delivery to the nerve terminal compartment (AP initiation) or to the desheathed vagus nerve (AP conduction). AP initiation in jugular C-fibres was unaffected by TTX, although it was inhibited by Na V 1.8 blocker (PF-01247324) and abolished by combination of TTX and PF-01247324. However, AP conduction in the majority of jugular C-fibres was abolished by TTX. By contrast, both AP initiation and conduction in nodose nociceptors was abolished by TTX or selective Na V 1.7 blockers. Distinction between the effect of a drug with respect to inhibiting AP in the nerve terminals within the airways vs. at conduction sites along the vagus nerve is relevant to therapeutic strategies involving inhaled Na V 1 blocking drugs. © 2018 The Authors. The Journal of Physiology © 2018 The Physiological Society.

2,5-hexanedione (HD) treatment alters calmodulin, Ca{sup 2+}/calmodulin-dependent protein kinase II, and protein kinase C in rats' nerve tissues

DOE Office of Scientific and Technical Information (OSTI.GOV)

Wang Qingshan; Hou Liyan; National Research Institute for Family Planning, Beijing 100081

2008-10-01

Calcium-dependent mechanisms, particularly those mediated by Ca{sup 2+}/calmodulin (CaM)-dependent protein kinase II (CaMKII), have been implicated in neurotoxicant-induced neuropathy. However, it is unknown whether similar mechanisms exist in 2,5-hexanedione (HD)-induced neuropathy. For that, we investigated the changes of CaM, CaMKII, protein kinase C (PKC) and polymerization ratios (PRs) of NF-L, NF-M and NF-H in cerebral cortex (CC, including total cortex and some gray), spinal cord (SC) and sciatic nerve (SN) of rats treated with HD at a dosage of 1.75 or 3.50 mmol/kg for 8 weeks (five times per week). The results showed that CaM contents in CC, SC andmore » SN were significantly increased, which indicated elevation of Ca{sup 2+} concentrations in nerve tissues. CaMKII contents and activities were also increased in CC and were positively correlated with gait abnormality, but it could not be found in SC and SN. The increases of PKC contents and activities were also observed in SN and were positively correlated with gait abnormality. Except for that of NF-M in CC, the PRs of NF-L, NF-M and NF-H were also elevated in nerve tissues, which was consistent with the activation of protein kinases. The results suggested that CaMKII might be partly (in CC but not in SC and SN) involved in HD-induced neuropathy. CaMKII and PKC might mediate the HD neurotoxicity by altering the NF phosphorylation status and PRs.« less

Rapid diagnosis of retina and optic nerve abnormalities in canine patients with and without cataracts using chromatic pupil light reflex testing.

PubMed

Grozdanic, Sinisa D; Kecova, Helga; Lazic, Tatjana

2013-09-01

To develop fast and reliable testing routines for diagnosing retina and optic nerve diseases in canine cataract patients based on chromatic properties of the pupillary light reflex response. Seventy-seven canine patients with a history of cataract and decreased vision (43 patients with cataracts and no evidence of retina or optic nerve disease, 21 patients with cataracts and retinal degeneration [RD], 13 patients with cataracts and retinal detachment [RDT]), 11 canine patients with optic neuritis (ON) and 23 healthy dogs were examined using chromatic pupillary light reflex (cPLR) analysis with red and blue light and electroretinography. Electroretinography analysis showed statistically significant deficits in a- and b-wave amplitudes in dogs with cataracts and RD, or cataracts and RDT, when compared to dogs with cataracts without evidence of retinal abnormalities. Evaluation of b-wave amplitudes showed that presence of 78.5-μV (or lower) amplitudes had high sensitivity of 100% (95% CI: 87.2-100%) and high specificity of 96.7% (95% CI: 88.4-100%) in RD and RDT. Evaluation of cPLR responses using red light showed that presence of the pupil end constriction diameter of 5.5 mm (or higher) had moderately high sensitivity of 76.5% (95% CI: 50.1-93.2%) and high specificity of 100% (95% CI: 91.2-100%) in detecting RD and RDT. Optic neuritis patients had absent cPLR responses, regardless of the visual status. Chromatic evaluation of the pupillary light reflex is a rapid and accurate test for diagnosing retina and optic nerve diseases in canine patients. © 2012 American College of Veterinary Ophthalmologists.

Melanopsin retinal ganglion cell loss in Alzheimer disease

PubMed Central

Ross‐Cisneros, Fred N.; Koronyo, Yosef; Hannibal, Jens; Gallassi, Roberto; Cantalupo, Gaetano; Sambati, Luisa; Pan, Billy X.; Tozer, Kevin R.; Barboni, Piero; Provini, Federica; Avanzini, Pietro; Carbonelli, Michele; Pelosi, Annalisa; Chui, Helena; Liguori, Rocco; Baruzzi, Agostino; Koronyo‐Hamaoui, Maya; Sadun, Alfredo A.; Carelli, Valerio

2015-01-01

Objective Melanopsin retinal ganglion cells (mRGCs) are photoreceptors driving circadian photoentrainment, and circadian dysfunction characterizes Alzheimer disease (AD). We investigated mRGCs in AD, hypothesizing that they contribute to circadian dysfunction. Methods We assessed retinal nerve fiber layer (RNFL) thickness by optical coherence tomography (OCT) in 21 mild‐moderate AD patients, and in a subgroup of 16 we evaluated rest–activity circadian rhythm by actigraphy. We studied postmortem mRGCs by immunohistochemistry in retinas, and axons in optic nerve cross‐sections of 14 neuropathologically confirmed AD patients. We coimmunostained for retinal amyloid β (Aβ) deposition and melanopsin to locate mRGCs. All AD cohorts were compared with age‐matched controls. Results We demonstrated an age‐related optic neuropathy in AD by OCT, with a significant reduction of RNFL thickness (p = 0.038), more evident in the superior quadrant (p = 0.006). Axonal loss was confirmed in postmortem AD optic nerves. Abnormal circadian function characterized only a subgroup of AD patients. Sleep efficiency was significantly reduced in AD patients (p = 0.001). We also found a significant loss of mRGCs in postmortem AD retinal specimens (p = 0.003) across all ages and abnormal mRGC dendritic morphology and size (p = 0.003). In flat‐mounted AD retinas, Aβ accumulation was remarkably evident inside and around mRGCs. Interpretation We show variable degrees of rest–activity circadian dysfunction in AD patients. We also demonstrate age‐related loss of optic nerve axons and specifically mRGC loss and pathology in postmortem AD retinal specimens, associated with Aβ deposition. These results all support the concept that mRGC degeneration is a contributor to circadian rhythm dysfunction in AD. ANN NEUROL 2016;79:90–109 PMID:26505992

Diffusion tensor imaging for anatomical localization of cranial nerves and cranial nerve nuclei in pontine lesions: initial experiences with 3T-MRI.

PubMed

Ulrich, Nils H; Ahmadli, Uzeyir; Woernle, Christoph M; Alzarhani, Yahea A; Bertalanffy, Helmut; Kollias, Spyros S

2014-11-01

With continuous refinement of neurosurgical techniques and higher resolution in neuroimaging, the management of pontine lesions is constantly improving. Among pontine structures with vital functions that are at risk of being damaged by surgical manipulation, cranial nerves (CN) and cranial nerve nuclei (CNN) such as CN V, VI, and VII are critical. Pre-operative localization of the intrapontine course of CN and CNN should be beneficial for surgical outcomes. Our objective was to accurately localize CN and CNN in patients with intra-axial lesions in the pons using diffusion tensor imaging (DTI) and estimate its input in surgical planning for avoiding unintended loss of their function during surgery. DTI of the pons obtained pre-operatively on a 3Tesla MR scanner was analyzed prospectively for the accurate localization of CN and CNN V, VI and VII in seven patients with intra-axial lesions in the pons. Anatomical sections in the pons were used to estimate abnormalities on color-coded fractional anisotropy maps. Imaging abnormalities were correlated with CN symptoms before and after surgery. The course of CN and the area of CNN were identified using DTI pre- and post-operatively. Clinical associations between post-operative improvements and the corresponding CN area of the pons were demonstrated. Our results suggest that pre- and post-operative DTI allows identification of key anatomical structures in the pons and enables estimation of their involvement by pathology. It may predict clinical outcome and help us to better understand the involvement of the intrinsic anatomy by pathological processes. Copyright © 2014 Elsevier Ltd. All rights reserved.

Bell palsy in lyme disease-endemic regions of canada: a cautionary case of occult bilateral peripheral facial nerve palsy due to Lyme disease.

PubMed

Ho, Karen; Melanson, Michel; Desai, Jamsheed A

2012-09-01

Lyme disease caused by the spirochete Borrelia burgdorferi is a multisystem disorder characterized by three clinical stages: dermatologic, neurologic, and rheumatologic. The number of known Lyme disease-endemic areas in Canada is increasing as the range of the vector Ixodes scapularis expands into the eastern and central provinces. Southern Ontario, Nova Scotia, southern Manitoba, New Brunswick, and southern Quebec are now considered Lyme disease-endemic regions in Canada. The use of field surveillance to map risk and endemic regions suggests that these geographic areas are growing, in part due to the effects of climate warming. Peripheral facial nerve palsy is the most common neurologic abnormality in the second stage of Lyme borreliosis, with up to 25% of Bell palsy (idiopathic peripheral facial nerve palsy) occurring due to Lyme disease. Here we present a case of occult bilateral facial nerve palsy due to Lyme disease initially diagnosed as Bell palsy. In Lyme disease-endemic regions of Canada, patients presenting with unilateral or bilateral peripheral facial nerve palsy should be evaluated for Lyme disease with serologic testing to avoid misdiagnosis. Serologic testing should not delay initiation of appropriate treatment for presumed Bell palsy.

Potential Involvement of Draxin in the Axonal Projection of Cranial Nerves, Especially Cranial Nerve X, in the Chick Hindbrain.

PubMed

Zhang, Sanbing; Cui, Huixian; Wang, Lei; Kang, Lin; Huang, Guannan; Du, Juan; Li, Sha; Tanaka, Hideaki; Su, Yuhong

2016-07-01

The appropriate projection of axons within the nervous system is a crucial component of the establishment of neural circuitry. Draxin is a repulsive axon guidance protein. Draxin has important functions in the guidance of three commissures in the central nervous system and in the migration of neural crest cells and dI3 interneurons in the chick spinal cord. Here, we report that the distribution of the draxin protein and the location of 23C10-positive areas have a strong temporal and spatial correlation. The overexpression of draxin, especially transmembrane draxin, caused 23C10-positive axon bundles to misproject in the dorsal hindbrain. In addition, the overexpression of transmembrane draxin caused abnormal formation of the ganglion crest of the IX and X cranial nerves, misprojection of some anti-human natural killer-1 (HNK-1)-stained structures in the dorsal roof of the hindbrain, and a simultaneous reduction in the efferent nerves of some motoneuron axons inside the hindbrain. Our data reveal that draxin might be involved in the fascicular projection of cranial nerves in the hindbrain. © 2016 The Histochemical Society.

Arsenic in drinking water and peripheral nerve conduction velocity among residents of a chronically arsenic-affected area in Inner Mongolia.

PubMed

Fujino, Yoshihisa; Guo, Xiaojuan; Shirane, Kiyoyumi; Liu, Jun; Wu, Kegong; Miyatake, Munetoshi; Tanabe, Kimiko; Kusuda, Tetsuya; Yoshimura, Takesumi

2006-09-01

It remains unclear whether chronic ingestion of arsenic in drinking water affects the peripheral nervous system. We examined the effects of arsenic exposure on nerve conduction velocity using electromyography. A cross-sectional study was conducted of a population living in an arsenic-affected village in Hetao Plain, Inner Mongolia, China. A total of 134 (93.7%) of 143 inhabitants took part in the study, and 36 (76.6%) of 47 inhabitants in a low-arsenic exposed village were recruited as a control group. Of the participants, 109 inhabitants in the arsenic-affected village and 32 in the low-arsenic exposed village aged > or =18 years were used for the analyses. An expert physician performed skin examinations, and median nerve conduction velocity was examined by electromyography. Arsenic levels in tube-well water and urine were measured. A mean level of arsenic in tube-well water in the arsenic-affected village was 158.3 microg/L, while that in the low-arsenic exposed village was 5.3 microg/L. No significant differences in the means of the motor nerve conduction velocity (MCV) and sensory nerve conduction velocity (SCV) were observed in relation to arsenic levels in tube wells, urine, and the duration of tube-well use. Further, no differences in mean MCV or SCV were found between the subjects with and without arsenic dermatosis, with mean SCV of 52.8 m/s (SD 6.3) in those without and 54.6 m/s (5.2) in subjects with arsenic dermatosis (p=0.206). These findings suggest that chronic arsenic poisoning from drinking water is unlikely to affect nerve conduction velocity, at least within the range of arsenic in drinking water examined in the present study.

[Osteosynthesis in the Surgical Treatment of Prognathism: State of The Art].

PubMed

Durão, Nuno; Amarante, José

2017-03-31

Prognathism is a common skeletal facial abnormality, associated with class III malocclusion, often with repercussions in quality of life. In addition to orthodontic treatment, sagittal split ramus osteotomy is the most common technique for its correction, and segment osteosynthesis is an important element of the post-surgical outcome. A search for relevant literature was conducted in the PubMed/MEDLINE database and in other relevant sources. The stability of different fixation methods, their repercussions on inferior alveolar nerve lesions, and the type of material are among the most researched subjects. Recent research about the type of osteosynthesis applied in the sagittal split ramus osteotomy for mandibular setback is discussed. Miniplates appear to be the better option for fixation of sagittal split osteotomy for mandibular setback. Bioabsorbable osteosynthesis may be an acceptable alternative to titanium.

Morphological patterns in children with ganglion related enteric neuronal abnormalities.

PubMed

Henna, Nausheen; Nagi, Abdul H; Sheikh, Muhammad A; Shaukat, Mahmood

2011-01-01

Hirschsprung's Disease (HD) is a developmental disorder of enteric nervous system characterised by the absence of ganglion cells in submucosal (Meissner's) and myenteric (Aurbach's) plexuses of distal bowel. The purpose of the present study was to observe and report the morphological patterns of ganglion related enteric neuronal abnormalities in children presented with clinical features of (HD) in a Pakistani population. A total of 92 patients with clinical presentation of HD were enrolled between March 2009 and October 2009. Among them, 8 were excluded according to the exclusion criteria. After detailed history and physical examination, paraffin embedded H and E stained sections were prepared from the serial open biopsies from colorectum. The data was analysed using SPSS-17. Frequencies and percentages are given for qualitative variables. Non-parametric Binomial Chi-Square test was applied to observe within group associations and p<0.05 was considered statistically significant. Among 84 patients, 13 (15.5%) proved to be normally ganglionic whereas 71 (84.5%) showed ganglion related enteric neuronal abnormalities namely isolated hypoganglionosis 9 (12.7%), immaturity of ganglion cells 9 (12.7%), isolated hyperganglionosis (IND Type B) 2 (2.8%) and Hirschsprung's disease 51 (71.8%). Among HD group, 34 (66.7%) belonged to isolated form and 17 (33.3%) showed combined ganglion related abnormalities. Hirschsprung's disease is common in Pakistani population, followed by hypoganglionosis, immaturity of ganglion cells and IND type B. The presence of hypertrophic nerve fibres was significant in HD, hyperganglionosis and hypoganglionosis, whereas, no hypertrophic nerve fibres were appreciated in immaturity of ganglion cell group.

Construction of nerve guide conduits from cellulose/soy protein composite membranes combined with Schwann cells and pyrroloquinoline quinone for the repair of peripheral nerve defect

DOE Office of Scientific and Technical Information (OSTI.GOV)

Luo, Lihua; Center of Molecular Medicine, School of Medicine, Hubei University of Arts and Sciences, Xiangyang 441053; Gan, Li

Regeneration and functional reconstruction of peripheral nerve defects remained a significant clinical challenge. Nerve guide conduits, with seed cells or neurotrophic factors (NTFs), had been widely used to improve the repair and regeneration of injured peripheral nerve. Pyrroloquinoline quinone (PQQ) was an antioxidant that can stimulate nerve growth factors (NGFs) synthesis and accelerate the Schwann cells (SCs) proliferation and growth. In present study, three kinds of nerve guide conduits were constructed: one from cellulose/SPI hollow tube (CSC), another from CSC combined with SCs (CSSC), and the third one from CSSC combined with PQQ (CSSPC), respectively. And then they were appliedmore » to bridge and repair the sciatic nerve defect in rats, using autograft as control. Effects of different nerve guide conduits on the nerve regeneration were comparatively evaluated by general analysis, sciatic function index (SFI) and histological analysis (HE and TEM). Newly-formed regenerative nerve fibers were observed and running through the transparent nerve guide conduits 12 weeks after surgery. SFI results indicated that the reconstruction of motor function in CSSPC group was better than that in CSSC and CSC groups. HE images from the cross-sections and longitudinal-sections of the harvested regenerative nerve indicated that regenerative nerve fibers had been formed and accompanied with new blood vessels and matrix materials in the conduits. TEM images also showed that lots of fresh myelinated and non-myelinated nerve fibers had been formed. Parts of vacuolar, swollen and abnormal axons occurred in CSC and CSSC groups, while the vacuolization and swell of axons was the least serious in CSSPC group. These results indicated that CSSPC group had the most ability to repair and reconstruct the nerve structure and functions due to the comprehensive contributions from hollow CSC tube, SCs and PQQ. As a result, the CSSPC may have the potential for the applications as nerve guide conduits in the field of nerve tissue engineering. - Highlights: • A novel nerve conduit was constructed and applied to repair nerve defect in rats. • Transparent hollow cellulose/soy protein isolate tube was used as conduit matrix. • Pyrroloquinoline quinine was adsorbed into the hollow tube as nerve growth factor. • Schwann cells were cultured into the hollow tube as seed cells. • The new nerve conduit could repair and reconstruct the peripheral nerve defects.« less

Traditional Japanese medicines inhibit compound action potentials in the frog sciatic nerve.

PubMed

Matsushita, Akitomo; Fujita, Tsugumi; Ohtsubo, Sena; Kumamoto, Eiichi

2016-02-03

Traditional Japanese (Kampo) medicines have a variety of clinical effects including pain alleviation, but evidence for a mechanism for their pain relief has not yet been elucidated fully. Considering that Kampo medicine contains many plant-derived chemicals having an ability to inhibit nerve action potential conduction, it is possible that this medicine inhibits nerve conduction. The purpose of the present study was to know how various Kampo medicines affect nerve conduction. We examined the effects of Kampo and crude medicines on compound action potentials (CAPs) recorded from the frog sciatic nerve by using the air-gap method. Daikenchuto, rikkosan, kikyoto, rikkunshito, shakuyakukanzoto and kakkonto concentration-dependently reduced the peak amplitude of the CAP. Among the Kampo medicines, daikenchuto was the most effective in inhibiting CAPs. Daikenchuto is composed of three kinds of crude medicine, Japanese pepper, processed ginger and ginseng radix. When the crude medicines were tested, Japanese pepper and processed ginger reduced CAP peak amplitudes, while ginseng radix hardly affected CAPs. Moreover, there was an interaction between the Japanese pepper and processed ginger activities in such that one medicine at low but not high concentrations increased the extent of the inhibition by the other one that was co-applied. Kampo medicines have an ability to inhibit nerve conduction. This action of daikenchuto is due to Japanese pepper and processed ginger but not ginseng radix, probably through an interaction between Japanese pepper and processed ginger in a manner dependent on their concentrations. Nerve conduction inhibition could contribute to at least a part of Kampo medicine's clinical effects such as pain alleviation. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Reconstruction of the Abdominal Vagus Nerve Using Sural Nerve Grafts in Canine Models

PubMed Central

Luo, Fen; Wang, Zhiming; Wang, Yin

2013-01-01

Background Recently, vagus nerve preservation or reconstruction of vagus has received increasing attention. The present study aimed to investigate the feasibility of reconstructing the severed vagal trunk using an autologous sural nerve graft. Methods Ten adult Beagle dogs were randomly assigned to two groups of five, the nerve grafting group (TG) and the vagal resection group (VG). The gastric secretion and emptying functions in both groups were assessed using Hollander insulin and acetaminophen tests before surgery and three months after surgery. All dogs underwent laparotomy under general anesthesia. In TG group, latency and conduction velocity of the action potential in a vagal trunk were measured, and then nerves of 4 cm long were cut from the abdominal anterior and posterior vagal trunks. Two segments of autologous sural nerve were collected for performing end-to-end anastomoses with the cut ends of vagal trunk (8–0 nylon suture, 3 sutures for each anastomosis). Dogs in VG group only underwent partial resections of the anterior and posterior vagal trunks. Laparotomy was performed in dogs of TG group, and latency and conduction velocity of the action potential in their vagal trunks were measured. The grafted nerve segment was removed, and stained with anti-neurofilament protein and toluidine blue. Results Latency of the action potential in the vagal trunk was longer after surgery than before surgery in TG group, while the conduction velocity was lower after surgery. The gastric secretion and emptying functions were weaker after surgery in dogs of both groups, but in TG group they were significantly better than in VG group. Anti-neurofilament protein staining and toluidine blue staining showed there were nerve fibers crossing the anastomosis of the vagus and sural nerves in dogs of TG group. Conclusion Reconstruction of the vagus nerve using the sural nerve is technically feasible. PMID:23555604

CMT2C with vocal cord paresis associated with short stature and mutations in the TRPV4 gene

PubMed Central

Chen, D.-H.; Sul, Y.; Weiss, M.; Hillel, A.; Lipe, H.; Wolff, J.; Matsushita, M.; Raskind, W.; Bird, T.

2010-01-01

Background: Recently, mutations in the transient receptor potential cation channel, subfamily V, member 4 gene (TRPV4) have been reported in Charcot-Marie-Tooth Type 2C (CMT2C) with vocal cord paresis. Other mutations in this same gene have been described in separate families with various skeletal dysplasias. Further clarification is needed of the different phenotypes associated with this gene. Methods: We performed clinical evaluation, electrophysiology, and genetic analysis of the TRPV4 gene in 2 families with CMT2C. Results: Two multigenerational families had a motor greater than sensory axonal neuropathy associated with variable vocal cord paresis. The vocal cord paresis varied from absent to severe, requiring permanent tracheotomy in 2 subjects. One family with mild neuropathy also manifested pronounced short stature, more than 2 SD below the average height for white Americans. There was one instance of dolichocephaly. A novel S542Y mutation in the TRPV4 gene was identified in this family. The other family had a more severe, progressive, motor neuropathy with sensory loss, but less remarkable short stature and an R315W mutation in TRPV4. Third cranial nerve involvement and sleep apnea occurred in one subject in each family. Conclusion: CMT2C with axonal neuropathy, vocal cord paresis, and short stature is a unique syndrome associated with mutations in the TRPV4 gene. Mutations in TRPV4 can cause abnormalities in bone, peripheral nerve, or both and may result in highly variable orthopedic and neurologic phenotypes. GLOSSARY CMAP = compound muscle action potential; CMT = Charcot-Marie-Tooth; CMT2C = Charcot-Marie-Tooth Type 2C; HMSN = hereditary motor and sensory neuropathy; NCV = nerve conduction velocity; RFLP = restriction fragment length polymorphism; SMA = spinal muscular atrophy; SNAP = sensory nerve action potential; SPSMA = scapuloperoneal spinal muscular atrophy. PMID:21115951

A Biosynthetic Nerve Guide Conduit Based on Silk/SWNT/Fibronectin Nanocomposite for Peripheral Nerve Regeneration

PubMed Central

Mottaghitalab, Fatemeh; Farokhi, Mehdi; Zaminy, Arash; Kokabi, Mehrdad; Soleimani, Masoud; Mirahmadi, Fereshteh

2013-01-01

As a contribution to the functionality of nerve guide conduits (NGCs) in nerve tissue engineering, here we report a conduit processing technique through introduction and evaluation of topographical, physical and chemical cues. Porous structure of NGCs based on freeze-dried silk/single walled carbon nanotubes (SF/SWNTs) has shown a uniform chemical and physical structure with suitable electrical conductivity. Moreover, fibronectin (FN) containing nanofibers within the structure of SF/SWNT conduits produced through electrospinning process have shown aligned fashion with appropriate porosity and diameter. Moreover, fibronectin remained its bioactivity and influenced the adhesion and growth of U373 cell lines. The conduits were then implanted to 10 mm left sciatic nerve defects in rats. The histological assessment has shown that nerve regeneration has taken places in proximal region of implanted nerve after 5 weeks following surgery. Furthermore, nerve conduction velocities (NCV) and more myelinated axons were observed in SF/SWNT and SF/SWNT/FN groups after 5 weeks post implantation, indicating a functional recovery for the injured nerves. With immunohistochemistry, the higher S-100 expression of Schwann cells in SF/SWNT/FN conduits in comparison to other groups was confirmed. In conclusion, an oriented conduit of biocompatible SF/SWNT/FN has been fabricated with acceptable structure that is particularly applicable in nerve grafts. PMID:24098649

Sensory and motor peripheral nerve function and lower-extremity quadriceps strength: the health, aging and body composition study.

PubMed

Strotmeyer, Elsa S; de Rekeneire, Nathalie; Schwartz, Ann V; Resnick, Helaine E; Goodpaster, Bret H; Faulkner, Kimberly A; Shorr, Ronald I; Vinik, Aaron I; Harris, Tamara B; Newman, Anne B

2009-11-01

To determine whether sensory and motor nerve function is associated cross-sectionally with quadriceps or ankle dorsiflexion strength in an older community-based population. Cross-sectional analyses within a longitudinal cohort study. Two U.S. clinical sites. Two thousand fifty-nine Health, Aging and Body Composition Study (Health ABC) participants (49.5% male, 36.7% black, aged 73-82) in 2000/01. Quadriceps and ankle strength were measured using an isokinetic dynamometer. Sensory and motor peripheral nerve function in the legs and feet was assessed using 10-g and 1.4-g monofilaments, vibration threshold, and peroneal motor nerve conduction amplitude and velocity. Monofilament insensitivity, poorest vibration threshold quartile (>60 mu), and poorest motor nerve conduction amplitude quartile (<1.7 mV) were associated with 11%, 7%, and 8% lower quadriceps strength (all P<.01), respectively, than in the best peripheral nerve function categories in adjusted linear regression models. Monofilament insensitivity and lowest amplitude quartile were both associated with 17% lower ankle strength (P<.01). Multivariate analyses were adjusted for demographic characteristics, diabetes mellitus, body composition, lifestyle factors, and chronic health conditions and included all peripheral nerve measures in the same model. Monofilament insensitivity (beta=-7.19), vibration threshold (beta=-0.097), and motor nerve conduction amplitude (beta=2.01) each contributed independently to lower quadriceps strength (all P<.01). Monofilament insensitivity (beta=-5.29) and amplitude (beta=1.17) each contributed independently to lower ankle strength (all P<.01). Neither diabetes mellitus status nor lean mass explained the associations between peripheral nerve function and strength. Reduced sensory and motor peripheral nerve function is related to poorer lower extremity strength in older adults, suggesting a mechanism for the relationship with lower extremity disability.

ANOMALOUS HEAD POSTURES IN STRABISMUS AND NYSTAGMUS - DIAGNOSIS AND MANAGEMENT -

PubMed Central

Teodorescu, Luminita

2015-01-01

Abnormal head positions are adopted in order to improve visual acuity, to avoid diplopia or to obtain a more comfortable binocular vision. The head can be turned or tilted toward right or left, with the chin rotated up or downwards or combination of these positions. The ophthalmologic examination including the assessment of versions leads to the diagnosis. When versions are free, the cause may be congenital nystagmus or strabismus with large angle. When versions are limited we suspect paralytic or restrictive strabismus. The head tilted to one shoulder suggests cyclotropia (IV Nerve Palsy) or congenital nystagmus. We present few of the above cases. An adequate surgical treatment can improve or correct the ocular deviation, diplopia and the abnormal head posture. Conclusions: The abnormal head posture must be assessed and treated early in order to correct the ocular position and head posture. All patient presenting abnormal head position HAD TO BE investigated by an ophthalmologist. PMID:26978880

Somatic gain-of-function mutations in PIK3CA in patients with macrodactyly.

PubMed

Rios, Jonathan J; Paria, Nandina; Burns, Dennis K; Israel, Bonnie A; Cornelia, Reuel; Wise, Carol A; Ezaki, Marybeth

2013-02-01

Macrodactyly is a discrete congenital anomaly consisting of enlargement of all tissues localized to the terminal portions of a limb, typically within a 'nerve territory'. The classic terminology for this condition is 'lipofibromatous hamartoma of nerve' or Type I macrodactyly. The peripheral nerve, itself, is enlarged both in circumference and in length. It is not related to neurofibromatosis (NF1), nor is it associated with vascular malformations, such as in the recently reported CLOVES syndrome. The specific nerve pathophysiology in this form of macrodactyly has not been well described and a genetic etiology for this specific form of enlargement is unknown. To identify the genetic cause of macrodactyly, we used whole-exome sequencing to identify somatic mutations present in the affected nerve of a single patient. We confirmed a novel mutation in PIK3CA (R115P) present in the patient's affected nerve tissue but not in blood DNA. Sequencing PIK3CA exons identified gain-of-function mutations (E542K, H1047L or H1047R) in the affected tissue of five additional unrelated patients; mutations were absent in blood DNA available from three patients. Immunocytochemistry confirmed AKT activation in cultured cells from the nerve of a macrodactyly patient. Additionally, we found that the most abnormal structure within the involved nerve in a macrodactylous digit is the perineurium, with additional secondary effects on the axon number and size. Thus, isolated congenital macrodactyly is caused by somatic activation of the PI3K/AKT cell-signaling pathway and is genetically and biochemically related to other overgrowth syndromes.

Effect of chronic administration of sildenafil citrate (Viagra) on the histology of the retina and optic nerve of adult male rat.

PubMed

Eltony, Sohair A; Abdelhameed, Sally Y

2017-04-01

Abnormal vision has been reported by 3% of patients treated with sildenafil citrate (Viagra). Although many men use Viagra for an extended period for treatment of erectile dysfunction, the implications of the long term-daily use of it on the retina and optic nerve are unclear. To investigate the effect of chronic daily use of sildenafil citrate in a dose equivalent to men preferred therapeutic dose on the histology of the retina and optic nerve of adult male rat. Eighteen adult male Wistar rats were equally divided into three groups. Group I: control. Group II: treated with sildenafil citrate orally (10mg/kg/day) for 8 weeks. Group III (withdrawal): treated as group II and then left for 4 weeks without treatment. Specimens from the retina and optic nerve were processed for light and electron microscopy. In sildenafil citrate treated group, the retina and optic nerve revealed vacuolations and congested blood capillaries with apoptotic endothelial and pericytic cells, and thickened basal lamina. Caspase-3 (apoptotic marker) and CD31 (endothelial marker) expression increased. Glial cells revealed morphological changes: Müller cells lost their processes, activated microglia, astrocytic clasmatodendrosis, degenerated oligodendrocytes surrounded by disintegrated myelin sheathes of the optic nerve fibers. The retina and optic nerve of the withdrawal group revealed less vacuolations and congestion, and partial recovery of the glial cells. Chronic treatment with sildenafil citrate (Viagra) caused toxic effect on the structure of the retina and optic nerve of the rat. Partial recovery was observed after drug withdrawal. Copyright © 2017 Elsevier Ltd. All rights reserved.

Patients affected by endemic pemphigus foliaceus in Colombia, South America exhibit autoantibodies to optic nerve sheath envelope cell junctions.

PubMed

Abreu-Velez, Ana Maria; Gao, Wendy; Howard, Michael S

2018-01-01

The majority of the patients affected by a new variant of endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre EPF or pemphigus Abreu-Manu), have experienced vision problems; we have previously reported several ocular abnormalities. Here, we aimed to investigate reactivity to optic nerves in these patients. We utilized bovine, rat and mouse optic nerves, and performed immunofluorescence and confocal microscopy to test for optical nerve autoreactivity. We tested 45 patients affected by this disease and 45 controls from the endemic area matched by age, sex and work activity. Overall, 37 of the 45 patient sera reacted to the optic nerve envelope that is composed of leptomeninges; the reactivity was polyclonal and present mostly at the cell junctions (P < 0.001). The immune response was directed against optic nerve sheath cell junctions and the vessels inside it, as well as other molecules inside the nerve. No control cases were positive. Of interest, all the patient autoantibodies co-localized with commercial antibodies to desmoplakins I-II, myocardium-enriched zonula occludens-1- associated protein (MYZAP), armadillo repeat gene deleted in velo-cardio-facial syndrome (ARVCF), and plakophilin-4 (p0071) from Progen Biotechnik (P < 0.001). We conclude that the majority of the patients affected by pemphigus Abreu-Manu have autoantibodies to optic nerve sheath envelope cell junctions. These antibodies also co-localize with armadillo repeat gene deleted in velo-cardio-facial syndrome, p0071 and desmoplakins I-II. The clinical significance of our findings remains unknown.

First branchial cleft anomalies in children: Experience with 30 cases.

PubMed

Li, Wanpeng; Zhao, Liming; Xu, Hongming; Li, Xiaoyan

2017-07-01

First branchial cleft anomalies (FBCA) are rare in the clinical setting, as they account for 1 to 8% of all branchial abnormalities. The purpose of this study is to explore the relationship between the fistula tract and facial nerve and the surgical method of FBCA. This retrospective study included 30 cases of FBCA in children managed from 2009 to 2016. All patients underwent surgery to remove the tract of the FBCA. We reviewed the clinical data of the patients to obtain their demographics and management. Thirty patients (11 male and 19 female) with anomalies of FBCA were diagnosed. The ages ranged from 1 to 13 years (median, 3 years). Twenty cases had a close relationship with the parotid gland. The facial nerve was identified in 20 of the 30 patients. The tract ran deep to the facial nerve in 3 cases, superficial to it in 21 cases, and passed between the branches of the nerve in 6 cases. The facial nerve was not identified in ten patients, as the tract was superficial to it. There were 2 cases of postoperative temporary facial paralysis (2/30, 6.7%). The symptoms gradually improved after one month, 1 case had permanent facial paralysis (1/30, 3.3%), and 1 case had postoperative recurrence. Complete excision of the tract is the only way to manage FBCA, and the course of the tracts vary and have different relationships with the facial nerve. There are 3 types: Superficial, deep to the facial nerve, and between the branches of the nerve. Therefore, surgical approaches differ among the various types, and careful preoperative planning and protecting the facial nerve during resection of the tract are essential.

First branchial cleft anomalies in children: Experience with 30 cases

PubMed Central

Li, Wanpeng; Zhao, Liming; Xu, Hongming; Li, Xiaoyan

2017-01-01

First branchial cleft anomalies (FBCA) are rare in the clinical setting, as they account for 1 to 8% of all branchial abnormalities. The purpose of this study is to explore the relationship between the fistula tract and facial nerve and the surgical method of FBCA. This retrospective study included 30 cases of FBCA in children managed from 2009 to 2016. All patients underwent surgery to remove the tract of the FBCA. We reviewed the clinical data of the patients to obtain their demographics and management. Thirty patients (11 male and 19 female) with anomalies of FBCA were diagnosed. The ages ranged from 1 to 13 years (median, 3 years). Twenty cases had a close relationship with the parotid gland. The facial nerve was identified in 20 of the 30 patients. The tract ran deep to the facial nerve in 3 cases, superficial to it in 21 cases, and passed between the branches of the nerve in 6 cases. The facial nerve was not identified in ten patients, as the tract was superficial to it. There were 2 cases of postoperative temporary facial paralysis (2/30, 6.7%). The symptoms gradually improved after one month, 1 case had permanent facial paralysis (1/30, 3.3%), and 1 case had postoperative recurrence. Complete excision of the tract is the only way to manage FBCA, and the course of the tracts vary and have different relationships with the facial nerve. There are 3 types: Superficial, deep to the facial nerve, and between the branches of the nerve. Therefore, surgical approaches differ among the various types, and careful preoperative planning and protecting the facial nerve during resection of the tract are essential. PMID:28672934

Augmented superior rectus transposition procedure in Duane retraction syndrome compared with sixth nerve palsy.

PubMed

Akbari, Mohammadreza; Shomali, Setareh; Mirmohammadsadeghi, Arash; Fard, Masoud Aghsaei

2018-05-01

Superior rectus transposition (SRT) with medial rectus recession has been used for the treatment of sixth nerve palsy and esotropic Duane retraction syndrome (DRS). The purpose of this study was to compare the results of augmented SRT (with scleral fixation) without medial rectus recession in DRS and sixth nerve palsy. Patients with unilateral esotropic DRS (DRS group) and sixth nerve palsy were included in this prospective, comparative study and underwent SRT. Preoperative forced duction testing was negative or slightly positive in both groups. Prospective measurements were compared between the two groups. There were 11 patients in the DRS group and 11 patients in the sixth nerve palsy group. The mean preoperative esotropia decreased from 20.9 ± 6.0 prism diopter (PD) at far to 13.2 ± 5.8 PD in the DRS group (P = 0.003). The same measurement improved from 28.0 ± 8.5 PD to 8.4 ± 7.3 PD in the sixth nerve palsy group (P = 0.003). In the sixth nerve palsy group, the improvement in primary gaze esotropia and abnormal head posture was more than the DRS group (Both P < 0.001).The average dose effect for SRT was 7.8 ± 2.2 PD in the DRS group and 19.2 ± 4.6 PD in the sixth nerve palsy group. Although objective intorsion was significantly induced after SRT, subjective torsion was not significant after surgery in both groups. SRT appears to be more effective in improving primary gaze deviation and head posture in sixth nerve palsy compared with DRS. Subjective torsional and vertical diplopia were rare in both groups.

Peripheral nerves are pathologically small in cerebellar ataxia neuropathy vestibular areflexia syndrome: a controlled ultrasound study.

PubMed

Pelosi, L; Mulroy, E; Leadbetter, R; Kilfoyle, D; Chancellor, A M; Mossman, S; Wing, L; Wu, T Y; Roxburgh, R H

2018-04-01

Sensory neuronopathy is a cardinal feature of cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS). Having observed that two patients with CANVAS had small median and ulnar nerves on ultrasound, we set out to examine this finding systematically in a cohort of patients with CANVAS, and compare them with both healthy controls and a cohort of patients with axonal neuropathy. We have previously reported preliminary findings in seven of these patients with CANVAS and seven healthy controls. We compared the ultrasound cross-sectional area of median, ulnar, sural and tibial nerves of 14 patients with CANVAS with 14 healthy controls and 14 age- and gender-matched patients with acquired primarily axonal neuropathy. We also compared the individual nerve cross-sectional areas of patients with CANVAS and neuropathy with the reference values of our laboratory control population. The nerve cross-sectional area of patients with CANVAS was smaller than that of both the healthy controls and the neuropathy controls, with highly significant differences at most sites (P < 0.001). Conversely, the nerve cross-sectional areas in the upper limb were larger in neuropathy controls than healthy controls (P < 0.05). On individual analysis, the ultrasound abnormality was sufficiently characteristic to be detected in all but one patient with CANVAS. Small nerves in CANVAS probably reflect nerve thinning from loss of axons due to ganglion cell loss. This is distinct from the ultrasound findings in axonal neuropathy, in which nerve size was either normal or enlarged. Our findings indicate a diagnostic role for ultrasound in CANVAS sensory neuronopathy and in differentiating neuronopathy from neuropathy. © 2018 EAN.

Hemifacial Spasm: A Neurosurgical Perspective

PubMed Central

Kong, Doo-Sik

2007-01-01

Hemifacial spasm (HFS) is characterized by tonic clonic contractions of the muscles innervated by the ipsilateral facial nerve. Compression of the facial nerve by an ectatic vessel is widely recognized as the most common underlying etiology. HFS needs to be differentiated from other causes of facial spasms, such as facial tic, ocular myokymia, and blepharospasm. To understand the overall craniofacial abnormalities and to perform the optimal surgical procedures for HFS, we are to review the prevalence, pathophysiology, differential diagnosis, details of each treatment modality, usefulness of brainstem auditory evoked potentials monitoring, debates on the facial EMG, clinical course, and complications from the literature published from 1995 to the present time. PMID:19096569

Changes in Properties of Auditory Nerve Synapses following Conductive Hearing Loss.

PubMed

Zhuang, Xiaowen; Sun, Wei; Xu-Friedman, Matthew A

2017-01-11

Auditory activity plays an important role in the development of the auditory system. Decreased activity can result from conductive hearing loss (CHL) associated with otitis media, which may lead to long-term perceptual deficits. The effects of CHL have been mainly studied at later stages of the auditory pathway, but early stages remain less examined. However, changes in early stages could be important because they would affect how information about sounds is conveyed to higher-order areas for further processing and localization. We examined the effects of CHL at auditory nerve synapses onto bushy cells in the mouse anteroventral cochlear nucleus following occlusion of the ear canal. These synapses, called endbulbs of Held, normally show strong depression in voltage-clamp recordings in brain slices. After 1 week of CHL, endbulbs showed even greater depression, reflecting higher release probability. We observed no differences in quantal size between control and occluded mice. We confirmed these observations using mean-variance analysis and the integration method, which also revealed that the number of release sites decreased after occlusion. Consistent with this, synaptic puncta immunopositive for VGLUT1 decreased in area after occlusion. The level of depression and number of release sites both showed recovery after returning to normal conditions. Finally, bushy cells fired fewer action potentials in response to evoked synaptic activity after occlusion, likely because of increased depression and decreased input resistance. These effects appear to reflect a homeostatic, adaptive response of auditory nerve synapses to reduced activity. These effects may have important implications for perceptual changes following CHL. Normal hearing is important to everyday life, but abnormal auditory experience during development can lead to processing disorders. For example, otitis media reduces sound to the ear, which can cause long-lasting deficits in language skills and verbal production, but the location of the problem is unknown. Here, we show that occluding the ear causes synapses at the very first stage of the auditory pathway to modify their properties, by decreasing in size and increasing the likelihood of releasing neurotransmitter. This causes synapses to deplete faster, which reduces fidelity at central targets of the auditory nerve, which could affect perception. Temporary hearing loss could cause similar changes at later stages of the auditory pathway, which could contribute to disorders in behavior. Copyright © 2017 the authors 0270-6474/17/370323-10$15.00/0.

Deficiency of electroneutral K+-Cl- cotransporter 3 causes a disruption in impulse propagation along peripheral nerves.

PubMed

Sun, Yuan-Ting; Lin, Thy-Sheng; Tzeng, Shun-Fen; Delpire, Eric; Shen, Meng-Ru

2010-10-01

Nerve conduction requires the fine tuning of ionic currents through delicate interactions between axons and Schwann cells. The K(+)-Cl(-) cotransporter (KCC) family includes four isoforms (KCC1-4) that play an important role in the maintenance of cellular osmotic homeostasis via the coupled electroneutral movement of K(+) and Cl(-) with concurrent water flux. Mutation in SLC12A6 gene encoding KCC3 results in an autosomal recessive disease, known as agenesis of the corpus callosum associated with peripheral neuropathy. Nevertheless, the role of KCC3 in nerve function remains a puzzle. In this study, the microscopic examination of KCC isoforms expressed in peripheral nerves showed high expression of KCC2-4 in nodal segments of the axons and in the perinucleus and microvilli of Schwann cells. The KCC inhibitor [[(dihydroindenyl)oxy]alkanoic acid] but not the Na(+)-K(+)-2Cl(-)-cotransport inhibitor (bumetanide) dose-dependently suppressed the amplitude and area of compound muscle action potential, indicating the involvement of KCC activity in peripheral nerve conduction. Furthermore, the amplitude and area under the curve were smaller, and the nerve conduction velocity was slower in nerves from KCC3(-/-) mice than in nerves from wild-type mice, while the expression pattern of KCC2 and KCC4 was similar in KCC3 kockout and wild-type strains. KCC3(-/-) mice also manifested a prominent motor deficit in the beam-walking test. This is the first study to demonstrate that the K(+)-Cl(-) cotransporter activity of KCC3 contributes to the propagation of action potentials along peripheral nerves. (c) 2010 Wiley-Liss, Inc.

Vibration-induced multifocal neuropathy in forestry workers: electrophysiological findings in relation to vibration exposure and finger circulation.

PubMed

Bovenzi, M; Giannini, F; Rossi, S

2000-11-01

To investigate neural conduction in the upper limbs of symptomatic forestry workers with and without exposure to hand-transmitted vibration. A further aim was to assess the possible relationships between vibration exposure, nerve conduction and finger circulation in the forestry workers who used chain saws. A detailed neurophysiological investigation was performed on the upper extremities of 20 chain saw workers, 20 forestry operators with heavy manual work but without vibration exposure, and 20 healthy male controls. All subjects were screened to exclude polyneuropathy. Measurements of sensory and motor nerve conduction (velocity and amplitude) were obtained bilaterally from the median, ulnar and radial nerves. To assess peripheral vascular function, the forestry workers underwent a cold test with plethysmographic measurement of finger systolic blood pressure (FSBP). In the chain saw operators, vibration exposure was evaluated according to the International Standard ISO 5349. Indices of daily vibration exposure and lifetime cumulative vibration dose were estimated for each chain saw operator. Sensory nerve conduction in several segments of the median and radial nerves was significantly reduced in the chain saw operators compared with that in the workers doing heavy manual work and the controls. The neurophysiological pattern more frequently observed in the chain saw operators was a multifocal nerve conduction impairment to several neural segments with predominant involvement of sensory rather than motor fibres. Sensory nerve conduction velocities in the hands of the chain saw operators were inversely related to both daily and lifetime cumulative vibration exposures. In the vibration-exposed forestry workers, neither were sensori-motor complaints associated with vascular symptoms (finger whiteness) nor were electrophysiological data related to cold-induced changes in FSBP. Exposure to hand-transmitted vibration, in addition to ergonomic stress factors, can contribute to peripheral nerve disorders occurring in forestry workers who operate chain saws. The findings of this study suggest the existence of an exposure-effect relationship for vibration-induced neuropathy. Different underlying mechanisms are likely to be involved in the pathogenesis of the neurological and vascular components of the hand-arm vibration syndrome.

Cohort study on respiratory and neurological disorders among workers in a bone glue factory in Egypt.

PubMed

Al-Batanony, M A; Abdel-Rasoul, G M; Abu-Salem, M A; Al-Ahmar, I A; Al-Badry, A S

2012-04-01

Glues are strong, liquid adhesive derived from animal tissues. It has been shown that glue sniffing is associated with demyelinating polyneuropathy. The low molecular weight agents which cause occupational lung disease have generally included the isocyanates exposure to which could result in asthma among workers. Toluene is also used widely in glue and adhesive industry and households where toluene exposure and abuse can occur. To study some respiratory and neurological disorders that may arise in workers in a bone glue factory in Queisna industrial zone, Menoufyia governorate, Egypt. In a historical cohort study, the exposed participants (n = 50) were recruited from workers in a bone glue factory in Queisna industrial zone, Menoufyia governorate. The unexposed group was selected from workers' relatives who had never worked in glue industry. All participants completed a pre-designed questionnaire on personal and occupational histories. Pulmonary function tests as well as electromyography (EMG) were performed for all participants. Urinary hippuric acid was also measure in all participants. The prevalence of cough, asthmatic attacks and paresthesia were significantly higher among exposed than unexposed participants. Abnormal spirometric measurements (particularly towards obstruction), abnormal EMG and positive urinary hippuric acid were significantly more prevalent among exposed than unexposed group. Spirometry and EMG should be included in the periodic medical examination for exposed workers for early detection of respiratory and neurological disorders. Urinary hippuric acid could be a useful indicator of the nerve conduction abnormalities and should be measured periodically for these workers.

Takotsubo cardiomyopathy associated with Miller-Fisher syndrome.

PubMed

Gill, Dalvir; Liu, Kan

2017-07-01

51-year-old female who presented with progressive paresthesia, numbness of the lower extremities, double vision, and trouble walking. Physical exam was remarkable for areflexia, and ptosis. Her initial EKG showed nonspecific ST segment changes and her Troponin T was elevated to 0.41ng/mL which peaked at 0.66ng/mL. Echocardiogram showed a depressed left ventricular ejection fraction to 35% with severely hypokinetic anterior wall and left ventricular apex was severely hypokinetic. EMG nerve conduction study showed severely decreased conduction velocity and prolonged distal latency in all nerves consistent with demyelinating disease. She was treated with 5days of intravenous immunoglobulin therapy to which she showed significant improvement in strength in her lower extremities. Echocardiogram repeated 4days later showing an improved left ventricular ejection fraction of 55% and no left ventricular wall motion abnormalities. Takotsubo cardiomyopathy is a rare complication of Miller-Fisher syndrome and literature review did not reveal any cases. Miller-Fisher syndrome is an autoimmune process that affects the peripheral nervous system causing autonomic dysfunction which may involve the heart. Due to significant autonomic dysfunction in Miller-Fisher syndrome, it could lead to arrhythmias, blood pressure changes, acute coronary syndrome and myocarditis, Takotsubo cardiomyopathy can be difficult to distinguish. The treatment of Takotsubo cardiomyopathy is supportive with beta-blockers and angiotensin-converting enzyme inhibitors are recommended until left ventricle ejection fraction improvement. Takotsubo cardiomyopathy is a rare complication during the acute phase of Miller-Fisher syndrome and must be distinguished from autonomic dysfunction as both diagnoses have different approaches to treatment. Published by Elsevier Inc.

Transition metals and polyol pathway in the development of diabetic neuropathy in rats.

PubMed

Nakamura, Jiro; Hamada, Yoji; Chaya, Sadao; Nakashima, Eitaro; Naruse, Keiko; Kato, Koichi; Yasuda, Yutaka; Kamiya, Hideki; Sakakibara, Fumihiko; Koh, Naoki; Hotta, Nigishi

2002-01-01

The transition metal-catalyzed reaction is a major source of oxygen free radicals, which play an important role in vascular dysfunction leading to ischemia in diabetic tissues. The inhibition of polyol pathway hyperactivity has been reported to ameliorate neurovascular abnormalities in diabetic rats and has been proposed to improve the oxygen free radical scavenging capacity. The present study was conducted to compare the effect of a transition metal chelating agent, trientine (TRI), on diabetic neuropathy with that of an aldose reductase inhibitor, NZ-314 (NZ). Diabetic rats were divided into three groups: (1). untreated, (2). TRI-treated, and (3). NZ-treated. TRI (20 mg/kg) or NZ (100 mg/kg) was administered by gavage or chow containing NZ, respectively, for 8 weeks. Motor nerve conduction velocity (MNCV), coefficient of variation of the R - R interval on electrocardiogram (CVr-r), sciatic nerve blood flow (SNBF), platelet aggregation activities, and serum concentrations of malondialdehyde were measured. Untreated diabetic rats showed delayed MNCV, decreased CV(R-R), and reduced SNBF compared to normal rats. TRI or NZ completely prevented these deficits. Platelet hyperaggregation activities in diabetic rats were prevented by NZ, but not by TRI. Increased concentrations of malondialdehyde in diabetic rats were partially but significantly ameliorated by either TRI or NZ. These observations suggest that increased free radical formation through the transition metal-catalyzed reaction plays an important role in the development of diabetic neuropathy and that the preventive effect of an aldose reductase inhibitor on diabetic neuropathy may also be mediated by decreasing oxygen free radicals. Copyright 2002 John Wiley & Sons, Ltd.

Visual impairment in children with congenital Zika syndrome.

PubMed

Ventura, Liana O; Ventura, Camila V; Lawrence, Linda; van der Linden, Vanessa; van der Linden, Ana; Gois, Adriana L; Cavalcanti, Milena M; Barros, Eveline A; Dias, Natalia C; Berrocal, Audina M; Miller, Marilyn T

2017-08-01

To describe the visual impairment associated with ocular and neurological abnormalities in a cohort of children with congenital Zika syndrome (CZS). This cross-sectional study included infants with microcephaly born in Pernambuco, Brazil, from May to December 2015. Immunoglobulin M antibody capture enzyme-linked immunosorbent assay for the Zika virus on the cerebrospinal fluid samples was positive for all infants. Clinical evaluation consisted of comprehensive ophthalmologic examination including visual acuity, visual function assessment, visual developmental milestone, neurologic examination, and neuroimaging. A total of 32 infants (18 males [56%]) were included. Mean age at examination was 5.7 ± 0.9 months (range, 4-7 months). Visual function and visual developmental milestone could not be tested in 1 child (3%). Visual impairment was detected in 32 infants (100%). Retinal and/or optic nerve findings were observed in 14 patients (44%). There was no statistical difference between the patients with ocular findings and those without (P = 0.180). All patients (100%) demonstrated neurological and neuroimaging abnormalities; 3 (9%) presented with late-onset of microcephaly. Children with CZS demonstrated visual impairment regardless of retina and/or optic nerve abnormalities. This finding suggests that cortical/cerebral visual impairment may be the most common cause of blindness identified in children with CZS. Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

[Investigation of maximal motor nerve conductivity and distal latency before and after galvanic cell bath (author's transl)].

PubMed

Trnavsky, G

1982-04-30

Measurements about maximal motor nerve conductivity of ulnaris and medianus were carried out before and after constant galvanisation from neck to hand. Significant results of conductivity, distal latency and amplitude of summation potential could not be registered neither by plus nor by minus pole at the hand.

Ocular toxocariasis presenting as bilateral scleritis with suspect retinal granuloma in the nerve fiber layer: a case report.

PubMed

Pak, Kang Yeun; Park, Sung Who; Byon, Ik Soo; Lee, Ji Eun

2016-08-18

This report details ocular toxocariasis presenting as bilateral scleritis with suspect retinal granuloma in the nerve fiber layer. The patient presented with scleritis, which did not improve with systemic steroid. Intraocular pressure was elevated, and well demarcated hyper-reflective round lesion were noted in both eyes. He had a history of general ache and concurrent onset of ocular symptoms the day after eating raw meat. Systemic work-ups revealed no remarkable abnormalities except antibody for toxocara. Oral albendazole and steroid were prescribed. The inflammation and swellings resolved without recurrence. In the current case, scleritis with suspect granuloma in the nerve fiber layer seems to be caused by toxocara. Ocular toxocariasis can be presented as atypical features. Serologic exams for toxocariasis would be considered not only in typical features but also in other uveitis or scleritis, particularly when the patient has a related history.

Prolonged phone-call posture causes changes of ulnar motor nerve conduction across elbow.

PubMed

Padua, Luca; Coraci, Daniele; Erra, Carmen; Doneddu, Pietro Emiliano; Granata, Giuseppe; Rossini, Paolo Maria

2016-08-01

Postures and work-hobby activities may play a role in the origin and progression of ulnar neuropathy at the elbow (UNE), whose occurrence appears to be increasing. The time spent on mobile-phone has increased in the last decades leading to an increased time spent with flexed elbow (prolonged-phone-posture, PPP). We aimed to assess the effect of PPP both in patients with symptoms of UNE and in symptom-free subjects. Patients with pure sensory symptoms of UNE and negative neurophysiological tests (MIN-UNE) and symptom-free subjects were enrolled. We evaluated ulnar motor nerve conduction velocity across elbow at baseline and after 6, 9, 12, 15, and 18min of PPP in both groups. Fifty-six symptom-free subjects and fifty-eight patients were enrolled. Globally 186 ulnar nerves from 114 subjects were studied. Conduction velocity of ulnar nerve across the elbow significantly changed over PPP time in patients with MIN-UNE, showing a different evolution between the two groups. PPP causes a modification of ulnar nerve functionality in patients with MIN-UNE. PPP may cause transient stress of ulnar nerve at elbow. Copyright © 2016 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

The Neuromotor Examination of the Preschool Child and Its Prognostic Significance

ERIC Educational Resources Information Center

Hadders-Algra, Mijna

2005-01-01

The present paper reviews the methods available for neurological or neuromotor evaluation at preschool age. General textbooks on pediatric neurology describe the neurological examination at preschool age in terms of the assessment of the evaluation of cranial nerves, muscle tone, muscle power, reflexes, and the presence of abnormal movements. They…

Assessment of diabetic neuropathy with emission tomography and magnetic resonance spectroscopy.

PubMed

Rao, Harshvardhan; Gaur, Neeraj; Tipre, Dnyanesh

2017-04-01

Diabetic neuropathies (DNs) are nerve-damaging disorders associated with diabetes. They are commonly attributed to peripheral nerves and primarily affect the limbs of the patient. They cause altered sensitivity to external stimuli along with loss in balance and reflexes of the affected patient. DNs are associated with a variety of clinical manifestations including autonomic failure and are caused by poor management of blood sugar levels. Imaging modalities provide vital information about early physiological changes in DNs. This review summarizes contributions by various teams of scientists in developing imaging methods to assess physiological changes in DNs and ongoing clinical trials where imaging modalities are applied to evaluate therapeutic intervention in DNs. Development of PET, single photon emission computed tomography, and magnetic resonance spectroscopy methods over the past 20 years are reviewed in the diagnostic assessment of DNs. Abnormal radiotracer pharmacokinetics and neurometabolite spectra in affected organs confirm physiological abnormalities in DN. With the use of the Siemens Biograph mMR and GE Signa - 60 cm (PET/MRI scanner), simultaneous acquisition of physiological and anatomical information could enhance understanding of DNs and accelerate drug development.

Papilloedema due to Chiari I malformation

PubMed Central

Zhang, Jason Chao; Bakir, Belal; Lee, Andrew; Yalamanchili, Sushma S

2011-01-01

The Chiari I malformation is a congenital abnormality characterised by downward displacement of the cerebellar tonsils through the foramen magnum into the cervical spine. It presents clinically most often in young adult women. Known ocular manifestations linked to Chiari I consist primarily of oculomotor paresis with cranial nerve VI palsy and convergence/divergence abnormalities. Papilloedema is a rare manifestation of Chiari I with a clinical presentation often similar to that of idiopathic intracranial hypertension. To highlight this unusual complication, the authors report a 64-year-old female who developed papilloedema as the only presenting neurological symptom resulting from a Chiari I malformation. PMID:22675036

Short Lingual Osteotomy Using a Piezosurgery Ultrasonic Bone-Cutting Device During Sagittal Split Ramus Osteotomy.

PubMed

Kawase-Koga, Yoko; Mori, Yoshiyuki; Kanno, Yuki; Hoshi, Kazuto; Takato, Tsuyoshi

2015-10-01

Short lingual osteotomy is a useful method for the performance of sagittal split ramus osteotomy involving interference between the proximal and distal bone fragments when lateral differences exist in the setback distance. However, this procedure occasionally results in abnormal fracture and nerve injury; expert surgical skill is thus required. We herein describe a novel technique involving the use of an ultrasonic bone-cutting device (Piezosurgery; Mectron Medical Technology, Carasco, Italy) for vertical osteotomy posterior to the mandibular foramen. Successful short lingual osteotomy was performed using this technique with avoidance of abnormal fracture and neurovascular bundle damage.

Early identification of 'acute-onset' chronic inflammatory demyelinating polyneuropathy.

PubMed

Sung, Jia-Ying; Tani, Jowy; Park, Susanna B; Kiernan, Matthew C; Lin, Cindy Shin-Yi

2014-08-01

Distinguishing patients with acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy prior to relapse is often challenging at the onset of their clinical presentation. In the present study, nerve excitability tests were used in conjunction with the clinical phenotype and disease staging, to differentiate between patients with acute-onset chronic inflammatory demyelinating polyneuropathy and patients with acute inflammatory demyelinating polyneuropathy at an early stage, with the aim to better guide treatment. Clinical assessment, staging and nerve excitability tests were undertaken on patients initially fulfilling the diagnostic criteria of acute inflammatory demyelinating polyneuropathy soon after symptom onset and their initial presentation. Patients were subsequently followed up for minimum of 12 months to determine if their clinical presentations were more consistent with acute-onset chronic inflammatory demyelinating polyneuropathy. Clinical severity as evaluated by Medical Research Council sum score and Hughes functional grading scale were not significantly different between the two cohorts. There was no difference between the time of onset of initial symptoms and nerve excitability test assessment between the two cohorts nor were there significant differences in conventional nerve conduction study parameters. However, nerve excitability test profiles obtained from patients with acute inflammatory demyelinating polyneuropathy demonstrated abnormalities in the recovery cycle of excitability, including significantly reduced superexcitability (P < 0.001) and prolonged relative refractory period (P < 0.01), without changes in threshold electrotonus. In contrast, in patients with acute-onset chronic inflammatory demyelinating polyneuropathy, a different pattern occurred with the recovery cycle shifted downward (increased superexcitability, P < 0.05; decreased subexcitability, P < 0.05) and increased threshold change in threshold electrotonus in both hyperpolarizing and depolarizing directions [depolarizing threshold electrotonus (90-100 ms) P < 0.005, hyperpolarizing threshold electrotonus (10-20 ms), P < 0.01, hyperpolarizing threshold electrotonus (90-100 ms), P < 0.05], perhaps suggesting early hyperpolarization. In addition, using excitability parameters superexcitability, subexcitability and hyperpolarizing threshold electrotonus (10-20 ms), the patients with acute inflammatory demyelinating polyneuropathy and acute-onset chronic inflammatory demyelinating polyneuropathy could be clearly separated into two non-overlapping groups. Studies of nerve excitability may be able to differentiate acute from acute-onset chronic inflammatory demyelinating polyneuropathy at an early stage. Characteristic nerve excitability parameter changes occur in early acute-onset chronic inflammatory demyelinating polyneuropathy, to match the clinical phenotype. Importantly, this pattern of change was strikingly different to that shown by patients with acute inflammatory demyelinating polyneuropathy, suggesting that nerve excitability techniques may be useful in distinguishing acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy at the initial stage. © The Author (2014). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Improving Structural and Functional Agreement in Patients with Glaucoma by Using Customized Perimetric Locations and Images of the Retinal Nerve Fiber Bundles

NASA Astrophysics Data System (ADS)

Alluwimi, Muhammed Saad

Glaucoma is the second leading cause of the blindness worldwide. It is a group of chronic, progressive, and potentially blinding optic neuropathies characterized by abnormalities of the optic nerve head and/or retinal nerve fiber layer (RNFL) associated with visual field abnormality. When diagnosing and managing patients with glaucoma, clinicians evaluate the agreement between structural and functional measures. However, it has been widely recognized that there is often a discordance between structural and functional (e.g., perimetry) measures in glaucoma, posing a challenge for clinicians to make their decisions. As explained in the literature, this discordance may relate to high normal between-subject variation, insufficient knowledge of the RNFL bundle organization, sparse spacing of the perimetric locations used to measure the functional performance of ganglion cells, high test-retest variation for the most commonly used stimulus for perimetry, and poor perimetric sampling of the macula. The aim of this thesis was to overcome this discordance by conducting three experiments: First, asymmetry analysis was used to reduce between-subject variation of the macular thickness and ganglion cell thickness measurements with OCT. This variation was decreased at particular regions of the macula. Outside the macula, the variation remained high leading to the second experiment in which customized closely-spaced perimetric locations were presented at wedge defects, guided by the OCT en face images of the RNFL bundles. A rapid suprathreshold perimetric strategy was used and perimetric defect was, in most cases, in correspondence with the structural defect. To threshold perimetric defects, an elongated blur-resistant stimulus was oriented within damaged RNFL bundles. It was found that contrast sensitivities were below the 95% normal limit in 37 of 44 locations. The latter experiment focused on wedge defects outside the macula, which led to the third experiment in which the goal was to investigate the feasibility of a basis to individualize perimetric locations within the macula guided by structural damage seen on OCT en face images. In preliminary data, it was feasible to individualize perimetric locations within the macula. In this thesis, the agreement between structural and functional measures was improved in glaucoma, by developing methods and techniques that provided a framework to help overcome challenges in clinical decision making.

Peripheral neuropathy in the twitcher mouse: accumulation of extracellular matrix in the endoneurium and aberrant expression of ion channels.

PubMed

Kagitani-Shimono, Kuriko; Mohri, Ikuko; Yagi, Takashi; Taniike, Masako; Suzuki, Kinuko

2008-05-01

Globoid cell leukodystrophy (GLD; Krabbe's disease), caused by a genetic galactosylceramidase deficiency, affects both the central and peripheral nervous systems (CNS and PNS). Allogenic hematopoietic stem-cell transplantation (HSCT) has been beneficial for clinical improvement of this disease. However, recent reports by Siddiqi et al. suggested that none of their transplanted patients achieved complete normalization of their peripheral nerve function, despite the well-documented remyelination of the CNS and PNS in the treated patients. We hypothesized that the PNS dysfunction in GLD is due to altered Schwann cell-axon interactions, resulting in structural abnormalities of the node of Ranvier and aberrant expression of ion channels caused by demyelination and that the persistence of this altered interaction is responsible for the dysfunction of the PNS after HSCT. Since there has not been any investigation of the Schwann cell-axonal relationship in twitcher mice, an authentic model of GLD, we first investigated structural abnormalities, focusing on the node of Ranvier in untreated twitcher mice, and compared the results with those obtained after receiving bone marrow transplantation (BMT). As expected, we found numerous supernumerary Schwann cells that formed structurally abnormal nodes of Ranvier. Similar findings, though at somewhat variable extent, were detected in mice treated with BMT. Activated supernumerary Schwann cells expressed GFAP immunoreactivity and generated Alcian blue-positive extracellular matrix (ECM) in the endoneurial space. The processes of these supernumerary Schwann cells often covered and obliterated the nodal regions. Furthermore, the distribution of Na(+) channel immunoreactivity was diffuse without the concentration at the nodes of Ranvier as seen in wild-type mice. Neither K(+) channels nor Neurexin IV/ Caspr/ Paranoidin (NCP-1) were detected in the twi/twi sciatic nerve. The results of our study suggest the importance of normalization of the Schwann cell-axon relationship for the functional recovery of peripheral nerves, when one considers therapeutic strategies for PNS pathology in GLD.

Paraparetic Guillain-Barré syndrome: Nondemyelinating reversible conduction failure restricted to the lower limbs.

PubMed

Kimachi, Takeshi; Yuki, Nobuhiro; Kokubun, Norito; Yamaguchi, Shuhei; Wakerley, Benjamin R

2017-02-01

Paraparetic Guillain-Barré syndrome (GBS) is a rare subtype of GBS characterized by leg weakness and areflexia in the absence of neurological involvement of the arms, cranial nerves, or respiratory muscles. Onset is characterized by lower back, buttock, or leg pain, followed by development of symmetric flaccid limb weakness in the absence of sensory disturbance. We describe an elderly woman who developed postinfectious symmetric flaccid leg weakness in the absence of sensory disturbance. Serial nerve conduction studies were carried out over 5 months. Antecedent infection, a monophasic disease course, and the presence of cerebrospinal fluid albuminocytological dissociation suggested a diagnosis of paraparetic GBS. Serial nerve conduction studies demonstrated nondemyelinating reversible conduction failure, which was restricted to the legs. Axonal neuropathy was supported by the presence of anti-GM1 IgG antibodies. These findings suggest that patients with paraparetic GBS have axonal neuropathy, which is restricted to the lower limbs. Muscle Nerve 55: 281-285, 2017. © 2016 Wiley Periodicals, Inc.

Intraoperative cranial nerve monitoring.

PubMed

Harper, C Michel

2004-03-01

The purpose of intraoperative monitoring is to preserve function and prevent injury to the nervous system at a time when clinical examination is not possible. Cranial nerves are delicate structures and are susceptible to damage by mechanical trauma or ischemia during intracranial and extracranial surgery. A number of reliable electrodiagnostic techniques, including nerve conduction studies, electromyography, and the recording of evoked potentials have been adapted to the study of cranial nerve function during surgery. A growing body of evidence supports the utility of intraoperative monitoring of cranial nerve nerves during selected surgical procedures.

Ophthalmic Manifestations of Congenital Zika Syndrome in Colombia and Venezuela.

PubMed

Yepez, Juan B; Murati, Felipe A; Pettito, Michele; Peñaranda, Carlos F; de Yepez, Jazmin; Maestre, Gladys; Arevalo, J Fernando

2017-05-01

The ocular manifestations and sequelae of Zika virus infection are not well known. Recently, the World Health Organization changed the declaration of Zika as a public health emergency and designated the viral outbreak and related microcephaly clusters as a long-term program of work. This change indicates the urgent need to evaluate and document ophthalmic manifestations in patients for timely management of this disease. In addition, confirmation whether the public health problem in Brazil extends to other regions in South America is needed. To report the ocular manifestations of congenital Zika syndrome with microcephaly in Colombia and Venezuela. This prospective case series included 43 patients from 2 ophthalmic centers in Colombia and Venezuela who underwent evaluation from October 1, 2015, through June 30, 2016, and were clinically diagnosed with congenital Zika syndrome. Twenty patients were Hispanic; 13, African; 8, white; and 2, Native American. Ophthalmic and systemic evaluations and serologic testing were performed on all infants. Patients underwent external ocular examination and dilated ophthalmoscopy. Serologic testing ruled out toxoplasmosis, rubella, cytomegalovirus, syphilis, and human immunodeficiency virus. Ophthalmic manifestations of congenital Zika syndrome. Of the 43 patients included in this series (28 female and 15 male), the mean (SD) age at examination was 2.1 (1.5) months. The mothers of all the children had no ophthalmic findings and did not report ocular symptoms during pregnancy. All patients had bilateral ophthalmic manifestations. Optic nerve findings included hypoplasia with the double-ring sign, pallor, and increased cup-disc ratio in 5 patients (11.6%). Macular abnormalities included mild to severe pigment mottling in 27 patients (63%) and lacunar maculopathy in 3 (6.9%). Chorioretinal scarring was present in 3 patients (7%). Eleven patients (26%) had a combination of lesions in the posterior pole. Five patients (12%) were diagnosed with congenital glaucoma, characterized by the clinical triad of epiphora, photophobia, and blepharospasm; increased intraocular pressure; corneal clouding at birth; and buphthalmos. These data reveal that 12% (95% CI, 5%-24%) of cases of congenital Zika with microcephaly had anterior segment abnormalities and 88% (95% CI, 76%-94%) had important macular and optic nerve abnormalities. The visual sequelae of these ophthalmic manifestations remain unknown. Congenital Zika syndrome in the current study had severe ocular abnormalities, and all patients had bilateral involvement. Ocular findings were focal macular pigment mottling, chorioretinal atrophy with a predilection for the macular area, congenital glaucoma and optical nerve hypoplasia, and optic disc abnormalities. Ophthalmic examination is recommended in patients with congenital Zika syndrome.

Ocular abnormalities in congenital Zika syndrome: are the ophthalmoscopic findings "the top of the iceberg"?

PubMed

de Oliveira Dias, João Rafael; Ventura, Camila V; de Paula Freitas, Bruno; Prazeres, Juliana; Ventura, Liana O; Bravo-Filho, Vasco; Aleman, Tomas; Ko, Albert Icksang; Zin, Andréa; Belfort, Rubens; Maia, Mauricio

2018-04-23

Zika virus (ZIKV) is an arbovirus mainly transmitted to humans by mosquitoes from Aedes genus. Other ways of transmission include the perinatal and sexual routes, blood transfusion, and laboratory exposure. Although the first human cases were registered in 1952 in African countries, outbreaks were only reported since 2007, when entire Pacific islands were affected. In March 2015, the first cases of ZIKV acute infection were notified in Brazil and, to date, 48 countries and territories in the Americas have confirmed local mosquito-borne transmission of ZIKV. Until 2015, ZIKV infection was thought to only cause asymptomatic or mild exanthematous febrile infections. However, after explosive ZIKV outbreaks in Polynesia and Latin American countries, it was confirmed that ZIKV could also lead to Guillain-Barré syndrome and congenital birth abnormalities. These abnormalities, which can include neurologic, ophthalmologic, audiologic, and skeletal findings, are now considered congenital Zika syndrome (CZS). Brain abnormalities in CZS include cerebral calcifications, malformations of cortical development, ventriculomegaly, lissencephaly, hypoplasia of the cerebellum and brainstem. The ocular findings, which are present in up to 70% of infants with CZS, include iris coloboma, lens subluxation, cataract, congenital glaucoma, and especially posterior segment findings. Loss of retinal pigment epithelium, the presence of a thin choroid, a perivascular choroidal inflammatory infiltrate, and atrophic changes within the optic nerve were seen in histologic analyses of eyes from deceased fetuses. To date, there is no ZIKV licensed vaccines or antiviral therapies are available for treatment. Preventive measures include individual protection from mosquito bites, control of mosquito populations and the use of barriers measures such as condoms during sexual intercourse or sexual abstinence for couples either at risk or after confirmed infection. A literature review based on studies that analyzed ocular findings in mothers and infants with CZS, with or without microcephaly, was conducted and a theoretical pathophysiologic explanation for ZIKV-ocular abnormalities was formulated. Copyright © 2018. Published by Elsevier Ltd.

Making sense of hypertrophic scar: a role for nerves.

PubMed

Scott, Jeffrey R; Muangman, Pornprom; Gibran, Nicole S

2007-01-01

Healed partial thickness wounds including burns and donor sites cause hypertrophic scar formation and patient discomfort. For many patients with hypertrophic scars, pruritus is the most distressing symptom, which leads to wound excoriation and chronic wound formation. In spite of the clinical significance of abnormal innervation in scars, the nervous system has been largely ignored in the pathophysiology of hypertrophic scars. Evidence that neuropeptides contribute to inflammatory responses to injury include inflammatory cell chemotaxis, cytokine and growth factor production. The neuropeptide substance P, which is released from nerve endings after injury, induces inflammation and mediates angiogenesis, keratinocyte proliferation, and fibrogenesis. Substance P activity is tightly regulated by neutral endopeptidase (NEP), a membrane bound metallopeptidase that degrades substance P at the cell membrane. Altered substance P levels may contribute to impaired cutaneous healing responses associated with diabetes mellitus or hypertrophic scar formation. Topical application of exogenous substance P or an NEP inhibitor enhances wound closure kinetics in diabetic murine wounds suggesting that diabetic wounds have insufficient substance P levels to promote a neuroinflammatory response necessary for normal wound repair. Conversely, increased nerve numbers and neuropeptide levels with reduced NEP levels in human and porcine hypertrophic scar samples suggest that excessive neuropeptide activity induces exuberant inflammation in hypertrophic scars. Given these observations about the role of neuropeptides in cutaneous repair, neuronal modulation of repair processes at two extremes of abnormal wound healing, chronic non-healing ulcers in type II diabetes mellitus and hypertrophic scars in deep partial thickness wounds, may provide therapeutic targets.

[Ocular coloboma and results of brain MRI: preliminary results].

PubMed

Denis, D; Girard, N; Levy-Mozziconacci, A; Berbis, J; Matonti, F

2013-03-01

Congenital ocular colobomas are the result of a failure in closure of the embryonal fissure. We present a prospective study (2007-2011) in which we report brain MRI findings in children with ocular coloboma. Thirty-five children (54 eyes) were included; 15 boys, 20 girls with a median age of 24.0 months (1.0-96.0) at first presentation. Within 2 to 3 months following complete ophthalmologic examination, brain MRI was performed. Colobomas were bilateral in 19 cases and unilateral in 16 cases. Eleven different types of coloboma were identified. Of 54 eyes, 74% demonstrated optic nerve coloboma, of which 28 were severe. Of 35 MRI's performed, abnormalities were present in 86%: gyration abnormalities (n=21), lateral ventricular dilatation (n=17), dilatation of the Virchow-Robin and subarachnoid spaces (n=14), signal abnormalities and brain stem malformations (n=14), white matter signal abnormalities (n=11), corpus callosum abnormalities (n=10). Most of these abnormalities were related. Gyration abnormalities were the most frequent. There was no significant association between the severity of the coloboma and the abnormalities found (P=1.0). Likewise, there was no significant association of gyration abnormalities with the severity of coloboma in children (P=1.0). This study shows, for the first time, the existence of frequent cerebral abnormalities on MRI in children with ocular coloboma. The most common abnormality being gyration abnormalities, in 60% of cases. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

Conductive micropatterned polyurethane films as tissue engineering scaffolds for Schwann cells and PC12 cells.

PubMed

Wu, Yaobin; Wang, Ling; Hu, Tianli; Ma, Peter X; Guo, Baolin

2018-05-15

Controlling cellular alignment and elongation has been demonstrated as an important parameter for developing nerve tissue engineering scaffolds. Many approaches have been developed to guide cellular orientation for nerve regeneration such as micropatterning techniques. However, most of materials used for developing micropatterning scaffolds lack of bioactivity and biofunctionability. Here we present a functional conductive micropatterned scaffold based on bioactive conductive biodegradable polyurethane prepared using a micro-molding technique. These conductive micropatterned scaffolds are able to not only induce the Schwann cells (SCs) alignment and elongation by the micropatterned surface but also enhance the nerve growth factor (NGF) gene expression of SCs by the bioactivity of these materials. Additionally, the combined effect of the bioactivity of such conductive materials and the micropatterned structure also dramatically promotes the neurite extension and elongation of PC12 cells in a highly aligned direction. These data suggest that these conductive micropatterned scaffolds that easily control cellular orientation and organization, and dramatically enhance NGF gene expression and significantly induce the neurite extension of PC12 cells, have a great potential for nerve regeneration applications. Copyright © 2018 Elsevier Inc. All rights reserved.

Turning Electromyography Reports Upside Down: A Pilot Study Surveying Referring Providers

PubMed Central

Shenoy, Anant M.; Baquis, Kate G.; Baquis, George D.

2016-01-01

Providers are expressing a desire for more efficient ways to retrieve relevant clinical data from the Electronic Health Record. In an effort to improve our Electromyography and Nerve Conduction Study reports, we surveyed referring providers on the effects of having the IMPRESSION at the start of the report. Our survey respondents felt that using this format for an Electromyography and Nerve Conduction Study report significantly improved the quality of the report while saving them time and/or mouse clicks when interpreting the report. Electro diagnosticians might consider using this format for their Electromyography and Nerve Conduction Study reports to improve referring provider satisfaction. PMID:27708744

Reversible conduction block in peripheral nerve using electrical waveforms.

PubMed

Bhadra, Niloy; Vrabec, Tina L; Bhadra, Narendra; Kilgore, Kevin L

2018-01-01

Electrical nerve block uses electrical waveforms to block action potential propagation. Two key features that distinguish electrical nerve block from other nonelectrical means of nerve block: block occurs instantly, typically within 1 s; and block is fully and rapidly reversible (within seconds). Approaches for achieving electrical nerve block are reviewed, including kilohertz frequency alternating current and charge-balanced polarizing current. We conclude with a discussion of the future directions of electrical nerve block. Electrical nerve block is an emerging technique that has many significant advantages over other methods of nerve block. This field is still in its infancy, but a significant expansion in the clinical application of this technique is expected in the coming years.

Neurophysiological aspects of peripheral neuropathies.

PubMed

MacKenzie, R A; Skuse, N F; Lethlean, A K

1976-01-01

1. Eighty-eight intrafascicular neural recordings were obtained in 10 normal subjects, 5 patients with axonal degeneration and 11 patients with demyelinating neuropathy. 2. Stimulus levels required for perception and fibre activation were higher in neuropathic subjects. Fibres transmitting touch perception had significantly lower conduction velocities in both patient groups, but were very much lower in the group with demyelinating neuropahty than the group with axonal degeneration. Maximum electrical stimulation evoked dispersed fibre responses in the axonal degeneration group and more dispersed, slowly conducting fibre potentials in the demyelinating group. In patients with hypertrophic Charcot-Marie-Tooth disorder, usually only a small group of slowly conducting low amplitude potentials was recorded. 3. Delivery of a train of supramaximal stimuli caused prolongation of latency and dispersion of fibre potentials in all microneurographic recordings. The changes were significantly greater in the axonal neuropathy group than in normals, and recovery was slower. The demyelinating neuropathies showed significantly greater changes than both the normal and the axonal neuropathy groups, and post-tetanic conduction slowing became even more marked after limb temperature was raised. 4. Surface SAP recordings showed normal refractory period in chronic axonal neuropathy but significant latency prolongation occurred in demyelinating neuropathy. 5. It is concluded that both receptor and nerve fibre abnormalities contribute to sensory dysfunction in degenerative and demyelinating neuropathies.

A System for Studying Facial Nerve Function in Rats through Simultaneous Bilateral Monitoring of Eyelid and Whisker Movements

PubMed Central

Heaton, James T.; Kowaleski, Jeffrey M.; Bermejo, Roberto; Zeigler, H. Philip; Ahlgren, David J.; Hadlock, Tessa A.

2008-01-01

The occurrence of inappropriate co-contraction of facially innervated muscles in humans (synkinesis) is a common sequela of facial nerve injury and recovery. We have developed a system for studying facial nerve function and synkinesis in restrained rats using non-contact opto-electronic techniques that enable simultaneous bilateral monitoring of eyelid and whisker movements. Whisking is monitored in high spatio-temporal resolution using laser micrometers, and eyelid movements are detected using infrared diode and phototransistor pairs that respond to the increased reflection when the eyelids cover the cornea. To validate the system, eight rats were tested with multiple five-minute sessions that included corneal air puffs to elicit blink and scented air flows to elicit robust whisking. Four rats then received unilateral facial nerve section and were tested at weeks 3–6. Whisking and eye blink behavior occurred both spontaneously and under stimulus control, with no detectable difference from published whisking data. Proximal facial nerve section caused an immediate ipsilateral loss of whisking and eye blink response, but some ocular closures emerged due to retractor bulbi muscle function. The independence observed between whisker and eyelid control indicates that this system may provide a powerful tool for identifying abnormal co-activation of facial zones resulting from aberrant axonal regeneration. PMID:18442856

Cornea nerve fiber quantification and construction of phenotypes in patients with fibromyalgia

PubMed Central

Oudejans, Linda; He, Xuan; Niesters, Marieke; Dahan, Albert; Brines, Michael; van Velzen, Monique

2016-01-01

Cornea confocal microscopy (CCM) is a novel non-invasive method to detect small nerve fiber pathology. CCM generally correlates with outcomes of skin biopsies in patients with small fiber pathology. The aim of this study was to quantify the morphology of small nerve fibers of the cornea of patients with fibromyalgia in terms of density, length and branching and further phenotype these patients using standardized quantitative sensory testing (QST). Small fiber pathology was detected in the cornea of 51% of patients: nerve fiber length was significantly decreased in 44% of patients compared to age- and sex-matched reference values; nerve fiber density and branching were significantly decreased in 10% and 28% of patients. The combination of the CCM parameters and sensory tests for central sensitization, (cold pain threshold, mechanical pain threshold, mechanical pain sensitivity, allodynia and/or windup), yielded four phenotypes of fibromyalgia patients in a subgroup analysis: one group with normal cornea morphology without and with signs of central sensitization, and a group with abnormal cornea morphology parameters without and with signs of central sensitization. In conclusion, half of the tested fibromyalgia population demonstrates signs of small fiber pathology as measured by CCM. The four distinct phenotypes suggest possible differences in disease mechanisms and may require different treatment approaches. PMID:27006259

Axonal Dysfunction Precedes Motor Neuronal Death in Amyotrophic Lateral Sclerosis

PubMed Central

Iwai, Yuta; Shibuya, Kazumoto; Misawa, Sonoko; Sekiguchi, Yukari; Watanabe, Keisuke; Amino, Hiroshi; Kuwabara, Satoshi

2016-01-01

Wide-spread fasciculations are a characteristic feature in amyotrophic lateral sclerosis (ALS), suggesting motor axonal hyperexcitability. Previous excitability studies have shown increased nodal persistent sodium conductances and decreased potassium currents in motor axons of ALS patients, both of the changes inducing hyperexcitability. Altered axonal excitability potentially contributes to motor neuron death in ALS, but the relationship of the extent of motor neuronal death and abnormal excitability has not been fully elucidated. We performed multiple nerve excitability measurements in the median nerve at the wrist of 140 ALS patients and analyzed the relationship of compound muscle action potential (CMAP) amplitude (index of motor neuronal loss) and excitability indices, such as strength-duration time constant, threshold electrotonus, recovery cycle and current-threshold relationships. Compared to age-matched normal controls (n = 44), ALS patients (n = 140) had longer strength-duration time constant (SDTC: a measure of nodal persistent sodium current; p < 0.05), greater threshold changes in depolarizing threshold electrotonus (p < 0.05) and depolarizing current threshold relationship (i.e. less accommodation; (p < 0.05), greater superexcitability (a measure of fast potassium current; p < 0.05) and reduced late subexcitability (a measure of slow potassium current; p < 0.05), suggesting increased persistent sodium currents and decreased potassium currents. The reduced potassium currents were found even in the patient subgroups with normal CMAP (> 5mV). Regression analyses showed that SDTC (R = -0.22) and depolarizing threshold electrotonus (R = -0.22) increased with CMAP decline. These findings suggest that motor nerve hyperexcitability occurs in the early stage of the disease, and precedes motor neuronal loss in ALS. Modulation of altered ion channel function could be a treatment option for ALS. PMID:27383069

Clinical, neurophysiological and morphological study of dominant intermediate Charcot-Marie-Tooth type C neuropathy.

PubMed

Thomas, Florian P; Guergueltcheva, Velina; Gondim, Francisco A A; Tournev, Ivailo; Rao, Chitharanjan V; Ishpekova, Boryana; Kinsella, Laurence J; Pan, Yi; Geller, Thomas J; Litvinenko, Ivan; De Jonghe, Peter; Scherer, Steven S; Jordanova, Albena

2016-03-01

Dominant intermediate Charcot-Marie-Tooth neuropathy subtype C (DI-CMTC) was associated with mutations in the YARS gene, encoding tyrosyl-tRNA synthetase, in two large unrelated Bulgarian and US pedigrees and one sporadic case. Here for the first time we describe the clinical, neurophysiological and histopathological features, and phenotypic differences between these two DI-CMTC families. Twenty-one affected individuals from the US family and 27 from the Bulgarian family were evaluated. The mean age of onset in US subjects was 10.7 years in men and 7.3 years in women, while in the Bulgarian participants it was 18.2 years in men and 33.7 years in women. The course was slowly progressive. Extensor digitorum brevis atrophy was uniform. Atrophy and/or weakness of upper and lower limb muscles were found in over 50 % of the subjects. Nerve conduction studies (NCS) were abnormal in all US adults and five of six children and all Bulgarian patients except one asymptomatic 25-year-old man. Median motor NCS were in the range of 29.5-45.6 m/s in the US family and 24.7-57.8 m/s in the Bulgarian family. Sural sensory nerve action potentials were absent in 14/21 and 4/12 NCS from adult US and Bulgarian participants, respectively. Analysis of sural nerve biopsies from US patients revealed age-dependent morphological changes of axonal degeneration, absence of onion bulbs, and <10 % fibers with segmental remyelination. Our findings provide further insights into the diagnosis and pathology of intermediate CMT. They also extend the phenotypic spectrum of peripheral neuropathies associated with aminoacyl-tRNA synthetase mutations.

Altered mechanisms underlying the abnormal glutamate release in amyotrophic lateral sclerosis at a pre-symptomatic stage of the disease.

PubMed

Bonifacino, Tiziana; Musazzi, Laura; Milanese, Marco; Seguini, Mara; Marte, Antonella; Gallia, Elena; Cattaneo, Luca; Onofri, Franco; Popoli, Maurizio; Bonanno, Giambattista

2016-11-01

Abnormal Glu release occurs in the spinal cord of SOD1(G93A) mice, a transgenic animal model for human ALS. Here we studied the mechanisms underlying Glu release in spinal cord nerve terminals of SOD1(G93A) mice at a pre-symptomatic disease stage (30days) and found that the basal release of Glu was more elevated in SOD1(G93A) with respect to SOD1 mice, and that the surplus of release relies on synaptic vesicle exocytosis. Exposure to high KCl or ionomycin provoked Ca(2+)-dependent Glu release that was likewise augmented in SOD1(G93A) mice. Equally, the Ca(2+)-independent hypertonic sucrose-induced Glu release was abnormally elevated in SOD1(G93A) mice. Also in this case, the surplus of Glu release was exocytotic in nature. We could determine elevated cytosolic Ca(2+) levels, increased phosphorylation of Synapsin-I, which was causally related to the abnormal Glu release measured in spinal cord synaptosomes of pre-symptomatic SOD1(G93A) mice, and increased phosphorylation of glycogen synthase kinase-3 at the inhibitory sites, an event that favours SNARE protein assembly. Western blot experiments revealed an increased number of SNARE protein complexes at the nerve terminal membrane, with no changes of the three SNARE proteins and increased expression of synaptotagmin-1 and β-Actin, but not of an array of other release-related presynaptic proteins. These results indicate that the abnormal exocytotic Glu release in spinal cord of pre-symptomatic SOD1(G93A) mice is mainly based on the increased size of the readily releasable pool of vesicles and release facilitation, supported by plastic changes of specific presynaptic mechanisms. Copyright © 2016 Elsevier Inc. All rights reserved.

Neurofilament light polypeptide gene N98S mutation in mice leads to neurofilament network abnormalities and a Charcot-Marie-Tooth Type 2E phenotype.

PubMed

Adebola, Adijat A; Di Castri, Theo; He, Chui-Zhen; Salvatierra, Laura A; Zhao, Jian; Brown, Kristy; Lin, Chyuan-Sheng; Worman, Howard J; Liem, Ronald K H

2015-04-15

Charcot-Marie-Tooth disease (CMT) is the most commonly inherited neurological disorder with a prevalence of 1 in 2500 people worldwide. Patients suffer from degeneration of the peripheral nerves that control sensory information of the foot/leg and hand/arm. Multiple mutations in the neurofilament light polypeptide gene, NEFL, cause CMT2E. Previous studies in transfected cells showed that expression of disease-associated neurofilament light chain variants results in abnormal intermediate filament networks associated with defects in axonal transport. We have now generated knock-in mice with two different point mutations in Nefl: P8R that has been reported in multiple families with variable age of onset and N98S that has been described as an early-onset, sporadic mutation in multiple individuals. Nefl(P8R/+) and Nefl(P8R/P8R) mice were indistinguishable from Nefl(+/+) in terms of behavioral phenotype. In contrast, Nefl(N98S/+) mice had a noticeable tremor, and most animals showed a hindlimb clasping phenotype. Immunohistochemical analysis revealed multiple inclusions in the cell bodies and proximal axons of spinal cord neurons, disorganized processes in the cerebellum and abnormal processes in the cerebral cortex and pons. Abnormal processes were observed as early as post-natal day 7. Electron microscopic analysis of sciatic nerves showed a reduction in the number of neurofilaments, an increase in the number of microtubules and a decrease in the axonal diameters. The Nefl(N98S/+) mice provide an excellent model to study the pathogenesis of CMT2E and should prove useful for testing potential therapies. © The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Confocal Adaptive Optics Imaging of Peripapillary Nerve Fiber Bundles: Implications for Glaucomatous Damage Seen on Circumpapillary OCT Scans.

PubMed

Hood, Donald C; Chen, Monica F; Lee, Dongwon; Epstein, Benjamin; Alhadeff, Paula; Rosen, Richard B; Ritch, Robert; Dubra, Alfredo; Chui, Toco Y P

2015-04-01

To improve our understanding of glaucomatous damage as seen on circumpapillary disc scans obtained with frequency-domain optical coherence tomography (fdOCT), fdOCT scans were compared to images of the peripapillary retinal nerve fiber (RNF) bundles obtained with an adaptive optics-scanning light ophthalmoscope (AO-SLO). The AO-SLO images and fdOCT scans were obtained on 6 eyes of 6 patients with deep arcuate defects (5 points ≤-15 db) on 10-2 visual fields. The AO-SLO images were montaged and aligned with the fdOCT images to compare the RNF bundles seen with AO-SLO to the RNF layer thickness measured with fdOCT. All 6 eyes had an abnormally thin (1% confidence limit) RNF layer (RNFL) on fdOCT and abnormal (hyporeflective) regions of RNF bundles on AO-SLO in corresponding regions. However, regions of abnormal, but equal, RNFL thickness on fdOCT scans varied in appearance on AO-SLO images. These regions could be largely devoid of RNF bundles (5 eyes), have abnormal-appearing bundles of lower contrast (6 eyes), or have isolated areas with a few relatively normal-appearing bundles (2 eyes). There also were local variations in reflectivity of the fdOCT RNFL that corresponded to the variations in AO-SLO RNF bundle appearance. Relatively similar 10-2 defects with similar fdOCT RNFL thickness profiles can have very different degrees of RNF bundle damage as seen on fdOCT and AO-SLO. While the results point to limitations of fdOCT RNFL thickness as typically analyzed, they also illustrate the potential for improving fdOCT by attending to variations in local intensity.

Comparison of multifocal visual evoked potential, static automated perimetry, and optical coherence tomography findings for assessing visual pathways in patients with pituitary adenomas.

PubMed

Qiao, Nidan; Zhang, Yichao; Ye, Zhao; Shen, Ming; Shou, Xuefei; Wang, Yongfei; Li, Shiqi; Wang, Min; Zhao, Yao

2015-10-01

There have been no studies investigating the correlation between structural [thickness of the retinal nerve fiber layer (RNFL) as determined by optical coherence tomography (OCT)] and functional [Humphrey visual field (HVF) or visual evoked potential (VEP) amplitude] measures of optic nerve integrity in patients with pituitary adenomas (PA). Patients with PAs were recruited between September 2010 and September 2013. OCT, standard automated perimetry (SAP), and multifical VEP (mfVEP) were performed. Agreement between OCT, SAP, and mfVEP values in classifying eyes/quadrants was determined using AC1 statistics. Pearson's correlation was used to examine relationships between structural and functional data. In total, 88.7% of the eyes tested showed abnormal SAP findings and 93.7% showed abnormal mfVEP findings. Only 14.8% of the eyes showed abnormal OCT findings. The agreement between SAP and mfVEP findings was 88.9% (AC1 = 0.87). The agreement between OCT and mfVEP findings was 24.2% (AC1 = -0.52), and that between OCT and SAP findings was 21.5% (AC1 = -0.56). The correlation values between RNFL thickness and the functional measurements were -0.601 for the mfVEP score (P = 0.000) and -0.441 for the SAP score (P = 0.000). The correlation between the mfVEP and SAP scores was -0.617 (P = 0.000). mfVEP, SAP, and OCT provided complementary information for detecting visual pathway abnormalities in patients with PAs. Good agreement was demonstrated between SAP and mfVEP and quantitative analysis of structure-function measurements revealed a moderate correlation.

Cold Exposure Exacerbates the Development of Diabetic Polyneuropathy in the Rat

PubMed Central

Kasselman, Lora J.; Veves, Aristidis; Gibbons, Christopher H.; Rutkove, Seward B.

2009-01-01

Diabetic polyneuropathy (DPN) and cold-induced nerve injury share several pathogenic mechanisms. This study explores whether cold exposure contributes to the development of DPN. Streptozotocin-induced diabetic rats and controls were exposed to a room temperature (23°C) or cold environment (10°C). H-reflex, tail and sciatic motor, and sensory nerve conduction studies were performed. Analyses of sural nerve, intraepidermal nerve fibers, and skin and nerve nitrotyrosine ELISAs were performed. Diabetic animals exposed to a cold environment had an increased H-reflex four weeks earlier than diabetic room temperature animals (P = .03). Cold-exposed diabetic animals also had greater reduction in motor conduction velocities at 20 weeks (P = .017), decreased skin nerve fiber density (P = .037), and increased skin nitrotyrosine levels (P = .047). Cold exposure appears to hasten the development of DPN in the rat STZ model of diabetes. These findings support that further study into the relationship between ambient temperature and DPN is warranted. PMID:20130819

Functional restoration of the paralyzed diaphragm in high cervical quadriplegia via phrenic nerve neurotization utilizing the functional spinal accessory nerve.

PubMed

Yang, Ming-liang; Li, Jian-jun; Zhang, Shao-cheng; Du, Liang-jie; Gao, Feng; Li, Jun; Wang, Yu-ming; Gong, Hui-ming; Cheng, Liang

2011-08-01

The authors report a case of functional improvement of the paralyzed diaphragm in high cervical quadriplegia via phrenic nerve neurotization using a functional spinal accessory nerve. Complete spinal cord injury at the C-2 level was diagnosed in a 44-year-old man. Left diaphragm activity was decreased, and the right diaphragm was completely paralyzed. When the level of metabolism or activity (for example, fever, sitting, or speech) slightly increased, dyspnea occurred. The patient underwent neurotization of the right phrenic nerve with the trapezius branch of the right spinal accessory nerve at 11 months postinjury. Four weeks after surgery, training of the synchronous activities of the trapezius muscle and inspiration was conducted. Six months after surgery, motion was observed in the previously paralyzed right diaphragm. The lung function evaluation indicated improvements in vital capacity and tidal volume. This patient was able to sit in a wheelchair and conduct outdoor activities without assisted ventilation 12 months after surgery.

Characterizing the reduction of stimulation artifact noise in a tripolar nerve cuff electrode by application of a conductive shield layer.

PubMed

Sabetian, Parisa; Sadeghlo, Bita; Zhang, Chengran Harvey; Yoo, Paul B

2017-02-01

Tripolar nerve cuff electrodes have been widely used for measuring peripheral nerve activity. However, despite the high signal-to-noise ratio levels that can be achieved with this recording configuration, the clinical use of cuff electrodes in closed-loop controlled neuroprostheses remains limited. This is largely attributed to artifact noise signals that contaminate the recorded neural activity. In this study, we investigated the use of a conductive shield layer (CSL) as a means of reducing the artifact noise recorded by nerve cuff electrodes. Using both computational simulations and in vivo experiments, we found that the CSL can result in up to an 85% decrease in the recorded artifact signal. Both the electrical conductivity and the surface area of the CSL were identified as important design criteria. Although this study shows that the CSL can significantly reduce artifact noise in tripolar nerve cuff electrodes, long-term implant studies are needed to validate our findings. Copyright © 2016 IPEM. Published by Elsevier Ltd. All rights reserved.

[Changes in the innervation of the taste buds in diabetic rats].

PubMed

Hevér, Helén; Altdorfer, Károly; Zelles, Tivadar; Batbayar, Bayarchimeg; Fehér, Erzsébet

2013-03-24

Abnormal sensations such as pain and impairment of taste are symptoms of approximately 10% of patients having diabetes mellitus. The aim of the study was to investigate and quantify the different neuropeptide containing nerve fibres in the vallate papilla of the diabetic rat. Immunohistochemical methods were used to study the changes of the number of different neuropeptide containing nerve terminals located in the vallate papillae in diabetic rats. Diabetes was induced in the rats with streptozotocin. Two weeks after streptozotocin treatment the number of the substance P, galanin, vasoactive intestinal polypeptide and neuropeptide Y immunoreactive nerve terminals was significantly increased (p<0.05) in the tunica mucosa of the tongue. The number of the lymphocytes and mast cells was also increased significantly. Some of the immunoreactive nerve terminals were located in the lingual epithelium both intragemmally and extragemmally and were seen to comprise dense bundles in the lamina propria just beneath the epithelium. No taste cells were immunoreactive for any of the investigated peptides. Vasoactive intestinal polypeptide and neuropeptide Y immunoreactive nerve fibres were not detected in the taste buds. For weeks after streptozotocin administration the number of the substance P, calcitonin gene related peptide and galanin immunoreactive nerve terminals was decreased both intragemmally and intergemmally. In case of immediate insulin treatment, the number of the immunoreactive nerve terminals was similar to that of the controls, however, insulin treatment given 1 week later to diabetic rats produced a decreased number of nerve fibers. Morphometry revealed no significant difference in papilla size between the control and diabetic groups, but there were fewer taste buds (per papilla). Increased number of immunoreactive nerve terminals and mast cells 2 weeks after the development of diabetes was the consequence of neurogenic inflammation which might cause vasoconstriction and lesions of the oral mucosa. Taste impairment, which developed 4 weeks after streptozotocin treatment could be caused by neuropathic defects and degeneration or morphological changes in the taste buds and nerve fibres.

Chitosan conduits combined with nerve growth factor microspheres repair facial nerve defects

PubMed Central

Liu, Huawei; Wen, Weisheng; Hu, Min; Bi, Wenting; Chen, Lijie; Liu, Sanxia; Chen, Peng; Tan, Xinying

2013-01-01

Microspheres containing nerve growth factor for sustained release were prepared by a compound method, and implanted into chitosan conduits to repair 10-mm defects on the right buccal branches of the facial nerve in rabbits. In addition, chitosan conduits combined with nerve growth factor or normal saline, as well as autologous nerve, were used as controls. At 90 days post-surgery, the muscular atrophy on the right upper lip was more evident in the nerve growth factor and normal sa-line groups than in the nerve growth factor-microspheres and autologous nerve groups. physiological analysis revealed that the nerve conduction velocity and amplitude were significantly higher in the nerve growth factor-microspheres and autologous nerve groups than in the nerve growth factor and normal saline groups. Moreover, histological observation illustrated that the di-ameter, number, alignment and myelin sheath thickness of myelinated nerves derived from rabbits were higher in the nerve growth factor-microspheres and autologous nerve groups than in the nerve growth factor and normal saline groups. These findings indicate that chitosan nerve conduits bined with microspheres for sustained release of nerve growth factor can significantly improve facial nerve defect repair in rabbits. PMID:25206635

Diffusion and ADC-map images detect ongoing demyelination on subcortical white matter in an adult metachromatic leukodystrophy patient with autoimmune Hashimoto thyroiditis

PubMed Central

Miura, Akiko; Kumabe, Yuri; Kimura, En; Yamashita, Satoshi; Ueda, Akihiko; Hirano, Teruyuki; Uchino, Makoto

2010-01-01

Adult-onset metachromatic leukodystrophy (MLD) often shows schizophrenia- or encephalopathy-like symptoms at an early stage, such as behavioural abnormalities, cognitive impairment, mood disorders and hallucinations. The authors report the case of an adult woman with MLD who had been given antipsychotic medication for schizophrenia. In the differential diagnosis, screening of auto-antibodies was important for ruling out other encephalopathies as she had a euthyroid Hashimoto thyroiditis. Diagnosis was based the results of MRI, nerve conduction velocity, sensory evoked potential, motor evoked potential, lysosomal enzyme activity and gene analysis studies. Brain MRI showed diffuse demyelination spreading from the deep white matter to subcortical area as high signals at the edges of these lesions in diffusion and apparent diffusion coefficient-map images with the U-fibres conserved. The authors diagnosed adult-onset MLD coexisting with euthyroid autoimmune Hashimoto thyroiditis. PMID:22798296

Multicenter trial of the proficiency of smart quantitative sensation tests.

PubMed

Dyck, Peter J; Argyros, Barbara; Russell, James W; Gahnstrom, Linde E; Nalepa, Susan; Albers, James W; Lodermeier, Karen A; Zafft, Andrew J; Dyck, P James B; Klein, Christopher J; Litchy, William J; Davies, Jenny L; Carter, Rickey E; Melton, L Joseph

2014-05-01

We assessed proficiency (accuracy and intra- and intertest reproducibility) of smart quantitative sensation tests (smart QSTs) in subjects without and with diabetic sensorimotor polyneuropathy (DSPN). Technologists from 3 medical centers using different but identical QSTs independently assessed 6 modalities of sensation of the foot (or leg) twice in patients without (n = 6) and with (n = 6) DSPN using smart computer assisted QSTs. Low rates of test abnormalities were observed in health and high rates in DSPN. Very high intraclass correlations were obtained between continuous measures of QSTs and neuropathy signs, symptoms, or nerve conductions (NCs). No significant intra- or intertest differences were observed. These results provide proof of concept that smart QSTs provide accurate assessment of sensation loss without intra- or intertest differences useful for multicenter trials. Smart technology makes possible efficient testing of body surface area sensation loss in symmetric length-dependent sensorimotor polyneuropathies. Copyright © 2013 Wiley Periodicals, Inc.

A Multicenter Trial of the Proficiency of Smart Quantitative Sensation Tests

PubMed Central

Dyck, Peter J.; Argyros, Barbara; Russell, James W.; Gahnstrom, Linde E.; Nalepa, Susan; Albers, James W.; Lodermeier, Karen A.; Zafft, Andrew J.; Dyck, P. James B.; Klein, Christopher J.; Litchy, William J.; Davies, Jenny L.; Carter, Rickey E.; Melton, L. Joseph

2014-01-01

Introduction We assessed proficiency (accuracy and intra- and inter-test reproducibility) of smart quantitative sensation tests (smart QSTs) in subjects without and with diabetic polyneuropathy (DSPN). Methods Technologists from 3 medical centers using different but identical QSTs assessed independently 6 modalities of sensation of foot (or leg) twice in patients without (n = 6) and with (n = 6) DSPN using smart computer assisted QSTs. Results Low rates of test abnormalities were observed in health and high rates in DSPN. Very high intra-class correlations were obtained between continuous measures of QSTs and neuropathy signs, symptoms, or nerve conductions (NCs). No significant intra- or inter-test differences were observed. Discussion These results provide proof of concept that smart QSTs provide accurate assessment of sensation loss without intra- or inter-test differences useful for multicenter trials. Smart technology makes possible efficient testing of body surface area sensation loss in symmetric length-dependent sensorimotor polyneuropathies. PMID:23929701

Sciatic Nerve Injury Related to Hip Replacement Surgery: Imaging Detection by MR Neurography Despite Susceptibility Artifacts

PubMed Central

Wolf, Marcel; Bäumer, Philipp; Pedro, Maria; Dombert, Thomas; Staub, Frank; Heiland, Sabine; Bendszus, Martin; Pham, Mirko

2014-01-01

Sciatic nerve palsy related to hip replacement surgery (HRS) is among the most common causes of sciatic neuropathies. The sciatic nerve may be injured by various different periprocedural mechanisms. The precise localization and extension of the nerve lesion, the determination of nerve continuity, lesion severity, and fascicular lesion distribution are essential for assessing the potential of spontaneous recovery and thereby avoiding delayed or inappropriate therapy. Adequate therapy is in many cases limited to conservative management, but in certain cases early surgical exploration and release of the nerve is indicated. Nerve-conduction-studies and electromyography are essential in the diagnosis of nerve injuries. In postsurgical nerve injuries, additional diagnostic imaging is important as well, in particular to detect or rule out direct mechanical compromise. Especially in the presence of metallic implants, commonly applied diagnostic imaging tests generally fail to adequately visualize nervous tissue. MRI has been deemed problematic due to implant-related artifacts after HRS. In this study, we describe for the first time the spectrum of imaging findings of Magnetic Resonance neurography (MRN) employing pulse sequences relatively insensitive to susceptibility artifacts (susceptibility insensitive MRN, siMRN) in a series of 9 patients with HRS procedure related sciatic nerve palsy. We were able to determine the localization and fascicular distribution of the sciatic nerve lesion in all 9 patients, which clearly showed on imaging predominant involvement of the peroneal more than the tibial division of the sciatic nerve. In 2 patients siMRN revealed direct mechanical compromise of the nerve by surgical material, and in one of these cases indication for surgical release of the sciatic nerve was based on siMRN. Thus, in selected cases of HRS related neuropathies, especially when surgical exploration of the nerve is considered, siMRN, with its potential to largely overcome implant related artifacts, is a useful diagnostic addition to nerve-conduction-studies and electromyography. PMID:24558483

Schwann cell glycogen selectively supports myelinated axon function.

PubMed

Brown, Angus M; Evans, Richard D; Black, Joel; Ransom, Bruce R

2012-09-01

Interruption of energy supply to peripheral axons is a cause of axon loss. We determined whether glycogen was present in mammalian peripheral nerve, and whether it supported axon conduction during aglycemia. We used biochemical assay and electron microscopy to determine the presence of glycogen, and electrophysiology to monitor axon function. Glycogen was present in sciatic nerve, its concentration varying directly with ambient glucose. Electron microscopy detected glycogen granules primarily in myelinating Schwann cell cytoplasm, and these diminished after exposure to aglycemia. During aglycemia, conduction failure in large myelinated axons (A fibers) mirrored the time course of glycogen loss. Latency to compound action potential (CAP) failure was directly related to nerve glycogen content at aglycemia onset. Glycogen did not benefit the function of slow-conducting, small-diameter unmyelinated axons (C fibers) during aglycemia. Blocking glycogen breakdown pharmacologically accelerated CAP failure during aglycemia in A fibers, but not in C fibers. Lactate was as effective as glucose in supporting sciatic nerve function, and was continuously released into the extracellular space in the presence of glucose and fell rapidly during aglycemia. Our findings indicated that glycogen is present in peripheral nerve, primarily in myelinating Schwann cells, and exclusively supports large-diameter, myelinated axon conduction during aglycemia. Available evidence suggests that peripheral nerve glycogen breaks down during aglycemia and is passed, probably as lactate, to myelinated axons to support function. Unmyelinated axons are not protected by glycogen and are more vulnerable to dysfunction during periods of hypoglycemia. . Copyright © 2012 American Neurological Association.

Schwann Cell Glycogen Selectively Supports Myelinated Axon Function

PubMed Central

Brown, Angus M; Evans, Richard D; Black, Joel; Ransom, Bruce R

2012-01-01

Objectives Interruption of energy supply to peripheral axons is a cause of axon loss. We determined if glycogen was present in mammalian peripheral nerve, and if it supported axon conduction during aglycemia. Methods We used biochemical assay and electron microscopy to determine the presence of glycogen, and electrophysiology to monitor axon function. Results Glycogen was present in sciatic nerve, its concentration varying directly with ambient [glucose]. Electron microscopy detected glycogen granules primarily in myelinating Schwann cell cytoplasm and these diminished after exposure to aglycemia. During aglycemia, conduction failure in large myelinated axons (A fibers) mirrored the time-course of glycogen loss. Latency to CAP failure was directly related to nerve glycogen content at aglycemia onset. Glycogen did not benefit the function of slow-conducting, small diameter unmyelinated axons (C fibers) during aglycemia. Blocking glycogen breakdown pharmacologically accelerated CAP failure during aglycemia in A fibers, but not in C fibers. Lactate was as effective as glucose in supporting sciatic nerve function, and was continuously released into the extracellular space in the presence of glucose and fell rapidly during aglycemia. Interpretation Our findings indicated that glycogen is present in peripheral nerve, primarily in myelinating Schwann cells, and exclusively supports large diameter, myelinated axon conduction during aglycemia. Available evidence suggests that peripheral nerve glycogen breaks down during aglycemia and is passed, probably as lactate, to myelinated axons to support function. Unmyelinated axons are not protected by glycogen and are more vulnerable to dysfunction during periods of hypoglycemia. PMID:23034913

Mechanisms defining the electrotonic potential abnormalities in simulated amyotrophic lateral sclerosis.

PubMed

Stephanova, D I; Krustev, S M; Negrev, N

2012-06-01

Electrotonic potentials allow the accommodative processes to polarizing stimuli to be assessed. Electrotonic potential transients in response to applied polarizing stimuli are caused by the kinetics of underlying axonal conductances. Here, we study these transients using our multi-layered model of the human motor nerve, in three simulated cases of the motor neuron disease amyotrophic lateral sclerosis (ALS): ALS1, ALS2 and ALS3 are three consecutively greater degrees of uniform axonal dysfunctions along the human motor nerve fibre. The results show that the responses in the ALS1 case are quite similar to the normal case. In contrast, in the ALS2 and ALS3 cases, long-lasting (100 ms) subthreshold depolarizing stimuli activate the classical "transient" Na(+) channels in the nodal and in the internodal axolemma beneath the myelin sheath; this leads to action potential generation during the early parts of the electrotonic responses in all compartments along the fibre length. The results also show that the electrotonic potentials in response to long-lasting (100 ms) subthreshold hyperpolarizing stimuli in the ALS1 and ALS2 cases are quiet similar to those of the normal case. However, the current kinetics in the ALS3 case differs from the normal case after the termination of the long-lasting hyperpolarizing stimuli. In the most abnormal ALS3 case, the activation of the Na(+) channels in the nodal and in the internodal axolemma leads to repetitive action potential generation in the late parts (100-200 ms) of the hyperpolarizing electrotonic responses. The results show that the repetitive firing, due to the progressively increased nodal and internodal ion channel dysfunction, are consistent with the loss of functional potassium channels involving both the fast and the slow potassium channel types. The results confirm that the electrotonic potentials in the three simulated ALS cases are specific indicators for the motor neuron disease ALS. The mechanisms underlying the simulated ALS are also discussed.

Optimal optic nerve sheath diameter threshold for the identification of elevated opening pressure on lumbar puncture in a Chinese population.

PubMed

Wang, Lijuan; Feng, Liangshu; Yao, Yan; Wang, Yuzhi; Chen, Ying; Feng, Jiachun; Xing, Yingqi

2015-01-01

Ultrasonography of the optic nerve sheath diameter (ONSD) is a non-invasive and rapid method that might be helpful in the identification of increased intracranial pressure (ICP). The use of an ONSD greater than 5 mm on ultrasound as an indicator of increased ICP in a Caucasian population has been studied. However, the cut-off point of this predictor in Chinese patients has not been established. Thus, we conducted this study to identify the ONSD criterion for the detection of elevated opening pressure on lumbar puncture (LP) in a Chinese population and to investigate the influencing factors. This study was a blind cross-sectional study. Patients who presented with suspected increased ICP were included. The opening pressure on LP of each participant was confirmed. We analyzed the clinical differences between the groups of patients with abnormal and normal opening pressures on LP. A receiver operating characteristic curve was constructed to determine the ONSD cut-off point for the identification of abnormal opening pressure on LP. In total, 279 patients were recruited, and 101 patients presented with elevated opening pressure on LP. ONSD was a significant independent predictor of elevated opening pressure on LP (p<0.001). However, no statistical significance was observed regarding the factors that might have affected this relationship including gender, age, body mass index, waistline, head circumference, hypertension and pathological subtype. The ONSD cut-off point for the identification of elevated opening pressure on LP was 4.1 mm; this cut-off yielded a sensitivity of 95% and a specificity of 92%. ONSD is a strong and accurate predictor of elevated opening pressure on LP. The cut-off point of this predictor in a Chinese population was remarkably lower than that found in a Caucasian population. Thus, ethnic differences should be noted when using the ONSD as an indicator of increased ICP.

Arm Nerve Conduction Velocity (NCV), Brain NCV, Reaction Time, and Intelligence.

ERIC Educational Resources Information Center

Reed, T. Edward; Jensen, Arthur R.

1991-01-01

Correlations among peripheral nerve conduction velocity (NCV), brain NCV, simple and choice reaction times, and a standard measure of intelligence were investigated for 200 male college students. No correlation was found between any arm NCV and the intelligence score. Neurophysiological bases of human information processing and intelligence are…

Intelligence, Reaction Times, and Peripheral Nerve Conduction Velocity.

ERIC Educational Resources Information Center

Vernon, Philip A.; Mori, Monica

1992-01-01

In 2 studies with 85 and 88 undergraduates, respectively, peripheral nerve conduction velocity (NCV) was significantly correlated with IQ score and reaction times, and NCV and reaction time contributed significantly, in combination, to prediction of IQ. Results are interpreted in terms of a neural efficiency model of intelligence. (Author/SLD)

A case of primary hypothyroidism causing central nervous system atherosclerosis in a dog.

PubMed

Blois, Shauna L; Poma, Roberto; Stalker, Margaret J; Allen, Dana G

2008-08-01

A 2-year-old, castrated male, Australian shepherd was presented with a history of chronic mild ataxia, obesity, and lethargy. The dog was treated with levothyroxine, but the ataxia worsened. Cranial nerve abnormalities developed and the dog was euthanized. Postmortem examination revealed marked thyroid gland atrophy and widespread, severe central nervous system atherosclerosis.

Medical Administration: Patient Regulating To and Within the Continental United States

DTIC Science & Technology

1990-03-30

the muscles, neuromuscular junctions, peripheral nerves, spinal cord, and brain. APY Electroencephalography See EVALUATIONS. APM Electromyography See...Oxygen Ther- apy See EVALUATIONS. SSTX Kidney Transplants See SURGERY/Urology. SSF Morbid Obesity Surgery Patients requiring surgery for morbid obesity...Scans. APG Cytogenetics Patients having, or suspected of having, genetic abnormalities, or chromosomal study is indi- cated. APY

Somatic gain-of-function mutations in PIK3CA in patients with macrodactyly

PubMed Central

Rios, Jonathan J.; Paria, Nandina; Burns, Dennis K.; Israel, Bonnie A.; Cornelia, Reuel; Wise, Carol A.; Ezaki, Marybeth

2013-01-01

Macrodactyly is a discrete congenital anomaly consisting of enlargement of all tissues localized to the terminal portions of a limb, typically within a ‘nerve territory’. The classic terminology for this condition is ‘lipofibromatous hamartoma of nerve’ or Type I macrodactyly. The peripheral nerve, itself, is enlarged both in circumference and in length. It is not related to neurofibromatosis (NF1), nor is it associated with vascular malformations, such as in the recently reported CLOVES syndrome. The specific nerve pathophysiology in this form of macrodactyly has not been well described and a genetic etiology for this specific form of enlargement is unknown. To identify the genetic cause of macrodactyly, we used whole-exome sequencing to identify somatic mutations present in the affected nerve of a single patient. We confirmed a novel mutation in PIK3CA (R115P) present in the patient's affected nerve tissue but not in blood DNA. Sequencing PIK3CA exons identified gain-of-function mutations (E542K, H1047L or H1047R) in the affected tissue of five additional unrelated patients; mutations were absent in blood DNA available from three patients. Immunocytochemistry confirmed AKT activation in cultured cells from the nerve of a macrodactyly patient. Additionally, we found that the most abnormal structure within the involved nerve in a macrodactylous digit is the perineurium, with additional secondary effects on the axon number and size. Thus, isolated congenital macrodactyly is caused by somatic activation of the PI3K/AKT cell-signaling pathway and is genetically and biochemically related to other overgrowth syndromes. PMID:23100325

Efficacy of Manual Therapy Including Neurodynamic Techniques for the Treatment of Carpal Tunnel Syndrome: A Randomized Controlled Trial.

PubMed

Wolny, Tomasz; Saulicz, Edward; Linek, Paweł; Shacklock, Michael; Myśliwiec, Andrzej

2017-05-01

The purpose of this randomized trial was to compare the efficacy of manual therapy, including the use of neurodynamic techniques, with electrophysical modalities on patients with mild and moderate carpal tunnel syndrome (CTS). The study included 140 CTS patients who were randomly assigned to the manual therapy (MT) group, which included the use of neurodynamic techniques, functional massage, and carpal bone mobilizations techniques, or to the electrophysical modalities (EM) group, which included laser and ultrasound therapy. Nerve conduction, pain severity, symptom severity, and functional status measured by the Boston Carpal Tunnel Questionnaire were assessed before and after treatment. Therapy was conducted twice weekly and both groups received 20 therapy sessions. A baseline assessment revealed group differences in sensory conduction of the median nerve (P < .01) but not in motor conduction (P = .82). Four weeks after the last treatment procedure, nerve conduction was examined again. In the MT group, median nerve sensory conduction velocity increased by 34% and motor conduction velocity by 6% (in both cases, P < .01). There was no change in median nerve sensory and motor conduction velocities in the EM. Distal motor latency was decreased (P < .01) in both groups. A baseline assessment revealed no group differences in pain severity, symptom severity, or functional status. Immediately after therapy, analysis of variance revealed group differences in pain severity (P < .01), with a reduction in pain in both groups (MT: 290%, P < .01; EM: 47%, P < .01). There were group differences in symptom severity (P < .01) and function (P < .01) on the Boston Carpal Tunnel Questionnaire. Both groups had an improvement in functional status (MT: 47%, P < .01; EM: 9%, P < .01) and a reduction in subjective CTS symptoms (MT: 67%, P < .01; EM: 15%, P < .01). Both therapies had a positive effect on nerve conduction, pain reduction, functional status, and subjective symptoms in individuals with CTS. However, the results regarding pain reduction, subjective symptoms, and functional status were better in the MT group. Copyright © 2017. Published by Elsevier Inc.

[Congenital mydriasis as an initial sign of septo-optic dysplasia].

PubMed

Carrascosa-Romero, M C; Ruiz-Cano, R; Martínez-López, F; Alfaro-Ponce, B; Pérez-Pardo, A

2013-10-01

Septo-optic dysplasia (SOD)[MIM182230] consisting of a heterogeneous and uncommon condition characterised by the classictriad: optic nerve hypoplasia, abnormalities of pituitary hormone, and defects of thebrain midline (including agenesis of the septum pellucidum and/or the corpus callosum; ithas also been described associated cortical malformations, it was referred to as SOD plus syndrome).We report the first known case in which the initial diagnostic sign of SOD was a bilateralmydriasis as a manifestation ofhypoplasia of both optic nerves, pituitary hypoplasia andcerebral dysgenesis with neuronal migration disorder.We discuss thedifferential diagnosis of congenital mydriasis. Copyright © 2010 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

Modeling the response of small myelinated axons in a compound nerve to kilohertz frequency signals

NASA Astrophysics Data System (ADS)

Pelot, N. A.; Behrend, C. E.; Grill, W. M.

2017-08-01

Objective. There is growing interest in electrical neuromodulation of peripheral nerves, particularly autonomic nerves, to treat various diseases. Electrical signals in the kilohertz frequency (KHF) range can produce different responses, including conduction block. For example, EnteroMedics’ vBloc® therapy for obesity delivers 5 kHz stimulation to block the abdominal vagus nerves, but the mechanisms of action are unclear. Approach. We developed a two-part computational model, coupling a 3D finite element model of a cuff electrode around the human abdominal vagus nerve with biophysically-realistic electrical circuit equivalent (cable) model axons (1, 2, and 5.7 µm in diameter). We developed an automated algorithm to classify conduction responses as subthreshold (transmission), KHF-evoked activity (excitation), or block. We quantified neural responses across kilohertz frequencies (5-20 kHz), amplitudes (1-8 mA), and electrode designs. Main results. We found heterogeneous conduction responses across the modeled nerve trunk, both for a given parameter set and across parameter sets, although most suprathreshold responses were excitation, rather than block. The firing patterns were irregular near transmission and block boundaries, but otherwise regular, and mean firing rates varied with electrode-fibre distance. Further, we identified excitation responses at amplitudes above block threshold, termed ‘re-excitation’, arising from action potentials initiated at virtual cathodes. Excitation and block thresholds decreased with smaller electrode-fibre distances, larger fibre diameters, and lower kilohertz frequencies. A point source model predicted a larger fraction of blocked fibres and greater change of threshold with distance as compared to the realistic cuff and nerve model. Significance. Our findings of widespread asynchronous KHF-evoked activity suggest that conduction block in the abdominal vagus nerves is unlikely with current clinical parameters. Our results indicate that compound neural or downstream muscle force recordings may be unreliable as quantitative measures of neural activity for in vivo studies or as biomarkers in closed-loop clinical devices.

Modeling the response of small myelinated axons in a compound nerve to kilohertz frequency signals.

PubMed

Pelot, N A; Behrend, C E; Grill, W M

2017-08-01

There is growing interest in electrical neuromodulation of peripheral nerves, particularly autonomic nerves, to treat various diseases. Electrical signals in the kilohertz frequency (KHF) range can produce different responses, including conduction block. For example, EnteroMedics' vBloc ® therapy for obesity delivers 5 kHz stimulation to block the abdominal vagus nerves, but the mechanisms of action are unclear. We developed a two-part computational model, coupling a 3D finite element model of a cuff electrode around the human abdominal vagus nerve with biophysically-realistic electrical circuit equivalent (cable) model axons (1, 2, and 5.7 µm in diameter). We developed an automated algorithm to classify conduction responses as subthreshold (transmission), KHF-evoked activity (excitation), or block. We quantified neural responses across kilohertz frequencies (5-20 kHz), amplitudes (1-8 mA), and electrode designs. We found heterogeneous conduction responses across the modeled nerve trunk, both for a given parameter set and across parameter sets, although most suprathreshold responses were excitation, rather than block. The firing patterns were irregular near transmission and block boundaries, but otherwise regular, and mean firing rates varied with electrode-fibre distance. Further, we identified excitation responses at amplitudes above block threshold, termed 're-excitation', arising from action potentials initiated at virtual cathodes. Excitation and block thresholds decreased with smaller electrode-fibre distances, larger fibre diameters, and lower kilohertz frequencies. A point source model predicted a larger fraction of blocked fibres and greater change of threshold with distance as compared to the realistic cuff and nerve model. Our findings of widespread asynchronous KHF-evoked activity suggest that conduction block in the abdominal vagus nerves is unlikely with current clinical parameters. Our results indicate that compound neural or downstream muscle force recordings may be unreliable as quantitative measures of neural activity for in vivo studies or as biomarkers in closed-loop clinical devices.

Pathophysiology of Small-Fiber Sensory System in Parkinson's Disease

PubMed Central

Lin, Chin-Hsien; Chao, Chi-Chao; Wu, Shao-Wei; Hsieh, Paul-Chen; Feng, Fang-Ping; Lin, Yea-Huey; Chen, Ya-Mei; Wu, Ruey-Meei; Hsieh, Sung-Tsang

2016-01-01

Abstract Sensory symptoms are frequent nonmotor complaints in patients with Parkinson's disease (PD). However, few investigations integrally explored the physiology and pathology of the thermonociceptive pathway in PD. We aim to investigate the involvement of the thermonociceptive pathway in PD. Twenty-eight PD patients (16 men, with a mean age and standard deviation of 65.6 ± 10.7 years) free of neuropathic symptoms and systemic disorders were recruited for the study and compared to 23 age- and gender-matched control subjects (12 men, with a mean age and standard deviation of 65.1 ± 9.9 years). We performed skin biopsy, contact heat-evoked potential (CHEP), and quantitative sensory tests (QST) to study the involvement of the thermonociceptive pathway in PD. The duration of PD was 7.1 ± 3.2 (range 2–17 years) years and the UPDRS part III score was 25.6 ± 9.7 (range 10–48) during the off period. Compared to control subjects, PD patients had reduced intra-epidermal nerve fiber (IENF) density (2.48 ± 1.65 vs 6.36 ± 3.19 fibers/mm, P < 0.001) and CHEP amplitude (18.02 ± 10.23 vs 33.28 ± 10.48 μV, P < 0.001). Twenty-three patients (82.1%) had abnormal IENF densities and 18 (64.3%) had abnormal CHEP. Nine patients (32.1%) had abnormal thermal thresholds in the feet. In total 27 patients (96.4%) had at least 1 abnormality in IENF, CHEP, or thermal thresholds of the foot, indicating dysfunctions in the small-fiber nerve system. In control subjects, CHEP amplitude linearly correlated with IENF density (P < 0.001). In contrast, this relationship disappeared in PD (P = 0.312) and CHEP amplitude was negatively correlated with motor severity of PD independent of age, gender, and anti-PD medication dose (P = 0.036), suggesting the influences of central components on thermonociceptive systems in addition to peripheral small-fiber nerves in PD. The present study suggested impairment of small-fiber sensory system at both peripheral and central levels is an intrinsic feature of PD, and skin biopsy, CHEP, and QST provided an integral approach for assessing such dysfunctions. PMID:26962835

Morphological and Functional Attenuation of Degeneration of Peripheral Neurons by Mesenchymal Stem Cell-Conditioned Medium in Spinocerebellar Ataxia Type 1-Knock-in Mice.

PubMed

Suto, Nana; Mieda, Tokue; Iizuka, Akira; Nakamura, Kazuhiro; Hirai, Hirokazu

2016-08-01

Spinocerebellar ataxia type 1 (SCA1) is caused by the ataxin-1 protein (ATXN1) with an abnormally expanded polyglutamine tract and is characterized by progressive neurodegeneration. We previously showed that intrathecal injection of mesenchymal stem cells (MSCs) during the nonsymptomatic stage mitigates the degeneration of the peripheral nervous system (PNS) neurons in SCA1-knock-in (SCA1-KI) mice. We tested in this study whether the therapeutic effects of MSCs in SCA1-KI mice could be reproduced with MSC-releasing factor(s). To test the effects of MSC-releasing factor(s), we used MSC-conditioned medium (MSC-CM). MSC-CM was intrathecally and/or intravenously injected into young SCA1-KI mice, and the therapeutic effects were assessed in the PNS at later ages using immunostaining, electrophysiology, and behavioral tests. MSC-CM attenuated the degeneration of axons and myelin of spinal motor neurons. Consequently, the injected SCA1-KI mice exhibited smaller reductions in nerve conduction velocity in spinal motor neurons and reduced motor incoordination than the untreated mice. These results suggest that factors released from MSC mitigate the morphological and functional abnormalities in the PNS that are observed in SCA1-KI mice in a paracrine manner. © 2016 John Wiley & Sons Ltd.

Juvenile-onset distal myopathy in Rottweiler dogs.

PubMed

Hanson, S M; Smith, M O; Walker, T L; Shelton, G D

1998-01-01

Two juvenile Rottweiler siblings were presented with the complaint of decreased activity and various postural abnormalities, including plantigrade and palmigrade stance and splayed forepaw digits. The neurologic examinations were otherwise normal. Electromyography revealed rare fibrillation potentials and positive sharp waves. Motor nerve conduction velocities were normal, whereas compound muscle action potentials from the interosseous muscles were decreased. These findings were consistent with a primary myopathy. A 3rd pup from a different litter and a 4th pup from a litter with 3 of 8 affected dogs had similar clinical presentations. Histopathologic changes in fresh-frozen muscle biopsy samples were similar in all pups and consisted of myofiber atrophy with mild myonecrosis, endomysial fibrosis and replacement of muscle with fatty tissue. These changes were more severe in distal muscles than in proximal muscles. Plasma carnitine concentrations (total and free) were decreased in all pups. Muscle carnitine concentrations (total and free) were decreased in 3 of 4 pups and the least affected pup had a borderline low free muscle carnitine concentration. Abnormalities involving major metabolic pathways were not found on quantification of organic and amino acids. Dystrophin immunocytochemistry was normal in 2 dogs tested. Distal myopathies in humans are classified under the dystrophic group of muscle disorders. These 4 cases represent a form of muscular dystrophy apparently not previously reported in dogs.

Influence of limb temperature on cutaneous silent periods.

PubMed

Kofler, Markus; Valls-Solé, Josep; Vasko, Peter; Boček, Václav; Štetkárová, Ivana

2014-09-01

The cutaneous silent period (CSP) is a spinal inhibitory reflex mediated by small-diameter afferents (A-delta fibers) and large-diameter efferents (alpha motoneurons). The effect of limb temperature on CSPs has so far not been assessed. In 27 healthy volunteers (11 males; age 22-58 years) we recorded median nerve motor and sensory action potentials, median nerve F-wave and CSPs induced by noxious digit II stimulation in thenar muscles in a baseline condition at room temperature, and after randomly submersing the forearm in 42 °C warm or 15 °C cold water for 20 min each. In cold limbs, distal and proximal motor and sensory latencies as well as F-wave latencies were prolonged. Motor and sensory nerve conduction velocities were reduced. Compound motor and sensory nerve action potential amplitudes did not differ significantly from baseline. CSP onset and end latencies were more delayed than distal and proximal median nerve motor and sensory latencies, whereas CSP duration was not affected. In warm limbs, opposite but smaller changes were seen in nerve conduction studies and CSPs. The observed CSP shift "en bloc" towards longer latencies without affecting CSP duration during limb cooling concurs with slower conduction velocity in both afferent and efferent fibers. Disparate conduction slowing in afferents and efferents, however, suggests that nociceptive EMG suppression is mediated by fibers of different size in the afferent than in the efferent arm, indirectly supporting the contribution of A-delta fibers as the main afferent input. Limb temperature should be taken into account when testing CSPs in the clinical setting, as different limb temperatures affect CSP latencies more than large-diameter fiber conduction function. Copyright © 2014 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

Chronic nerve compression alters Schwann cell myelin architecture in a murine model

PubMed Central

Gupta, Ranjan; Nassiri, Nima; Hazel, Antony; Bathen, Mary; Mozaffar, Tahseen

2011-01-01

Introduction Myelinating Schwann cells compartmentalize their outermost layer to form actin-rich channels known as Cajal bands. Here, we investigate changes in Schwann cell architecture and cytoplasmic morphology in a novel mouse model of carpal tunnel syndrome. Methods Chronic nerve compression (CNC) injury was created in wild-type and slow-Wallerian degeneration (WldS) mice. Over 12 weeks, nerves were electrodiagnostically assessed, and Schwann cell morphology was thoroughly evaluated. Results A decline in nerve conduction velocity and increase in g-ratio is observed without early axonal damage. Schwann cells display shortened internodal lengths and severely disrupted Cajal bands. Quite surprisingly, the latter is reconstituted without improvements to nerve conduction velocity. Discussion Chronic entrapment injuries like carpal tunnel syndrome are primarily mediated by the Schwann cell response, wherein decreases in internodal length and myelin thickness disrupt the efficiency of impulse propagation. Restitution of Cajal bands is not sufficient for remyelination post-CNC injury. PMID:22246880

Abnormal electroretinogram associated with developmental brain anomalies.

PubMed Central

Cibis, G W; Fitzgerald, K M

1995-01-01

PURPOSE: We have encountered abnormal ERGs associated with optic nerve hypoplasia, macular, optic nerve and chorioretinal colobomata and developmental brain anomalies. Brain anomalies include cortical dysgenesis, lissencephaly, porencephaly, cerebellar and corpus callosum hypoplasia. We describe six exemplar cases. METHODS: Scotopic and photopic ERGs adherent to international standards were performed as well as photopic ERGs to long-duration stimuli. CT or MRI studies were also done. The ERGs were compared to age-matched normal control subjects. RESULTS: ERG changes include reduced amplitude b-waves to blue and red stimuli under scotopic testing conditions. Implicit times were often delayed. The photopic responses also showed reduced amplitude a- and b-waves with implicit time delays. The long-duration photopic ERG done in one case shows attenuation of both ON- and OFF-responses. CONCLUSIONS: Common underlying developmental genetic or environmental unifying casualties are speculated to be at fault in causing these cases of associated retinal and brain abnormalities. No single etiology is expected. Multiple potential causes acting early in embryogenesis effecting neuronal induction, migration and differentiation are theorized. These occur at a time when brain and retinal cells are sufficiently undifferentiated to be similarly effected. We call these cases examples of Brain Retina Neuroembryodysgenesis (BRNED). Homeobox and PAX genes with global neuronal developmental influences are gene candidates to unify the observed disruption of brain and retinal cell development. The ERG can provide a valuable clinical addition in understanding and ultimately classifying these disorders. Images FIGURE 1 FIGURE 4 FIGURE 5 FIGURE 6 FIGURE 7 FIGURE 8 PMID:8719676

Reproducibility of retinal nerve fiber layer thickness measures using eye tracking in children with nonglaucomatous optic neuropathy.

PubMed

Rajjoub, Raneem D; Trimboli-Heidler, Carmelina; Packer, Roger J; Avery, Robert A

2015-01-01

To determine the intra- and intervisit reproducibility of circumpapillary retinal nerve fiber layer (RNFL) thickness measures using eye tracking-assisted spectral-domain optical coherence tomography (SD OCT) in children with nonglaucomatous optic neuropathy. Prospective longitudinal study. Circumpapillary RNFL thickness measures were acquired with SD OCT using the eye-tracking feature at 2 separate study visits. Children with normal and abnormal vision (visual acuity ≥ 0.2 logMAR above normal and/or visual field loss) who demonstrated clinical and radiographic stability were enrolled. Intra- and intervisit reproducibility was calculated for the global average and 9 anatomic sectors by calculating the coefficient of variation and intraclass correlation coefficient. Forty-two subjects (median age 8.6 years, range 3.9-18.2 years) met inclusion criteria and contributed 62 study eyes. Both the abnormal and normal vision cohort demonstrated the lowest intravisit coefficient of variation for the global RNFL thickness. Intervisit reproducibility remained good for those with normal and abnormal vision, although small but statistically significant increases in the coefficient of variation were observed for multiple anatomic sectors in both cohorts. The magnitude of visual acuity loss was significantly associated with the global (ß = 0.026, P < .01) and temporal sector coefficient of variation (ß = 0.099, P < .01). SD OCT with eye tracking demonstrates highly reproducible RNFL thickness measures. Subjects with vision loss demonstrate greater intra- and intervisit variability than those with normal vision. Copyright © 2015 Elsevier Inc. All rights reserved.

Spine Topographical Distribution of Skin α-Synuclein Deposits in Idiopathic Parkinson Disease.

PubMed

Donadio, Vincenzo; Incensi, Alex; Rizzo, Giovanni; Scaglione, Cesa; Capellari, Sabina; Fileccia, Enrico; Avoni, Patrizia; Liguori, Rocco

2017-05-01

Phosphorylated α-synuclein (p-syn) in skin nerves mainly in the proximal sites is a promising neurodegenerative biomarker for idiopathic Parkinson disease (IPD). However, the p-syn spine distribution particularly in patients with unilateral motor dysfunctions remains undefined. This study aimed to investigate in IPD p-syn differences between left and right cervical spine sites in patients with prevalent unilateral motor symptoms, and cervical and thoracic spine sites in patients with bilateral motor symptoms. We enrolled 28 IPD patients fulfilling clinical diagnostic criteria associated with abnormal nigro-striatal DatScan and cardiac MIBG: 15 with prevalently unilateral motor symptoms demonstrated by DatScan; 13 with bilateral motor symptoms and DatScan abnormalities. Patients underwent skin biopsy searching for intraneural p-syn deposits: skin samples were taken from C7 paravertebral left and right sites in unilateral patients and from cervical (C7) and thoracic (Th12) paravertebral spine regions in bilateral patients. Unilateral patients displayed 20% of abnormal p-syn deposits in the affected motor site, 60% in both sites and 20% only in the non-affected site. P-syn was found in all patients in C7 but in only 62% of patients in Th12. Our data showed that cervical p-syn deposits displayed a uniform distribution between both sides not following the motor dysfunction in unilateral patients, and skin nerve p-syn deposits demonstrated a spine gradient with the cervical site expressing the highest positivity. © 2017 American Association of Neuropathologists, Inc. All rights reserved.

Chitin biological absorbable catheters bridging sural nerve grafts transplanted into sciatic nerve defects promote nerve regeneration.

PubMed

Wang, Zhi-Yong; Wang, Jian-Wei; Qin, Li-Hua; Zhang, Wei-Guang; Zhang, Pei-Xun; Jiang, Bao-Guo

2018-06-01

To investigate the efficacy of chitin biological absorbable catheters in a rat model of autologous nerve transplantation. A segment of sciatic nerve was removed to produce a sciatic nerve defect, and the sural nerve was cut from the ipsilateral leg and used as a graft to bridge the defect, with or without use of a chitin biological absorbable catheter surrounding the graft. The number and morphology of regenerating myelinated fibers, nerve conduction velocity, nerve function index, triceps surae muscle morphology, and sensory function were evaluated at 9 and 12 months after surgery. All of the above parameters were improved in rats in which the nerve graft was bridged with chitin biological absorbable catheters compared with rats without catheters. The results of this study indicate that use of chitin biological absorbable catheters to surround sural nerve grafts bridging sciatic nerve defects promotes recovery of structural, motor, and sensory function and improves muscle fiber morphology. © 2018 John Wiley & Sons Ltd.

Effect of Enriched (Complex) Environment on Nerve Conduction Velocity: New Data and Review of Implications for the Speed of Information Processing.

ERIC Educational Resources Information Center

Reed, T. Edward

1993-01-01

Results with 54 mice confirm that increased stimulation or usage, as would be provided by environmental enrichment (EE), increases peripheral nerve conduction velocity. These results suggest a role at the physiological level for EE (or deprivation) in affecting measured intelligence. (SLD)

Conduction studies of the normal sural nerve.

PubMed

Horowitz, S H; Krarup, C

1992-03-01

The sural nerve was studied orthodromically using the near-nerve technique in 273 normal subjects (155 females, 118 males) aged 5 to 90 years. The sensory action potential (SAP), evoked at the dorsum of the foot, was recorded at the lateral malleolus and midcalf, and at the midcalf when evoked at the lateral malleolus. In addition, the SAP was recorded at intermediate distal sites and at proximal sites at the popliteal fossa, the gluteal fold, and the S-1 root. The amplitude of the SAP recorded at midcalf was 32% higher in females than in males. This was probably due to volume-conduction properties, as differences between genders were less noticeable at more distal recording sites. The amplitude decreased steeply and exponentially with age. Conduction distance had a strong influence on the amplitude of the SAP, which decreased with increasing distance following a power relationship with an exponent of 1.4 to 1.7. This decrease was due to temporal dispersion with decreased summation and increased phase cancellation. The conduction velocity was slightly lower along the very distal course of the nerve than along more proximal segments.

Experiences with preventing carpal tunnel syndrome in an automotive plant.

PubMed

Žídková, Věra; Nakládalová, Marie; Zapletalová, Jana; Nakládal, Zdeněk; Kollárová, Helena

2017-02-21

Carpal tunnel syndrome (CTS) is a common occupational disease. The aim was to assess the effect of preventive measures in automotive assembly workers. The analysis summarizes data from annual crosssectional studies. The 7-year analysis of data was based on medical records obtained from an occupational physician and inspections carried out at the workplace where targeted preventive measures were introduced, including better ergonomic arrangement of the workplace, technical adjustments facilitating the work, preventive nerve conduction studies (NCS) testing of the median nerve once a year, switching of workers and their targeted rotation within the workplace. The NCS testing of median nerve conduction at the wrist was the basic objective method for assessment of the prevalence and severity of CTS. Over the study period, the sample comprised 1804 workers at risk for repetitive overuse of the upper extremities, of whom 281 were females with a mean age of 38.5 years and 1523 were males with a mean age of 31.4 years. Over the study period, a total of 13 cases of CTS were recognized as an occupational disease in the plant, 8 of which occurred within the first 2 years from the initiation of production. Introduction of preventive measures decreased the prevalence of median neuropathy from 18.3% of examined extremities in 2011 to 10.5% in 2013 (p = 0.003). In early 2014, the production pace increased and this was accompanied by a rise in abnormal NCS findings to 16.9%. Over the study period, the rate of sensorimotor neuropathy decreased in favor of merely sensory neuropathies, which have been most frequent since 2013. The percentage of employees whose contracts were terminated due to median neuropathy decreased steadily from 5.5% to 0.4%. Targeted prevention of work-related CTS is effective as evidenced by the decrease in the prevalence of median neuropathy detected by NCS. Int J Occup Med Environ Health 2017;30(1):45-54. This work is available in Open Access model and licensed under a CC BY-NC 3.0 PL license.

Effect of diet induced obesity or type 1 or type 2 diabetes on corneal nerves and peripheral neuropathy in C57Bl/6J mice

PubMed Central

Yorek, Matthew S.; Obrosov, Alexander; Shevalye, Hanna; Holmes, Amey; Harper, Matthew M.; Kardon, Randy H.; Yorek, Mark A.

2015-01-01

We determined the impact diet induced obesity (DIO) and types 1 and 2 diabetes has on peripheral neuropathy with emphasis on corneal nerve structural changes in C57Bl/6J mice. Endpoints examined included nerve conduction velocity, response to thermal and mechanical stimuli and innervation of the skin and cornea. DIO mice and to a greater extent type 2 diabetic mice were insulin resistant. DIO and both types 1 and 2 diabetic mice developed motor and sensory nerve conduction deficits. In the cornea of DIO and type 2 diabetic mice there was a decrease in sub-epithelial corneal nerves, innervation of the corneal epithelium and corneal sensitivity. Type 1 diabetic mice did not present with any significant changes in corneal nerve structure until after 20 weeks of hyperglycemia. DIO and type 2 diabetic mice developed corneal structural damage more rapidly than type 1 diabetic mice even though hemoglobin A1C values were significantly higher in type 1 diabetic mice. This suggests that DIO with or without hyperglycemia contributes to development and progression of peripheral neuropathy and nerve structural damage in the cornea. PMID:25858759

Comparison of four different nerve conduction techniques of the superficial fibular sensory nerve.

PubMed

Saffarian, Mathew R; Condie, Nathan C; Austin, Erica A; Mccausland, Katie E; Andary, Michael T; Sylvain, James R; Mull, Iian R; Zemper, Eric D; Jannausch, Mary L

2017-09-01

There are many different nerve conduction study (NCS) techniques to study the superficial fibular sensory nerve (SFSN). We present reference distal latency values and comparative data regarding 4 different NCS for the SFSN. Four different NCS techniques, Spartan technique, Izzo techniques (medial and intermediate dorsal cutaneous branches), and Daube technique, were performed on (114) healthy volunteers. A total of 108 subjects with 164 legs were included. The mean latency of the Spartan technique was longest (3.9 ± 0.3 ms) while the Daube technique was the shortest (3.6 ± 0.7 ms). The mean amplitude of the Daube technique displayed the highest (15.2 ± 8.2 μV) with the Spartan technique having the lowest (8.7 ± 4.2 μV). Among the absent sensory nerve action potentials (SNAPs), the Spartan technique was absent only twice (1.2%) and the Izzo Medial technique was absent more than the other techniques (2.9%). All 4 techniques were reliable methods for obtaining the superficial fibular nerve SNAP, present in 95% of individuals. Muscle Nerve 56: 458-462, 2017. © 2017 Wiley Periodicals, Inc.

A novel method for measuring hydraulic conductivity at the human blood-nerve barrier in vitro.

PubMed

Helton, E Scott; Palladino, Steven; Ubogu, Eroboghene E

2017-01-01

Microvascular barrier permeability to water is an essential biophysical property required for the homeostatic maintenance of unique tissue microenvironments. This is of particular importance in peripheral nerves where strict control of ionic concentrations is needed for axonal signal transduction. Previous studies have associated inflammation, trauma, toxin exposure and metabolic disease with increases in water influx and hydrostatic pressure in peripheral nerves with resultant endoneurial edema that may impair axonal function. The regulation of water permeability across endoneurial microvessels that form the blood-nerve barrier (BNB) is poorly understood. Variations exist in apparatus and methods used to measure hydraulic conductivity. The objective of the study was to develop a simplified hydraulic conductivity system using commercially available components to evaluate the BNB. We determined the mean hydraulic conductivity of cultured confluent primary and immortalized human endoneurial endothelial cell layers as 2.00×10 -7 and 2.17×10 -7 cm/s/cm H₂O respectively, consistent with restrictive microvascular endothelial cells in vitro. We also determined the mean hydraulic conductivity of immortalized human brain microvascular endothelial cell layers, a commonly used blood-brain barrier (BBB) cell line, as 0.20×10 -7 cm/s/cm H₂O, implying a mean 10-fold higher resistance to transendothelial water flux in the brain compared to peripheral nerves. To our knowledge, this is the first reported measurement of human BNB and BBB hydraulic conductivities. This model represents an important tool to further characterize the human BNB and deduce the molecular determinants and signaling mechanisms responsible for BNB hydraulic conductivity in normal and disease states in vitro. Copyright © 2016 Elsevier Inc. All rights reserved.

Impact of mild traumatic brain injury on auditory brain stem dysfunction in mouse model.

PubMed

Amanipour, Reza M; Frisina, Robert D; Cresoe, Samantha A; Parsons, Teresa J; Xiaoxia Zhu; Borlongan, Cesario V; Walton, Joseph P

2016-08-01

The auditory brainstem response (ABR) is an electrophysiological test that examines the functionality of the auditory nerve and brainstem. Traumatic brain injury (TBI) can be detected if prolonged peak latency is observed in ABR measurements, since latency measures the neural conduction time in the brainstem, and an increase in latency can be a sign of pathological lesion at the auditory brainstem level. The ABR is elicited by brief sounds that can be used to measure hearing sensitivity as well as temporal processing. Reduction in peak amplitudes and increases in latency are indicative of dysfunction in the auditory nerve and/or central auditory pathways. In this study we used sixteen young adult mice that were divided into two groups: sham and mild traumatic brain injury (mTBI), with ABR measurements obtained prior to, and at 2, 6, and 14 weeks after injury. Abnormal ABRs were observed for the nine TBI cases as early as two weeks after injury and the deficits lasted for fourteen weeks after injury. Results indicated a significant reduction in the Peak 1 (P1) and Peak 4 (P4) amplitudes to the first noise burst, as well as an increase in latency response for P1 and P4 following mTBI. These results are the first to demonstrate auditory sound processing deficits in a rodent model of mild TBI.

Rhabdomyolysis associated with initiation of febuxostat therapy for hyperuricaemia in a patient with chronic kidney disease.

PubMed

Kang, Y; Kim, M J; Jang, H N; Bae, E J; Yun, S; Cho, H S; Chang, S-H; Park, D J

2014-06-01

Febuxostat is now recommended as the first-line pharmacological urate-lowering therapy for gout in the American College of Rheumatology guidelines. There is no case of rhabdomyolysis associated with febuxostat among reported side effects of the drug. Our objective is to report on a case of rhabdomyolysis associated with initiation of febuxostat in a patient with chronic kidney disease (CKD). A 73-year-old male patient visited our emergency room due to progressive weakness in both lower extremities starting 3 days earlier. Ten days before presentation, his primary physician had changed his prescription from allopurinol to febuxostat (80 mg) because of poor control of uric acid levels. There was tenderness in both thighs. Initial creatinine kinase (CK) was 7652 U/L (0-170 U/L), and a bone scan using (99m) Tc-HDP revealed strong uptake in soft tissues in both thighs and buttocks. Electromyography (EMG) and nerve conduction velocity (NCV) showed abnormal spontaneous activities (ASA), suggesting myopathy, not nerve damage. On day 7 of admission, after conservative management and febuxostat withdrawal, he could walk on the ward. He is being followed in our clinic as an outpatient with no sequelae. This report is first case of rhabdomyolysis associated with initiation of febuxostat. Febuxostat should be withdrawn when rhabdomyolysis is confirmed. © 2014 John Wiley & Sons Ltd.

Xeroderma pigmentosum neurological abnormalities correlate with colony-forming ability after ultraviolet radiation

DOE Office of Scientific and Technical Information (OSTI.GOV)

Andrews, A.D.; Barrett, S.F.; Robbins, J.H.

1978-04-01

Xeroderma pigmentosum is an autosomal recessive disease in which DNA repair processes are defective. All xeroderma pigmentosum patients develop premature aging of sun exposed skin, and some develop neurological abnormalities due to premature death of nerve cells. Sensitivity to ultraviolet radiation of 24 xeroderma pigmentosum fibroblast strains was studied in vitro by measuring each strain's ability to divide and form colonies after irradiation. The most sensitive strains were derived from patients who had an early onset of neurological abnormalities; less sensitive strains were from patients with a later onset; and the most resistant strains were from patients without neurological abnormalities.more » The uv sensitivities of strains from each member of a sibling pair with xeroderma pigmentosum were identical, indicating that uv sensitivity of xeroderma pigmentosum strains is determined by the patient's inherited DNA repair defect. The results suggest that effective DNA repair is required to maintain the functional integrity of the human nervous system by preventing premature death of neurons.« less

Patellar tendon vibration reduces the increased facilitation from quadriceps to soleus in post-stroke hemiparetic individuals.

PubMed

Maupas, Eric; Dyer, Joseph-Omer; Melo, Sibele de Andrade; Forget, Robert

2017-09-01

Stimulation of the femoral nerve in healthy people can facilitate soleus H-reflex and electromyography (EMG) activity. In stroke patients, such facilitation of transmission in spinal pathways linking the quadriceps and soleus muscles is enhanced and related to co-activation of knee and ankle extensors while sitting and walking. Soleus H-reflex facilitation can be depressed by vibration of the quadriceps in healthy people, but the effects of such vibration have never been studied on the abnormal soleus facilitation observed in people after stroke. To determine whether vibration of the quadriceps can modify the enhanced heteronymous facilitation of the soleus muscle observed in people with spastic stroke after femoral nerve stimulation and compare post-vibration effects on soleus facilitation in control and stroke individuals. Modulation of voluntary soleus EMG activity induced by femoral nerve stimulation (2×motor threshold) was assessed before, during and after vibration of the patellar tendon in 10 healthy controls and 17 stroke participants. Voluntary soleus EMG activity was facilitated by femoral nerve stimulation in 4/10 (40%) controls and 11/17 (65%) stroke participants. The level of facilitation was greater in the stroke than control group. Vibration significantly reduced early heteronymous facilitation in both groups (50% of pre-vibration values). However, the delay in recovery of soleus facilitation after vibration was shorter for the stroke than control group. The control condition with the vibrator turned off had no effect on the modulation. Patellar tendon vibration can reduce the facilitation between knee and ankle extensors, which suggests effective presynaptic inhibition but decreased post-activation depression in the lower limb of people after chronic hemiparetic stroke. Further studies are warranted to determine whether such vibration could be used to reduce the abnormal extension synergy of knee and ankle extensors in people after hemiparetic stroke. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Sensitivity and specificity of diagnostic ultrasound in the diagnosis of phrenic neuropathy.

PubMed

Boon, Andrea J; Sekiguchi, Hiroshi; Harper, Caitlin J; Strommen, Jeffrey A; Ghahfarokhi, Leili S; Watson, James C; Sorenson, Eric J

2014-09-30

To determine the sensitivity and specificity of B-mode ultrasound in the diagnosis of neuromuscular diaphragmatic dysfunction, including phrenic neuropathy. A prospective study of patients with dyspnea referred to the EMG laboratory over a 2-year time frame for evaluation of neuromuscular respiratory failure who were recruited consecutively and examined with ultrasound for possible diaphragm dysfunction. Sonographic outcome measures were absolute thickness of the diaphragm and degree of increased thickness with maximal inspiration. The comparison standard for diagnosis of diaphragm dysfunction was the final clinical diagnosis of clinicians blinded to the diaphragm ultrasound results, but taking into account other diagnostic workup, including chest radiographs, fluoroscopy, phrenic nerve conduction studies, diaphragm EMG, and/or pulmonary function tests. Of 82 patients recruited over a 2-year period, 66 were enrolled in the study. Sixteen patients were excluded because of inconclusive or insufficient reference testing. One hemidiaphragm could not be adequately visualized; therefore, hemidiaphragm assessment was conducted in a total of 131 hemidiaphragms in 66 patients. Of the 82 abnormal hemidiaphragms, 76 had abnormal sonographic findings (atrophy or decreased contractility). Of the 49 normal hemidiaphragms, none had a false-positive ultrasound. Diaphragmatic ultrasound was 93% sensitive and 100% specific for the diagnosis of neuromuscular diaphragmatic dysfunction. B-mode ultrasound imaging of the diaphragm is a highly sensitive and specific tool for diagnosis of neuromuscular diaphragm dysfunction. This study provides Class II evidence that diaphragmatic ultrasound performed by well-trained individuals accurately identifies patients with neuromuscular diaphragmatic respiratory failure (sensitivity 93%; specificity 100%). © 2014 American Academy of Neurology.

Utility of Brainstem Trigeminal Evoked Potentials in Patients With Primary Trigeminal Neuralgia Treated by Microvascular Decompression.

PubMed

Zhu, Jin; Zhang, Xin; Zhao, Hua; Tang, Yin-Da; Ying, Ting-Ting; Li, Shi-Ting

2017-09-01

To investigate the characteristics of brainstem trigeminal evoked potentials (BTEP) waveform in patients with and without trigeminal neuralgia (TN), and to discuss the utility of BTEP in patients with primary TN treated by microvascular decompression (MVD). A retrospective review of 43 patients who underwent BTEP between January 2016 and June 2016, including 33 patients with TN who underwent MVD and 10 patients without TN. Brainstem trigeminal evoked potentials characteristics of TN and non-TN were summarized, in particular to compare the BTEP changes between pre- and post-MVD, and to discover the relationship between BTEP changes and surgical outcome. Brainstem trigeminal evoked potentials can be recorded in patients without trigeminal neuralgia. Abnormal BTEP could be recorded when different branches were stimulated. After decompression, the original W2, W3 disappeared and then replaced by a large wave in most patients, or original wave poorly differentiated improved in some patients, showed as shorter latency and (or) amplitude increased. Brainstem trigeminal evoked potentials waveform of healthy side in patients with trigeminal neuralgia was similar to the waveform of patients without TN. In 3 patients, after decompression the W2, W3 peaks increased, and the latency, duration, IPLD did not change significantly. Until discharge, 87.9% (29/33) of the patients presented complete absence of pain without medication (BNI I) and 93.9% (31/33) had good pain control without medication (BNI I-II). Brainstem trigeminal evoked potentials can reflect the conduction function of the trigeminal nerve to evaluate the functional level of the trigeminal nerve conduction pathway. The improvement and restoration of BTEP waveforms are closely related to the postoperative curative effect.

Peripheral neuropathy in liver cirrhosis.

PubMed

Kharbanda, Parampreet S; Prabhakar, Sudesh; Chawla, Yogesh K; Das, Chandi P; Syal, Puneet

2003-08-01

Neuropathy in association with chronic liver disease, including cirrhosis, is recognized; however, there are differences in the incidence and type of neuropathy reported. The causal relationship of liver disease to neuropathy has been questioned. This study was designed to evaluate the incidence and character of peripheral neuropathy in patients with liver cirrhosis. The effect of alcohol consumption, severity of liver disease and encephalopathy on the incidence and severity of neuropathy were also studied. Patients having an identifiable cause of peripheral neuropathy, except alcohol, were excluded from the study. Patients with evidence of vitamin B12 deficiency or diabetes were also excluded from the study. In this study, 33 patients with liver cirrhosis were evaluated clinically and electrophysiologically to detect any evidence of peripheral neuropathy. Nerve conduction studies were performed in the upper and lower limbs using surface electrodes. These patients also underwent a detailed clinical examination. Clinical signs of peripheral neuropathy were found in seven (21%) patients. Nerve conduction studies were abnormal in 24 (73%) patients. The pattern of involvement was predominantly of an axonal sensory motor polyneuropathy. Neuropathy was found both in patients with alcohol-related and non-alcohol-related cirrhosis. The presence of encephalopathy did not have a significant bearing on the incidence and severity of neuropathy. The neuropathy was also not significantly related to the severity of liver disease. The present study reveals that a significant number of patients with liver cirrhosis show evidence of peripheral neuropathy, which is present regardless of the etiology of cirrhosis, and is subclinical in a majority of these patients. The cause of neuropathy was probably the liver disease itself, as the incidence and severity of neuropathy in the alcohol-related cirrhosis, although higher, was not significantly different from the neuropathy in patients with non-alcohol-related cirrhosis.

Guillain-Barré syndrome: clinical profile and management

PubMed Central

Sudulagunta, Sreenivasa Rao; Sodalagunta, Mahesh Babu; Sepehrar, Mona; Khorram, Hadi; Bangalore Raja, Shiva Kumar; Kothandapani, Shyamala; Noroozpour, Zahra; Aheta Sham, Mohammed; Prasad, Nagendra; Sunny, Sony Parethu; Mohammed, Munawar Dhanish; Gangadharappa, Rekha; Nidsale Sudarshan, Ranjitha

2015-01-01

Introduction: Guillain-Barré syndrome (GBS) is a fulminant polyradiculoneuropathy that is acute, frequently severe and autoimmune in nature. Etiology of GBS is incompletely understood, prognosis is usually good with early detection and prompt treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and electrodiagnostic features of patients with GBS and mode of management, complications and prognostic factors. Methods: Data of 1,166 patients admitted with GBS or presented to outpatient department (previous medical records) with GBS between January 2003 and January 2014 were analyzed. Results: No difference in genders noted. Around 35% of patients are above 50 years of age. Poor control of diabetes with mean HbA1c of 8.1 ± 2.11 is found on analysis. Seasonal occurrence in GBS is prominent in winter 484 (41.50%) and mechanically ventilated were 449 (38.50%) patients. 48 (4.11%) deaths were attributed to GBS. Neurological analysis revealed cranial nerve involvement in 407 (34.90%) patients, facial palsy in 401 (34.39%) and ataxia in 88 (7.54%) patients. Most patients in plasma exchange group belonged to the lower socio-economic status. Mean cerebrospinal fluid (CSF) protein levels was (n=962) 113.8 ± 11.8 mg/dl. Conduction block determined indirectly by absent H-reflex was noted in 891 (90.64%) patients. No difference in complications and outcome is found in treatment regimens of intravenous immunoglobulin (IVIG) and plasma exchange. Conclusion: Seasonal occurrence predominantly in winter is noted. Peak flow test may be a predictor of assessing requirement of mechanical ventilation and prognosis. Conduction block is the major abnormality noted in electrophysiological studies and proximal nerve segment assessing with Erb’s point stimulation has high predictive value. IVIG treatment is more expensive but is associated with less duration of hospital stay. PMID:26421004

Guillain-Barré syndrome: clinical profile and management.

PubMed

Sudulagunta, Sreenivasa Rao; Sodalagunta, Mahesh Babu; Sepehrar, Mona; Khorram, Hadi; Bangalore Raja, Shiva Kumar; Kothandapani, Shyamala; Noroozpour, Zahra; Aheta Sham, Mohammed; Prasad, Nagendra; Sunny, Sony Parethu; Mohammed, Munawar Dhanish; Gangadharappa, Rekha; Nidsale Sudarshan, Ranjitha

2015-01-01

Guillain-Barré syndrome (GBS) is a fulminant polyradiculoneuropathy that is acute, frequently severe and autoimmune in nature. Etiology of GBS is incompletely understood, prognosis is usually good with early detection and prompt treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and electrodiagnostic features of patients with GBS and mode of management, complications and prognostic factors. Data of 1,166 patients admitted with GBS or presented to outpatient department (previous medical records) with GBS between January 2003 and January 2014 were analyzed. No difference in genders noted. Around 35% of patients are above 50 years of age. Poor control of diabetes with mean HbA1c of 8.1 ± 2.11 is found on analysis. Seasonal occurrence in GBS is prominent in winter 484 (41.50%) and mechanically ventilated were 449 (38.50%) patients. 48 (4.11%) deaths were attributed to GBS. Neurological analysis revealed cranial nerve involvement in 407 (34.90%) patients, facial palsy in 401 (34.39%) and ataxia in 88 (7.54%) patients. Most patients in plasma exchange group belonged to the lower socio-economic status. Mean cerebrospinal fluid (CSF) protein levels was (n=962) 113.8 ± 11.8 mg/dl. Conduction block determined indirectly by absent H-reflex was noted in 891 (90.64%) patients. No difference in complications and outcome is found in treatment regimens of intravenous immunoglobulin (IVIG) and plasma exchange. Seasonal occurrence predominantly in winter is noted. Peak flow test may be a predictor of assessing requirement of mechanical ventilation and prognosis. Conduction block is the major abnormality noted in electrophysiological studies and proximal nerve segment assessing with Erb's point stimulation has high predictive value. IVIG treatment is more expensive but is associated with less duration of hospital stay.

Abducens Nerve in Patients with Type 3 Duane’s Retraction Syndrome

PubMed Central

Hwang, Jeong-Min

2016-01-01

Background We have previously reported that the presence of the abducens nerve was variable in patients with type 3 Duane’s retraction syndrome (DRS), being present in 2 of 5 eyes (40%) and absent in 3 (60%) on magnetic resonance imaging (MRI). The previous study included only 5 eyes with unilateral DRS type 3. Objectives To supplement existing scarce pathologic information by evaluating the presence of the abducens nerve using high resolution thin-section MRI system in a larger number of patients with DRS type 3, thus to provide further insight into the pathogenesis of DRS. Data Extraction A retrospective review of medical records on ophthalmologic examination and high resolution thin-section MRI at the brainstem level and orbit was performed. A total of 31 patients who showed the typical signs of DRS type 3, including abduction and adduction deficit, globe retraction, narrowing of fissure on adduction and upshoot and/or downshoot, were included. The abducens nerve and any other extraocular muscle abnormalities discovered by MRI were noted. Results DRS was unilateral in 26 patients (84%) and bilateral in 5 patients (16%). Two out of 5 bilateral patients had DRS type 3 in the right eye and DRS type 1 in the left eye. Of the 34 affected orbits with DRS type 3 in 31 patients, the abducens nerve was absent or hypoplastic in 31 eyes (91%) and present in 3 eyes (9%). Patients with a present abducens nerve showed more limitation in adduction compared to patients with an absent abducens nerve (P = 0.030). Conclusions The abducens nerve is absent or hypoplastic in 91% of DRS type 3. Patients with a present abducens nerve showed more prominent limitation of adduction. As DRS type 3 partly share the same pathophysiology with type 1 and 2 DRS, the classification of DRS may have to be revised according to MRI findings. PMID:27352171

Neural tissue engineering scaffold with sustained RAPA release relieves neuropathic pain in rats.

PubMed

Ding, Tan; Zhu, Chao; Kou, Zhen-Zhen; Yin, Jun-Bin; Zhang, Ting; Lu, Ya-Cheng; Wang, Li-Ying; Luo, Zhuo-Jing; Li, Yun-Qing

2014-09-01

To investigate the effect of locally slow-released rapamycin (RAPA) from bionic peripheral nerve stent to reduce the incidence of neuropathic pain or mitigate the degree of pain after nerve injury. We constructed a neural tissue engineering scaffold with sustained release of RAPA to repair 20mm defects in rat sciatic nerves. Four presurgical and postsurgical time windows were selected to monitor the changes in the expression of pain-related dorsal root ganglion (DRG) voltage-gated sodium channels 1.3 (Nav1.3), 1.7 (Nav1.7), and 1.8 (Nav1.8) through immunohistochemistry (IHC) and Western Blot, along with the observation of postsurgical pathological pain in rats by pain-related behavior approaches. Relatively small upregulation of DRG sodium channels was observed in the experimental group (RAPA+poly(lactic-co-glycolic acid) (PLGA)+stent) after surgery, along with low degrees of neuropathic pain and anxiety, which were similar to those in the Autologous nerve graft group. Autoimmune inflammatory response plays a leading role in the occurrence of post-traumatic neuropathic pain, and that RAPA significantly inhibits the abnormal upregulation of sodium channels to reduce pain by alleviating inflammatory response. Copyright © 2014 Elsevier Inc. All rights reserved.

ARA 290 improves symptoms in patients with sarcoidosis-associated small nerve fiber loss and increases corneal nerve fiber density.

PubMed

Dahan, Albert; Dunne, Ann; Swartjes, Maarten; Proto, Paolo L; Heij, Lara; Vogels, Oscar; van Velzen, Monique; Sarton, Elise; Niesters, Marieke; Tannemaat, Martijn R; Cerami, Anthony; Brines, Michael

2013-11-08

Small nerve fiber loss and damage (SNFLD) is a frequent complication of sarcoidosis that is associated with autonomic dysfunction and sensory abnormalities, including pain syndromes that severely degrade the quality of life. SNFLD is hypothesized to arise from the effects of immune dysregulation, an essential feature of sarcoidosis, on the peripheral and central nervous systems. Current therapy of sarcoidosis-associated SNFLD consists primarily of immune suppression and symptomatic treatment; however, this treatment is typically unsatisfactory. ARA 290 is a small peptide engineered to activate the innate repair receptor that antagonizes inflammatory processes and stimulates tissue repair. Here we show in a blinded, placebo-controlled trial that 28 d of daily subcutaneous administration of ARA 290 in a group of patients with documented SNFLD significantly improves neuropathic symptoms. In addition to improved patient-reported symptom-based outcomes, ARA 290 administration was also associated with a significant increase in corneal small nerve fiber density, changes in cutaneous temperature sensitivity, and an increased exercise capacity as assessed by the 6-minute walk test. On the basis of these results and of prior studies, ARA 290 is a potential disease-modifying agent for treatment of sarcoidosis-associated SNFLD.