Acoustic reflex patterns in amyotrophic lateral sclerosis.

PubMed

Canale, Andrea; Albera, Roberto; Lacilla, Michelangelo; Canosa, Antonio; Albera, Andrea; Sacco, Francesca; Chiò, Adriano; Calvo, Andrea

2017-02-01

The aim of the study is to investigate acoustic reflex testing in amyotrophic lateral sclerosis patients. Amplitude, latency, and rise time of stapedial reflex were recorded for 500 and 1000 Hz contralateral stimulus. Statistical analysis was performed by the Wilcoxon test and the level of significance was set at 5 %. Fifty-one amyotrophic lateral sclerosis patients and ten sex- and age-matched control subjects were studied. Patients were further divided in two groups: amyotrophic lateral sclerosis-bulbar (38 cases, with bulbar signs at evaluation) and amyotrophic lateral sclerosis-spinal (13 cases, without bulbar signs at evaluation). Stapedial reflex was present in all patients. There was a statistically significant difference in the mean amplitude, latency, and rise time between the amyotrophic lateral sclerosis patients as compared with the controls. Amplitude was lower in both the amyotrophic lateral sclerosis-bulbar and the amyotrophic lateral sclerosis-spinal patients than in the controls (p < 0.05) and rise time was longer in both patient groups compared with the controls (p < 0.05). These results confirm the presence of abnormal acoustic reflex patterns in amyotrophic lateral sclerosis cases with bulbar signs and, moreover, suggesting a possible subclinical involvement of the stapedial motor neuron even in amyotrophic lateral sclerosis-spinal patients. Amplitude and rise time seem to be good sensitive parameters for investigating subclinical bulbar involvement.

[Complications in brachial plexus surgery].

PubMed

Martínez, Fernando; Pinazzo, Samantha; Moragues, Rodrigo; Suarez, Elizabeth

2015-01-01

Although traumatic brachial plexus injuries are relatively rare in trauma patients, their effects on the functionality of the upper limb can be very disabling. The authors' objective was to assess the complications in a series of patients operated for brachial plexus injuries. This was a retrospective evaluation of patients operated on by the authors between August 2009 and March 2013. We performed 36 surgeries on 33 patients. The incidence of complications was 27.7%. Of these, only 1 (2.7%) was considered serious and associated with the procedure (iatrogenic injury of brachial artery). There was another serious complication (hypoxia in patients with airway injury) but it was not directly related to the surgical procedure. All other complications were considered minor (wound dehiscence, hematoma, infection). There was no mortality in our series. The complications in our series are similar to those reported in the literature. Serious complications (vascular, neural) are rare and represent less than 5% in all the different series. Given the rate of surgical complications and the poor functional perspective for a brachial plexus injury without surgery, we believe that surgery should be the treatment of choice. Copyright © 2013 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

What causes amyotrophic lateral sclerosis?

PubMed Central

Martin, Sarah; Al Khleifat, Ahmad; Al-Chalabi, Ammar

2017-01-01

Amyotrophic lateral sclerosis is a neurodegenerative disease predominantly affecting upper and lower motor neurons, resulting in progressive paralysis and death from respiratory failure within 2 to 3 years. The peak age of onset is 55 to 70 years, with a male predominance. The causes of amyotrophic lateral sclerosis are only partly known, but they include some environmental risk factors as well as several genes that have been identified as harbouring disease-associated variation. Here we review the nature, epidemiology, genetic associations, and environmental exposures associated with amyotrophic lateral sclerosis. PMID:28408982

Ankle-Brachial Index

MedlinePlus

... in which the arteries in your legs or arms are narrowed or blocked. People with peripheral artery ... ankle with your blood pressure measured at your arm. A low ankle-brachial index number can indicate ...

Obstetric brachial plexus injury

PubMed Central

Thatte, Mukund R.; Mehta, Rujuta

2011-01-01

Obstetric brachial plexus injury (OBPI), also known as birth brachial plexus injury (BBPI), is unfortunately a rather common injury in newborn children. Incidence varies between 0.15 and 3 per 1000 live births in various series and countries. Although spontaneous recovery is known, there is a large subset which does not recover and needs primary or secondary surgical intervention. An extensive review of peer-reviewed publications has been done in this study, including clinical papers, review articles and systematic review of the subject. In addition, the authors’ experience of several hundred cases over the last 15 years has been added and has influenced the ultimate text. Causes of OBPI, indications of primary nerve surgery and secondary reconstruction of shoulder, etc. are discussed in detail. Although all affected children do not require surgery in infancy, a substantial proportion of them, however, require it and are better off for it. Secondary surgery is needed for shoulder elbow and hand problems. Results of nerve surgery are very encouraging. Children with OBPI should be seen early by a hand surgeon dealing with brachial plexus injuries. Good results are possible with early and appropriate intervention even in severe cases. PMID:22279269

Clinical research of comprehensive rehabilitation in treating brachial plexus injury patients.

PubMed

Zhou, Jun-Ming; Gu, Yu-Dong; Xu, Xiao-Jun; Zhang, Shen-Yu; Zhao, Xin

2012-07-01

Brachial plexus injury is one of the difficult medical problems in the world. The aim of this study was to observe the clinical therapeutic effect of comprehensive rehabilitation in treating dysfunction after brachial plexus injury. Forty-three cases of dysfunction after brachial plexus injury were divided into two groups randomly. The treatment group, which totaled 21 patients (including 14 cases of total brachial plexus injury and seven cases of branch brachial plexus injury), was treated with comprehensive rehabilitation including transcutaneous electrical nerve stimulation, mid-frequency electrotherapy, Tuina therapy, and occupational therapy. The control group, which totaled 22 patients (including 16 cases of total brachial plexus injury and six cases of branch brachial plexus injury), was treated with home-based electrical nerve stimulation and occupational therapy. Each course was of 30 days duration and the patients received four courses totally. After four courses, the rehabilitation effect was evaluated according to the brachial plexus function evaluation standard and electromyogram (EMG) assessment. In the treatment group, there was significant difference in the scores of brachial plexus function pre- and post-treatment (P < 0.01) in both "total" and "branch" injury. The scores of two "total injury" groups had statistical differences (P < 0.01), while the scores of two "branch injury" groups had statistical differences (P < 0.05) after four courses. EMG suggested that the appearance of regeneration potentials of the recipient nerves in the treatment group was earlier than the control group and had significant differences (P < 0.05). Comprehensive rehabilitation was more effective in treating dysfunction after brachial plexus injury than nonintegrated rehabilitation.

Is there pain with neuropathic characteristics in patients with amyotrophic lateral sclerosis? A cross-sectional study.

PubMed

Moisset, Xavier; Cornut-Chauvinc, Catherine; Clavelou, Pierre; Pereira, Bruno; Dallel, Radhouane; Guy, Nathalie

2016-05-01

Amyotrophic lateral sclerosis is a progressive debilitating and lethal disorder, characterized by degeneration of motor neurons that warrant palliative care. Pain is frequent in patients with amyotrophic lateral sclerosis and significantly impacts on quality of life. To describe pain and assess the prevalence of pain with neuropathic characteristics in patients with amyotrophic lateral sclerosis. Cross-sectional survey from March 2009 to October 2013. Amyotrophic lateral sclerosis patients underwent multidisciplinary assessment and completed questionnaires measuring the severity and impact of pain and anxiety. The Douleur Neuropathique-4 questionnaire was used to look for pain with neuropathic characteristics. Of 96 clinical evaluations, 93 were usable for analysis (age at onset: 62 ± 12.5 years; disease duration: 34 ± 33 months). The overall pain prevalence was 66%, with 9% experiencing pain with neuropathic characteristics. Pain was most often located in the neck and shoulders (38% of pain patients). Neck and shoulder pain was associated with neck (p = 0.04) and proximal upper limb muscular weakness (p = 0.02), respectively. Pain was not associated with disease duration, respiratory or nutritional parameters, but with higher anxiety scores (p = 0.01). Patients with neuropathic characteristics pain did not differ significantly from patients with or without pain, except that they had higher minimal pain intensity score (p < 0.05). Neuropathic characteristics pain was frequently spontaneous (rarely evoked) and described as numbness, burning, electric shock, tingling, and pins-and-needle. Even if amyotrophic lateral sclerosis is a disease of the motor system, pain is frequent and can rarely have neuropathic characteristics. Pain must be always sought and appropriately treated to limit quality of life impairment. © The Author(s) 2015.

Rhabdomyolysis resulting in concurrent Horner's syndrome and brachial plexopathy: a case report.

PubMed

Lee, Susan C; Geannette, Christian; Wolfe, Scott W; Feinberg, Joseph H; Sneag, Darryl B

2017-08-01

This case report describes a 29-year-old male who presented with immediate onset of Horner's syndrome and ipsilateral brachial plexopathy after sleeping with his arm dangling outside a car window for 8 h. Outside workup and imaging revealed rhabdomyolysis of the left neck musculature. Subsequent electrodiagnostic testing and high-resolution brachial plexus magnetic resonance imaging at the authors' institution attributed the Horner's syndrome and concurrent brachial plexopathy to rhabdomyolysis of the longus colli and scalene musculature, which had compressed-and consequently scar tethered-the cervical sympathetic trunk and brachial plexus. This case of co-existent Horner's syndrome and brachial plexopathy demonstrates the role of high-resolution brachial plexus MRI in diagnosing plexopathy and the importance of being familiar with plexus and paravertebral muscle anatomy.

Concepts of nerve regeneration and repair applied to brachial plexus reconstruction.

PubMed

Bertelli, Jayme Augusto; Ghizoni, Marcos Flávio

2006-01-01

Brachial plexus injury is a serious condition that usually affects young adults. Progress in brachial plexus repair is intimately related to peripheral nerve surgery, and depends on clinical and experimental studies. We review the rat brachial plexus as an experimental model, together with its behavioral evaluation. Techniques to repair nerves, such as neurolysis, nerve coaptation, nerve grafting, nerve transfer, fascicular transfer, direct muscle neurotization, and end-to-side neurorraphy, are discussed in light of the authors' experimental studies. Intradural repair of the brachial plexus by graft implants into the spinal cord and motor rootlet transfer offer new possibilities in brachial plexus reconstruction. The clinical experience of intradural repair is presented. Surgical planning in root rupture or avulsion is proposed. In total avulsion, the authors are in favor of the reconstruction of thoraco-brachial and abdomino-antebrachial grasping, and on the transfer of the brachialis muscle to the wrist extensors if it is reinnervated. Surgical treatment of painful conditions and new drugs are also discussed.

True Brachial Artery Aneurysm Presenting as a Non-Pulsatile Mass.

PubMed

Pradhananga, A; Chao, X

2017-01-01

Brachial artery aneurysms are rare disease that can be encountered. It is divided into true and false. The frequency of true aneurysm of the brachial artery is so much unusual. So, we present a case of a 59 year old male who presented to us with complaint of mass in left upper limb since many years ago. Now, there was sudden onset of progressive pain with coldness, numbness, tingling sensation and blackish discolouration of skin from 8 hours. The left upper limb was pulseless and color Doppler ultrasound showed a non-pulsatile aneurysm at the mid level of left brachial artery associated with arterial occlusion in its distal branch by thrombus. The patient was successfully revascularised by interposing a saphenous vein graft. Thus,this case suggest that the prompt diagnosis of true brachial artery aneurysm by ultrasound or color Doppler ultrasound and the proper treatment by surgical repair can save limb from dangerous sequel Keywords: brachial artery aneurysm; color doppler ultrasound; true aneurysm; ultrasound.

Risk factors for clavicle fracture concurrent with brachial plexus injury.

PubMed

Karahanoglu, Ertugrul; Kasapoglu, Taner; Ozdemirci, Safak; Fadıloglu, Erdem; Akyol, Aysegul; Demirdag, Erhan; Yalvac, E Serdar; Kandemir, N Omer

2016-04-01

The aim of this study was to evaluate the risk factors for clavicle fracture concurrent with brachial plexus injuries. A retrospective study was conducted at a tertiary centre. The hospital records of 62,288 vaginal deliveries were evaluated retrospectively. There were 35 cases of brachial plexus injury. Of these patients, nine had brachial plexus injuries with clavicle fracture and 26 without clavicle fracture. The analysed risk factors for clavicle fracture concurrent with brachial plexus injury were gestational diabetes, labour induction and augmentation, prolonged second stage of labour, estimated foetal weight above 4000 g, birth weight above 4000 g, risky working hours, and the requirement of manoeuvres to free the impacted shoulder from behind the symphysis pubis. Labour augmentation with oxytocin increased the risk of clavicle fracture in cases of brachial plexus injury (OR 6.67; 95% CI 1.26-35.03). A birth weight higher than 4000 g also increased the risk of clavicle fracture. Risky working hours, gestational diabetes, estimated foetal weight higher than 4000 g, and requirement of shoulder dystocia manoeuvres did not increase the risk of clavicle fracture. Labour augmentation and actual birth weight higher than 4000 g were identified as risk factors for clavicle fracture in cases of brachial plexus injury.

High-Fat and Ketogenic Diets in Amyotrophic Lateral Sclerosis

PubMed Central

Paganoni, Sabrina; Wills, Anne-Marie

2015-01-01

Amyotrophic lateral sclerosis is a fatal neurodegenerative disease. Epidemiologic data suggest that malnutrition is a common feature in amyotrophic lateral sclerosis and being overweight or obese confers a survival advantage in this patient population. In amyotrophic lateral sclerosis mouse models, a high-fat diet has been shown to lead to weight gain and prolonged survival. However, little research has been conducted to test whether nutritional interventions might ameliorate the disease course in humans. Here we review the currently available evidence supporting the potential role of dietary interventions as a therapeutic tool for amyotrophic lateral sclerosis. Ultimately, determining whether a high-fat or ketogenic diet could be beneficial in amyotrophic lateral sclerosis will require large randomized, placebo-controlled clinical trials. PMID:23666040

Anatomical study of prefixed versus postfixed brachial plexuses in adult human cadaver.

PubMed

Guday, Edengenet; Bekele, Asegedech; Muche, Abebe

2017-05-01

The brachial plexus is usually formed by the fusion of anterior primary rami of the fifth to eighth cervical and the first thoracic spinal nerves. Variations in the formation of the brachial plexus may occur. Variations in brachial plexus anatomy are important to radiologists, surgeons and anaesthesiologists performing surgical procedures in the neck, axilla and upper limb regions. These variations may lead to deviation from the expected dermatome distribution as well as differences in the motor innervation of muscles of the upper limb. This study is aimed to describe the anatomical variations of brachial plexus in its formation among 20 Ethiopian cadavers. Observational based study was conducted by using 20 cadavers obtained from the Department of Human Anatomy at University of Gondar, Bahir Dar, Addis Ababa, Hawasa, Hayat Medical College and St Paul Hospital Millennium Medical College. Data analysis was conducted using thematic approaches. A total of 20 cadavers examined bilaterally for the formation of brachial plexus. Of the 40 sides, 30 sides (75%) were found normal, seven sides (17.5%) prefixed, three sides (7.5%) postfixed and one side of the cadaver lacks cord formation. The brachial plexus formation in most subjects is found to be normal. Among the variants, the numbers of the prefixed brachial plexuses are greater than the postfixed brachial plexuses. © 2016 Royal Australasian College of Surgeons.

Ankle-Brachial Index, Toe-Brachial Index, and Pulse Volume Recording in Healthy Young Adults

PubMed Central

Masaki, Hisao; Yunoki, Yasuhiro; Tabuchi, Atushi; Morita, Ichiro; Mohri, Satoshi; Tanemoto, Kazuo

2015-01-01

Objective: To clarify the characteristics of ankle-brachial index (ABI), toe-brachial index (TBI), and pulse volume recording (PVR) of the ankle with brachial-ankle pulse wave velocity (baPWV) in healthy young adults. Material and Methods: We analyzed ABI, TBI, baPWV, and PVR in the ankle of healthy adults aged 20 to 25 years (median, 20 years) using an automatic oscillometric device between 2002 and 2013. The ABI, baPWV, and PVR in 1282 legs of 641 subjects (301 men and 340 women) and the TBI in 474 toes of 237 subjects (117 men and 120 women) were evaluated. Results: The measured values showed no bilateral differences. ABI and baPWV were higher in men than in women, but TBI was similar in both sexes. ABI <1.0 was observed in 18.1% of the legs in men and in 25.6% in women. TBI <0.7 was observed in 16.2% of the toes in men and 19.1% in women. For ankle PVR, the % mean arterial pressure was higher in women than in men. The upstroke time was <180 ms in most subjects. Conclusions: For young people, ABI <1.0 or TBI <0.7 may not always indicate vascular abnormalities. When evaluating circulatory indexes, age and sex should be considered. PMID:26421072

Primary benign brachial plexus tumors: an experience of 115 operated cases.

PubMed

Desai, Ketan I

2012-01-01

Primary benign brachial plexus tumors are rare. They pose a great challenge to the neurosurgeon, because the majority of patients present with minimal or no neurological deficits. Radical to complete excision of the tumor with preservation of neurological function of the involved nerve is an ideal surgical treatment option with benign primary brachial plexus tumor surgery. We present a review article of our 10-year experience with primary benign brachial plexus tumors surgically treated at King Edward Memorial Hospital and P.D. Hinduja National Hospital from 2000 to 2009. The clinical presentations, radiological features, surgical strategies, and the eventual outcome following surgery are analyzed, discussed, and compared with available series in the world literature. Various difficulties and problems faced in the management of primary benign brachial plexus tumors are analyzed. Irrespective of the tumor size, the indications for surgical intervention are also discussed. The goal of our study was to optimize the treatment of patients with benign brachial plexus tumors with minimal neurological deficits. It is of paramount importance that brachial plexus tumors be managed by a peripheral nerve surgeon with expertise and experience in this field to minimize the neurological insult following surgery.

[Microsurgical treatment of brachial plexus injuries].

PubMed

Päzolt, H J

1986-01-01

Injuries of the brachial plexus are found to occur primarily to juvenile patients as a consequence of motorcycle accidents. While it is a severe injury, its prognosis has been substantively improved by the availability of microsurgical treatment, using long nerve transplants. Further improvement of results will be possible by early operation, a desirable objective. 44 patients with brachial palsy received treatment, including surgery in 41 cases. An account is given in this paper of intraoperative findings, surgical techniques, and results from follow-up checks. The need is underlined for long-term intensive after-care for the purpose of occupational reintegration.

[Brachial plexus. Anesthesia and analgesia].

PubMed

Schulz-Stübner, S

2003-07-01

This review explains the different approaches to the brachial plexus (posterior cervical, interscalene, supra- and infraclavicular, and axillary) and their advantages and disadvantages (indications, contraindications, and complications) for surgery and postoperative or chronic pain management. One of the focussed areas of this review is the use of continuous catheter techniques. Information about the most commonly used local anesthetics as well as adjuncts suggested in the literature is summarized. As essential components for the success of those techniques, organizational and documentation requirements are described. In summary, regional techniques for single shot or continuous block of the brachial plexus are an efficient and safe way of providing anesthesia and analgesia for surgery or pain in the region of the shoulder, arm, or hand.

[Surgical treatment of children with brachial plexus paralysis].

PubMed

Grossman, J A; Ramos, L E; Tidwell, M; Price, A; Papazian, O; Alfonso, I

1998-08-01

A variety of surgical procedures exist for early repair of the nerve injury in obstetrical brachial plexus palsy, including neuroma excision and nerve grafting, neurolysis and neurotization. Secondary deformities of the shoulder, forearm, and hand can similarly be reconstructed using soft tissue and skeletal procedures. This review describes our surgical approach to maximize the ultimate functional outcome in infants and children with obstetrical brachial plexus palsy.

Brachial Plexus Injuries in Adults: Evaluation and Diagnostic Approach

PubMed Central

Sakellariou, Vasileios I.; Badilas, Nikolaos K.; Mazis, George A.; Stavropoulos, Nikolaos A.; Kotoulas, Helias K.; Kyriakopoulos, Stamatios; Tagkalegkas, Ioannis; Sofianos, Ioannis P.

2014-01-01

The increased incidence of motor vehicle accidents during the past century has been associated with a significant increase in brachial plexus injuries. New imaging studies are currently available for the evaluation of brachial plexus injuries. Myelography, CT myelography, and magnetic resonance imaging (MRI) are indicated in the evaluation of brachial plexus. Moreover, a series of specialized electrodiagnostic and nerve conduction studies in association with the clinical findings during the neurologic examination can provide information regarding the location of the lesion, the severity of trauma, and expected clinical outcome. Improvements in diagnostic approaches and microsurgical techniques have dramatically changed the prognosis and functional outcome of these types of injuries. PMID:24967130

Hand Sensorimotor Function in Older Children With Neonatal Brachial Plexus Palsy.

PubMed

Brown, Susan H; Wernimont, Cory W; Phillips, Lauren; Kern, Kathy L; Nelson, Virginia S; Yang, Lynda J-S

2016-03-01

Routine sensory assessments in neonatal brachial plexus palsy are infrequently performed because it is generally assumed that sensory recovery exceeds motor recovery. However, studies examining sensory function in neonatal brachial plexus palsy have produced equivocal findings. The purpose of this study was to examine hand sensorimotor function in older children with neonatal brachial plexus palsy using standard clinical and research-based measures of tactile sensibility. Seventeen children with neonatal brachial plexus palsy (mean age: 11.6 years) and 19 age-matched controls participated in the study. Functional assessments included grip force, monofilament testing, and hand dexterity (Nine-Hole Peg, Jebsen-Taylor Hand Function). Tactile spatial perception involving the discrimination of pin patterns and movement-enhanced object recognition (stereognosis) were also assessed. In the neonatal brachial plexus palsy group, significant deficits in the affected hand motor function were observed compared with the unaffected hand. Median monofilament scores were considered normal for both hands. In contrast, tactile spatial perception was impaired in the neonatal brachial plexus palsy group. This impairment was seen as deficits in both pin pattern and object recognition accuracy as well as the amount of time required to identify patterns and objects. Tactile pattern discrimination time significantly correlated with performance on both functional assessment tests (P < 0.01). This study provides evidence that tactile perception deficits may accompany motor deficits in neonatal brachial plexus palsy even when measures of tactile registration (i.e., monofilament testing) are normal. These results may reflect impaired processing of somatosensory feedback associated with reductions in goal-directed upper limb use and illustrate the importance of including a broader range of sensory assessments in neonatal brachial plexus palsy. Copyright © 2016 Elsevier Inc. All rights

Child neurology: Brachial plexus birth injury: what every neurologist needs to know.

PubMed

Pham, Christina B; Kratz, Johannes R; Jelin, Angie C; Gelfand, Amy A

2011-08-16

While most often transient, brachial plexus birth injury can cause permanent neurologic injury. The major risk factors for brachial plexus birth injury are fetal macrosomia and shoulder dystocia. The degree of injury to the brachial plexus should be determined in the neonatal nursery, as those infants with the most severe injury--root avulsion--should be referred early for surgical evaluation so that microsurgical repair of the plexus can occur by 3 months of life. Microsurgical repair options include nerve grafts and nerve transfers. All children with brachial plexus birth injury require ongoing physical and occupational therapy and close follow-up to monitor progress.

Tolerance of the Brachial Plexus to High-Dose Reirradiation

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

Chen, Allen M., E-mail: achen5@kumc.edu; Yoshizaki, Taeko; Velez, Maria A.

Purpose: To study the tolerance of the brachial plexus to high doses of radiation exceeding historically accepted limits by analyzing human subjects treated with reirradiation for recurrent tumors of the head and neck. Methods and Materials: Data from 43 patients who were confirmed to have received overlapping dose to the brachial plexus after review of radiation treatment plans from the initial and reirradiation courses were used to model the tolerance of this normal tissue structure. A standardized instrument for symptoms of neuropathy believed to be related to brachial plexus injury was utilized to screen for toxicity. Cumulative dose was calculatedmore » by fusing the initial dose distributions onto the reirradiation plan, thereby creating a composite plan via deformable image registration. The median elapsed time from the initial course of radiation therapy to reirradiation was 24 months (range, 3-144 months). Results: The dominant complaints among patients with symptoms were ipsilateral pain (54%), numbness/tingling (31%), and motor weakness and/or difficulty with manual dexterity (15%). The cumulative maximum dose (Dmax) received by the brachial plexus ranged from 60.5 Gy to 150.1 Gy (median, 95.0 Gy). The cumulative mean (Dmean) dose ranged from 20.2 Gy to 111.5 Gy (median, 63.8 Gy). The 1-year freedom from brachial plexus–related neuropathy was 67% and 86% for subjects with a cumulative Dmax greater than and less than 95.0 Gy, respectively (P=.05). The 1-year complication-free rate was 66% and 87%, for those reirradiated within and after 2 years from the initial course, respectively (P=.06). Conclusion: The development of brachial plexus–related symptoms was less than expected owing to repair kinetics and to the relatively short survival of the subject population. Time-dose factors were demonstrated to be predictive of complications.« less

Evaluation of an education day for families of children with obstetrical brachial plexus palsy.

PubMed

Ho, Emily S; Ulster, Alissa A

2011-09-01

Children with obstetrical brachial plexus palsy may have chronic physical impairment in their affected upper extremity. Affected children and their families may benefit from psychosocial interventions including therapeutic relationships with health professionals, meeting other families living with obstetrical brachial plexus palsy, support groups, and social work. One method of addressing psychosocial needs is through a support and education day. The purpose of this quality improvement project is to evaluate parental perceptions of a support and education day called the "Brachial Plexus Family Day." Families of children with obstetrical brachial plexus palsy who attended the Brachial Plexus Family Day completed a questionnaire to evaluate the different programs offered during the day. The families also ranked the importance of different psychosocial supports offered in the clinic. Sixty-three out of 69 families completed the questionnaire. Each program of the Brachial Plexus Family Day was rated as good or excellent by the respondents. Ninety-seven percent of respondents rated meeting other families and children with obstetrical brachial plexus palsy as helpful supports. Attending a Brachial Plexus Family day event (86%), followed by connecting with a doctor (60%), and physical or occupational therapist (59%) were the highest ranked supports reported by the families. The parents and caregivers that attended the Brachial Plexus Family Day rated the program highly. This group also valued the opportunity to connect with other families and children affected with the same condition.

The natural history and management of brachial plexus birth palsy.

PubMed

Buterbaugh, Kristin L; Shah, Apurva S

2016-12-01

Brachial plexus birth palsy (BPBP) is an upper extremity paralysis that occurs due to traction injury of the brachial plexus during childbirth. Approximately 20 % of children with brachial plexus birth palsy will have residual neurologic deficits. These permanent and significant impacts on upper limb function continue to spur interest in optimizing the management of a problem with a highly variable natural history. BPBP is generally diagnosed on clinical examination and does not typically require cross-sectional imaging. Physical examination is also the best modality to determine candidates for microsurgical reconstruction of the brachial plexus. The key finding on physical examination that determines need for microsurgery is recovery of antigravity elbow flexion by 3-6 months of age. When indicated, both microsurgery and secondary shoulder and elbow procedures are effective and can substantially improve functional outcomes. These procedures include nerve transfers and nerve grafting in infants and secondary procedures in children, such as botulinum toxin injection, shoulder tendon transfers, and humeral derotational osteotomy.

Perineural Spread of Nonmelanoma Skin Cancer to the Brachial Plexus: Identifying Anatomic Pathway(s).

PubMed

Marek, Tomas; Howe, B Matthew; Amrami, Kimberly K; Spinner, Robert J

2018-06-01

Perineural spread leading to brachial plexopathy has recently been described in cases of melanoma. The occurrence and mechanism for nonmelanoma skin cancer spread to the brachial plexus is poorly understood. A retrospective chart review of the Mayo Clinic database was conducted to identify patients with nonmelanoma skin cancer and brachial plexopathy between 2000 and 2017. Inclusion criteria were a history of nonmelanoma skin cancer, a clinical diagnosis of brachial plexopathy, imaging features of perineural spread, and a positive result of examination of a biopsy specimen showing tumor in a skin nerve. Thirty-seven patients with a history of nonmelanoma skin cancer and brachial plexopathy were identified. Inclusion criteria were fulfilled in 2 cases of cutaneous squamous cell carcinoma. One case of recurrent basal cell carcinoma with perineural spread confirmed in the brachial plexus by pathologic examination was excluded because confirmatory evidence of perineural spread from the skin to the brachial plexus was not available. Perineural spread of nonmelanoma skin cancer leading to brachial plexopathy is rare. Our 2 cases and the cases found in the literature demonstrate different entry points to the neural highway resulting in neurologic deficits. The cervical plexus serves as a hub for further spread in certain cases of perineural spread of skin cancer. Copyright © 2018 Elsevier Inc. All rights reserved.

Polycyclic aromatic hydrocarbons, brachial artery distensibility and blood pressure among children residing near an oil refinery

PubMed Central

Trasande, Leonardo; Urbina, Elaine M.; Khoder, Mamdouh; Alghamdi, Mansour; Shabaj, Ibrahim; Alam, Mohammed S.; Harrison, Roy M.; Shamy, Magdy

2017-01-01

Background Polycyclic aromatic hydrocarbons (PAH) are produced by the burning and processing of fuel oils, and have been associated with oxidant stress, insulin resistance and hypertension in adults. Few studies have examined whether adolescents are susceptible to cardiovascular effects of PAHs. Objective To study associations of PAH exposure with blood pressure (BP) and brachial artery distensibility (BAD), an early marker of arterial wall stiffness, in young boys attending three schools in Jeddah, Saudi Arabia in varying proximity to an oil refinery. Methods Air samples collected from the three schools were analyzed for PAHs. PAH metabolites (total hydroxyphenanthrenes and 1-hydroxypyrene) were measured in urine samples from 184 adolescent males, in whom anthropometrics, heart rate, pulse pressure, brachial artery distensibility and blood pressure were measured. Descriptive, bivariate and multivariable analyses were performed to assess relationships of school location and urinary PAH metabolites with cardiovascular measures. Results Total suspended matter was significantly higher (444 ± 143 µg/m3) at the school near the refinery compared to a school located near a ring road (395 ± 65 µg/m3) and a school located away from vehicle traffic (232 ± 137 µg/m3), as were PAHs. Systolic (0.47 SD units, p = 0.006) and diastolic (0.53 SD units, p < 0.001) BP Z-scores were highest at the school near the refinery, with a 4.36-fold increase in prehypertension (p = 0.001), controlling for confounders. No differences in pulse pressure, BAD and heart rate were noted in relationship to school location. Urinary total hydroxyphenanthrenes and 1-hydroxypyrene were not associated with cardiovascular outcomes. Conclusions Proximity to an oil refinery in Saudi Arabia is associated with prehypertension and increases in PAH and particulate matter exposures. Further study including insulin resistance measurements, better control for confounding, and longitudinal measurement is

Polycyclic aromatic hydrocarbons, brachial artery distensibility and blood pressure among children residing near an oil refinery.

PubMed

Trasande, Leonardo; Urbina, Elaine M; Khoder, Mamdouh; Alghamdi, Mansour; Shabaj, Ibrahim; Alam, Mohammed S; Harrison, Roy M; Shamy, Magdy

2015-01-01

Polycyclic aromatic hydrocarbons (PAH) are produced by the burning and processing of fuel oils, and have been associated with oxidant stress, insulin resistance and hypertension in adults. Few studies have examined whether adolescents are susceptible to cardiovascular effects of PAHs. To study associations of PAH exposure with blood pressure (BP) and brachial artery distensibility (BAD), an early marker of arterial wall stiffness, in young boys attending three schools in Jeddah, Saudi Arabia in varying proximity to an oil refinery. Air samples collected from the three schools were analyzed for PAHs. PAH metabolites (total hydroxyphenanthrenes and 1-hydroxypyrene) were measured in urine samples from 184 adolescent males, in whom anthropometrics, heart rate, pulse pressure, brachial artery distensibility and blood pressure were measured. Descriptive, bivariate and multivariable analyses were performed to assess relationships of school location and urinary PAH metabolites with cardiovascular measures. Total suspended matter was significantly higher (444 ± 143 μg/m(3)) at the school near the refinery compared to a school located near a ring road (395 ± 65 μg/m(3)) and a school located away from vehicle traffic (232 ± 137 μg/m(3)), as were PAHs. Systolic (0.47 S D units, p = 0.006) and diastolic (0.53 SD units, p < 0.001) BP Z-scores were highest at the school near the refinery, with a 4.36-fold increase in prehypertension (p = 0.001), controlling for confounders. No differences in pulse pressure, BAD and heart rate were noted in relationship to school location. Urinary total hydroxyphenanthrenes and 1-hydroxypyrene were not associated with cardiovascular outcomes. Proximity to an oil refinery in Saudi Arabia is associated with prehypertension and increases in PAH and particulate matter exposures. Further study including insulin resistance measurements, better control for confounding, and longitudinal measurement is indicated. Copyright © 2014 Elsevier Inc. All

High resolution neurography of the brachial plexus by 3 Tesla magnetic resonance imaging.

PubMed

Cejas, C; Rollán, C; Michelin, G; Nogués, M

2016-01-01

The study of the structures that make up the brachial plexus has benefited particularly from the high resolution images provided by 3T magnetic resonance scanners. The brachial plexus can have mononeuropathies or polyneuropathies. The mononeuropathies include traumatic injuries and trapping, such as occurs in thoracic outlet syndrome due to cervical ribs, prominent transverse apophyses, or tumors. The polyneuropathies include inflammatory processes, in particular chronic inflammatory demyelinating polyneuropathy, Parsonage-Turner syndrome, granulomatous diseases, and radiation neuropathy. Vascular processes affecting the brachial plexus include diabetic polyneuropathy and the vasculitides. This article reviews the anatomy of the brachial plexus and describes the technique for magnetic resonance neurography and the most common pathologic conditions that can affect the brachial plexus. Copyright © 2016 SERAM. Published by Elsevier España, S.L.U. All rights reserved.

Is SOD1 loss of function involved in amyotrophic lateral sclerosis?

PubMed Central

Saccon, Rachele A.; Bunton-Stasyshyn, Rosie K. A.; Fisher, Elizabeth M.C.; Fratta, Pietro

2013-01-01

Mutations in the gene superoxide dismutase 1 (SOD1) are causative for familial forms of the neurodegenerative disease amyotrophic lateral sclerosis. When the first SOD1 mutations were identified they were postulated to give rise to amyotrophic lateral sclerosis through a loss of function mechanism, but experimental data soon showed that the disease arises from a—still unknown—toxic gain of function, and the possibility that loss of function plays a role in amyotrophic lateral sclerosis pathogenesis was abandoned. Although loss of function is not causative for amyotrophic lateral sclerosis, here we re-examine two decades of evidence regarding whether loss of function may play a modifying role in SOD1–amyotrophic lateral sclerosis. From analysing published data from patients with SOD1–amyotrophic lateral sclerosis, we find a marked loss of SOD1 enzyme activity arising from almost all mutations. We continue to examine functional data from all Sod1 knockout mice and we find obvious detrimental effects within the nervous system with, interestingly, some specificity for the motor system. Here, we bring together historical and recent experimental findings to conclude that there is a possibility that SOD1 loss of function may play a modifying role in amyotrophic lateral sclerosis. This likelihood has implications for some current therapies aimed at knocking down the level of mutant protein in patients with SOD1–amyotrophic lateral sclerosis. Finally, the wide-ranging phenotypes that result from loss of function indicate that SOD1 gene sequences should be screened in diseases other than amyotrophic lateral sclerosis. PMID:23687121

Genotyping of presenilin-1 polymorphism in amyotrophic lateral sclerosis.

PubMed

Panas, M; Karadima, G; Kalfakis, N; Psarrou, O; Floroskoufi, P; Kladi, A; Petersen, M B; Vassilopoulos, D

2000-12-01

The mechanisms underlying motor neuron degeneration in amyotrophic lateral sclerosis are not fully understood. Recent studies suggest that apoptosis is involved in the abnormal neural death that occurs in this devastating disease. Presenilin-1, a transmembrane protein, seems to be implicated in apoptosis. To determine whether presenilin-1 intron 8 polymorphism has an influence in the course of amyotrophic lateral sclerosis, we examined this polymorphism genotypes in a large group of patients (n = 72) with amyotrophic lateral sclerosis and in a random sample of 213 healthy individuals. The results showed a significant difference in genotype (P < 0.04) and allele (P < 0.03) distribution between patients controls. These results suggest a possible intervention of presenilin-1 in the pathogenesis of amyotrophic lateral sclerosis.

Amyotrophic Lateral Sclerosis

MedlinePlus

Amyotrophic lateral sclerosis (ALS) is a nervous system disease that attacks nerve cells called neurons in your brain and spinal cord. These neurons ... breathing machine can help, but most people with ALS die from respiratory failure. The disease usually strikes ...

Effects of posture on shear rates in human brachial and superficial femoral arteries

PubMed Central

Newcomer, S. C.; Sauder, C. L.; Kuipers, N. T.; Laughlin, M. H.; Ray, C. A.

2012-01-01

Shear rate is significantly lower in the superficial femoral compared with the brachial artery in the supine posture. The relative shear rates in these arteries of subjects in the upright posture (seated and/or standing) are unknown. The purpose of this investigation was to test the hypothesis that upright posture (seated and/or standing) would produce greater shear rates in the superficial femoral compared with the brachial artery. To test this hypothesis, Doppler ultrasound was used to measure mean blood velocity (MBV) and diameter in the brachial and superficial femoral arteries of 21 healthy subjects after being in the supine, seated, and standing postures for 10 min. MBV was significantly higher in the brachial compared with the superficial femoral artery during upright postures. Superficial femoral artery diameter was significantly larger than brachial artery diameter. However, posture had no significant effect on either brachial or superficial femoral artery diameter. The calculated shear rate was significantly greater in the brachial (73 ± 5, 91 ± 11, and 97 ± 13 s−1) compared with the superficial femoral (53 ± 4, 39 ± 77, and 44 ± 5 s−1) artery in the supine, seated, and standing postures, respectively. Contrary to our hypothesis, our current findings indicate that mean shear rate is lower in the superficial femoral compared with the brachial artery in the supine, seated, and standing postures. These findings of lower shear rates in the superficial femoral artery may be one mechanism for the higher propensity for atherosclerosis in the arteries of the leg than of the arm. PMID:18245564

Brachial Plexus Injury from CT-Guided RF Ablation Under General Anesthesia

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

Shankar, Sridhar, E-mail: shankars@ummhc.org; Sonnenberg, Eric van; Silverman, Stuart G.

2005-06-15

Brachial plexus injury in a patient under general anesthesia (GA) is not uncommon, despite careful positioning and, particularly, awareness of the possibility. The mechanism of injury is stretching and compression of the brachial plexus over a prolonged period. Positioning the patient within the computed tomography (CT) gantry for abdominal or chest procedures can simulate a surgical procedure, particularly when GA is used. The potential for brachial plexus injury is increased if the case is prolonged and the patient's arms are raised above the head to avoid CT image degradation from streak artifacts. We report a case of profound brachial plexusmore » palsy following a CT-guided radiofrequency ablation procedure under GA. Fortunately, the patient recovered completely. We emphasize the mechanism of injury and detail measures to combat this problem, such that radiologists are aware of this potentially serious complication.« less

38 CFR 3.318 - Presumptive service connection for amyotrophic lateral sclerosis.

Code of Federal Regulations, 2010 CFR

2010-07-01

... connection for amyotrophic lateral sclerosis. 3.318 Section 3.318 Pensions, Bonuses, and Veterans' Relief... sclerosis. (a) Except as provided in paragraph (b) of this section, the development of amyotrophic lateral... under this section: (1) If there is affirmative evidence that amyotrophic lateral sclerosis was not...

Effects of early nerve repair on experimental brachial plexus injury in neonatal rats.

PubMed

Bourke, Gráinne; McGrath, Aleksandra M; Wiberg, Mikael; Novikov, Lev N

2018-03-01

Obstetrical brachial plexus injury refers to injury observed at the time of delivery, which may lead to major functional impairment in the upper limb. In this study, the neuroprotective effect of early nerve repair following complete brachial plexus injury in neonatal rats was examined. Brachial plexus injury induced 90% loss of spinal motoneurons and 70% decrease in biceps muscle weight at 28 days after injury. Retrograde degeneration in spinal cord was associated with decreased density of dendritic branches and presynaptic boutons and increased density of astrocytes and macrophages/microglial cells. Early repair of the injured brachial plexus significantly delayed retrograde degeneration of spinal motoneurons and reduced the degree of macrophage/microglial reaction but had no effect on muscle atrophy. The results demonstrate that early nerve repair of neonatal brachial plexus injury could promote survival of injured motoneurons and attenuate neuroinflammation in spinal cord.

A novel rat model of brachial plexus injury with nerve root stumps.

PubMed

Fang, Jintao; Yang, Jiantao; Yang, Yi; Li, Liang; Qin, Bengang; He, Wenting; Yan, Liwei; Chen, Gang; Tu, Zhehui; Liu, Xiaolin; Gu, Liqiang

2018-02-01

The C5-C6 nerve roots are usually spared from avulsion after brachial plexus injury (BPI) and thus can be used as donors for nerve grafting. To date, there are no appropriate animal models to evaluate spared nerve root stumps. Hence, the aim of this study was to establish and evaluate a rat model with spared nerve root stumps in BPI. In rupture group, the proximal parts of C5-T1 nerve roots were held with the surrounding muscles and the distal parts were pulled by a sudden force after the brachial plexus was fully exposed, and the results were compared with those of sham group. To validate the model, the lengths of C5-T1 spared nerve root stumps were measured and the histologies of the shortest one and the corresponding spinal cord were evaluated. C5 nerve root stump was found to be the shortest. Histology findings demonstrated that the nerve fibers became more irregular and the continuity decreased; numbers and diameters of myelinated axons and thickness of myelin sheaths significantly decreased over time. The survival of motoneurons was reduced, and the death of motoneurons may be related to the apoptotic process. Our model could successfully create BPI model with nerve root stumps by traction, which could simulate injury mechanisms. While other models involve root avulsion or rupturing by distal nerve transection. This model would be suitable for evaluating nerve root stumps and testing new therapeutic strategies for neuroprotection through nerve root stumps in the future. Copyright © 2017. Published by Elsevier B.V.

Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis.

PubMed

Vercruysse, Pauline; Sinniger, Jérôme; El Oussini, Hajer; Scekic-Zahirovic, Jelena; Dieterlé, Stéphane; Dengler, Reinhard; Meyer, Thomas; Zierz, Stephan; Kassubek, Jan; Fischer, Wilhelm; Dreyhaupt, Jens; Grehl, Torsten; Hermann, Andreas; Grosskreutz, Julian; Witting, Anke; Van Den Bosch, Ludo; Spreux-Varoquaux, Odile; Ludolph, Albert C; Dupuis, Luc

2016-04-01

Amyotrophic lateral sclerosis, the most common adult-onset motor neuron disease, leads to death within 3 to 5 years after onset. Beyond progressive motor impairment, patients with amyotrophic lateral sclerosis suffer from major defects in energy metabolism, such as weight loss, which are well correlated with survival. Indeed, nutritional intervention targeting weight loss might improve survival of patients. However, the neural mechanisms underlying metabolic impairment in patients with amyotrophic lateral sclerosis remain elusive, in particular due to the lack of longitudinal studies. Here we took advantage of samples collected during the clinical trial of pioglitazone (GERP-ALS), and characterized longitudinally energy metabolism of patients with amyotrophic lateral sclerosis in response to pioglitazone, a drug with well-characterized metabolic effects. As expected, pioglitazone decreased glycaemia, decreased liver enzymes and increased circulating adiponectin in patients with amyotrophic lateral sclerosis, showing its efficacy in the periphery. However, pioglitazone did not increase body weight of patients with amyotrophic lateral sclerosis independently of bulbar involvement. As pioglitazone increases body weight through a direct inhibition of the hypothalamic melanocortin system, we studied hypothalamic neurons producing proopiomelanocortin (POMC) and the endogenous melanocortin inhibitor agouti-related peptide (AGRP), in mice expressing amyotrophic lateral sclerosis-linked mutant SOD1(G86R). We observed lower Pomc but higher Agrp mRNA levels in the hypothalamus of presymptomatic SOD1(G86R) mice. Consistently, numbers of POMC-positive neurons were decreased, whereas AGRP fibre density was elevated in the hypothalamic arcuate nucleus of SOD1(G86R) mice. Consistent with a defect in the hypothalamic melanocortin system, food intake after short term fasting was increased in SOD1(G86R) mice. Importantly, these findings were replicated in two other amyotrophic

Association of Brachial-Ankle Pulse Wave Velocity with Asymptomatic Intracranial Arterial Stenosis in Hypertension Patients.

PubMed

Wang, Yan; Zhang, Jin; Qain, Yuesheng; Tang, Xiaofeng; Ling, Huawei; Chen, Kemin; Li, Yan; Gao, Pingjin; Zhu, Dingliang

2016-08-01

Intracranial arterial stenosis is a common cause of ischemic stroke in Asians. We therefore sought to explore the relationship of brachial-ankle pulse wave velocity and intracranial arterial stenosis in 834 stroke-free hypertensive patients. Intracranial arterial stenosis was evaluated through computerized tomographic angiography. Brachial-ankle pulse wave velocity was measured by an automated cuff device. The top decile of brachial-ankle pulse wave velocity was significantly associated with intracranial arterial stenosis (P = .027, odds ratio = 1.82; 95% confidence interval: 1.07-3.10). The patients with the top decile of brachial-ankle pulse wave velocity showed 56% higher risk for the presence of intracranial arterial stenosis to the whole population, which was more significant in patients younger than 65 years old. We also found that brachial-ankle pulse wave velocity related to both intracranial arterial stenosis and homocysteine. Our study showed the association of brachial-ankle pulse wave velocity with asymptomatic intracranial arterial stenosis in hypertension patients, especially in relative younger subjects. Brachial-ankle pulse wave velocity might be a relatively simple and repeatable measurement to detect hypertension patients in high risk of intracranial arterial stenosis. Copyright © 2016. Published by Elsevier Inc.

Brachial artery stiffness estimation using ARTSENS.

PubMed

Kiran, V Raj; Nabeel, P M; Joseph, Jayaraj; Sivaprakasam, Mohanasankar

2017-07-01

Central and peripheral arteries stiffening prominently affect hemodynamics thus increasing the risk of coronary heart disease, chronic kidney disease and end stage renal disease. There are several commercially available non-invasive measurement technologies for the evaluation of stiffness that are expensive, demand dedicated expertise and fall short for mass screening. Considering this, we have developed ARTSENS ® , a highly compact and portable image-free ultrasound device for evaluation of arterial stiffness. The capability of the device to perform accurate measurements of carotid artery stiffness has been validated through extensive in-vivo studies. In this paper we demonstrate the feasibility of using ARTSENS ® for measuring brachial artery stiffness. An inter-operator repeatability study was done based on in-vivo experiments on 9 young healthy subjects. The study included measurement of distension, end diastolic lumen diameter, arterial compliance and stiffness index performed both on carotid artery and brachial artery by two operators successively. The degree of agreement between the measurements made by operators has been investigated based on Bland-Altman plots and paired t-test. The measurements were populated within the limits of agreement. No statistically significant difference (p-values from paired t-test for end-diastolic diameter, distension, stiffness index, arterial compliance were 0.36, 0.24, 0.47 and 0.11 respectively) was seen for the brachial artery measurements performed by the two operators. The correlation between the measurement made by the operators was highly significant (r=0.86, p-value=0.003).

Characteristic features of hereditary neuropathy with liability to pressure palsy (HNPP) presenting with brachial plexopathy in soldiers.

PubMed

Kim, Kyoung-Eun

2014-11-15

A brachial plexus lesion is not common in hereditary neuropathy with liability to pressure palsy (HNPP). We report the clinical and electrodiagnostic features of young soldiers with HNPP presenting with brachial plexopathy. By reviewing 2year medical records from Korean military hospitals, we identified soldiers with brachial plexus lesions. Among them, patients diagnosed with HNPP were determined and clinical and electrophysiological findings were compared between HNPP and non-HNPP patients with a brachial plexus lesion. Thirteen patients (6.8%) were diagnosed with HNPP among 189 patients with a brachial plexus lesion. Push-ups, as either a punishment or an exercise, was the most frequent preceding event in HNPP patients (76.9%), whereas it was rare in non-HNPP patients. The distal motor latency of the median nerve showed the highest sensitivity (90.9%) and specificity (100%) for HNPP in patients with a brachial plexus lesion. In conclusion, HNPP should be suspected in patients with brachial plexopathy if brachial plexopathy develops after push-ups or if the distal motor latency of median nerves is prolonged. Copyright © 2014 Elsevier B.V. All rights reserved.

Targeted Riluzole Delivery by Antioxidant Nanovectors for Treating Amyotrophic Lateral Sclerosis

DTIC Science & Technology

2013-10-01

treating amyotrophic lateral sclerosis . PRINCIPAL INVESTIGATOR: Raymond J. Grill CONTRACTING ORGANIZATION: University of Texas Health...treating Amyotrophic lateral sclerosis 5a. CONTRACT NUMBER W 5b. GRANT NUMBER W81XWH-12-1-0612 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S...survival in a mouse model of amyotrophic lateral sclerosis . This project involves work performed at both UT-Health and Rice University; combining the

The clinical applicability of an automated plethysmographic determination of the ankle-brachial index after vascular surgery.

PubMed

van der Slegt, Jasper; Verbogt, Nathalie Pa; Mulder, Paul Gh; Steunenberg, Stijn L; Steunenberg, Bastiaan E; van der Laan, Lijckle

2016-10-01

An automated ankle-brachial index device could lead to potential time savings and more accuracy in ankle-brachial index-determination after vascular surgery. This prospective cross-sectional study compared postprocedural ankle-brachial indices measured by a manual method with ankle-brachial indices of an automated plethysmographic method. Forty-two patients were included. No significant difference in time performing a measurement was observed (1.1 min, 95% CI: -0.2 to +2.4; P = 0.095). Mean ankle-brachial index with the automated method was 0.105 higher (95% CI: 0.017 to 0.193; P = 0.020) than with the manual method, with limits of agreement of -0.376 and +0.587. Total variance amounted to 0.0759 and the correlation between both methods was 0.60. Reliability expressed as maximum absolute difference (95% level) between duplicate ankle-brachial index-measurements under identical conditions was 0.350 (manual) and 0.152 (automated), although not significant (p = 0.053). Finally, the automated method had 34% points higher failure rate than the manual method. In conclusion based on this study, the automated ankle-brachial index-method seems not to be clinically applicable for measuring ankle-brachial index postoperatively in patients with vascular disease. © The Author(s) 2016.

Degeneration of serotonergic neurons in amyotrophic lateral sclerosis: a link to spasticity.

PubMed

Dentel, Christel; Palamiuc, Lavinia; Henriques, Alexandre; Lannes, Béatrice; Spreux-Varoquaux, Odile; Gutknecht, Lise; René, Frédérique; Echaniz-Laguna, Andoni; Gonzalez de Aguilar, Jose-Luis; Lesch, Klaus Peter; Meininger, Vincent; Loeffler, Jean-Philippe; Dupuis, Luc

2013-02-01

Spasticity is a common and disabling symptom observed in patients with central nervous system diseases, including amyotrophic lateral sclerosis, a disease affecting both upper and lower motor neurons. In amyotrophic lateral sclerosis, spasticity is traditionally thought to be the result of degeneration of the upper motor neurons in the cerebral cortex, although degeneration of other neuronal types, in particular serotonergic neurons, might also represent a cause of spasticity. We performed a pathology study in seven patients with amyotrophic lateral sclerosis and six control subjects and observed that central serotonergic neurons suffer from a degenerative process with prominent neuritic degeneration, and sometimes loss of cell bodies in patients with amyotrophic lateral sclerosis. Moreover, distal serotonergic projections to spinal cord motor neurons and hippocampus systematically degenerated in patients with amyotrophic lateral sclerosis. In SOD1 (G86R) mice, a transgenic model of amyotrophic lateral sclerosis, serotonin levels were decreased in brainstem and spinal cord before onset of motor symptoms. Furthermore, there was noticeable atrophy of serotonin neuronal cell bodies along with neuritic degeneration at disease onset. We hypothesized that degeneration of serotonergic neurons could underlie spasticity in amyotrophic lateral sclerosis and investigated this hypothesis in vivo using tail muscle spastic-like contractions in response to mechanical stimulation as a measure of spasticity. In SOD1 (G86R) mice, tail muscle spastic-like contractions were observed at end-stage. Importantly, they were abolished by 5-hydroxytryptamine-2b/c receptors inverse agonists. In line with this, 5-hydroxytryptamine-2b receptor expression was strongly increased at disease onset. In all, we show that serotonergic neurons degenerate during amyotrophic lateral sclerosis, and that this might underlie spasticity in mice. Further research is needed to determine whether inverse

Estimation of brachial artery volume flow by duplex ultrasound imaging predicts dialysis access maturation.

PubMed

Ko, Sae Hee; Bandyk, Dennis F; Hodgkiss-Harlow, Kelley D; Barleben, Andrew; Lane, John

2015-06-01

This study validated duplex ultrasound measurement of brachial artery volume flow (VF) as predictor of dialysis access flow maturation and successful hemodialysis. Duplex ultrasound was used to image upper extremity dialysis access anatomy and estimate access VF within 1 to 2 weeks of the procedure. Correlation of brachial artery VF with dialysis access conduit VF was performed using a standardized duplex testing protocol in 75 patients. The hemodynamic data were used to develop brachial artery flow velocity criteria (peak systolic velocity and end-diastolic velocity) predictive of three VF categories: low (<600 mL/min), acceptable (600-800 mL/min), or high (>800 mL/min). Brachial artery VF was then measured in 148 patients after a primary (n = 86) or revised (n = 62) upper extremity dialysis access procedure, and the VF category correlated with access maturation or need for revision before hemodialysis usage. Access maturation was conferred when brachial artery VF was >600 mL/min and conduit imaging indicated successful cannulation based on anatomic criteria of conduit diameter >5 mm and skin depth <6 mm. Measurements of VF from the brachial artery and access conduit demonstrated a high degree of correlation (R(2) = 0.805) for autogenous vein (n = 45; R(2) = 0.87) and bridge graft (n = 30; R(2) = 0.78) dialysis accesses. Access VF of >800 mL/min was predicted when the brachial artery lumen diameter was >4.5 mm, peak systolic velocity was >150 cm/s, and the diastolic-to-systolic velocity ratio was >0.4. Brachial artery velocity spectra indicating VF <800 mL/min was associated (P < .0001) with failure of access maturation. Revision was required in 15 of 21 (71%) accesses with a VF of <600 mL/min, 4 of 40 accesses (10%) with aVF of 600 to 800 mL/min, and 2 of 87 accesses (2.3%) with an initial VF of >800 mL/min. Duplex testing to estimate brachial artery VF and assess the conduit for ease of cannulation can be performed in 5 minutes during the initial postoperative

Spinal Cord Gray Matter Atrophy in Amyotrophic Lateral Sclerosis.

PubMed

Paquin, M-Ê; El Mendili, M M; Gros, C; Dupont, S M; Cohen-Adad, J; Pradat, P-F

2018-01-01

There is an emerging need for biomarkers to better categorize clinical phenotypes and predict progression in amyotrophic lateral sclerosis. This study aimed to quantify cervical spinal gray matter atrophy in amyotrophic lateral sclerosis and investigate its association with clinical disability at baseline and after 1 year. Twenty-nine patients with amyotrophic lateral sclerosis and 22 healthy controls were scanned with 3T MR imaging. Standard functional scale was recorded at the time of MR imaging and after 1 year. MR imaging data were processed automatically to measure the spinal cord, gray matter, and white matter cross-sectional areas. A statistical analysis assessed the difference in cross-sectional areas between patients with amyotrophic lateral sclerosis and controls, correlations between spinal cord and gray matter atrophy to clinical disability at baseline and at 1 year, and prediction of clinical disability at 1 year. Gray matter atrophy was more sensitive to discriminate patients with amyotrophic lateral sclerosis from controls ( P = .004) compared with spinal cord atrophy ( P = .02). Gray matter and spinal cord cross-sectional areas showed good correlations with clinical scores at baseline ( R = 0.56 for gray matter and R = 0.55 for spinal cord; P < .01). Prediction at 1 year with clinical scores ( R 2 = 0.54) was improved when including a combination of gray matter and white matter cross-sectional areas ( R 2 = 0.74). Although improvements over spinal cord cross-sectional areas were modest, this study suggests the potential use of gray matter cross-sectional areas as an MR imaging structural biomarker to monitor the evolution of amyotrophic lateral sclerosis. © 2018 by American Journal of Neuroradiology.

Avulsion of the brachial plexus in a great horned owl (Bubo virginaus)

USGS Publications Warehouse

Moore, M.P.; Stauber, E.; Thomas, N.J.

1989-01-01

Avulsion of the brachial plexus was documented in a Great Horned Owl (Bubo virginianus). A fractured scapula was also present. Cause of these injuries was not known but was thought to be due to trauma. Differentiation of musculoskeletal injury from peripheral nerve damage can be difficult in raptors. Use of electromyography and motor nerve conduction velocity was helpful in demonstrating peripheral nerve involvement. A brachial plexus avulsion was suspected on the basis of clinical signs, presence of electromyographic abnormalities in all muscles supplied by the nerves of the brachial plexus and absence of median-ulnar motor nerve conduction velocities.

Three-tesla magnetic resonance neurography of the brachial plexus in cervical radiculopathy.

PubMed

Yoshida, Takeshi; Sueyoshi, Takeshi; Suwazono, Shugo; Suehara, Masahito

2015-09-01

There have been no reports of the use of 3-Tesla magnetic resonance neurography (3T MRN) to characterize cervical radiculopathy. In particular, there are no reports of MRN of brachial plexus involvement in patients with cervical radiculopathy. We reviewed retrospectively 12 consecutive patients with cervical radiculopathy who underwent 3T MRN. The median age was 54.5 years. Eleven of 12 patients were men. The distribution of nerve-root signal abnormality was correlated with intervertebral foraminal stenosis and the presence of muscles that exhibited weakness and/or signs of denervation on electromyography. MRN abnormalities were found to extend into the distal part of the brachial plexus in 10 patients. This study demonstrates that MRN is potentially useful for diagnosis in patients with suspected cervical radiculopathy. Moreover, the finding of brachial plexus involvement on MRN may indicate a possible pathophysiological relationship between cervical radiculopathy and brachial plexopathy. © 2014 Wiley Periodicals, Inc.

Current Concept in Adult Peripheral Nerve and Brachial Plexus Surgery.

PubMed

Rasulic, Lukas

2017-01-01

Peripheral nerve injuries and brachial plexus injuries are relatively frequent. Significance of these injuries lies in the fact that the majority of patients with these types of injuries constitute working population. Since these injuries may create disability, they present substantial socioeconomic problem nowadays. This article will present current state-of-the-art achievements of minimal invasive brachial plexus and peripheral nerve surgery. It is considered that the age of the patient, the mechanism of the injury, and the associated vascular and soft-tissue injuries are factors that primarily influence the extent of recovery of the injured nerve. The majority of patients are treated using classical open surgical approach. However, new minimally invasive open and endoscopic approaches are being developed in recent years-endoscopic carpal and cubital tunnel release, targeted minimally invasive approaches in brachial plexus surgery, endoscopic single-incision sural nerve harvesting, and there were even attempts to perform endoscopic brachial plexus surgery. The use of the commercially available nerve conduits for bridging short nerve gap has shown promising results. Multidisciplinary approach individually designed for every patient is of the utmost importance for the successful treatment of these injuries. In the future, integration of biology and nanotechnology may fabricate a new generation of nerve conduits that will allow nerve regeneration over longer nerve gaps and start new chapter in peripheral nerve surgery.

Mitochondrial dysfunction in blood cells from amyotrophic lateral sclerosis patients.

PubMed

Ehinger, Johannes K; Morota, Saori; Hansson, Magnus J; Paul, Gesine; Elmér, Eskil

2015-06-01

Mitochondrial dysfunction is implicated in amyotrophic lateral sclerosis, where the progressive degeneration of motor neurons results in muscle atrophy, paralysis and death. Abnormalities in both central nervous system and muscle mitochondria have previously been demonstrated in patient samples, indicating systemic disease. In this case-control study, venous blood samples were acquired from 24 amyotrophic lateral sclerosis patients and 21 age-matched controls. Platelets and peripheral blood mononuclear cells were isolated and mitochondrial oxygen consumption measured in intact and permeabilized cells with additions of mitochondrial substrates, inhibitors and titration of an uncoupler. Respiratory values were normalized to cell count and for two markers of cellular mitochondrial content, citrate synthase activity and mitochondrial DNA, respectively. Mitochondrial function was correlated with clinical staging of disease severity. Complex IV (cytochrome c-oxidase)-activity normalized to mitochondrial content was decreased in platelets from amyotrophic lateral sclerosis patients both when normalized to citrate synthase activity and mitochondrial DNA copy number. In mononuclear cells, complex IV-activity was decreased when normalized to citrate synthase activity. Mitochondrial content was increased in amyotrophic lateral sclerosis patient platelets. In mononuclear cells, complex I activity declined and mitochondrial content increased progressively with advancing disease stage. The findings are, however, based on small subsets of patients and need to be confirmed. We conclude that when normalized to mitochondria-specific content, complex IV-activity is reduced in blood cells from amyotrophic lateral sclerosis patients and that there is an apparent compensatory increase in cellular mitochondrial content. This supports systemic involvement in amyotrophic lateral sclerosis and suggests further study of mitochondrial function in blood cells as a future biomarker for the

Use of Sugammadex in a Patient with Amyotrophic Lateral Sclerosis

PubMed Central

Kelsaka, Ebru; Karakaya, Deniz; Zengin, Eyüp Cağatayn

2013-01-01

Objective To report on general anesthesia management in amyotrophic lateral sclerosis. Case Presentation and Intervention A 47-year-old man presented with fracture of the humerus. The patient was diagnosed with amyotrophic lateral sclerosis. General anesthesia was induced with propofol, rocuronium and remifentanil. After uneventful surgical repair, TOF (train-of-four) ratio reached >0.90 at the end of operation. However, muscle strength and tidal volume were inadequate. After sugammadex 2 mg kg−1 i.v. was given, the patient was extubated 120 s later. Conclusion This case highlights that rocuronium and sugammadex can be used safely in patients with amyotrophic lateral sclerosis undergoing surgery with general anesthesia. PMID:23075763

Palpation- and ultrasound-guided brachial plexus blockade in Hispaniolan Amazon parrots (Amazona ventralis).

PubMed

da Cunha, Anderson F; Strain, George M; Rademacher, Nathalie; Schnellbacher, Rodney; Tully, Thomas N

2013-01-01

To compare palpation-guided with ultrasound-guided brachial plexus blockade in Hispaniolan Amazon parrots. Prospective randomized experimental trial. Eighteen adult Hispaniolan Amazon parrots (Amazona ventralis) weighing 252-295 g. After induction of anesthesia with isoflurane, parrots received an injection of lidocaine (2 mg kg(-1)) in a total volume of 0.3 mL at the axillary region. The birds were randomly assigned to equal groups using either palpation or ultrasound as a guide for the brachial plexus block. Nerve evoked muscle potentials (NEMP) were used to monitor effectiveness of brachial plexus block. The palpation-guided group received the local anesthetic at the space between the pectoral muscle, triceps, and supracoracoideus aticimus muscle, at the insertion of the tendons of the caudal coracobrachial muscle, and the caudal scapulohumeral muscle. For the ultrasound-guided group, the brachial plexus and the adjacent vessels were located with B-mode ultrasonography using a 7-15 MHz linear probe. After location, an 8-5 MHz convex transducer was used to guide injections. General anesthesia was discontinued 20 minutes after lidocaine injection and the birds recovered in a padded cage. Both techniques decreased the amplitude of NEMP. Statistically significant differences in NEMP amplitudes, were observed within the ultrasound-guided group at 5, 10, 15, and 20 minutes after injection and within the palpation-guided group at 10, 15, and 20 minutes after injection. There was no statistically significant difference between the two groups. No effect on motor function, muscle relaxation or wing droop was observed after brachial plexus block. The onset of the brachial plexus block tended to be faster when ultrasonography was used. Brachial plexus injection can be performed in Hispaniolan Amazon parrots and nerve evoked muscle potentials were useful to monitor the effects on nerve conduction in this avian species. Neither technique produced an effective block at the

Models of brachial to finger pulse wave distortion and pressure decrement.

PubMed

Gizdulich, P; Prentza, A; Wesseling, K H

1997-03-01

To model the pulse wave distortion and pressure decrement occurring between brachial and finger arteries. Distortion reversion and decrement correction were also our aims. Brachial artery pressure was recorded intra-arterially and finger pressure was recorded non-invasively by the Finapres technique in 53 adult human subjects. Mean pressure was subtracted from each pressure waveform and Fourier analysis applied to the pulsations. A distortion model was estimated for each subject and averaged over the group. The average inverse model was applied to the full finger pressure waveform. The pressure decrement was modelled by multiple regression on finger systolic and diastolic levels. Waveform distortion could be described by a general, frequency dependent model having a resonance at 7.3 Hz. The general inverse model has an anti-resonance at this frequency. It converts finger to brachial pulsations thereby reducing average waveform distortion from 9.7 (s.d. 3.2) mmHg per sample for the finger pulse to 3.7 (1.7) mmHg for the converted pulse. Systolic and diastolic level differences between finger and brachial arterial pressures changed from -4 (15) and -8 (11) to +8 (14) and +8 (12) mmHg, respectively, after inverse modelling, with pulse pressures correct on average. The pressure decrement model reduced both the mean and the standard deviation of systolic and diastolic level differences to 0 (13) and 0 (8) mmHg. Diastolic differences were thus reduced most. Brachial to finger pulse wave distortion due to wave reflection in arteries is almost identical in all subjects and can be modelled by a single resonance. The pressure decrement due to flow in arteries is greatest for high pulse pressures superimposed on low means.

Management of traumatic brachial artery injuries: A report on 49 patients

PubMed Central

Ekim, Hasan; Tuncer, Mustafa

2009-01-01

BACKGROUND AND OBJECTIVE: The brachial artery is the most frequently injured artery in the upper extremity due to its vulnerability. The purpose of our study was to review our experience with brachial artery injuries over a 9-year period, describing the type of injury, surgical procedures, complications, and associated injuries. PATIENTS AND METHODS: Forty-nine patients with brachial artery injury underwent surgical repair procedures at our hospital, from the beginning of May 1999 to the end of June 2008. The brachial artery injuries were diagnosed by physical examination and Doppler ultrasonography. Depending on the mode of presentation, patients were either taken immediately to the operating room for bleeding control and vascular repair or were assessed by preoperative duplex ultrasonography. RESULTS: This study group consisted of 43 males and 6 females, ranging in age from 6 to 65 years with a mean (SD) age of 27.9 (6.7) years. The mechanism of trauma was penetrating in 45 patients and blunt in the remaining 4 patients. Stab injury was the most frequent form of penetrating trauma (24 of 45). Treatment included primary arterial repair in 5 cases, end-to-end anastomosis in 28 cases, interposition vein graft in 15 cases, and interposition-ringed polytetrafluoroethylene graft in 1 case. Associated injuries were common and included venous injury (14), bone fracture (5), and peripheral nerve injury (11). Fifteen patients developed postoperative complications. One patient underwent an above-elbow amputation. CONCLUSIONS: Prompt and appropriate management of the brachial artery injuries, attention to associated injuries, and a readiness to revise the vascular repair early in the event of failure will maximize patient survival and upper extremity salvage. PMID:19318753

Distal Predominance of Electrodiagnostic Abnormalities in Early Stage Amyotrophic Lateral Sclerosis.

PubMed

Shayya, Luay; Babu, Suma; Pioro, Erik P; Li, Jianbo; Li, Yuebing

2018-05-09

We compare the electrodiagnostic (EDX) yield of limb muscles in revealing lower motor neuron (LMN) dysfunction by electromyography (EMG) in early stage amyotrophic lateral sclerosis (ALS). Single-site retrospective review Results: This study includes 122 consecutive patients with possible ALS as defined by revised El Escorial Criteria. Distal limb muscles show more frequent EMG abnormalities than proximal muscles. EDX yield is higher in the limb where weakness begins and when clinical signs of LMN dysfunction are evident. Adoption of Awaji criteria increases the yield of EMG positive segments significantly in the cervical (p<0.0005) and lumbosacral regions (P<0.0001), and upgrades 19 patients into probable and 1 patient into definite categories. Electromyographic abnormalities are distal limb-predominant in early stage ALS. A redefinition of an EDX-positive cervical or lumbosacral segment, with an emphasis on distal limb muscles, may result in an earlier ALS diagnosis. This article is protected by copyright. All rights reserved. © 2018 Wiley Periodicals, Inc.

Exercise-induced brachial artery vasodilation: effects of antioxidants and exercise training in elderly men

PubMed Central

Donato, Anthony J.; Uberoi, Abhimanyu; Bailey, Damian M.; Walter Wray, D.

2010-01-01

Aging, vascular function, and exercise are thought to have a common link in oxidative stress. Of the 28 subjects studied (young, 26 ± 2 yr; old, 71 ± 6 yr), 12 took part in a study to validate an antioxidant cocktail (AOC: vitamins C, E, and α-lipoic acid), while the remaining 8 young and 8 old subjects performed submaximal forearm handgrip exercise with placebo or AOC. Old subjects repeated forearm exercise with placebo or AOC following knee-extensor (KE) exercise training. Brachial arterial diameter and blood velocity (Doppler ultrasound) were measured at rest and during exercise. During handgrip exercise, brachial artery vasodilation in the old subjects was attenuated compared with that in young subjects following placebo (maximum = ∼3.0 and ∼6.0%, respectively). In contrast to the previously documented attenuation in exercise-induced brachial artery vasodilation in the young group with AOC, in the old subjects the AOC restored vasodilation (maximum = ∼7.0%) to match the young. KE training also improved exercise-induced brachial artery vasodilation. However, in the trained state, AOC administration no longer augmented brachial artery vasodilation in the elderly, but rather attenuated it. These data reveal an age-related pro-/antioxidant imbalance that impacts vascular function and show that exercise training is capable of restoring equilibrium such that vascular function is improved and the AOC-mediated reduction in free radicals now negatively impacts brachial artery vasodilation, as seen in the young. PMID:19966056

Coping strategies among patients with newly diagnosed amyotrophic lateral sclerosis.

PubMed

Jakobsson Larsson, Birgitta; Nordin, Karin; Askmark, Håkan; Nygren, Ingela

2014-11-01

To prospectively identify different coping strategies among newly diagnosed amyotrophic lateral sclerosis patients and whether they change over time and to determine whether physical function, psychological well-being, age and gender correlated with the use of different coping strategies. Amyotrophic lateral sclerosis is a fatal disease with impact on both physical function and psychological well-being. Different coping strategies are used to manage symptoms and disease progression, but knowledge about coping in newly diagnosed amyotrophic lateral sclerosis patients is scarce. This was a prospective study with a longitudinal and descriptive design. A total of 33 patients were included and evaluation was made at two time points, one to three months and six months after diagnosis. Patients were asked to complete the Motor Neuron Disease Coping Scale and the Hospital Anxiety and Depression Scale. Physical function was estimated using the revised Amyotrophic Lateral Sclerosis Functional Rating Scale. The most commonly used strategies were support and independence. Avoidance/venting and information seeking were seldom used at both time points. The use of information seeking decreased between the two time points. Men did not differ from women, but patients ≤64 years used positive action more often than older patients. Amyotrophic Lateral Sclerosis Functional Rating Scale was positively correlated with positive action at time point 1, but not at time point 2. Patients' psychological well-being was correlated with the use of different coping strategies. Support and independence were the most used coping strategies, and the use of different strategies changed over time. Psychological well-being was correlated with different coping strategies in newly diagnosed amyotrophic lateral sclerosis patients. The knowledge about coping strategies in early stage of the disease may help the nurses to improve and develop the care and support for these patients. © 2014 John Wiley

Quantification of the Interrelationship between Brachial-Ankle and Carotid-Femoral Pulse Wave Velocity in a Workplace Population.

PubMed

Cheng, Yi-Bang; Li, Yan; Sheng, Chang-Sheng; Huang, Qi-Fang; Wang, Ji-Guang

2016-04-01

Brachial-ankle pulse wave velocity (PWV) is increasingly used for the measurement of arterial stiffness. In the present study, we quantified the interrelationship between brachial-ankle and carotid-femoral PWV in a workplace population, and investigated the associations with cardiovascular risk factors and carotid intima-media thickness (IMT). Brachial-ankle and carotid-femoral PWV were measured using the Omron-Colin VP1000 and SphygmoCor devices, respectively. We investigated the interrelationship by the Pearson's correlation analysis and Bland-Altman plot, and performed sensitivity and specificity analyses. The 954 participants (mean ± standard deviation age 42.6 ± 14.2 years) included 630 (66.0%) men and 203 (21.3%) hypertensive patients. Brachial-ankle (13.4 ± 2.7 m/s) and carotid-femoral PWV (7.3 ± 1.6 m/s) were significantly correlated in all subjects (r = 0.75) as well as in men (r = 0.72) and women (r = 0.80) separately. For arterial stiffness defined as a carotid-femoral PWV of 10 m/s or higher, the sensitivity and specificity of brachial-ankle PWV of 16.7 m/s or higher were 72 and 94%, respectively. The area under the receiver operating characteristic curve was 0.953. In multiple stepwise regression, brachial-ankle and carotid-femoral PWV were significantly (p < 0.001) associated with age (partial r = 0.33 and 0.34, respectively) and systolic blood pressure (partial r = 0.71 and 0.66, respectively). In addition, brachial-ankle and carotid-femoral PWV were significantly (p < 0.001) associated with carotid IMT (r = 0.57 and 0.55, respectively) in unadjusted analysis, but not in analysis adjusted for cardiovascular risk factors (p ≥ 0.08). Brachial-ankle and carotid-femoral PWV were closely correlated, and had similar determinants. Brachial-ankle PWV can behave as an ease-of-use alternative measure of arterial stiffness for assessing cardiovascular risk.

A novel technique for teaching the brachial plexus.

PubMed

Lefroy, Henrietta; Burdon-Bailey, Victoria; Bhangu, Aneel; Abrahams, Peter

2011-09-01

The brachial plexus has posed problems for both students and teachers throughout generations of medical education. The anatomy is intricate, and traditional pictorial representations can be difficult to understand and learn. Few innovative teaching methods have been reported. The basic anatomy of the brachial plexus is core knowledge required by medical students to aid clinical examination and diagnosis. A more detailed understanding is necessary for a variety of specialists, including surgeons, anaesthetists and radiologists. Here, we present a novel, cheap and interactive method of teaching the brachial plexus. Using coloured pipe cleaners, teachers and students can construct three-dimensional models using different colours to denote the origin and outflow of each nerve. The three-dimensional nature of the model also allows for a better understanding of certain intricacies of the plexus. Students may use these models as adjuncts for self study, didactic lectures and tutorials. Compared with traditional textbooks and whiteboards, the pipe-cleaner model was preferred by medical students, and provided a higher level of student satisfaction. This was demonstrated and analysed using student feedback forms. Our model could be incorporated into current curricula to provide an effective and enjoyable way of rapidly teaching a difficult concept. Other such novel methods for teaching complex anatomical principles should be encouraged and explored. © Blackwell Publishing Ltd 2011.

Incidence of intravenous contrast extravasation: increased risk for patients with deep brachial catheter placement from the emergency department.

PubMed

Hardie, Andrew D; Kereshi, Borko

2014-06-01

Deep brachial intravenous catheter (IV) placement can be performed in emergency department patients with difficult vascular access, but the safety of deep brachial IV for iodinated contrast administration has not been assessed. This study compares the relative risk for extravasation of deep brachial IV compared with antecubital IV during power injected computed tomography (CT) examinations. A departmental practice quality improvement was performed to assess the rate of IV extravasation for all CT examinations during a 1 year period. De-identified data was analyzed with a waiver of informed consent to identify the rate and relative risk of iodinated contrast extravasation by catheter type. A total of 10,750 injections were performed, with 82 extravasation events (0.8 %). There were 51 extravasations of antecubital IV from approximately 8,599 placed (0.6 %). For 123 deep brachial IV placed, there were eight extravasations (6.5 %). The relative risk of a deep brachial IV extravasation was 9.4 compared to 0.4 for antecubital placement. Deep brachial IV demonstrated a markedly higher rate of contrast extravasation than antecubital IV. For power injected iodinated contrast administration, it is recommended to avoid the use of deep brachial IV whenever possible.

Percutaneous Coil Embolization for the Treatment of a Giant Brachial Artery Pseudoaneurym in a Child.

PubMed

Yuksel, Ahmet; Nas, Omer Fatih; Kan, Irem Iris; Tok, Mustafa; Erdogan, Cuneyt

2017-11-01

Brachial artery pseudoaneurysm is a rare phenomenon. When a diagnosis of brachial artery pseudoaneurysm is established, early and appropriate treatment should be performed as soon as possible to prevent possible complications, such as hemorrhage, rupture, and upper limb and finger losses. Open surgical repair is usually the cornerstone of treatment; however, we here report a case of giant brachial pseudoaneurysm in a 2-year-old girl, which was successfully treated with percutaneous coil embolization. Copyright © 2017 Elsevier Inc. All rights reserved.

Vascular patterns of upper limb: an anatomical study with accent on superficial brachial artery

PubMed Central

Kachlik, David; Konarik, Marek; Baca, Vaclav

2011-01-01

The aim of the study was to evaluate the terminal segmentation of the axillary artery and to present four cases of anomalous branching of the axillary artery, the superficial brachial artery (arteria brachialis superficialis), which is defined as the brachial artery that runs superficially to the median nerve. Totally, 130 cadaveric upper arms embalmed by classical formaldehyde technique from collections of the Department of Anatomy, Third Faculty of Medicine, Charles University in Prague, were macroscopically dissected with special focus on the branching arrangement of the axillary artery. The most distal part of the axillary artery (infrapectoral part) terminated in four cases as a bifurcation into two terminal branches: the superficial brachial artery and profunda brachii artery, denominated according to their relation to the median nerve. The profunda brachii artery primarily gave rise to the main branches of the infrapectoral part of the axillary artery. The superficial brachial artery descended to the cubital fossa where it assumed the usual course of the brachial artery in two cases and in the other two cases its branches (the radial and ulnar arteries) passed superficially to the flexors. The incidence of the superficial brachial artery in our study was 5% of cases. The reported incidence is a bit contradictory, from 0.12% to 25% of cases. The anatomical knowledge of the axillary region is of crucial importance for neurosurgeons and specialists using the radiodiagnostic techniques, particularly in cases involving traumatic injuries. The improved knowledge would allow more accurate diagnostic interpretations and surgical treatment. PMID:21342134

FUS GENE MUTATIONS IN FAMILIAL AND SPORADIC AMYOTROPHIC LATERAL SCLEROSIS

PubMed Central

Rademakers, Rosa; Stewart, Heather; DeJesus-Hernandez, Mariely; Krieger, Charles; Graff-Radford, Neill; Fabros, Marife; Briemberg, Hannah; Cashman, Neil; Eisen, Andrew; Mackenzie, Ian R. A.

2010-01-01

Introduction Mutations in the fused in sarcoma (FUS) gene have recently been found to cause familial amyotrophic lateral sclerosis (FALS). Methods We screened FUS in a cohort of 200 ALS patients [32 FALS and 168 sporadic ALS (SALS)]. Results In one FALS proband, we identified a mutation (p.R521C) that was also present in her affected daughter. Their clinical phenotype was remarkably similar and atypical of classic ALS, with symmetric proximal pelvic and pectoral weakness. Distal weakness and upper motor neuron features only developed late. Neuropathological examination demonstrated FUS-immunoreactive neuronal and glial inclusions in the spinal cord and many extramotor regions, but no TDP-43 pathology. We also identified a novel mutation (p.G187S) in one SALS patient. Overall, FUS mutations accounted for 3% of our non-SOD1, non-TARDBP FALS cases and 0.6% of SALS. Discussion This study demonstrates that the phenotype with FUS mutations extends beyond classical ALS. It suggests there are specific clinicogenetic correlations and provides the first detailed neuropathological description. PMID:20544928

Quantification of the Interrelationship between Brachial-Ankle and Carotid-Femoral Pulse Wave Velocity in a Workplace Population

PubMed Central

Cheng, Yi-Bang; Li, Yan; Sheng, Chang-Sheng; Huang, Qi-Fang; Wang, Ji-Guang

2016-01-01

Background Brachial-ankle pulse wave velocity (PWV) is increasingly used for the measurement of arterial stiffness. In the present study, we quantified the interrelationship between brachial-ankle and carotid-femoral PWV in a workplace population, and investigated the associations with cardiovascular risk factors and carotid intima-media thickness (IMT). Methods Brachial-ankle and carotid-femoral PWV were measured using the Omron-Colin VP1000 and SphygmoCor devices, respectively. We investigated the interrelationship by the Pearson's correlation analysis and Bland-Altman plot, and performed sensitivity and specificity analyses. Results The 954 participants (mean ± standard deviation age 42.6 ± 14.2 years) included 630 (66.0%) men and 203 (21.3%) hypertensive patients. Brachial-ankle (13.4 ± 2.7 m/s) and carotid-femoral PWV (7.3 ± 1.6 m/s) were significantly correlated in all subjects (r = 0.75) as well as in men (r = 0.72) and women (r = 0.80) separately. For arterial stiffness defined as a carotid-femoral PWV of 10 m/s or higher, the sensitivity and specificity of brachial-ankle PWV of 16.7 m/s or higher were 72 and 94%, respectively. The area under the receiver operating characteristic curve was 0.953. In multiple stepwise regression, brachial-ankle and carotid-femoral PWV were significantly (p < 0.001) associated with age (partial r = 0.33 and 0.34, respectively) and systolic blood pressure (partial r = 0.71 and 0.66, respectively). In addition, brachial-ankle and carotid-femoral PWV were significantly (p < 0.001) associated with carotid IMT (r = 0.57 and 0.55, respectively) in unadjusted analysis, but not in analysis adjusted for cardiovascular risk factors (p ≥ 0.08). Conclusions Brachial-ankle and carotid-femoral PWV were closely correlated, and had similar determinants. Brachial-ankle PWV can behave as an ease-of-use alternative measure of arterial stiffness for assessing cardiovascular risk. PMID:27195246

A unique retinal epitheliopathy is associated with amyotrophic lateral sclerosis/Parkinsonism-Dementia complex of Guam.

PubMed

Steele, John C; Wresch, Robert; Hanlon, Samuel D; Keystone, Jay; Ben-Shlomo, Yoav

2015-08-01

The aim of this work was to examine whether a linear retinal pigment epitheliopathy is associated with the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam. A total of 918 Guamanian Chamorros, with and without amyotrophic lateral sclerosis/parkinsonism-dementia complex, were examined cross-sectionally for linear retinal pigment epitheliopathy (LRPE). Overall, 239 Guamanians, who were neurologically asymptomatic, were followed for up to 20 years to determine the risk of developing amyotrophic lateral sclerosis/parkinsonism-dementia complex. The epitheliopathy was present in 59.7% (117 of 196) patients with amyotrophic lateral sclerosis/parkinsonism-dementia complex, but in only 24.7% (178 of 722) of subjects who were neurologically asymptomatic (age- and sex-adjusted risk difference: 35.0%; 95% confidence interval [CI]: 27.5-42.6; p < 0.0001). Prospectively, 15 of 50 cases with epitheliopathy developed amyotrophic lateral sclerosis/parkinsonism-dementia complex, compared to 4 of 189 cases without epitheliopathy (age- and sex-adjusted hazard ratio: 13.1; 95% CI: 4.0-43.1; P < 0.0001). Amyotrophic lateral sclerosis/parkinsonism-dementia complex is associated with an LRPE and predicts future neurological disease. Identifying the cause of this retinopathy could provide an understanding about the pathogenesis of amyotrophic lateral sclerosis/parkinsonism-dementia complex and related diseases. © 2015 International Parkinson and Movement Disorder Society.

The blind pushing technique for peripherally inserted central catheter placement through brachial vein puncture.

PubMed

Lee, Jae Myeong; Cho, Young Kwon; Kim, Han Myun; Song, Myung Gyu; Song, Soon-Young; Yeon, Jae Woo; Yoon, Dae Young; Lee, Sam Yeol

2018-03-01

The objective of this study was to conduct a prospective clinical trial evaluating the technical feasibility and short-term clinical outcome of the blind pushing technique for placement of pretrimmed peripherally inserted central catheters (PICCs) through brachial vein access. Patients requiring PICC placement at any of the three participating institutions were prospectively enrolled between January and December 2016. The review boards of all participating institutions approved this study, and informed consent was obtained from all patients. PICC placement was performed using the blind pushing technique and primary brachial vein access. The following data were collected from unified case report forms: access vein, obstacles during PICC advancement, procedure time, and postprocedural complications. During the 12-month study period, 1380 PICCs were placed in 1043 patients. Of these, 1092 PICCs placed in 837 patients were enrolled, with 834 PICCs (76%) and 258 PICCs (34%) placed through brachial vein and nonbrachial vein access, respectively. In both arms, obstacles were most commonly noted in the subclavian veins (n = 220) and axillary veins (n = 94). Successful puncture of the access vein was achieved at first try in 1028 PICCs (94%). The technical success rate was 99%, with 1055 PICCs (97%) placed within 120 seconds of procedure time and 1088 PICCs (99%) having the tip located at the ideal position. Follow-up Doppler ultrasound detected catheter-associated upper extremity deep venous thrombosis (UEDVT) for 18 PICCs in 16 patients and late symptomatic UEDVT for 16 PICCs in 16 patients (3.1%). Catheter-associated UEDVT was noted for 28 PICCs (82%) and 6 PICCs (18%) placed through brachial vein and nonbrachial vein access, respectively. The incidence of obstacles and the procedure time (<120 seconds) differed significantly between brachial vein and nonbrachial vein access (P = .001). There was no statistically significant difference between brachial vein and

The prognostic value of concurrent phrenic nerve palsy in newborn babies with neonatal brachial plexus palsy.

PubMed

Yoshida, Kiyoshi; Kawabata, Hidehiko

2015-06-01

To investigate the prognostic value of concurrent phrenic nerve palsy for predicting spontaneous motor recovery in neonatal brachial plexus palsy. We reviewed the records of 366 neonates with brachial plexus palsy. The clinical and follow-up data of patients with and without phrenic nerve palsy were compared. Of 366 newborn babies with neonatal brachial plexus palsy, 21 (6%) had concurrent phrenic nerve palsy. Sixteen of these neonates had upper-type palsy and 5 had total-type palsy. Poor spontaneous motor recovery was observed in 13 neonates with concurrent phrenic nerve palsy (62%) and in 129 without concurrent phrenic nerve palsy (39%). Among neonates born via vertex delivery, poor motor recovery was observed in 7 of 9 (78%) neonates with concurrent phrenic nerve palsy and 115 of 296 (39%) without concurrent phrenic nerve palsy. Concurrent phrenic nerve palsy in neonates with brachial plexus palsy has prognostic value in predicting poor spontaneous motor recovery of the brachial plexus, particularly after vertex delivery. Therapeutic IV. Copyright © 2015 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

Neonatal brachial plexus palsy--management and prognostic factors.

PubMed

Yang, Lynda J-S

2014-06-01

Successful treatment of patients with neonatal brachial plexus palsy (NBPP) begins with a thorough understanding of the anatomy of the brachial plexus and of the pathophysiology of nerve injury via which the brachial plexus nerves stretched in the perinatal period manifest as a weak or paralyzed upper extremity in the newborn. NBPP can be classified by systems that can guide the prognosis and the management as these systems are based on the extent and severity of nerve injury, anatomy of nerve injury, and clinical presentation. Serial physical examinations, supplemented by a thorough maternal and perinatal history, are critical to the formulation of the treatment plan that relies upon occupational/physical therapy and rehabilitation management but may include nerve reconstruction and secondary musculoskeletal surgeries. Adjunctive imaging and electrodiagnostic studies provide additional information to guide prognosis and treatment. As research improves not only the technical aspects of NBPP treatment but also the ability to assess the activity and participation as well as body structure and function of NBPP patients, the functional outcomes for affected infants have an overall optimistic prognosis, with the majority recovering adequate functional use of the affected arm. Of importance are (i) early referral to interdisciplinary specialty clinics that can provide up-to-date advances in clinical care and (ii) increasing research/awareness of the psychosocial and patient-reported quality-of-life issues that surround the chronic disablement of NBPP. Copyright © 2014 Elsevier Inc. All rights reserved.

[History of hereditary motor and sensory neuropathy with proximal dominant involvement (HMSN-P)].

PubMed

Takashima, Hiroshi

2013-01-01

We established a new disease autosomal dominant hereditary motor and sensory neuropathy with proximal dominant involvement (HMSNP) in 1997, in Okinawa, Japan. This disease is characterized by proximal dominant neurogenic atrophy with fasciculations, painful muscle cramp, obvious sensory nerve involvement, areflexia, high incidence of elevated creatine kinase levels, hyperlipidemia and hyperglycemia. (MIM %604484). HMSNP is so called or HMSNO (HMSN OKINAWA type),. These clinical features resembled those of Kennedy-Alter-Sung syndrome. Most HMSNP patients have severe muscle atrophy and finally the tracheostomy and artificial ventilation are required. Therefore, we initially thought to classify HMSNP into a subtype of motor neuron disease (MND) like familial amyotrophic lateral sclerosis (FALS) or spinal muscular atrophy (SMA). However, the general consensus for MND was no sensory involvement. Therefore, as the disease showed severe sensory involvement, we categorized HMSNP in subtype of HMSN at that time. We also reported the pathology of HMSNP, showing severely decreased anterior horn cells, decreased posterior horn cells, and loss of posterior funiculus in the spinal cord.

Prevalence of brachial plexus injuries in patients with scapular fractures: A National Trauma Data Bank review

PubMed Central

Chamata, Edward; Mahabir, Raman; Jupiter, Daniel; Weber, Robert A

2014-01-01

BACKGROUND: Studies investigating the prevalence of brachial plexus injuries associated with scapular fractures are sparse, and are frequently limited by small sample sizes and often restricted to single-centre experience. OBJECTIVE: To determine the prevalence of brachial plexus injuries associated with scapular fractures; to determine how the prevalence varies with the region of the scapula injured; and to assess which specific nerves of the brachial plexus were involved. METHODS: The present study was a retrospective review of data from the National Trauma Data Bank over a five-year period (2007 to 2011). RESULTS: Of 68,118 patients with scapular fractures, brachial plexus injury was present in 1173 (1.72%). In patients with multiple scapular fractures, the prevalence of brachial plexus injury was 3.12%, and ranged from 1.52% to 2.22% in patients with single scapular fractures depending on the specific anatomical location of the fracture. Of the 426 injuries with detailed information on nerve injury, 208 (49%) involved the radial nerve, 113 (26.5%) the ulnar nerve, 65 (15%) the median nerve, 36 (8.5%) the axillary nerve and four (1%) the musculocutaneous nerve. CONCLUSION: The prevalence of brachial plexus injuries in patients with scapular fractures was 1.72%. The prevalence was similar across anatomical regions for single scapular fracture and was higher with multiple fractures. The largest percentage of nerve injuries were to the radial nerve. PMID:25535462

C8 cross transfer for the treatment of caudal brachial plexus avulsion in three dogs.

PubMed

Moissonnier, Pierre; Carozzo, Claude; Thibaut, Jean-Laurent; Escriou, Catherine; Hidalgo, Antoine; Blot, Stéphane

2017-01-01

To evaluate the cervical nerve 8 cross-transfer technique (C8CT) as a part of surgical treatment of caudal brachial plexus avulsion (BPA) in the dog. Case series. Client-owned dogs suspected to have caudal BPA based on neurological examination and electrophysiological testing (n = 3). The distal stump of the surgically transected contralateral C8 ventral branch (donor) was bridged to the proximal stump of the avulsed C8 ventral branch (recipient) and secured with 9-0 polypropylene suture under an operating microscope. A carpal panarthrodesis was performed on the injured limb after C8CT. Surgical exploration confirmed avulsion of nerve roots C7, C8, and T1 in all cases. There was no evidence of an iatrogenic effect on the donor forelimb. Gradual improvement in function of the affected forelimb occurred in all dogs, with eventual recovery of voluntary elbow extension. Reinnervation was evident in EMG recordings 6 months postoperatively in all three dogs. Stimulation of the donor C8 ventral branch led to motor evoked potentials in the avulsed side triceps brachialis and radial carpus extensor muscles. Variable functional outcome was observed in the 3 dogs during clinical evaluation 3-4 years after surgery. Digital abrasion wounds, distal interphalangeal infectious arthritis, and self-mutilation necessitated distal phalanx amputation of digits 3 and 4 in 2 dogs. C8CT provided partial reconnection of the donor C8 ventral branch to the avulsed brachial plexus in the 3 dogs of this series. Reinnervation resulted in active elbow extension and promoted functional recovery in the affected limb. © 2017 The American College of Veterinary Surgeons.

Effectiveness of primary conservative management for infants with obstetric brachial plexus palsy.

PubMed

Bialocerkowski, Andrea; Kurlowicz, Kirsty; Vladusic, Sharon; Grimmer, Karen

Obstetric brachial plexus palsy, a complication of childbirth, occurs in 1-3 per 1000 live births internationally. Traction and/or compression of the brachial plexus is thought to be the primary mechanism of injury and this may occur in utero, during the descent through the birth canal or during delivery. This results in a spectrum of injuries that vary in severity, extent of damage and functional use of the affected upper limb. Most infants receive treatment, such as conservative management (physiotherapy, occupational therapy) or surgery; however, there is controversy regarding the most appropriate form of management. To date, no synthesised evidence is available regarding the effectiveness of primary conservative management for obstetric brachial plexus palsy. The objective of this review was to systematically assess the literature and present the best available evidence that investigated the effectiveness of primary conservative management for infants with obstetric brachial plexus palsy. A systematic literature search was performed using 14 databases: TRIP, MEDLINE, CINAHL, AMED, Web of Science, Proquest 5000, Evidence Based Medicine Reviews, Expanded Academic ASAP, Meditext, Science Direct, Physiotherapy Evidence Database, Proquest Digital Dissertations, Open Archives Initiative Search Engine, Australian Digital Thesis Program. Those studies that were reported in English and published over the last decade (July 1992 to June 2003) were included in this review. Quantitative studies that investigated the effectiveness of primary conservative management for infants with obstetric brachial plexus palsy were eligible for inclusion in this review. This excluded studies that solely investigated the effect of primary surgery for these infants, management of secondary deformities and the investigation of the effects of pharmacological agents, such as botulinum toxin. Two independent reviewers assessed the eligibility of each study for inclusion into the review, the

Dietary BMAA Exposure in an Amyotrophic Lateral Sclerosis Cluster from Southern France

PubMed Central

Masseret, Estelle; Banack, Sandra; Boumédiène, Farid; Abadie, Eric; Brient, Luc; Pernet, Fabrice; Juntas-Morales, Raoul; Pageot, Nicolas; Metcalf, James; Cox, Paul; Camu, William

2013-01-01

Background Dietary exposure to the cyanotoxin BMAA is suspected to be the cause of amyotrophic lateral sclerosis in the Western Pacific Islands. In Europe and North America, this toxin has been identified in the marine environment of amyotrophic lateral sclerosis clusters but, to date, only few dietary exposures have been described. Objectives We aimed at identifying cluster(s) of amyotrophic lateral sclerosis in the Hérault district, a coastal district from Southern France, and to search, in the identified area(s), for the existence of a potential dietary source of BMAA. Methods A spatio-temporal cluster analysis was performed in the district, considering all incident amyotrophic lateral sclerosis cases identified from 1994 to 2009 by our expert center. We investigated the cluster area with serial collections of oysters and mussels that were subsequently analyzed blind for BMAA concentrations. Results We found one significant amyotrophic lateral sclerosis cluster (p = 0.0024), surrounding the Thau lagoon, the most important area of shellfish production and consumption along the French Mediterranean coast. BMAA was identified in mussels (1.8 µg/g to 6.0 µg/g) and oysters (0.6 µg/g to 1.6 µg/g). The highest concentrations of BMAA were measured during summer when the highest picocyanobacteria abundances were recorded. Conclusions While it is not possible to ascertain a direct link between shellfish consumption and the existence of this ALS cluster, these results add new data to the potential association of BMAA with sporadic amyotrophic lateral sclerosis, one of the most severe neurodegenerative disorder. PMID:24349504

Dietary BMAA exposure in an amyotrophic lateral sclerosis cluster from southern France.

PubMed

Masseret, Estelle; Banack, Sandra; Boumédiène, Farid; Abadie, Eric; Brient, Luc; Pernet, Fabrice; Juntas-Morales, Raoul; Pageot, Nicolas; Metcalf, James; Cox, Paul; Camu, William

2013-01-01

Dietary exposure to the cyanotoxin BMAA is suspected to be the cause of amyotrophic lateral sclerosis in the Western Pacific Islands. In Europe and North America, this toxin has been identified in the marine environment of amyotrophic lateral sclerosis clusters but, to date, only few dietary exposures have been described. We aimed at identifying cluster(s) of amyotrophic lateral sclerosis in the Hérault district, a coastal district from Southern France, and to search, in the identified area(s), for the existence of a potential dietary source of BMAA. A spatio-temporal cluster analysis was performed in the district, considering all incident amyotrophic lateral sclerosis cases identified from 1994 to 2009 by our expert center. We investigated the cluster area with serial collections of oysters and mussels that were subsequently analyzed blind for BMAA concentrations. We found one significant amyotrophic lateral sclerosis cluster (p = 0.0024), surrounding the Thau lagoon, the most important area of shellfish production and consumption along the French Mediterranean coast. BMAA was identified in mussels (1.8 µg/g to 6.0 µg/g) and oysters (0.6 µg/g to 1.6 µg/g). The highest concentrations of BMAA were measured during summer when the highest picocyanobacteria abundances were recorded. While it is not possible to ascertain a direct link between shellfish consumption and the existence of this ALS cluster, these results add new data to the potential association of BMAA with sporadic amyotrophic lateral sclerosis, one of the most severe neurodegenerative disorder.

Juvenile-onset Sporadic Amyotrophic Lateral Sclerosis with a Frameshift FUS Gene Mutation Presenting Unique Neuroradiological Findings and Cognitive Impairment.

PubMed

Hirayanagi, Kimitoshi; Sato, Masayuki; Furuta, Natsumi; Makioka, Kouki; Ikeda, Yoshio

2016-01-01

A 24-year-old Japanese woman developed anterocollis, weakness of the proximal arms, and subsequent cognitive impairment. A neurological examination revealed amyotrophic lateral sclerosis (ALS) without a family history. Systemic muscle atrophy progressed rapidly. Cerebral MRI clearly exhibited high signal intensities along the bilateral pyramidal tracts. An analysis of the FUS gene revealed a heterozygous two-base pair deletion, c.1507-1508delAG (p.G504WfsX515). A subset of juvenile-onset familial/sporadic ALS cases with FUS gene mutations reportedly demonstrates mental retardation or learning difficulty. Our study emphasizes the importance of conducting a FUS gene analysis in juvenile-onset ALS cases, even when no family occurrence is confirmed.

Correlation between ultrasound imaging, cross-sectional anatomy, and histology of the brachial plexus: a review.

PubMed

van Geffen, Geert J; Moayeri, Nizar; Bruhn, Jörgen; Scheffer, Gert J; Chan, Vincent W; Groen, Gerbrand J

2009-01-01

The anatomy of the brachial plexus is complex. To facilitate the understanding of the ultrasound appearance of the brachial plexus, we present a review of important anatomic considerations. A detailed correlation of reconstructed, cross-sectional gross anatomy and histology with ultrasound sonoanatomy is provided.

Microstructural Correlates of Emotional Attribution Impairment in Non-Demented Patients with Amyotrophic Lateral Sclerosis.

PubMed

Crespi, Chiara; Cerami, Chiara; Dodich, Alessandra; Canessa, Nicola; Iannaccone, Sandro; Corbo, Massimo; Lunetta, Christian; Falini, Andrea; Cappa, Stefano F

2016-01-01

Impairments in the ability to recognize and attribute emotional states to others have been described in amyotrophic lateral sclerosis patients and linked to the dysfunction of key nodes of the emotional empathy network. Microstructural correlates of such disorders are still unexplored. We investigated the white-matter substrates of emotional attribution deficits in a sample of amyotrophic lateral sclerosis patients without cognitive decline. Thirteen individuals with either probable or definite amyotrophic lateral sclerosis and 14 healthy controls were enrolled in a Diffusion Tensor Imaging study and administered the Story-based Empathy Task, assessing the ability to attribute mental states to others (i.e., Intention and Emotion attribution conditions). As already reported, a significant global reduction of empathic skills, mainly driven by a failure in Emotion Attribution condition, was found in amyotrophic lateral sclerosis patients compared to healthy subjects. The severity of this deficit was significantly correlated with fractional anisotropy along the forceps minor, genu of corpus callosum, right uncinate and inferior fronto-occipital fasciculi. The involvement of frontal commissural fiber tracts and right ventral associative fronto-limbic pathways is the microstructural hallmark of the impairment of high-order processing of socio-emotional stimuli in amyotrophic lateral sclerosis. These results support the notion of the neurofunctional and neuroanatomical continuum between amyotrophic lateral sclerosis and frontotemporal dementia.

Screening for Peripheral Artery Disease and Cardiovascular Disease Risk Assessment with Ankle Brachial Index in Adults

MedlinePlus

... Force Recommendations Screening for Peripheral Artery Disease and Cardiovascular Disease Risk Assessment with Ankle Brachial Index in Adults ... on Screening for Peripheral Artery Disease (PAD) and Cardiovascular Disease (CVD) Risk Assessment with Ankle Brachial Index (ABI) ...

Immune system alterations in amyotrophic lateral sclerosis.

PubMed

Hovden, H; Frederiksen, J L; Pedersen, S W

2013-11-01

Amyotrophic lateral sclerosis is a disease of which the underlying cause and pathogenesis are unknown. Cumulatative data clearly indicates an active participation by the immune system in the disease. An increasingly recognized theory suggests a non-cell autonomous mechanism, meaning that multiple cells working together are necessary for the pathogenesis of the disease. Observed immune system alterations could indicate an active participation in this mechanism. Damaged motor neurons are able to activate microglia, astrocytes and the complement system, which further can influence each other and contribute to neurodegeneration. Infiltrating peripheral immune cells appears to correlate with disease progression, but their significance and composition is unclear. The deleterious effects of this collaborating system of cells appear to outweigh the protective aspects, and revealing this interplay might give more insight into the disease. Markers from the classical complement pathway are elevated where its initiator C1q appears to derive primarily from motor neurons. Activated microglia and astrocytes are found in close proximity to dying motor neurons. Their activation status and proliferation seemingly increases with disease progression. Infiltrating monocytes, macrophages and T cells are associated with these areas, although with mixed reports regarding T cell composition. This literature review will provide evidence supporting the immune system as an important part of ALS disease mechanism and present a hypothesis to direct the way for further studies. © 2013 John Wiley & Sons A/S.

Postfixed brachial plexus radiculopathy due to thoracic disc herniation in a collegiate wrestler: a case report.

PubMed

Kuzma, Scott A; Doberstein, Scott T; Rushlow, David R

2013-01-01

To present the unique case of a collegiate wrestler with C7 neurologic symptoms due to T1-T2 disc herniation. A 23-year-old male collegiate wrestler injured his neck in a wrestling tournament match and experienced pain, weakness, and numbness in his left upper extremity. He completed that match and 1 additional match that day with mild symptoms. Evaluation by a certified athletic trainer 6 days postinjury showed radiculopathy in the C7 distribution of his left upper extremity. He was evaluated further by the team physician, a primary care physician, and a neurosurgeon. Cervical spine injury, stinger/burner, peripheral nerve injury, spinal cord injury, thoracic outlet syndrome, brachial plexus radiculopathy. The patient initially underwent nonoperative management with ice, heat, massage, electrical stimulation, shortwave diathermy, and nonsteroidal anti-inflammatory drugs without symptom resolution. Cervical spine radiographs were negative for bony pathologic conditions. Magnetic resonance imaging showed evidence of T1-T2 disc herniation. The patient underwent surgery to resolve the symptoms and enable him to participate for the remainder of the wrestling season. Whereas brachial plexus radiculopathy commonly is seen in collision sports, a postfixed brachial plexus in which the T2 nerve root has substantial contribution to the innervation of the upper extremity is a rare anatomic variation with which many health care providers are unfamiliar. The injury sustained by the wrestler appeared to be C7 radiculopathy due to a brachial plexus traction injury. However, it ultimately was diagnosed as radiculopathy due to a T1-T2 thoracic intervertebral disc herniation causing impingement of a postfixed brachial plexus and required surgical intervention. Athletic trainers and physicians need to be aware of the anatomic variations of the brachial plexus when evaluating and caring for patients with suspected brachial plexus radiculopathies.

Presence of time-dependent diffusion in the brachial plexus.

PubMed

Mahbub, Zaid B; Peters, Andrew M; Gowland, Penny A

2018-02-01

This work describes the development of a method to measure the variation of apparent diffusion coefficient (ADC) with diffusion time (Δ) in the brachial plexus, as a potential method of probing microstructure. Diffusion-weighted MRI with body signal suppression was used to highlight the nerves from surrounding tissues, and sequence parameters were optimized for sensitivity to change with diffusion time. A porous media-restricted diffusion model based on the Latour-Mitra equation was fitted to the diffusion time-dependent ADC data from the brachial plexus nerves and cord. The ADC was observed to reduce at long diffusion times, confirming that diffusion was restricted in the nerves and cord in healthy subjects. T2 of the nerves was measured to be 80 ± 5 ms, the diffusion coefficient was found to vary from (1.5 ± 0.1) × 10 -3 mm 2 /s at a diffusion time of 18.3 ms to (1.0 ± 0.2) × 10 -3 mm 2 /s at a diffusion time of 81.3 ms. A novel method of probing restricted diffusion in the brachial plexus was developed. Resulting parameters were comparable with values obtained previously on biological systems. Magn Reson Med 79:789-795, 2018. © 2017 International Society for Magnetic Resonance in Medicine. © 2017 International Society for Magnetic Resonance in Medicine.

Serum microRNAs in patients with genetic amyotrophic lateral sclerosis and pre-manifest mutation carriers.

PubMed

Freischmidt, Axel; Müller, Kathrin; Zondler, Lisa; Weydt, Patrick; Volk, Alexander E; Božič, Anže Lošdorfer; Walter, Michael; Bonin, Michael; Mayer, Benjamin; von Arnim, Christine A F; Otto, Markus; Dieterich, Christoph; Holzmann, Karlheinz; Andersen, Peter M; Ludolph, Albert C; Danzer, Karin M; Weishaupt, Jochen H

2014-11-01

Knowledge about the nature of pathomolecular alterations preceding onset of symptoms in amyotrophic lateral sclerosis is largely lacking. It could not only pave the way for the discovery of valuable therapeutic targets but might also govern future concepts of pre-manifest disease modifying treatments. MicroRNAs are central regulators of transcriptome plasticity and participate in pathogenic cascades and/or mirror cellular adaptation to insults. We obtained comprehensive expression profiles of microRNAs in the serum of patients with familial amyotrophic lateral sclerosis, asymptomatic mutation carriers and healthy control subjects. We observed a strikingly homogenous microRNA profile in patients with familial amyotrophic lateral sclerosis that was largely independent from the underlying disease gene. Moreover, we identified 24 significantly downregulated microRNAs in pre-manifest amyotrophic lateral sclerosis mutation carriers up to two decades or more before the estimated time window of disease onset; 91.7% of the downregulated microRNAs in mutation carriers overlapped with the patients with familial amyotrophic lateral sclerosis. Bioinformatic analysis revealed a consensus sequence motif present in the vast majority of downregulated microRNAs identified in this study. Our data thus suggest specific common denominators regarding molecular pathogenesis of different amyotrophic lateral sclerosis genes. We describe the earliest pathomolecular alterations in amyotrophic lateral sclerosis mutation carriers known to date, which provide a basis for the discovery of novel therapeutic targets and strongly argue for studies evaluating presymptomatic disease-modifying treatment in amyotrophic lateral sclerosis. © 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.

Effectiveness of primary conservative management for infants with obstetric brachial plexus palsy.

PubMed

Bialocerkowski, Andrea; Kurlowicz, Kirsty; Vladusic, Sharon; Grimmer, Karen

2005-03-01

Background  Obstetric brachial plexus palsy, a complication of childbirth, occurs in 1-3 per 1000 live births internationally. Traction and/or compression of the brachial plexus is thought to be the primary mechanism of injury and this may occur in utero, during the descent through the birth canal or during delivery. This results in a spectrum of injuries that vary in severity, extent of damage and functional use of the affected upper limb. Most infants receive treatment, such as conservative management (physiotherapy, occupational therapy) or surgery; however, there is controversy regarding the most appropriate form of management. To date, no synthesised evidence is available regarding the effectiveness of primary conservative management for obstetric brachial plexus palsy. Objectives  The objective of this review was to systematically assess the literature and present the best available evidence that investigated the effectiveness of primary conservative management for infants with obstetric brachial plexus palsy. Search strategy  A systematic literature search was performed using 14 databases: TRIP, MEDLINE, CINAHL, AMED, Web of Science, Proquest 5000, Evidence Based Medicine Reviews, Expanded Academic ASAP, Meditext, Science Direct, Physiotherapy Evidence Database, Proquest Digital Dissertations, Open Archives Initiative Search Engine, Australian Digital Thesis Program. Those studies that were reported in English and published over the last decade (July 1992 to June 2003) were included in this review. Selection criteria  Quantitative studies that investigated the effectiveness of primary conservative management for infants with obstetric brachial plexus palsy were eligible for inclusion in this review. This excluded studies that solely investigated the effect of primary surgery for these infants, management of secondary deformities and the investigation of the effects of pharmacological agents, such as botulinum toxin. Data collection and analysis�

True Brachial Artery Aneurysm in a Patient with Vascular Access for Haemodialysis and Kidney Graft.

PubMed

Correia, Mafalda; Marinho, André; Mendes, Carolina; Antunes, Luís; Gonçalves, Óscar

2017-01-01

True brachial artery aneurysms are rare and some of them have been described as a late complication in patients with vascular access for haemodialysis and kidney graft. The purpose of this paper is to present a clinical case of a patient with a true brachial artery aneurysm and its following treatment. This case concerns a caucasian male patient with 43 years old who had vesicoureteral reflux at 7 years old, and subsequent end-stage renal disease, and started on haemodialysis at the age of twelve. The patient had homolateral radial and brachiocephalic arteriovenous fistulas (AVF), two sequential kidney grafts and was under immunosuppressant therapy for several years. As part of the medical history he also had bilateral amaurosis, Hepatitis B and C and was submitted to total parathyroidectomy and a following auto-transplant. In the latest years the patient presented with several aneurysms related to the vascular access. After the finding of an anastomotic false aneurysm and venous aneurysms complicating the AVF, the patient had removal of the aneurysms and ligation of the AVF. Later, he was diagnosed, in different times, with two true brachial artery aneurysms. At the time of the diagnosis of the first true aneurysm, the patient presented with local pain and occasional paraesthesias relative to compression symptoms. At physical examination the patient had a brachial pulsatile mass and a palpable radial pulse. The Doppler ultrasound exam revealed a true brachial artery aneurysm with 4,5 cm diameter. After two years, the patient was once again diagnosed with a true brachial artery aneurysm with 3,1 cm diameter. At this time the patient was asymptomatic and had palpable brachial mass and radial pulse. At the time of the diagnosis of the first true brachial aneurysm the patient was submitted to partial aneurysmectomy and brachiobrachial graft with PTFE 8. The patency of the graft persisted until the diagnosis of the second aneurysm. After two years, the patient was

Constraint-Induced Movement Therapy for Children with Obstetric Brachial Plexus Palsy: Two Single-Case Series

ERIC Educational Resources Information Center

Buesch, Francisca Eugster

2010-01-01

The objective of this pilot study was to investigate the feasibility of constraint-induced movement therapy (CIMT) in children with obstetric brachial plexus palsy and receive preliminary information about functional improvements. Two patients (age 12 years) with obstetric brachial plexus palsy were included for a 126-h home-based CIMT…

Swelling in the upper arm: the presentation and management of an isolated brachial artery aneurysm

PubMed Central

Alagaratnam, S; Lau, T; Munro, M; Loh, A

2011-01-01

True aneurysms of the brachial artery are uncommon. We describe the presentation and surgical management of an isolated, brachial artery aneurysm in a 64-year-old woman. Excision of the aneurysm and long saphenous venous interposition grafting was performed with no postoperative complications and histology demonstrated true aneurysmal degeneration. PMID:21943445

Microstructural Correlates of Emotional Attribution Impairment in Non-Demented Patients with Amyotrophic Lateral Sclerosis

PubMed Central

Cerami, Chiara; Dodich, Alessandra; Canessa, Nicola; Iannaccone, Sandro; Corbo, Massimo; Lunetta, Christian; Falini, Andrea; Cappa, Stefano F.

2016-01-01

Impairments in the ability to recognize and attribute emotional states to others have been described in amyotrophic lateral sclerosis patients and linked to the dysfunction of key nodes of the emotional empathy network. Microstructural correlates of such disorders are still unexplored. We investigated the white-matter substrates of emotional attribution deficits in a sample of amyotrophic lateral sclerosis patients without cognitive decline. Thirteen individuals with either probable or definite amyotrophic lateral sclerosis and 14 healthy controls were enrolled in a Diffusion Tensor Imaging study and administered the Story-based Empathy Task, assessing the ability to attribute mental states to others (i.e., Intention and Emotion attribution conditions). As already reported, a significant global reduction of empathic skills, mainly driven by a failure in Emotion Attribution condition, was found in amyotrophic lateral sclerosis patients compared to healthy subjects. The severity of this deficit was significantly correlated with fractional anisotropy along the forceps minor, genu of corpus callosum, right uncinate and inferior fronto-occipital fasciculi. The involvement of frontal commissural fiber tracts and right ventral associative fronto-limbic pathways is the microstructural hallmark of the impairment of high-order processing of socio-emotional stimuli in amyotrophic lateral sclerosis. These results support the notion of the neurofunctional and neuroanatomical continuum between amyotrophic lateral sclerosis and frontotemporal dementia. PMID:27513746

Brachial plexus injury management through upper extremity amputation with immediate postoperative prostheses.

PubMed

Malone, J M; Leal, J M; Underwood, J; Childers, S J

1982-02-01

Management of patients with brachial plexus injuries requires a team approach so that all aspects of their care are addressed simultaneously. This report examines elective amputation and prosthetic rehabilitation in a patient with brachial plexus avulsion of the left arm. The best possibility for good prosthetic rehabilitation is the early application of prosthetic devices with intensive occupational therapy. Using this type of approach, we have achieved significant improvement in amputation rehabilitation of upper extremity amputees treated with immediate postoperative conventional electric and myoelectric prostheses.

Lockhart Clarke’s contribution to the description of amyotrophic lateral sclerosis

PubMed Central

Turner, Martin R.; Swash, Michael; Ebers, George C.

2011-01-01

The definition of the clinicopathological entity of amyotrophic lateral sclerosis evolved over half a century. Although the definitive term amyotrophic lateral sclerosis that acknowledged both upper and lower motor neuron involvement was attributed to Jean-Martin Charcot in 1874, his initial case was published nearly a decade earlier; and it is accepted that, from at least the 1830s, several others (including Charles Bell, François-Amilcar Aran and Jean Cruveilhier) had already recognized a progressive lower motor neuron-only syndrome within a broader, clinically-defined group of disorders, termed progressive muscular atrophy. Although William Gowers first grouped the three phenotypes of amyotrophic lateral sclerosis, progressive muscular atrophy and progressive bulbar palsy together as part of the same syndrome, the term motor neuron disease, as an over-arching label, was not suggested until nearly a century later by W. Russell Brain. Augustus Jacob Lockhart Clarke (1817–80) is best known for his descriptions of spinal cord anatomy. However, in two detailed case reports from the 1860s, he carried out rigorous post-mortem neuropathological studies of what appear to be classical cases of amyotrophic lateral sclerosis. Furthermore, he recognized the additional involvement of the corticospinal tracts that distinguished this from progressive muscular atrophy. Several aspects of the exquisite clinical histories documented as part of both studies, one by Charles Bland Radcliffe, resonate with contemporary debates concerning the evolution of disease in amyotrophic lateral sclerosis. These ‘past masters’ still have much to teach us. PMID:20576696

Association between alcohol consumption and amyotrophic lateral sclerosis: a meta-analysis of five observational studies.

PubMed

E, Meng; Yu, Sufang; Dou, Jianrui; Jin, Wu; Cai, Xiang; Mao, Yiyang; Zhu, Daojian; Yang, Rumei

2016-08-01

The purpose of this study is to examine the association between alcohol consumption and amyotrophic lateral sclerosis. Published literature on the association between alcohol consumption and amyotrophic lateral sclerosis was retrieved from the PubMed and Embase databases. Two authors independently extracted the data. The quality of the identified studies was evaluated according to the Newcastle-Ottawa scale. Subgroup and sensitivity analyses were performed and publication bias was assessed. Five articles, including one cohort study and seven case-control studies, and a total of 431,943 participants, were identified. The odds ratio for the association between alcohol consumption and amyotrophic lateral sclerosis was 0.57 (95 % confidence interval 0.51-0.64). Subgroup and sensitivity analyses confirmed the result. Evidence for publication bias was detected. Alcohol consumption reduced the risk of developing amyotrophic lateral sclerosis compared with non-drinking. Alcohol, therefore, has a potentially neuroprotective effect on the development of amyotrophic lateral sclerosis.

Comparison of two automatic methods for the assessment of brachial artery flow-mediated dilation.

PubMed

Faita, Francesco; Masi, Stefano; Loukogeorgakis, Stavros; Gemignani, Vincenzo; Okorie, Mike; Bianchini, Elisabetta; Charakida, Marietta; Demi, Marcello; Ghiadoni, Lorenzo; Deanfield, John Eric

2011-01-01

Brachial artery flow-mediated dilation (FMD) is associated with risk factors providing information on cardiovascular prognosis. Despite the large effort to standardize the methodology, the FMD examination is still characterized by problems of reproducibility and reliability that can be partially overcome with the use of automatic systems. We developed real-time software for the assessment of brachial FMD (FMD Studio, Institute of Clinical Physiology, Pisa, Italy) from ultrasound images. The aim of this study is to compare our system with another automatic method (Brachial Analyzer, MIA LLC, IA, USA) which is currently considered as a reference method in FMD assessment. The agreement between systems was assessed as follows. Protocol 1: Mean baseline (Basal), maximal (Max) brachial artery diameter after forearm ischemia and FMD, calculated as maximal percentage diameter increase, have been evaluated in 60 recorded FMD sequences. Protocol 2: Values of diameter and FMD have been evaluated in 618 frames extracted from 12 sequences. All biases are negligible and standard deviations of the differences are satisfactory (protocol 1: -0.27 ± 0.59%; protocol 2: -0.26 ± 0.61%) for FMD measurements. Analysis times were reduced (-33%) when FMD Studio is used. Rejected examinations due to the poor quality were 2% with the FMD Studio and 5% with the Brachial Analyzer. In conclusion, the compared systems show a optimal grade of agreement and they can be used interchangeably. Thus, the use of a system characterized by real-time functionalities could represent a referral method for assessing endothelial function in clinical trials.

Comparison of brachial artery vasoreactivity in elite power athletes and age-matched controls.

PubMed

Welsch, Michael A; Blalock, Paul; Credeur, Daniel P; Parish, Tracie R

2013-01-01

Elite endurance athletes typically have larger arteries contributing to greater skeletal muscle blood flow, oxygen and nutrient delivery and improved physical performance. Few studies have examined structural and functional properties of arteries in power athletes. To compare the size and vasoreactivity of the brachial artery of elite power athletes to age-matched controls. It was hypothesized brachial artery diameters of athletes would be larger, have less vasodilation in response to cuff occlusion, but more constriction after a cold pressor test than age-matched controls. Eight elite power athletes (age = 23 ± 2 years) and ten controls (age = 22 ± 1 yrs) were studied. High-resolution ultrasonography was used to assess brachial artery diameters at rest and following 5 minutes of forearm occlusion (Brachial Artery Flow Mediated Dilation = BAFMD) and a cold pressor test (CPT). Basic fitness measures included a handgrip test and 3-minute step test. Brachial arteries of athletes were larger (Athletes 5.39 ± 1.51 vs. 3.73 ± 0.71 mm, p<0.05), had greater vasodilatory (BAFMD%: Athletes: 8.21 ± 1.78 vs. 5.69 ± 1.56%) and constrictor (CPT %: Athletes: -2.95 ± 1.07 vs. -1.20 ± 0.48%) responses, compared to controls. Vascular operating range (VOR = Peak dilation+Peak Constriction) was also greater in athletes (VOR: Athletes: 0.55 ± 0.15 vs. 0.25 ± 0.18 mm, p<0.05). Athletes had superior handgrip strength (Athletes: 55.92 ± 17.06 vs. 36.77 ± 17.06 kg, p<0.05) but similar heart rate responses at peak (Athletes: 123 ± 16 vs. 130 ± 25 bpm, p>0.05) and 1 minute recovery (Athletes: 88 ± 21 vs. 98 ± 26 bpm, p>0.05) following the step test. Elite power athletes have larger brachial arteries, and greater vasoreactivity (greater vasodilatory and constrictor responses) than age-matched controls, contributing to a significantly greater VOR. These data extend the existence of an 'athlete's artery' as previously shown for elite endurance athletes to elite power athletes

Brachial-Ankle PWV: Current Status and Future Directions as a Useful Marker in the Management of Cardiovascular Disease and/or Cardiovascular Risk Factors.

PubMed

Tomiyama, Hirofumi; Matsumoto, Chisa; Shiina, Kazuki; Yamashina, Akira

2016-01-01

Since 2001, brachial-ankle pulse wave velocity (brachial-ankle PWV) measurement has been applied for risk stratification of patients with atherosclerotic cardiovascular disease and/or its risk factors in Japan. Measurement of the brachial-ankle PWV is simple and well standardized, and its reproducibility and accuracy are acceptable. Several cross-sectional studies have demonstrated a significant correlation between the brachial-ankle PWV and known risk factors for cardiovascular disease; the correlation is stronger in subjects with cardiovascular disease than in those without cardiovascular disease. We conducted a meta-analysis, which demonstrated that the brachial-ankle PWV is an independent predictor of future cardiovascular events. Furthermore, the treatment of cardiovascular risk factors and lifestyle modifications have been shown to improve the brachial-ankle PWV. Thus, at present, brachial-ankle PWV is close to being considered as a useful marker in the management of atherosclerotic cardiovascular disease and/or its risk factors.

A pilot study of the relationship between Doppler-estimated carotid and brachial artery flow and cardiac index.

PubMed

Weber, U; Glassford, N J; Eastwood, G M; Bellomo, R; Hilton, A K

2015-10-01

We measured carotid and brachial artery blood flow by Doppler ultrasound in 11 human volunteers, and related these to cardiac index and to each other. The median (IQR [range]) carotid arterial blood flow was 0.334 (0.223-0.381 [0.052-0.563]) l.min(-1) on the right and 0.315 (0.223-0.369 [0.061-0.690]) l.min(-1) on the left. The brachial arterial blood flow was 0.049 (0.033-0.062 [0.015-0.204]) l.min(-1) on the right and 0.039 (0.027-0.054 [0.011-0.116]) on the left. Cardiac index was 3.2 (2.8-3.5 [1.9-5.4]) l.min(-1) .m(-2) . There was a moderate to good correlation between right-and left-sided flows (brachial: ρ = 0.45; carotid: ρ = 0.567). Brachial and carotid flow had no or a negative correlation with cardiac index (right brachial: ρ = -0.145, left brachial: ρ = -0.349; right carotid: ρ = -0.376, left carotid: ρ = -0.285). In contrast to some previous studies, we found that Doppler-estimated peripheral arterial blood flows only show a weak correlation with cardiac index and cannot be used to provide non-invasive estimates of cardiac index in man. © 2015 The Association of Anaesthetists of Great Britain and Ireland.

Bilateral transit time assessment of upper and lower limbs as a surrogate ankle brachial index marker.

PubMed

Foo, Jong Yong Abdiel

2008-01-01

Ankle brachial index is useful in monitoring the pathogenesis of peripheral arterial occlusive diseases. Sphygmomanometer is the standard instrument widely used but frequent prolonged monitoring can be less comfortable for patients. Pulse transit time is known to be inversely correlated with blood pressure and a ratio-based pulse transit time measurement has been proposed as a surrogate ankle brachial index marker. In this study, 17 normotensive adults (9 men; aged 25.4 +/- 3.9 years) were recruited. Two postural change test activities were performed to induce changes in the stiffness of the arterial wall of the moved periphery. Results showed that only readings from the limbs that adopted a new posture registered significant blood pressure and pulse transit time changes (P < .05). Furthermore, there was significant correlation between the ankle brachial index and pulse transit time ratio measure for both test activities (R(2) > or = 0.704). The findings herein suggest that pulse transit time ratio is a surrogate and accommodating ankle brachial index marker.

Preventing brachial plexus injury during shoulder surgery: a real-time cadaveric study.

PubMed

Kam, Andrew W; Lam, Patrick H; Haen, Pieter S W A; Tan, Martin; Shamsudin, Aminudin; Murrell, George A C

2018-05-01

Brachial plexopathy is not uncommon after shoulder surgery. Although thought to be due to stretch neuropathy, its etiology is poorly understood. This study aimed to identify arm positions and maneuvers that may risk causing brachial plexopathy during shoulder arthroplasty. Tensions in the cords of the brachial plexuses of 6 human cadaveric upper limbs were measured using load cells while each limb was placed in different arm positions and while they underwent shoulder hemiarthroplasty and revision reverse arthroplasty. Arthroplasty procedures in 4 specimens were performed with standard limb positioning (unsupported), and 2 specimens were supported from under the elbow (supported). Each cord then underwent biomechanical testing to identify tension corresponding to 10% strain (the stretch neuropathy threshold in animal models). Tensions exceeding 15 N, 11 N, and 9 N in the lateral, medial, and posterior cords, respectively, produced 10% strain. Shoulder abduction >70° and combined external rotation >60° with extension >50° increased medial cord tension above the 10% strain threshold. Medial cord tensions (mean ± standard error of the mean) in unsupported specimens increased over baseline during hemiarthroplasty (sounder insertion [4.7 ± 0.6 N, P = .04], prosthesis impaction [6.1 ± 0.8 N, P = .04], and arthroplasty reduction [5.0 ± 0.7 N, P = .04]) and revision reverse arthroplasty (retractor positioning [7.2 ± 0.8 N, P = .02]). Supported specimens experienced lower tensions than unsupported specimens. Shoulder abduction >70°, combined external rotation >60° with extension >50°, and downward forces on the humeral shaft may risk causing brachial plexopathy. Retractor placement, sounder insertion, humeral prosthesis impaction, and arthroplasty reduction increase medial cord tensions during shoulder arthroplasty. Supporting the arm from under the elbow protected the brachial plexus in this cadaveric model

Patients' self-perceived burden, caregivers' burden and quality of life for amyotrophic lateral sclerosis patients: a cross-sectional study.

PubMed

Geng, Dan; Ou, RuWei; Miao, XiaoHui; Zhao, LiHong; Wei, QianQian; Chen, XuePing; Liang, Yan; Shang, HuiFang; Yang, Rong

2017-10-01

This study surveys the quality of life of amyotrophic lateral sclerosis patients and the factors associated with amyotrophic lateral sclerosis patients' self-perceived burden and their caregivers' burden. Burdens of patients with amyotrophic lateral sclerosis and their caregivers in Chinese population are largely unknown. A cross-sectional study was conducted among 81 pairs of amyotrophic lateral sclerosis patients and their caregivers. Amyotrophic lateral sclerosis patients' self-perceived burden and caregivers' burden were assessed by the Self-Perceived Burden Scale and Zarit-Burden Interview, respectively. Quality of life of amyotrophic lateral sclerosis patients was measured using the World Health Organization Quality of Life-Bref. The amyotrophic lateral sclerosis Functional Rating Scale-Revised questionnaire was used to estimate patients' physical function. Both patients and caregivers reported a mild to moderate burden. The World Health Organization quality of life-Bref scores were decreased in respondents with lower amyotrophic lateral sclerosis Functional Rating Scale-Revised, higher Self-Perceived Burden Scale and higher Zarit-Burden Interview scores. Self-Perceived Burden Scale scores were associated with patients' knowledge of amyotrophic lateral sclerosis, respiratory function and female sex. Zarit-Burden Interview scores were associated with caregivers' age, patients' motor function and out-of-pocket payment. With increase in amyotrophic lateral sclerosis patients' self-perceived burden and caregivers' burden, quality of life of amyotrophic lateral sclerosis patients decreased. Female patients, who had known more about the disease, and those with severe respiratory dysfunction were subject to higher self-perceived burden. Older caregivers and caregivers of patients with severe motor dysfunction and more out-of-pocket payment experienced more care burdens. Our study suggests that paying more attention to female amyotrophic lateral sclerosis patients

Association of left ventricular structural and functional abnormalities with aortic and brachial blood pressure variability in hypertensive patients: the SAFAR study.

PubMed

Chi, C; Yu, S-K; Auckle, R; Argyris, A A; Nasothimiou, E; Tountas, C; Aissopou, E; Blacher, J; Safar, M E; Sfikakis, P P; Zhang, Y; Protogerou, A D

2017-10-01

Both brachial blood pressure (BP) level and its variability (BPV) significantly associate with left ventricular (LV) structure and function. Recent studies indicate that aortic BP is superior to brachial BP in the association with LV abnormalities. However, it remains unknown whether aortic BPV better associate with LV structural and functional abnormalities. We therefore aimed to investigate and compare aortic versus brachial BPV, in terms of the identification of LV abnormalities. Two hundred and three participants who underwent echocardiography were included in this study. Twenty-four-hour aortic and brachial ambulatory BP was measured simultaneously by a validated BP monitor (Mobil-O-Graph, Stolberg, Germany) and BPV was calculated with validated formulae. LV mass and LV diastolic dysfunction (LVDD) were evaluated by echocardiography. The prevalence of LV hypertrophy (LVH) and LVDD increased significantly with BPV indices (P⩽0.04) in trend tests. After adjustment to potential confounders, only aortic average real variability (ARV), but not brachial ARV or weighted s.d. (wSD, neither aortic nor brachial) significantly associated with LV mass index (P=0.02). Similar results were observed in logistic regression. After adjustment, only aortic ARV significantly associated with LVH (odds ratio (OR) and 95% confidence interval (CI): 2.28 (1.08, 4.82)). As for LVDD, neither the brachial nor the aortic 24-hour wSD, but the aortic and brachial ARV, associated with LVDD significantly, with OR=2.28 (95% CI: (1.03, 5.02)) and OR=2.36 (95% CI: (1.10, 5.05)), respectively. In summary, aortic BPV, especially aortic ARV, seems to be superior to brachial BPV in the association of LV structural and functional abnormalities.

Brachial Plexus-Associated Neuropathy After High-Dose Radiation Therapy for Head-and-Neck Cancer

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

Chen, Allen M., E-mail: allen.chen@ucdmc.ucdavis.edu; Hall, William H.; Li, Judy

2012-09-01

Purpose: To identify clinical and treatment-related predictors of brachial plexus-associated neuropathies after radiation therapy for head-and-neck cancer. Methods and Materials: Three hundred thirty patients who had previously completed radiation therapy for head-and-neck cancer were prospectively screened using a standardized instrument for symptoms of neuropathy thought to be related to brachial plexus injury. All patients were disease-free at the time of screening. The median time from completion of radiation therapy was 56 months (range, 6-135 months). One-hundred fifty-five patients (47%) were treated by definitive radiation therapy, and 175 (53%) were treated postoperatively. Radiation doses ranged from 50 to 74 Gy (median,more » 66 Gy). Intensity-modulated radiation therapy was used in 62% of cases, and 133 patients (40%) received concurrent chemotherapy. Results: Forty patients (12%) reported neuropathic symptoms, with the most common being ipsilateral pain (50%), numbness/tingling (40%), motor weakness, and/or muscle atrophy (25%). When patients with <5 years of follow-up were excluded, the rate of positive symptoms increased to 22%. On univariate analysis, the following factors were significantly associated with brachial plexus symptoms: prior neck dissection (p = 0.01), concurrent chemotherapy (p = 0.01), and radiation maximum dose (p < 0.001). Cox regression analysis confirmed that both neck dissection (p < 0.001) and radiation maximum dose (p < 0.001) were independently predictive of symptoms. Conclusion: The incidence of brachial plexus-associated neuropathies after radiation therapy for head-and-neck cancer may be underreported. In view of the dose-response relationship identified, limiting radiation dose to the brachial plexus should be considered when possible.« less

Intraoperative brachial plexus injury during emergence following movement with arms restrained: a preventable complication?

PubMed Central

Chandler, Mark H; DiMatteo, Laura; Hasenboehler, Erik A; Temple, Michael

2007-01-01

Background Despite considerable analysis and preventive strategies, brachial plexus injuries remain fairly common in the perioperative setting. These injuries range from brief periods of numbness or discomfort in the immediate postoperative period to, in rare cases, profound, prolonged losses of sensation and function. We present a case of an orthopedic surgery patient who suffered a brachial plexus injury while under anesthesia after trying to sit upright with his arms restrained. Case presentation After the uneventful placement of an intramedullary tibial nail, an 18 year old patient tried to sit upright with his arms restrained while still under the influence of anesthesia. In the immediate postoperative period, the patient complained of a profound loss of sensation in his left arm and an inability to flex his left elbow, suppinate his arm, or abduct and rotate his shoulder. Neurological examination and subsequent studies revealed a C5-6 brachial plexus injury. The patient underwent range of motion physical therapy and, over the next three months, regained the full function and sensation of his left arm. Conclusion Restraining arms during general anesthesia to prevent injury remains a wise practice. However, to avoid injuring the brachial plexus while the arms are restrained, extra caution must be used to prevent unexpected patient movement and to ensure gentle emergence. PMID:18271944

c-jun-N-Terminal Kinase (JNK) for the Treatment of Amyotrophic Lateral Sclerosis

DTIC Science & Technology

2014-10-01

AWARD NUMBER: W81XWH-12-1-0431 TITLE: “c-jun-N-Terminal Kinase (JNK) for the Treatment of Amyotrophic Lateral Sclerosis ” PRINCIPAL INVESTIGATOR...TITLE AND SUBTITLE “c-jun-N-Terminal Kinase (JNK) for the Treatment of Amyotrophic Lateral Scelerosis” 5a. CONTRACT NUMBER 5b. GRANT NUMBER W81XWH

Brachial plexus injury mimicking a spinal-cord injury

PubMed Central

Macyszyn, Luke J.; Gonzalez-Giraldo, Ernesto; Aversano, Michael; Heuer, Gregory G.; Zager, Eric L.; Schuster, James M.

2010-01-01

Objective: High-energy impact to the head, neck, and shoulder can result in cervical spine as well as brachial plexus injuries. Because cervical spine injuries are more common, this tends to be the initial focus for management. We present a case in which the initial magnetic resonance imaging (MRI) was somewhat misleading and a detailed neurological exam lead to the correct diagnosis. Clinical presentation: A 19-year-old man presented to the hospital following a shoulder injury during football practice. The patient immediately complained of significant pain in his neck, shoulder, and right arm and the inability to move his right arm. He was stabilized in the field for a presumed cervical-spine injury and transported to the emergency department. Intervention: Initial radiographic assessment (C-spine CT, right shoulder x-ray) showed no bony abnormality. MRI of the cervical-spine showed T2 signal change and cord swelling thought to be consistent with a cord contusion. With adequate pain control, a detailed neurological examination was possible and was consistent with an upper brachial plexus avulsion injury that was confirmed by CT myelogram. The patient failed to make significant neurological recovery and he underwent spinal accessory nerve grafting to the suprascapular nerve to restore shoulder abduction and external rotation, while the phrenic nerve was grafted to the musculocutaneous nerve to restore elbow flexion. Conclusion: Cervical spinal-cord injuries and brachial plexus injuries can occur by the same high energy mechanisms and can occur simultaneously. As in this case, MRI findings can be misleading and a detailed physical examination is the key to diagnosis. However, this can be difficult in polytrauma patients with upper extremity injuries, head injuries or concomitant spinal-cord injury. Finally, prompt diagnosis and early surgical renerveration have been associated with better long-term recovery with certain types of injury. PMID:22956928

Types and severity of operated supraclavicular brachial plexus injuries caused by traffic accidents.

PubMed

Kaiser, Radek; Waldauf, Petr; Haninec, Pavel

2012-07-01

Brachial plexus injuries occur in up to 5% of polytrauma cases involving motorcycle accidents and in approximately 4% of severe winter sports injuries. One of the criteria for a successful operative therapy is the type of lesion. Upper plexus palsy has the best prognosis, whereas lower plexus palsy is surgically untreatable. The aim of this study was to evaluate a group of patients with brachial plexus injury caused by traffic accidents, categorize the injuries according to type of accident, and look for correlations between type of palsy (injury) and specific accidents. A total of 441 brachial plexus reconstruction patients from our department were evaluated retrospectively(1993 to 2011). Sex, age, neurological status, and the type and cause of injury were recorded for each case. Patients with BPI caused by a traffic accident were assessed in detail. Traffic accidents were the cause of brachial plexus injury in most cases (80.7%). The most common type of injury was avulsion of upper root(s) (45.7%) followed by rupture (28.2%), complete avulsion (16.9%) and avulsion of lower root(s) (9.2%). Of the patients, 73.9% had an upper,22.7% had a complete and only 3.4% had a lower brachial plexus palsy. The main cause was motorcycle accidents(63.2%) followed by car accidents (23.5%), bicycle accidents(10.7%) and pedestrian collisions (3.1%) (p<0.001).Patients involved in car accidents had a higher percentage of lower avulsion (22.7%) and a lower percentage of upper avulsion (29.3%), whereas cyclists had a higher percentage of upper avulsion (68.6%) based on the data from the entire group of patients (p<0.001). Lower plexus palsy was significantly increased in patients after car accidents (9.3%,p<0.05). In the two main groups (car and motorcycle accidents),significantly more upper and fewer lower palsies were present. In the bicycle accident group, upper palsy was the most common (89%). Study results indicate that the most common injury was an upper plexus palsy. It was

AMX0035 in Patients With Amyotrophic Lateral Sclerosis (ALS)

ClinicalTrials.gov

2018-05-21

Amyotrophic Lateral Sclerosis; Motor Neuron Disease; Neuromuscular Diseases; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Nervous System Diseases; Central Nervous System Diseases

Neuron-specific antioxidant OXR1 extends survival of a mouse model of amyotrophic lateral sclerosis.

PubMed

Liu, Kevin X; Edwards, Benjamin; Lee, Sheena; Finelli, Mattéa J; Davies, Ben; Davies, Kay E; Oliver, Peter L

2015-05-01

Amyotrophic lateral sclerosis is a devastating neurodegenerative disorder characterized by the progressive loss of spinal motor neurons. While the aetiological mechanisms underlying the disease remain poorly understood, oxidative stress is a central component of amyotrophic lateral sclerosis and contributes to motor neuron injury. Recently, oxidation resistance 1 (OXR1) has emerged as a critical regulator of neuronal survival in response to oxidative stress, and is upregulated in the spinal cord of patients with amyotrophic lateral sclerosis. Here, we tested the hypothesis that OXR1 is a key neuroprotective factor during amyotrophic lateral sclerosis pathogenesis by crossing a new transgenic mouse line that overexpresses OXR1 in neurons with the SOD1(G93A) mouse model of amyotrophic lateral sclerosis. Interestingly, we report that overexpression of OXR1 significantly extends survival, improves motor deficits, and delays pathology in the spinal cord and in muscles of SOD1(G93A) mice. Furthermore, we find that overexpression of OXR1 in neurons significantly delays non-cell-autonomous neuroinflammatory response, classic complement system activation, and STAT3 activation through transcriptomic analysis of spinal cords of SOD1(G93A) mice. Taken together, these data identify OXR1 as the first neuron-specific antioxidant modulator of pathogenesis and disease progression in SOD1-mediated amyotrophic lateral sclerosis, and suggest that OXR1 may serve as a novel target for future therapeutic strategies. © The Author (2015). Published by Oxford University Press on behalf of the Guarantors of Brain.

High-Resolution 7T MR Imaging of the Motor Cortex in Amyotrophic Lateral Sclerosis.

PubMed

Cosottini, M; Donatelli, G; Costagli, M; Caldarazzo Ienco, E; Frosini, D; Pesaresi, I; Biagi, L; Siciliano, G; Tosetti, M

2016-03-01

Amyotrophic lateral sclerosis is a progressive motor neuron disorder that involves degeneration of both upper and lower motor neurons. In patients with amyotrophic lateral sclerosis, pathologic studies and ex vivo high-resolution MR imaging at ultra-high field strength revealed the co-localization of iron and activated microglia distributed in the deep layers of the primary motor cortex. The aims of the study were to measure the cortical thickness and evaluate the distribution of iron-related signal changes in the primary motor cortex of patients with amyotrophic lateral sclerosis as possible in vivo biomarkers of upper motor neuron impairment. Twenty-two patients with definite amyotrophic lateral sclerosis and 14 healthy subjects underwent a high-resolution 2D multiecho gradient-recalled sequence targeted on the primary motor cortex by using a 7T scanner. Image analysis consisted of the visual evaluation and quantitative measurement of signal intensity and cortical thickness of the primary motor cortex in patients and controls. Qualitative and quantitative MR imaging parameters were correlated with electrophysiologic and laboratory data and with clinical scores. Ultra-high field MR imaging revealed atrophy and signal hypointensity in the deep layers of the primary motor cortex of patients with amyotrophic lateral sclerosis with a diagnostic accuracy of 71%. Signal hypointensity of the deep layers of the primary motor cortex correlated with upper motor neuron impairment (r = -0.47; P < .001) and with disease progression rate (r = -0.60; P = .009). The combined high spatial resolution and sensitivity to paramagnetic substances of 7T MR imaging demonstrate in vivo signal changes of the cerebral motor cortex that resemble the distribution of activated microglia within the cortex of patients with amyotrophic lateral sclerosis. Cortical thinning and signal hypointensity of the deep layers of the primary motor cortex could constitute a marker of upper motor neuron

Application of botulinum toxin to treat sialorrhea in amyotrophic lateral sclerosis patients: a literature review

PubMed Central

de Oliveira, Ademar Francisco; Silva, Gêssyca Adryene de Menezes; Almeida, Débora Milenna Xavier

2016-01-01

ABSTRACT Amyotrophic lateral sclerosis is a progressive and fatal neurodegenerative disease characterized by the degeneration of motor neurons, which are the central nervous system cells that control voluntary muscle movements. The excessive salivation (sialorrhea) is present in approximately 50% of amyotrophic lateral sclerosis cases. Thus, some alternative therapeutic methods are sought, such as anticholinergic drugs and surgery. Recently the use of botulinum toxin applied at a midpoint of the salivary glands, often guided by ultrasound, have demonstrated positive results. The objective was to review the literature to demonstrate an alternative method to treatments of sialorrhea in patients with amyotrophic lateral sclerosis. In recent studies, the efficacy of botulinum toxin is confirmed, although new applications are required. Since the side effects are negligible, this is an alternative to treat amyotrophic lateral sclerosis, and other patients with diseases that present sialorrhea. PMID:27759834

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.

Brachial artery endothelial function is unchanged after acute sprint interval exercise in sedentary men and women.

PubMed

Shenouda, Ninette; Skelly, Lauren E; Gibala, Martin J; MacDonald, Maureen J

2018-05-03

What is the central question of this study? What is the acute brachial artery endothelial function response to sprint interval exercise and are there sex-based differences? What is the main finding and its importance? Brachial artery endothelial function did not change in either men or women following an acute session of SIT consisting of 3 × 20 s 'all-out' cycling sprints. Our findings suggest this low-volume protocol may not be sufficient to induce functional changes in the brachial artery of sedentary, but otherwise healthy adults. Sprint interval training (SIT) is a potent metabolic stimulus, but studies examining its acute effects on brachial artery endothelial function are limited. The influence of oestradiol on the acute arterial response to this type of exercise is also unknown. We investigated the brachial artery endothelial function response to a single session of SIT in sedentary healthy men (n = 8; 22 ± 4 years) and premenopausal women tested in the mid-follicular phase of the menstrual cycle (n = 8; 21 ± 3 years). Participants performed 3 × 20 s 'all-out' cycling sprints interspersed with 2 min of active recovery. Brachial artery flow-mediated dilatation (FMD) and haemodynamic parameters were measured before and 1 and 24 h post-exercise. Despite attenuations in some haemodynamic parameters at 1 h post-exercise, there were no changes in absolute (P = 0.23), relative (P = 0.23) or allometrically scaled FMD (P = 0.38) following a single session of SIT. Resting and peak dilatory diameters did not change in men or women (P > 0.05 for all) and there were no interactions between time and sex for any measure (P > 0.05). Oestradiol was not correlated with relative FMD at baseline (r = -0.22, P = 0.42) or with the change in relative FMD from baseline to 1 h post-exercise (r = 0.24, P = 0.40). Overall, brachial artery FMD appears to be unchanged in men and women following an acute session of SIT, and the higher oestradiol

Non-invasive assessment of peripheral arterial disease: Automated ankle brachial index measurement and pulse volume analysis compared to duplex scan.

PubMed

Lewis, Jane Ea; Williams, Paul; Davies, Jane H

2016-01-01

This cross-sectional study aimed to individually and cumulatively compare sensitivity and specificity of the (1) ankle brachial index and (2) pulse volume waveform analysis recorded by the same automated device, with the presence or absence of peripheral arterial disease being verified by ultrasound duplex scan. Patients (n=205) referred for lower limb arterial assessment underwent ankle brachial index measurement and pulse volume waveform recording using volume plethysmography, followed by ultrasound duplex scan. The presence of peripheral arterial disease was recorded if ankle brachial index <0.9; pulse volume waveform was graded as 2, 3 or 4; or if haemodynamically significant stenosis >50% was evident with ultrasound duplex scan. Outcome measure was agreement between the measured ankle brachial index and interpretation of pulse volume waveform for peripheral arterial disease diagnosis, using ultrasound duplex scan as the reference standard. Sensitivity of ankle brachial index was 79%, specificity 91% and overall accuracy 88%. Pulse volume waveform sensitivity was 97%, specificity 81% and overall accuracy 85%. The combined sensitivity of ankle brachial index and pulse volume waveform was 100%, specificity 76% and overall accuracy 85%. Combining these two diagnostic modalities within one device provided a highly accurate method of ruling out peripheral arterial disease, which could be utilised in primary care to safely reduce unnecessary secondary care referrals.

The ratio of N-acetyl aspartate to glutamate correlates with disease duration of amyotrophic lateral sclerosis.

PubMed

Sako, Wataru; Abe, Takashi; Izumi, Yuishin; Harada, Masafumi; Kaji, Ryuji

2016-05-01

Glutamate (Glu)-induced excitotoxicity has been implicated in the neuronal loss of amyotrophic lateral sclerosis. To test the hypothesis that Glu in the primary motor cortex contributes to disease severity and/or duration, the Glu level was investigated using MR spectroscopy. Seventeen patients with amyotrophic lateral sclerosis were diagnosed according to the El Escorial criteria for suspected, possible, probable or definite amyotrophic lateral sclerosis, and enrolled in this cross-sectional study. We measured metabolite concentrations, including N-acetyl aspartate (NAA), creatine, choline, inositol, Glu and glutamine, and performed partial correlation between each metabolite concentration or NAA/Glu ratio and disease severity or duration using age as a covariate. Considering our hypothesis that Glu is associated with neuronal cell death in amyotrophic lateral sclerosis, we investigated the ratio of NAA to Glu, and found a significant correlation between NAA/Glu and disease duration (r=-0.574, p=0.02). The "suspected" amyotrophic lateral sclerosis patients showed the same tendency as possible, probable and definite amyotrophic lateral sclerosis patients in regard to correlation of NAA/Glu ratio with disease duration. The other metabolites showed no significant correlation. Our findings suggested that glutamatergic neurons are less vulnerable compared to other neurons and this may be because inhibitory receptors are mainly located presynaptically, which supports the notion of Glu-induced excitotoxicity. Copyright © 2015 Elsevier Ltd. All rights reserved.

Relation of Long-term Exposure to Air Pollution to Brachial Artery Flow-Mediated Dilation and Reactive Hyperemia

PubMed Central

Wilker, Elissa H.; Ljungman, Petter L.; Rice, Mary B.; Kloog, Itai; Schwartz, Joel; Gold, Diane R.; Koutrakis, Petros; Vita, Joseph A.; Mitchell, Gary F.; Vasan, Ramachandran S.; Benjamin, Emelia J.; Hamburg, Naomi M.; Mittleman, Murray A.

2014-01-01

Long-term exposure to ambient air pollution has been associated with cardiovascular morbidity and mortality. Impaired vascular responses may in part explain these findings, but the association of such long-term exposure with measures of both conduit artery and microvascular function have not been widely reported. We evaluated the association between residential proximity to a major roadway (primary or secondary highway) and spatially resolved average fine particulate matter (PM2.5) and baseline brachial artery diameter and mean flow velocity, flow mediated dilation % and hyperemic flow velocity, in the Framingham Offspring and Third Generation Cohorts. We examined 5,112 participants (2,731 (53%) women, mean age 49±14 years). Spatially resolved average PM2.5 was associated with lower flow mediated dilation% and hyperemic flow velocity. An interquartile range difference in PM2.5 (1.99 μg/m3) was associated with −0.16% (95%CI: −0.27%, −0.05%) lower FMD% and −0.72 (95%CI: −1.38, −0.06) cm/s lower hyperemic flow velocity %. Residential proximity to a major roadway was negatively associated with flow mediated dilation %. Compared to living ≥400 m away, living <50 m from a major roadway was associated with 0.32% lower flow mediated dilation (95% confidence interval (CI): −0.58%, −0.06%), but results for hyperemic flow velocity had wide confidence intervals −0.68 cm/s (95%CI: −2.29, 0.93). In conclusion, residential proximity to a major roadway and higher levels of spatially resolved estimates of PM2.5 at participant residences are associated with impaired conduit artery and microvascular function in this large community-based cohort of middle-aged and elderly adults. PMID:24793676

Brachial artery protected by wrapped latissimus dorsi muscle flap in high voltage electrical injury

PubMed Central

Gencel, E.; Eser, C.; Kokacya, O.; Kesiktas, E.; Yavuz, M.

2016-01-01

Summary High voltage electrical injury can disrupt the vascular system and lead to extremity amputations. It is important to protect main vessels from progressive burn necrosis in order to salvage a limb. The brachial artery should be totally isolated from the burned area by a muscle flap to prevent vessel disruption. In this study, we report the use of a wrap-around latissimus dorsi muscle flap to protect a skeletonized brachial artery in a high voltage electrical injury in order to salvage the upper extremity and restore function. The flap wrapped around the exposed brachial artery segment and luminal status of the artery was assessed using magnetic resonance angiography. No vascular intervention was required. The flap survived completely with good elbow function. Extremity amputation was not encountered. This method using a latissimus dorsi flap allows the surgeon to protect the main upper extremity artery and reconstruct arm defects, which contributes to restoring arm function in high voltage electrical injury. PMID:28149236

Brachial artery protected by wrapped latissimus dorsi muscle flap in high voltage electrical injury.

PubMed

Gencel, E; Eser, C; Kokacya, O; Kesiktas, E; Yavuz, M

2016-06-30

High voltage electrical injury can disrupt the vascular system and lead to extremity amputations. It is important to protect main vessels from progressive burn necrosis in order to salvage a limb. The brachial artery should be totally isolated from the burned area by a muscle flap to prevent vessel disruption. In this study, we report the use of a wrap-around latissimus dorsi muscle flap to protect a skeletonized brachial artery in a high voltage electrical injury in order to salvage the upper extremity and restore function. The flap wrapped around the exposed brachial artery segment and luminal status of the artery was assessed using magnetic resonance angiography. No vascular intervention was required. The flap survived completely with good elbow function. Extremity amputation was not encountered. This method using a latissimus dorsi flap allows the surgeon to protect the main upper extremity artery and reconstruct arm defects, which contributes to restoring arm function in high voltage electrical injury.

[Pattern of paralysis and reconstructive operations after traumatic brachial plexus lesions].

PubMed

Rühmann, O; Schmolke, S; Carls, J; Wirth, C J

2002-12-01

The aim of this study was to evaluate persistent patterns of paralysis after traumatic brachial plexus lesions. As a result, consecutive reconstructive operations according to our differential therapy concept are presented. Between 04/1994 and 12/2000 in 104 patients with brachial plexus palsy, the grade of muscle power of the affected upper extremities was evaluated prospectively. The neuromuscular patterns of defect showed, in most cases, insufficient muscle power grades of 0-2 for the deltoid muscle (90%), supraspinatus muscle (82%), infraspinatus muscle (93%), elbow flexors (67% to 77%), hand and finger extensors (69% to 71%), and the abductor and extensors of the thumb (67% to 70%). In corresponding frequency, the following operations were performed between 04/1994 and 06/2002: shoulder arthrodesis (n 26), trapezius transfer (n 80), rotation osteotomy of humerus (n 10), triceps to biceps transposition (n 11), transposition of forearm flexors or extensors/Steindler operation (n 12), latissimus transfer (n 7), pectoralis transfer (n 1), teres major transfer (n 1), transposition of forearm flexors to the tendons of extensor digitorum (n 19) and of the extensor pollicis longus (n 9), and wrist arthrodesis (n 5). On malfunction of muscles following brachial plexus lesions, taking into account the individual neuromuscular defect, passive joint function, and bony deformities, different procedures such as muscle transposition, arthrodesis, and corrective osteotomy can be performed to improve function of the upper extremity.

A comprehensive analysis of rare genetic variation in amyotrophic lateral sclerosis in the UK.

PubMed

Morgan, Sarah; Shatunov, Aleksey; Sproviero, William; Jones, Ashley R; Shoai, Maryam; Hughes, Deborah; Al Khleifat, Ahmad; Malaspina, Andrea; Morrison, Karen E; Shaw, Pamela J; Shaw, Christopher E; Sidle, Katie; Orrell, Richard W; Fratta, Pietro; Hardy, John; Pittman, Alan; Al-Chalabi, Ammar

2017-06-01

Amyotrophic lateral sclerosis is a progressive neurodegenerative disease of motor neurons. About 25 genes have been verified as relevant to the disease process, with rare and common variation implicated. We used next generation sequencing and repeat sizing to comprehensively assay genetic variation in a panel of known amyotrophic lateral sclerosis genes in 1126 patient samples and 613 controls. About 10% of patients were predicted to carry a pathological expansion of the C9orf72 gene. We found an increased burden of rare variants in patients within the untranslated regions of known disease-causing genes, driven by SOD1, TARDBP, FUS, VCP, OPTN and UBQLN2. We found 11 patients (1%) carried more than one pathogenic variant (P = 0.001) consistent with an oligogenic basis of amyotrophic lateral sclerosis. These findings show that the genetic architecture of amyotrophic lateral sclerosis is complex and that variation in the regulatory regions of associated genes may be important in disease pathogenesis. © The Author (2017). Published by Oxford University Press on behalf of the Guarantors of Brain.

Adding a PECS II block for proximal arm arteriovenous access - a randomised study.

PubMed

Quek, K H; Low, E Y; Tan, Y R; Ong, A S C; Tang, T Y; Kam, J W; Kiew, A S C

2018-05-01

Brachial plexus block is often utilised for proximal arm arteriovenous access creation. However, the medial upper arm and axilla are often inadequately anaesthetised, requiring repeated, intraoperative local anaesthetic supplementation, or conversion into general anaesthesia. We hypothesised that the addition of a PECS II block would improve anaesthesia and analgesia for proximal arm arteriovenous access surgery. In this prospective, double-blinded, randomised proof-of-concept study, 36 consenting adults with end-stage renal disease aged between 21 and 90 years received either a combined supraclavicular and PECS II block (Group PECS, n = 18), or combined supraclavicular and sham block (Group SCB, n = 18) for proximal arm arteriovenous access surgery. Primary outcome was whether patients required intraoperative local anaesthetic supplementation by the surgeon. In Group PECS, 33.3% (6/18) needed local anaesthetic supplementation vs. 100% (18/18) in Group SCB. Group SCB had three times (RR 3.0, 95% CI 1.6-5.8; P < 0.001) the risk of requiring intraoperative local anaesthetic supplementation. Group PECS required lower volume of supplemental local anaesthetic compared to Group SCB (0.0 ml, IQR 0.0-6.3 ml vs. 15.0 ml, IQR 7.4-17.8 ml; P < 0.001). Group SCB had twice [RR 2.2, 95% CI 1.1-4.4; (P = 0.019)] the risk of needing additional sedation or analgesia. There were no significant differences between the groups with respect to postoperative visual analogue scale pain scores, time to first rescue analgesia or patient satisfaction. The results suggest that adding a PECS II block to a supraclavicular block improves regional anaesthesia for patients with end-stage renal disease undergoing proximal arm arteriovenous access surgery. © 2018 The Acta Anaesthesiologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

Addition of dexmedetomidine to bupivacaine in supraclavicular brachial plexus block.

PubMed

Aksu, Recep; Bicer, Cihangir

2017-06-26

Research is ongoing to determine the lowest dose of local anesthetics in brachial plexus block that provides adequate anesthesia and postoperative analgesia and reduces complications related to local anesthetics. Patients 18-65 years of age who underwent upper limb surgery and who received ultrasound-guided supraclavicular brachial plexus block at the Erciyes University Faculty of Medicine Hospital between February 2014 and January 2015 were included in the study (n=50). Supraclavicular brachial plexus blocks were performed on Group B cases by adding 30 ml 0.33% bupivacaine and on Group BD cases by adding 15 ml 0.33% bupivacaine and 1 µg / kg dexmedetomidine. Block success was evaluated by the onset and block duration of motor and sensory block and the duration of analgesia. The block success of Group B and Group BD was 92.6% and 89.3%, respectively (P = 1.000). Onset time of sensory block, degree of sensory block, duration of sensory block, onset time of motor block, degree of motor block and duration of motor block were similar in both groups in the intergroup comparison (P > 0.05). Duration of analgesia and the operative conditions of groups were similar (P > 0.05). In the implementation of ultrasound-guided supraclavicular brachial plexus block, block success, sensory and motor block and analgesia duration were similar for patients anaesthetized with 30 ml of bupivacaine in comparison with dexmedetomidine+bupivacaine (when the bupivacaine dose was reduced by 50% by the addition of the adjuvant).

Frontal slab composite magnetic resonance neurography of the brachial plexus: implications for infraclavicular block approaches.

PubMed

Raphael, David T; McIntee, Diane; Tsuruda, Jay S; Colletti, Patrick; Tatevossian, Ray

2005-12-01

Magnetic resonance neurography (MRN) is an imaging method by which nerves can be selectively highlighted. Using commercial software, the authors explored a variety of approaches to develop a three-dimensional volume-rendered MRN image of the entire brachial plexus and used it to evaluate the accuracy of infraclavicular block approaches. With institutional review board approval, MRN of the brachial plexus was performed in 10 volunteer subjects. MRN imaging was performed on a GE 1.5-tesla magnetic resonance scanner (General Electric Healthcare Technologies, Waukesha, WI) using a phased array torso coil. Coronal STIR and T1 oblique sagittal sequences of the brachial plexus were obtained. Multiple software programs were explored for enhanced display and manipulation of the composite magnetic resonance images. The authors developed a frontal slab composite approach that allows single-frame reconstruction of a three-dimensional volume-rendered image of the entire brachial plexus. Automatic segmentation was supplemented by manual segmentation in nearly all cases. For each of three infraclavicular approaches (posteriorly directed needle below midclavicle, infracoracoid, or caudomedial to coracoid), the targeting error was measured as the distance from the MRN plexus midpoint to the approach-targeted site. Composite frontal slabs (coronal views), which are single-frame three-dimensional volume renderings from image-enhanced two-dimensional frontal view projections of the underlying coronal slices, were created. The targeting errors (mean +/- SD) for the approaches-midclavicle, infracoracoid, caudomedial to coracoid-were 0.43 +/- 0.67, 0.99 +/- 1.22, and 0.65 +/- 1.14 cm, respectively. Image-processed three-dimensional volume-rendered MNR scans, which allow visualization of the entire brachial plexus within a single composite image, have educational value in illustrating the complexity and individual variation of the plexus. Suggestions for improved guidance during

The Prevalence, Rate of Progression, and Treatment of Elbow Flexion Contracture in Children with Brachial Plexus Birth Palsy

PubMed Central

Sheffler, Lindsey C.; Lattanza, Lisa; Hagar, Yolanda; Bagley, Anita; James, Michelle A.

2012-01-01

Background: Elbow flexion contracture is a well-known complication of brachial plexus birth palsy that adversely affects upper-extremity function. The prevalence, risk factors, and rate of progression of elbow flexion contracture associated with brachial plexus birth palsy have not been established, and the effectiveness of nonoperative treatment involving nighttime splinting or serial casting has not been well studied. Methods: The medical records of 319 patients with brachial plexus birth palsy who had been seen at our institution between 1992 and 2009 were retrospectively reviewed to identify patients with an elbow flexion contracture (≥10°). The chi-square test for trend and the Kaplan-Meier estimator were used to evaluate risk factors for contracture, including age, sex, and the extent of brachial plexus involvement. Longitudinal models were used to estimate the rate of contracture progression and the effectiveness of nonoperative treatment. Results: An elbow flexion contracture was present in 48% (152) of the patients with brachial plexus birth palsy. The median age of onset was 5.1 years (range, 0.25 to 14.8 years). The contracture was ≥30° in 36% (fifty-four) of these 152 patients and was accompanied by a documented radial head dislocation in 6% (nine). The prevalence of contracture increased with increasing age (p < 0.001) but was not significantly associated with sex or with the extent of brachial plexus involvement. The magnitude of the contracture increased by 4.4% per year before treatment (p < 0.01). The magnitude of the contracture decreased by 31% when casting was performed (p < 0.01) but thereafter increased again at the same rate of 4.4% per year. The magnitude of the contracture did not improve when splinting was performed but the rate of increase thereafter decreased to <0.1% per year (p = 0.04). Conclusions: The prevalence of elbow flexion contracture in children with brachial plexus birth palsy may be greater than clinicians perceive

Sup-ER orthosis: an innovative treatment for infants with birth related brachial plexus injury.

PubMed

Durlacher, Kim M; Bellows, Doria; Verchere, Cynthia

2014-01-01

Impairments in active and passive range of upper extremity supination and shoulder external rotation are common sequelae for children with delayed recovery from birth related brachial plexus injury. Orthotic intervention may complement traditional treatment strategies commonly employed in the newborn period. These authors describe their custom fabricated orthosis designed to balance shoulder growth and muscular function, and improve prognosis of long term functional outcomes for children with birth related brachial plexus injury. - Victoria Priganc, PhD, OTR, CHT, CLT, Practice Forum Editor. Copyright © 2014 Hanley & Belfus. Published by Elsevier Inc. All rights reserved.

Occupational therapy intervention with radiation-induced brachial plexopathy.

PubMed

Cooper, J

1998-06-01

Occupational therapy intervention minimizes disability and facilitates optimum functional independence. The range of dysfunction experienced by patients with radiation-induced brachial plexopathy includes physical, psychological, emotional and social difficulties. The occupational therapist works as part of the multiprofessional team to use a client-centred, problem-solving approach to address the problems and enable the patient to adapt to the altered body image and disabilities.

Information-seeking Behavior and Information Needs in Patients With Amyotrophic Lateral Sclerosis: Analyzing an Online Patient Community.

PubMed

Oh, Juyeon; Kim, Jung A

2017-07-01

A few studies have examined the specific informational needs of the population with amyotrophic lateral sclerosis. The aims of this study were to describe the information-seeking behavior and information needs of patients with amyotrophic lateral sclerosis and their families in Korea by analyzing messages from an online patient community. A total of 1047 messages from the question and answer forum of the "Lou Gehrig's Disease Network" (http://cafe.daum.net/alsfree) from January 2010 to September 2015 were collected. The word frequency, main questions, and asker of the messages were analyzed and coded. Terms such as "hospital," "mother," "father," "gastrostomy," and "ALS" were most frequently identified. The most commonly mentioned main topic was about disease-specific information, while the most frequent subcategory was symptoms or management of symptoms. Other prominent categories concerned information about treatment, rehabilitation, and the medical system. The people who wrote the questions were mostly the son/daughter of patients with amyotrophic lateral sclerosis. Patients with amyotrophic lateral sclerosis and their family members commonly obtained information by posting their inquiries online and have a variety of questions regarding amyotrophic lateral sclerosis in this study. The findings of this study can be used as a base of information for developing educational programs and resources for patients with amyotrophic lateral sclerosis and their families.

Noninvasive Evaluation of Varying Pulse Pressures in vivo Using Brachial Sphymomanometry, Applanation Tonometry, and Pulse Wave Ultrasound Manometry.

PubMed

Li, Ronny X; Ip, Ada; Sanz-Miralles, Elena; Konofagou, Elisa E

2017-06-01

The routine assessment and monitoring of hypertension may benefit from the evaluation of arterial pulse pressure (PP) at more central locations (e.g. the aorta) rather solely at the brachial artery. Pulse Wave Ultrasound Manometry (PWUM) was previously developed by our group to provide direct, noninvasive aortic PP measurements using ultrasound elasticity imaging. Using PWUM, radial applanation tonometry, and brachial sphygmomanometry, this study investigated the feasibility of noninvasively obtaining direct PP measurements at multiple arterial locations in normotensive, pre-hypertensive, and hypertensive human subjects. Two-way ANOVA indicated a significantly higher aortic PP in the hypertensive subjects, while radial and brachial PP were not significantly different among the subject groups. No strong correlation (r 2 < 0.45) was observed between aortic and radial/brachial PP in normal and pre-hypertensive subjects, suggesting that increases in PP throughout the arterial tree may not be uniform in relatively compliant arteries. However, there was a relatively strong positive correlation between aortic PP and both radial and brachial PP in hypertensive subjects (r 2 = 0.68 and 0.87, respectively). PWUM provides a low-cost, non-invasive, and direct means of measuring the pulse pressure in large central arteries such as the aorta. When used in conjunction with peripheral measurement devices, PWUM allows for the routine screening of hypertension and monitoring of BP-lowering drugs based on the PP from multiple arterial sites.

Finger movement at birth in brachial plexus birth palsy

PubMed Central

Nath, Rahul K; Benyahia, Mohamed; Somasundaram, Chandra

2013-01-01

AIM: To investigate whether the finger movement at birth is a better predictor of the brachial plexus birth injury. METHODS: We conducted a retrospective study reviewing pre-surgical records of 87 patients with residual obstetric brachial plexus palsy in study 1. Posterior subluxation of the humeral head (PHHA), and glenoid retroversion were measured from computed tomography or Magnetic resonance imaging, and correlated with the finger movement at birth. The study 2 consisted of 141 obstetric brachial plexus injury patients, who underwent primary surgeries and/or secondary surgery at the Texas Nerve and Paralysis Institute. Information regarding finger movement was obtained from the patient’s parent or guardian during the initial evaluation. RESULTS: Among 87 patients, 9 (10.3%) patients who lacked finger movement at birth had a PHHA > 40%, and glenoid retroversion < -12°, whereas only 1 patient (1.1%) with finger movement had a PHHA > 40%, and retroversion < -8° in study 1. The improvement in glenohumeral deformity (PHHA, 31.8% ± 14.3%; and glenoid retroversion 22.0° ± 15.0°) was significantly higher in patients, who have not had any primary surgeries and had finger movement at birth (group 1), when compared to those patients, who had primary surgeries (nerve and muscle surgeries), and lacked finger movement at birth (group 2), (PHHA 10.7% ± 15.8%; Version -8.0° ± 8.4°, P = 0.005 and P = 0.030, respectively) in study 2. No finger movement at birth was observed in 55% of the patients in this study group. CONCLUSION: Posterior subluxation and glenoid retroversion measurements indicated significantly severe shoulder deformities in children with finger movement at birth, in comparison with those lacked finger movement. However, the improvement after triangle tilt surgery was higher in patients who had finger movement at birth. PMID:23362472

Cost analysis of brachial plexus injuries: variability of compensation by insurance companies before and after surgery.

PubMed

Felici, N; Zaami, S; Ciancolini, G; Marinelli, E; Tagliente, D; Cannatà, C

2014-04-01

Traumatic paralysis of the brachial plexus is an extremely disabling pathology. The type of trauma most frequently suffered by this group of patients is due to motorcycle injuries. It therefore affects a population of young patients. In the majority of cases, these patients receive compensation for permanent damage from insurance companies. Surgery of the brachial plexus enables various forms of functional recovery, depending on the number of roots of the brachial plexus involved in the injury. The aim of this study is to compare the functional deficit and the extent of the related compensation before and after surgical intervention, and to evaluate the saving in economic terms (understood as the cost of compensation paid by insurance companies) obtainable through surgical intervention. The authors analysed the functional recovery obtained through surgery in 134 patients divided into 4 groups on the basis of the number of injured roots. The levels of compensation payable to the patient before surgical intervention, and 3 years after, were then compared. The results showed that the saving obtainable through surgical treatment of brachial plexus injuries may exceed 65% of the economic value of the compensation that would have been attributable to the same patients if they had not undergone surgical treatment. © Georg Thieme Verlag KG Stuttgart · New York.

24-hour aortic blood pressure variability showed a stronger association with carotid damage than 24-hour brachial blood pressure variability: The SAFAR study.

PubMed

Yu, Shikai; Chi, Chen; Protogerou, Athanase D; Safar, Michel E; Blacher, Jacques; Argyris, Antonis A; Nasothimiou, Efthimia G; Sfikakis, Petros P; Papaioannou, Theodore G; Xu, Henry; Zhang, Yi; Xu, Yawei

2018-03-01

We aim to compare 24-hour aortic blood pressure variability (BPV) with brachial BPV in relation to carotid damage as estimated by carotid intima-media thickness (CIMT) and cross-sectional area (CCSA). Four hundred and forty five individuals received brachial and aortic 24-hour ambulatory BP monitoring with a validated device (Mobil-O-Graph). Systolic BPV was estimated by average real variability (ARV) and time-weighted standard deviation (wSD). In multiple logistic regression analysis, CIMT > 900 μm was significantly and independently associated with aortic ARV (OR = 1.38; 95% CI: 1.04-1.84), aortic wSD (OR = 1.65; 95% CI: 1.19-2.29) and brachial ARV (OR = 1.53; 95% CI: 1.07-2.18), but not with brachial wSD. CCSA > 90th percentile was significantly and independently associated with aortic ARV (OR = 1.50; 95% CI: 1.07-2.10) and wSD (OR = 1.70; 95% CI: 1.12-2.56), but not with brachial BPVs. In receiver operator characteristics curve analysis, aortic wSD identified CCSA > 90th percentile better than brachial wSD (AUC: 0.73 vs 0.68, P < .01). In conclusion, aortic 24-hour systolic BPV showed a slightly stronger association with carotid damage than brachial BPV. ©2018 Wiley Periodicals, Inc.

Dose–Volume Modeling of Brachial Plexus-Associated Neuropathy After Radiation Therapy for Head-and-Neck Cancer: Findings From a Prospective Screening Protocol

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

Chen, Allen M., E-mail: amchen@mednet.ucla.edu; Wang, Pin-Chieh; Daly, Megan E.

2014-03-15

Purpose: Data from a prospective screening protocol administered for patients previously irradiated for head-and-neck cancer was analyzed to identify dosimetric predictors of brachial plexus-associated neuropathy. Methods and Materials: Three hundred fifty-two patients who had previously completed radiation therapy for squamous cell carcinoma of the head and neck were prospectively screened from August 2007 to April 2013 using a standardized self-administered instrument for symptoms of neuropathy thought to be related to brachial plexus injury. All patients were disease-free at the time of screening. The median time from radiation therapy was 40 months (range, 6-111 months). A total of 177 patients (50%)more » underwent neck dissection. Two hundred twenty-one patients (63%) received concurrent chemotherapy. Results: Fifty-one patients (14%) reported brachial plexus-related neuropathic symptoms with the most common being ipsilateral pain (50%), numbness/tingling (40%), and motor weakness and/or muscle atrophy (25%). The 3- and 5-year estimates of freedom from brachial plexus-associated neuropathy were 86% and 81%, respectively. Clinical/pathological N3 disease (P<.001) and maximum radiation dose to the ipsilateral brachial plexus (P=.01) were significantly associated with neuropathic symptoms. Cox regression analysis revealed significant dose–volume effects for brachial plexus-associated neuropathy. The volume of the ipsilateral brachial plexus receiving >70 Gy (V70) predicted for symptoms, with the incidence increasing with V70 >10% (P<.001). A correlation was also observed for the volume receiving >74 Gy (V74) among patients treated without neck dissection, with a cutoff of 4% predictive of symptoms (P=.038). Conclusions: Dose–volume guidelines were developed for radiation planning that may limit brachial plexus-related neuropathies.« less

The First Experience of Triple Nerve Transfer in Proximal Radial Nerve Palsy.

PubMed

Emamhadi, Mohammadreza; Andalib, Sasan

2018-01-01

Injury to distal portion of posterior cord of brachial plexus leads to palsy of radial and axillary nerves. Symptoms are usually motor deficits of the deltoid muscle; triceps brachii muscle; and extensor muscles of the wrist, thumb, and fingers. Tendon transfers, nerve grafts, and nerve transfers are options for surgical treatment of proximal radial nerve palsy to restore some motor functions. Tendon transfer is painful, requires a long immobilization, and decreases donor muscle strength; nevertheless, nerve transfer produces promising outcomes. We present a patient with proximal radial nerve palsy following a blunt injury undergoing triple nerve transfer. The patient was involved in a motorcycle accident with complete palsy of the radial and axillary nerves. After 6 months, on admission, he showed spontaneous recovery of axillary nerve palsy, but radial nerve palsy remained. We performed triple nerve transfer, fascicle of ulnar nerve to long head of the triceps branch of radial nerve, flexor digitorum superficialis branch of median nerve to extensor carpi radialis brevis branch of radial nerve, and flexor carpi radialis branch of median nerve to posterior interosseous nerve, for restoration of elbow, wrist, and finger extensions, respectively. Our experience confirmed functional elbow, wrist, and finger extensions in the patient. Triple nerve transfer restores functions of the upper limb in patients with debilitating radial nerve palsy after blunt injuries. Copyright © 2017 Elsevier Inc. All rights reserved.

Relation of blood pressure and organ damage: comparison between feasible, noninvasive central hemodynamic measures and conventional brachial measures.

PubMed

Lindroos, Annika S; Langén, Ville L; Kantola, Ilkka; Salomaa, Veikko; Juhanoja, Eeva P; Sivén, Sam S; Jousilahti, Pekka; Jula, Antti M; Niiranen, Teemu J

2018-06-01

The present cross-sectional study investigated whether central SBP and pulse pressure (PP) measured noninvasively with a novel cuff-based stand-alone monitor are more strongly associated with hypertensive end-organ damage than corresponding brachial measures. We investigated the cross-sectional association of central versus brachial SBP and PP with echocardiographic left ventricular mass index (LVMI), LV hypertrophy (LVH), carotid intima-media thickness (IMT), and increased IMT (IMT ≥ 75th percentile) among 246 participants drawn from the general population (mean age 57.2 years, 55.3% women). All blood pressure (BP) measures were positively correlated with LVMI and IMT (P < 0.001 for all). Brachial and central SBP correlated equally strongly with LVMI (r = 0.42 versus 0.40, P for difference 0.19) and IMT (r = 0.32 versus 0.33, P = 0.60). However, brachial PP correlated more strongly than central PP with LVMI (r = 0.34 versus 0.27, P = 0.03) and IMT (r = 0.40 versus 0.35, P = 0.04). In multivariable-adjusted logistic models, all four BP measures were significantly associated with LVH and increased IMT (P ≤ 0.03 for all). However, the diagnostic accuracy of logistic regression models that included brachial or central hemodynamic parameters was similar for LVH [area under curve (AUC) for SBP: 0.74 versus 0.76, P = 0.16; AUC for PP: 0.75 versus 0.73, P = 0.35] and IMT (AUC for SBP: 0.61 versus 0.61, P = 0.67; AUC for PP: 0.63 versus 0.61, P = 0.29). Our findings suggest that central SBP and PP measured with a stand-alone noninvasive BP monitor do not improve diagnostic accuracy for end-organ damage over corresponding brachial measures.

Brachial Plexopathy in Apical Non-Small Cell Lung Cancer Treated With Definitive Radiation: Dosimetric Analysis and Clinical Implications

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

Eblan, Michael J.; Corradetti, Michael N.; Lukens, J. Nicholas

2013-01-01

Purpose: Data are limited on the clinical significance of brachial plexopathy in patients with apical non-small cell lung cancers (NSCLC) treated with definitive radiation therapy. We report the rates of radiation-induced brachial plexopathy (RIBP) and tumor-related brachial plexopathy (TRBP) and associated dosimetric parameters in apical NSCLC patients. Methods and Materials: Charts of NSCLC patients with primary upper lobe or superiorly located nodal disease who received {>=}50 Gy of definitive conventionally fractionated radiation or chemoradiation were retrospectively reviewed for evidence of brachial plexopathy and categorized as RIBP, TRBP, or trauma-related. Dosimetric data were gathered on ipsilateral brachial plexuses (IBP) contoured accordingmore » to Radiation Therapy Oncology Group atlas guidelines. Results: Eighty patients were identified with a median follow-up and survival time of 17.2 and 17.7 months, respectively. The median prescribed dose was 66.6 Gy (range, 50.4-84.0), and 71% of patients received concurrent chemotherapy. RIBP occurred in 5 patients with an estimated 3-year rate of 12% when accounting for competing risk of death. Seven patients developed TRBP (estimated 3-year rate of 13%), comprising 24% of patients who developed locoregional failures. Grade 3 brachial plexopathy was more common in patients who experienced TRBP than RIBP (57% vs 20%). No patient who received {<=}78 Gy to the IBP developed RIBP. On multivariable competing risk analysis, IBP V76 receiving {>=}1 cc, and primary tumor failure had the highest hazard ratios for developing RIBP and TRBP, respectively. Conclusions: RIBP is a relatively uncommon complication in patients with apical NSCLC tumors receiving definitive doses of radiation, while patients who develop primary tumor failures are at high risk for developing morbid TRBP. These findings suggest that the importance of primary tumor control with adequate doses of radiation outweigh the risk of RIBP in this

c-jun-N-Terminal Kinase (JNK) for the Treatment of Amyotrophic Lateral Sclerosis

DTIC Science & Technology

2013-10-01

Treatment of Amyotrophic Lateral Sclerosis ” PRINCIPAL INVESTIGATOR: Dr. Philip LoGrasso CONTRACTING ORGANIZATION: The Scripps Research... Lateral Sclerosis ” 5a. CONTRACT NUMBER W81XWH-12-1-0431 5b. GRANT NUMBER W81XWH-12-1-0431 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) Philip...Annual 3. DATES COVERED 30September2012-29September2013 4. TITLE AND SUBTITLE “c-jun-N-Terminal Kinase (JNK) for the Treatment of Amyotrophic

Acute repair of traumatic pan-brachial plexus injury: technical considerations and approaches.

PubMed

Abou-Al-Shaar, Hussam; Karsy, Michael; Ravindra, Vijay; Joyce, Evan; Mahan, Mark A

2018-01-01

Particularly challenging after complete brachial plexus avulsion is reestablishing effective hand function, due to limited neurological donors to reanimate the arm. Acute repair of avulsion injuries may enable reinnervation strategies for achieving hand function. This patient presented with pan-brachial plexus injury. Given its irreparable nature, the authors recommended multistage reconstruction, including contralateral C-7 transfer for hand function, multiple intercostal nerves for shoulder/triceps function, shoulder fusion, and spinal accessory nerve-to-musculocutaneous nerve transfer for elbow flexion. The video demonstrates distal contraction from electrical stimulation of the avulsed roots. Single neurorrhaphy of the contralateral C-7 transfer was performed along with a retrosternocleidomastoid approach. The video can be found here: https://youtu.be/GMPfno8sK0U .

Rehabilitation program for children with brachial plexus and peripheral nerve injury.

PubMed

Ramos, L E; Zell, J P

2000-03-01

An aggressive and integrated physical and occupational therapy program is essential in the treatment of congenital brachial plexus injuries and other severe upper extremity nerve injuries. This article addresses the evaluation, identification of needs, establishment of goals, and the approaches to rehabilitation treatment for patients with brachial plexus palsy and other peripheral nerve injuries. Rehabilitative therapy can preserve and build on gains made possible by medical or surgical interventions; however, therapy is vital to these children regardless of whether surgery is indicated. The therapist uses a problem-solving approach to evaluate the patient and select appropriate occupational and physical therapy treatment modalities. Therapy is continually adjusted based on each child's unique needs. An understanding of the therapy principles aids in making appropriate referrals and prescriptions, and helps to coordinate care between the therapist, pediatrician, neurologist, and surgeon.

Subscapularis slide correction of the shoulder internal rotation contracture after brachial plexus birth injury: technique and outcomes.

PubMed

Immerman, Igor; Valencia, Herbert; DiTaranto, Patricia; DelSole, Edward M; Glait, Sergio; Price, Andrew E; Grossman, John A I

2013-03-01

Internal rotation contracture is the most common shoulder deformity in patients with brachial plexus birth injury. The purpose of this investigation is to describe the indications, technique, and results of the subscapularis slide procedure. The technique involves the release of the subscapularis muscle origin off the scapula, with preservation of anterior shoulder structures. A standard postoperative protocol is used in all patients and includes a modified shoulder spica with the shoulder held in 60 degrees of external rotation and 30 degrees of abduction, aggressive occupational and physical therapy, and subsequent shoulder manipulation under anesthesia with botulinum toxin injections as needed. Seventy-one patients at 2 institutions treated with subscapularis slide between 1997 and 2010, with minimum follow-up of 39.2 months, were identified. Patients were divided into 5 groups based on the index procedure performed: subscapularis slide alone (group 1); subscapularis slide with a simultaneous microsurgical reconstruction (group 2); primary microsurgical brachial plexus reconstruction followed later by a subscapularis slide (group 3); primary microsurgical brachial plexus reconstruction followed later by a subscapularis slide combined with tendon transfers for shoulder external rotation (group 4); and subscapularis slide with simultaneous tendon transfers, with no prior brachial plexus surgery (group 5). Full passive external rotation equivalent to the contralateral side was achieved in the operating room in all cases. No cases resulted in anterior instability or internal rotation deficit. Internal rotation contracture of the shoulder after brachial plexus birth injury can be effectively managed with the technique of subscapularis slide.

Hand Function in Children with an Upper Brachial Plexus Birth Injury: Results of the Nine-Hole Peg Test

ERIC Educational Resources Information Center

Immerman, Igor; Alfonso, Daniel T.; Ramos, Lorna E.; Grossman, Leslie A.; Alfonso, Israel; Ditaranto, Patricia; Grossman, John A. I.

2012-01-01

Aim: The aim of this study was to evaluate hand function in children with Erb upper brachial plexus palsy. Method: Hand function was evaluated in 25 children (eight males; 17 females) with a diagnosed upper (C5/C6) brachial plexus birth injury. Of these children, 22 had undergone primary nerve reconstruction and 13 of the 25 had undergone…

Characterizing Social Communication Changes in Amyotrophic Lateral Sclerosis

ERIC Educational Resources Information Center

Fisher, Fiona; Philpott, April; Andrews, Sophie C.; Maule, Roxanne; Douglas, Jacinta

2017-01-01

Background: Speech and language impairments are well-established in individuals with amyotrophic lateral sclerosis (ALS). However, knowledge about particular aspects of social communication and everyday conversational abilities is limited. Aims: To investigate self- and informant-report ratings of social communicative abilities in ALS participants…

Axillary Brachial Plexus Blockade for the Reflex Sympathetic Dystrophy Syndrome.

ERIC Educational Resources Information Center

Ribbers, G. M.; Geurts, A. C. H.; Rijken, R. A. J.; Kerkkamp, H. E. M.

1997-01-01

Reflex sympathetic dystrophy syndrome (RSD) is a neurogenic pain syndrome characterized by pain, vasomotor and dystrophic changes, and often motor impairments. This study evaluated the effectiveness of brachial plexus blockade with local anaesthetic drugs as a treatment for this condition. Three patients responded well; three did not. (DB)

Communication vulnerable in patients with Amyotrophic Lateral Sclerosis: A systematic review.

PubMed

Neto, Lavoisier Leite; Constantini, Ana Carolina; Chun, Regina Yu Shon

2017-01-01

Individuals with Amyotrophic Lateral Sclerosis (ALS) exhibit speech disorders since the early stages that decrease the communication rate and interfere in social participation. To conduct a literature review on communication vulnerable and Augmentative and Alternative Communication (AAC) in Amyotrophic Lateral Sclerosis. Descriptors of the Health Sciences Descriptors (DeCS) were used: Amyotrophic Lateral Sclerosis, Health Vulnerability, Communication Barriers, Nonverbal Communication, and Communication Aids for Disabled. Articles in Portuguese and English from 2010 to 2015, fully available in the Virtual Health Library, PubMed, and Scopus were used. Duplicate articles and those not related to communication/language were excluded. Of the 94 articles found, 37 met the criteria. All of them were published in the USA and Europe, none was Brazilian; 27% of 2012 to 2014; 40.5% descriptive studies and 24.3% case studies; 45.9% addressed ALS and 24.3%, other serious motor alterations, including ALS. A large proportion (89.2%) addressed AAC, 70.3% Brain-Computer Interface (BCI). The results show that the researches recurrently addressed communication vulnerable, although not necessarily in these terms. The device which was most employed was the BCI, mainly in advanced stages of the disease.

Cuff-Based Oscillometric Central and Brachial Blood Pressures Obtained Through ABPM are Similarly Associated with Renal Organ Damage in Arterial Hypertension.

PubMed

Fernández-Llama, Patricia; Pareja, Júlia; Yun, Sergi; Vázquez, Susana; Oliveras, Anna; Armario, Pedro; Blanch, Pedro; Calero, Francesca; Sierra, Cristina; de la Sierra, Alejandro

2017-01-01

Central blood pressure (BP) has been suggested to be a better estimator of hypertension-associated risks. We aimed to evaluate the association of 24-hour central BP, in comparison with 24-hour peripheral BP, with the presence of renal organ damage in hypertensive patients. Brachial and central (calculated by an oscillometric system through brachial pulse wave analysis) office BP and ambulatory BP monitoring (ABPM) data and aortic pulse wave velocity (PWV) were measured in 208 hypertensive patients. Renal organ damage was evaluated by means of the albumin to creatinine ratio and the estimated glomerular filtration rate. Fifty-four patients (25.9%) were affected by renal organ damage, displaying either microalbuminuria (urinary albumin excretion ≥30 mg/g creatinine) or an estimated glomerular filtration rate (eGFR) <60 ml/min/1.73 m2. Compared to those without renal abnormalities, hypertensive patients with kidney damage had higher values of office brachial systolic BP (SBP) and pulse pressure (PP), and 24-h, daytime, and nighttime central and brachial SBP and PP. They also had a blunted nocturnal decrease in both central and brachial BP, and higher values of aortic PWV. After adjustment for age, gender, and antihypertensive treatment, only ABPM-derived BP estimates (both central and brachial) showed significant associations with the presence of renal damage. Odds ratios for central BP estimates were not significantly higher than those obtained for brachial BP. Compared with peripheral ABPM, cuff-based oscillometric central ABPM does not show a closer association with presence of renal organ damage in hypertensive patients. More studies, however, need to be done to better identify the role of central BP in clinical practice. © 2017 The Author(s). Published by S. Karger AG, Basel.

Respiratory failure in a patient with antecedent poliomyelitis: amyotrophic lateral sclerosis or post-polio syndrome?

PubMed

Terao, Shin-ichi; Miura, Naofumi; Noda, Aiji; Yoshida, Mari; Hashizume, Yoshio; Ikeda, Hiroshi; Sobue, Gen

2006-10-01

We report a 69-year-old man who developed paralytic poliomyelitis in childhood and then decades later suffered from fatal respiratory failure. Six months before this event, he had progressive weight loss and shortness of breath. He had severe muscular atrophy of the entire right leg as a sequela of the paralytic poliomyelitis. He showed mild weakness of the facial muscle and tongue, dysarthria, and severe muscle atrophy from the neck to proximal upper extremities and trunk, but no obvious pyramidal signs. Electromyogram revealed neurogenic changes in the right leg, and in the paraspinal, sternocleidomastoid, and lingual muscles. There was a slight increase in central motor conduction time from the motor cortex to the lumbar anterior horn. Pulmonary function showed restrictive ventilation dysfunction, which was the eventual cause of death. Some neuropathological features were suggestive of amyotrophic lateral sclerosis (ALS), namely Bunina bodies. In patients with a history of paralytic poliomyelitis who present after a long stable period with advanced fatal respiratory failure, one may consider not only respiratory impairment from post-polio syndrome but also the onset of ALS.

Brachial Plexus in the Pampas Fox (Lycalopex gymnocercus): a Descriptive and Comparative Analysis.

PubMed

de Souza Junior, Paulo; da Cruz de Carvalho, Natan; de Mattos, Karine; Abidu Figueiredo, Marcelo; Luiz Quagliatto Santos, André

2017-03-01

Twenty thoracic limbs of ten Lycalopex gymnocercus were dissected to describe origin and distribution of the nerves forming brachial plexuses. The brachial plexus resulted from the connections between the ventral branches of the last three cervical nerves (C6, C7, and C8) and first thoracic nerve (T1). These branches connected the suprascapular, subscapular, axillary, musculocutaneous, radial, median and ulnar nerves to the intrinsic musculature and connected the brachiocephalic, thoracodorsal, lateral thoracic, long thoracic, cranial pectoral and caudal pectoral nerves to the extrinsic musculature. The C7 ventral branches contribute most to the formation of the nerves (62.7%), followed by C8 (58.8%), T1 (40.0%) and C6 (24.6%). Of the 260 nerves dissected, 69.2% resulted from a combination of two or three branches, while only 30.8% originated from a single branch. The origin and innervation area of the pampas fox brachial plexus, in comparison with other domestic and wild species, were most similar to the domestic dog and wild canids from the neotropics. The results of this study can serve as a base for comparative morphofunctional analysis involving this species and development of nerve block techniques. Anat Rec, 300:537-548, 2017. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Phrenic Nerve Transfer for Reconstruction of Elbow Extension in Severe Brachial Plexus Injuries.

PubMed

Flores, Leandro P; Socolovsky, Mariano

2016-09-01

Background Restoring elbow extension is an important objective to pursue when repairing the brachial plexus in patients with a flail arm. Based upon the good results obtained using the phrenic nerve to restore elbow flexion and shoulder stability, we hypothesized that this nerve could also be employed to reconstruct elbow extension in patients with severe brachial plexus injuries. Methods A retrospective study of 10 patients in which the phrenic nerve targeted the radial nerve (7 patients) or the branch to the long head of the triceps (3 patients) as a surgical strategy for reconstruction of the brachial plexus. Results The mean postoperative follow-up time was 34 months. At final follow-up, elbow extension graded as M4 was measured in three patients, Medical Research Council MRC M3 in five patients, and M2 in one patient, while one patient experienced no measurable recovery (M0). No patient complained or demonstrated any signs of respiratory insufficiency postoperatively. Conclusions The phrenic nerve is a reliable donor for reanimation of elbow extension in such cases, and the branch to the long head of the triceps should be considered as a better target for the nerve transfer. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Early onset of bilateral brachial plexopathy during mantle radiotherapy for Hodgkin's disease.

PubMed

Churn, M; Clough, V; Slater, A

2000-01-01

We report a case of brachial plexus neuropathy occurring in a 50-year-old man treated with standard mantle radiotherapy for early-stage Hodgkin's disease. A dose of 35 Gy in 20 fractions was given to the mantle field, following by a boost to the right side of the neck (8 Gy in four fractions). The onset of symptoms was early in the course of treatment and a gradual and almost full recovery was observed over 3 years after completion ofradiotherapy. The diagnosis was supported by electromyography. The temporal relationship of the radiotherapy and the onset of the brachial plexus neuropathy suggests a cause and effect, but this association is rarely reported after mantle radiotherapy. We review the aetiology of this condition and postulate possible mechanisms in this patient.

Diagnosis and treatment of the hemiplegic patient with brachial plexus injury.

PubMed

Meredith, J; Taft, G; Kaplan, P

1981-10-01

Brachial plexus injury was observed as a complication in 5 of 12 hemiplegic patients admitted over a 5-week period to an inpatient unit of the Rehabilitation Institute of Chicago. These patients exhibited unusual patterns of muscle atrophy and return of function in the impaired upper extremity. Occupational therapists may play an important part in the diagnosis and treatment of this complication of hemiplegia by promptly recognizing its subtle clinical signs and instituting appropriate therapy. Electromyography may be recommended to confirm this diagnosis. The treatment of choice is to maintain correct positioning of the limb both day and night, to use facilitation techniques for specific muscles in order to prevent atrophy, and to maintain passive range of motion as much as possible. Prevention of brachial plexus injury depends largely on the education of patient, family, and staff as to the potential hazards to a frail extremity that has no protective responses.

Ultrasound guided therapeutic injections of the cervical spine and brachial plexus.

PubMed

Cormick, Wes

2014-02-01

Introduction : Recent applications in ultrasound imaging include ultrasound assessment and ultrasound guided therapeutic injections of the spine and brachial plexus. Discussion : Ultrasound is an ideal modality for these regions as it allows accurate safe and quick injection of single or multiple sites. It has the added advantages of lack of ionising radiation, and can be done without requiring large expensive radiology equipment. Conclusion : Brachial plexus pathology may be present in patients presenting for shoulder symptoms where very little is found at imaging the shoulder. It is important to understand the anatomy and normal variants that may exist to be able to recognise when pathology is present. When pathology is demonstrated it is easy to do a trial of therapy with ultrasound guided injection of steroid around the nerve lesion. This review will outline the normal anatomy and variants and common pathology, which can be amenable to ultrasound guided injection of steroid.

c-jun-N-Terminal Kinase (JNK) for the Treatment of Amyotrophic Lateral Sclerosis

DTIC Science & Technology

2015-03-01

1 AWARD NUMBER: W81XWH-12-1-0431 TITLE: “c-jun-N-Terminal Kinase (JNK) for the Treatment of Amyotrophic Lateral Sclerosis ” PRINCIPAL...TITLE AND SUBTITLE “c-jun-N-Terminal Kinase (JNK) for the Treatment of Amyotrophic Lateral Scelerosis” 5a. CONTRACT NUMBER 5b. GRANT NUMBER... Lateral   Sclerosis ”   Final  Report:  Project  Period  Sept  2012-­‐Dec  2014     Personnel  List:     Feng,  Yangbo

System xC- is a mediator of microglial function and its deletion slows symptoms in amyotrophic lateral sclerosis mice.

PubMed

Mesci, Pinar; Zaïdi, Sakina; Lobsiger, Christian S; Millecamps, Stéphanie; Escartin, Carole; Seilhean, Danielle; Sato, Hideyo; Mallat, Michel; Boillée, Séverine

2015-01-01

Amyotrophic lateral sclerosis is the most common adult-onset motor neuron disease and evidence from mice expressing amyotrophic lateral sclerosis-causing SOD1 mutations suggest that neurodegeneration is a non-cell autonomous process where microglial cells influence disease progression. However, microglial-derived neurotoxic factors still remain largely unidentified in amyotrophic lateral sclerosis. With excitotoxicity being a major mechanism proposed to cause motor neuron death in amyotrophic lateral sclerosis, our hypothesis was that excessive glutamate release by activated microglia through their system [Formula: see text] (a cystine/glutamate antiporter with the specific subunit xCT/Slc7a11) could contribute to neurodegeneration. Here we show that xCT expression is enriched in microglia compared to total mouse spinal cord and absent from motor neurons. Activated microglia induced xCT expression and during disease, xCT levels were increased in both spinal cord and isolated microglia from mutant SOD1 amyotrophic lateral sclerosis mice. Expression of xCT was also detectable in spinal cord post-mortem tissues of patients with amyotrophic lateral sclerosis and correlated with increased inflammation. Genetic deletion of xCT in mice demonstrated that activated microglia released glutamate mainly through system [Formula: see text]. Interestingly, xCT deletion also led to decreased production of specific microglial pro-inflammatory/neurotoxic factors including nitric oxide, TNFa and IL6, whereas expression of anti-inflammatory/neuroprotective markers such as Ym1/Chil3 were increased, indicating that xCT regulates microglial functions. In amyotrophic lateral sclerosis mice, xCT deletion surprisingly led to earlier symptom onset but, importantly, this was followed by a significantly slowed progressive disease phase, which resulted in more surviving motor neurons. These results are consistent with a deleterious contribution of microglial-derived glutamate during symptomatic

The extracellular domain of neurotrophin receptor p75 as a candidate biomarker for amyotrophic lateral sclerosis.

PubMed

Shepheard, Stephanie R; Chataway, Tim; Schultz, David W; Rush, Robert A; Rogers, Mary-Louise

2014-01-01

Objective biomarkers for amyotrophic lateral sclerosis would facilitate the discovery of new treatments. The common neurotrophin receptor p75 is up regulated and the extracellular domain cleaved from injured neurons and peripheral glia in amyotrophic lateral sclerosis. We have tested the hypothesis that urinary levels of extracellular neurotrophin receptor p75 serve as a biomarker for both human motor amyotrophic lateral sclerosis and the SOD1(G93A) mouse model of the disease. The extracellular domain of neurotrophin receptor p75 was identified in the urine of amyotrophic lateral sclerosis patients by an immuno-precipitation/western blot procedure and confirmed by mass spectrometry. An ELISA was established to measure urinary extracellular neurotrophin receptor p75. The mean value for urinary extracellular neurotrophin receptor p75 from 28 amyotrophic lateral sclerosis patients measured by ELISA was 7.9±0.5 ng/mg creatinine and this was significantly higher (p<0.001) than 12 controls (2.6±0.2 ng/mg creatinine) and 19 patients with other neurological disease (Parkinson's disease and Multiple Sclerosis; 4.1±0.2 ng/mg creatinine). Pilot data of disease progression rates in 14 MND patients indicates that p75NTR(ECD) levels were significantly higher (p = 0.0041) in 7 rapidly progressing patients as compared to 7 with slowly progressing disease. Extracellular neurotrophin receptor p75 was also readily detected in SOD1(G93A) mice by immuno-precipitation/western blot before the onset of clinical symptoms. These findings indicate a significant relation between urinary extracellular neurotrophin receptor p75 levels and disease progression and suggests that it may be a useful marker of disease activity and progression in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis

PubMed Central

Wijesekera, Lokesh C; Leigh, P Nigel

2009-01-01

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterised by progressive muscular paralysis reflecting degeneration of motor neurones in the primary motor cortex, corticospinal tracts, brainstem and spinal cord. Incidence (average 1.89 per 100,000/year) and prevalence (average 5.2 per100,000) are relatively uniform in Western countries, although foci of higher frequency occur in the Western Pacific. The mean age of onset for sporadic ALS is about 60 years. Overall, there is a slight male prevalence (M:F ratio~1.5:1). Approximately two thirds of patients with typical ALS have a spinal form of the disease (limb onset) and present with symptoms related to focal muscle weakness and wasting, where the symptoms may start either distally or proximally in the upper and lower limbs. Gradually, spasticity may develop in the weakened atrophic limbs, affecting manual dexterity and gait. Patients with bulbar onset ALS usually present with dysarthria and dysphagia for solid or liquids, and limbs symptoms can develop almost simultaneously with bulbar symptoms, and in the vast majority of cases will occur within 1–2 years. Paralysis is progressive and leads to death due to respiratory failure within 2–3 years for bulbar onset cases and 3–5 years for limb onset ALS cases. Most ALS cases are sporadic but 5–10% of cases are familial, and of these 20% have a mutation of the SOD1 gene and about 2–5% have mutations of the TARDBP (TDP-43) gene. Two percent of apparently sporadic patients have SOD1 mutations, and TARDBP mutations also occur in sporadic cases. The diagnosis is based on clinical history, examination, electromyography, and exclusion of 'ALS-mimics' (e.g. cervical spondylotic myelopathies, multifocal motor neuropathy, Kennedy's disease) by appropriate investigations. The pathological hallmarks comprise loss of motor neurones with intraneuronal ubiquitin-immunoreactive inclusions in upper motor neurones and TDP-43 immunoreactive inclusions in

"Understanding my ALS". Experiences and reflections of persons with amyotrophic lateral sclerosis and relatives on participation in peer group rehabilitation.

PubMed

Madsen, Louise Sofia; Jeppesen, Jørgen; Handberg, Charlotte

2018-01-26

The aim of this study was to gain insight into experiences and reflections of persons with amyotrophic lateral sclerosis and relatives concerning the peer group rehabilitation programme "More Life - Less Illness". This qualitative study used the Interpretive Description methodology with Symbolic Interactionism as the analytical framework. Eighteen programme participants representing persons with amyotrophic lateral sclerosis (n = 8) and relatives (n = 10) were included. Data consisted of individual interviews and participant observation. The analysis revealed two categorical themes, "Sense of Community Building" and "Understanding my ALS", which represented the participants' experiences and reflections on peer group rehabilitation. Through the analysis, it became apparent that "Sense of Community Building" gave rise to an increased and personalised understanding of amyotrophic lateral sclerosis among the participants. As a part of the continuous processing of the knowledge gained, "Facing Facts" and "Retaining Normality" appeared as subthemes regarding the participants' ability to live a less dependent and more meaningful life. This study of peer group rehabilitation for persons with amyotrophic lateral sclerosis and relatives indicates that programme participation leads to positive experiences in terms of living a shared meaningful life despite severe disability. The findings may guide practice to develop longitudinal peer group rehabilitation programmes with joint inclusion of persons with amyotrophic lateral sclerosis and relatives. Implications for Rehabilitation Peer group rehabilitation may facilitate an increased and personalised understanding of what it means to live with amyotrophic lateral sclerosis. A programme design with six months of sequential sessions enables a continuous processing of shared experiences and gained knowledge. Joint participation of persons with amyotrophic lateral sclerosis and their relatives supports both their internal

Clinical Neurogenetics: Amyotrophic Lateral Sclerosis

PubMed Central

Harms, Matthew B.; Baloh, Robert H.

2013-01-01

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease, about which our understanding is expanding rapidly as its genetic causes are uncovered. The pace of new gene discovery over the last 5 years has accelerated, providing new insights into the pathogenesis of disease and highlighting biological pathways for target for therapeutic development. This article reviews our current understanding of the heritability of ALS, provides an overview of each of the major ALS genes, highlighting their phenotypic characteristics and frequencies as a guide for clinicians evaluating patients with ALS. PMID:24176417

Timing of rehabilitation in children with obstetric upper trunk brachial plexus palsy.

PubMed

Yilmaz, Volkan; Umay, Ebru; Tezel, Nihal; Gundogdu, Ibrahim

2018-06-01

The initiation timing of rehabilitation in children with obstetric brachial plexus palsy is controversial. The aim of the present study is to evaluate the effectiveness of rehabilitation timing to the functional outcomes of patients with obstetric upper trunk brachial plexus palsy. Twenty-nine patients, who did not previously received any rehabilitation programme but attended our outpatient clinic, were included for the study. The electrophysiological findings, obstetric characteristics, and demographic features of the patients were recorded. The range of motion (ROM) of shoulders, elbows, and wrists and the strength of the muscles associated with these joints were evaluated. Modified Mallet Scale (MMS) was used for functional evaluation. A 4-week rehabilitation programme was performed twice at 2-month intervals. Patients were divided into three groups according to their ages as follows: 1-3 years old (group 1), 3-5 years old (group 2), and 5-7 years old (group 3). The ROMs, muscle strengths, and MMS scores of the patients were all evaluated. Two out of 29 patients were female (6.9%) and 27 were male (93.1%). All 29 patients had right upper extremity palsy (100%). The MMS scores, ROMs, and muscle strength of the upper extremities had improved in all the groups following the standardized rehabilitation programme. A rehabilitation programme is the best choice of treatment before surgical procedures in patients with mild to moderate obstetric upper trunk brachial plexus palsy regardless of age and the initiation time.

Brachial plexus assessment with three-dimensional isotropic resolution fast spin echo MRI: comparison with conventional MRI at 3.0 T

PubMed Central

Tagliafico, A; Succio, G; Neumaier, C E; Baio, G; Serafini, G; Ghidara, M; Calabrese, M; Martinoli, C

2012-01-01

Objective The purpose of our study was to determine whether a three-dimensional (3D) isotropic resolution fast spin echo sequence (FSE-cube) has similar image quality and diagnostic performance to a routine MRI protocol for brachial plexus evaluation in volunteers and symptomatic patients at 3.0 T. Institutional review board approval and written informed consent were guaranteed. Methods In this prospective study FSE-cube was added to the standard brachial plexus examination protocol in eight patients (mean age, 50.2 years) with brachial plexus pathologies and in six volunteers (mean age, 54 years). Nerve visibility, tissue contrast, edge sharpness, image blurring, motion artefact and acquisition time were calculated for FSE-cube sequences and for the standard protocol on a standardised five-point scale. The visibility of brachial plexus nerve and surrounding tissues at four levels (roots, interscalene area, costoclavicular space and axillary level) was assessed. Results Image quality and nerve visibility did not significantly differ between FSE-cube and the standard protocol (p>0.05). Acquisition time was statistically and clinically significantly shorter with FSE-cube (p<0.05). Pathological findings were seen equally well with FSE-cube and the standard protocol. Conclusion 3D FSE-cube provided similar image quality in a shorter acquisition time and enabled excellent visualisation of brachial plexus anatomy and pathology in any orientation, regardless of the original scanning plane. PMID:21343321

A Potential Biomarker in Amyotrophic Lateral Sclerosis: Can Assessment of Brain Iron Deposition with SWI and Corticospinal Tract Degeneration with DTI Help?

PubMed

Sheelakumari, R; Madhusoodanan, M; Radhakrishnan, A; Ranjith, G; Thomas, B

2016-02-01

Iron-mediated oxidative stress plays a pivotal role in the pathogenesis of amyotrophic lateral sclerosis. This study aimed to assess iron deposition qualitatively and quantitatively by using SWI and microstructural changes in the corticospinal tract by using DTI in patients with amyotrophic lateral sclerosis. Seventeen patients with amyotrophic lateral sclerosis and 15 age- and sex-matched controls underwent brain MR imaging with SWI and DTI. SWI was analyzed for both signal-intensity scoring and quantitative estimation of iron deposition in the anterior and posterior banks of the motor and sensory cortices and deep gray nuclei. The diffusion measurements along the corticospinal tract at the level of pons and medulla were obtained by ROI analysis. Patients with amyotrophic lateral sclerosis showed reduced signal-intensity grades in the posterior bank of the motor cortex bilaterally. Quantitative analysis confirmed significantly higher iron content in the posterior bank of the motor cortex in patients with amyotrophic lateral sclerosis. In contrast, no significant differences were noted for the anterior bank of the motor cortex, anterior and posterior banks of the sensory cortex, and deep nuclei. Receiver operating characteristic comparison showed a cutoff of 35μg Fe/g of tissue with an area under the curve of 0.78 (P = .008) for the posterior bank of the motor cortex in discriminating patients with amyotrophic lateral sclerosis from controls. Fractional anisotropy was lower in the pyramidal tracts of patients with amyotrophic lateral sclerosis at the pons and medulla on either side, along with higher directionally averaged mean diffusivity values. The combination of SWI and DTI revealed an area under the curve of 0.784 for differentiating patients with amyotrophic lateral sclerosis from controls. Measurements of motor cortex iron deposition and diffusion tensor parameters of the corticospinal tract may be useful biomarkers for the diagnosis of clinically suspected

Influence of pregnancy and smoking on brachial artery flow-mediated dilation values and time until maximum response.

PubMed

Nicolau, Luis G C; Martins, Wellington P; Gallarreta, Francisco M P; Lima, Jailson C; Filho, Francisco Mauad

2011-08-01

To evaluate the effect of pregnancy and smoking on endothelial function using brachial artery flow-mediated dilation (FMD) and to determine the time necessary until the occurrence of maximum brachial artery dilation after stimulus. This study was an observational study evaluating 133 women, who were grouped as follows: non-smoking pregnant women (N = 47), smoking pregnant women (N = 33), non-smoking women (N = 34), and smoking pregnant women (N = 19). The diameter of the brachial artery was measured at baseline and at 30, 60, 90 and 120 s after stimulus. The relative change of brachial artery was determined for each of these four moments. FMD measured at 60 s after stimulus was compared between the groups. The maximum FMD was observed at 60 s after cuff release in all groups. FMD was greater among non-smoking pregnant women compared to smoking pregnant women (11.50 ± 5.77 vs. 8.74 ± 4.83; p = 0.03) and also between non-smoking non-pregnant women compared to smoking non-pregnant women (10.52 ± 4.76 vs. 7.21 ± 5.57; p = 0.03). Maximum FMD was observed approximately 60 s after stimulus in all groups regardless of smoking and pregnancy status. The smoking habit seems to lead to endothelial dysfunction both in pregnant and non-pregnant women, as demonstrated by the lower FMD in smokers.

Prevalence of Neuropathic Pain in Patients with Traumatic Brachial Plexus Injury: A Multicenter Prospective Hospital-Based Study.

PubMed

Ciaramitaro, Palma; Padua, Luca; Devigili, Grazia; Rota, Eugenia; Tamburin, Stefano; Eleopra, Roberto; Cruccu, Giorgio; Truini, Andrea

2017-12-01

Prevalence and clinical characteristics of neuropathic pain due to traumatic brachial plexus injury. Observational epidemiological study. Hospital-based multicenter study. One hundred seven prospectively enrolled patients with brachial plexus injury. All the patients underwent clinical examination and neurophysiological testing for a definitive diagnosis of the brachial plexus lesion. The DN4 questionnaire was used to identify neuropathic pain, and the Neuropathic Pain Symptom Inventory (NPSI) to evaluate the different symptoms of neuropathic pain. The SF36 questionnaire and the Beck Depression Inventory (BDI) were used to assess quality of life and mood disturbances in patients with neuropathic pain. Of the 107 enrolled patients, 74 had pain (69%); neuropathic pain, as assessed by means of the DN4, was identified in 60 (56%) of these patients. According to the NPSI, the most frequent and severe pain type was the spontaneous burning pain. Clinical and neurophysiological findings showed that pain is unrelated to age but is associated with the severity of peripheral nerve damage. The SF36 questionnaire and BDI showed that neuropathic pain impairs quality of life and causes depression. Our study provides information on the prevalence, characteristics, and variables associated with neuropathic pain due to traumatic brachial plexus injuries that might provide a basis for improving the clinical management of this condition. © 2017 American Academy of Pain Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com

Comparison Between Ultrasound-Guided Supraclavicular and Interscalene Brachial Plexus Blocks in Patients Undergoing Arthroscopic Shoulder Surgery: A Prospective, Randomized, Parallel Study.

PubMed

Ryu, Taeha; Kil, Byung Tae; Kim, Jong Hae

2015-10-01

Although supraclavicular brachial plexus block (SCBPB) was repopularized by the introduction of ultrasound, its usefulness in shoulder surgery has not been widely reported. The objective of this study was to compare motor and sensory blockades, the incidence of side effects, and intraoperative opioid analgesic requirements between SCBPB and interscalene brachial plexus block (ISBPB) in patients undergoing arthroscopic shoulder surgery. Patients were randomly assigned to 1 of 2 groups (ISBPB group: n = 47; SCBPB group: n = 46). The side effects of the brachial plexus block (Horner's syndrome, hoarseness, and subjective dyspnea), the sensory block score (graded from 0 [no cold sensation] to 100 [intact sensation] using an alcohol swab) for each of the 5 dermatomes (C5-C8 and T1), and the motor block score (graded from 0 [complete paralysis] to 6 [normal muscle force]) for muscle forces corresponding to the radial, ulnar, median, and musculocutaneous nerves were evaluated 20 min after the brachial plexus block. Fentanyl was administered in 50 μg increments when the patients complained of pain that was not relieved by the brachial plexus block. There were no conversions to general anesthesia due to a failed brachial plexus block. The sensory block scores for the C5 to C8 dermatomes were significantly lower in the ISBPB group. However, the percentage of patients who received fentanyl was comparable between the 2 groups (27.7% [ISBPB group] and 30.4% [SCBPB group], P = 0.77). SCBPB produced significantly lower motor block scores for the radial, ulnar, and median nerves than did ISBPB. A significantly higher incidence of Horner's syndrome was observed in the ISBPB group (59.6% [ISBPB group] and 19.6% [SCBPB group], P < 0.001). No patient complained of subjective dyspnea. Despite the weaker degree of sensory blockade provided by SCBPB in comparison to ISBPB, opioid analgesic requirements are similar during arthroscopic shoulder surgery under both brachial plexus blocks

Hypermetabolism is a deleterious prognostic factor in patients with amyotrophic lateral sclerosis.

PubMed

Jésus, P; Fayemendy, P; Nicol, M; Lautrette, G; Sourisseau, H; Preux, P-M; Desport, J-C; Marin, B; Couratier, P

2018-01-01

The aim of this study was to investigate patients with amyotrophic lateral sclerosis in order to determine their nutritional, neurological and respiratory parameters, and survival according to metabolic level. Nutritional assessment included resting energy expenditure (REE) measured by indirect calorimetry [hypermetabolism if REE variation (ΔREE) > 10%] and fat mass (FM) using impedancemetry. Neurological assessment included the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised score. Survival analysis used the Kaplan-Meier method and multivariate Cox model. A total of 315 patients were analysed. Median age at diagnosis was 65.9 years and 55.2% of patients were hypermetabolic. With regard to the metabolic level (ΔREE: < 10%, 10-20% and >20%), patients with ΔREE > 20% initially had a lower FM(29.7% vs. 32.1% in those with ΔREE ≤10%; P = 0.0054). During follow-up, the median slope of Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised tended to worsen more in patients with ΔREE > 20% (-1.4 vs. -1.0 points/month in those with ΔREE ≤10%; P = 0.07). Overall median survival since diagnosis was 18.4 months. ΔREE > 20% tended to increase the risk of dying compared with ΔREE ≤10% (hazard ratio, 1.33; P = 0.055). In multivariate analysis, an increased REE:FM ratio was independently associated with death (hazard ratio, 1.005; P = 0.001). Hypermetabolism is present in more than half of patients with amyotrophic lateral sclerosis. It modifies the body composition at diagnosis, and patients with hypermetabolism >20% have a worse prognosis than those without hypermetabolism. © 2017 EAN.

Novel Neuroprotective Multicomponent Therapy for Amyotrophic Lateral Sclerosis Designed by Networked Systems

PubMed Central

Herrando-Grabulosa, Mireia; Mulet, Roger; Pujol, Albert; Mas, José Manuel; Navarro, Xavier; Aloy, Patrick; Coma, Mireia; Casas, Caty

2016-01-01

Amyotrophic Lateral Sclerosis is a fatal, progressive neurodegenerative disease characterized by loss of motor neuron function for which there is no effective treatment. One of the main difficulties in developing new therapies lies on the multiple events that contribute to motor neuron death in amyotrophic lateral sclerosis. Several pathological mechanisms have been identified as underlying events of the disease process, including excitotoxicity, mitochondrial dysfunction, oxidative stress, altered axonal transport, proteasome dysfunction, synaptic deficits, glial cell contribution, and disrupted clearance of misfolded proteins. Our approach in this study was based on a holistic vision of these mechanisms and the use of computational tools to identify polypharmacology for targeting multiple etiopathogenic pathways. By using a repositioning analysis based on systems biology approach (TPMS technology), we identified and validated the neuroprotective potential of two new drug combinations: Aliretinoin and Pranlukast, and Aliretinoin and Mefloquine. In addition, we estimated their molecular mechanisms of action in silico and validated some of these results in a well-established in vitro model of amyotrophic lateral sclerosis based on cultured spinal cord slices. The results verified that Aliretinoin and Pranlukast, and Aliretinoin and Mefloquine promote neuroprotection of motor neurons and reduce microgliosis. PMID:26807587

Prediction of early and late preeclampsia by flow-mediated dilation of the brachial artery*

PubMed Central

Brandão, Augusto Henriques Fulgêncio; Evangelista, Aline Aarão; Martins, Raphaela Menin Franco; Leite, Henrique Vítor; Cabral, Antônio Carlos Vieira

2014-01-01

Objective To assess the accuracy in the prediction of both early and late preeclampsia by flow-mediated dilation of the brachial artery (FMD), a biophysical marker for endothelial dysfunction. Materials and Methods A total of 91 patients, considered at high risk for development of preeclampsia were submitted to brachial artery FMD between 24 and 28 weeks of gestation. Results Nineteen out of the selected patients developed preeclampsia, 8 in its early form and 11 in the late form. With a cut-off value of 6.5%, the FMD sensitivity for early preeclampsia prediction was 75.0%, with specificity of 73.3%, positive predictive value (PPV) of 32.4% and negative predictive value (NPV) of 91.9%. For the prediction of late preeclampsia, sensitivity = 83.3%, specificity = 73.2%, PPV = 34.4% and NPV = 96.2% were observed. And for the prediction of all associated forms of preeclampsia, sensitivity = 84.2%, specificity = 73.6%, PPV = 45.7% and NPV = 94.6% were observed. Conclusion FMD of the brachial artery is a test with good accuracy in the prediction of both early and late preeclampsia, which may represent a positive impact on the follow-up of pregnant women at high risk for developing this syndrome. PMID:25741086

MAPT as a predisposing gene for sporadic amyotrophic lateral sclerosis in the Chinese Han population

PubMed Central

Fang, Pu; Xu, Wenyuan; Wu, Chengsi; Zhu, Min; Li, Xiaobing; Hong, Daojun

2013-01-01

A previous study of European Caucasian patients with sporadic amyotrophic lateral sclerosis demonstrated that a polymorphism in the microtubule-associated protein Tau (MAPT) gene was significantly associated with sporadic amyotrophic lateral sclerosis pathogenesis. Here, we tested this association in 107 sporadic amyotrophic lateral sclerosis patients and 100 healthy controls from the Chinese Han population. We screened the mutation-susceptible regions of MAPT – the 3' and 5' untranslated regions as well as introns 9, 10, 11, and 12 – by direct sequencing, and identified 33 genetic variations. Two of these, 105788 A > G in intron 9 and 123972 T > A in intron 11, were not present in the control group. The age of onset in patients with the 105788 A > G and/or the 123972 T > A variant was younger than that in patients without either genetic variation. Moreover, the pa-tients with a genetic variation were more prone to bulbar palsy and breathing difficulties than those with the wild-type genotype. This led to a shorter survival period in patients with a MAPT genetic variant. Our study suggests that the MAPT gene is a potential risk gene for sporadic amyotrophic lateral sclerosis in the Chinese Han population. PMID:25206632

The phrenic nerve transfer in the treatment of a septuagenarian with brachial plexus avulsion injury: a case report.

PubMed

Jiang, Ye; Lao, Jie

2018-05-01

Phrenic nerve transfer has been a well-established procedure for restoring elbow flexion function in patients with brachial plexus avulsion injury. Concerning about probably detrimental respiratory effects brought by the operation, however, stirred up quite a bit of controversy. We present a case report of the successful application of phrenic nerve as donor to reinnervate the biceps in a septuagenarian with brachial plexus avulsion injury, not accompanied with significant clinical respiratory problem.

A comparative study of nerve stimulator versus ultrasound-guided supraclavicular brachial plexus block.

PubMed

Duncan, Mithun; Shetti, Akshaya N; Tripathy, Debendra Kumar; Roshansingh, D; Krishnaveni, N

2013-01-01

With the advent of ultrasound (US) guidance, this technique saw resurgence in the late 1990s. As US guidance provides real-time view of the block needle, the brachial plexus, and its spatial relationship to the surrounding vital structures; it not only increased the success rates, but also brought down the complication rates. Most of the studies show use of US guidance for performing brachial plexus block, results in near 100% success with or without complications. This study has been designed to examine the technique and usefulness of state-of-the-art US technology-guided supraclavicular brachial plexus block and compare it with routine nerve stimulator (NS)-guided technique. To note block execution time, time of onset of sensory and motor block, quality of block and success rates. Randomized controlled trial. A total of 60 patients were enrolled in this prospective randomized study and were randomly divided into two groups: US (Group US) and NS (Group NS). Both groups received 1:1 mixture of 0.5% bupivacaine and 2% lignocaine with 1:200000 adrenaline. The amount of local anaesthetic injected calculated according to the body weight and not crossing the toxic dosage (Inj. bupivacaine 2 mg/kg, Inj. lignocaine with adrenaline 7 mg/kg). The parameters compared between the two groups are block execution time, time of onset of sensory and motor block, quality of sensory and motor block, success rates are noted. The failed blocks are supplemented with general anesthesia. The data were analyzed using the SPSS (version 19) software. The parametric data were analyzed with student "t" test and the nonparametric data were analyzed with Chi-square test A P < 0.05 was considered significant. There was no significant difference between patient groups with regard to demographic data, the time of onset of sensory and motor block. Comparing the two groups, we found that the difference in the block execution time and success rates is not statistically significant. A failure rate of

Spatiotemporal Coupling of the Tongue in Amyotrophic Lateral Sclerosis

ERIC Educational Resources Information Center

Kuruvilla, Mili S.; Green, Jordan R.; Yunusova, Yana; Hanford, Kathy

2012-01-01

Purpose: The primary aim of the investigation was to identify deficits in spatiotemporal coupling between tongue regions in amyotrophic lateral sclerosis (ALS). The relations between disease-related changes in tongue movement patterns and speech intelligibility were also determined. Methods: The authors recorded word productions from 11…

Cultivating stem cells for treating amyotrophic lateral sclerosis

PubMed Central

Li, Shengwen Calvin; Yin, Hong Zhen; Loudon, William G; Weiss, John H

2012-01-01

This editorial addresses the current challenges and future directions in the use of stem cells as an approach for treating amyotrophic lateral sclerosis. A wide variety of literature has been reviewed to enlighten the reader on the many facets of stem cell research that are important to consider before using them for a cell based therapy. PMID:23516096

Supportive care needs of patients with amyotrophic lateral sclerosis/motor neuron disease and their caregivers: A scoping review.

PubMed

Oh, Juyeon; Kim, Jung A

2017-12-01

To identify the supportive care needs of amyotrophic lateral sclerosis/motor neuron disease patients and their caregivers, categorise and summarise them into a Supportive Care Needs Framework and identify gaps in literature. Little is known about the supportive care needs of amyotrophic lateral sclerosis/motor neuron disease patients and their caregivers, and this subject has not previously been systemically reviewed. Scoping review. We conducted a scoping review from the MEDLINE, EMBASE, CINAHL and Cochrane databases for the period January 2000-July 2016, using the following inclusion criteria: (i) written in English only, (ii) published in peer-reviewed journals, (iii) at least part of the research considered the supportive care needs perspective of amyotrophic lateral sclerosis/motor neuron disease patients or their caregivers and (iv) the population sample included patients of amyotrophic lateral sclerosis/motor neuron disease or their caregivers. Thirty-seven articles were included. Our review shows that amyotrophic lateral sclerosis/motor neuron disease patients and their caregivers' supportive care needs were mentioned across all seven domains of the Supportive Care Needs Framework. Most common were practical needs (n = 24), followed by Informational needs (n = 19), Social needs (n = 18), Psychological needs (n = 16), Physical needs (n = 15), Emotional needs (n = 13) and Spiritual needs (n = 8). From the perspectives of amyotrophic lateral sclerosis/motor neuron disease patients and their caregivers, there is a significant need for more practical, social, informational, psychological, physical, emotional and spiritual support. The Supportive Care Needs Framework has potential utility in the development of patient-centred support services or healthcare policies and serves as an important base for further studies; especially, specific examples of each supportive care needs domain can guide in clinical settings when healthcare professionals

The interaction between breathing and swallowing in amyotrophic lateral sclerosis.

PubMed

Erdem, Nazan Simsek; Karaali, Kamil; Ünal, Ali; Kızılay, Ferah; Öğüş, Candan; Uysal, Hilmi

2016-12-01

The aim of the study is to determine the association between respiratory swallow patterns in amyotrophic lateral sclerosis (ALS) patients. Furthermore, it aims to clarify the role of the dysphagia limit in defining the relationship between swallowing disorders and respiratory disorders. Functional rating scales were used to describe swallowing and respiratory function. Swallowing was observed using the dysphagia limit. Dysphagia limit is the volume at which a second or more swallows are required to swallow the whole bolus. Laryngeal and chest movement sensors, pulmonary function tests, submental, and diaphragm electromyography activity were used to evaluate the relationship between swallowing and respiratory phase. Of the 27 patients included in the study, 14 were dysphagic and 13 were non-dysphagic. Tests showed normal respiratory function in 11 of the non-dysphagic patients and 3 of the dysphagic patients. There was a high correlation between the dysphagia limit and Amyotrophic Lateral Sclerosis Functional Rating Scale swallowing parameters. Non-dysphagic patients were able to swallow during inspiration but only six patients in the dysphagic group were able to swallow during inspiration. The occurrence of dysphagia in ALS is related to piecemeal deglutition and respiration consistency during swallowing. Detecting the timing of disturbances in the relationship between swallowing and respiration may be a way of identifying dysphagia. Dysphagia limit may be a useful, complementary test for assessing swallowing disturbances in amyotrophic lateral sclerosis.

MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis.

PubMed

MacNair, Laura; Xiao, Shangxi; Miletic, Denise; Ghani, Mahdi; Julien, Jean-Pierre; Keith, Julia; Zinman, Lorne; Rogaeva, Ekaterina; Robertson, Janice

2016-01-01

Tar DNA-binding protein 43 (TDP-43) is an RNA-binding protein normally localized to the nucleus of cells, where it elicits functions related to RNA metabolism such as transcriptional regulation and alternative splicing. In amyotrophic lateral sclerosis, TDP-43 is mislocalized from the nucleus to the cytoplasm of diseased motor neurons, forming ubiquitinated inclusions. Although mutations in the gene encoding TDP-43, TARDBP, are found in amyotrophic lateral sclerosis, these are rare. However, TDP-43 pathology is common to over 95% of amyotrophic lateral sclerosis cases, suggesting that abnormalities of TDP-43 play an active role in disease pathogenesis. It is our hypothesis that a loss of TDP-43 from the nucleus of affected motor neurons in amyotrophic lateral sclerosis will lead to changes in RNA processing and expression. Identifying these changes could uncover molecular pathways that underpin motor neuron degeneration. Here we have used translating ribosome affinity purification coupled with microarray analysis to identify the mRNAs being actively translated in motor neurons of mutant TDP-43(A315T) mice compared to age-matched non-transgenic littermates. No significant changes were found at 5 months (presymptomatic) of age, but at 10 months (symptomatic) the translational profile revealed significant changes in genes involved in RNA metabolic process, immune response and cell cycle regulation. Of 28 differentially expressed genes, seven had a ≥ 2-fold change; four were validated by immunofluorescence labelling of motor neurons in TDP-43(A315T) mice, and two of these were confirmed by immunohistochemistry in amyotrophic lateral sclerosis cases. Both of these identified genes, DDX58 and MTHFSD, are RNA-binding proteins, and we show that TDP-43 binds to their respective mRNAs and we identify MTHFSD as a novel component of stress granules. This discovery-based approach has for the first time revealed translational changes in motor neurons of a TDP-43 mouse model

Vascular endothelial growth factor gene therapy improves nerve regeneration in a model of obstetric brachial plexus palsy.

PubMed

Hillenbrand, Matthias; Holzbach, Thomas; Matiasek, Kaspar; Schlegel, Jürgen; Giunta, Riccardo E

2015-03-01

The treatment of obstetric brachial plexus palsy has been limited to conservative therapies and surgical reconstruction of peripheral nerves. In addition to the damage of the brachial plexus itself, it also leads to a loss of the corresponding motoneurons in the spinal cord, which raises the need for supportive strategies that take the participation of the central nervous system into account. Based on the protective and regenerative effects of VEGF on neural tissue, our aim was to analyse the effect on nerve regeneration by adenoviral gene transfer of vascular endothelial growth factor (VEGF) in postpartum nerve injury of the brachial plexus in rats. In the present study, we induced a selective crush injury to the left spinal roots C5 and C6 in 18 rats within 24 hours after birth and examined the effect of VEGF-gene therapy on nerve regeneration. For gene transduction an adenoviral vector encoding for VEGF165 (AdCMV.VEGF165) was used. In a period of 11 weeks, starting 3 weeks post-operatively, functional regeneration was assessed weekly by behavioural analysis and force measurement of the upper limb. Morphometric evaluation was carried out 8 months post-operatively and consisted of a histological examination of the deltoid muscle and the brachial plexus according to defined criteria of degeneration. In addition, atrophy of the deltoid muscle was evaluated by weight determination comparing the left with the right side. VEGF expression in the brachial plexus was quantified by an enzyme-linked immunosorbent assay (ELISA). Furthermore the motoneurons of the spinal cord segment C5 were counted comparing the left with the right side. On the functional level, VEGF-treated animals showed faster nerve regeneration. It was found less degeneration and smaller mass reduction of the deltoid muscle in VEGF-treated animals. We observed significantly less degeneration of the brachial plexus and a greater number of surviving motoneurons (P < 0·05) in the VEGF group. The results of

Conjugal amyotrophic lateral sclerosis: a case report from Scotland.

PubMed

Fernandes, P M; Macleod, M R; Bateman, A; Abrahams, S; Pal, S

2017-03-29

Conjugal amyotrophic lateral sclerosis is rare, with significant effects on psychological and care needs. We report a case of conjugal amyotrophic lateral sclerosis disease from central Scotland. This case is particularly unusual as both patients were diagnosed within an 18-month period and experienced the disease simultaneously, with similar symptomatology and progression. Patient A was a 71-year-old man who presented with unilateral arm weakness and wasting. Patient B was a 68-year-old woman who presented with unilateral shoulder and elbow weakness. Diagnosis of amyotrophic lateral sclerosis was made within a few months of presentation in both cases, based on typical clinical symptomatology together with supportive neurophysiological testing. Interventions included enteral feeding and non-invasive ventilation. The time period between symptom onset and death was 5 years for Patient A and 3.5 years for Patient B. This case illustrates two main points: the care issues surrounding cases of conjugal neurological disease, and the psychological issues in these patients. There are significant care issues arising when co-habiting couples both develop severe functionally limiting neurological diseases at the same time. The more slowly progressive nature of Patient A's disease may be at least partially explained by the support he was able to receive from Patient B before she developed symptoms. Secondly, there are important psychological effects of living with someone with the same - but more advanced - progressive and incurable neurological disease. Thus, Patient B was reluctant to have certain interventions that she had observed being given to her husband. Lastly, no plausible shared environmental risk factors were identified, implying that the co-occurrence of ALS in this couple was a random association.

Analysis of amyotrophic lateral sclerosis as a multistep process: a population-based modelling study.

PubMed

Al-Chalabi, Ammar; Calvo, Andrea; Chio, Adriano; Colville, Shuna; Ellis, Cathy M; Hardiman, Orla; Heverin, Mark; Howard, Robin S; Huisman, Mark H B; Keren, Noa; Leigh, P Nigel; Mazzini, Letizia; Mora, Gabriele; Orrell, Richard W; Rooney, James; Scott, Kirsten M; Scotton, William J; Seelen, Meinie; Shaw, Christopher E; Sidle, Katie S; Swingler, Robert; Tsuda, Miho; Veldink, Jan H; Visser, Anne E; van den Berg, Leonard H; Pearce, Neil

2014-11-01

Amyotrophic lateral sclerosis shares characteristics with some cancers, such as onset being more common in later life, progression usually being rapid, the disease affecting a particular cell type, and showing complex inheritance. We used a model originally applied to cancer epidemiology to investigate the hypothesis that amyotrophic lateral sclerosis is a multistep process. We generated incidence data by age and sex from amyotrophic lateral sclerosis population registers in Ireland (registration dates 1995-2012), the Netherlands (2006-12), Italy (1995-2004), Scotland (1989-98), and England (2002-09), and calculated age and sex-adjusted incidences for each register. We regressed the log of age-specific incidence against the log of age with least squares regression. We did the analyses within each register, and also did a combined analysis, adjusting for register. We identified 6274 cases of amyotrophic lateral sclerosis from a catchment population of about 34 million people. We noted a linear relationship between log incidence and log age in all five registers: England r(2)=0·95, Ireland r(2)=0·99, Italy r(2)=0·95, the Netherlands r(2)=0·99, and Scotland r(2)=0·97; overall r(2)=0·99. All five registers gave similar estimates of the linear slope ranging from 4·5 to 5·1, with overlapping confidence intervals. The combination of all five registers gave an overall slope of 4·8 (95% CI 4·5-5·0), with similar estimates for men (4·6, 4·3-4·9) and women (5·0, 4·5-5·5). A linear relationship between the log incidence and log age of onset of amyotrophic lateral sclerosis is consistent with a multistage model of disease. The slope estimate suggests that amyotrophic lateral sclerosis is a six-step process. Identification of these steps could lead to preventive and therapeutic avenues. UK Medical Research Council; UK Economic and Social Research Council; Ireland Health Research Board; The Netherlands Organisation for Health Research and Development (ZonMw); the

Case-control study of amyotrophic lateral sclerosis

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

Deapen, D.M.; Henderson, B.E.

1986-05-01

The authors conducted a study of 518 amyotrophic lateral sclerosis patients identified between 1977 and 1979 and 518 controls to investigate putative risk factors for this disease. Occupations at risk of electrical exposure were reported more often by patients (odds ratio (OR) = 3.8, 95% confidence interval (CI) = 1.4-13.0) as were electrical shocks producing unconsciousness (OR = 2.8, 95% CI = 1.0-9.9). Although an overall excess of physical trauma associated with unconsciousness was observed in the amyotrophic lateral sclerosis patients (OR = 1.6, 95% CI = 1.0-2.4), the effect was inversely associated with duration of the unconscious episodes, suggestingmore » an effect of recall bias. Only slight differences were found for surgical traumata to the nervous system. Parkinsonism was reported more often among first degree relatives of cases (OR = 2.7, 95% CI = 1.1-7.6). The frequencies of prior poliomyelitis or other central nervous system diseases were similar for patients and controls. Occupational exposure to selected toxic substances was similar for patients and controls except for the manufacture of plastics (OR = 3.7, 95% CI = 1.0-20.5), although few details of these exposures were provided. No differences in occupations with exposure to animal skins or hides were observed.« less

Neurogenic Thoracic Outlet Syndrome Caused by Vascular Compression of the Brachial Plexus: A Report of Two Cases

PubMed Central

Hanna, Amgad; Bodden, Larry O'Neil; Siebiger, Gabriel R. L.

2018-01-01

Thoracic outlet syndrome (TOS) is caused by compression of the brachial plexus and/or subclavian vessels as they pass through the cervicothoracobrachial region, exiting the chest. There are three main types of TOS: neurogenic TOS, arterial TOS, and venous TOS. Neurogenic TOS accounts for approximately 95% of all cases, and it is usually caused by physical trauma (posttraumatic etiology), chronic repetitive motion (functional etiology), or bone or muscle anomalies (congenital etiology). We present two cases in which neurogenic TOS was elicited by vascular compression of the inferior portion of the brachial plexus. PMID:29497457

Comparison between isotropic linear-elastic law and isotropic hyperelastic law in the finite element modeling of the brachial plexus.

PubMed

Perruisseau-Carrier, A; Bahlouli, N; Bierry, G; Vernet, P; Facca, S; Liverneaux, P

2017-12-01

Augmented reality could help the identification of nerve structures in brachial plexus surgery. The goal of this study was to determine which law of mechanical behavior was more adapted by comparing the results of Hooke's isotropic linear elastic law to those of Ogden's isotropic hyperelastic law, applied to a biomechanical model of the brachial plexus. A model of finite elements was created using the ABAQUS ® from a 3D model of the brachial plexus acquired by segmentation and meshing of MRI images at 0°, 45° and 135° of shoulder abduction of a healthy subject. The offset between the reconstructed model and the deformed model was evaluated quantitatively by the Hausdorff distance and qualitatively by the identification of 3 anatomical landmarks. In every case the Hausdorff distance was shorter with Ogden's law compared to Hooke's law. On a qualitative aspect, the model deformed by Ogden's law followed the concavity of the reconstructed model whereas the model deformed by Hooke's law remained convex. In conclusion, the results of this study demonstrate that the behavior of Ogden's isotropic hyperelastic mechanical model was more adapted to the modeling of the deformations of the brachial plexus. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Tendon transfer to reconstruct wrist extension in children with obstetric brachial plexus palsy.

PubMed

Al-Qattan, M M

2003-04-01

This study reports on 20 children with obstetric brachial plexus palsy who underwent a tendon transfer to reconstruct wrist extension. The mean age at the time of tendon transfer was 8 years. There were seven patients with Erb's palsy and the remaining 13 had total palsy. The flexor carpi ulnaris was utilized 15 times and the flexor carpi radialis five times. The transferred tendon was sutured to the tendon of the extensor carpi radialis brevis. The result of the transfer was assessed according to a modified Medical Research Council (MRC) muscle grading system. A good result was obtained in 18 patients (modified MRC grade of 4) and a fair result (modified MRC grade of 3) in two. The choice of tendon transfer to reconstruct the wrist drop deformity in various conditions including adult traumatic brachial plexus injuries is discussed.

Ultrasound-guided approach for axillary brachial plexus, femoral nerve, and sciatic nerve blocks in dogs.

PubMed

Campoy, Luis; Bezuidenhout, Abraham J; Gleed, Robin D; Martin-Flores, Manuel; Raw, Robert M; Santare, Carrie L; Jay, Ariane R; Wang, Annie L

2010-03-01

To describe an ultrasound-guided technique and the anatomical basis for three clinically useful nerve blocks in dogs. Prospective experimental trial. Four hound-cross dogs aged 2 +/- 0 years (mean +/- SD) weighing 30 +/- 5 kg and four Beagles aged 2 +/- 0 years and weighing 8.5 +/- 0.5 kg. Axillary brachial plexus, femoral, and sciatic combined ultrasound/electrolocation-guided nerve blocks were performed sequentially and bilaterally using a lidocaine solution mixed with methylene blue. Sciatic nerve blocks were not performed in the hounds. After the blocks, the dogs were euthanatized and each relevant site dissected. Axillary brachial plexus block Landmark blood vessels and the roots of the brachial plexus were identified by ultrasound in all eight dogs. Anatomical examination confirmed the relationship between the four ventral nerve roots (C6, C7, C8, and T1) and the axillary vessels. Three roots (C7, C8, and T1) were adequately stained bilaterally in all dogs. Femoral nerve block Landmark blood vessels (femoral artery and femoral vein), the femoral and saphenous nerves and the medial portion of the rectus femoris muscle were identified by ultrasound in all dogs. Anatomical examination confirmed the relationship between the femoral vessels, femoral nerve, and the rectus femoris muscle. The femoral nerves were adequately stained bilaterally in all dogs. Sciatic nerve block. Ultrasound landmarks (semimembranosus muscle, the fascia of the biceps femoris muscle and the sciatic nerve) could be identified in all of the dogs. In the four Beagles, anatomical examination confirmed the relationship between the biceps femoris muscle, the semimembranosus muscle, and the sciatic nerve. In the Beagles, all but one of the sciatic nerves were stained adequately. Ultrasound-guided needle insertion is an accurate method for depositing local anesthetic for axillary brachial plexus, femoral, and sciatic nerve blocks.

"One-stop-shop" ultrasound diagnosis of functional, structural and physicomechanical properties of the brachial artery.

PubMed

Balzer, J; Boos, M; Rassaf, T; Heiss, Ch; Preik, M; Matern, S; Schoebel, F; Kelm, M; Lauer, T

2007-05-01

The pathogenesis of atherosclerosis comprises endothelial dysfunction, thickening as well as impaired compliance of the arterial vessel wall. Early assessment of these alterations of the vessel wall at the same site of the vascular tree has yet been hampered by the lack of highly sensitive diagnostic approaches suitable for clinical routine. We therefore aimed to develop and validate a single non-invasive examination of the brachial artery for simultaneous and highly accurate measurement of functional, structural and physicomechanical parameters of the brachial artery. 20 healthy individuals were investigated using high resolution ultrasound. Flow-mediated dilation (FMD), fractional diameter changes (FDC) and intima-media-thickness (IMT) were measured in the same segment of the brachial artery. Coefficients of variation, day-to-day-variability, between- and within-observer-variability were investigated in 5 individuals. All measurements were performed manually and by an automated PC-based analyzing system. Mean values for all measured parameters were 7.65 +/- 0.8% for FMD, 0.02 +/- 0.002 for FDC, 0.351 +/- 0.007 mm for IMT and followed an even distribution throughout the study population. Automated analysis of coefficient of variation, day-to-day-, between- and within-observer variabilities were: 0. 78%, 1.3%, 0.8%, 0.8% (FMD); 4.7%, 2.8%, 4.2%, 2.7% (FDC); 1.8%, 1.1%, 1.9%, 1.1% (IMT). Coefficient of variation, day-to-day-, between- and within-observer variabilities for the manual readings were significantly higher. Functional, structural and physicomechanical parameters of the brachial artery can be quantified consecutively, time-saving and highly reproducibly as an "one-stop-shop" in a single session using high resolution ultrasound with digitized post-processing. This highlights the future possibility of early, sensitive and non-invasive diagnostic testing of vascular function in patients prone to vascular disease.

User-guided automated segmentation of time-series ultrasound images for measuring vasoreactivity of the brachial artery induced by flow mediation

NASA Astrophysics Data System (ADS)

Sehgal, Chandra M.; Kao, Yen H.; Cary, Ted W.; Arger, Peter H.; Mohler, Emile R.

2005-04-01

Endothelial dysfunction in response to vasoactive stimuli is closely associated with diseases such as atherosclerosis, hypertension and congestive heart failure. The current method of using ultrasound to image the brachial artery along the longitudinal axis is insensitive for measuring the small vasodilatation that occurs in response to flow mediation. The goal of this study is to overcome this limitation by using cross-sectional imaging of the brachial artery in conjunction with the User-Guided Automated Boundary Detection (UGABD) algorithm for extracting arterial boundaries. High-resolution ultrasound imaging was performed on rigid plastic tubing, on elastic rubber tubing phantoms with steady and pulsatile flow, and on the brachial artery of a healthy volunteer undergoing reactive hyperemia. The area of cross section of time-series images was analyzed by UGABD by propagating the boundary from one frame to the next. The UGABD results were compared by linear correlation with those obtained by manual tracing. UGABD measured the cross-sectional area of the phantom tubing to within 5% of the true area. The algorithm correctly detected pulsatile vasomotion in phantoms and in the brachial artery. A comparison of area measurements made using UGABD with those made by manual tracings yielded a correlation of 0.9 and 0.8 for phantoms and arteries, respectively. The peak vasodilatation due to reactive hyperemia was two orders of magnitude greater in pixel count than that measured by longitudinal imaging. Cross-sectional imaging is more sensitive than longitudinal imaging for measuring flow-mediated dilatation of brachial artery, and thus may be more suitable for evaluating endothelial dysfunction.

Relation between digital peripheral arterial tonometry and brachial artery ultrasound measures of vascular function in patients with coronary artery disease and in healthy volunteers.

PubMed

Lee, Craig R; Bass, Almasa; Ellis, Kyle; Tran, Bryant; Steele, Savanna; Caughey, Melissa; Stouffer, George A; Hinderliter, Alan L

2012-03-01

Digital peripheral arterial tonometry (PAT) is an emerging, noninvasive method to assess vascular function. The physiology underlying this phenotype, however, remains unclear. Therefore, we evaluated the relation between digital PAT and established brachial artery ultrasound measures of vascular function under basal conditions and after reactive hyperemia. Using a cross-sectional study design, digital PAT and brachial artery ultrasonography with pulsed wave Doppler were simultaneously completed at baseline and after reactive hyperemia in both those with established coronary artery disease (n = 99) and healthy volunteers with low cardiovascular disease risk (n = 40). Under basal conditions, the digital pulse volume amplitude demonstrated a significant positive correlation with the brachial artery velocity-time integral that was independent of the arterial diameter, in both the healthy volunteer (r(s) = 0.64, p <0.001) and coronary artery disease (r(s) = 0.63, p <0.001) cohorts. Similar positive relations were observed with the baseline brachial artery blood flow velocity and blood flow. In contrast, no relation between the reactive hyperemia-evoked digital PAT ratio and either brachial artery flow-mediated dilation or shear stress was observed in either cohort (p = NS). In conclusion, these findings demonstrate that the digital PAT measures of vascular function more closely reflect basal blood flow in the brachial artery than reactive hyperemia-induced changes in the arterial diameter or flow velocity, and the presence of vascular disease does not modify the physiology underlying the digital PAT phenotype. Copyright © 2012 Elsevier Inc. All rights reserved.

Factor analysis of the Zarit Burden Interview in family caregivers of patients with amyotrophic lateral sclerosis.

PubMed

Oh, Juyeon; Kim, Jung A

2018-02-01

The Zarit Burden Interview has been used in many studies to assess caregiver burden in family caregivers of patients with amyotrophic lateral sclerosis, but the factor structure of the Zarit Burden Interview in the caregivers of amyotrophic lateral sclerosis patients is unknown. The aim of this study was to explore the factor structure of the Zarit Burden Interview in family caregivers of amyotrophic lateral sclerosis patients using exploratory factor analysis. The exploratory factor analysis was performed using generalized least squares with oblique rotation in a sample of 202 family caregivers. Three factors had an eigenvalue greater than 1 and accounted for 60.33% of the total variance. The three factors were named as follows: (factor 1) "Social restrictions" (items 2, 3, and 10-15); (factor 2) "Self-criticism" (items 20-21); and (factor 3) "Anger and frustration" (items 1, 4-6, 9, and 16-19). The correlation between factors 1 and 3 was much higher (r = 0.79) than that between factors 1 and 2 (r = 0.14) or factors 2 and 3 (r = 0.15). The findings of this study enriched our understanding of several meaningful dimensions of the caregiving burden in caregivers of an amyotrophic lateral sclerosis population and provided opportunities for future intervention.

Investigation of brachial plexus traction lesions by peripheral and spinal somatosensory evoked potentials.

PubMed Central

Jones, S J

1979-01-01

Peripheral, spinal and cortical somatosensory evoked potentials were recorded in 26 patients with unilateral traction injuries of the brachial plexus ganglia. Of 10 cases explored surgically the recordings correctly anticipated the major site of the lesion in eight. PMID:422958

Expression of microRNAs in human post-mortem amyotrophic lateral sclerosis spinal cords provides insight into disease mechanisms.

PubMed

Figueroa-Romero, Claudia; Hur, Junguk; Lunn, J Simon; Paez-Colasante, Ximena; Bender, Diane E; Yung, Raymond; Sakowski, Stacey A; Feldman, Eva L

2016-03-01

Amyotrophic lateral sclerosis is a late-onset and terminal neurodegenerative disease. The majority of cases are sporadic with unknown causes and only a small number of cases are genetically linked. Recent evidence suggests that post-transcriptional regulation and epigenetic mechanisms, such as microRNAs, underlie the onset and progression of neurodegenerative disorders; therefore, altered microRNA expression may result in the dysregulation of key genes and biological pathways that contribute to the development of sporadic amyotrophic lateral sclerosis. Using systems biology analyses on postmortem human spinal cord tissue, we identified dysregulated mature microRNAs and their potential targets previously implicated in functional process and pathways associated with the pathogenesis of ALS. Furthermore, we report a global reduction of mature microRNAs, alterations in microRNA processing, and support for a role of the nucleotide binding protein, TAR DNA binding protein 43, in regulating sporadic amyotrophic lateral sclerosis-associated microRNAs, thereby offering a potential underlying mechanism for sporadic amyotrophic lateral sclerosis. Copyright © 2015 Elsevier Inc. All rights reserved.

Open Anterior Release of the Superior Transverse Scapular Ligament for Decompression of the Suprascapular Nerve During Brachial Plexus Surgery.

PubMed

Elzinga, Kate E; Curran, Matthew W T; Morhart, Michael J; Chan, K Ming; Olson, Jaret L

2016-07-01

Reconstruction of the suprascapular nerve (SSN) after brachial plexus injury often involves nerve grafting or a nerve transfer. To restore shoulder abduction and external rotation, a branch of the spinal accessory nerve is commonly transferred to the SSN. To allow reinnervation of the SSN, any potential compression points should be released to prevent a possible double crush syndrome. For that reason, the authors perform a release of the superior transverse scapular ligament at the suprascapular notch in all patients undergoing reconstruction of the upper trunk of the brachial plexus. Performing the release through a standard anterior open supraclavicular approach to the brachial plexus avoids the need for an additional posterior incision or arthroscopic procedure. Copyright © 2016 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

Prognostic Factors in Amyotrophic Lateral Sclerosis: A Population-Based Study.

PubMed

Moura, Mirian Conceicao; Novaes, Maria Rita Carvalho Garbi; Eduardo, Emanoel Junio; Zago, Yuri S S P; Freitas, Ricardo Del Negro Barroso; Casulari, Luiz Augusto

2015-01-01

To determine the prognostic factors associated with survival in amyotrophic lateral sclerosis at diagnosis. This retrospective population-based study evaluated 218 patients treated with riluzole between 2005 and 2014 and described their clinical and demographic profiles after the analysis of clinical data and records from the mortality information system in the Federal District, Brazil. Cox multivariate regression analysis was conducted for the parameters found. The study sample consisted of 132 men and 86 women with a mean age at disease onset of 57.2±12.3 years; 77.6% of them were Caucasian. The mean periods between disease onset and diagnosis were 22.7 months among men and 23.5 months among women, and the mean survival periods were 45.7±47.0 months among men and 39.3±29.8 months among women. In addition, 80.3% patients presented non-bulbar-onset amyotrophic lateral sclerosis, and 19.7% presented bulbar-onset. Cox regression analysis indicated worse prognosis for body mass index (BMI) <25 kg/m2 (relative risk [RR]: 3.56, 95% confidence interval [CI]: 1.44-8.86), age >75 years (RR: 12.47, 95% CI: 3.51-44.26), and bulbar-onset (RR: 4.56, 95% CI: 2.06-10.12). Electromyography did not confirm the diagnosis in 55.6% of the suspected cases and in 27.9% of the bulbar-onset cases. The factors associated with lower survival in amyotrophic lateral sclerosis were age >75 years, BMI <25 kg/m2, and bulbar-onset.

Prognostic Factors in Amyotrophic Lateral Sclerosis: A Population-Based Study

PubMed Central

Moura, Mirian Conceicao; Novaes, Maria Rita Carvalho Garbi; Eduardo, Emanoel Junio; Zago, Yuri S. S. P.; Freitas, Ricardo Del Negro Barroso; Casulari, Luiz Augusto

2015-01-01

Objective To determine the prognostic factors associated with survival in amyotrophic lateral sclerosis at diagnosis. Methods This retrospective population-based study evaluated 218 patients treated with riluzole between 2005 and 2014 and described their clinical and demographic profiles after the analysis of clinical data and records from the mortality information system in the Federal District, Brazil. Cox multivariate regression analysis was conducted for the parameters found. Results The study sample consisted of 132 men and 86 women with a mean age at disease onset of 57.2±12.3 years; 77.6% of them were Caucasian. The mean periods between disease onset and diagnosis were 22.7 months among men and 23.5 months among women, and the mean survival periods were 45.7±47.0 months among men and 39.3±29.8 months among women. In addition, 80.3% patients presented non-bulbar-onset amyotrophic lateral sclerosis, and 19.7% presented bulbar-onset. Cox regression analysis indicated worse prognosis for body mass index (BMI) <25 kg/m2 (relative risk [RR]: 3.56, 95% confidence interval [CI]: 1.44–8.86), age >75 years (RR: 12.47, 95% CI: 3.51–44.26), and bulbar-onset (RR: 4.56, 95% CI: 2.06–10.12). Electromyography did not confirm the diagnosis in 55.6% of the suspected cases and in 27.9% of the bulbar-onset cases. Conclusions The factors associated with lower survival in amyotrophic lateral sclerosis were age >75 years, BMI <25 kg/m2, and bulbar-onset. PMID:26517122

Reactivity to low-flow as a potential determinant for brachial artery flow-mediated vasodilatation.

PubMed

Aizawa, Kunihiko; Elyas, Salim; Adingupu, Damilola D; Casanova, Francesco; Gooding, Kim M; Strain, W David; Shore, Angela C; Gates, Phillip E

2016-06-01

Previous studies have reported a vasoconstrictor response in the radial artery during a cuff-induced low-flow condition, but a similar low-flow condition in the brachial artery results in nonuniform reactivity. This variable reactivity to low-flow influences the subsequent flow-mediated dilatation (FMD) response following cuff-release. However, it is uncertain whether reactivity to low-flow is important in data interpretation in clinical populations and older adults. This study aimed to determine the influence of reactivity to low-flow on the magnitude of brachial artery FMD response in middle-aged and older individuals with diverse cardiovascular risk profiles. Data were analyzed from 165 individuals, divided into increased cardiovascular risk (CVR: n = 115, 85M, 67.0 ± 8.8 years) and healthy control (CTRL: n = 50, 30M, 63.2 ± 7.2 years) groups. Brachial artery diameter and blood velocity data obtained from Doppler ultrasound were used to calculate FMD, reactivity to low-flow and estimated shear rate (SR) using semiautomated edge-detection software. There was a significant association between reactivity to low-flow and FMD in overall (r = 0.261), CTRL (r = 0.410) and CVR (r = 0.189, all P < 0.05) groups. Multivariate regression analysis found that reactivity to low-flow, peak SR, and baseline diameter independently contributed to FMD along with sex, the presence of diabetes, and smoking (total R(2) = 0.450). There was a significant association between reactivity to low-flow and the subsequent FMD response in the overall dataset, and reactivity to low-flow independently contributed to FMD These findings suggest that reactivity to low-flow plays a key role in the subsequent brachial artery FMD response and is important in the interpretation of FMD data. © 2016 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of the American Physiological Society and The Physiological Society.

Increased in vivo glial activation in patients with amyotrophic lateral sclerosis: assessed with [(11)C]-PBR28.

PubMed

Zürcher, Nicole R; Loggia, Marco L; Lawson, Robert; Chonde, Daniel B; Izquierdo-Garcia, David; Yasek, Julia E; Akeju, Oluwaseun; Catana, Ciprian; Rosen, Bruce R; Cudkowicz, Merit E; Hooker, Jacob M; Atassi, Nazem

2015-01-01

Evidence from human post mortem, in vivo and animal model studies implicates the neuroimmune system and activated microglia in the pathology of amyotrophic lateral sclerosis. The study aim was to further evaluate in vivo neuroinflammation in individuals with amyotrophic lateral sclerosis using [(11)C]-PBR28 positron emission tomography. Ten patients with amyotrophic lateral sclerosis (seven males, three females, 38-68 years) and ten age- and [(11)C]-PBR28 binding affinity-matched healthy volunteers (six males, four females, 33-65 years) completed a positron emission tomography scan. Standardized uptake values were calculated from 60 to 90 min post-injection and normalized to whole brain mean. Voxel-wise analysis showed increased binding in the motor cortices and corticospinal tracts in patients with amyotrophic lateral sclerosis compared to healthy controls (p FWE < 0.05). Region of interest analysis revealed increased [(11)C]-PBR28 binding in the precentral gyrus in patients (normalized standardized uptake value = 1.15) compared to controls (1.03, p < 0.05). In patients those values were positively correlated with upper motor neuron burden scores (r = 0.69, p < 0.05), and negatively correlated with the amyotrophic lateral sclerosis functional rating scale (r = -0.66, p < 0.05). Increased in vivo glial activation in motor cortices, that correlates with phenotype, complements previous histopathological reports. Further studies will determine the role of [(11)C]-PBR28 as a marker of treatments that target neuroinflammation.

Comparative study of phrenic nerve transfers with and without nerve graft for elbow flexion after global brachial plexus injury.

PubMed

Liu, Yuzhou; Lao, Jie; Gao, Kaiming; Gu, Yudong; Zhao, Xin

2014-01-01

Nerve transfer is a valuable surgical technique in peripheral nerve reconstruction, especially in brachial plexus injuries. Phrenic nerve transfer for elbow flexion was proved to be one of the optimal procedures in the treatment of brachial plexus injuries in the study of Gu et al. The aim of this study was to compare phrenic nerve transfers with and without nerve graft for elbow flexion after brachial plexus injury. A retrospective review of 33 patients treated with phrenic nerve transfer for elbow flexion in posttraumatic global root avulsion brachial plexus injury was carried out. All the 33 patients were confirmed to have global root avulsion brachial plexus injury by preoperative and intraoperative electromyography (EMG), physical examination and especially by intraoperative exploration. There were two types of phrenic nerve transfers: type1 - the phrenic nerve to anterolateral bundle of anterior division of upper trunk (14 patients); type 2 - the phrenic nerve via nerve graft to anterolateral bundle of musculocutaneous nerve (19 patients). Motor function and EMG evaluation were performed at least 3 years after surgery. The efficiency of motor function in type 1 was 86%, while it was 84% in type 2. The two groups were not statistically different in terms of Medical Research Council (MRC) grade (p=1.000) and EMG results (p=1.000). There were seven patients with more than 4 month's delay of surgery, among whom only three patients regained biceps power to M3 strength or above (43%). A total of 26 patients had reconstruction done within 4 months, among whom 25 patients recovered to M3 strength or above (96%). There was a statistically significant difference of motor function between the delay of surgery within 4 months and more than 4 months (p=0.008). Phrenic nerve transfers with and without nerve graft for elbow flexion after brachial plexus injury had no significant difference for biceps reinnervation according to MRC grading and EMG. A delay of the surgery

[Antegrade catheterization of the brachial artery during treatment of arteriovenous angiodysplasia of the forearm and hand].

PubMed

Tsygankov, V N; Varava, A B

2013-01-01

The authors share herein their experience with an antegrade brachial access for treatment of patients presenting with arteriovenous angiodysplasia localizing on the distal portions of the upper limbs, also describing the choice of the site for puncture and the technique of antegrade catheterization of the brachial artery. This is followed by reporting the results of successful use of this access in a total of 27 patients. The access is simple to create, making it possible to easily perform the intervention using instruments of standard length. It is also safe, requires no bed rest in the postoperative period, and is well tolerated by the patients.

N-acetylcysteine neither lowers plasma homocysteine concentrations nor improves brachial artery endothelial function in cardiac transplant recipients.

PubMed

Miner, S E S; Cole, D E C; Evrovski, J; Forrest, Q; Hutchison, S J; Holmes, K; Ross, H J

2002-05-01

N-acetylcysteine is a novel antioxidant that has been reported to reduce plasma homocysteine concentrations and improve endothelial function. Cardiac transplant recipients have a high incidence of coronary endothelial dysfunction and hyperhomocysteinemia, both of which may lead to the development of transplantation coronary artery disease. It was hypothesized that N-acetylcysteine would reduce plasma homocysteine concentrations and improve brachial endothelial function in cardiac transplant recipients. A cohort of stable cardiac transplant recipients was recruited from the outpatient clinic at the Toronto General Hospital, Toronto, Ontario. Brachial artery endothelial functions were studied according to standard techniques to determine flow-mediated dilation of the brachial artery. Plasma homocysteine concentrations were assayed using high performance liquid chromatography with electrochemical detection and pulsed integrated amperometry. After baseline testing, patients were treated in an unblinded fashion with N-acetylcysteine 500 mg/day. After 10 weeks of therapy, patients returned for follow-up endothelial function and homocysteine testing. Thirty-one patients were initially enrolled. Two patients withdrew due to excessive gastrointestinal upset. Two patients did not return for follow-up testing. The remaining 27 patients tolerated the treatment well. At baseline, 85% of the patients had hyperhomocysteinemia (greater than 15 mol/L) with a mean plasma concentration of 18.6 4.7 mol/L. No changes in homocysteine concentrations were seen at follow-up. At baseline, the average flow-mediated dilation was only 4.7 6.3%. No changes were seen at follow-up. Hyperhomocysteinemia and brachial endothelial dysfunction are common in stable cardiac transplant recipients and are unaffected by supplementation with N-acetylcysteine.

Predicting functional decline and survival in amyotrophic lateral sclerosis.

PubMed

Ong, Mei-Lyn; Tan, Pei Fang; Holbrook, Joanna D

2017-01-01

Better predictors of amyotrophic lateral sclerosis disease course could enable smaller and more targeted clinical trials. Partially to address this aim, the Prize for Life foundation collected de-identified records from amyotrophic lateral sclerosis sufferers who participated in clinical trials of investigational drugs and made them available to researchers in the PRO-ACT database. In this study, time series data from PRO-ACT subjects were fitted to exponential models. Binary classes for decline in the total score of amyotrophic lateral sclerosis functional rating scale revised (ALSFRS-R) (fast/slow progression) and survival (high/low death risk) were derived. Data was segregated into training and test sets via cross validation. Learning algorithms were applied to the demographic, clinical and laboratory parameters in the training set to predict ALSFRS-R decline and the derived fast/slow progression and high/low death risk categories. The performance of predictive models was assessed by cross-validation in the test set using Receiver Operator Curves and root mean squared errors. A model created using a boosting algorithm containing the decline in four parameters (weight, alkaline phosphatase, albumin and creatine kinase) post baseline, was able to predict functional decline class (fast or slow) with fair accuracy (AUC = 0.82). However similar approaches to build a predictive model for decline class by baseline subject characteristics were not successful. In contrast, baseline values of total bilirubin, gamma glutamyltransferase, urine specific gravity and ALSFRS-R item score-climbing stairs were sufficient to predict survival class. Using combinations of small numbers of variables it was possible to predict classes of functional decline and survival across the 1-2 year timeframe available in PRO-ACT. These findings may have utility for design of future ALS clinical trials.

Neuro-Myelomatosis of the Brachial Plexus - An Unusual Site of Disease Visualized by FDG-PET/CT: A Case Report.

PubMed

Fukunaga, Hisanori; Mutoh, Tatsushi; Tatewaki, Yasuko; Shimomura, Hideo; Totsune, Tomoko; Terao, Chiaki; Miyazawa, Hidemitsu; Taki, Yasuyuki

2017-05-01

BACKGROUND Peripheral or cranial nerve root dysfunction secondary to invasion of the CNS in multiple myeloma is a rare clinical event that is frequently mistaken for other diagnoses. We describe the clinical utility of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET)/CT scanning for diagnosing neuro-myelomatosis. CASE REPORT A 63-year-old woman whose chief complaints were right shoulder and upper extremity pain underwent MRI and 18F-FDG PET/CT scan. MRI revealed a non-specific brachial plexus tumor. 18F-FDG PET/CT demonstrated intense FDG uptake in multiple intramedullary lesions and in the adjacent right brachial plexus, indicating extramedullary neural involvement associated with multiple myeloma, which was confirmed later by a bone marrow biopsy. CONCLUSIONS This is the first reported case of neuro-myelomatosis of the brachial plexus. It highlights the utility of the 18F-FDG PET/CT scan as a valuable diagnostic modality.

Illness trajectories in patients with amyotrophic lateral sclerosis: How illness progression is related to life narratives and interpersonal relationships.

PubMed

Cipolletta, Sabrina; Gammino, Giorgia Rosamaria; Palmieri, Arianna

2017-12-01

To identify illness trajectories in amyotrophic lateral sclerosis by analysing personal, social and functional dimensions related to amyotrophic lateral sclerosis progression. Previous studies have considered some psychological distinct variables that may moderate illness progression, but no research has combined an extensive qualitative understanding of amyotrophic lateral sclerosis patients' psychological characteristics and illness progression. A mixed-methods approach was used to combine quantitative and qualitative measures. Illness progression was assessed through a longitudinal design. Eighteen patients with amyotrophic lateral sclerosis attending a Neurology Department in northern Italy participated in the study. Semi-structured interviews to explore personal experience, and dependency grids to assess the distribution of dependency; ALSFRS-R and neuropsychological screening were, respectively, used to measure physical and cognitive impairment. To assess the progression of the disease, ALSFRS-R was re-administered after 8 months and mortality rate was considered. Data were analysed using the grounded theory approach. Illness progression changed according to the perception of the disease, the trust placed in medical care, self-construction and the distribution of dependency. Based on these categories, cases that had similar experiences were grouped, and four illness trajectories were identified: aggressiveness, threat, constriction and guilt. The findings suggest that it is possible to identify different illness trajectories in amyotrophic lateral sclerosis. Personalised intervention strategies may be construed based on the different trajectories identified. © 2017 John Wiley & Sons Ltd.

Assessment and nutrition education in patients with amyotrophic lateral sclerosis.

PubMed

Almeida, Claudinéa S; Stanich, Patricia; Salvioni, Cristina C S; Diccini, Solange

2016-11-01

Neurological patients with amyotrophic lateral sclerosis (ALS)often deteriorate to a worsening nutritional status. The aim of this study was to compare the nutritional status and food intake after nutrition education in patients with ALS. Clinical, anthropometric and functional variables were analyzed. Fifty-three patients were monitored at an early stage of the disease. The average score on the functionality scale was 33 points. Initially only 3.8% were classified as low body weight. After three months, 50% showed significant variation in anthropometric measures related to muscle mass and body fat reserves without association with clinical variables. After nutritional guidance, there was an increase in the intake of all food groups, especially the dairy group (p <0.05).The change of the nutritional status occurs early in patients with amyotrophic lateral sclerosis, even in those previously eutrophic or over weight. There was an increase in food intake after nutritional guidance according to the food guide adapted to the Brazilian population.

Use of brachial plexus blockade and medetomidine-ketamine-isoflurane anaesthesia for repair of radio-ulna fracture in an adult cheetah (Acinonyx jubatus).

PubMed

Kimeli, Peter; Mogoa, Eddy M; Mwangi, Willy E; Kipyegon, Ambrose N; Kirui, Gilbert; Muasya, Daniel W; Mande, John D; Kariuki, Edward; Mijele, Dominic

2014-10-10

Regional anaesthetic techniques have been used in combination with systemic analgesics during small animal surgery to provide multimodal analgesia. Brachial plexus nerves block using local anaesthetics provides analgesia of the thoracic limb through desensitization of the nerves that provide sensory and motor innervation. This has been shown to reduce intra-operative anesthetic requirements and provide postoperative pain relief. Decreasing the doses of general anaesthetics allows more stable cardiopulmonary function during anaesthesia and the development of less side effects. The present case reports a successful use of brachial plexus blockade to supplement medetomidine-ketamine-isoflurane anaesthesia for repair of radio-ulna fracture in an adult cheetah (acinonyx jubatus). An adult male Cheetah weighing about 65 kg was presented with a history of leg carrying lameness of the left forelimb sustained following a car accident a week earlier. Clinical examination under general anaesthesia revealed slight dehydration and a swelling with a wound on the caudo-medial aspect of the left radio-ulna region. Crepitation was present on manipulation and radiography confirmed a complete transverse radio-ulna fracture of the left forelimb, which required open reduction and internal fixation. Brachial plexus blockade using lignocaine hydrochloride was used to supplement medetomidine-ketamine-isoflurane anaesthesia for the surgical procedure. Isoflurane anaesthesia was maintained at 0.5 - 2.0% throughout the surgical procedure, which was uneventful. Temperature and cardio-pulmonary parameters remained stable intra-operatively. Limb paralysis extended for 5 hours post-operatively, suggesting prolonged anaesthesia. To the researchers' knowledge, this is the first reported case of the use of brachial plexus blockade to supplement general anaesthesia to facilitate forelimb surgery in an adult cheetah. The use of brachial plexus block with a light plane of general anaesthesia proved to

Calcium in the pathomechanism of amyotrophic lateral sclerosis - Taking center stage?

PubMed

Patai, Roland; Nógrádi, Bernát; Engelhardt, József I; Siklós, László

2017-02-19

Amyotrophic lateral sclerosis is an incurable, relentlessly progressive disease primarily affecting motor neurons. The cause of the disease, except for the mutations identified in a small fraction of patients, is unknown. The major mechanisms contributing to the degeneration of motor neurons have already been disclosed and characterized, including excitotoxicity, oxidative stress, mitochondrial dysfunction, and immune/inflammatory processes. During the progression of the disease these toxic processes are not discrete, but each facilitates the deleterious effect of the other. However, due to their common reciprocal calcium dependence, calcium ions may act as a common denominator and through a positive feedback loop may combine the individual pathological processes into a unified escalating mechanism of neuronal destruction. This mini-review provides an overview of the mutual calcium dependence of the major toxic mechanisms associated with amyotrophic lateral sclerosis. Copyright © 2016 Elsevier Inc. All rights reserved.

Ethnic and demographic incidence of amyotrophic lateral sclerosis (ALS) in Brazil: A population based study.

PubMed

Moura, Mirian Conceicao; Casulari, Luiz Augusto; Carvalho Garbi Novaes, Maria Rita

2016-01-01

Our objectives were to examine demographic and ethnic factors associated with amyotrophic lateral sclerosis in Brazil. The method used was a retrospective study of death certificates performed in June 2015, identifying the incidence of amyotrophic lateral sclerosis over 10 years, from January 2004 to December 2013, related to gender, age and race. Results revealed 8942 death certificates with 8152 as the underlying cause and 790 as a secondary cause. The average age was 62.7 ± 13.2 years, with a predominance of males (1·3:1). The adjusted mortality rate over 20 years was 0.61 to 0.89/100,000 person-years, and over 45 years was 1.77 to 2.3/100,000 person-years. There was a predominance of amyotrophic lateral sclerosis in Caucasians compared to the general population above 20 years (2010 Census), with an odds ratio (OR) of 2.92 (95% CI 2.78-3.07). The OR in blacks was 0.04 (95% CI: 0.03-0.04), in mestizos was 0.05 (0.04-0.07), and in Indians was 0.02 (0.01-0.04). The mean age was lower than in European populations (48.5 ± 12.3 years) (p < 0.0001). In conclusion, the incidence of amyotrophic lateral sclerosis in Brazil is close to other Latin American populations, with a lower age at death and clear predominance in Caucasians.

Etiogenic factors present in the cerebrospinal fluid from amyotrophic lateral sclerosis patients induce predominantly pro-inflammatory responses in microglia.

PubMed

Mishra, Pooja-Shree; Vijayalakshmi, K; Nalini, A; Sathyaprabha, T N; Kramer, B W; Alladi, Phalguni Anand; Raju, T R

2017-12-16

Microglial cell-associated neuroinflammation is considered as a potential contributor to the pathophysiology of sporadic amyotrophic lateral sclerosis. However, the specific role of microglia in the disease pathogenesis remains to be elucidated. We studied the activation profiles of the microglial cultures exposed to the cerebrospinal fluid from these patients which recapitulates the neurodegeneration seen in sporadic amyotrophic lateral sclerosis. This was done by investigating the morphological and functional changes including the expression levels of prostaglandin E2 (PGE2), cyclooxygenase-2 (COX-2), TNF-α, IL-6, IFN-γ, IL-10, inducible nitric oxide synthase (iNOS), arginase, and trophic factors. We also studied the effect of chitotriosidase, the inflammatory protein found upregulated in the cerebrospinal fluid from amyotrophic lateral sclerosis patients, on these cultures. We report that the cerebrospinal fluid from amyotrophic lateral sclerosis patients could induce an early and potent response in the form of microglial activation, skewed primarily towards a pro-inflammatory profile. It was seen in the form of upregulation of the pro-inflammatory cytokines and factors including IL-6, TNF-α, iNOS, COX-2, and PGE2. Concomitantly, a downregulation of beneficial trophic factors and anti-inflammatory markers including VEGF, glial cell line-derived neurotrophic factor, and IFN-γ was seen. In addition, chitotriosidase-1 appeared to act specifically via the microglial cells. Our findings demonstrate that the cerebrospinal fluid from amyotrophic lateral sclerosis patients holds enough cues to induce microglial inflammatory processes as an early event, which may contribute to the neurodegeneration seen in the sporadic amyotrophic lateral sclerosis. These findings highlight the dynamic role of microglial cells in the pathogenesis of the disease, thus suggesting the need for a multidimensional and temporally guarded therapeutic approach targeting the inflammatory

Amyotrophic Lateral Sclerosis Patients' Perspectives on Use of Mechanical Ventilation.

ERIC Educational Resources Information Center

Young, Jenny M.; And Others

1994-01-01

Interviewed 13 amyotrophic lateral sclerosis patients. All believed that they alone should make decision regarding use of mechanical ventilation. Factors they considered important were quality of life, severity of disability, availability of ventilation by means of nasal mask, possible admission to long-term care facility, ability to discontinue…

Amyotrophic Lateral Sclerosis: An Introduction to Psychosocial and Behavioral Adaptations.

ERIC Educational Resources Information Center

Hoffman, R. Leigh; Decker, Thomas W.

1993-01-01

Defines amyotrophic lateral sclerosis (ALS) as motor-neuron disease that is terminal. Discusses symptoms associated with ALS and identifies treatment options. Reviews psychological and behavioral adaptations in regard to ALS clients, their families, and professionals who work with them. Discusses support groups as method of reducing stress for ALS…

Management of Shoulder Problems Following Obstetric Brachial Plexus Injury

PubMed Central

Nixon, Matthew; Trail, Ian

2013-01-01

Obstetric brachial plexus injuries are common, with an incidence of 0.42 per 1000 live births in the UK, and with 25% of patients being left with permanent disability without intervention. The shoulder is the most commonly affected joint and, as a result of the subsequent imbalance of musculature, the abnormal deforming forces cause dysplasia of the glenohumeral joint. In the growing child, this presents with changing pattern of pathology, which requires a multidisciplinary approach and a broad range of treatment modalities to optimize function. PMID:27582903

Ankle Brachial Index: simple non-invasive estimation of peripheral artery disease

NASA Astrophysics Data System (ADS)

Pieniak, Marcin; Cieślicki, Krzysztof; Żyliński, Marek; Górski, Piotr; Murgrabia, Agnieszka; Cybulski, Gerard

2014-11-01

According to international guidelines, patients with Peripheral Artery Disease (PAD) are burdened with high cardiovascular risk. One of the simplest, non-invasive methods for PAD detection is the ankle-brachial index (ABI) measurement. The ABI is calculated as the ratio of systolic blood pressure at the ankle (pressure in the posterior tibial artery or the dorsal artery) to the systolic pressure in the arm (in the brachial artery) when the body is in a horizontal position. The physiological value of the ABI is assumed to be between 1 and 1.3; however, these limits vary from study to study. A value less than 0.9 indicates PAD. Some authors propose also measuring the ABI on both sides of the body to highlight possible differences in blood pressure between the opposite arterial segments. The aim of this study was to perform a meta-analysis of the ABI diagnostic criteria used in different publications. Additionally, ABI measurements were performed on 19 healthy patients in age ranged from 20 to 63 years. The results showed a slight dependence between age and the differences between the values obtained from left and right sides of the body.

Inhibition of β-Glucocerebrosidase Activity Preserves Motor Unit Integrity in a Mouse Model of Amyotrophic Lateral Sclerosis.

PubMed

Henriques, Alexandre; Huebecker, Mylene; Blasco, Hélène; Keime, Céline; Andres, Christian R; Corcia, Philippe; Priestman, David A; Platt, Frances M; Spedding, Michael; Loeffler, Jean-Philippe

2017-07-12

Recent metabolomic reports connect dysregulation of glycosphingolipids, particularly ceramide and glucosylceramide, to neurodegeneration and to motor unit dismantling in amyotrophic lateral sclerosis at late disease stage. We report here altered levels of gangliosides in the cerebrospinal fluid of amyotrophic lateral sclerosis patients in early disease stage. Conduritol B epoxide is an inhibitor of acid beta-glucosidase, and lowers glucosylceramide degradation. Glucosylceramide is the precursor for all of the more complex glycosphingolipids. In SOD1 G86R mice, an animal model of amyotrophic lateral sclerosis, conduritol B epoxide preserved ganglioside distribution at the neuromuscular junction, delayed disease onset, improved motor function and preserved motor neurons as well as neuromuscular junctions from degeneration. Conduritol B epoxide mitigated gene dysregulation in the spinal cord and restored the expression of genes involved in signal transduction and axonal elongation. Inhibition of acid beta-glucosidase promoted faster axonal elongation in an in vitro model of neuromuscular junctions and hastened recovery after peripheral nerve injury in wild type mice. Here, we provide evidence that glycosphingolipids play an important role in muscle innervation, which degenerates in amyotrophic lateral sclerosis from the early disease stage. This is a first proof of concept study showing that modulating the catabolism of glucosylceramide may be a therapeutic target for this devastating disease.

Patient satisfaction and self-reported outcomes after complete brachial plexus avulsion injury.

PubMed

Franzblau, Lauren E; Shauver, Melissa J; Chung, Kevin C

2014-05-01

Reconstructive surgery for complete brachial plexus avulsion injuries only partially restores function, and many patients are dissatisfied with results that surgeons consider good. Preoperative expectations have been shown to influence postoperative satisfaction but are poorly understood in patients with complete brachial plexus avulsion injuries. Qualitative methodology can elucidate patient beliefs and attitudes, which are difficult to quantify. The purpose of this study was to examine patient-reported outcomes, including satisfaction, and to understand the patient perspective. We used qualitative interviews and questionnaires to assess patient-reported outcomes. Two members of the research team analyzed interview data using Grounded Theory methodology. Data from participants who had and did not have reconstructive surgery were compared. Twelve patients participated in this study. Of the 7 participants who had reconstructive surgery, 4 felt their expectations had been met and 5 were satisfied with their outcomes. Reconstruction did not produce statistically significant improvements in upper extremity function, pain, or work ability. All patients reported dissatisfaction with upper extremity ability, and 9 expressed hope for innovative treatments (e.g., stem cell therapy, nerve reinsertion) that could potentially provide better outcomes than existing procedures and enable return to work. Satisfaction with surgical outcomes after complete avulsion brachial plexus injury depends heavily on whether preoperative expectations are met, but patients are unfamiliar with nerve avulsion and do not always know what to expect. Low satisfaction with upper extremity ability and the lack of statistically significant differences produced by reconstruction suggest that current treatments may not be meeting patients' needs. Physicians must provide robust preoperative education to encourage realistic expectations and direct patients toward resources for pain management to facilitate

Proportionate mortality of Italian soccer players: is amyotrophic lateral sclerosis an occupational disease?

PubMed

Belli, Stefano; Vanacore, Nicola

2005-01-01

The objective of the study is to investigate the mortality experience of Italian soccer players and to discuss the findings in the light of possible long term effects of doping. Standardized proportionate mortality ratio (SPMR) and standardized proportionate cancer mortality ratio (SPCMR) were computed for 350 deceased subjects deriving from a list of about 24,000 active Italian soccer players from 1960 to 1996 in the three top leagues (A, B and C). When considering SPMRs, there is a substantial adherence of observed to expected mortality, with the only exception of mortality for diseases of the nervous system (13 obs. vs. 6 exp.) mainly explained by an excess of amyotrophic lateral sclerosis (8 obs. vs 0.69 exp.). As far as SPCMRs are concerned, some digestive cancers (namely: colon cancer, liver cancer and pancreas cancer) show a doubled risk. A high risk for amyotrophic lateral sclerosis is observed among Italian soccer players. Epidemiological data on association between sport and Amyotrophic Lateral Sclerosis (ALS) are contrasting. On the basis of the overall available evidence we suggest a possible connection between dietary supplements or drugs used to enhance sporting performance and ALS pathogenesis. Further epidemiological studies are needed to confirm these specific mortality risks among soccer players.

Apo-Ferritin as a Therapeutic Treatment for Amyotrophic Lateral Sclerosis

DTIC Science & Technology

2012-09-01

infusions with or without H- ferritin shows a significant extension of lifespan and a clear trend of increased survival (Figures 7 and 8). Given that the......August 2012 4. TITLE AND SUBTITLE 5a. CONTRACT NUMBER Apo- Ferritin as a Therapeutic Treatment for Amyotrophic Lateral Sclerosis 5b. GRANT NUMBER

The Impact of Pediatric Brachial Plexus Injury on Families

PubMed Central

Allgier, Allison; Overton, Myra; Welge, Jeffrey; Mehlman, Charles T.

2015-01-01

Purpose To determine the impact on families of children with brachial plexus injuries in order to best meet their clinical and social needs. Methods Our cross-sectional study included families with children between the ages of 1 and 18 with birth or non-neonatal brachial plexus injuries (BPI). The consenting parent or guardian completed a demographic questionnaire and the validated Impact on Family Scale during a single assessment. Total scores can range from 0-100, with the higher the score indicating a higher impact on the family. Factor analysis and item-total correlations were used to examine structure, individual items, and dimensions of family impact. Results One hundred two caregivers participated. Overall, families perceived various dimensions of impact on having a child with a BPI. Total family impact was 43. The 2 individual items correlating most strongly with the overall total score were from the financial dimension of the Impact on Family Scale. The strongest demographic relationship was traveling nationally for care and treatment of the BPI. Severity of injury was marginally correlated with impact on the family. Parent-child agreement about the severity of the illness was relatively high. Conclusion Caretakers of children with a BPI perceived impact on their families in the form of personal strain, family/social factors, financial stress, and mastery. A multidisciplinary clinical care team should address the various realms of impact on family throughout the course of treatment. Level of Evidence II Prognostic PMID:25936738

An Investigation of Perspectives of Respite Admission Among People Living With Amyotrophic Lateral Sclerosis and the Hospitals That Support Them.

PubMed

Nakai, Michiko; Narita, Yugo; Tomimoto, Hidekazu

2017-07-01

Amyotrophic lateral sclerosis is a progressive disease with rapid degeneration. Respite care is an essential service for improving the well-being of both patients with this disease and their family caregivers, but accessibility of respite services is limited. This study investigates perspectives on respite admission among people living with amyotrophic lateral sclerosis and the hospitals supporting them. We conducted semistructured interviews among 3 patients with amyotrophic lateral sclerosis and 12 family members, exploring demographic information and their awareness and experience of respite admission. We also interviewed 16 representatives from hospitals about awareness of and preparation for respite admission for patients with this disease, the role of regional networks for intractable diseases, and knowledge about communication support schemes. We found significant differences in the revised Amyotrophic Lateral Sclerosis Functional Rating Scale between patients who had and had not received respite admission. Qualitative analysis of the data indicated that respite admission was a contributory factor in continuing and stabilizing home care. Limited provision of social services and hospital care quality were barriers to respite admission. Respite admission was essential to continued home care for patients with amyotrophic lateral sclerosis. Severe-stage patients were eligible for respite admission. Its accessibility, however, was limited, especially for patients living in rural areas. Supporting hospitals had limited capacity to respond to patients' needs. Individualized care and communication were internal barriers to respite admission.

Measuring surgeons' treatment preferences and satisfaction with nerve reconstruction techniques for children with unique brachial plexus birth palsies.

PubMed

Shah, Amee K; Zurakowski, David; Jessel, Rebecca H; Kuo, Anne; Waters, Peter M

2006-09-15

This study surveyed microsurgeons on treatments chosen for infants with brachial plexus birth palsies who have had failure of antigravity biceps and/or triceps function due to nerve surgery or natural history. Questionnaires were sent to surgeons participating in a prospective multicenter brachial plexus birth palsy study. With a response rate of 82 percent, the sample comprised 22 surgeons with extensive experience in treating brachial plexus birth palsy. The survey gathered collective information on two unique clinical groups: (1) infants with no antigravity biceps function but intact antigravity deltoid and radial nerve function and (2) infants with no antigravity radial nerve function (wrist and digital extension, triceps) but intact antigravity biceps and deltoid function. Analysis of data and age-based trends was performed using the Fisher's exact test. With failure of biceps recovery, surgeons preferred microsurgery for children 6 to 18 months old and tendon transfers for children older than 18 months. Both procedures were preferred over observation alone (p < 0.001). With regard to microsurgery techniques, with increasing age, surgeons used nerve transfers more than resected neuroma and grafting. With tendon transfers, regional transfers were performed more than 90 percent of the time at all ages. For patients with no antigravity radial nerve function, most cases at all ages were managed by observation rather than microsurgery or tendon transfers (p < 0.001). The authors' data indicate a general consensus in treatment choices for the two cases of microsurgical failure in infants with brachial plexus birth palsies as well as in satisfaction among experienced surgeons in using these treatments.

The Vienna psychosocial assessment procedure for bionic reconstruction in patients with global brachial plexus injuries.

PubMed

Hruby, Laura Antonia; Pittermann, Anna; Sturma, Agnes; Aszmann, Oskar Christian

2018-01-01

Global brachial plexopathies cause major sensory and motor deficits in the affected arm and hand. Many patients report of psychosocial consequences including chronic pain, decreased self-sufficiency, and poor body image. Bionic reconstruction, which includes the amputation and prosthetic replacement of the functionless limb, has been shown to restore hand function in patients where classic reconstructions have failed. Patient selection and psychological evaluation before such a life-changing procedure are crucial for optimal functional outcomes. In this paper we describe a psychosocial assessment procedure for bionic reconstruction in patients with complete brachial plexopathies and present psychosocial outcome variables associated with bionic reconstruction. Between 2013 and 2017 psychosocial assessments were performed in eight patients with global brachial plexopathies. We conducted semi-structured interviews exploring the psychosocial adjustment related to the accident, the overall psychosocial status, as well as motivational aspects related to an anticipated amputation and expectations of functional prosthetic outcome. During the interview patients were asked to respond freely. Their answers were transcribed verbatim by the interviewer and analyzed afterwards on the basis of a pre-defined item scoring system. The interview was augmented by quantitative evaluation of self-reported mental health and social functioning (SF-36 Health Survey), body image (FKB-20) and deafferentation pain (VAS). Additionally, psychosocial outcome variables were presented for seven patients before and after bionic reconstruction. Qualitative data revealed several psychological stressors with long-term negative effects on patients with complete brachial plexopathies. 88% of patients felt functionally limited to a great extent due to their disability, and all of them reported constant, debilitating pain in the deafferented hand. After bionic reconstruction the physical component summary

Watson-Jones Lecture, 1976. Some lesions of the brachial plexus.

PubMed Central

Bonney, G.

1977-01-01

Three types of lesion of the brachial plexus are discussed: entrapment syndrome; tumours; and traumatic lesions. In the first the importance of the pathological anatomy is stressed; in the second the rewarding results of accurate diagnosis and careful treatment are noted; and in the third the expanding possibilities of neural reconstruction and of specific treatment for pain are described. Images Fig. 1 Fig. 3 Fig. 4 Fig. 5 Fig. 8 Fig. 9 Fig. 10 PMID:879635

Wheelchair economy class syndrome in amyotrophic lateral sclerosis.

PubMed

Kimura, Fumiharu; Ishida, Simon; Furutama, Daisuke; Hirata, Yuuji; Sato, Toshihiko; Hosokawa, Takashi; Hanafusa, Toshiaki

2006-03-01

A wheelchair-bound 61-year-old diabetic man with amyotrophic lateral sclerosis (ALS) developed sudden respiratory failure. Specific findings for hypoxemia and hypocapnia were incompatible with type II respiratory failure seen in the terminal stages of ALS. 'Economy class syndrome' was diagnosed, with massive thrombosis in the pulmonary arteries and deep vein thrombosis. This case offers a warning for long-term wheelchair users, particularly hypoxemic ALS patients, regarding the risks of treatable pulmonary thromboembolism.

A comparative study of brachial plexus sonography and magnetic resonance imaging in chronic inflammatory demyelinating neuropathy and multifocal motor neuropathy.

PubMed

Goedee, H S; Jongbloed, B A; van Asseldonk, J-T H; Hendrikse, J; Vrancken, A F J E; Franssen, H; Nikolakopoulos, S; Visser, L H; van der Pol, W L; van den Berg, L H

2017-10-01

To compare the performance of neuroimaging techniques, i.e. high-resolution ultrasound (HRUS) and magnetic resonance imaging (MRI), when applied to the brachial plexus, as part of the diagnostic work-up of chronic inflammatory demyelinating neuropathy (CIDP) and multifocal motor neuropathy (MMN). Fifty-one incident, treatment-naive patients with CIDP (n = 23) or MMN (n = 28) underwent imaging of the brachial plexus using (i) a standardized MRI protocol to assess enlargement or T2 hyperintensity and (ii) bilateral HRUS to determine the extent of nerve (root) enlargement. We found enlargement of the brachial plexus in 19/51 (37%) and T2 hyperintensity in 29/51 (57%) patients with MRI and enlargement in 37/51 (73%) patients with HRUS. Abnormal results were only found in 6/51 (12%) patients with MRI and 12/51 (24%) patients with HRUS. A combination of the two imaging techniques identified 42/51 (83%) patients. We found no association between age, disease duration or Medical Research Council sum-score and sonographic nerve size, MRI enlargement or presence of T2 hyperintensity. Brachial plexus sonography could complement MRI in the diagnostic work-up of patients with suspected CIDP and MMN. Our results indicate that combined imaging studies may add value to the current diagnostic consensus criteria for chronic inflammatory neuropathies. © 2017 EAN.

Morphometric Atlas Selection for Automatic Brachial Plexus Segmentation

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

Van de Velde, Joris, E-mail: joris.vandevelde@ugent.be; Department of Radiotherapy, Ghent University, Ghent; Wouters, Johan

Purpose: The purpose of this study was to determine the effects of atlas selection based on different morphometric parameters, on the accuracy of automatic brachial plexus (BP) segmentation for radiation therapy planning. The segmentation accuracy was measured by comparing all of the generated automatic segmentations with anatomically validated gold standard atlases developed using cadavers. Methods and Materials: Twelve cadaver computed tomography (CT) atlases (3 males, 9 females; mean age: 73 years) were included in the study. One atlas was selected to serve as a patient, and the other 11 atlases were registered separately onto this “patient” using deformable image registration. Thismore » procedure was repeated for every atlas as a patient. Next, the Dice and Jaccard similarity indices and inclusion index were calculated for every registered BP with the original gold standard BP. In parallel, differences in several morphometric parameters that may influence the BP segmentation accuracy were measured for the different atlases. Specific brachial plexus-related CT-visible bony points were used to define the morphometric parameters. Subsequently, correlations between the similarity indices and morphometric parameters were calculated. Results: A clear negative correlation between difference in protraction-retraction distance and the similarity indices was observed (mean Pearson correlation coefficient = −0.546). All of the other investigated Pearson correlation coefficients were weak. Conclusions: Differences in the shoulder protraction-retraction position between the atlas and the patient during planning CT influence the BP autosegmentation accuracy. A greater difference in the protraction-retraction distance between the atlas and the patient reduces the accuracy of the BP automatic segmentation result.« less

Unusual cause of brachial palsy with diaphragmatic palsy.

PubMed

Gupta, Vishal; Pandita, Aakash; Panghal, Astha; Hassan, Neha

2018-05-12

We report a preterm neonate born with respiratory distress. The neonate was found to have diaphragmatic palsy and brachial palsy. The neonate was born by caesarean section and there was no history of birth trauma. On examination, there was bilateral congenital talipes equinovarus and a scar was present on the forearm. The mother had a history of chickenpox during the 16 weeks of pregnancy for which no treatment was sought. On investigation, PCR for varicella was found to be positive in the neonate. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Pan-brachial plexus neuropraxia following lightning: A rare case report.

PubMed

Patnaik, Ashis; Mahapatra, Ashok Kumar; Jha, Menka

2015-01-01

Neurological complications following lightning are rare and occur in form of temporary neurological deficits of central origin. Involvement of peripheral nervous system is extremely rare and only a few cases have been described in the literature. Isolated unilateral pan-brachial plexus neuropraxia has never been reported in the literature. Steroids have long been used for treatment of neuropraxia. However, their use in lightning neural injury is unique and requires special mention. We report a rare case of lightning-induced unilateral complete flaccid paralysis along with sensory loss in a young patient. Lightning typically causes central nervous involvement in various types of motor and sensory deficit. Surprisingly, the nerve conduction study showed the involvement of peripheral nervous system involvement. Steroids were administered and there was significant improvement in neurological functions within a short span of days. Patients' functions in the affected limb were normal in one month. Our case was interesting since it is the first such case in the literature where lightning has caused such a rare instance of unilateral pan-brachial plexus lesion. Such cases when seen, raises the possibility of more common central nervous system pathology rather than peripheral involvement. However, such lesions can be purely benign forms of peripheral nerve neuropraxia, which can be managed by steroid treatment without leaving any long-term neurological deficits.

Prevalence and variables associated with an abnormal ankle-brachial index among patients with human immunodeficiency virus/acquired immunodeficiency syndrome.

PubMed

Hinojosa, Carlos A; Nunez-Salgado, Ana E; Anaya-Ayala, Javier E; Laparra-Escareno, Hugo; Ortiz-Lopez, Laura J; Herrera-Caceres, Jaime O; Crabtree-Ramirez, Brenda E; Sierra-Madero, Juan G

2018-01-01

Objectives The longer survival of patients with human immunodeficiency virus/acquired immunodeficiency syndrome and the introduction of the highly active antiretroviral therapy have increased the number of chronic conditions; among these, cardiovascular diseases. The aim of this study is to determine patient, disease, and factors associated with peripheral arterial disease in a population of patients with human immunodeficiency virus/acquired immunodeficiency syndrome. Methods A prospective nested case-control study of a cohort of patients with human immunodeficiency virus/acquired immunodeficiency syndrome was conducted in a tertiary medical center in Mexico City. A sample size of 206 patients was calculated. Medical history, relevant laboratory data, peripheral arterial exam, and screening ankle-brachial index tests were obtained. Results The prevalence of abnormal ankle-brachial indexes was 20% (42 patients). Patient's mean age was 44 years ±13. The majority (98.5%) were actively receiving highly active antiretroviral therapy; active smoking was reported in 55 (27%), arterial hypertension and type 2 diabetes mellitus were found in 24 (12%) and 22 (11%) patients. Median time from the human immunodeficiency virus diagnosis was eight years (Interquartile range ±11); the mean CD4 count was 481, with a mean viral load of 13,557 copies (SD ± 69025.27) and 1889.18 (SD ± 9052.77) for patients with normal and abnormal ankle-brachial index and a median of 40 (IQ ± 2). Viral load ( p = 0.04) and number of years with human immunodeficiency virus/acquired immunodeficiency syndrome ( p = 0.04) were significantly associated with abnormal ankle-brachial indexes. Conclusions Abnormal ankle-brachial index seems to be more frequent in Mexican patients with human immunodeficiency virus/acquired immunodeficiency syndrome when compared with the general population at the same age. The most important factors associated with arterial disease were the viral

The diagnostic accuracy of 1.5T magnetic resonance imaging for detecting root avulsions in traumatic adult brachial plexus injuries.

PubMed

Wade, Ryckie G; Itte, Vinay; Rankine, James J; Ridgway, John P; Bourke, Grainne

2018-03-01

Identification of root avulsions is of critical importance in traumatic brachial plexus injuries because it alters the reconstruction and prognosis. Pre-operative magnetic resonance imaging is gaining popularity, but there is limited and conflicting data on its diagnostic accuracy for root avulsion. This cohort study describes consecutive patients requiring brachial plexus exploration following trauma between 2008 and 2016. The index test was magnetic resonance imaging at 1.5 Tesla and the reference test was operative exploration of the supraclavicular plexus. Complete data from 29 males was available. The diagnostic accuracy of magnetic resonance imaging for root avulsion(s) of C5-T1 was 79%. The diagnostic accuracy of a pseudomeningocoele as a surrogate marker of root avulsion(s) of C5-T1 was 68%. We conclude that pseudomeningocoles were not a reliable sign of root avulsion and magnetic resonance imaging has modest diagnostic accuracy for root avulsions in the context of adult traumatic brachial plexus injuries. III.

Endoplasmic reticulum stress and proteasomal system in amyotrophic lateral sclerosis.

PubMed

Karademir, Betul; Corek, Ceyda; Ozer, Nesrin Kartal

2015-11-01

Protein processing including folding, unfolding and degradation is involved in the mechanisms of many diseases. Unfolded protein response and/or endoplasmic reticulum stress are accepted to be the first steps which should be completed via protein degradation. In this direction, proteasomal system and autophagy play important role as the degradation pathways and controlled via complex mechanisms. Amyotrophic lateral sclerosis is a multifactorial neurodegenerative disease which is also known as the most catastrophic one. Mutation of many different genes are involved in the pathogenesis such as superoxide dismutase 1, chromosome 9 open reading frame 72 and ubiquilin 2. These genes are mainly related to the antioxidant defense systems, endoplasmic reticulum stress related proteins and also protein aggregation, degradation pathways and therefore mutation of these genes cause related disorders.This review focused on the role of protein processing via endoplasmic reticulum and proteasomal system in amyotrophic lateral sclerosis which are the main players in the pathology. In this direction, dysfunction of endoplasmic reticulum associated degradation and related cell death mechanisms that are autophagy/apoptosis have been detailed. Copyright © 2015 Elsevier Inc. All rights reserved.

Cognitive and behavioural deficits associated with the orbitomedial prefrontal cortex in amyotrophic lateral sclerosis.

PubMed

Meier, Sandra L; Charleston, Alison J; Tippett, Lynette J

2010-11-01

Amyotrophic lateral sclerosis, a progressive disease affecting motor neurons, may variably affect cognition and behaviour. We tested the hypothesis that functions associated with orbitomedial prefrontal cortex are affected by evaluating the behavioural and cognitive performance of 18 participants with amyotrophic lateral sclerosis without dementia and 18 healthy, matched controls. We measured Theory of Mind (Faux Pas Task), emotional prosody recognition (Aprosodia Battery), reversal of behaviour in response to changes in reward (Probabilistic Reversal Learning Task), decision making without risk (Holiday Apartment Task) and aberrant behaviour (Neuropsychiatric Inventory). We also assessed dorsolateral prefrontal function, using verbal and written fluency and planning (One-touch Stockings of Cambridge), to determine whether impairments in tasks sensitive to these two prefrontal regions co-occur. The patient group was significantly impaired at identifying social faux pas, recognizing emotions and decision-making, indicating mild, but consistent impairment on most measures sensitive to orbitomedial prefrontal cortex. Significant levels of aberrant behaviour were present in 50% of patients. Patients were also impaired on verbal fluency and planning. Individual subject analyses involved computing classical dissociations between tasks sensitive to different prefrontal regions. These revealed heterogeneous patterns of impaired and spared cognitive abilities: 33% of participants had classical dissociations involving orbitomedial prefrontal tasks, 17% had classical dissociations involving dorsolateral prefrontal tasks, 22% had classical dissociations between tasks of both regions, and 28% had no classical dissociations. These data indicate subtle changes in behaviour, emotional processing, decision-making and altered social awareness, associated with orbitomedial prefrontal cortex, may be present in a significant proportion of individuals with amyotrophic lateral sclerosis

Proximal Nephron

PubMed Central

Zhuo, Jia L.; Li, Xiao C.

2013-01-01

The kidney plays a fundamental role in maintaining body salt and fluid balance and blood pressure homeostasis through the actions of its proximal and distal tubular segments of nephrons. However, proximal tubules are well recognized to exert a more prominent role than distal counterparts. Proximal tubules are responsible for reabsorbing approximately 65% of filtered load and most, if not all, of filtered amino acids, glucose, solutes, and low molecular weight proteins. Proximal tubules also play a key role in regulating acid-base balance by reabsorbing approximately 80% of filtered bicarbonate. The purpose of this review article is to provide a comprehensive overview of new insights and perspectives into current understanding of proximal tubules of nephrons, with an emphasis on the ultrastructure, molecular biology, cellular and integrative physiology, and the underlying signaling transduction mechanisms. The review is divided into three closely related sections. The first section focuses on the classification of nephrons and recent perspectives on the potential role of nephron numbers in human health and diseases. The second section reviews recent research on the structural and biochemical basis of proximal tubular function. The final section provides a comprehensive overview of new insights and perspectives in the physiological regulation of proximal tubular transport by vasoactive hormones. In the latter section, attention is particularly paid to new insights and perspectives learnt from recent cloning of transporters, development of transgenic animals with knockout or knockin of a particular gene of interest, and mapping of signaling pathways using microarrays and/or physiological proteomic approaches. PMID:23897681

Intra-familial aggregation and heritability of aortic versus brachial pulse pressure after imputing pretreatment values in a community of African ancestry.

PubMed

Redelinghuys, Michelle; Norton, Gavin R; Maseko, Muzi J; Majane, Olebogeng H I; Woodiwiss, Angela J

2012-06-01

To compare the intra-familial aggregation and heritability of central (aortic) (PPc) versus peripheral (brachial) (PPp) pulse pressure after imputing pretreatment blood pressures (BPs) in treated participants in a community of black African ancestry. Central PPc [generalized transfer function (GTF) and radial P2-derived] was determined with applanation tonometry at the radial artery (SphygmoCor software) in 946 participants from 258 families with 23 families including three generations from an urban developing community of black Africans. In the 24.1% of participants receiving antihypertensive treatment, pretreatment brachial BP was imputed from published overall averaged effects of therapy grouped by class and dose, specific for groups of black African descent. From these data PPc was estimated from proportionate differences in central aortic and brachial PP. Heritability estimates were determined from SAGE software. Echocardiography was evaluated in 507 participants in order to determine stroke volume. With adjustments for confounders, parent-child (P < 0.05) and sibling-sibling (P < 0.0005) correlations were noted for log PPc, whilst for log PPp only sibling-sibling correlations were noted. No mother-father correlations were noted for either PPc or PPp. Independent of confounders the heritability for log GTF-derived (h = 0.33 ± 0.07, P < 0.0001) and P2-derived (h = 0.30 ± 0.07, P < 0.0001) PPc was greater than the heritability for log PPp (h = 0.11 ± 0.06, P < 0.05) (P < 0.05 for comparison of heritability estimates). After imputing pretreatment BP values, central aortic PP is significantly more inherited than brachial PP. These data suggest that in groups of African descent the genetic determinants of PP may be underestimated when employing brachial rather than central aortic PP measurements.

An anatomic description of intrinsic brachial muscles in the crab-eating fox (Cerdocyon thous, Linnaeus 1776) and report of a variant arterial distribution.

PubMed

Vélez, J; Ramírez, J; Aristizábal, O

2018-04-01

The crab-eating fox (Cerdocyon thous) is a wild canid distributed throughout South America. It is one of the wild canids reported being hit by vehicles and injured in snares, thus inducing trauma or injury to the musculoskeletal system, possibly occurring in the brachial region. The main objective of this research was to provide an anatomic description of the crab-eating fox's intrinsic brachial muscles including shape, origin, insertion, innervation and arterial blood supply, compared with that of the domestic dog. We dissected from superficial to deep two thoracic limbs of seven dead specimens donated to the University of Caldas by CORPOCALDAS. These muscles presented anatomic characteristics similar to those reported in the domestic dog (Canis lupus familiaris) but with a variant in arterial blood supply, allowing us to suggest that surgical procedures that need the knowledge of intrinsic brachial muscles in the crab-eating fox may be homologous to the domestic dog. However, one should consider its variant arterial distribution by part of the collateral radial artery and deep brachial artery to prevent incorrect incisions that may damage these arteries. © 2017 Blackwell Verlag GmbH.

Transfer of supinator motor branches to the posterior interosseous nerve in C7-T1 brachial plexus palsy.

PubMed

Bertelli, Jayme Augusto; Ghizoni, Marcos Flavio

2010-07-01

In C7-T1 palsies of the brachial plexus, shoulder and elbow function are preserved, but finger motion is absent. Finger flexion has been reconstructed by tendon or nerve transfers. Finger extension has been restored ineffectively by attaching the extensor tendons to the distal aspect of the dorsal radius (termed tenodesis) or by tendon transfers. In these palsies, supinator muscle function is preserved, because innervation stems from the C-6 root. The feasibility of transferring supinator branches to the posterior interosseous nerve has been documented in a previous anatomical study. In this paper, the authors report the clinical results of supinator motor nerve transfer to the posterior interosseous nerve in 4 patients with a C7-T1 root lesion. Four adult patients with C7-T1 root lesions underwent surgery between 5 and 7 months postinjury. The patients had preserved motion of the shoulder, elbow, and wrist, but they had complete palsy of finger motion. They underwent finger flexion reconstruction via transfer of the brachialis muscle, and finger and thumb extension were restored by transferring the supinator motor branches to the posterior interosseous nerve. This nerve transfer was performed through an incision over the proximal third of the radius. Dissection was carried out between the extensor carpi radialis brevis and the extensor digitorum communis. The patients were followed up as per regular protocol and underwent a final evaluation 12 months after surgery. To document the extent of recovery, the authors assessed the degree of active metacarpophalangeal joint extension of the long fingers. The thumb span was evaluated by measuring the distance between the thumb pulp and the lateral aspect of the index finger. Surgery to transfer the supinator motor branches to the posterior interosseous nerve was straightforward. Twelve months after surgery, all patients were capable of opening their hand and could fully extend their metacarpophalangeal joints. The

Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment.

PubMed

Goldstein, Laura H; Abrahams, Sharon

2013-04-01

Increased awareness of cognitive and behavioural change in amyotrophic lateral sclerosis has been driven by various clinic-based and population-based studies. A frontotemporal syndrome occurs in a substantial proportion of patients, a subgroup of whom present with frontotemporal dementia. Deficits are characterised by executive and working-memory impairments, extending to changes in language and social cognition. Behaviour and social cognition abnormalities are closely similar to those reported in behavioural variant frontotemporal dementia, implying a clinical spectrum linking amyotrophic lateral sclerosis and frontotemporal dementia. Cognitive impairment should be considered in clinical management, but few specialist assessment resources are available, and thus the cognitive status of most patients is unknown. Standard assessment procedures are not appropriate to detect dysfunction due to progressive physical disability; techniques that better measure the problems encountered by this group of patients are needed to further establish disease effects. Screening instruments are needed that are validated specifically for amyotrophic lateral sclerosis, encompass the heterogeneity of impairment, and accommodate physical disability. Copyright © 2013 Elsevier Ltd. All rights reserved.

Copper mediated neurological disorder: visions into amyotrophic lateral sclerosis, Alzheimer and Menkes disease.

PubMed

Ahuja, Anami; Dev, Kapil; Tanwar, Ranjeet S; Selwal, Krishan K; Tyagi, Pankaj K

2015-01-01

Copper (Cu) is a vital redox dynamic metal that is possibly poisonous in superfluous. Metals can traditionally or intricately cause propagation in reactive oxygen species (ROS) accretion in cells and this may effect in programmed cell death. Accumulation of Cu causes necrosis that looks to be facilitated by DNA damage, followed by activation of P53. Cu dyshomeostasis has also been concerned in neurodegenerative disorders such as Alzheimer, Amyotrophic lateral sclerosis (ALS) or Menkes disease and is directly related to neurodegenerative syndrome that usually produces senile dementia. These mortal syndromes are closely related with an immense damage of neurons and synaptic failure in the brain. This review focuses on copper mediated neurological disorders with insights into amyotrophic lateral sclerosis, Alzheimer and Menkes disease. Copyright © 2014 Elsevier GmbH. All rights reserved.

Synaptic sprouting increases the uptake capacities of motoneurons in amyotrophic lateral sclerosis mice

PubMed Central

Millecamps, Stéphanie; Nicolle, Delphine; Ceballos-Picot, Irène; Mallet, Jacques; Barkats, Martine

2001-01-01

Using adenoviruses encoding reporter genes as retrograde tracers, we assessed the capacity of motoneurons to take up and retrogradely transport adenoviral particles injected into the muscles of transgenic mice expressing the G93A human superoxide dismutase mutation, a model of amyotrophic lateral sclerosis. Surprisingly, transgene expression in the motoneurons was significantly higher in symptomatic mice than in control or presymptomatic mice. Using botulinum toxin to induce nerve sprouting at neuromuscular junctions, we showed that the unexpectedly high level of motoneurons retrograde transduction results, at least in part, from newly acquired uptake properties of the sprouts. These findings demonstrate the remarkable uptake properties of amyotrophic lateral sclerosis motoneurons in response to denervation and the rationale of using intramuscular injections of adenoviruses to overexpress therapeutic proteins in motor neuron diseases. PMID:11404466

Neurotization of elements of the brachial plexus.

PubMed

Friedman, A H

1991-01-01

Satisfactory therapy for an avulsion injury of the brachial plexus has yet to be described. Dorsal root entry zone lesions will usually mitigate the searing pain which is so disabling in some of these patients. Neurotization procedures are effective in restoring limited function to these patients. The most useful isolated movement of the upper extremity is elbow flexion, which is thus the primary target of neurotization procedures. Intercostal nerves and elements of the cervical plexus are the most commonly used donor nerves for neurotization procedures. From our experience and from a review of the literature, it appears that these procedures will be successful in approximately 50% of cases. It must be stressed that before performing a nerve transfer, the surgeon must be certain that the patient is not a candidate for a simple nerve graft.

The Vienna psychosocial assessment procedure for bionic reconstruction in patients with global brachial plexus injuries

PubMed Central

Sturma, Agnes

2018-01-01

Background Global brachial plexopathies cause major sensory and motor deficits in the affected arm and hand. Many patients report of psychosocial consequences including chronic pain, decreased self-sufficiency, and poor body image. Bionic reconstruction, which includes the amputation and prosthetic replacement of the functionless limb, has been shown to restore hand function in patients where classic reconstructions have failed. Patient selection and psychological evaluation before such a life-changing procedure are crucial for optimal functional outcomes. In this paper we describe a psychosocial assessment procedure for bionic reconstruction in patients with complete brachial plexopathies and present psychosocial outcome variables associated with bionic reconstruction. Methods Between 2013 and 2017 psychosocial assessments were performed in eight patients with global brachial plexopathies. We conducted semi-structured interviews exploring the psychosocial adjustment related to the accident, the overall psychosocial status, as well as motivational aspects related to an anticipated amputation and expectations of functional prosthetic outcome. During the interview patients were asked to respond freely. Their answers were transcribed verbatim by the interviewer and analyzed afterwards on the basis of a pre-defined item scoring system. The interview was augmented by quantitative evaluation of self-reported mental health and social functioning (SF-36 Health Survey), body image (FKB-20) and deafferentation pain (VAS). Additionally, psychosocial outcome variables were presented for seven patients before and after bionic reconstruction. Results Qualitative data revealed several psychological stressors with long-term negative effects on patients with complete brachial plexopathies. 88% of patients felt functionally limited to a great extent due to their disability, and all of them reported constant, debilitating pain in the deafferented hand. After bionic reconstruction the

Evaluation of Internal Construct Validity and Unidimensionality of the Brachial Assessment Tool, A Patient-Reported Outcome Measure for Brachial Plexus Injury.

PubMed

Hill, Bridget; Pallant, Julie; Williams, Gavin; Olver, John; Ferris, Scott; Bialocerkowski, Andrea

2016-12-01

To evaluate the internal construct validity and dimensionality of a new patient-reported outcome measure for people with traumatic brachial plexus injury (BPI) based on the International Classification of Functioning, Disability and Health definition of activity. Cross-sectional study. Outpatient clinics. Adults (age range, 18-82y) with a traumatic BPI (N=106). There were 106 people with BPI who completed a 51-item 5-response questionnaire. Responses were analyzed in 4 phases (missing responses, item correlations, exploratory factor analysis, and Rasch analysis) to evaluate the properties of fit to the Rasch model, threshold response, local dependency, dimensionality, differential item functioning, and targeting. Not applicable, as this study addresses the development of an outcome measure. Six items were deleted for missing responses, and 10 were deleted for high interitem correlations >.81. The remaining 35 items, while demonstrating fit to the Rasch model, showed evidence of local dependency and multidimensionality. Items were divided into 3 subscales: dressing and grooming (8 items), arm and hand (17 items), and no hand (6 items). All 3 subscales demonstrated fit to the model with no local dependency, minimal disordered thresholds, no unidimensionality or differential item functioning for age, time postinjury, or self-selected dominance. Subscales were combined into 3 subtests and demonstrated fit to the model, no misfit, and unidimensionality, allowing calculation of a summary score. This preliminary analysis supports the internal construct validity of the Brachial Assessment Tool, a unidimensional targeted 4-response patient-reported outcome measure designed to solely assess activity after traumatic BPI regardless of level of injury, age at recruitment, premorbid limb dominance, and time postinjury. Further examination is required to determine test-retest reliability and responsiveness. Copyright © 2016 American Congress of Rehabilitation Medicine

The TRK-Fused Gene Is Mutated in Hereditary Motor and Sensory Neuropathy with Proximal Dominant Involvement

PubMed Central

Ishiura, Hiroyuki; Sako, Wataru; Yoshida, Mari; Kawarai, Toshitaka; Tanabe, Osamu; Goto, Jun; Takahashi, Yuji; Date, Hidetoshi; Mitsui, Jun; Ahsan, Budrul; Ichikawa, Yaeko; Iwata, Atsushi; Yoshino, Hiide; Izumi, Yuishin; Fujita, Koji; Maeda, Kouji; Goto, Satoshi; Koizumi, Hidetaka; Morigaki, Ryoma; Ikemura, Masako; Yamauchi, Naoko; Murayama, Shigeo; Nicholson, Garth A.; Ito, Hidefumi; Sobue, Gen; Nakagawa, Masanori; Kaji, Ryuji; Tsuji, Shoji

2012-01-01

Hereditary motor and sensory neuropathy with proximal dominant involvement (HMSN-P) is an autosomal-dominant neurodegenerative disorder characterized by widespread fasciculations, proximal-predominant muscle weakness, and atrophy followed by distal sensory involvement. To date, large families affected by HMSN-P have been reported from two different regions in Japan. Linkage and haplotype analyses of two previously reported families and two new families with the use of high-density SNP arrays further defined the minimum candidate region of 3.3 Mb in chromosomal region 3q12. Exome sequencing showed an identical c.854C>T (p.Pro285Leu) mutation in the TRK-fused gene (TFG) in the four families. Detailed haplotype analysis suggested two independent origins of the mutation. Pathological studies of an autopsied patient revealed TFG- and ubiquitin-immunopositive cytoplasmic inclusions in the spinal and cortical motor neurons. Fragmentation of the Golgi apparatus, a frequent finding in amyotrophic lateral sclerosis, was also observed in the motor neurons with inclusion bodies. Moreover, TAR DNA-binding protein 43 kDa (TDP-43)-positive cytoplasmic inclusions were also demonstrated. In cultured cells expressing mutant TFG, cytoplasmic aggregation of TDP-43 was demonstrated. These findings indicate that formation of TFG-containing cytoplasmic inclusions and concomitant mislocalization of TDP-43 underlie motor neuron degeneration in HMSN-P. Pathological overlap of proteinopathies involving TFG and TDP-43 highlights a new pathway leading to motor neuron degeneration. PMID:22883144

Proximal Tibial Bone Graft

MedlinePlus

... All Site Content AOFAS / FootCareMD / Treatments Proximal Tibial Bone Graft Page Content What is a bone graft? Bone grafts may be needed for various ... the proximal tibia. What is a proximal tibial bone graft? Proximal tibial bone graft (PTBG) is a ...

Executive deficits, not processing speed relates to abnormalities in distinct prefrontal tracts in amyotrophic lateral sclerosis.

PubMed

Pettit, Lewis D; Bastin, Mark E; Smith, Colin; Bak, Thomas H; Gillingwater, Thomas H; Abrahams, Sharon

2013-11-01

Cognitive impairment in amyotrophic lateral sclerosis is characterized by deficits on tests of executive function; however, the contribution of abnormal processing speed is unknown. Methods are confounded by tasks that depend on motor speed in patients with physical disability. Structural and functional magnetic resonance imaging studies have revealed multi-system cerebral involvement, with evidence of reduced white matter volume and integrity in predominant frontotemporal regions. The current study has two aims. First, to investigate whether cognitive impairments in amyotrophic lateral sclerosis are due to executive dysfunction or slowed processing speed using methodology that accommodates motor disability. This is achieved using a dual-task paradigm and tasks that manipulate stimulus presentation times and do not rely on response motor speed. Second, to identify relationships between specific cognitive impairments and the integrity of distinct white matter tracts. Thirty patients with amyotrophic lateral sclerosis and 30 age- and education-matched control subjects were administered an experimental dual-task procedure that combined a visual inspection time task and digit recall. In addition, measures of executive function (including letter fluency) and processing speed (visual inspection time and rapid serial letter identification) were administered. Integrity of white matter tracts was determined using region of interest analyses of diffusion tensor magnetic resonance imaging data. Patients with amyotrophic lateral sclerosis did not show impairments on tests of processing speed, but executive deficits were revealed once visual inspection time was combined with digit recall (dual-task) and in letter fluency. In addition to the corticospinal tracts, significant differences in fractional anisotropy and mean diffusivity were found between groups in a number of prefrontal and temporal white matter tracts including the anterior cingulate, anterior thalamic radiation

The relation between amyotrophic lateral sclerosis and inorganic selenium in drinking water: a population-based case-control study

PubMed Central

2010-01-01

Background A community in northern Italy was previously reported to have an excess incidence of amyotrophic lateral sclerosis among residents exposed to high levels of inorganic selenium in their drinking water. Methods To assess the extent to which such association persisted in the decade following its initial observation, we conducted a population-based case-control study encompassing forty-one newly-diagnosed cases of amyotrophic lateral sclerosis and eighty-two age- and sex-matched controls. We measured long-term intake of inorganic selenium along with other potentially neurotoxic trace elements. Results We found that consumption of drinking water containing ≥ 1 μg/l of inorganic selenium was associated with a relative risk for amyotrophic lateral sclerosis of 5.4 (95% confidence interval 1.1-26) after adjustment for confounding factors. Greater amounts of cumulative inorganic selenium intake were associated with progressively increasing effects, with a relative risk of 2.1 (95% confidence interval 0.5-9.1) for intermediate levels of cumulative intake and 6.4 (95% confidence interval 1.3-31) for high intake. Conclusion Based on these results, coupled with other epidemiologic data and with findings from animal studies that show specific toxicity of the trace element on motor neurons, we hypothesize that dietary intake of inorganic selenium through drinking water increases the risk for amyotrophic lateral sclerosis. PMID:21134276

Reconstructive operations for the upper limb after brachial plexus palsy.

PubMed

Rühmann, Oliver; Schmolke, Stephan; Bohnsack, Michael; Carls, Jörg; Flamme, Christian; Wirth, Carl Joachim

2004-07-01

Limited function due to paralysis following brachial plexus lesions can be improved by secondary operations of the bony and soft tissue. Between April 1994 and December 2000, 109 patients suffering from arm-plexus lesions underwent a total of 144 reconstructive operations guided by our concept of integrated therapy. The average age at the time of surgery was 32 years (range: 15-59). The following operations were performed: shoulder arthrodesis (23), trapezius transfer (74), rotation osteotomy of humerus (9), triceps to biceps transposition (9), transposition of forearm flexors or extensors (8), latissimus transfer (7), pectoralis transfer (1), teres major transfer (1), transposition of flexor carpi ulnaris to the tendons of extensor digitorum (10), and wrist arthrodesis (2). Prospectively, in all patients, the grade of muscle power of the affected upper extremity was evaluated prior to surgery. The follow-up period for all 144 operations was, on average, 22 months (range: 6-74). By means of operative measures, almost all patients obtained an improvement of shoulder function (100%) and stability (>90%), elbow flexion (85%), and hand, finger, and thumb (100%). When muscles malfunction after brachial plexus lesions, one should take into account the individual neuromuscular defect, passive joint function, and bony deformities; different procedures such as muscle transpositions, arthrodeses, and corrective osteotomies can then be performed to improve function of the upper extremity. Each form of operative treatment presents patients with certain benefits and all are integrated into a total treatment plan for the affected extremity.

Use of botulinum toxin type A in the management of neonatal brachial plexus palsy.

PubMed

Michaud, Linda J; Louden, Emily J; Lippert, William C; Allgier, Allison J; Foad, Susan L; Mehlman, Charles T

2014-12-01

To evaluate functional outcomes and the impact on surgical interventions after the use of botulinum neurotoxin type A (BoNT-A) for muscle imbalance, cocontractions, or contractures with neonatal brachial plexus palsy. A retrospective cohort study. A brachial plexus center in a tertiary children's hospital. Fifty-nine patients with neonatal brachial plexus palsy (75 injection procedures, 91 muscles and/or muscle groups) received BoNT-A injections (mean age at injection, 36.2 months; range, 6-123 months; 31 boys; 30 right-sided injuries, 28 left-sided injuries, 1 bilateral injury). Data collected retrospectively from medical records, from procedure notes and clinic visits before BoNT-A use, at ≤6 months follow-up (BoNT-A active [BA]) and at ≥7 months follow-up (BoNT-A not active [BNA]) included demographics, injection indication, side, and site(s), previous surgical history, occupational therapy and/or physical therapy plan, and outcome measurements. Outcomes assessed before and after injections included active and passive range of motion, Mallet and Toronto scores, parent comments about arm function, preinjection surgical considerations, and postinjection surgical history. Injection procedures included 51 to shoulder internal rotators, 15 triceps, 15 pronator teres, 9 biceps, and 1 flexor carpi ulnaris. Active and passive shoulder external rotation (SER) range of motion improved after shoulder internal rotator injections (P = .0003 and P = .002, respectively), as did Mallet scores with BA; the latter were sustained with BNA. Surgical intervention was averted, modified, or deferred after BoNT-A in 45% (n = 20) under surgical consideration before BoNT-A. Active elbow flexion improved in 67% (P = .005), sustained BNA (P = .004) after triceps injections; 2 of 7 patients averted surgery. Active supination improved with BA (P = .002), with gains sustained BNA (P = .016). Passive elbow extension improved after biceps injections by an average 17° (P�

75 FR 35711 - Schedule for Rating Disabilities; Evaluation of Amyotrophic Lateral Sclerosis

Federal Register 2010, 2011, 2012, 2013, 2014

2010-06-23

... revising the evaluation criterion for amyotrophic lateral sclerosis (ALS) to provide a 100-percent evaluation for any veteran with service-connected ALS. This change is necessary to adequately compensate... provide a total disability rating for any veteran with service- connected ALS. DATES: Comments must be...

Retropharyngeal Contralateral C7 Nerve Transfer to the Lower Trunk for Brachial Plexus Birth Injury: Technique and Results.

PubMed

Vu, Anthony T; Sparkman, Darlene M; van Belle, Christopher J; Yakuboff, Kevin P; Schwentker, Ann R

2018-05-01

Brachial plexus birth injuries with multiple nerve root avulsions present a particularly difficult reconstructive challenge because of the limited availability of donor nerves. The contralateral C7 has been described for brachial plexus reconstruction in adults but has not been well-studied in the pediatric population. We present our technique and results for retropharyngeal contralateral C7 nerve transfer to the lower trunk for brachial plexus birth injury. We performed a retrospective review. Any child aged less than 2 years was included. Charts were analyzed for patient demographic data, operative variables, functional outcomes, complications, and length of follow-up. We had a total of 5 patients. Average nerve graft length was 3 cm. All patients had return of hand sensation to the ulnar nerve distribution as evidenced by a pinch test, unprompted use of the recipient limb without mirror movement, and an Active Movement Scale (AMS) of at least 2/7 for finger and thumb flexion; one patient had an AMS of 7/7 for finger and thumb flexion. Only one patient had return of ulnar intrinsic hand function with an AMS of 3/7. Two patients had temporary triceps weakness in the donor limb and one had clinically insignificant temporary phrenic nerve paresis. No complications were related to the retropharyngeal nerve dissection in any patient. Average follow-up was 3.3 years. The retropharyngeal contralateral C7 nerve transfer is a safe way to supply extra axons to the severely injured arm in brachial plexus birth injuries with no permanent donor limb deficits. Early functional recovery in these patients, with regard to hand function and sensation, is promising. Therapeutic V. Copyright © 2018 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

Quantifying Disease Progression in Amyotrophic Lateral Sclerosis

PubMed Central

Simon, Neil G; Turner, Martin R; Vucic, Steve; Al-Chalabi, Ammar; Shefner, Jeremy; Lomen-Hoerth, Catherine; Kiernan, Matthew C

2014-01-01

Amyotrophic lateral sclerosis (ALS) exhibits characteristic variability of onset and rate of disease progression, with inherent clinical heterogeneity making disease quantitation difficult. Recent advances in understanding pathogenic mechanisms linked to the development of ALS impose an increasing need to develop strategies to predict and more objectively measure disease progression. This review explores phenotypic and genetic determinants of disease progression in ALS, and examines established and evolving biomarkers that may contribute to robust measurement in longitudinal clinical studies. With targeted neuroprotective strategies on the horizon, developing efficiencies in clinical trial design may facilitate timely entry of novel treatments into the clinic. PMID:25223628

Amyotrophic lateral sclerosis and environmental factors

PubMed Central

Bozzoni, Virginia; Pansarasa, Orietta; Diamanti, Luca; Nosari, Guido; Cereda, Cristina; Ceroni, Mauro

2016-01-01

Summary Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder that affects central and peripheral motor neuron cells. Its etiology is unknown, although a relationship between genetic background and environmental factors may play a major role in triggering the neurodegeneration. In this review, we analyze the role of environmental factors in ALS: heavy metals, electromagnetic fields and electric shocks, pesticides, β-N-methylamino-L-alanine, physical activity and the controversial role of sports. The literature on the single issues is analyzed in an attempt to clarify, as clearly as possible, whether each risk factor significantly contributes to the disease pathogenesis. After summarizing conflicting observations and data, the authors provide a final synthetic statement. PMID:27027889

Airflow obstruction was associated with elevation of brachial-ankle pulse wave velocity but not ankle-brachial index in aged patients with chronic obstructive pulmonary disease.

PubMed

Chen, Rui; He, Wanbing; Zhang, Kun; Zheng, Houzhen; Lin, Lin; Nie, Ruqiong; Wang, Jingfeng; Huang, Hui

2015-09-01

Both brachial-ankle pulse wave velocity (baPWV) and ankle-brachial index (ABI) are important predictors for cardiovascular disease (CVD). Patients with chronic obstructive pulmonary disease (COPD) are at high risk of CVD. But the association between airflow obstruction and baPWV or ABI was still unclear. The study was aimed to investigate the influencing factors on arterial stiffness in aged COPD patients. 67 aged patients with COPD and 67 age- and sex-matched controls without COPD were enrolled in this study. COPD patients were grouped into four groups according to the Global Initiative for Chronic Obstructive Lung Disease Guidelines (GOLD). Both baPWV and ABI were evaluated. Spirometry indices, blood pressure, smoking history and related laboratory parameters were also collected. Comparing with controls, all COPD patients had significantly higher baPWV (1933 ± 355 cm/s versus 1515 ± 256 cm/s, P < 0.001) but not ABI (P = 0.196). And baPWV values were significantly highest at GOLD stage 4. Forced expiratory volume in 1 s (FEV1) was the most significant factor influencing baPWV, after adjusting for age, systolic blood pressure and other traditional cardiovascular risk factors (β = -0.463, P = 0.014). Arterial stiffness was serious in aged patients with COPD. Spirometry index FEV1 was a possible important predictor for the severity of arterial stiffness of COPD patients. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

High dietary sodium reduces brachial artery flow-mediated dilation in humans with salt-sensitive and salt-resistant blood pressure

PubMed Central

Matthews, Evan L.; Brian, Michael S.; Ramick, Meghan G.; Lennon-Edwards, Shannon; Edwards, David G.

2015-01-01

Recent studies demonstrate that high dietary sodium (HS) impairs endothelial function in those with salt-resistant (SR) blood pressure (BP). The effect of HS on endothelial function in those with salt-sensitive (SS) BP is not currently known. We hypothesized that HS would impair brachial artery flow-mediated dilation (FMD) to a greater extent in SS compared with SR adults. Ten SR (age 42 ± 5 yr, 5 men, 5 women) and 10 SS (age 39 ± 5 yr, 5 men, 5 women) healthy, normotensive participants were enrolled in a controlled feeding study consisting of a run-in diet followed by a 7-day low dietary sodium (LS) (20 mmol/day) and a 7-day HS (300 mmol/day) diet in random order. Brachial artery FMD and 24-h BP were assessed on the last day of each diet. SS BP was individually assessed and defined as a change in 24-h mean arterial pressure (MAP) of >5 mmHg between the LS and HS diets (ΔMAP: SR −0.6 ± 1.2, SS 7.7 ± 0.4 mmHg). Brachial artery FMD was lower in both SS and SR individuals during the HS diet (P < 0.001), and did not differ between groups (P > 0.05) (FMD: SR LS 10.6 ± 1.3%, SR HS 7.2 ± 1.5%, SS LS 12.5 ± 1.7%, SS HS 7.8 ± 1.4%). These data indicate that an HS diet impairs brachial artery FMD to a similar extent in adults with SS BP and SR BP. PMID:26078434

OCT/PS-OCT imaging of brachial plexus neurovascular structures

NASA Astrophysics Data System (ADS)

Raphael, David T.; Zhang, Jun; Zhang, Yaoping; Chen, Zhongping; Miller, Carol; Zhou, Li

2004-07-01

Introduction: Optical coherence tomography (OCT) allows high-resolution imaging (less than 10 microns) of tissue structures. A pilot study with OCT and polarization-sensitive OCT (PS-OCT) was undertaken to image ex-vivo neurovascular structures (vessels, nerves) of the canine brachial plexus. Methods: OCT is an interferometry-based optical analog of B-mode ultrasound, which can image through non-transparent biological tissues. With approval of the USC Animal Care and Use Committee, segments of the supra- and infraclavicular brachial plexus were excised from euthanized adult dogs, and the ex-vivo specimens were placed in cold pH-buffered physiologic solution. An OCT beam, in micrometer translational steps, scanned the fixed-position bisected specimens in transverse and longitudinal views. Two-dimensional images were obtained from identified arteries and nerves, with specific sections of interest stained with hematoxylin-eosin for later imaging through a surgical microscope. Results: with the beam scan direction transverse to arteries, the resulting OCT images showed an identifiable arterial lumen and arterial wall tissue layers. By comparison, transverse beam OCT images of nerves revealed a multitude of smaller nerve bundles contained within larger circular-shaped fascicles. PS-OCT imaging was helpful in showing the characteristic birefringence exhibited by arrayed neural structures. Discussion: High-resolution OCT imaging may be useful in the optical identification of neurovascular structures during attempted regional nerve blockade. If incorporated into a needle-shaped catheter endoscope, such a technology could prevent intraneural and intravascular injections immediately prior to local anesthetic injection. The major limitation of OCT is that it can form a coherent image of tissue structures only to a depth of 1.5 - 2 mm.

Speech Intelligibility and Marital Communication in Amyotrophic Lateral Sclerosis: An Exploratory Study

ERIC Educational Resources Information Center

Joubert, Karin; Bornman, Juan; Alant, Erna

2011-01-01

Amyotrophic lateral sclerosis (ALS), a rapidly progressive neuromuscular disease, has a devastating impact not only on individuals diagnosed with ALS but also their spouses. Speech intelligibility, often compromised as a result of dysarthria, affects the couple's ability to maintain effective, intimate communication. The purpose of this…

76 FR 78823 - Schedule for Rating Disabilities; Evaluation of Amyotrophic Lateral Sclerosis

Federal Register 2010, 2011, 2012, 2013, 2014

2011-12-20

... revising the disability evaluation criterion provided for amyotrophic lateral sclerosis (ALS) to provide an evaluation of 100 percent for any veteran with service-connected ALS. This change is necessary to adequately... to provide a total disability rating for any veteran with service-connected ALS. DATES: Effective...

Metabolomics in amyotrophic lateral sclerosis: how far can it take us?

PubMed

Blasco, H; Patin, F; Madji Hounoum, B; Gordon, P H; Vourc'h, P; Andres, C R; Corcia, P

2016-03-01

Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease. Alongside identification of aetiologies, development of biomarkers is a foremost research priority. Metabolomics is one promising approach that is being utilized in the search for diagnosis and prognosis markers. Our aim is to provide an overview of the principal research in metabolomics applied to ALS. References were identified using PubMed with the terms 'metabolomics' or 'metabolomic' and 'ALS' or 'amyotrophic lateral sclerosis' or 'MND' or 'motor neuron disorders'. To date, nine articles have reported metabolomics research in patients and a few additional studies examined disease physiology and drug effects in patients or models. Metabolomics contribute to a better understanding of ALS pathophysiology but, to date, no biomarker has been validated for diagnosis, principally due to the heterogeneity of the disease and the absence of applied standardized methodology for biomarker discovery. A consensus on best metabolomics methodology as well as systematic independent validation will be an important accomplishment on the path to identifying the long-awaited biomarkers for ALS and to improve clinical trial designs. © 2016 EAN.

Safety, tolerability, and cerebrospinal fluid penetration of ursodeoxycholic Acid in patients with amyotrophic lateral sclerosis.

PubMed

Parry, Gareth J; Rodrigues, Cecilia M P; Aranha, Marcia M; Hilbert, Sarah J; Davey, Cynthia; Kelkar, Praful; Low, Walter C; Steer, Clifford J

2010-01-01

Amyotrophic lateral sclerosis is a progressive degenerative disease, which typically leads to death in 3 to 5 years. Neuronal cell death offers a potential target for therapeutic intervention. Ursodeoxycholic acid is a cytoprotective, endogenous bile acid that has been shown to be neuroprotective in experimental Huntington and Alzheimer diseases, retinal degeneration, and ischemic and hemorrhagic stroke. The objective of this research was to study the safety and the tolerability of ursodeoxycholic acid in amyotrophic lateral sclerosis and document effective and dose-dependent cerebrospinal fluid penetration. Eighteen patients were randomly assigned to receive ursodeoxycholic acid at doses of 15, 30, and 50 mg/kg of body weight per day. Serum and cerebrospinal fluid were obtained for analysis after 4 weeks of treatment. Treatment-emergent clinical and laboratory events were monitored weekly. Our data indicated that ursodeoxycholic acid is well tolerated by all subjects at all doses. We also showed that ursodeoxycholic acid is well absorbed after oral administration and crosses the blood-brain barrier in a dose-dependent manner. These results show excellent safety and tolerability of ursodeoxycholic acid. The drug penetrates the cerebrospinal fluid in a dose-dependent manner. A large, placebo-controlled clinical trial is needed to assess the efficacy of ursodeoxycholic acid in treating amyotrophic lateral sclerosis.

Association Between Dietary Intake and Function in Amyotrophic Lateral Sclerosis.

PubMed

Nieves, Jeri W; Gennings, Chris; Factor-Litvak, Pam; Hupf, Jonathan; Singleton, Jessica; Sharf, Valerie; Oskarsson, Björn; Fernandes Filho, J Americo M; Sorenson, Eric J; D'Amico, Emanuele; Goetz, Ray; Mitsumoto, Hiroshi

2016-12-01

There is growing interest in the role of nutrition in the pathogenesis and progression of amyotrophic lateral sclerosis (ALS). To evaluate the associations between nutrients, individually and in groups, and ALS function and respiratory function at diagnosis. A cross-sectional baseline analysis of the Amyotrophic Lateral Sclerosis Multicenter Cohort Study of Oxidative Stress study was conducted from March 14, 2008, to February 27, 2013, at 16 ALS clinics throughout the United States among 302 patients with ALS symptom duration of 18 months or less. Nutrient intake, measured using a modified Block Food Frequency Questionnaire (FFQ). Amyotrophic lateral sclerosis function, measured using the ALS Functional Rating Scale-Revised (ALSFRS-R), and respiratory function, measured using percentage of predicted forced vital capacity (FVC). Baseline data were available on 302 patients with ALS (median age, 63.2 years [interquartile range, 55.5-68.0 years]; 178 men and 124 women). Regression analysis of nutrients found that higher intakes of antioxidants and carotenes from vegetables were associated with higher ALSFRS-R scores or percentage FVC. Empirically weighted indices using the weighted quantile sum regression method of "good" micronutrients and "good" food groups were positively associated with ALSFRS-R scores (β [SE], 2.7 [0.69] and 2.9 [0.9], respectively) and percentage FVC (β [SE], 12.1 [2.8] and 11.5 [3.4], respectively) (all P < .001). Positive and significant associations with ALSFRS-R scores (β [SE], 1.5 [0.61]; P = .02) and percentage FVC (β [SE], 5.2 [2.2]; P = .02) for selected vitamins were found in exploratory analyses. Antioxidants, carotenes, fruits, and vegetables were associated with higher ALS function at baseline by regression of nutrient indices and weighted quantile sum regression analysis. We also demonstrated the usefulness of the weighted quantile sum regression method in the evaluation of diet. Those responsible for nutritional

Bipolar Transfer of Latissimus Dorsi Myocutaneous Flap for Restoration of Elbow Flexion in Late Traumatic Brachial Plexus Injury: Evaluation of 13 Cases.

PubMed

Azab, Ahmed Abo-Hashem; Alsabbahi, Mohammad Salah

2017-02-01

Restoration of elbow flexion following traumatic brachial plexus injury represents a great challenge to the reconstructive surgeons. Functional muscle transfers come next to the sophisticated types of nerve surgery in this area. Many transfers are well known for restoration of elbow flexion; bipolar or unipolar latissimus dorsi, triceps brachii, sternocleidomastoid, pectoralis major, and Steindler flexorplasty. Evaluation of the outcome of bipolar transfer of latissimus dorsi myocutaneous flap when used to restore elbow flexion in late traumatic brachial plexus injury. Thirteen patients were included in this case series with careful evaluation both preoperatively and postoperatively both clinically and using electromyography. Almost 84.6% of patients (11 of 13) developed G3-4 on the Medical Research Council grading with relatively minimal both donor-site and recipient-site morbidity. Bipolar transfer of latissimus dorsi myocutaneous flap is a reliable method for restoration of elbow flexion in patients suffering from late sequelae of traumatic brachial plexus injury.

Use of the DEKA Arm for amputees with brachial plexus injury: A case series

PubMed Central

Fantini, Christopher; Latlief, Gail; Phillips, Samuel; Sasson, Nicole; Sepulveda, Eve

2017-01-01

Objective Patients with upper limb amputation and brachial plexus injuries have high rates of prosthesis rejection. Study purpose is to describe experiences of subjects with transhumeral amputation and brachial plexus injury, who were fit with, and trained to use, a DEKA Arm. Methods This was a mixed-methods study utilizing qualitative (e.g. interview, survey) and quantitative data (e.g. self-report and performance measures). Subject 1, a current prosthesis user, had a shoulder arthrodesis. Subject 2, not a prosthesis user, had a subluxed shoulder. Both were trained in laboratory and participated in a trial of home use. Descriptive analyses of processes and outcomes were conducted. Results Subject 1 was fitted with the transhumeral configuration (HC) DEKA Arm using a compression release stabilized socket. He had 12 hours of prosthetic training and participated in all home study activities. Subject 1 had improved dexterity and prosthetic satisfaction with the DEKA Arm and reported better quality of life (QOL) at the end of participation. Subject 2 was fit with the shoulder configuration (SC) DEKA Arm using a modified X-frame socket. He had 30 hours of training and participated in 3 weeks of home activities. He reported less functional disability at the end of training as compared to baseline, but encountered personal problems and exacerbation of PTSD symptoms and withdrew from home use portion at 3 weeks. Both subjects reported functional benefits from use, and expressed a desire to receive a DEKA Arm in the future. Discussion This paper reported on two different strategies for prosthetic fitting and their outcomes. The advantages and limitations of each approach were discussed. Conclusion Use of both the HC and SC DEKA Arm for patients with TH amputation and brachial plexus injury was reported. Lessons learned may be instructive to clinicians considering prosthetic choices for future cases. PMID:28628623

Use of the DEKA Arm for amputees with brachial plexus injury: A case series.

PubMed

Resnik, Linda; Fantini, Christopher; Latlief, Gail; Phillips, Samuel; Sasson, Nicole; Sepulveda, Eve

2017-01-01

Patients with upper limb amputation and brachial plexus injuries have high rates of prosthesis rejection. Study purpose is to describe experiences of subjects with transhumeral amputation and brachial plexus injury, who were fit with, and trained to use, a DEKA Arm. This was a mixed-methods study utilizing qualitative (e.g. interview, survey) and quantitative data (e.g. self-report and performance measures). Subject 1, a current prosthesis user, had a shoulder arthrodesis. Subject 2, not a prosthesis user, had a subluxed shoulder. Both were trained in laboratory and participated in a trial of home use. Descriptive analyses of processes and outcomes were conducted. Subject 1 was fitted with the transhumeral configuration (HC) DEKA Arm using a compression release stabilized socket. He had 12 hours of prosthetic training and participated in all home study activities. Subject 1 had improved dexterity and prosthetic satisfaction with the DEKA Arm and reported better quality of life (QOL) at the end of participation. Subject 2 was fit with the shoulder configuration (SC) DEKA Arm using a modified X-frame socket. He had 30 hours of training and participated in 3 weeks of home activities. He reported less functional disability at the end of training as compared to baseline, but encountered personal problems and exacerbation of PTSD symptoms and withdrew from home use portion at 3 weeks. Both subjects reported functional benefits from use, and expressed a desire to receive a DEKA Arm in the future. This paper reported on two different strategies for prosthetic fitting and their outcomes. The advantages and limitations of each approach were discussed. Use of both the HC and SC DEKA Arm for patients with TH amputation and brachial plexus injury was reported. Lessons learned may be instructive to clinicians considering prosthetic choices for future cases.

78 FR 72573 - Specially Adapted Housing Eligibility for Amyotrophic Lateral Sclerosis Beneficiaries

Federal Register 2010, 2011, 2012, 2013, 2014

2013-12-03

... amyotrophic lateral sclerosis (ALS) rated totally disabling under the VA Schedule for Rating Disabilities. The...-connected ALS. VA previously amended its Schedule for Rating Disabilities to assign a 100-percent disability evaluation for any veteran who has service-connected ALS based on the recognition that ALS is a rapidly...

A PET/CT approach to spinal cord metabolism in amyotrophic lateral sclerosis.

PubMed

Marini, Cecilia; Cistaro, Angelina; Campi, Cristina; Calvo, Andrea; Caponnetto, Claudia; Nobili, Flavio Mariano; Fania, Piercarlo; Beltrametti, Mauro C; Moglia, Cristina; Novi, Giovanni; Buschiazzo, Ambra; Perasso, Annalisa; Canosa, Antonio; Scialò, Carlo; Pomposelli, Elena; Massone, Anna Maria; Bagnara, Maria Caludia; Cammarosano, Stefania; Bruzzi, Paolo; Morbelli, Silvia; Sambuceti, Gianmario; Mancardi, Gianluigi; Piana, Michele; Chiò, Adriano

2016-10-01

In amyotrophic lateral sclerosis, functional alterations within the brain have been intensively assessed, while progression of lower motor neuron damage has scarcely been defined. The aim of the present study was to develop a computational method to systematically evaluate spinal cord metabolism as a tool to monitor disease mechanisms. A new computational three-dimensional method to extract the spinal cord from (18)F-FDG PET/CT images was evaluated in 30 patients with spinal onset amyotrophic lateral sclerosis and 30 controls. The algorithm identified the skeleton on the CT images by using an extension of the Hough transform and then extracted the spinal canal and the spinal cord. In these regions, (18)F-FDG standardized uptake values were measured to estimate the metabolic activity of the spinal canal and cord. Measurements were performed in the cervical and dorsal spine and normalized to the corresponding value in the liver. Uptake of (18)F-FDG in the spinal cord was significantly higher in patients than in controls (p < 0.05). By contrast, no significant differences were observed in spinal cord and spinal canal volumes between the two groups. (18)F-FDG uptake was completely independent of age, gender, degree of functional impairment, disease duration and riluzole treatment. Kaplan-Meier analysis showed a higher mortality rate in patients with standardized uptake values above the fifth decile at the 3-year follow-up evaluation (log-rank test, p < 0.01). The independence of this value was confirmed by multivariate Cox analysis. Our computational three-dimensional method enabled the evaluation of spinal cord metabolism and volume and might represent a potential new window onto the pathophysiology of amyotrophic lateral sclerosis.

Proximal Hypospadias

PubMed Central

Kraft, Kate H.; Shukla, Aseem R.; Canning, Douglas A.

2011-01-01

Hypospadias results from abnormal development of the penis that leaves the urethral meatus proximal to its normal glanular position. Meatal position may be located anywhere along the penile shaft, but more severe forms of hypospadias may have a urethral meatus located at the scrotum or perineum. The spectrum of abnormalities may also include ventral curvature of the penis, a dorsally redundant prepuce, and atrophic corpus spongiosum. Due to the severity of these abnormalities, proximal hypospadias often requires more extensive reconstruction in order to achieve an anatomically and functionally successful result. We review the spectrum of proximal hypospadias etiology, presentation, correction, and possible associated complications. PMID:21516286

Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons.

PubMed

Gopal, Pallavi P; Nirschl, Jeffrey J; Klinman, Eva; Holzbaur, Erika L F

2017-03-21

Ribonucleoprotein (RNP) granules are enriched in specific RNAs and RNA-binding proteins (RBPs) and mediate critical cellular processes. Purified RBPs form liquid droplets in vitro through liquid-liquid phase separation and liquid-like non-membrane-bound structures in cells. Mutations in the human RBPs TAR-DNA binding protein 43 (TDP-43) and RNA-binding protein FUS cause amyotrophic lateral sclerosis (ALS), but the biophysical properties of these proteins have not yet been studied in neurons. Here, we show that TDP-43 RNP granules in axons of rodent primary cortical neurons display liquid-like properties, including fusion with rapid relaxation to circular shape, shear stress-induced deformation, and rapid fluorescence recovery after photobleaching. RNP granules formed from wild-type TDP-43 show distinct biophysical properties depending on axonal location, suggesting maturation to a more stabilized structure is dependent on subcellular context, including local density and aging. Superresolution microscopy demonstrates that the stabilized population of TDP-43 RNP granules in the proximal axon is less circular and shows spiculated edges, whereas more distal granules are both more spherical and more dynamic. RNP granules formed by ALS-linked mutant TDP-43 are more viscous and exhibit disrupted transport dynamics. We propose these altered properties may confer toxic gain of function and reflect differential propensity for pathological transformation.

Feasibility and Safety of Distal and Proximal Combined Endovascular Approach with a Balloon-Guiding Catheter for Subclavian Artery Total Occlusion.

PubMed

Yamamoto, Taiki; Ohshima, Tomotaka; Ishikawa, Kojiro; Goto, Shunsaku; Tamari, Yosuke

2017-04-01

Symptomatic subclavian artery total occlusion is widely treated with an endovascular procedure that often results in distal vertebral artery embolism. Therefore, protection devices are important. Establishing a filter or balloon device in the vertebral artery can protect against this distal embolism. However, the use of embolic protection devices is not easy, and it makes the procedure more complicated. Here, we report a case of symptomatic subclavian artery total occlusion that was treated successfully with a balloon-guiding catheter and the pull-through technique. A 67-year-old man developed intermittent motor weakness in his left arm. Aortic angiography demonstrated a complete occlusion of the left proximal subclavian artery and a retrograde flow through the left vertebral artery to the distal brachial artery. In this case, we used a balloon-guiding catheter and the pull-through technique to prevent distal embolism. The balloon-guiding system was useful not only for embolic protection but also for scaffold during excavation and for the centering effect against invisible vessels. The pull-through technique enabled our devices to deliver easily and smoothly. The patient was treated successfully without complications. The distal and proximal combined endovascular treatment with a transbrachial balloon-guiding catheter is a beneficial treatment option for patients with subclavian artery total occlusion. Copyright © 2017 Elsevier Inc. All rights reserved.

Psychometric Evaluation of the Brachial Assessment Tool Part 1: Reproducibility.

PubMed

Hill, Bridget; Williams, Gavin; Olver, John; Ferris, Scott; Bialocerkowski, Andrea

2018-04-01

To evaluate reproducibility (reliability and agreement) of the Brachial Assessment Tool (BrAT), a new patient-reported outcome measure for adults with traumatic brachial plexus injury (BPI). Prospective repeated-measure design. Outpatient clinics. Adults with confirmed traumatic BPI (N=43; age range, 19-82y). People with BPI completed the 31-item 4-response BrAT twice, 2 weeks apart. Results for the 3 subscales and summed score were compared at time 1 and time 2 to determine reliability, including systematic differences using paired t tests, test retest using intraclass correlation coefficient model 1,1 (ICC 1,1 ), and internal consistency using Cronbach α. Agreement parameters included standard error of measurement, minimal detectable change, and limits of agreement. BrAT. Test-retest reliability was excellent (ICC 1,1 =.90-.97). Internal consistency was high (Cronbach α=.90-.98). Measurement error was relatively low (standard error of measurement range, 3.1-8.8). A change of >4 for subscale 1, >6 for subscale 2, >4 for subscale 3, and >10 for the summed score is indicative of change over and above measurement error. Limits of agreement ranged from ±4.4 (subscale 3) to 11.61 (summed score). These findings support the use of the BrAT as a reproducible patient-reported outcome measure for adults with traumatic BPI with evidence of appropriate reliability and agreement for both individual and group comparisons. Further psychometric testing is required to establish the construct validity and responsiveness of the BrAT. Copyright © 2017 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

Brugada syndrome in a patient with amyotrophic lateral sclerosis: a case report.

PubMed

Battineni, Anusha; Gummi, Rohit; Mullaguri, Naresh; Govindarajan, Raghav

2017-07-14

Amyotrophic lateral sclerosis is a fatal neuromuscular disorder characterized by progressive death of the upper and lower motor neurons in the central nervous system. Patients with this disease die mostly as a result of respiratory failure; however, owing to prolonged survival through assisted ventilation, cardiovascular causes are increasingly responsible for mortality. We report what is to the best of our knowledge the first case of type 2 Brugada syndrome causing ventricular tachyarrhythmia and cardiac arrest in a patient with upper limb onset amyotrophic lateral sclerosis. A 48-year-old Caucasian woman with a significant past medical history of papillary thyroid carcinoma status postresection, pulmonary embolism on anticoagulation, and a recent diagnosis of right upper limb-onset amyotrophic lateral sclerosis presented to the emergency department of our hospital with acute on chronic shortness of breath. On further evaluation, she was found to have hypoxic and hypercapnic respiratory failure and was placed on bilevel positive airway pressure ventilation. Her 12-lead electrocardiogram showed sinus rhythm with J-point elevation, saddle-shaped ST segment elevation, predominantly in V1 and V2 with no significant QTc prolongation. No troponin elevation was noted in her laboratory workup. Because she was unable to protect her airway, a decision was made to intubate her. After 1 minute of induction with etomidate and succinylcholine, she went into pulseless ventricular tachycardia and fibrillation requiring three cycles of cardiopulmonary resuscitation with high-quality chest compressions, three doses of epinephrine, and a loading dose of amiodarone prior to return of spontaneous circulation. She was further evaluated by cardiology services and was diagnosed with type 2 Brugada syndrome, for which she was started on quinidine. Her respiratory failure and the drugs she received for intubation likely caused her ventricular tachycardia to occur in conjunction with an

Characteristics of Speaking Rate in the Dysarthria Associated with Amyotrophic Lateral Sclerosis.

ERIC Educational Resources Information Center

Turner, Greg S.; Weismer, Gary

1993-01-01

The ability to alter speaking rate was studied in nine adult subjects with amyotrophic lateral sclerosis and nine control subjects. Results suggest that the relationship between speaking rate, articulation rate, pause duration, and pause frequency remained largely intact for the dysarthric speakers. Data showed greater dependence on pausing by the…

Speech Deterioration in Amyotrophic Lateral Sclerosis (ALS) after Manifestation of Bulbar Symptoms

ERIC Educational Resources Information Center

Makkonen, Tanja; Ruottinen, Hanna; Puhto, Riitta; Helminen, Mika; Palmio, Johanna

2018-01-01

Background: The symptoms and their progression in amyotrophic lateral sclerosis (ALS) are typically studied after the diagnosis has been confirmed. However, many people with ALS already have severe dysarthria and loss of adequate speech at the time of diagnosis. Speech-and-language therapy interventions should be targeted timely based on…

Clinical and neuropathological study about the neurotization of the suprascapular nerve in obstetric brachial plexus lesions

PubMed Central

2009-01-01

Background The lack of recovery of active external rotation of the shoulder is an important problem in children suffering from brachial plexus lesions involving the suprascapular nerve. The accessory nerve neurotization to the suprascapular nerve is a standard procedure, performed to improve shoulder motion in patients with brachial plexus palsy. Methods We operated on 65 patients with obstetric brachial plexus palsy (OBPP), aged 5-35 months (average: 19 months). We assessed the recovery of passive and active external rotation with the arm in abduction and in adduction. We also looked at the influence of the restoration of the muscular balance between the internal and the external rotators on the development of a gleno-humeral joint dysplasia. Intraoperatively, suprascapular nerve samples were taken from 13 patients and were analyzed histologically. Results Most patients (71.5%) showed good recovery of the active external rotation in abduction (60°-90°). Better results were obtained for the external rotation with the arm in abduction compared to adduction, and for patients having only undergone the neurotization procedure compared to patients having had complete plexus reconstruction. The neurotization operation has a positive influence on the glenohumeral joint: 7 patients with clinical signs of dysplasia before the reconstructive operation did not show any sign of dysplasia in the postoperative follow-up. Conclusion The neurotization procedure helps to recover the active external rotation in the shoulder joint and has a good prevention influence on the dysplasia in our sample. The nerve quality measured using histopathology also seems to have a positive impact on the clinical results. PMID:19744351

Tendon transfer options about the shoulder in patients with brachial plexus injury.

PubMed

Elhassan, Bassem; Bishop, Allen T; Hartzler, Robert U; Shin, Alexander Y; Spinner, Robert J

2012-08-01

The purpose of this study was to evaluate the early outcome of shoulder tendon transfer in patients with brachial plexus injury and to determine the factors associated with favorable outcomes. Fifty-two patients with traumatic brachial plexus injury and a paralytic shoulder were included in the study. All patients were evaluated at a mean of nineteen months (range, twelve to twenty-eight months) postoperatively. Twelve patients had a C5-6 injury, twenty-two had a C5-7 injury, five had a C5-8 injury, and thirteen had a C5-T1 injury. Transfer of the lower portion of the trapezius muscle was performed either in isolation or as part of multiple tendon transfers to improve shoulder function. Additional muscles transferred included the middle and upper portions of the trapezius, levator scapulae, upper portion of the serratus anterior, teres major, latissimus dorsi, and pectoralis major. All patients had a stable shoulder postoperatively. Shoulder external rotation improved substantially in all patients from no external rotation (hand-on-belly position) to a mean of 20° (p = 0.001). Patients who underwent additional transfers had marginal improvement of shoulder flexion, from a mean of 10° preoperatively to 60° postoperatively, and of shoulder abduction, from a mean of 10° to 50° (p = 0.01 for each). Mean pain on a visual analog scale improved from 6 points preoperatively to 2 points postoperatively. The mean Disabilities of the Arm, Shoulder and Hand (DASH) score improved from 59 to 47 points (p = 0.001). The mean Subjective Shoulder Value improved from 5% to 40% (p = 0.001). Greater age, higher body mass index, and more extensive nerve injury were associated with a poorer DASH score in a multivariate analysis (p = 0.003). Tendon transfers about the shoulder can improve shoulder function in patients with brachial plexus injury resulting in a paralytic shoulder. Significant improvement of shoulder external rotation but only marginal improvements of shoulder

Local infiltration analgesia versus continuous interscalene brachial plexus block for shoulder replacement pain: a randomized clinical trial.

PubMed

Bjørnholdt, Karen T; Jensen, Jan M; Bendtsen, Thomas F; Søballe, Kjeld; Nikolajsen, Lone

2015-12-01

Shoulder replacement involves significant post-operative pain, which is often managed by continuous interscalene brachial plexus block. Catheter displacement and complications limit the beneficial effect of the block. Local infiltration analgesia (LIA) has provided good results in knee replacement. We aimed to assess the effectiveness of LIA for pain after shoulder replacement. Patients scheduled for primary shoulder replacement under general anaesthesia were randomized to receive either local infiltration analgesia (LIA) (150 ml ropivacaine 0.2 % with epinephrine intra-operatively) or interscalene brachial plexus catheter (ISC) (ropivacaine 0.75 %, 7 ml bolus followed by 48-h 5 ml/h infusion). The primary outcome was opioid consumption during the first 24 post-operative hours. Secondary outcomes were pain ratings, supplementary analgesics, and side effects for 3 days, and complications until 3 months after surgery. Data were analysed for 61 patients (LIA 30, ISC 31). Twenty-four-hour opioid consumption was higher in the LIA group compared with the ISC group: median (IQR) 95 mg (70-150 mg) versus 40 mg (8-76 mg) (P = 0.0001). No significant difference in opioid consumption was found between groups during the following 3 days. The LIA group had higher pain scores at 0, 2, 4, and 8 h. Two patients in the ISC group had long-lasting complications. The LIA technique cannot be recommended for shoulder replacement unless substantially modified. Occurrence of inadequate analgesia and complications following interscalene brachial plexus block prompt further studies into pain management after shoulder replacement.

Lingual-Alveolar Contact Pressure during Speech in Amyotrophic Lateral Sclerosis: Preliminary Findings

ERIC Educational Resources Information Center

Searl, Jeff; Knollhoff, Stephanie; Barohn, Richard J.

2017-01-01

Purpose: This preliminary study on lingual-alveolar contact pressures (LACP) in people with amyotrophic lateral sclerosis (ALS) had several aims: (a) to evaluate whether the protocol induced fatigue, (b) to compare LACP during speech (LACP-Sp) and during maximum isometric pressing (LACP-Max) in people with ALS (PALS) versus healthy controls, (c)…

Comparing the Efficacy of Triple Nerve Transfers with Nerve Graft Reconstruction in Upper Trunk Obstetric Brachial Plexus Injury.

PubMed

O'Grady, Kathleen M; Power, Hollie A; Olson, Jaret L; Morhart, Michael J; Harrop, A Robertson; Watt, M Joe; Chan, K Ming

2017-10-01

Upper trunk obstetric brachial plexus injury can cause profound shoulder and elbow dysfunction. Although neuroma excision with interpositional sural nerve grafting is the current gold standard, distal nerve transfers have a number of potential advantages. The goal of this study was to compare the clinical outcomes and health care costs between nerve grafting and distal nerve transfers in children with upper trunk obstetric brachial plexus injury. In this prospective cohort study, children who underwent triple nerve transfers were followed with the Active Movement Scale for 2 years. Their outcomes were compared to those of children who underwent nerve graft reconstruction. To assess health care use, a cost analysis was also performed. Twelve patients who underwent nerve grafting were compared to 14 patients who underwent triple nerve transfers. Both groups had similar baseline characteristics and showed improved shoulder and elbow function following surgery. However, the nerve transfer group displayed significantly greater improvement in shoulder external rotation and forearm supination 2 years after surgery (p < 0.05). The operative time and length of hospital stay were significantly lower (p < 0.05), and the overall cost was approximately 50 percent less in the nerve transfer group. Triple nerve transfer for upper trunk obstetric brachial plexus injury is a feasible option, with better functional shoulder external rotation and forearm supination, faster recovery, and lower cost compared with traditional nerve graft reconstruction. Therapeutic, II.

Brachial artery responses to ambient pollution, temperature, and humidity in people with type 2 diabetes: a repeated-measures study.

PubMed

Zanobetti, Antonella; Luttmann-Gibson, Heike; Horton, Edward S; Cohen, Allison; Coull, Brent A; Hoffmann, Barbara; Schwartz, Joel D; Mittleman, Murray A; Li, Yongsheng; Stone, Peter H; de Souza, Celine; Lamparello, Brooke; Koutrakis, Petros; Gold, Diane R

2014-03-01

Extreme weather and air pollution are associated with increased cardiovascular risk in people with diabetes. In a population with diabetes, we conducted a novel assessment of vascular brachial artery responses both to ambient pollution and to weather (temperature and water vapor pressure, a measure of humidity). Sixty-four 49- to 85-year-old Boston residents with type 2 diabetes completed up to five study visits (279 repeated measures). Brachial artery diameter (BAD) was measured by ultrasound before and after brachial artery occlusion [i.e., flow-mediated dilation (FMD)] and before and after nitroglycerin-mediated dilation (NMD). Ambient concentrations of fine particulate mass (PM2.5), black carbon (BC), organic carbon (OC), elemental carbon, particle number, and sulfate were measured at our monitoring site; ambient concentrations of carbon monoxide, nitrogen dioxide, and ozone were obtained from state monitors. Particle exposure in the home and during each trip to the clinic (home/trip exposure) was measured continuously and as a 5-day integrated sample. We used linear models with fixed effects for participants, adjusting for date, season, temperature, and water vapor pressure on the day of each visit, to estimate associations between our outcomes and interquartile range increases in exposure. Baseline BAD was negatively associated with particle pollution, including home/trip-integrated BC (-0.02 mm; 95% CI: -0.04, -0.003, for a 0.28 μg/m3 increase in BC), OC (-0.08 mm; 95% CI: -0.14, -0.03, for a 1.61 μg/m3 increase) as well as PM2.5, 5-day average ambient PM2.5, and BC. BAD was positively associated with ambient temperature and water vapor pressure. However, exposures were not consistently associated with FMD or NMD. Brachial artery diameter, a predictor of cardiovascular risk, decreased in association with particle pollution and increased in association with ambient temperature in our study population of adults with type 2 diabetes. Zanobetti A, Luttmann

Brachial Artery Responses to Ambient Pollution, Temperature, and Humidity in People with Type 2 Diabetes: A Repeated-Measures Study

PubMed Central

Luttmann-Gibson, Heike; Horton, Edward S.; Cohen, Allison; Coull, Brent A.; Hoffmann, Barbara; Schwartz, Joel D.; Mittleman, Murray A.; Li, Yongsheng; Stone, Peter H.; de Souza, Celine; Lamparello, Brooke; Koutrakis, Petros; Gold, Diane R.

2014-01-01

Background: Extreme weather and air pollution are associated with increased cardiovascular risk in people with diabetes. Objectives: In a population with diabetes, we conducted a novel assessment of vascular brachial artery responses both to ambient pollution and to weather (temperature and water vapor pressure, a measure of humidity). Methods: Sixty-four 49- to 85-year-old Boston residents with type 2 diabetes completed up to five study visits (279 repeated measures). Brachial artery diameter (BAD) was measured by ultrasound before and after brachial artery occlusion [i.e., flow-mediated dilation (FMD)] and before and after nitroglycerin-mediated dilation (NMD). Ambient concentrations of fine particulate mass (PM2.5), black carbon (BC), organic carbon (OC), elemental carbon, particle number, and sulfate were measured at our monitoring site; ambient concentrations of carbon monoxide, nitrogen dioxide, and ozone were obtained from state monitors. Particle exposure in the home and during each trip to the clinic (home/trip exposure) was measured continuously and as a 5-day integrated sample. We used linear models with fixed effects for participants, adjusting for date, season, temperature, and water vapor pressure on the day of each visit, to estimate associations between our outcomes and interquartile range increases in exposure. Results: Baseline BAD was negatively associated with particle pollution, including home/trip–integrated BC (–0.02 mm; 95% CI: –0.04, –0.003, for a 0.28 μg/m3 increase in BC), OC (–0.08 mm; 95% CI: –0.14, –0.03, for a 1.61 μg/m3 increase) as well as PM2.5, 5-day average ambient PM2.5, and BC. BAD was positively associated with ambient temperature and water vapor pressure. However, exposures were not consistently associated with FMD or NMD. Conclusion: Brachial artery diameter, a predictor of cardiovascular risk, decreased in association with particle pollution and increased in association with ambient temperature in our study

The TRK-fused gene is mutated in hereditary motor and sensory neuropathy with proximal dominant involvement.

PubMed

Ishiura, Hiroyuki; Sako, Wataru; Yoshida, Mari; Kawarai, Toshitaka; Tanabe, Osamu; Goto, Jun; Takahashi, Yuji; Date, Hidetoshi; Mitsui, Jun; Ahsan, Budrul; Ichikawa, Yaeko; Iwata, Atsushi; Yoshino, Hiide; Izumi, Yuishin; Fujita, Koji; Maeda, Kouji; Goto, Satoshi; Koizumi, Hidetaka; Morigaki, Ryoma; Ikemura, Masako; Yamauchi, Naoko; Murayama, Shigeo; Nicholson, Garth A; Ito, Hidefumi; Sobue, Gen; Nakagawa, Masanori; Kaji, Ryuji; Tsuji, Shoji

2012-08-10

Hereditary motor and sensory neuropathy with proximal dominant involvement (HMSN-P) is an autosomal-dominant neurodegenerative disorder characterized by widespread fasciculations, proximal-predominant muscle weakness, and atrophy followed by distal sensory involvement. To date, large families affected by HMSN-P have been reported from two different regions in Japan. Linkage and haplotype analyses of two previously reported families and two new families with the use of high-density SNP arrays further defined the minimum candidate region of 3.3 Mb in chromosomal region 3q12. Exome sequencing showed an identical c.854C>T (p.Pro285Leu) mutation in the TRK-fused gene (TFG) in the four families. Detailed haplotype analysis suggested two independent origins of the mutation. Pathological studies of an autopsied patient revealed TFG- and ubiquitin-immunopositive cytoplasmic inclusions in the spinal and cortical motor neurons. Fragmentation of the Golgi apparatus, a frequent finding in amyotrophic lateral sclerosis, was also observed in the motor neurons with inclusion bodies. Moreover, TAR DNA-binding protein 43 kDa (TDP-43)-positive cytoplasmic inclusions were also demonstrated. In cultured cells expressing mutant TFG, cytoplasmic aggregation of TDP-43 was demonstrated. These findings indicate that formation of TFG-containing cytoplasmic inclusions and concomitant mislocalization of TDP-43 underlie motor neuron degeneration in HMSN-P. Pathological overlap of proteinopathies involving TFG and TDP-43 highlights a new pathway leading to motor neuron degeneration. Copyright © 2012 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

Against All Odds: Positive Life Experiences of People with Advanced Amyotrophic Lateral Sclerosis.

ERIC Educational Resources Information Center

Young, Jenny M.; McNicoll, Paule

1998-01-01

Describes the nature of positive life experiences of 13 people coping exceptionally well while living with advanced amyotrophic lateral sclerosis (ALS), or Lou Gehrig's, disease and the resulting significant physical disabilities. Emerging themes were the use of cognitive reappraisal, reframing, and intellectual stimulation as coping mechanisms;…

Speech Movement Measures as Markers of Bulbar Disease in Amyotrophic Lateral Sclerosis

ERIC Educational Resources Information Center

Shellikeri, Sanjana; Green, Jordan R.; Kulkarni, Madhura; Rong, Panying; Martino, Rosemary; Zinman, Lorne; Yunusova, Yana

2016-01-01

Purpose: The goal of this study was to identify the effects of amyotrophic lateral sclerosis (ALS) on tongue and jaw control, both cross-sectionally and longitudinally. The data were examined in the context of their utility as a diagnostic marker of bulbar disease. Method: Tongue and jaw movements were recorded cross-sectionally (n = 33…

Application of botulinum toxin to treat sialorrhea in amyotrophic lateral sclerosis patients: a literature review.

PubMed

Oliveira, Ademar Francisco de; Silva, Gêssyca Adryene de Menezes; Almeida, Débora Milenna Xavier

2016-01-01

Amyotrophic lateral sclerosis is a progressive and fatal neurodegenerative disease characterized by the degeneration of motor neurons, which are the central nervous system cells that control voluntary muscle movements. The excessive salivation (sialorrhea) is present in approximately 50% of amyotrophic lateral sclerosis cases. Thus, some alternative therapeutic methods are sought, such as anticholinergic drugs and surgery. Recently the use of botulinum toxin applied at a midpoint of the salivary glands, often guided by ultrasound, have demonstrated positive results. The objective was to review the literature to demonstrate an alternative method to treatments of sialorrhea in patients with amyotrophic lateral sclerosis. In recent studies, the efficacy of botulinum toxin is confirmed, although new applications are required. Since the side effects are negligible, this is an alternative to treat amyotrophic lateral sclerosis, and other patients with diseases that present sialorrhea. RESUMO Esclerose lateral amiotrófica é uma doença neurodegenerativa progressiva e fatal, caracterizada pela degeneração dos neurônios motores, as células do sistema nervoso central que controlam os movimentos voluntários dos músculos. A salivação excessiva (sialorreia) está presente em cerca de 50% dos casos de esclerose lateral amiotrófica. Dessa forma, surgem medidas terapêuticas alternativas como drogas anticolinérgicas e cirurgia, e recentemente, o uso da toxina botulínica, aplicada em um ponto central das glândulas salivares, muitas vezes guiado por ultrassonografia, demostrou resultados positivos. Objetivou-se revisar a literatura no intuito de demonstrar um método alternativo aos tratamentos de sialorreia em pacientes com esclerose lateral amiotrófica. Em estudos recentes, a eficácia do tratamento com toxina botulínica foi confirmada e, mesmo requerendo novas aplicações, os efeitos colaterais são ínfimos. Ela surge então como alternativa não só ao

[Non-invasive mechanical ventilation with a facial interface during sedation for a percutaneous endoscopic gastrostomy in a patient with amyotrophic lateral sclerosis].

PubMed

González-Frasquet, M C; García-Covisa, N; Vidagany-Espert, L; Herranz-Gordo, A; Llopis-Calatayud, J E

2015-11-01

Amyotrophic lateral sclerosis is a chronic neurodegenerative disease of the central nervous system which affects the motor neurons and produces a progressive muscle weakness, leading to atrophy and muscle paralysis, and ultimately death. Performing a percutaneous endoscopic gastrostomy with sedation in patients with amyotrophic lateral sclerosis can be a challenge for the anesthesiologist. The case is presented of a 76-year-old patient who suffered from advanced stage amyotrophic lateral sclerosis, ASA III, in which a percutaneous endoscopic gastrostomy was performed with deep sedation, for which non-invasive ventilation was used as a respiratory support to prevent hypoventilation and postoperative respiratory complications. Copyright © 2014 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

Experimental study of brachial plexus and vessel compression: evaluation of combined central and peripheral electrodiagnostic approach.

PubMed

Yang, Chaoqun; Xu, Jianguang; Chen, Jie; Li, Shulin; Cao, Yu; Zhu, Yi; Xu, Lei

2017-08-01

We sought to investigate the reliability of a new electrodiagnostic method for identifying Electrodiagnosis of Brachial Plexus & Vessel Compression Syndrome (BPVCS) in rats that involves the application of transcranial electrical stimulation motor evoked potentials (TES-MEPs) combined with peripheral nerve stimulation compound muscle action potentials (PNS-CMAPs). The latencies of the TES-MEP and PNS-CMAP were initially elongated in the 8-week group. The amplitudes of TES-MEP and PNS-CMAP were initially attenuated in the 16-week group. The isolateral amplitude ratio of the TES-MEP to the PNS-CMAP was apparently decreased, and spontaneous activities emerged at 16 weeks postoperatively. Superior and inferior trunk models of BPVCS were created in 72 male Sprague Dawley (SD) rats that were divided into six experimental groups. The latencies, amplitudes and isolateral amplitude ratios of the TES-MEPs and PNS-CMAPs were recorded at different postoperative intervals. Electrophysiological and histological examinations of the rats' compressed brachial plexus nerves were utilized to establish preliminary electrodiagnostic criteria for BPVCS.

A system for real-time measurement of the brachial artery diameter in B-mode ultrasound images.

PubMed

Gemignani, Vincenzo; Faita, Francesco; Ghiadoni, Lorenzo; Poggianti, Elisa; Demi, Marcello

2007-03-01

The measurement of the brachial artery diameter is frequently used in clinical studies for evaluating the flow-mediated dilation and, in conjunction with the blood pressure value, for assessing arterial stiffness. This paper presents a system for computing the brachial artery diameter in real-time by analyzing B-mode ultrasound images. The method is based on a robust edge detection algorithm which is used to automatically locate the two walls of the vessel. The measure of the diameter is obtained with subpixel precision and with a temporal resolution of 25 samples/s, so that the small dilations induced by the cardiac cycle can also be retrieved. The algorithm is implemented on a standalone video processing board which acquires the analog video signal from the ultrasound equipment. Results are shown in real-time on a graphical user interface. The system was tested both on synthetic ultrasound images and in clinical studies of flow-mediated dilation. Accuracy, robustness, and intra/inter observer variability of the method were evaluated.

Impact of weight loss on ankle-brachial index and interartery blood pressures.

PubMed

Espeland, Mark A; Lewis, Cora E; Bahnson, Judy; Knowler, William C; Regensteiner, Judith G; Gaussoin, Sarah A; Beavers, Daniel; Johnson, Karen C

2014-04-01

To assess whether weight loss improves markers of peripheral artery disease and vascular stenosis. The Action for Health in Diabetes randomized clinical trial compared intensive lifestyle intervention (ILI) for weight loss to a control condition of diabetes support and education (DSE) in overweight or obese adults with type 2 diabetes. Annual ankle and brachial blood pressures over four years were used to compute ankle-brachial indices (ABIs) and to assess interartery blood pressure differences in 5018 participants. ILI, compared to DSE, produced 7.8% (Year 1) to 3.6% (Year 4) greater weight losses. These did not affect prevalence of low (<0.90) ABI (3.60% in DSE versus 3.14% in ILI; P = 0.20) or elevated (>1.40) ABI (7.52% in DSE versus 7.59% in ILI: P = 0.90), but produced smaller mean (SE) maximum interartery systolic blood pressure differences among ankle sites [19.7 (0.2) mmHg for ILI versus 20.6 (0.2) mmHg for DSE (P < 0.001)] and between arms [5.8 (0.1) mmHg for ILI versus 6.1 (0.1) mmHg for DSE (P = 0.01)]. Four years of intensive behavioral weight loss intervention did not significantly alter prevalence of abnormal ABI, however, it did reduce differences in systolic blood pressures among arterial sites. Copyright © 2013 The Obesity Society.

Non-invasive determination of instantaneous brachial blood flow using the oscillometric method.

PubMed

Liu, Shing-Hong; Wang, Jia-Jung; Cheng, Da-Chuan

2009-08-01

The oscillometric method has been widely used to measure arterial systolic and diastolic blood pressures, but its potential for arterial blood flow measurements still remains to be explored. The aim of this study was to non-invasively determine arterial blood flow using an oscillometric blood flow measurement system. The system consists of a pneumatic elastic cuff, an air-pumping motor, a releaser valve, a pressure transducer, and an airflow meter. To build a non-linear cuff model, we measured airflow pumped into the pneumatic cuff and cuff pressure using an airflow meter and pressure transducer during the inflation period, respectively. During the deflation period, only the pressure transducer was used to record cuff pressure. Based on the cuff model, the oscillometric blood flow waveform was obtained by integrating the oscillometric pressure waveform. We compared arterial blood flow derived from the maximum amplitude of the oscillometric blood flow waveform with Doppler-measured blood flow calculated with the diameters and blood velocities of the brachial arteries in 32 subjects who underwent diagnostic evaluations for peripheral arterial embolism. A linear correlation coefficient of r = 0.716 was found between the oscillometry- and Doppler-based blood flow measurements in the 32 subjects. These results suggest that blood flow passing through the brachial artery can be quantified non-invasively using the oscillometric approach after appropriate calibration.

Marijuana in the management of amyotrophic lateral sclerosis.

PubMed

Carter, G T; Rosen, B S

2001-01-01

Marijuana has been proposed as treatment for a widening spectrum of medical conditions. Marijuana is a substance with many properties that may be applicable to the management of amyotrophic lateral sclerosis (ALS). These include analgesia, muscle relaxation, bronchodilation, saliva reduction, appetite stimulation, and sleep induction. In addition, marijuana has now been shown to have strong antioxidative and neuroprotective effects, which may prolong neuronal cell survival. In areas where it is legal to do so, marijuana should be considered in the pharmacological management of ALS. Further investigation into the usefulness of marijuana in this setting is warranted.

Respiratory therapies for amyotrophic lateral sclerosis: a primer.

PubMed

Gruis, Kirsten L; Lechtzin, Noah

2012-09-01

Respiratory complications are a common cause of morbidity and mortality in amyotrophic lateral sclerosis (ALS). Treatment of respiratory insufficiency with noninvasive ventilation (NIV) improves ALS patients' quality of life and survival. Evidence-based practice guidelines for the management of ALS patients recommend treatment of respiratory insufficiency with NIV as well as consideration of insufflation/exsufflation to improve clearance of airway secretions. Despite these recommendations respiratory therapies remain underused. In this review we provide a practical guide for the clinician to prescribe and manage respiratory therapies for the patient with ALS. Copyright © 2012 Wiley Periodicals, Inc.

[Amyotrophic lateral sclerosis--diagnosis and treatment].

PubMed

Jung, H H; Neumann, M; Bloch, K E

2012-07-04

Amyotrophic lateral sclerosis (ALS) represents the most common motoneuron disorder in adulthood. It is characterized by selective degeneration of the motoneurons. About 10% of patients have a genetically determined ALS. Clinically, ALS is characterized by coexistence of signs of the first motoneuron, such as spasticity and hyperreflexia, as well as the second motoneuron, such as muscular atrophy and fasciculations. If such signs are present in at least three regions and if other possible causes have been excluded, a definite diagnosis of ALS can be made based on the revised El-Escorial criteria. Initial manifestations are often focalized and generalization develops during the course. The glutamate antagonist riluzole is worldwide the only approved ALS treatment. However, symptomatic treatments to ameliorate spasticity, drooling, speech and swallowing problems, and assisted ventilation to treat respiratory failure are essential.

Parecoxib added to ropivacaine prolongs duration of axillary brachial plexus blockade and relieves postoperative pain.

PubMed

Liu, Xiaoming; Zhao, Xuan; Lou, Jian; Wang, Yingwei; Shen, Xiaofang

2013-02-01

Cyclooxygenase (COX)-2 antagonist is widely used for intravenous postoperative pain relief. Recent studies reported COX-2 in the spinal dorsal horn could modulate spinal nociceptive processes. Epidural parecoxib in rats showed no neurotoxicity. These findings suggested applying a COX-2 antagonist directly to the central or peripheral nerve might provide better analgesia. We therefore determined: (1) whether the addition of parecoxib to ropivacaine injected locally on the nerve block affected the sensory and motor block times of the brachial plexus nerve block; and (2) whether parecoxib injected locally on the nerve or intravenously had a similar analgesic adjuvant effect. We conducted a randomized controlled trial from January 2009 to November 2010 with 150 patients scheduled for elective forearm surgery, using a multiple-nerve stimulation technique. Patients were randomly allocated into one of three groups: Group A (n = 50) received ropivacaine 0.25% alone on the brachial plexus nerve; Group B (n = 50) received ropivacaine together with 20 mg parecoxib locally on the nerve block; and Group C (n = 50) received 20 mg parecoxib intravenously. We recorded the duration of the sensory and motor blocks, and the most severe pain score during a 24-hour postoperative period. Parecoxib added locally on the nerve block prolonged the motor and sensory block times compared with Group A. However, parecoxib injected intravenously had no such effect. Pain intensity scores in Group B were lower than those in Groups A and C. Parecoxib added to ropivacaine locally on the nerve block prolonged the duration of the axillary brachial plexus blockade and relieved postoperative pain for patients having forearm orthopaedic surgery. Level I, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Pain and brachial plexus lesions: evaluation of initial outcomes after reconstructive microsurgery and validation of a new pain severity scale.

PubMed

Bonilla, Gonzalo; Di Masi, Gilda; Battaglia, Danilo; Otero, José María; Socolovsky, Mariano

2011-01-01

Peripheral nerve lesions usually are associated with neuropathic pain. In the present paper, we describe a simple scale to quantify pain after brachial plexus injuries and apply this scale to a series of patients to determine initial outcomes after reconstructive surgery. Fifty-one patients with traumatic brachial plexus avulsion injuries were treated over the period of one calendar year at one center by the same surgical team. Of these, 28 patients who were available for follow-up reported some degree of neuropathic pain radiating towards the hand or forearm and underwent reconstructive microsurgery and direct pain management, including trunk and nerve neurolysis and repair. A special pain severity rating scale was developed and used to assess patients' pain before and after surgery, over a minimum follow-up of 6 months. An independent researcher, not part of the surgical team, performed all pre- and postoperative evaluations. Of the 28 patients with brachial plexus traction injuries who met eligibility criteria, 93% were male, and most were young (mean age, 27.6 years). The mean preoperative severity of pain using our scale was 30.9 out of a maximum of 37 (± 0.76 SD), which fell to a mean of 6.9 (± 0.68 SD) 6 months post-procedure. On average, pain declined by 78% across the whole series, a decline that was statistically significant (p < .001). Subset analysis revealed similar improvements across all the different parameters of pain. We have designed and tested a simple and reliable method by which to quantify neuropathic pain after traumatic brachial plexus injuries. Initial surgical treatment of the paralysis--including nerve, trunk and root reconstruction, and neurolysis--comprises an effective means by which to initially treat neuropathic pain. Ablative or neuromodulative procedures, like dorsal root entry zone, should be reserved for refractory cases.

Targeted Riluzole Delivery by Antioxidant Nanovectors for Treating Amyotrophic Lateral Sclerosis

DTIC Science & Technology

2014-10-01

8. Special Reporting Requirements…………………………………… 6 9. Appendices/Quadchart…………………………………………… n /a 1 1. Introduction: Amyotrophic lateral... acetyltransferase ) and quantified image analysis. These studies are ongoing, but should be complete by the middle of January, 2015. What opportunities

Dual Nerve Transfers for Restoration of Shoulder Function After Brachial Plexus Avulsion Injury.

PubMed

Chu, Bin; Wang, Huan; Chen, Liang; Gu, Yudong; Hu, Shaonan

2016-06-01

The purpose of this study was to investigate the effectiveness of shoulder function restoration by dual nerve transfers, spinal accessory nerve to the suprascapular nerve and 2 intercostal nerves to the anterior branch of the axillary nerve, in patients with shoulder paralysis that resulted from brachial plexus avulsion injury. It was a retrospective analysis to assess the impact of a variety of factors on reanimation of shoulder functions with dual nerve transfers. A total of 19 patients were included in this study. Most of these patients sustained avulsions of C5, C6, and C7 nerve roots (16 patients). Three of them had avulsions of C5 and C6 roots only. Through a posterior approach, direct coaptation of the intercostal nerves and the anterior branch of the axillary nerve was performed, along with accessory nerve transfer to the suprascapular nerve. Satisfactory shoulder function recovery (93.83° of shoulder abduction and 54.00° of external rotation on average) was achieved after a 62-month follow-up. This dual nerve transfer procedure provided us with a reliable and effective method for shoulder function reconstruction after brachial plexus root avulsion, especially C5/C6/C7 avulsion. The level of evidence is therapeutic IV.

Correlations between brachial endothelial function and cardiovascular risk factors: a survey of 2,511 Chinese subjects

PubMed Central

Yang, Ping-Ting; Yuan, Hong; Wang, Ya-Qin; Cao, Xia; Wu, Liu-Xin

2014-01-01

Objective We examined the relationship of several cardiovascular risk factors (CVRF) to brachial artery flow-mediated dilatation (FMD) in Chinese subjects. Methods This was a cross-sectional study. In 2,511 Chinese adults (age 46.86±9.52 years, 1,891 men and 620 women) recruited from people who underwent health screening at The Third Xiangya Hospital, patients’ CVRF [age, body mass index (BMI), waist circumference (WC), blood pressure (BP), cholesterol parameters, creatinine (Cr), uric acid (UA), glucose level and smoking] and prevalence of present disease (hypertension, diabetes mellitus, coronary heart disease and hyperlipidemia) were investigated. Results Multivariate analysis revealed that FMD negative correlated with age (β=–0.29, P<0.001), gender (β=–0.12, P<0.001), BMI (β=–0.12, P=0.001), WC (β=–0.10, P=0.011), systolic BP (SBP) (β=–0.12, P<0.001), fasting glucose (β=–0.04, P=0.009), total cholesterol (TC) (β=–0.04, P=0.014), smoking (β=–0.05, P=0.003), and baseline brachial artery diameter (β=–0.35, P<0.001). FMD decreased with increasing age in both genders. In women, FMD was higher than men and age-related decline in FMD was steepest after age 40; FMD was similar in men above 55 years old. Conclusions In Chinese subjects, FMD may be a usefully marker of CVRF. Age, gender, BMI, WC, SBP, fasting glucose, TC, smoking, and baseline brachial artery diameter were independent variables related to the impairment of FMD. The influence of CVRF on endothelial function is more in women than men. PMID:25364521

Association Between Dietary Intake and Function in Amyotrophic Lateral Sclerosis

PubMed Central

Nieves, Jeri W.; Gennings, Chris; Factor-Litvak, Pam; Hupf, Jonathan; Singleton, Jessica; Sharf, Valerie; Oskarsson, Björn; Fernandes Filho, J. Americo M.; Sorenson, Eric J.; D’Amico, Emanuele; Goetz, Ray; Mitsumoto, Hiroshi

2017-01-01

IMPORTANCE There is growing interest in the role of nutrition in the pathogenesis and progression of amyotrophic lateral sclerosis (ALS). OBJECTIVE To evaluate the associations between nutrients, individually and in groups, and ALS function and respiratory function at diagnosis. DESIGN, SETTING, AND PARTICIPANTS A cross-sectional baseline analysis of the Amyotrophic Lateral Sclerosis Multicenter Cohort Study of Oxidative Stress study was conducted from March 14, 2008, to February 27, 2013, at 16 ALS clinics throughout the United States among 302 patients with ALS symptom duration of 18 months or less. EXPOSURES Nutrient intake, measured using a modified Block Food Frequency Questionnaire (FFQ). MAIN OUTCOMES AND MEASURES Amyotrophic lateral sclerosis function, measured using the ALS Functional Rating Scale–Revised (ALSFRS-R), and respiratory function, measured using percentage of predicted forced vital capacity (FVC). RESULTS Baseline data were available on 302 patients with ALS (median age, 63.2 years [interquartile range, 55.5–68.0 years]; 178 men and 124 women). Regression analysis of nutrients found that higher intakes of antioxidants and carotenes from vegetables were associated with higher ALSFRS-R scores or percentage FVC. Empirically weighted indices using the weighted quantile sum regression method of “good” micronutrients and “good” food groups were positively associated with ALSFRS-R scores (β [SE], 2.7 [0.69] and 2.9 [0.9], respectively) and percentage FVC (β [SE], 12.1 [2.8] and 11.5 [3.4], respectively) (all P < .001). Positive and significant associations with ALSFRS-R scores (β [SE], 1.5 [0.61]; P = .02) and percentage FVC (β [SE], 5.2 [2.2]; P = .02) for selected vitamins were found in exploratory analyses. CONCLUSIONS AND RELEVANCE Antioxidants, carotenes, fruits, and vegetables were associated with higher ALS function at baseline by regression of nutrient indices and weighted quantile sum regression analysis. We also demonstrated

A Review of Brachial Plexus Birth Palsy: Injury and Rehabilitation.

PubMed

Raducha, Jeremy E; Cohen, Brian; Blood, Travis; Katarincic, Julia

2017-11-01

Brachial plexus injuries during the birthing process can leave infants with upper extremity deficits corresponding to the location of the lesion within the complex plexus anatomy. Manifestations can range from mild injuries with complete resolution to severe and permanent disability. Overall, patients have a high rate of spontaneous recovery (66-92%).1,2 Initially, all lesions are managed with passive range motion and observation. Prevention and/or correction of contractures with occupational therapy and serial splinting/casting along with encouraging normal development are the main goals of non-operative treatment. Surgical intervention may be war- ranted, depending on functional recovery. [Full article available at http://rimed.org/rimedicaljournal-2017-11.asp].

A systematic review of the sensitivity and specificity of the toe-brachial index for detecting peripheral artery disease.

PubMed

Tehan, Peta Ellen; Santos, Derek; Chuter, Vivienne Helaine

2016-08-01

The toe-brachial index (TBI) is used as an adjunct to the ankle-brachial index (ABI) for non-invasive lower limb vascular screening. With increasing evidence suggesting limitations of the ABI for diagnosis of vascular complications, particularly in specific populations including diabetes cohorts, the TBI is being used more widely. The aim of this review was to determine the sensitivity and specificity of the TBI for detecting peripheral artery disease (PAD) in populations at risk of this disease. A database search was conducted to identify current work relating to the sensitivity and specificity of toe-brachial indices up to July 2015. Only studies using valid diagnostic imaging as a reference standard were included. The QUADAS-2 tool was used to critically appraise included articles. Seven studies met the inclusion criteria. Sensitivity of the TBI for PAD was reported in all seven studies and ranged from 45% to 100%; specificity was reported by five studies only and ranged from 16% to 100%. In conclusion, this review suggests that the TBI has variable diagnostic accuracy for the presence of PAD in specific populations at risk of developing the disease. There was a notable lack of large-scale diagnostic accuracy studies determining the diagnostic accuracy of the TBI in detecting PAD in different at-risk cohorts. However, standardised normal values need to be established for the TBI to conclusively determine the diagnostic accuracy of this test. © The Author(s) 2016.

Collagen nerve guides for surgical repair of brachial plexus birth injury.

PubMed

Ashley, William W; Weatherly, Trisha; Park, Tae Sung

2006-12-01

Standard brachial plexus repair techniques often involve autologous nerve graft placement and neurotization. However, when performed to treat severe injuries, this procedure can sometimes yield poor results. Moreover, harvesting the autologous graft is time-consuming and exposes the patient to additional surgical risks. To improve surgical outcomes and reduce surgical risks associated with autologous nerve graft retrieval and placement, the authors use collagen matrix tubes (Neurogen) instead of autologous nerve graft material. Between 1991 and 2005, the authors surgically treated 65 infants who had suffered brachial plexus injury at birth. During this time, seven patients were treated using collagen matrix tubes (Neurogen). This study is a retrospective analysis of the initial five patients who were treated using the tubes. Two patients underwent tube placement recently and were excluded from the analysis because of the inadequate follow-up period. Four of the five patients experienced a good recovery (motor scale composite [MSC] > 0.6), and three exhibited an excellent recovery (MSC > 0.75) at 2 years postoperatively. The MSC improved by an average of 69 and 78% at 1 and 2 years, respectively. The movement scores improved to greater than or equal to 50% range of motion in most patients, and the contractures were usually mild or moderate. Follow-up physical and occupational therapy evaluations confirm these patients' functional status. When last seen, four of five of these children could feed and dress themselves. Technically, the use of the collagen matrix tubes was straightforward and efficient, and there were no complications. The outcomes in this small series are encouraging.

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis.

PubMed

Johnson, Janel O; Pioro, Erik P; Boehringer, Ashley; Chia, Ruth; Feit, Howard; Renton, Alan E; Pliner, Hannah A; Abramzon, Yevgeniya; Marangi, Giuseppe; Winborn, Brett J; Gibbs, J Raphael; Nalls, Michael A; Morgan, Sarah; Shoai, Maryam; Hardy, John; Pittman, Alan; Orrell, Richard W; Malaspina, Andrea; Sidle, Katie C; Fratta, Pietro; Harms, Matthew B; Baloh, Robert H; Pestronk, Alan; Weihl, Conrad C; Rogaeva, Ekaterina; Zinman, Lorne; Drory, Vivian E; Borghero, Giuseppe; Mora, Gabriele; Calvo, Andrea; Rothstein, Jeffrey D; Drepper, Carsten; Sendtner, Michael; Singleton, Andrew B; Taylor, J Paul; Cookson, Mark R; Restagno, Gabriella; Sabatelli, Mario; Bowser, Robert; Chiò, Adriano; Traynor, Bryan J

2014-05-01

MATR3 is an RNA- and DNA-binding protein that interacts with TDP-43, a disease protein linked to amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. Using exome sequencing, we identified mutations in MATR3 in ALS kindreds. We also observed MATR3 pathology in ALS-affected spinal cords with and without MATR3 mutations. Our data provide more evidence supporting the role of aberrant RNA processing in motor neuron degeneration.

A cadaveric study to determine the minimum volume of methylene blue to completely color the nerves of brachial plexus in cats. An update in forelimb and shoulder surgeries.

PubMed

Mencalha, Rodrigo; Fernandes, Neide; Sousa, Carlos Augusto dos Santos; Abidu-Figueiredo, Marcelo

2014-06-01

To determine the minimum volume of methylene blue (MB) to completely color the brachial plexus (BP) nerves, simulating an effective anesthetic block in cats. Fifteen adult male cat cadavers were injected through subscapular approach with volumes of 2, 3, 4, 5 and 6 ml in both forelimbs, for a total of 30 brachial plexus blocks (BPB). After infusions, the specimens were carefully dissected preserving each nervous branch. The measurement of the effective area was indicated by the impregnation of MB. Nerves were divided into four segments from the origin at the spinal level until the insertion into the thoracic limb muscles. The blocks were considered effective only when all the nerves were strongly or totally colored. Volumes of 2, 3 and 4 ml were considered insufficient suggesting a failed block, however, volumes of 5 and 6 ml were associated with a successful block. The injection of methylene blue, in a volume of 6 ml, completely colored the brachial plexus. At volumes of 5 and 6 ml the brachial plexus blocks were considered a successful regional block, however, volumes of 2, 3 and 4 ml were considered a failed regional block.

Associations between ankle-brachial index and cognitive function: results from the Lifestyle Interventions and Independence for Elders trial

USDA-ARS?s Scientific Manuscript database

OBJECTIVE: The objective of this study was to evaluate cross-sectional and longitudinal associations between ankle-brachial index (ABI) and indicators of cognitive function. DESIGN: Randomized clinical trial (Lifestyle Interventions and Independence for Elders Trial). SETTING: Eight US academic ce...

Dexmedetomidine prolongs the effect of bupivacaine in supraclavicular brachial plexus block

PubMed Central

Agarwal, Sandhya; Aggarwal, Ritu; Gupta, Praveen

2014-01-01

Background: We compared the effects of adding dexmedetomidine to a 30 ml solution of 0.325% bupivacaine in supraclavicular brachial plexus block. Onset and duration of sensory and motor block along with the duration of analgesia were the primary endpoints. Materials and Methods: Fifty patients posted for upper limb surgeries were enrolled for a prospective, randomized, double-blind, placebo-controlled trial. Patients were divided into two groups, the control group S and the study group SD. In group S (n = 25), 30 ml of 0.325% bupivacaine + 1 ml normal saline; and in group SD (n = 25), 30 ml of 0.325% bupivacaine + 1 ml (100 μg) dexmedetomidine were given for supraclavicular brachial plexus block using the peripheral nerve stimulator. Onset and duration of sensory and motor blocks were assessed along with the duration of analgesia, sedation, and adverse effects, if any. Hemodynamic parameters, like heart rate (HR), systolic arterial blood pressure (SBP), and diastolic arterial blood pressure (DBP) were also monitored. Results: Demographic data and surgical characteristics were comparable in both the groups. The onset times for sensory and motor blocks were significantly shorter in SD than S group (P < 0.001), while the duration of blocks was significantly longer (P < 0.001) in SD group. Except for the initial recordings (at 0, 5, 10, and 15 min), heart rate levels in group SD were significantly lower (P < 0.001). SBP and DBP levels in SD group at 15, 30, 45, 60, 90 and 120 min were significantly lower than in S group (P < 0.001). In fact, when the percentage changes in HR/SBP/DBP were compared from 0-5/0-10/0-15/0-30/0-45/0-60/0-90/0-120 min in SD with S group, they came out to be highly significant (P < 0.001) in group SD. The duration of analgesia (DOA) was significantly longer in SD group than S group (P < 0.001). Except that, bradycardia was observed in one patient in the group SD, no other adverse effects were observed in either of the groups. Conclusion

UBC-Nepal Expedition: acute alterations in sympathetic nervous activity do not influence brachial artery endothelial function at sea level and high altitude.

PubMed

Tymko, Michael M; Tremblay, Joshua C; Steinback, Craig D; Moore, Jonathan P; Hansen, Alex B; Patrician, Alexander; Howe, Connor A; Hoiland, Ryan L; Green, Daniel J; Ainslie, Philip N

2017-11-01

Evidence indicates that increases in sympathetic nervous activity (SNA), and acclimatization to high altitude (HA), may reduce endothelial function as assessed by brachial artery flow-mediated dilatation (FMD); however, it is unclear whether such changes in FMD are due to direct vascular constraint, or consequential altered hemodynamics (e.g., shear stress) associated with increased SNA as a consequence of exposure to HA. We hypothesized that 1 ) at rest, SNA would be elevated and FMD would be reduced at HA compared with sea-level (SL); and 2 ) at SL and HA, FMD would be reduced when SNA was acutely increased, and elevated when SNA was acutely decreased. Using a novel, randomized experimental design, brachial artery FMD was assessed at SL (344 m) and HA (5,050 m) in 14 participants during mild lower-body negative pressure (LBNP; -10 mmHg) and lower-body positive pressure (LBPP; +10 mmHg). Blood pressure (finger photoplethysmography), heart rate (electrocardiogram), oxygen saturation (pulse oximetry), and brachial artery blood flow and shear rate (Duplex ultrasound) were recorded during LBNP, control, and LBPP trials. Muscle SNA was recorded (via microneurography) in a subset of participants ( n = 5). Our findings were 1 ) at rest, SNA was elevated ( P < 0.01), and absolute FMD was reduced ( P = 0.024), but relative FMD remained unaltered ( P = 0.061), at HA compared with SL; and 2 ) despite significantly altering SNA with LBNP (+60.3 ± 25.5%) and LBPP (-37.2 ± 12.7%) ( P < 0.01), FMD was unaltered at SL ( P = 0.448) and HA ( P = 0.537). These data indicate that acute and mild changes in SNA do not directly influence brachial artery FMD at SL or HA. NEW & NOTEWORTHY The role of the sympathetic nervous system on endothelial function remains unclear. We used lower-body negative and positive pressure to manipulate sympathetic nervous activity at sea level and high altitude and measured brachial endothelial function via flow-mediated dilation. We found that

A Retrospective Study Evaluating the Effect of Low Doses of Perineural Dexamethasone on Ropivacaine Brachial Plexus Peripheral Nerve Block Analgesic Duration.

PubMed

Schnepper, Gregory D; Kightlinger, Benjamin I; Jiang, Yunyun; Wolf, Bethany J; Bolin, Eric D; Wilson, Sylvia H

2017-09-23

Examination of the effectiveness of perineural dexamethasone administered in very low and low doses on ropivacaine brachial plexus block duration. Retrospective evaluation of brachial plexus block duration in a large cohort of patients receiving peripheral nerve blocks with and without perineural dexamethasone in a prospectively collected quality assurance database. A single academic medical center. A total of 1,942 brachial plexus blocks placed over a 16-month period were reviewed. Demographics, nerve block location, and perineural dexamethasone utilization and dose were examined in relation to block duration. Perineural dexamethasone was examined as none (0 mg), very low dose (2 mg or less), and low dose (greater than 2 mg to 4 mg). Continuous catheter techniques, local anesthetics other than ropivacaine, and block locations with fewer than 15 subjects were excluded. Associations between block duration and predictors of interest were examined using multivariable regression models. A subgroup analysis of the impact of receiving dexamethasone on block duration within each block type was also conducted using a univariate linear regression approach. A total of 1,027 subjects were evaluated. More than 90% of brachial plexus blocks contained perineural dexamethasone (≤4 mg), with a median dose of 2 mg. Increased block duration was associated with receiving any dose of perineural dexamethasone (P < 0.0001), female gender (P = 0.022), increased age (P = 0.048), and increased local anesthetic dose (P = 0.01). In a multivariable model, block duration did not differ with very low- or low-dose perineural dexamethasone after controlling for other factors (P = 0.420). Perineural dexamethasone prolonged block duration compared with ropivacaine alone; however, duration was not greater with low-dose compared with very low-dose perineural dexamethasone. © 2017 American Academy of Pain Medicine. All rights reserved. For permissions, please e

Proximal Junctional Kyphosis.

PubMed

Kim, Han Jo; Iyer, Sravisht

2016-05-01

Proximal junctional kyphosis (PJK) is a common complication following adult spinal deformity surgery. It is defined by two criteria: a proximal junctional sagittal Cobb angle (1) ≥ 10° and (2) at least 10° greater than the preoperative measurement. PJK is multifactorial in origin and likely stems from surgical, radiographic, and patient-related risk factors. The diagnosis of PJK represents a broad spectrum of disease ranging from asymptomatic patients with recurrence of deformity to those presenting with increased pain, functional deficit, and, in the most severe cases, neurologic deficits. Recent studies have demonstrated increased pain levels in select patients with PJK. In keeping with the broad spectrum of the disease, classification schemes are needed to better describe and stratify the severity of PJK. The most severe form is proximal junctional failure. A consensus on a uniform definition of proximal junctional failure is needed to allow for more systematic study of this phenomenon.

Comprehensive assessment of impaired peripheral and coronary artery endothelial functions in smokers using brachial artery ultrasound and oxygen-15-labeled water PET.

PubMed

Ochi, Noriki; Yoshinaga, Keiichiro; Ito, Yoichi M; Tomiyama, Yuuki; Inoue, Mamiko; Nishida, Mutsumi; Manabe, Osamu; Shibuya, Hitoshi; Shimizu, Chikara; Suzuki, Eriko; Fujii, Satoshi; Katoh, Chietsugu; Tamaki, Nagara

2016-10-01

Comprehensive evaluation of endothelium-dependent and endothelium-independent vascular functions in peripheral arteries and coronary arteries in smokers has never been performed previously. Through the use of brachial artery ultrasound and oxygen-15-labeled water positron emission tomography (PET), we sought to investigate peripheral and coronary vascular dysfunctions in smokers. Eight smokers and 10 healthy individuals underwent brachial artery ultrasound at rest, during reactive hyperemia [250mmHg cuff occlusion (flow-mediated dilatation (FMD)], and following sublingual nitroglycerin (NTG) administration. Myocardial blood flow (MBF) was assessed through O-15-labeled water PET at rest, during adenosine triphosphate (ATP) administration, and during a cold pressor test (CPT). Through ultrasound, smokers were shown to have significantly reduced %FMD compared to controls (6.62±2.28% vs. 11.29±2.75%, p=0.0014). As assessed by O-15-labeled water PET, smokers were shown to have a significantly lower CPT response than were controls (21.1±9.5% vs. 50.9±16.9%, p=0.0004). There was no relationship between %FMD and CPT response (r=0.40, p=0.097). Endothelium-independent vascular dilatation was similar for both groups in terms of coronary flow reserve with PET (p=0.19). Smokers tended to have lower %NTG in the brachial artery (p=0.055). Smokers exhibited impaired coronary endothelial function as well as peripheral brachial artery endothelial function. In addition, there was no correlation between PET and ultrasound measurements, possibly implying that while smokers may have systemic vascular endothelial dysfunction, the characteristics of that dysfunction may be different in peripheral arteries and coronary arteries. Copyright © 2016. Published by Elsevier Ltd.

Amyotrophic lateral sclerosis progression and stability of brain-computer interface communication.

PubMed

Silvoni, Stefano; Cavinato, Marianna; Volpato, Chiara; Ruf, Carolin A; Birbaumer, Niels; Piccione, Francesco

2013-09-01

Our objective was to investigate the relationship between brain-computer interface (BCI) communication skill and disease progression in amyotrophic lateral sclerosis (ALS). We sought also to assess stability of BCI communication performance over time and whether it is related to the progression of neurological impairment before entering the locked-in state. A three years follow-up, BCI evaluation in a group of ALS patients (n = 24) was conducted. For a variety of reasons only three patients completed the three years follow-up. BCI communication skill and disability level, using the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised, were assessed at admission and at each of the three follow-ups. Multiple non-parametric statistical methods were used to ensure reliability of the dependent variables: correlations, paired test and factor analysis of variance. Results demonstrated no significant relationship between BCI communication skill (BCI-CS) and disease evolution. The patients who performed the follow-up evaluations preserved their BCI-CS over time. Patients' age at admission correlated positively with the ability to achieve control over a BCI. In conclusion, disease evolution in ALS does not affect the ability to control a BCI for communication. BCI performance can be maintained in the different stages of the illness.

Motoneuron firing in amyotrophic lateral sclerosis (ALS)

PubMed Central

de Carvalho, Mamede; Eisen, Andrew; Krieger, Charles; Swash, Michael

2014-01-01

Amyotrophic lateral sclerosis is an inexorably progressive neurodegenerative disorder involving the classical motor system and the frontal effector brain, causing muscular weakness and atrophy, with variable upper motor neuron signs and often an associated fronto-temporal dementia. The physiological disturbance consequent on the motor system degeneration is beginning to be well understood. In this review we describe aspects of the motor cortical, neuronal, and lower motor neuron dysfunction. We show how studies of the changes in the pattern of motor unit firing help delineate the underlying pathophysiological disturbance as the disease progresses. Such studies are beginning to illuminate the underlying disordered pathophysiological processes in the disease, and are important in designing new approaches to therapy and especially for clinical trials. PMID:25294995

Amyotrophic lateral sclerosis: update and new developments

PubMed Central

Pratt, Ashley J; Getzoff, Elizabeth D; Perry, J Jefferson P

2012-01-01

Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease. It is typically characterized by adult-onset degeneration of the upper and lower motor neurons, and is usually fatal within a few years of onset. A subset of ALS patients has an inherited form of the disease, and a few of the known mutant genes identified in familial cases have also been found in sporadic forms of ALS. Precisely how the diverse ALS-linked gene products dictate the course of the disease, resulting in compromised voluntary muscular ability, is not entirely known. This review addresses the major advances that are being made in our understanding of the molecular mechanisms giving rise to the disease, which may eventually translate into new treatment options. PMID:23019386

Obstetrical brachial plexus injury (OBPI): Canada's national clinical practice guideline

PubMed Central

Coroneos, Christopher J; Voineskos, Sophocles H; Christakis, Marie K; Thoma, Achilleas; Bain, James R; Brouwers, Melissa C

2017-01-01

Objective The objective of this study was to establish an evidence-based clinical practice guideline for the primary management of obstetrical brachial plexus injury (OBPI). This clinical practice guideline addresses 4 existing gaps: (1) historic poor use of evidence, (2) timing of referral to multidisciplinary care, (3) Indications and timing of operative nerve repair and (4) distribution of expertise. Setting The guideline is intended for all healthcare providers treating infants and children, and all specialists treating upper extremity injuries. Participants The evidence interpretation and recommendation consensus team (Canadian OBPI Working Group) was composed of clinicians representing each of Canada's 10 multidisciplinary centres. Outcome measures An electronic modified Delphi approach was used for consensus, with agreement criteria defined a priori. Quality indicators for referral to a multidisciplinary centre were established by consensus. An original meta-analysis of primary nerve repair and review of Canadian epidemiology and burden were previously completed. Results 7 recommendations address clinical gaps and guide identification, referral, treatment and outcome assessment: (1) physically examine for OBPI in newborns with arm asymmetry or risk factors; (2) refer newborns with OBPI to a multidisciplinary centre by 1 month; (3) provide pregnancy/birth history and physical examination findings at birth; (4) multidisciplinary centres should include a therapist and peripheral nerve surgeon experienced with OBPI; (5) physical therapy should be advised by a multidisciplinary team; (6) microsurgical nerve repair is indicated in root avulsion and other OBPI meeting centre operative criteria; (7) the common data set includes the Narakas classification, limb length, Active Movement Scale (AMS) and Brachial Plexus Outcome Measure (BPOM) 2 years after birth/surgery. Conclusions The process established a new network of opinion leaders and researchers for further

[Efficacy of a massage and exercise programme on the ankle-brachial index and blood pressure in patients with diabetes mellitus type 2 and peripheral arterial disease: a randomized clinical trial].

PubMed

Castro-Sánchez, Adelaida María; Moreno-Lorenzo, Carmen; Matarán-Peñarrocha, Guillermo A; Feriche-Fernández-Castanys, Belén; Sánchez Labraca, Nuria; Sánchez Joya, María del Mar

2010-02-06

Type 2 diabetes mellitus is a highly prevalent disease that can favour the development of peripheral arterial disease. The objective of this study was to analyse the efficacy of a massage and exercise programme on the ankle-brachial index and arterial pressure of patients with diabetes mellitus type 2 and peripheral arterial disease. An experimental study with placebo control group was performed. Sixty-six type 2 diabetes patients with Leriche-Fontaine stage II peripheral arterial disease were randomly assigned to an intervention (exercise and massage) or placebo control (simulated magnetotherapy) group. Study variables were arterial pressure and ankle-brachial index. After 10 weeks of treatment, significant (P<0.05) differences between the intervention and placebo groups were found in right and left ankle-brachial index values and in systolic and diastolic pressures in right and left lower extremities. A combined programme of exercise and massage improves arterial blood pressure and ankle brachial index values in type 2 diabetics with peripheral arterial disease. Copyright 2009 Elsevier España, S.L. All rights reserved.

A Bibliometric Assessment of Global Ice Bucket Challenge (Amyotrophic Lateral Sclerosis) Research.

PubMed

Ram, Shri

2016-10-01

This study is a quantitative and qualitative assessment of the global research trends on amyotrophic lateral sclerosis (ALS) (popularly known as Ice Bucket Challenge), through related literatures retrieved from SCOPUS multidisciplinary database for the period 1974-2013. This study is aimed at analyzing the literature on ALS in terms of document type, language, annual growth, productive country, journal, authors, subject, and most cited articles. The bibliographic data for this study was retrieved from the SCOPUS database using keywords 'amyotrophic lateral sclerosis', 'motor neurone disease', 'Charcot disease', 'Lou Gehrig's disease', 'Ice Bucket Challenge' available in title, abstract, and keyword fields of Scopus database from 1974 to 2013. The literature analysis included 21,750 articles during the period from 1974 to 2013 in different areas of ALS. USA was the most productive country in terms of literature produced, while Neurology was the most productive journal. An intensive awareness created by 'Ice Bucket Challenge' has attracted masses, and an intensive growth of literature is pertinent on ALS. The results of this study are expressed in terms of growth of literature, output of individual countries, and authors, and will be helpful in collaborative research in future.

SEPT9 Mutations and a Conserved 17q25 Sequence in Sporadic and Hereditary Brachial Plexus Neuropathy

PubMed Central

Klein, Christopher J.; Wu, Yanhong; Cunningham, Julie M.; Windebank, Anthony J.; Dyck, P. James B.; Friedenberg, Scott M.; Klein, Diane M.; Dyck, Peter J.

2009-01-01

Background The clinical characteristics of sporadic brachial plexus neuropathy (S-BPN) and hereditary brachial plexus neuropathy (H-BPN) are similar. At times of attack inflammation in brachial plexus nerves has been identified in both conditions. SEPT-9 mutations (Arg88Trp, Ser93Phe, 5UTR-131G to C) occur in some families with H-BPN. These mutations were not found in American H-BPN kindreds with a conserved 500 Kb sequence of DNA at 17q25 (the location of SEPT-9) where a founder mutation has been suggested. Objective To study 17q25 and SEPT-9 in S-BPN (56 patients) and H-BPN (13 kindreds). Methods Allele analysis at 17q25, SEPT-9 DNA sequencing and mRNA analysis from lymphoblast cultures. Results A conserved 17q25 sequence was found in 5 of 13 H-BPN kindreds and one S-BPN patient. This conserved sequence was not found in the family with a SEPT-9 mutation (Arg88Trp) or controls (182). SEPT-9 mRNA expression did not differ between forms of H-BPN and controls. No known mutations of SEPT-9 were found in S-BPN. Conclusions/Relevance Rare S-BPN patients have the same conserved 17q25 sequence found in many American H-BPN kindreds. BPN patients with this conserved sequence do not appear to have SEPT-9 mutations or alterations of its mRNA expression levels in lymphoblast cultures. BPN patients with this conserved sequence may have the most common genetic cause in the Americas by a founder effect mutation. PMID:19204161

Prevention of brachial plexus injury-12 years of shoulder dystocia training: an interrupted time-series study.

PubMed

Crofts, J F; Lenguerrand, E; Bentham, G L; Tawfik, S; Claireaux, H A; Odd, D; Fox, R; Draycott, T J

2016-01-01

To investigate management and outcomes of incidences of shoulder dystocia in the 12 years following the introduction of an obstetric emergencies training programme. Interrupted time-series study comparing management and neonatal outcome of births complicated by shoulder dystocia over three 4-year periods: (i) Pre-training (1996-99), (ii) Early training (2001-04), and (iii) Late training (2009-12). Southmead Hospital, Bristol, UK, with approximately 6000 births per annum. Infants and their mothers who experienced shoulder dystocia. A bi-monthly multi-professional 1-day intrapartum emergencies training course, that included a 30-minute practical session on shoulder dystocia management, commenced in 2000. Neonatal morbidity (brachial plexus injury, humeral fracture, clavicular fracture, 5-minute Apgar score <7) and documented management of shoulder dystocia (resolution manoeuvres performed, traction applied, head-to-body delivery interval). Compliance with national guidance improved with continued training. At least one recognised resolution manoeuvre was used in 99.8% (561/562) of cases of shoulder dystocia in the late training period, demonstrating a continued improvement from 46.3% (150/324, P < 0.001) pre-training, and 92% (241/262, P < 0.001) in the early training period. In parallel there was reduction in the brachial plexus injury at birth (24/324 [7.4%, P < 0.01], pre-training, 6/262 [2.3%] early training, and 7/562 [1.3%] late training. There are significant benefits to long-term, embedded training programmes with improvements in both management and outcomes. A decade after the introduction of training there were no cases of brachial plexus injury lasting over 12 months in 562 cases of shoulder dystocia. © 2015 Royal College of Obstetricians and Gynaecologists.

Outcomes associated with a structured prenatal counseling program for shoulder dystocia with brachial plexus injury.

PubMed

Daly, Mary Veronica; Bender, Christina; Townsend, Kathryn E; Hamilton, Emily F

2012-08-01

We examined outcomes that were associated with a novel program to identify patients who are at high risk for shoulder dystocia with brachial plexus injury. The program included a checklist of key risk factors and a multifactorial algorithm to estimate risk of shoulder dystocia with brachial plexus injury. We examined rates of cesarean delivery and shoulder dystocia in 8767 deliveries by clinicians who were enrolled in the program and in 11,958 patients of clinicians with no access to the program. Key risk factors were identified in 1071 of 8767 mothers (12.2%), of whom 40 of 8767 women (0.46%) had results in the high-risk category. The rate of primary cesarean delivery rate was stable (21.2-20.8%; P = .57). Shoulder dystocia rates fell by 56.8% (1.74-0.75%; P = .002). The rates of shoulder dystocia and cesarean birth showed no changes in the group with no access to the program. With the introduction of this program, overall shoulder dystocia rates fell by more than one-half with no increase in the primary cesarean delivery rate. Copyright © 2012 Mosby, Inc. All rights reserved.

78 FR 43003 - Biomedical Laboratory Research and Development Service, Special Emphasis Panel-Amyotrophic...

Federal Register 2010, 2011, 2012, 2013, 2014

2013-07-18

... Emphasis Panel--Amyotrophic Lateral Sclerosis (ALS) Brain Bank, Notice of Meeting The Department of... Special Emphasis Panel--ALS Brain Bank will meet on July 23, 2013, from 9 a.m. until 4 p.m. in Building 57... VA ALS Brain Bank for its continued funding. The VA ALS Brain Bank is a project of high programmatic...

Transplantation of autologous peripheral blood mononuclear cells in the subarachnoid space for amyotrophic lateral sclerosis: a safety analysis of 14 patients

PubMed Central

Li, Xiao-yan; Liang, Zhan-hua; Han, Chao; Wei, Wen-juan; Song, Chun-li; Zhou, Li-na; Liu, Yang; Li, Ying; Ji, Xiao-fei; Liu, Jing

2017-01-01

There is a small amount of clinical data regarding the safety and feasibility of autologous peripheral blood mononuclear cell transplantation into the subarachnoid space for the treatment of amyotrophic lateral sclerosis. The objectives of this retrospective study were to assess the safety and efficacy of peripheral blood mononuclear cell transplantation in 14 amyotrophic lateral sclerosis patients to provide more objective data for future clinical trials. After stem cell mobilization and collection, autologous peripheral blood mononuclear cells (1 × 109) were isolated and directly transplanted into the subarachnoid space of amyotrophic lateral sclerosis patients. The primary outcome measure was incidence of adverse events. Secondary outcome measures were electromyography 1 week before operation and 4 weeks after operation, Functional Independence Measurement, Berg Balance Scale, and Dysarthria Assessment Scale 1 week preoperatively and 1, 2, 4 and 12 weeks postoperatively. There was no immediate or delayed transplant-related cytotoxicity. The number of leukocytes, serum alanine aminotransferase and creatinine levels, and body temperature were within the normal ranges. Radiographic evaluation showed no serious transplant-related adverse events. Muscle strength grade, results of Functional Independence Measurement, Berg Balance Scale, and Dysarthria Assessment Scale were not significantly different before and after treatment. These findings suggest that peripheral blood mononuclear cell transplantation into the subarachnoid space for the treatment of amyotrophic lateral sclerosis is safe, but its therapeutic effect is not remarkable. Thus, a large-sample investigation is needed to assess its efficacy further. PMID:28469667

Increased phospho-adducin immunoreactivity in a murine model of amyotrophic lateral sclerosis.

PubMed

Shan, X; Hu, J H; Cayabyab, F S; Krieger, C

2005-01-01

Adducins alpha, beta and gamma are proteins that link spectrin and actin in the regulation of cytoskeletal architecture and are substrates for protein kinase C and other signaling molecules. Previous studies have shown that expressions of phosphorylated adducin (phospho-adducin) and protein kinase C are increased in spinal cord tissue from patients who died with amyotrophic lateral sclerosis, a neurodegenerative disorder of motoneurons and other cells. However, the distribution of phospho-adducin immunoreactivity has not been described in the mammalian spinal cord. We have evaluated the distribution of immunoreactivity to serine/threonine-dependent phospho-adducin at a region corresponding to the myristoylated alanine-rich C kinase substrate-related domain of adducin in spinal cords of mice over-expressing mutant human superoxide dismutase, an animal model of amyotrophic lateral sclerosis, and in control littermates. We find phospho-adducin immunoreactivity in control spinal cord in ependymal cells surrounding the central canal, neurons and astrocytes. Phospho-adducin immunoreactivity is localized to the cell bodies, dendrites and axons of some motoneurons, as well as to astrocytes in the gray and white matter. Spinal cords of mutant human superoxide dismutase mice having motoneuron loss exhibit significantly increased phospho-adducin immunoreactivity in ventral and dorsal horn spinal cord regions, but not in ependyma surrounding the central canal, compared with control animals. Increased phospho-adducin immunoreactivity localizes predominantly to astrocytes and likely increases as a consequence of the astrogliosis that occurs in the mutant human superoxide dismutase mouse with disease progression. These findings demonstrate increased immunoreactivity against phosphorylated adducin at the myristoylated alanine-rich C kinase substrate domain in a murine model of amyotrophic lateral sclerosis. As adducin is a substrate for protein kinase C at the myristoylated

Methods of Communication at End of Life for the Person with Amyotrophic Lateral Sclerosis

ERIC Educational Resources Information Center

Brownlee, Alisa; Bruening, Lisa M.

2012-01-01

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that results in loss of most motor functions by the time of death. Most persons with ALS experience a dysarthria that eventually renders oral/vocal communication unintelligible. This article reviews the communication needs of persons with ALS and the range of communication…

Risk Factors for Brachial Plexus Birth Injury

PubMed Central

Louden, Emily; Marcotte, Michael; Mehlman, Charles; Lippert, William; Huang, Bin; Paulson, Andrea

2018-01-01

Over the course of decades, the incidence of brachial plexus birth injury (BPBI) has increased despite advances in healthcare which would seem to assist in decreasing the rate. The aim of this study is to identify previously unknown risk factors for BPBI and the risk factors with potential to guide preventative measures. A case control study of 52 mothers who had delivered a child with a BPBI injury and 132 mothers who had delivered without BPBI injury was conducted. Univariate, multivariable and logistic regressions identified risk factors and their combinations. The odds of BPBI were 2.5 times higher when oxytocin was used and 3.7 times higher when tachysystole occurred. The odds of BPBI injury are increased when tachysystole and oxytocin occur during the mother’s labor. Logistic regression identified a higher risk for BPBI when more than three of the following variables (>30 lbs gained during the pregnancy, stage 2 labor >61.5 min, mother’s age >26.4 years, tachysystole, or fetal malpresentation) were present in any combination. PMID:29596309

Automated analysis of brachial ultrasound time series

NASA Astrophysics Data System (ADS)

Liang, Weidong; Browning, Roger L.; Lauer, Ronald M.; Sonka, Milan

1998-07-01

Atherosclerosis begins in childhood with the accumulation of lipid in the intima of arteries to form fatty streaks, advances through adult life when occlusive vascular disease may result in coronary heart disease, stroke and peripheral vascular disease. Non-invasive B-mode ultrasound has been found useful in studying risk factors in the symptom-free population. Large amount of data is acquired from continuous imaging of the vessels in a large study population. A high quality brachial vessel diameter measurement method is necessary such that accurate diameters can be measured consistently in all frames in a sequence, across different observers. Though human expert has the advantage over automated computer methods in recognizing noise during diameter measurement, manual measurement suffers from inter- and intra-observer variability. It is also time-consuming. An automated measurement method is presented in this paper which utilizes quality assurance approaches to adapt to specific image features, to recognize and minimize the noise effect. Experimental results showed the method's potential for clinical usage in the epidemiological studies.

A Thematic Analysis of Online Discussion Boards for Brachial Plexus Injury.

PubMed

Morris, Marie T; Daluiski, Aaron; Dy, Christopher J

2016-08-01

Patients with brachial plexus injury (BPI) and their family members contribute to Internet discussion groups dedicated to BPI. We hypothesized that a thematic analysis of posts from BPI Internet discussion groups would reveal common themes related to the BPI patient experience, providing topics for patient education and counseling. Internet discussion boards were identified using the search term "brachial plexus injury support group" in Google, Bing, and Yahoo! search engines. Two discussion boards had substantially more posts than other Web sites and were chosen for analyses. Posts from January 1, 2015, through January 1, 2016, were examined. Using an iterative and established process, 2 investigators (M.T.M. and C.J.D) independently analyzed each post using thematic analysis in 3 steps (open coding, axial coding, and selective coding) to determine common themes. In this process, each post was reviewed 3 times. A total of 328 posts from the 2 leading discussion boards were analyzed. Investigators reached a consensus on themes for all posts. One central theme focused on emotional aspects of BPI. Four other central themes regarding information support were identified: BPI disease, BPI treatment, recovery after BPI treatment, and process of seeking care for BPI. Examination of posts on Internet support groups for BPI revealed recurring concerns, questions, and opinions of patients and their family members. The most common themes related to disease information, treatment, recovery, and the emotional element of BPI. These findings provide a helpful starting point in refining topics for patient education and support that are targeted on patients' interests and concerns. Copyright © 2016 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

Structural MRI correlates of amyotrophic lateral sclerosis progression.

PubMed

Senda, Joe; Atsuta, Naoki; Watanabe, Hirohisa; Bagarinao, Epifanio; Imai, Kazunori; Yokoi, Daichi; Riku, Yuichi; Masuda, Michihito; Nakamura, Ryoichi; Watanabe, Hazuki; Ito, Mizuki; Katsuno, Masahisa; Naganawa, Shinji; Sobue, Gen

2017-11-01

Amyotrophic lateral sclerosis (ALS) presents with varying degrees of brain degeneration that can extend beyond the corticospinal tract (CST). Furthermore, the clinical course and progression of ALS varies widely. Brain degeneration detected using structural MRI could reflect disease progression. On study registration, 3-Tesla volumetric MRI and diffusion tensor imaging scans were obtained at baseline in 38 healthy controls and 67 patients with sporadic ALS. Patients had Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) scores of ≥36 and did not have the chromosome 9, open reading frame 72 repeat expansion. Six months later, changes in ALSFRS-R (ΔALSFRS-R) scores were calculated and patients were grouped into three categories, namely, patients with slow progression with ΔALSFRS-R scores ≤3 (n=19), intermediate progression with ΔALSFRS-R scores =4, 5 and 6 (n=36) and rapid progression with ΔALSFRS-R scores ≥7 (n=12). We analysed voxel-based morphometry and tract-based spatial statistics among these subgroups and controls. In comparison with controls, patients with ALS showed grey matter atrophy and decreased fractional anisotropy beyond the motor cortex and CST, especially in the frontotemporal lobes and basal ganglia. Moreover, the degree of change was highly proportional to ΔALSFRS-R at the 6-month assessment. A more rapid disease progression and poorer functional decline were associated with greater involvement of the extra-motor cortex and basal ganglia, suggesting that the spatial extent of brain involvement can be an indicator of the progression in ALS. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Depression and anxiety in a case series of amyotrophic lateral sclerosis: frequency and association with clinical features

PubMed Central

Prado, Laura de Godoy Rousseff; Bicalho, Isabella Carolina Santos; Vidigal-Lopes, Mauro; Prado, Vitor de Godoy Rousseff; Gomez, Rodrigo Santiago; de Souza, Leonardo Cruz; Teixeira, Antônio Lúcio

2017-01-01

ABSTRACT Objective To investigate the frequency of anxiety and depression and their association with clinical features of amyotrophic lateral sclerosis. Methods This is a cross-sectional and descriptive study including a consecutive series of patients with sporadic amyotrophic lateral sclerosis according to Awaji’s criteria. Patients underwent clinical and psychiatric assessment (anxiety and depression symptoms). Results We included 76 patients. The men/women ratio was 1.6:1. Participants’ mean age at disease onset was 55 years (SD±12.1). Sixty-six patients (86.8%) were able to complete psychiatric evaluation. Clinically significant anxiety was found in 23 patients (34.8%) while clinically significant depression was found in 24 patients (36.4%). When we compared patients with and without depression a significant difference was seen only in the frequency of anxiety symptoms (p<0.001). We did further analysis comparing subgroups of patients classified according to the presence or not of anxiety and or depression, without any significant difference regarding sex, age at onset, initial form, disease duration or functional measures. A positive correlation between anxiety and depressive symptoms was found (p<0.001). Conclusion Anxiety and depressive symptoms were highly correlated and frequent in patients with amyotrophic lateral sclerosis. In addition, anxiety and depression were not associated with disease duration and presentation, sex, age at onset, and functional score. PMID:28444090

MR Neurography of Brachial Plexus at 3.0 T with Robust Fat and Blood Suppression.

PubMed

Wang, Xinzeng; Harrison, Crystal; Mariappan, Yogesh K; Gopalakrishnan, Karthik; Chhabra, Avneesh; Lenkinski, Robert E; Madhuranthakam, Ananth J

2017-05-01

Purpose To develop and evaluate magnetic resonance (MR) neurography of the brachial plexus with robust fat and blood suppression for increased conspicuity of nerves at 3.0 T in clinically feasible acquisition times. Materials and Methods This prospective study was HIPAA compliant, with institutional review board approval and written informed consent. A low-refocusing-flip-angle three-dimensional (3D) turbo spin-echo (TSE) sequence was modified to acquire both in-phase and out-of-phase echoes, required for chemical shift (Dixon) reconstruction, in the same repetition by using partial echoes combined with modified homodyne reconstruction with phase preservation. This multiecho TSE modified Dixon (mDixon) sequence was optimized by using simulations and phantom studies and in three healthy volunteers. The sequence was tested in five healthy volunteers and was evaluated in 10 patients who had been referred for brachial plexopathy at 3.0 T. The images were evaluated against the current standard of care, images acquired with a 3D TSE short inversion time inversion recovery (STIR) sequence, qualitatively by using the Wilcoxon signed-rank test and quantitatively by using the Friedman two-way analysis of variance, with P < .05 considered to indicate a statistically significant difference. Results Multiecho TSE-mDixon involving partial-echo and homodyne reconstruction with phase preservation achieved uniform fat suppression in half the imaging time compared with multiacquisition TSE-mDixon. Compared with 3D TSE STIR, fat suppression, venous suppression, and nerve visualization were significantly improved (P < .05), while arterial suppression was better but not significantly so (P = .06), with increased apparent signal-to-noise ratio in the dorsal nerve root ganglion and C6 nerve (P < .001) with the multiecho TSE-mDixon sequence. Conclusion The multiecho 3D TSE-mDixon sequence provides robust fat and blood suppression, resulting in increased conspicuity of the nerves, in

Depression and Anxiety in Traumatic Brachial Plexus Injury Patients Are Associated With Reduced Motor Outcome After Surgical Intervention for Restoration of Elbow Flexion.

PubMed

Wilson, Thomas J; Chang, Kate W C; Yang, Lynda J-S

2016-06-01

Depression has been associated with poor outcomes in neurosurgical patients, including increased pain, poorer functional recovery, delayed return to work, and decreased patient satisfaction. No reports exist regarding an association of psychiatric diagnoses with outcomes after brachial plexus reconstruction. As outcomes and patient satisfaction become increasingly important to payers and physician reimbursement, assessing modifiable preoperative risk factors for their association with poor outcome and patient satisfaction is imperative. To analyze patients undergoing brachial plexus reconstruction to assess the relationship of depression/anxiety with functional outcome. Data were collected retrospectively on all patients who underwent brachial plexus reconstruction to restore elbow flexion between 2005 and 2013. Elbow flexion, graded via the Medical Research Council scale, was assessed at latest follow-up. Multiple variables, including the presence of Axis I psychiatric diagnoses, were assessed for their association with the dichotomous outcome of Medical Research Council scale score ≥3 (antigravity) vs <3 elbow flexion. Standard statistical methods were used. Thirty-seven patients met inclusion criteria. The median postsurgical follow-up time was 21 months. Operations included neurolysis (n = 3), nerve graft repair (n = 6), and nerve transfer (n = 28). Depression was present in 10 of 37 patients (27%). Of variables tested, only depression was associated with poor elbow flexion outcome (odds ratio: 6.038; P = .04). Preoperative depression is common after brachial plexus injury. The presence of depression is associated with reduced elbow flexion recovery after reconstruction. Our data suggest assessment and treatment of preoperative mental health is important in designing a comprehensive postoperative management plan to optimize outcomes and patient satisfaction. MRC, Medical Research CouncilTBI, traumatic brain injury.

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis.

PubMed

Kabashi, Edor; Valdmanis, Paul N; Dion, Patrick; Spiegelman, Dan; McConkey, Brendan J; Vande Velde, Christine; Bouchard, Jean-Pierre; Lacomblez, Lucette; Pochigaeva, Ksenia; Salachas, Francois; Pradat, Pierre-Francois; Camu, William; Meininger, Vincent; Dupre, Nicolas; Rouleau, Guy A

2008-05-01

Recently, TDP-43 was identified as a key component of ubiquitinated aggregates in amyotrophic lateral sclerosis (ALS), an adult-onset neurological disorder that leads to the degeneration of motor neurons. Here we report eight missense mutations in nine individuals--six from individuals with sporadic ALS (SALS) and three from those with familial ALS (FALS)--and a concurring increase of a smaller TDP-43 product. These findings further corroborate that TDP-43 is involved in ALS pathogenesis.

The role of exercise in amyotrophic lateral sclerosis.

PubMed

Chen, Amy; Montes, Jacqueline; Mitsumoto, Hiroshi

2008-08-01

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting the motor nervous system. It causes progressive and cumulative physical disabilities in patients, and leads to eventual death due to respiratory muscle failure. The disease is diverse in its presentation, course, and progression. We do not yet fully understand the cause or causes of the disease, nor the mechanisms for its progression; thus, we lack effective means for treating this disease. Currently, we rely on a multidisciplinary approach to symptomatically manage and care for patients who have ALS. In this article, the authors review the literature on the role of exercise in patients who have ALS, and briefly compare what is known about exercise in other neuromuscular diseases.

Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?

PubMed Central

Webster, Christopher P.; Smith, Emma F.; Shaw, Pamela J.; De Vos, Kurt J.

2017-01-01

Protein homeostasis (proteostasis), the correct balance between production and degradation of proteins, is essential for the health and survival of cells. Proteostasis requires an intricate network of protein quality control pathways (the proteostasis network) that work to prevent protein aggregation and maintain proteome health throughout the lifespan of the cell. Collapse of proteostasis has been implicated in the etiology of a number of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), the most common adult onset motor neuron disorder. Here, we review the evidence linking dysfunctional proteostasis to the etiology of ALS and discuss how ALS-associated insults affect the proteostasis network. Finally, we discuss the potential therapeutic benefit of proteostasis network modulation in ALS. PMID:28512398

Genetics of amyotrophic lateral sclerosis: an update

PubMed Central

2013-01-01

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder involving both upper motor neurons (UMN) and lower motor neurons (LMN). Enormous research has been done in the past few decades in unveiling the genetics of ALS, successfully identifying at least fifteen candidate genes associated with familial and sporadic ALS. Numerous studies attempting to define the pathogenesis of ALS have identified several plausible determinants and molecular pathways leading to motor neuron degeneration, which include oxidative stress, glutamate excitotoxicity, apoptosis, abnormal neurofilament function, protein misfolding and subsequent aggregation, impairment of RNA processing, defects in axonal transport, changes in endosomal trafficking, increased inflammation, and mitochondrial dysfunction. This review is to update the recent discoveries in genetics of ALS, which may provide insight information to help us better understanding of the disease neuropathogenesis. PMID:23941283

A comprehensive review of amyotrophic lateral sclerosis

PubMed Central

Zarei, Sara; Carr, Karen; Reiley, Luz; Diaz, Kelvin; Guerra, Orleiquis; Altamirano, Pablo Fernandez; Pagani, Wilfredo; Lodin, Daud; Orozco, Gloria; Chinea, Angel

2015-01-01

Amyotrophic lateral sclerosis (ALS) is a late-onset fatal neurodegenerative disease affecting motor neurons with an incidence of about 1/100,000. Most ALS cases are sporadic, but 5–10% of the cases are familial ALS. Both sporadic and familial ALS (FALS) are associated with degeneration of cortical and spinal motor neurons. The etiology of ALS remains unknown. However, mutations of superoxide dismutase 1 have been known as the most common cause of FALS. In this study, we provide a comprehensive review of ALS. We cover all aspects of the disease including epidemiology, comorbidities, environmental risk factor, molecular mechanism, genetic factors, symptoms, diagnostic, treatment, and even the available supplement and management of ALS. This will provide the reader with an advantage of receiving a broad range of information about the disease. PMID:26629397

Therapeutic neuroprotective agents for amyotrophic lateral sclerosis

PubMed Central

Pandya, Rachna S.; Zhu, Haining; Li, Wei; Bowser, Robert; Friedlander, Robert M.

2014-01-01

Amyotrophic lateral sclerosis (ALS) is a fatal chronic neurodegenerative disease whose hallmark is proteinaceous, ubiquitinated, cytoplasmic inclusions in motor neurons and surrounding cells. Multiple mechanisms proposed as responsible for ALS pathogenesis include dysfunction of protein degradation, glutamate excitotoxicity, mitochondrial dysfunction, apoptosis, oxidative stress, and inflammation. It is therefore essential to gain a better understanding of the underlying disease etiology and search for neuroprotective agents that might delay disease onset, slow progression, prolong survival, and ultimately reduce the burden of disease. Because riluzole, the only Food and Drug Administration (FDA)-approved treatment, prolongs the ALS patient’s life by only 3 months, new therapeutic agents are urgently needed. In this review, we focus on studies of various small pharmacological compounds targeting the proposed pathogenic mechanisms of ALS and discuss their impact on disease progression. PMID:23864030

Influence of arm position on catheter placement during real-time ultrasound-guided right infraclavicular proximal axillary venous catheterization.

PubMed

Ahn, J H; Kim, I S; Shin, K M; Kang, S S; Hong, S J; Park, J H; Kim, H J; Lee, S H; Kim, D Y; Jung, J H

2016-03-01

Real-time ultrasound-guided infraclavicular proximal axillary venous catheterization is used in many clinical situations and provides the advantages of catheter stabilization, a reduced risk of catheter-related infection, and comfort for the patient without limitation of movement. However, unintended catheter tip dislocation and accidental arterial puncture occur occasionally. This study was designed to investigate the influence of arm position on catheter placement and complications. Patients were randomized to either the neutral group (n=240) or the abduction group (n=241). In the neutral group, patients were positioned with the head and shoulders placed in an anatomically neutral position and the arms kept by the side during catheterization. In the abduction group, the right upper arm was abducted at 90° from the trunk during catheterization. After real-time ultrasound-guided catheterization was carried out in the right infraclavicular proximal axillary vein, misplacement of the catheter and all complications were evaluated with ultrasound and chest radiography. The success rate of complete catheterization before evaluating the placement of the catheter was high in both groups (97.1 vs 98.8%, P=not significant). The incidence of accidental arterial puncture was not different (1.7 vs 0%, P=not significant). The incidence of misplacement of the catheter was higher in the neutral group than in the abduction group (3.9 vs 0.4%, P=0.01). There were no complications, such as haemothorax, pneumothorax, or injury to the brachial plexus and phrenic nerve, in either group. Upper arm abduction may minimize the risk of misplacement of the catheter during real-time ultrasound-guided infraclavicular proximal axillary venous catheterization. The trial was registered with the Clinical Trial Registry of Korea: https://cris.nih.go.kr/cris/index.jsp. Identifier: KCT0001417. © The Author 2015. Published by Oxford University Press on behalf of the British Journal of Anaesthesia

True Brachial Artery Aneurysm after Arteriovenous Fistula for Hemodialysis: Five Cases and Literature Review.

PubMed

Fendri, Jihene; Palcau, Laura; Cameliere, Lucie; Coffin, Olivier; Felisaz, Aurelien; Gouicem, Djelloul; Dufranc, Julie; Laneelle, Damien; Berger, Ludovic

2017-02-01

The donor artery after a long-standing arteriovenous fistula (AVF) for hemodialysis usually evolves exceptionally toward a true aneurysmal degeneration (AD). The purpose of this article was to describe true brachial artery AD in end-stage renal disease patients after AVF creation, as well as its influencing factors and treatment strategies. We present a retrospective, observational, single-center study realized in Caen University Hospital's Vascular Surgery Department from May 1996 to November 2015. The inclusion criteria were true AD of the brachial artery after a vascular access for hemodialysis. A literature research, using the same criteria, was performed on the articles published between 1994 and 2015. The used databases included MEDLINE (via PubMed), EMBASE via OVID, Cochrane Library Database, and ResearchGate. Our series includes 5 patients. Twenty-one articles were found in the literature: 17 case reports, 3 series, and 1 review. The same triggering factors for AD (high flow and immunosuppressive treatment) were found. The mean age at the time of AVF creation, first renal transplantation, and AD's diagnosis were respectively 26 (range 15-49), 29.2, and 48.6 years (range 37-76) in our series versus 34 (range 27-39), 40.4 (range 28-55), and 55.5 years (range 35-75) in cases found in the literature. The time spread after AVF creation and aneurysmal diagnosis was about 20.6 years (range 18-25) in our study versus 20.5 years (range 9-29) in the case reports. Our surgical attitude corresponds principally to that described in the literature. Nevertheless, we describe for the first time one case of arterial transposition to exclude the brachial aneurysm using superficial femoral artery. Arterial aneurysm is a rare, but significant complication after a long-term creation of hemodialysis access. High flow and immunosuppression may accelerate this process. Young age of the patients may act as a benefic factor and delay the AD. Arterial transposition could be an

Outcome following nonoperative treatment of brachial plexus birth injuries.

PubMed

DiTaranto, Patricia; Campagna, Liliana; Price, Andrew E; Grossman, John A I

2004-02-01

Ninety-one infants who sustained a brachial plexus birth injury were treated with only physical and occupational therapy. The children were evaluated at 3-month intervals and followed for a minimum of 2 years. Sixty-three children with an upper or upper-middle plexus injury recovered good to excellent shoulder and hand function. In all of these children, critical marker muscles recovered M4 by 6 months of age. Twelve infants sustained a global palsy, with critical marker muscles remaining at M0-M1 at 6 months, resulting in a useless extremity. Sixteen infants with upper and upper-middle plexus injuries failed to recover greater than M1-M2 deltoid and biceps by 6 months, resulting in a very poor final outcome. These data provide useful guidelines for selection of infants for surgical reconstruction to improve ultimate outcome.

Sedentary Behavior and Light Physical Activity Are Associated with Brachial and Central Blood Pressure in Hypertensive Patients

PubMed Central

Gerage, Aline M.; Benedetti, Tania R. B.; Farah, Breno Q.; Santana, Fábio da S.; Ohara, David; Andersen, Lars B.; Ritti-Dias, Raphael M.

2015-01-01

Background Physical activity is recommended as a part of a comprehensive lifestyle approach in the treatment of hypertension, but there is a lack of data about the relationship between different intensities of physical activity and cardiovascular parameters in hypertensive patients. The purpose of this study was to investigate the association between the time spent in physical activities of different intensities and blood pressure levels, arterial stiffness and autonomic modulation in hypertensive patients. Methods In this cross-sectional study, 87 hypertensive patients (57.5 ± 9.9 years of age) had their physical activity assessed over a 7 day period using an accelerometer and the time spent in sedentary activities, light physical activities, moderate physical activities and moderate-to-vigorous physical activities was obtained. The primary outcomes were brachial and central blood pressure. Arterial stiffness parameters (augmentation index and pulse wave velocity) and cardiac autonomic modulation (sympathetic and parasympathetic modulation in the heart) were also obtained as secondary outcomes. Results Sedentary activities and light physical activities were positively and inversely associated, respectively, with brachial systolic (r = 0.56; P < 0.01), central systolic (r = 0.51; P < 0.05), brachial diastolic (r = 0.45; P < 0.01) and central diastolic (r = 0.42; P < 0.05) blood pressures, after adjustment for sex, age, trunk fat, number of antihypertensive drugs, accelerometer wear time and moderate-to-vigorous physical activities. Arterial stiffness parameters and cardiac autonomic modulation were not associated with the time spent in sedentary activities and in light physical activities (P > 0.05). Conclusion Lower time spent in sedentary activities and higher time spent in light physical activities are associated with lower blood pressure, without affecting arterial stiffness and cardiac autonomic modulation in hypertensive patients. PMID:26717310

A comparative study of clonidine and dexmedetomidine as an adjunct to bupivacaine in supraclavicular brachial plexus block.

PubMed

Tripathi, Archana; Sharma, Khushboo; Somvanshi, Mukesh; Samal, Rajib Lochan

2016-01-01

Various additives are mixed with local anesthetic agents to increase the quality of block in regional anesthesia. We compared clonidine and dexmedetomidine as an adjunct to bupivacaine in supraclavicular brachial plexus block with respect to the onset and duration of sensory and motor block and duration of analgesia. Sixty American Society of Anesthesiologists Grades I and II patients scheduled for various orthopedic surgeries of the upper limb under supraclavicular brachial plexus block were divided into two equal groups in a randomized, double-blind manner. Patients were assigned randomly to one of the two groups. In Group C (n = 30), 39 ml of 0.25% bupivacaine plus 1 ml (1 μg/kg) clonidine and in Group D (n = 30), 39 ml of 0.25% bupivacaine plus 1 ml (1 μg/kg) dexmedetomidine were given. The onset and duration of sensory and motor block, duration of analgesia, and quality of anesthesia were studied in both the groups. There was no statistically significant difference in the onset of sensory and motor block in both the groups. The durations of sensory and motor block were 316.67 ± 45.21 and 372.67 ± 44.48 min, respectively, in Group C, whereas they were 502.67 ± 43.78 and 557.67 ± 38.83 min, respectively, in Group D. The duration of analgesia was 349.33 ± 42.91 min, significantly less in Group C compared to 525.33 ± 42.89 min in Group D (P < 0.001). The quality of anesthesia was significantly better in dexmedetomidine group compared to clonidine group (P < 0.001). The addition of dexmedetomidine prolongs the durations of sensory and motor block and duration of analgesia and improves the quality of anesthesia as compared with clonidine when injected with bupivacaine in supraclavicular brachial plexus block.

Evaluation of nerve transfer options for treating total brachial plexus avulsion injury: a retrospective study of 73 participants

PubMed Central

Gao, Kai-ming; Hu, Jing-jing; Lao, Jie; Zhao, Xin

2018-01-01

Despite recent great progress in diagnosis and microsurgical repair, the prognosis in total brachial plexus-avulsion injury remains unfavorable. Insufficient number of donors and unreasonable use of donor nerves might be key factors. To identify an optimal treatment strategy for this condition, we conducted a retrospective review. Seventy-three patients with total brachial plexus avulsion injury were followed up for an average of 7.3 years. Our analysis demonstrated no significant difference in elbow-flexion recovery between phrenic nerve-transfer (25 cases), phrenic nerve-graft (19 cases), intercostal nerve (17 cases), or contralateral C7-transfer (12 cases) groups. Restoration of shoulder function was attempted through anterior accessory nerve (27 cases), posterior accessory nerve (10 cases), intercostal nerve (5 cases), or accessory + intercostal nerve transfer (31 cases). Accessory nerve + intercostal nerve transfer was the most effective method. A significantly greater amount of elbow extension was observed in patients with intercostal nerve transfer (25 cases) than in those with contralateral C7 transfer (10 cases). Recovery of median nerve function was noticeably better for those who received entire contralateral C7 transfer (33 cases) than for those who received partial contralateral C7 transfer (40 cases). Wrist and finger extension were reconstructed by intercostal nerve transfer (31 cases). Overall, the recommended surgical treatment for total brachial plexus-avulsion injury is phrenic nerve transfer for elbow flexion, accessory nerve + intercostal nerve transfer for shoulder function, intercostal nerves transfer for elbow extension, entire contralateral C7 transfer for median nerve function, and intercostal nerve transfer for finger extension. The trial was registered at ClinicalTrials.gov (identifier: NCT03166033). PMID:29623932

Evaluation of nerve transfer options for treating total brachial plexus avulsion injury: A retrospective study of 73 participants.

PubMed

Gao, Kai-Ming; Hu, Jing-Jing; Lao, Jie; Zhao, Xin

2018-03-01

Despite recent great progress in diagnosis and microsurgical repair, the prognosis in total brachial plexus-avulsion injury remains unfavorable. Insufficient number of donors and unreasonable use of donor nerves might be key factors. To identify an optimal treatment strategy for this condition, we conducted a retrospective review. Seventy-three patients with total brachial plexus avulsion injury were followed up for an average of 7.3 years. Our analysis demonstrated no significant difference in elbow-flexion recovery between phrenic nerve-transfer (25 cases), phrenic nerve-graft (19 cases), intercostal nerve (17 cases), or contralateral C 7 -transfer (12 cases) groups. Restoration of shoulder function was attempted through anterior accessory nerve (27 cases), posterior accessory nerve (10 cases), intercostal nerve (5 cases), or accessory + intercostal nerve transfer (31 cases). Accessory nerve + intercostal nerve transfer was the most effective method. A significantly greater amount of elbow extension was observed in patients with intercostal nerve transfer (25 cases) than in those with contralateral C 7 transfer (10 cases). Recovery of median nerve function was noticeably better for those who received entire contralateral C 7 transfer (33 cases) than for those who received partial contralateral C 7 transfer (40 cases). Wrist and finger extension were reconstructed by intercostal nerve transfer (31 cases). Overall, the recommended surgical treatment for total brachial plexus-avulsion injury is phrenic nerve transfer for elbow flexion, accessory nerve + intercostal nerve transfer for shoulder function, intercostal nerves transfer for elbow extension, entire contralateral C 7 transfer for median nerve function, and intercostal nerve transfer for finger extension. The trial was registered at ClinicalTrials.gov (identifier: NCT03166033).

Care management in amyotrophic lateral sclerosis.

PubMed

Soriani, M-H; Desnuelle, C

2017-05-01

Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive and fatal neurodegenerative disease characterized by progressive weakness of voluntary muscles of movement as well as those for swallowing, speech and respiration. In the absence of curative treatment, care can improve quality of life, prolong survival, and support ALS patients and their families, and also help them to anticipate and prepare for the end of life. Multidisciplinary management in tertiary centers is recommended in close collaboration with general practitioners, home carers and a dedicated health network. Patients' follow-up deals mainly with motor impairment and physical disability, adaptation, nutrition and respiratory function. Involvement of palliative care as part of the multidisciplinary team management offers patients the possibility of discussing their end of life issues. This review summarizes the different aspects of ALS care, from delivering the diagnosis to the end of life, and the organization of its management. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

The effect of pretest rest time on automated measures of toe systolic blood pressure and the toe brachial index.

PubMed

Sadler, Sean G; Hawke, Fiona E; Chuter, Vivienne H

2015-10-01

Evaluation of peripheral blood supply is fundamental to risk categorization and subsequent ongoing monitoring of patients with lower extremity peripheral arterial disease. Toe systolic blood pressure (TSBP) and the toe brachial index (TBI) are both valid and reliable vascular screening techniques that are commonly used in clinical practice. However, the effect of pretest rest duration on the magnitude of these measurements is unclear. Eighty individuals meeting current guidelines for lower extremity peripheral arterial disease screening volunteered to participate. The Systoe and MicroLife automated devices were used to measure toe and brachial systolic blood pressures, respectively, following 5, 10 and 15 min of rest in a horizontal supine position. A ratio of TSBP to brachial pressure was used to calculate the TBI and change in TBI at each time interval was investigated. A significant increase in TSBP [3.66 mmHg; 95% confidence interval (CI): 1.44-5.89; P≤0.001] and the TBI (0.03; 95% CI: 0.01-0.05; P≤0.001) occurred between 5 and 10 min. Between 10 and 15 min, there was a nonsignificant decrease in TSBP (-0.73 mmHg; 95% CI: -1.48 to 2.93; P=1.000) and the TBI (0.00; 95% CI: -0.02 to 0.02; P=1.000). Ten minutes of pretest rest is recommended for measurement of TSBP and for both pressure measurements used in the calculation of a TBI to ensure that stable pressures are measured.

Capacitive proximity sensor

DOEpatents

Kronberg, James W.

1994-01-01

A proximity sensor based on a closed field circuit. The circuit comprises a ring oscillator using a symmetrical array of plates that creates an oscillating displacement current. The displacement current varies as a function of the proximity of objects to the plate array. Preferably the plates are in the form of a group of three pair of symmetric plates having a common center, arranged in a hexagonal pattern with opposing plates linked as a pair. The sensor produces logic level pulses suitable for interfacing with a computer or process controller. The proximity sensor can be incorporated into a load cell, a differential pressure gauge, or a device for measuring the consistency of a characteristic of a material where a variation in the consistency causes the dielectric constant of the material to change.

[The plexal hand (involvement of the hand in traumatic lesions of the brachial plexus in adults)].

PubMed

Alnot, J Y; Salon, A

1993-01-01

After spontaneous recovery following brachial plexus injuries, or after nerve regeneration following nervous surgery on traumatic brachial plexus, there usually is a variable residual distal deficit involving the hand and wrist. This deficit is defined by both a functional approach, and its anatomical nervous correspondence, in order to predict which motor muscles will be available for palliative surgery, and to establish the most reasonable therapeutic plan. Total palsy of the hand will only be mentioned, whereas three main presentations of the "plexic hand" are of a greater surgical interest and will be detailed: the "wrist drop", due to C5 C6 +/- C7 supra-clavicular lesions, the hand presenting with a deficit of digit flexion, pinch and intrinsic functions, due to C8 T1 +/- C7 supra-clavicular lesions, non-standardized deficits due to infra- and retro-clavicular lesions, whose therapeutic indications and prognosis are closer to more classical trunk palsies. The review of 44 "plexic hands" after distal palliative surgery indicates the need to modify the fundamental rules of tendinous transfer surgery, and suggests new principles properly adapted to the surgery of plexic hand palsies.

Variations of the origin of collateral branches emerging from the posterior aspect of the brachial plexus

PubMed Central

2007-01-01

Background The frequency of variation found in the arrangement and distribution of the branches in the brachial plexus, make this anatomical region extremely complicated. The medical concerns involved with these variations include anesthetic blocks, surgical approaches, interpreting tumor or traumatic nervous compressions having unexplained clinical symptoms (sensory loss, pain, wakefulness and paresis), and the possibility of these structures becoming compromised. The clinical importance of these variations is discussed in the light of their differential origins. Methods The anatomy of brachial plexus structures from 46 male and 11 female cadaverous specimens were studied. The 40–80 year-old specimens were obtained from the Universidad Industrial de Santander's Medical Faculty's Anatomy Department (dissection laboratory). Parametric measures were used for calculating results. Results Almost half (47.1%) of the evaluated plexuses had collateral variations. Subscapular nerves were the most varied structure, including the presence of a novel accessory nerve. Long thoracic nerve variations were present, as were the absence of C5 or C7 involvement, and late C7 union with C5–C6. Conclusion Further studies are needed to confirm the existence of these variations in a larger sample of cadaver specimens. PMID:17587464

Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review)

PubMed Central

Miller, R G.; Jackson, C E.; Kasarskis, E J.; England, J D.; Forshew, D; Johnston, W; Kalra, S; Katz, J S.; Mitsumoto, H; Rosenfeld, J; Shoesmith, C; Strong, M J.; Woolley, S C.

2009-01-01

Objective: To systematically review evidence bearing on the management of patients with amyotrophic lateral sclerosis (ALS). Methods: The authors analyzed studies from 1998 to 2007 to update the 1999 practice parameter. Topics covered in this section include breaking the news, multidisciplinary clinics, symptom management, cognitive and behavioral impairment, communication, and palliative care for patients with ALS. Results: The authors identified 2 Class I studies, 8 Class II studies, and 30 Class III studies in ALS, but many important areas have been little studied. More high-quality, controlled studies of symptomatic therapies and palliative care are needed to guide management and assess outcomes in patients with ALS. Recommendations: Multidisciplinary clinic referral should be considered for managing patients with ALS to optimize health care delivery and prolong survival (Level B) and may be considered to enhance quality of life (Level C). For the treatment of refractory sialorrhea, botulinum toxin B should be considered (Level B) and low-dose radiation therapy to the salivary glands may be considered (Level C). For treatment of pseudobulbar affect, dextromethorphan and quinidine should be considered if approved by the US Food and Drug Administration (Level B). For patients who develop fatigue while taking riluzole, withholding the drug may be considered (Level C). Because many patients with ALS demonstrate cognitive impairment, which in some cases meets criteria for dementia, screening for cognitive and behavioral impairment should be considered in patients with ALS (Level B). Other management strategies all lack strong evidence. GLOSSARY ALS = amyotrophic lateral sclerosis; ALS-FTD = amyotrophic lateral sclerosis with a dementia meeting the Neary criteria for frontotemporal dementia; ALSbi = amyotrophic lateral sclerosis with behavioral impairment; ALSci = amyotrophic lateral sclerosis with cognitive impairment; BTxA = botulinum toxin type A; BTxB = botulinum

Three-Dimensional Magnetic Resonance Imaging of Glenohumeral Dysplasia in Neonatal Brachial Plexus Palsy.

PubMed

Eismann, Emily A; Laor, Tal; Cornwall, Roger

2016-01-20

Existing quantitative measurements of glenohumeral dysplasia in children with unresolved neonatal brachial plexus palsy (NBPP) have been mostly limited to the axial plane. The purpose of this study was to describe the three-dimensional (3D) pathoanatomy of glenohumeral dysplasia using 3D magnetic resonance imaging (MRI) reformations. 3D MRI reformations of the scapula, glenoid labrum, and proximal part of the humerus were created from a volume-acquisition proton-density-weighted MRI sequence of both the affected and the unaffected shoulder of seventeen children less than six years of age with unresolved NBPP who had not undergone shoulder surgery. Glenoid retroversion and posterior humeral head displacement were measured on axial 2D images. Humeral head displacement in all planes, labral circumference, glenoid retroversion, glenoid declination, and scapular morphometric values were measured on 3D reformations. Contiguity of the humeral head with the labrum and the shape of the glenoid were classified. Measurements were compared between the affected and unaffected sides. On 3D evaluation, the humeral head was completely posteriorly translated in ten patients but was never outside the glenoid labrum. Instead, in these patients, the humeral head was eccentrically articulating with the dysplastic glenoid and was contained by a posteriorly elongated labrum. Glenoid dysplasia was not limited to the axial plane. Less declination of the glenoid in the coronal plane correlated with greater 3D glenoid retroversion. Glenoid retroversion resulted from underdevelopment of the posterior aspect of the glenoid rather than overdevelopment of the anterior aspect of the glenoid. 3D measurements of greater glenoid retroversion and less declination correlated with 2D measurements of glenoid retroversion and posterior humeral head displacement. Posterior humeral head displacement in NBPP should not be considered a simple "dislocation." Glenohumeral dysplasia is not limited to the axial

The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosis.

PubMed

Chen, Han-Jou; Mitchell, Jacqueline C; Novoselov, Sergey; Miller, Jack; Nishimura, Agnes L; Scotter, Emma L; Vance, Caroline A; Cheetham, Michael E; Shaw, Christopher E

2016-05-01

Detergent-resistant, ubiquitinated and hyperphosphorylated Tar DNA binding protein 43 (TDP-43, encoded by TARDBP) neuronal cytoplasmic inclusions are the pathological hallmark in ∼95% of amyotrophic lateral sclerosis and ∼60% of frontotemporal lobar degeneration cases. We sought to explore the role for the heat shock response in the clearance of insoluble TDP-43 in a cellular model of disease and to validate our findings in transgenic mice and human amyotrophic lateral sclerosis tissues. The heat shock response is a stress-responsive protective mechanism regulated by the transcription factor heat shock factor 1 (HSF1), which increases the expression of chaperones that refold damaged misfolded proteins or facilitate their degradation. Here we show that manipulation of the heat shock response by expression of dominant active HSF1 results in a dramatic reduction of insoluble and hyperphosphorylated TDP-43 that enhances cell survival, whereas expression of dominant negative HSF1 leads to enhanced TDP-43 aggregation and hyperphosphorylation. To determine which chaperones were mediating TDP-43 clearance we over-expressed a range of heat shock proteins (HSPs) and identified DNAJB2a (encoded by DNAJB2, and also known as HSJ1a) as a potent anti-aggregation chaperone for TDP-43. DNAJB2a has a J domain, allowing it to interact with HSP70, and ubiquitin interacting motifs, which enable it to engage the degradation of its client proteins. Using functionally deleted DNAJB2a constructs we demonstrated that TDP-43 clearance was J domain-dependent and was not affected by ubiquitin interacting motif deletion or proteasome inhibition. This indicates that TDP-43 is maintained in a soluble state by DNAJB2a, leaving the total levels of TDP-43 unchanged. Additionally, we have demonstrated that the levels of HSF1 and heat shock proteins are significantly reduced in affected neuronal tissues from a TDP-43 transgenic mouse model of amyotrophic lateral sclerosis and patients with

Brachial arteriovenous fistula as a complication of placement of a peripherally inserted central venous catheter: a case report and review of the literature.

PubMed

Tran, Hoang S; Burrows, Brian J; Zang, William A; Han, David C

2006-09-01

Peripherally inserted central venous catheter (PICC) lines have become a frequently used method of intravenous access for long-term administration of antibiotics, chemotherapy, and parenteral nutrition. Catheter-related complications involving the arterial tree are rare. We report a case of a 25-year-old woman with a history of difficult PICC line placement that presented with an arteriovenous fistula in the left arm. Duplex ultrasound confirmed the diagnosis of a brachial artery-to-brachial vein arteriovenous fistula (AVF), and the patient underwent surgical repair. To our knowledge, this is the first reported case of an AVF resulting from PICC line placement. Correction of AVF is indicated to alleviate symptoms as well as to prevent future complications.

Amyotrophic Lateral Sclerosis: A Focus on Disease Progression

PubMed Central

Calvo, Ana C.; Manzano, Raquel; Mendonça, Deise M. F.; Muñoz, María J.; Zaragoza, Pilar

2014-01-01

Since amyotrophic lateral sclerosis (ALS) was discovered and described in 1869 as a neurodegenerative disease in which motor neuron death is induced, a wide range of biomarkers have been selected to identify therapeutic targets. ALS shares altered molecular pathways with other neurodegenerative diseases, such as Alzheimer's, Huntington's, and Parkinson's diseases. However, the molecular targets that directly influence its aggressive nature remain unknown. What is the first link in the neurodegenerative chain of ALS that makes this disease so peculiar? In this review, we will discuss the progression of the disease from the viewpoint of the potential biomarkers described to date in human and animal model samples. Finally, we will consider potential therapeutic strategies for ALS treatment and future, innovative perspectives. PMID:25157374

Phrenic nerve block caused by interscalene brachial plexus block: breathing effects of different sites of injection.

PubMed

Bergmann, Lars; Martini, Stefan; Kesselmeier, Miriam; Armbruster, Wolf; Notheisen, Thomas; Adamzik, Michael; Eichholz, Rϋdiger

2016-07-29

Interscalene brachial plexus (ISB) block is often associated with phrenic nerve block and diaphragmatic paresis. The goal of our study was to test if the anterior or the posterior ultrasound guided approach of the ISB is associated with a lower incidence of phrenic nerve blocks and impaired lung function. This was a prospective, randomized and single-blinded study of 84 patients scheduled for elective shoulder surgery who fullfilled the inclusion and exclusion critereria. Patients were randomized in two groups to receive either the anterior (n = 42) or the posterior (n = 42) approach for ISB. Clinical data were recorded. In both groups patients received ISB with a total injection volume of 15 ml of ropivacaine 1 %. Spirometry was conducted at baseline (T0) and 30 min (T30) after accomplishing the block. Changes in spirometrical variables between T0 and T30 were investigated by Wilcoxon signed-rank test for each puncture approach. The temporal difference between the posterior and the anterior puncture approach groups were again analyzed by the Wilcoxon-Mann-Whitney test. The spirometric results showed a significant decrease in vital capacity, forced expiratory volume per second, and maximum nasal inspiratory breathing after the Interscalene brachial plexus block; indicating a phrenic nerve block (p <0.001, Wilcoxon signed-rank). A significant difference in the development of the spirometric parameters between the anterior and the posterior group could not be identified (Wilcoxon-Mann-Whitney test). Despite the changes in spirometry, no cases of dyspnea were reported. A different site of injection (anterior or posterior) did not show an effect in reducing the cervical block spread of the local anesthetic and the incidence of phrenic nerve blocks during during ultrasound guided Interscalene brachial plexus block. Clinical breathing effects of phrenic nerve blocks are, however, usually well compensated, and subjective dyspnea did not occur in our patients

Capacitive proximity sensor

DOEpatents

Kronberg, J.W.

1994-05-31

A proximity sensor based on a closed field circuit is disclosed. The circuit comprises a ring oscillator using a symmetrical array of plates that creates an oscillating displacement current. The displacement current varies as a function of the proximity of objects to the plate array. Preferably the plates are in the form of a group of three pair of symmetric plates having a common center, arranged in a hexagonal pattern with opposing plates linked as a pair. The sensor produces logic level pulses suitable for interfacing with a computer or process controller. The proximity sensor can be incorporated into a load cell, a differential pressure gauge, or a device for measuring the consistency of a characteristic of a material where a variation in the consistency causes the dielectric constant of the material to change. 14 figs.

Usefulness of IDEAL T2 imaging for homogeneous fat suppression and reducing susceptibility artefacts in brachial plexus MRI at 3.0 T.

PubMed

Tagliafico, Alberto; Bignotti, Bianca; Tagliafico, Giulio; Martinoli, Carlo

2016-01-01

To quantitatively and qualitatively compare fat-suppressed MR imaging quality using iterative decomposition of water and fat with echo asymmetry and least-squares estimation (IDEAL) with that using frequency-selective fat-suppressed (FSFS) T2 images of the brachial plexus at 3.0 T. Prospective MR image analysis was performed in 40 volunteers and 40 patients at a single centre. Oblique-sagittal and coronal IDEAL fat-suppressed T2 images and FSFS T2 images were compared. Visual assessment was performed by two independent musculoskeletal radiologists with respect to: (1) susceptibility artefacts around the neck, (2) homogeneity of fat suppression, (3) image sharpness and (4) tissue resolution contrast of pathologies. The signal-to-noise ratios (SNR) for each image sequence were assessed. Compared to FSFS sequences, IDEAL fat-suppressed T2 images significantly reduced artefacts around the brachial plexus and significantly improved homogeneous fat suppression (p < 0.05). IDEAL significantly improved sharpness and lesion-to-tissue contrast (p < 0.05). The mean SNRs were significantly improved on T2-weighted IDEAL images (p < 0.05). IDEAL technique improved image quality by reducing artefacts around the brachial plexus while maintaining a high SNR and provided superior homogeneous fat suppression than FSFS sequences.

Comparison of Regression Analysis and Transfer Function in Estimating the Parameters of Central Pulse Waves from Brachial Pulse Wave.

PubMed

Chai, Rui; Xu, Li-Sheng; Yao, Yang; Hao, Li-Ling; Qi, Lin

2017-01-01

This study analyzed ascending branch slope (A_slope), dicrotic notch height (Hn), diastolic area (Ad) and systolic area (As) diastolic blood pressure (DBP), systolic blood pressure (SBP), pulse pressure (PP), subendocardial viability ratio (SEVR), waveform parameter (k), stroke volume (SV), cardiac output (CO), and peripheral resistance (RS) of central pulse wave invasively and non-invasively measured. Invasively measured parameters were compared with parameters measured from brachial pulse waves by regression model and transfer function model. Accuracy of parameters estimated by regression and transfer function model, was compared too. Findings showed that k value, central pulse wave and brachial pulse wave parameters invasively measured, correlated positively. Regression model parameters including A_slope, DBP, SEVR, and transfer function model parameters had good consistency with parameters invasively measured. They had same effect of consistency. SBP, PP, SV, and CO could be calculated through the regression model, but their accuracies were worse than that of transfer function model.

Brachial artery vasodilatation during prolonged lower limb exercise: role of shear rate

PubMed Central

Padilla, Jaume; Simmons, Grant H.; Vianna, Lauro C.; Davis, Michael J.; Laughlin, M. Harold; Fadel, Paul J.

2012-01-01

We recently observed a marked increase in brachial artery (BA) diameter during prolonged leg cycling exercise. The purpose of the present study was to test the hypothesis that this increase in BA diameter during lower limb exercise is shear stress mediated. Accordingly, we determined whether recapitulation of cycling-induced BA shear rate with forearm heating, a known stimulus evoking shear-induced conduit artery dilatation, would elicit comparable profiles and magnitudes of BA vasodilatation to those observed during cycling. In 12 healthy men, BA diameter and blood velocity were measured simultaneously using Doppler ultrasonography at baseline and every 5 min during 60 min of either steady-state semi-recumbent leg cycling (120 W) or forearm heating. At the onset of cycling, the BA diameter was reduced (−3.9 ± 1.2% at 5 min; P < 0.05), but it subsequently increased throughout the remainder of the exercise bout (+15.1 ± 1.6% at 60 min; P < 0.05). The increase in BA diameter during exercise was accompanied by an approximately 2.5-fold rise in BA mean shear rate (P < 0.05). Similar increases in BA mean shear with forearm heating elicited an equivalent magnitude of BA vasodilatation to that observed during cycling (P > 0.05). Herein, we found that in the absence of exercise the extent of the BA vasodilator response was reproduced when the BA was exposed to comparable magnitudes of shear rate via forearm heating. These results are consistent with the hypothesis that shear stress plays a key role in signalling brachial artery vasodilatation during dynamic leg exercise. PMID:21784788

Depression in amyotrophic lateral sclerosis

PubMed Central

ATASSI, NAZEM; COOK, AMANDA; PINEDA, CRISTIANA M. E.; YERRAMILLI-RAO, PADMAJA; PULLEY, DARLENE; CUDKOWICZ, MERIT

2011-01-01

Depression is an under-recognized comorbidity associated with amyotrophic lateral sclerosis (ALS). The goals of this study were to prospectively estimate the prevalence of depression and other ALS related symptoms and to study the impact of depression on enrollment in research studies. One hundred and twenty-seven people with ALS completed the ALS Depression Inventory (ADI-12) and answered questions about ALS related symptoms and research study enrollment preferences. Demographics, ALS symptoms, medications, functional status, and research enrollment were compared between depressed and non-depressed patients. Results showed that the prevalence of mild and severe depression was 29% and 6%, respectively. More than one-third of our ALS patients were receiving anti-depressants to treat depression, sialorrhea, and pseudobulbar affect. Depression prevalence was not correlated with disease duration or progression. Except for anxiety, none of the ALS related symptoms predicted depression. The presence of depression did not have an effect on the decision to enroll in research studies. In conclusion, major depression is less common in our ALS cohort than in the general population. The diagnosis of depression can be masked by some ALS related symptoms and it has no impact on enrollment in ALS clinical trials. PMID:21091399

Isolated lower brachial plexus (Klumpke) palsy with compound arm presentation: case report.

PubMed

Buchanan, Edward P; Richardson, Randal; Tse, Raymond

2013-08-01

Klumpke palsy has yet to be clearly documented in the newborn, because previous reports lack any description of the obstetrical history, clinical progression, or outcome. Based on a high incidence of breach presentation in the few clinical series that report Klumpke palsy, hyperabduction with arm overhead during delivery has been the presumed mechanism. We report a child with isolated lower brachial plexus palsy and Horner syndrome who presented at birth with a vertex compound arm presentation. Recognition of this condition and details of the clinical progression and outcome are important, because guidelines for management are currently not available. Copyright © 2013. Published by Elsevier Inc.

Interrelationships Among Flow-Mediated Vasodilation, Nitroglycerine-Induced Vasodilation, Baseline Brachial Artery Diameter, Hyperemic Shear Stress, and Cardiovascular Risk Factors.

PubMed

Maruhashi, Tatsuya; Iwamoto, Yumiko; Kajikawa, Masato; Oda, Nozomu; Kishimoto, Shinji; Matsui, Shogo; Hashimoto, Haruki; Aibara, Yoshiki; Yusoff, Farina Mohamad; Hidaka, Takayuki; Kihara, Yasuki; Chayama, Kazuaki; Noma, Kensuke; Nakashima, Ayumu; Goto, Chikara; Hida, Eisuke; Higashi, Yukihito

2017-12-29

Flow-mediated vasodilation (FMD) of the brachial artery has been used for the assessment of endothelial function. Considering the mechanism underlying the vasodilatory response of the brachial artery to reactive hyperemia, hyperemic shear stress (HSS), a stimulus for FMD; nitroglycerine-induced vasodilation (NID), an index of endothelium-independent vasodilation; and baseline brachial artery diameter (BAD) are also involved in vasodilatory response. The purpose of this study was to investigate the interrelationships among FMD, HSS, NID, baseline BAD, and cardiovascular risk factors. We measured FMD, HSS, NID, and baseline BAD simultaneously in 1033 participants (633 men and 400 women; mean age: 58.6±17.0 years). Framingham risk score was negatively correlated with FMD, HSS, and NID and was positively correlated with baseline BAD. HSS and NID were positively correlated with FMD, and baseline BAD was negatively correlated with FMD. In participants with normal NID, FMD was correlated with HSS, NID, and baseline BAD, all of which were independent variables of FMD in multivariate analysis. In participants with impaired NID, FMD was correlated with NID and baseline BAD, both of which were independent variables of FMD in multivariate analysis, but there was no association between FMD and HSS. NID and baseline BAD were independent variables of FMD regardless of the status of endothelium-independent vasodilation, whereas there was a significant association between FMD and HSS in participants with normal NID but not in those with impaired NID. The influence of HSS on FMD seems to be dependent on the status of endothelium-independent vasodilation. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

Local injection of autologous bone marrow cells to regenerate muscle in patients with traumatic brachial plexus injury: a pilot study.

PubMed

Hogendoorn, S; Duijnisveld, B J; van Duinen, S G; Stoel, B C; van Dijk, J G; Fibbe, W E; Nelissen, R G H H

2014-01-01

Traumatic brachial plexus injury causes severe functional impairment of the arm. Elbow flexion is often affected. Nerve surgery or tendon transfers provide the only means to obtain improved elbow flexion. Unfortunately, the functionality of the arm often remains insufficient. Stem cell therapy could potentially improve muscle strength and avoid muscle-tendon transfer. This pilot study assesses the safety and regenerative potential of autologous bone marrow-derived mononuclear cell injection in partially denervated biceps. Nine brachial plexus patients with insufficient elbow flexion (i.e., partial denervation) received intramuscular escalating doses of autologous bone marrow-derived mononuclear cells, combined with tendon transfers. Effect parameters included biceps biopsies, motor unit analysis on needle electromyography and computerised muscle tomography, before and after cell therapy. No adverse effects in vital signs, bone marrow aspiration sites, injection sites, or surgical wound were seen. After cell therapy there was a 52% decrease in muscle fibrosis (p = 0.01), an 80% increase in myofibre diameter (p = 0.007), a 50% increase in satellite cells (p = 0.045) and an 83% increase in capillary-to-myofibre ratio (p < 0.001) was shown. CT analysis demonstrated a 48% decrease in mean muscle density (p = 0.009). Motor unit analysis showed a mean increase of 36% in motor unit amplitude (p = 0.045), 22% increase in duration (p = 0.005) and 29% increase in number of phases (p = 0.002). Mononuclear cell injection in partly denervated muscle of brachial plexus patients is safe. The results suggest enhanced muscle reinnervation and regeneration. Cite this article: Bone Joint Res 2014;3:38-47.

Report by the Spanish Foundation for the Brain on the social impact of amyotrophic lateral sclerosis and other neuromuscular disorders.

PubMed

Camacho, A; Esteban, J; Paradas, C

A thorough knowledge of the socioeconomic scope of neuromuscular disease is essential for managing resources and raising social awareness. Our group reviewed current data on the epidemiology, mortality and dependence rates, and socioeconomic impact of amyotrophic lateral sclerosis and neuromuscular diseases in Spain. We also recorded how neurological care for these patients is organised. Neuromuscular disorders are a very heterogeneous group of diseases, and some are very rare. These disorders account for between 2.8% and 18% of the total motives for a neurological consultation. In Spain, prevalence and incidence figures for amyotrophic lateral sclerosis are similar to those in other countries; however, figures for patients with other neuromuscular diseases are not known. Since the diseases are chronic, progressive, and debilitating, they cause considerable disability and dependence, which in turn directly affects healthcare and social costs associated with the disease. The costs generated by one patient with amyotrophic lateral sclerosis or Duchenne disease have been calculated at about 50 000 euros per year. Neuromuscular disease shows aetiological, diagnostic, and prognostic complexity, and it requires multidisciplinary management. Follow-up for these patients should be entrusted to specialised units. Copyright © 2015 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

Structures of the G85R Variant of SOD1 in Familial Amyotrophic Lateral Sclerosis

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

Cao, Xiaohang; Antonyuk, Svetlana V.; Seetharaman, Sai V.

2008-07-21

Mutations in the gene encoding human copper-zinc superoxide dismutase (SOD1) cause a dominant form of the progressive neurodegenerative disease amyotrophic lateral sclerosis. Transgenic mice expressing the human G85R SOD1 variant develop paralytic symptoms concomitant with the appearance of SOD1-enriched proteinaceous inclusions in their neural tissues. The process(es) through which misfolding or aggregation of G85R SOD1 induces motor neuron toxicity is not understood. Here we present structures of the human G85R SOD1 variant determined by single crystal x-ray diffraction. Alterations in structure of the metal-binding loop elements relative to the wild type enzyme suggest a molecular basis for the metal ionmore » deficiency of the G85R SOD1 protein observed in the central nervous system of transgenic mice and in purified recombinant G85R SOD1. These findings support the notion that metal-deficient and/or disulfide-reduced mutant SOD1 species contribute to toxicity in SOD1-linked amyotrophic lateral sclerosis.« less

Amyotrophic lateral sclerosis with sensory neuropathy: part of a multisystem disorder?

PubMed Central

Isaacs, Jeremy D; Dean, Andrew F; Shaw, Christopher E; Al‐Chalabi, Ammar; Mills, Kerry R; Leigh, P Nigel

2007-01-01

Sensory involvement is thought not to be a feature of amyotrophic lateral sclerosis (ALS). However, in the setting of a specialist motor neuron disease clinic, we have identified five patients with sporadic ALS and a sensory neuropathy for which an alternative cause could not be identified. In three individuals, sensory nerve biopsy was performed, demonstrating axonal loss without features of an alternative aetiology. These findings support the hypothesis that ALS is a multisystem neurodegenerative disorder that may occasionally include sensory neuropathy among its non‐motor features. PMID:17575021

Spatial Elucidation of Spinal Cord Lipid- and Metabolite- Regulations in Amyotrophic Lateral Sclerosis

NASA Astrophysics Data System (ADS)

Hanrieder, Jörg; Ewing, Andrew G.

2014-06-01

Amyotrophic lateral sclerosis (ALS) is a devastating, rapidly progressing disease of the central nervous system that is characterized by motor neuron degeneration in the brain stem and the spinal cord. We employed time of flight secondary ion mass spectrometry (ToF-SIMS) to profile spatial lipid- and metabolite- regulations in post mortem human spinal cord tissue from ALS patients to investigate chemical markers of ALS pathogenesis. ToF-SIMS scans and multivariate analysis of image and spectral data were performed on thoracic human spinal cord sections. Multivariate statistics of the image data allowed delineation of anatomical regions of interest based on their chemical identity. Spectral data extracted from these regions were compared using two different approaches for multivariate statistics, for investigating ALS related lipid and metabolite changes. The results show a significant decrease for cholesterol, triglycerides, and vitamin E in the ventral horn of ALS samples, which is presumably a consequence of motor neuron degeneration. Conversely, the biogenic mediator lipid lysophosphatidylcholine and its fragments were increased in ALS ventral spinal cord, pointing towards neuroinflammatory mechanisms associated with neuronal cell death. ToF-SIMS imaging is a promising approach for chemical histology and pathology for investigating the subcellular mechanisms underlying motor neuron degeneration in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis: increased solubility of skin collagen

NASA Technical Reports Server (NTRS)

Ono, S.; Yamauchi, M.

1992-01-01

We studied the solubility of skin collagen from six patients with amyotrophic lateral sclerosis (ALS) and six controls. The amount of collagen extracted with neutral salt solution was significantly greater in patients with ALS than in controls. In addition, there was a statistically significant increase in the proportion of collagen extracted from ALS patients with increased duration of illness. The collagen solubilized by pepsin and cyanogen bromide treatments was significantly higher in ALS patients than in controls, and its proportion was positively and significantly associated with duration of illness in ALS patients. These results indicate that the metabolism of skin collagen may be affected in the disease process of ALS, causing an increase in immature soluble collagen in the tissue, which is the opposite to that which occurs in the normal aging process.

Retrieval of a subintimal fractured guide wire from the brachial artery following saphenous vein graft stenting.

PubMed

Danson, Edward J; Ward, Michael

2015-06-01

We present a case of a 58-year-old woman with diabetes mellitus with a history of angina, coronary artery bypass 24 years previously and who underwent retrieval of a fractured coronary buddy wire from the right brachial artery following attempted coronary intervention to a saphenous vein graft via the right radial route. Attempted removal of the guide wire had caused guide catheter-induced dissection of the vein graft in addition to a distal stent edge dissection before fracture in the brachial artery. The fractured end of the buddy wire was found to be in the subintimal space and could only be retrieved by advancing the wire into the subclavian artery by means of wrapping its free portion around the guiding catheter. Its fractured end could then be snared into the guiding catheter but could only be withdrawn from behind the stented segment in the vein graft by means of a trap balloon in the guiding catheter. Successful stenting of a guide catheter-induced dissection and distal stent edge dissection within the vein graft was then performed. This case highlights the hazards of deploying stents over buddy wires and of fractured guide wires in coronary intervention. © 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc.

Acquired Brachial Cutaneous Dyschromatosis in a Middle Aged Male

PubMed Central

Choi, Min Jung; Byun, Ji Yeon; Choi, Hae Young

2018-01-01

Acquired brachial cutaneous dyschromatosis (ABCD) is an acquired disorder of pigmentary change that presents as chronic, asymptomatic, geographic-shaped, gray-brown patches, consisting of mixed hyper and hypopigmented macules on the dorsal aspect of the forearms. We report a case of a 40-year-old male who presented with asymptomatic, multiple brown-colored macules on the outer aspects of both arms. He had no history of hypertension and had never taken angiotensin converting enzyme inhibitors. He also denied chronic sun exposure history. Histologic examination demonstrated epidermal atrophy, increased basal layer pigmentation, and several telangiectatic vessels in the upper dermis. Solar elastosis was not remarkable. The patient's clinical and histopathologic features were consistent with a diagnosis of ABCD. Poikiloderma of Civatte, melasma, acquired bilateral telangiectatic macules and other pigmentary disorders should be considered in the differential diagnosis of ABCD. Herein, we report a case of ABCD in a middle-aged male without hypertension and medication. PMID:29853750

Redox Regulation in Amyotrophic Lateral Sclerosis

PubMed Central

Parakh, Sonam; Spencer, Damian M.; Halloran, Mark A.; Soo, Kai Y.; Atkin, Julie D.

2013-01-01

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that results from the death of upper and lower motor neurons. Due to a lack of effective treatment, it is imperative to understand the underlying mechanisms and processes involved in disease progression. Regulations in cellular reduction/oxidation (redox) processes are being increasingly implicated in disease. Here we discuss the possible involvement of redox dysregulation in the pathophysiology of ALS, either as a cause of cellular abnormalities or a consequence. We focus on its possible role in oxidative stress, protein misfolding, glutamate excitotoxicity, lipid peroxidation and cholesterol esterification, mitochondrial dysfunction, impaired axonal transport and neurofilament aggregation, autophagic stress, and endoplasmic reticulum (ER) stress. We also speculate that an ER chaperone protein disulphide isomerase (PDI) could play a key role in this dysregulation. PDI is essential for normal protein folding by oxidation and reduction of disulphide bonds, and hence any disruption to this process may have consequences for motor neurons. Addressing the mechanism underlying redox regulation and dysregulation may therefore help to unravel the molecular mechanism involved in ALS. PMID:23533690

Distal nerve transfer versus supraclavicular nerve grafting: comparison of elbow flexion outcome in neonatal brachial plexus palsy with C5-C7 involvement.

PubMed

Heise, Carlos O; Siqueira, Mario G; Martins, Roberto S; Foroni, Luciano H; Sterman-Neto, Hugo

2017-09-01

Ulnar and median nerve transfers to arm muscles have been used to recover elbow flexion in infants with neonatal brachial plexus palsy, but there is no direct outcome comparison with the classical supraclavicular nerve grafting approach. We retrospectively analyzed patients with C5-C7 neonatal brachial plexus palsy submitted to nerve surgery and recorded elbow flexion recovery using the active movement scale (0-7) at 12 and 24 months after surgery. We compared 13 patients submitted to supraclavicular nerve grafting with 21 patients submitted to distal ulnar or median nerve transfer to biceps motor branch. We considered elbow flexion scores of 6 or 7 as good results. The mean elbow flexion score and the proportion of good results were better using distal nerve transfers than supraclavicular grafting at 12 months (p < 0.01), but not at 24 months. Two patients with failed supraclavicular nerve grafting at 12 months showed good elbow flexion recovery after ulnar nerve transfers. Distal nerve transfers provided faster elbow flexion recovery than supraclavicular nerve grafting, but there was no significant difference in the outcome after 24 months of surgery. Patients with failed supraclavicular grafting operated early can still benefit from late distal nerve transfers. Supraclavicular nerve grafting should remain as the first line surgical treatment for children with neonatal brachial plexus palsy.

Active Targets For Capacitive Proximity Sensors

NASA Technical Reports Server (NTRS)

Jenstrom, Del T.; Mcconnell, Robert L.

1994-01-01

Lightweight, low-power active targets devised for use with improved capacitive proximity sensors described in "Capacitive Proximity Sensor Has Longer Range" (GSC-13377), and "Capacitive Proximity Sensors With Additional Driven Shields" (GSC-13475). Active targets are short-distance electrostatic beacons; they generate known alternating electro-static fields used for alignment and/or to measure distances.

An Autopsy Case of Amyotrophic Lateral Sclerosis with Diaphragm Pacing.

PubMed

Ito, Hisashi; Kamei, Tetsumasa; Odake, Sanae; Nakano, Masayuki; Okeda, Riki; Kohriki, Shunsaku; Kawachi, Jun; Onders, Raymond P; Yoshii, Fumihito

Respiratory insufficiency is a critical problem in amyotrophic lateral sclerosis (ALS) patients. We herein present the case of an autopsied patient with sporadic ALS who underwent diaphragm pacing (DP). The pathology showed several localized adhesions with a markedly atrophied diaphragm. A marked loss of motor neurons with Bunina bodies and phosphorylated TDP-43 positive inclusions was found in the spinal cord and primary motor cortex. Mild hyalinization and a few multinucleated giant cells were present around the electrode tracks in the diaphragm. However, no infiltration of inflammatory cells was detected. Our findings suggest that full-time DP might not cause severe damage to adjacent diaphragm tissue.

Energy Homeostasis and Abnormal RNA Metabolism in Amyotrophic Lateral Sclerosis

PubMed Central

Liu, Yu-Ju; Tsai, Po-Yi; Chern, Yijuang

2017-01-01

Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that is clinically characterized by progressive muscle weakness and impaired voluntary movement due to the loss of motor neurons in the brain, brain stem and spinal cord. To date, no effective treatment is available. Ample evidence suggests that impaired RNA homeostasis and abnormal energy status are two major pathogenesis pathways in ALS. In the present review article, we focus on recent studies that report molecular insights of both pathways, and discuss the possibility that energy dysfunction might negatively regulate RNA homeostasis via the impairment of cytoplasmic-nuclear shuttling in motor neurons and subsequently contribute to the development of ALS. PMID:28522961

Is There a Role for Exercise in the Management of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis?

ERIC Educational Resources Information Center

Plowman, Emily K.

2015-01-01

Purpose: The role of exercise in the management of people with amyotrophic lateral sclerosis (PALS) is controversial and currently unclear. The purpose of this review article is to review literature examining the impact of limb, respiratory, and oral motor exercise on function, disease progression, and survival in PALS and the transgenic ALS…

[Physiokinesitherapeutic management of traumatic lesions of the brachial plexus. Indications and results].

PubMed

Gusso, M I; Innocenti, M; Mauceri, M C

1985-03-31

An account is given of the indications and results of physical and rehabilitative management of brachial plexus lesions when used electively or as a complement to surgery. Material from the Florence Traumatological and Orthopaedics Centre and the V. Putti Mutilated Patients Centre, Bologna (formerly directed by Prof. O. Scaglietti) dating from 1940 to 1980 is used to explain the indications for conservative and surgical management, in the conviction that sound results depend on exact indication. A selected sample of 51 patients treated conservatively and 194 surgically is presented. The results were satisfactory in about 50% of each group, with a slight advantage in favour of physiotherapy, this being obviously employed when lesions were less severe.

Brachial plexus palsy with the use of haloperidol and a geriatric chair.

PubMed

King, T; Mallet, L

1991-10-01

An 81-year-old white man was admitted to an intermediate care facility because of increased wandering and confusion secondary to dementia. On the first day after admission, the patient tried to leave the facility and was hitting and kicking the employees. Haloperidol 0.5 mg tid was prescribed to help control his behavior. He became more agitated and confused; haloperidol was then increased to 1 mg qid and the patient was confined to a geriatric chair to prevent injuries. Cogwheel movements, rigidity, and marked sedation were documented. A right brachial plexus palsy was diagnosed. This case demonstrated the hazards of two commonly used interventions in a nursing home: antipsychotic agents and the geriatric chair.

Center effect on ankle-brachial index measurement when using the reference method (Doppler and manometer): results from a large cohort study.

PubMed

Vierron, Emilie; Halimi, Jean-Michel; Tichet, Jean; Balkau, Beverley; Cogneau, Joel; Giraudeau, Bruno

2009-07-01

The ankle-brachial index (ABI) is a simple and noninvasive tool used to detect peripheral arterial disease (PAD). We aimed to assess, in a French multicenter cohort, the center effect associated with arterial pressure (AP) and ABI measurements using the reference method and using a semiautomatic device. This study included baseline and 9-year follow-up data from 3,664 volunteers of 10 health examination centers of the DESIR (Data from an Epidemiological Study on the Insulin Resistance) syndrome French cohort. Ankle and brachial AP were measured at inclusion by the reference method (a mercury sphygmomanometer coupled with a Doppler probe for ankle measurements) and at 9 years by a semiautomatic device (Omron HEM-705CP). The center effect was assessed by the intraclass correlation coefficient (ICC), ratio of the between-center variance to the total variance of the measurement. At inclusion, the sample mean age was 47.5 (s.d. 9.9) years; 49.3% were men. Although ICCs were smaller than 0.05 for brachial AP measurements, they were close to 0.18 and 0.20 for ankle systolic AP (SAP) and ABI measurements, respectively, when the reference method was used. No center effect for measures other than ankle SAP was detected. With the semiautomatic device method, all ICCs, including those for ankle SAP and ABI measurements, were between 0.005 and 0.04. We found an important center effect on ABI measured with a sphygmomanometer and a Doppler probe but not a semiautomatic device. A center effect should be taken into account when planning any multicenter study on ABI measurement.

RNA-Targeted Therapies and Amyotrophic Lateral Sclerosis

PubMed Central

Le Masson, Gwendal

2018-01-01

Amyotrophic lateral sclerosis (ALS) is a fatal motor disease in adults. Its pathophysiology remains mysterious, but tremendous advances have been made with the discovery of the most frequent mutations of its more common familial form linked to the C9ORF72 gene. Although most cases are still considered sporadic, these genetic mutations have revealed the role of RNA production, processing and transport in ALS, and may be important players in all ALS forms. There are no disease-modifying treatments for adult human neurodegenerative diseases, including ALS. As in spinal muscular atrophy, RNA-targeted therapies have been proposed as potential strategies for treating this neurodegenerative disorder. Successes achieved in various animal models of ALS have proven that RNA therapies are both safe and effective. With careful consideration of the applicability of such therapies in humans, it is possible to anticipate ongoing in vivo research and clinical trial development of RNA therapies for treating ALS. PMID:29342921

RNA-Targeted Therapies and Amyotrophic Lateral Sclerosis.

PubMed

Mathis, Stéphane; Le Masson, Gwendal

2018-01-15

Amyotrophic lateral sclerosis (ALS) is a fatal motor disease in adults. Its pathophysiology remains mysterious, but tremendous advances have been made with the discovery of the most frequent mutations of its more common familial form linked to the C9ORF72 gene. Although most cases are still considered sporadic, these genetic mutations have revealed the role of RNA production, processing and transport in ALS, and may be important players in all ALS forms. There are no disease-modifying treatments for adult human neurodegenerative diseases, including ALS. As in spinal muscular atrophy, RNA-targeted therapies have been proposed as potential strategies for treating this neurodegenerative disorder. Successes achieved in various animal models of ALS have proven that RNA therapies are both safe and effective. With careful consideration of the applicability of such therapies in humans, it is possible to anticipate ongoing in vivo research and clinical trial development of RNA therapies for treating ALS.

Characterizing Methods of Measuring Flow-Mediated Dilation in the Brachial Artery

NASA Technical Reports Server (NTRS)

Callender, Ariane R.

2010-01-01

Regulation of vascular tone is one of the many important functions of the vascular endothelium. Endothelial dysfunction is a critical early event in the pathogenesis of atherosclerosis and occurs in the absence of angiographic disease. Flow-Mediated Dilation (FMD) is a noninvasive technique commonly used to evaluate endothelium-dependent vasodilation in humans and gauge the health of the cardiovascular system. Reductions in brachial artery FMD have been strongly correlated with disease progression and are predictive of future cardiac events. The flow stimulus for brachial artery FMD occurs as a result of the increased shear stress following deflation of an occlusion cuff around the upper arm. Using 2-dimensional ultrasound, changes in arterial diameter up to 5-minutes following cuff deflation are calculated from baseline image measurements. Along with pulsed Doppler measures of flow velocity through the artery, flow-mediated, endothelium-dependent vasodilation can be assessed. There is debate among investigators, however, about the proper positioning of the occlusion cuff during FMD testing. It is thought that placement of the cuff around the upper arm may not accurately reflect the impact of nitric oxide, a critically important molecule released as a result of the increased shear stress created by the FMD technique. Data suggest that the production of other endogenous metabolites may also contribute to FMD-related changes when positioning the cuff around the upper arm. To overcome the potential influence of such molecules, researchers now suggest that the occlusion cuff be placed below the elbow allowing a more precise estimate of nitric oxide mediated dilation. The purpose of this study is to compare the differences in FMD between the two methodologies of occlusion cuff placement. In addition, this study will determine the method that is easier for ultrasound technicians to perform and will produce a low coefficient of variance between technicians. Ultimately

Botulinum toxin for the treatment of motor imbalance in obstetrical brachial plexus palsy.

PubMed

Arad, Ehud; Stephens, Derek; Curtis, Christine G; Clarke, Howard M

2013-06-01

Residual muscle imbalance is a common problem affecting obstetrical brachial plexus palsy patients. The goal of this study was to examine the efficacy of botulinum toxin type A (Botox) in improving this muscle imbalance. The authors retrospectively reviewed obstetrical brachial plexus palsy patients treated with Botox for muscle imbalance as an isolated procedure. Outcomes were the change in Active Movement Scale scores from pre-Botox scores to scores at 1 month after Botox and 1 year after Botox. Twenty-seven patients were included, 19 treated for shoulder imbalance and eight treated for elbow imbalance. Active Movement Scale scores (mean±SD) for shoulder external rotation improved from 0.6±1.0 before Botox to 2.6±2.14 (p<0.01) at 1 month after Botox, and declined to 1.3±1.2 (p<0.01) at 1 year after Botox. Scores for elbow flexion were 3.3±2.1 before Botox, unchanged at 4.4±1.8 (p=0.07) 1 month after Botox, and improved to 5.8±0.5 (p<0.01) at 1 year after Botox. Scores for elbow supination were 2.9±1.7 before Botox and 3.4±1.5 (p=0.2) at 1 month after Botox, and improved to 3.9±2.0 (p<0.01) at 1 year after Botox. Botox for shoulder movement imbalance produces improvement in external rotation that is not sufficiently sustained over time to be of clinical benefit. However, Botox for elbow movement imbalance produces a sustained and clinically useful improvement. Therapeutic, IV.

Outcome Following Spinal Accessory to Suprascapular (Spinoscapular) Nerve Transfer in Infants with Brachial Plexus Birth Injuries

PubMed Central

Ruchelsman, David E.; Ramos, Lorna E.; Alfonso, Israel; Price, Andrew E.; Grossman, Agatha

2009-01-01

The purpose of this study is to evaluate the value of distal spinal accessory nerve (SAN) transfer to the suprascapular nerve (SSN) in children with brachial plexus birth injuries in order to better define the application and outcome of this transfer in these infants. Over a 3-year period, 34 infants with brachial plexus injuries underwent transfer of the SAN to the SSN as part of the primary surgical reconstruction. Twenty-five patients (direct repair, n = 20; interposition graft, n = 5) achieved a minimum follow-up of 24 months. Fourteen children underwent plexus reconstruction with SAN-to-SSN transfer at less than 9 months of age, and 11 underwent surgical reconstruction at the age of 9 months or older. Mean age at the time of nerve transfer was 11.6 months (range, 5–30 months). At latest follow-up, active shoulder external rotation was measured in the arm abducted position and confirmed by review of videos. The Gilbert and Miami shoulder classification scores were utilized to report shoulder-specific functional outcomes. The effects of patient age at the time of nerve transfer and the use of interpositional nerve graft were analyzed. Overall mean active external rotation measured 69.6°; mean Gilbert score was 4.1 and the mean Miami score was 7.1, corresponding to overall good shoulder functional outcomes. Similar clinical and shoulder-specific functional outcomes were obtained in patients undergoing early (<9 months of age, n = 14) and late (>9 months of age, n = 11) SAN-to-SSN transfer and primary plexus reconstruction. Nine patients (27%) were lost to follow-up and are not included in the analysis. Optimum results were achieved following direct transfer (n = 20). Results following the use of an interpositional graft (n = 5) were rated satisfactory. No patient required a secondary shoulder procedure during the study period. There were no postoperative complications. Distal SAN-to-SSN (spinoscapular) nerve transfer is a reliable

Alternative Fuels in Epilepsy and Amyotrophic Lateral Sclerosis.

PubMed

Tefera, Tesfaye W; Tan, Kah Ni; McDonald, Tanya S; Borges, Karin

2017-06-01

This review summarises the recent findings on metabolic treatments for epilepsy and Amyotrophic Lateral Sclerosis (ALS) in honour of Professor Ursula Sonnewald. The metabolic impairments in rodent models of these disorders as well as affected patients are being discussed. In both epilepsy and ALS, there are defects in glucose uptake and reduced tricarboxylic acid (TCA) cycling, at least in part due to reduced amounts of C4 TCA cycle intermediates. In addition there are impairments in glycolysis in ALS. A reduction in glucose uptake can be addressed by providing the brain with alternative fuels, such as ketones or medium-chain triglycerides. As anaplerotic fuels, such as the triglyceride of heptanoate, triheptanoin, refill the TCA cycle C4/C5 intermediate pool that is deficient, they are ideal to boost TCA cycling and thus the oxidative metabolism of all fuels.

Assessment of a multiple biomarker panel for diagnosis of amyotrophic lateral sclerosis.

PubMed

Chen, Xueping; Chen, Yongping; Wei, Qianqian; Ou, Ruwei; Cao, Bei; Zhao, Bi; Shang, Hui-Fang

2016-09-15

The aim of the study was to assess a panel of promising biomarkers for their ability to improve diagnosis of sporadic amyotrophic lateral sclerosis (ALS). Forty patients with sporadic ALS and 40 controls with other neurological diseases were evaluated. Levels of phosphorylated neurofilament heavy chain (pNfH), S100-β, cystatin C, and chitotriosidase (CHIT) in cerebrospinal fluid were assayed using two-site solid-phase sandwich ELISA. Patients with sporadic ALS showed higher levels of pNfH and CHIT than controls, but lower levels of cystatin C. Multivariate logistic regression that adjusted for patient age and sex identified significant associations between sporadic ALS and levels of pNfH, CHIT and cystatin C. Levels of pNfH correlated positively with rate of progression and decline based on the Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised. Based on receiver operating curve analysis, a pNfH cut-off of 437 ng/L discriminated patients from controls with a sensitivity of 97.3 % and specificity of 83.8 %. A CHIT cut-off of 1593.779 ng/L discriminated patients from controls with a sensitivity of 83.8 % and specificity of 81.1 %. Combining the two biomarkers gave a sensitivity of 83.8 % and specificity of 91.9 %. Levels of pNfH in cerebrospinal fluid may be a reliable biomarker for diagnosing ALS, and combining this biomarker with levels of CHIT may improve diagnostic accuracy.

Daytime Mouthpiece for Continuous Noninvasive Ventilation in Individuals With Amyotrophic Lateral Sclerosis.

PubMed

Bédard, Marie-Eve; McKim, Douglas A

2016-10-01

Noninvasive ventilation (NIV) is commonly used to provide ventilatory support for individuals with amyotrophic lateral sclerosis (ALS). Once 24-h ventilation is required, the decision between invasive tracheostomy ventilation and palliation is often faced. This study describes the use and outcomes of daytime mouthpiece ventilation added to nighttime mask ventilation for continuous NIV in subjects with ALS as an effective alternative. This was a retrospective study of 39 subjects with ALS using daytime mouthpiece ventilation over a 17-y period. Thirty-one subjects were successful with mouthpiece ventilation, 2 were excluded, 2 stopped because of lack of motivation, and 4 with bulbar subscores of the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (b-ALSFRS-R) between 0 and 3 physically failed to use it consistently. No subject in the successful group had a b-ALSFRS-R score of <6. Thirty of the successful subjects were able to generate a maximum insufflation capacity - vital capacity difference with lung volume recruitment. The median (range) survival to tracheostomy or death from initiation of nocturnal NIV and mouthpiece ventilation were 648 (176-2,188) and 286 (41-1,769) d, respectively. Peak cough flow with lung-volume recruitment >180 L/min at initiation of mouthpiece ventilation was associated with a longer survival (637 ± 468 vs 240 ± 158 d (P = .01). Mouthpiece ventilation provides effective ventilation and prolonged survival for individuals with ALS requiring full-time ventilatory support and maintaining adequate bulbar function. Copyright © 2016 by Daedalus Enterprises.

The application of paramagnetic contrast-based T2 effect to 3D heavily T2W high-resolution MR imaging of the brachial plexus and its branches.

PubMed

Wang, Lixia; Niu, Yanfeng; Kong, Xiangquan; Yu, Qun; Kong, Xiangchuang; Lv, Yinzhang; Shi, Heshui; Li, Chungao; Wu, Wenjun; Wang, Bing; Liu, Dingxi

2016-03-01

To introduce a new 3D magnetic resonance neurography (MRN) method involving a paramagnetic contrast-based T2 effect coupled with an advanced 3D heavily T2W SPACE-STIR high resolution imaging sequence that would enhance the contrast between nervous tissue and surrounding tissues. Thirty subjects (average age, 39.6±17.0 years; 18 male and 12 female) were enrolled, including three patients with brachial plexopathy and 27 healthy volunteers. Subjective scores from two neuroradiologists, evaluating noncontrast MRN (cMRN) and 3D SPACE-STIR contrast enhanced MRN (ceMRN) 3D data using a 3-point scoring system, were compared using Wilcoxon signed-rank test. Contrast-to-noise ratios (CNRs), SNRs, and contrast ratios within the brachial plexus on cMRN vs. ceMRN MIP and source images were also compared using the paired t-test. The average score for cMRN (0.77±0.43) was significantly lower than ceMRN (1.73±0.45) (p<0.001). Lower nerve vs. vein CNRs were found on cMRN vs. ceMRN, respectively (p<0.001 for both source and MIP images). All nerve-to-surrounding tissue contrast ratios (i.e., fat, muscle, veins, and bone) were higher for ceMRN compared with cMRN for both source and MIP images (all p<0.05). The improved 3D visualization of the brachial plexus and its branches, using this new contrast-enhanced MRN method, can provide high resolution imaging which may be of significant value in the assessment of brachial plexopathy. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

The anti-inflammatory peptide stearyl-norleucine-VIP delays disease onset and extends survival in a rat model of inherited amyotrophic lateral sclerosis.

PubMed

Goursaud, Stéphanie; Schäfer, Sabrina; Dumont, Amélie O; Vergouts, Maxime; Gallo, Alessandro; Desmet, Nathalie; Deumens, Ronald; Hermans, Emmanuel

2015-01-01

Vasoactive intestinal peptide (VIP) has potent immune modulatory actions that may influence the course of neurodegenerative disorders associated with chronic inflammation. Here, we show the therapeutic benefits of a modified peptide agonist stearyl-norleucine-VIP (SNV) in a transgenic rat model of amyotrophic lateral sclerosis (mutated superoxide dismutase 1, hSOD1(G93A)). When administered by systemic every-other-day intraperitoneal injections during a period of 80 days before disease, SNV delayed the onset of motor dysfunction by no less than three weeks, while survival was extended by nearly two months. SNV-treated rats showed reduced astro- and microgliosis in the lumbar ventral spinal cord and a significant degree of motor neuron preservation. Throughout the treatment, SNV promoted the expression of the anti-inflammatory cytokine interleukin-10 as well as neurotrophic factors commonly considered as beneficial in amyotrophic lateral sclerosis management (glial derived neuroptrophic factor, insulin like growth factor, brain derived neurotrophic factor). The peptide nearly totally suppressed the expression of tumor necrosis factor-α and repressed the production of the pro-inflammatory mediators interleukin-1β, nitric oxide and of the transcription factor nuclear factor kappa B. Inhibition of tumor necrosis factor-α likely accounted for the observed down-regulation of nuclear factor kappa B that modulates the transcription of genes specifically involved in amyotrophic lateral sclerosis (sod1 and the glutamate transporter slc1a2). In line with this, levels of human superoxide dismutase 1 mRNA and protein were decreased by SNV treatment, while the expression and activity of the glutamate transporter-1 was promoted. Considering the large diversity of influences of this peptide on both clinical features of the disease and associated biochemical markers, we propose that SNV or related peptides may constitute promising candidates for amyotrophic lateral sclerosis

DNA/RNA Helicase Gene Mutations in a Form of Juvenile Amyotrophic Lateral Sclerosis (ALS4)

PubMed Central

Chen, Ying-Zhang; Bennett, Craig L.; Huynh, Huy M.; Blair, Ian P.; Puls, Imke; Irobi, Joy; Dierick, Ines; Abel, Annette; Kennerson, Marina L.; Rabin, Bruce A.; Nicholson, Garth A.; Auer-Grumbach, Michaela; Wagner, Klaus; De Jonghe, Peter; Griffin, John W.; Fischbeck, Kenneth H.; Timmerman, Vincent; Cornblath, David R.; Chance, Phillip F.

2004-01-01

Juvenile amyotrophic lateral sclerosis (ALS4) is a rare autosomal dominant form of juvenile amyotrophic lateral sclerosis (ALS) characterized by distal muscle weakness and atrophy, normal sensation, and pyramidal signs. Individuals affected with ALS4 usually have an onset of symptoms at age <25 years, a slow rate of progression, and a normal life span. The ALS4 locus maps to a 1.7-Mb interval on chromosome 9q34 flanked by D9S64 and D9S1198. To identify the molecular basis of ALS4, we tested 19 genes within the ALS4 interval and detected missense mutations (T3I, L389S, and R2136H) in the Senataxin gene (SETX). The SETX gene encodes a novel 302.8-kD protein. Although its function remains unknown, SETX contains a DNA/RNA helicase domain with strong homology to human RENT1 and IGHMBP2, two genes encoding proteins known to have roles in RNA processing. These observations of ALS4 suggest that mutations in SETX may cause neuronal degeneration through dysfunction of the helicase activity or other steps in RNA processing. PMID:15106121

Military Service, Deployments, and Exposures in Relation to Amyotrophic Lateral Sclerosis Etiology and Survival

PubMed Central

Beard, John D.; Kamel, Freya

2015-01-01

Rates of amyotrophic lateral sclerosis (ALS) have been reported to be higher among US military veterans, who currently number more than 21 million, but the causal factor(s) has not been identified. We conducted a review to examine the weight of evidence for associations between military service, deployments, and exposures and ALS etiology and survival. Thirty articles or abstracts published through 2013 were reviewed. Although the current evidence suggests a positive association with ALS etiology, it is too limited to draw firm conclusions regarding associations between military service and ALS etiology or survival. Some evidence suggests that deployment to the 1990–1991 Persian Gulf War may be associated with ALS etiology, but there is currently no strong evidence that any particular military exposure is associated with ALS etiology. Future studies should address the limitations of previous ones, such as reliance on mortality as a surrogate for incidence, a dearth of survival analyses, lack of clinical data, low statistical power, and limited exposure assessment. The Genes and Environmental Exposures in Veterans with Amyotrophic Lateral Sclerosis (GENEVA) Study is one such study, but additional research is needed to determine whether military-related factors are associated with ALS and to assess potential prevention strategies. PMID:25365170

Regression analysis and transfer function in estimating the parameters of central pulse waves from brachial pulse wave.

PubMed

Chai Rui; Li Si-Man; Xu Li-Sheng; Yao Yang; Hao Li-Ling

2017-07-01

This study mainly analyzed the parameters such as ascending branch slope (A_slope), dicrotic notch height (Hn), diastolic area (Ad) and systolic area (As) diastolic blood pressure (DBP), systolic blood pressure (SBP), pulse pressure (PP), subendocardial viability ratio (SEVR), waveform parameter (k), stroke volume (SV), cardiac output (CO) and peripheral resistance (RS) of central pulse wave invasively and non-invasively measured. These parameters extracted from the central pulse wave invasively measured were compared with the parameters measured from the brachial pulse waves by a regression model and a transfer function model. The accuracy of the parameters which were estimated by the regression model and the transfer function model was compared too. Our findings showed that in addition to the k value, the above parameters of the central pulse wave and the brachial pulse wave invasively measured had positive correlation. Both the regression model parameters including A_slope, DBP, SEVR and the transfer function model parameters had good consistency with the parameters invasively measured, and they had the same effect of consistency. The regression equations of the three parameters were expressed by Y'=a+bx. The SBP, PP, SV, CO of central pulse wave could be calculated through the regression model, but their accuracies were worse than that of transfer function model.

Recovery of brachial plexus lesions resulting from heavy backpack use: A follow-up case series

PubMed Central

2011-01-01

Background Brachial plexus lesions as a consequence of carrying a heavy backpack have been reported, but the typical clinical course and long-term consequences are not clear. Here we evaluated the clinical course and pattern of recovery of backpack palsy (BPP) in a large series of patients. Methods Thirty-eight consecutive patients with idiopathic BPP were identified from our population of 193,450 Finnish conscripts by means of computerised register. A physiotherapist provided instructions for proper hand use and rehabilitative exercises at disease onset. The patients were followed up for 2 to 8 years from the diagnosis. We also searched for genetic markers of hereditary neuropathy with pressure palsies. Mann-Whitney U-test was used to analyze continuous data. The Fischer's exact test was used to assess two-way tables. Results Eighty percent of the patients recovered totally within 9 months after the onset of weakness. Prolonged symptoms occurred in 15% of the patients, but daily activities were not affected. The weight of the carried load at the symptom onset significantly affected the severity of the muscle strength loss in the physiotherapeutic testing at the follow-up. The initial electromyography did not predict recovery. Genetic testing did not reveal de novo hereditary neuropathy with pressure palsies. Conclusions The prognosis of BPP is favorable in the vast majority of cases. Electromyography is useful for diagnosis. To prevent brachial plexus lesions, backpack loads greater than 40 kg should be avoided. PMID:21429232

Brachial blood flow under relative levels of blood flow restriction is decreased in a nonlinear fashion.

PubMed

Mouser, J Grant; Ade, Carl J; Black, Christopher D; Bemben, Debra A; Bemben, Michael G

2018-05-01

Blood flow restriction (BFR), the application of external pressure to occlude venous return and restrict arterial inflow, has been shown to increase muscular size and strength when combined with low-load resistance exercise. BFR in the research setting uses a wide range of pressures, applying a pressure based upon an individual's systolic pressure or a percentage of occlusion pressure; not a directly determined reduction in blood flow. The relationship between relative pressure and blood flow has not been established. To measure blood flow in the arm under relative levels of BFR. Forty-five people (18-40 years old) participated. Arterial occlusion pressure in the right arm was measured using a 5-cm pneumatic cuff. Blood flow in the brachial artery was measured at rest and at pressures between 10% and 90% of occlusion using ultrasound. Blood flow decreased in a nonlinear, stepped fashion. Blood flow decreased at 10% of occlusion and remained constant until decreasing again at 40%, where it remained until 90% of occlusion. The decrease in brachial blood flow is not proportional to the applied relative pressure. The prescription of blood flow restriction should take into account the stimulus provided at each relative level of blood flow. © 2017 Scandinavian Society of Clinical Physiology and Nuclear Medicine. Published by John Wiley & Sons Ltd.

Aortic-Brachial Pulse Wave Velocity Ratio: A Measure of Arterial Stiffness Gradient Not Affected by Mean Arterial Pressure.

PubMed

Fortier, Catherine; Desjardins, Marie-Pier; Agharazii, Mohsen

2018-03-01

Aortic stiffness, measured by carotid-femoral pulse wave velocity (cf-PWV), is used for the prediction of cardiovascular risk. This mini-review describes the nonlinear relationship between cf-PWV and operational blood pressure, presents the proposed methods to adjust for this relationship, and discusses a potential place for aortic-brachial PWV ratio (a measure of arterial stiffness gradient) as a blood pressure-independent measure of vascular aging. PWV is inherently dependent on the operational blood pressure. In cross-sectional studies, PWV adjustment for mean arterial pressure (MAP) is preferred, but still remains a nonoptimal approach, as the relationship between PWV and blood pressure is nonlinear and varies considerably among individuals due to heterogeneity in genetic background, vascular tone, and vascular remodeling. Extrapolations from the blood pressure-independent stiffness parameter β (β 0 ) have led to the creation of stiffness index β, which can be used for local stiffness. A similar approach has been used for cardio-ankle PWV to generate a blood pressure-independent cardio-ankle vascular index (CAVI). It was recently demonstrated that stiffness index β and CAVI remain slightly blood pressure-dependent, and a more appropriate formula has been proposed to make the proper adjustments. On the other hand, the negative impact of aortic stiffness on clinical outcomes is thought to be mediated through attenuation or reversal of the arterial stiffness gradient, which can also be influenced by a reduction in peripheral medium-sized muscular arteries in conditions that predispose to accelerate vascular aging. Arterial stiffness gradient, assessed by aortic-brachial PWV ratio, is emerging to be at least as good as cf-PWV for risk prediction, but has the advantage of not being affected by operating MAP. The negative impacts of aortic stiffness on clinical outcomes are proposed to be mediated through attenuation or reversal of arterial stiffness gradient

[Efficacy and safety of ultrasound-guided or neurostimulator-guided bilateral axillary brachial plexus block].

PubMed

Xu, C S; Zhao, X L; Zhou, H B; Qu, Z J; Yang, Q G; Wang, H J; Wang, G

2017-10-17

Objective: To explore the efficacy and safety of bilateral axillary brachial plexus block under the guidance of ultrasound or neurostimulator. Methods: From February 2012 to April 2014, 120 patients undergoing bilateral hand/forearm surgery in Beijing Jishuitan Hospital were enrolled and anaesthetized with bilateral axillary brachial plexus block. All patients were divided into two groups randomly using random number table: the ultrasound-guided group (group U, n =60) and the neurostimulator-guidedgroup (group N, n =60). The block was performed with 0.5% ropivacaine. Patients' age, sex and operation duration were recorded. Moreover, success rate, performance time, onset of sensor and motor block, performance pain, patient satisfaction degree and the incidence of related complications were also documented. Venous samples were collected at selected time points and the total and the plasma concentrations of ropivacaine were analyzed with HPLC. Results: The performance time, the onset of sensor block and the onset of motor block of group U were (8.2±1.5), (14.2± 2.2)and (24.0±3.5)min respectively, which were markedly shorter than those in group N( (14.6±3.9), (19.9±3.8), (28.8±4.2)min, respectively), and the differences were statistically significant( t =11.74, 10.09, 6.73, respectively, all P <0.01). The performance pain score of group N was (25.5± 13.2), which was obviously more serious than group U (31.7± 11.2) and a significant statistical difference was detected ( t =2.856, P <0.05). The patient satisfaction degree of group U was 95.0%, which was significantly higher than group N (83.3%) and a markedly statistical difference was detected (χ(2)=4.227, P <0.05). Fifty min after performance, the total plasma concentration of ropivacaine of group U was(1.76±0.48)mg/L, which was significantly lower than group N (1.88±0.53)mg/L and a significant statistical difference was detected ( t =2.43, P <0.05), while no significant differences were detected at the

Proximity operations considerations affecting spacecraft design

NASA Technical Reports Server (NTRS)

Staas, Steven K.

1991-01-01

Experience from several recent spacecraft development programs, such as Space Station Freedom (SSF) and the Orbital Maneuvering Vehicle (OMV) has shown the need for factoring proximity operations considerations into the vehicle design process. Proximity operations, those orbital maneuvers and procedures which involve operation of two or more spacecraft at ranges of less than one nautical mile, are essential to the construction, servicing, and operation of complex spacecraft. Typical proximity operations considerations which drive spacecraft design may be broken into two broad categories; flight profile characteristics and concerns, and use of various spacecraft systems during proximity operations. Proximity operations flight profile concerns include the following: (1) relative approach/separation line; (2) relative orientation of the vehicles; (3) relative translational and rotational rates; (4) vehicle interaction, in the form of thruster plume impingement, mating or demating operations, or uncontrolled contact/collision; and (5) active vehicle piloting. Spacecraft systems used during proximity operations include the following: (1) sensors, such as radar, laser ranging devices, or optical ranging systems; (2) effector hardware, such as thrusters; (3) flight control software; and (4) mating hardware, needed for docking or berthing operations. A discussion of how these factors affect vehicle design follows, addressing both active and passive/cooperative vehicles.

Numerical Optimization of the Position in Femoral Head of Proximal Locking Screws of Proximal Femoral Nail System; Biomechanical Study.

PubMed

Konya, Mehmet Nuri; Verim, Özgür

2017-09-29

Proximal femoral fracture rates are increasing due to osteoporosis and traffic accidents. Proximal femoral nails are routinely used in the treatment of these fractures in the proximal femur. To compare various combinations and to determine the ideal proximal lag screw position in pertrochanteric fractures (Arbeitsgemeinschaft für Osteosynthesefragen classification 31-A1) of the femur by using optimized finite element analysis. Biomechanical study. Computed tomography images of patients' right femurs were processed with Mimics. Afterwards a solid femur model was created with SolidWorks 2015 and transferred to ANSYS Workbench 16.0 for response surface optimization analysis which was carried out according to anterior-posterior (-10°proximal lag screw positions in the fracture region. The optimum position of the proximal lag screw was determined based on the von Mises stress values occurring on the fracture line. Initial analysis of the system was realized under the surgeon's normal positioning conditions (anterior-posterior, inferior-superior=0°; tip-apex distance=12 mm). The maximum and minimum (compression) von Mises stresses were found to be 438 MPa and 0.003 MPa, respectively, and risky stresses for the system occurred in the regions where the proximal lag screw passes through the proximal femoral nail hole, the small diameter portion of stem joints with a large diameter and lag screw mounts to the stem. The most suitable position of the proximal lag screw was found at the middle position of the tip-apex distance (20 mm) and femoral neck (anterior-posterior, inferior-superior=0°), according to von Mises compression stress values occurring on the fracture line. In our study, we couldn't find any correlation between proximal lag screw movement and tip-apex distance on stresses of the fracture surfaces, but the proximal lag screw

Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis.

PubMed

Tibshirani, Michael; Zhao, Beibei; Gentil, Benoit J; Minotti, Sandra; Marques, Christine; Keith, Julia; Rogaeva, Ekaterina; Zinman, Lorne; Rouaux, Caroline; Robertson, Janice; Durham, Heather D

2017-11-01

Amyotrophic lateral sclerosis is a fatal neurodegenerative disease with paralysis resulting from dysfunction and loss of motor neurons. A common neuropathological finding is attrition of motor neuron dendrites, which make central connections vital to motor control. The chromatin remodelling complex, neuronal Brahma-related gene 1 (Brg1)-associated factor complex (nBAF), is critical for neuronal differentiation, dendritic extension and synaptic function. We have identified loss of the crucial nBAF subunits Brg1, Brg1-associated factor 53b and calcium responsive transactivator in cultured motor neurons expressing FUS or TAR-DNA Binding Protein 43 (TDP-43) mutants linked to familial ALS. When plasmids encoding wild-type or mutant human FUS or TDP-43 were expressed in motor neurons of dissociated spinal cord cultures prepared from E13 mice, mutant proteins in particular accumulated in the cytoplasm. Immunolabelling of nBAF subunits was reduced in proportion to loss of nuclear FUS or TDP-43 and depletion of Brg1 was associated with nuclear retention of Brg1 mRNA. Dendritic attrition (loss of intermediate and terminal dendritic branches) occurred in motor neurons expressing mutant, but not wild-type, FUS or TDP-43. This attrition was delayed by ectopic over-expression of Brg1 and was reproduced by inhibiting Brg1 activity either through genetic manipulation or treatment with the chemical inhibitor, (E)-1-(2-Hydroxyphenyl)-3-((1R, 4R)-5-(pyridin-2-yl)-2, 5-diazabicyclo[2.2.1]heptan-2-yl)prop-2-en-1-one, demonstrating the importance of Brg1 to maintenance of dendritic architecture. Loss of nBAF subunits was also documented in spinal motor neurons in autopsy tissue from familial amyotrophic sclerosis (chromosome 9 open reading frame 72 with G4C2 nucleotide expansion) and from sporadic cases with no identified mutation, pointing to dysfunction of nBAF chromatin remodelling in multiple forms of ALS. © The Author 2017. Published by Oxford University Press. All rights reserved

Clinical Assessment of the Infant and Child Following Perinatal Brachial Plexus Injury

PubMed Central

Duff, Susan V.; DeMatteo, Carol

2015-01-01

STUDY DESIGN Literature review INTRODUCTION After perinatal brachial plexus injury (PBPI), clinicians play an important role in injury classification as well as the assessment of recovery and secondary conditions. Early assessment guides the initial plan of care and influences follow-up and long-term outcome. PURPOSE To review methods used to assess, classify and monitor the extent and influence of PBPI with an emphasis on guidelines for clinicians. METHODS We use The International Classification of Functioning, Disability, and Health (ICF) model to provide a guide to assessment after PBPI for rehabilitation clinicians. DISCUSSION With information gained from targeted assessments, clinicians can design interventions to increase the opportunities infants and children have for optimal recovery and to attain skills that allow participation in areas of interest. PMID:25840493

Risk factors for amyotrophic lateral sclerosis

PubMed Central

Ingre, Caroline; Roos, Per M; Piehl, Fredrik; Kamel, Freya; Fang, Fang

2015-01-01

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease. It is typically fatal within 2–5 years of symptom onset. The incidence of ALS is largely uniform across most parts of the world, but an increasing ALS incidence during the last decades has been suggested. Although recent genetic studies have substantially improved our understanding of the causes of ALS, especially familial ALS, an important role of non-genetic factors in ALS is recognized and needs further study. In this review, we briefly discuss several major genetic contributors to ALS identified to date, followed by a more focused discussion on the most commonly examined non-genetic risk factors for ALS. We first review factors related to lifestyle choices, including smoking, intake of antioxidants, physical fitness, body mass index, and physical exercise, followed by factors related to occupational and environmental exposures, including electromagnetic fields, metals, pesticides, β-methylamino-L-alanine, and viral infection. Potential links between ALS and other medical conditions, including head trauma, metabolic diseases, cancer, and inflammatory diseases, are also discussed. Finally, we outline several future directions aiming to more efficiently examine the role of non-genetic risk factors in ALS. PMID:25709501

Metabolic Reprogramming in Amyotrophic Lateral Sclerosis.

PubMed

Szelechowski, M; Amoedo, N; Obre, E; Léger, C; Allard, L; Bonneu, M; Claverol, S; Lacombe, D; Oliet, S; Chevallier, S; Le Masson, G; Rossignol, R

2018-03-02

Mitochondrial dysfunction in the spinal cord is a hallmark of amyotrophic lateral sclerosis (ALS), but the neurometabolic alterations during early stages of the disease remain unknown. Here, we investigated the bioenergetic and proteomic changes in ALS mouse motor neurons and patients' skin fibroblasts. We first observed that SODG93A mice presymptomatic motor neurons display alterations in the coupling efficiency of oxidative phosphorylation, along with fragmentation of the mitochondrial network. The proteome of presymptomatic ALS mice motor neurons also revealed a peculiar metabolic signature with upregulation of most energy-transducing enzymes, including the fatty acid oxidation (FAO) and the ketogenic components HADHA and ACAT2, respectively. Accordingly, FAO inhibition altered cell viability specifically in ALS mice motor neurons, while uncoupling protein 2 (UCP2) inhibition recovered cellular ATP levels and mitochondrial network morphology. These findings suggest a novel hypothesis of ALS bioenergetics linking FAO and UCP2. Lastly, we provide a unique set of data comparing the molecular alterations found in human ALS patients' skin fibroblasts and SODG93A mouse motor neurons, revealing conserved changes in protein translation, folding and assembly, tRNA aminoacylation and cell adhesion processes.

Sphingolipid Metabolism Is Dysregulated at Transcriptomic and Metabolic Levels in the Spinal Cord of an Animal Model of Amyotrophic Lateral Sclerosis.

PubMed

Henriques, Alexandre; Croixmarie, Vincent; Bouscary, Alexandra; Mosbach, Althéa; Keime, Céline; Boursier-Neyret, Claire; Walter, Bernard; Spedding, Michael; Loeffler, Jean-Philippe

2017-01-01

Lipid metabolism is drastically dysregulated in amyotrophic lateral sclerosis and impacts prognosis of patients. Animal models recapitulate alterations in the energy metabolism, including hypermetabolism and severe loss of adipose tissue. To gain insight into the molecular mechanisms underlying disease progression in amyotrophic lateral sclerosis, we have performed RNA-sequencing and lipidomic profiling in spinal cord of symptomatic SOD1 G86R mice. Spinal transcriptome of SOD1 G86R mice was characterized by differential expression of genes related to immune system, extracellular exosome, and lysosome. Hypothesis-driven identification of metabolites showed that lipids, including sphingomyelin(d18:0/26:1), ceramide(d18:1/22:0), and phosphatidylcholine(o-22:1/20:4) showed profound altered levels. A correlation between disease severity and gene expression or metabolite levels was found for sphingosine, ceramide(d18:1/26:0), Sgpp2, Sphk1 , and Ugt8a . Joint-analysis revealed a significant enrichment of glycosphingolipid metabolism in SOD1 G86R mice, due to the down-regulation of ceramide, glucosylceramide, and lactosylceramide and the overexpression of genes involved in their recycling in the lysosome. A drug-gene interaction database was interrogated to identify potential drugs able to modulate the dysregulated genes from the signaling pathway. Our results suggest that complex lipids are pivotally changed during the first phase of motor symptoms in an animal model of amyotrophic lateral sclerosis.

HDAC inhibition inhibits brachial plexus avulsion induced neuropathic pain.

PubMed

Zhao, Yingbo; Wu, Tianjian

2018-05-09

Introduction Neuropathic pain induced by brachial plexus avulsion (BPA) is a pathological condition. We hypothesized that inhibition of histone deacetylase (HDAC) could suppress BPA-induced neuropathic pain through inhibition of transient reception potential (TRP) overexpression and protein kinase B (Akt) mediated mammalian target of rapamycin (mTOR) activation. Methods We generated a rat BPA model, administered HDAC inhibitor Tricostatin A (TSA) for 7 days post-surgery and assessed the effects on HDAC expression, Akt phosphorylation, neuroinflammation and mTOR activation. Results TSA treatment alleviated BPA induced mechanical hyperalgesia, suppressed Akt phosphorylation and increased HDAC. We found suppressed pro-inflammatory cytokine levels, TRP cation channel subfamily V member 1 (TRPV1) and TRP melastatin 8 (TRPM8) expression and mTOR activity in TSA treated BPA rats. Discussion Our results suggest that altered HDAC and Akt signaling are involved in BPA-induced neuropathic pain and that inhibition of HDAC could be an effective therapeutic approach in reducing neuropathic pain. This article is protected by copyright. All rights reserved. © 2018 Wiley Periodicals, Inc.

Contribution of TARDBP mutations to sporadic amyotrophic lateral sclerosis.

PubMed

Daoud, H; Valdmanis, P N; Kabashi, E; Dion, P; Dupré, N; Camu, W; Meininger, V; Rouleau, G A

2009-02-01

Mutations in the TARDBP gene, which encodes the TAR DNA binding protein (TDP-43), have been described in individuals with familial and sporadic amyotrophic lateral sclerosis (ALS). We screened the TARDBP gene in 285 French sporadic ALS patients to assess the frequency of TARDBP mutations in ALS. Six individuals had potentially deleterious mutations of which three were novel including a Y374X truncating mutation and P363A and A382P missense mutations. This suggests that TARDBP mutations may predispose to ALS in approximately 2% of the individuals followed in this study. Our findings, combined with those from other collections, brings the total number of mutations in unrelated ALS patients to 17, further suggesting that mutations in the TARDBP gene have an important role in the pathogenesis of ALS.

“Neuropathology of amyotrophic lateral sclerosis and its variants”

PubMed Central

Saberi, Shahram; Stauffer, Jennifer E.; Schulte, Derek J.; Ravits, John

2015-01-01

Summary Amyotrophic lateral sclerosis (ALS) is a clinical syndrome named for its neuropathological hallmark: degeneration of motor neurons in the spinal anterior horn and motor cortex and loss of axons in the lateral columns of the spinal cord. The signature neuropathological molecular signature common to almost all sporadic ALS and most familial ALS is TDP-43 immunoreactive neuronal cytoplasmic inclusions. The neuropathological and molecular neuropathological features of ALS variants primarly lateral sclerosis and progressive muscular atrophy are less certain, but also appear to share the primary features of ALS. A number of genetic causes including mutations in SOD1, FUS, and C9orf72 comprise a disease spectrum and all demonstrate distinctive molecular and neuropathological signatures. Neuropathology will continue to play to a key role in solving the puzzle of ALS pathogenesis. PMID:26515626

Subacute diabetic proximal neuropathy

NASA Technical Reports Server (NTRS)

Pascoe, M. K.; Low, P. A.; Windebank, A. J.; Litchy, W. J.

1997-01-01

OBJECTIVE: To evaluate the clinical, electrophysiologic, autonomic, and neuropathologic characteristics and the natural history of subacute diabetic proximal neuropathy and its response to immunotherapy. MATERIAL AND METHODS: For the 12-year period from 1983 to 1995, we conducted a retrospective review of medical records of Mayo Clinic patients with diabetes who had subacute onset and progression of proximal weakness. The responses of treated versus untreated patients were compared statistically. RESULTS: During the designated study period, 44 patients with subacute diabetic proximal neuropathy were encountered. Most patients were middle-aged or elderly, and no sex preponderance was noted. The proximal muscle weakness often was associated with reduced or absent lower extremity reflexes. Associated weight loss was a common finding. Frequently, patients had some evidence of demyelination on nerve conduction studies, but it invariably was accompanied by concomitant axonal degeneration. The cerebrospinal fluid protein concentration was usually increased. Diffuse and substantial autonomic failure was generally present. In most cases, a sural nerve biopsy specimen suggested demyelination, although evidence of an inflammatory infiltrate was less common. Of 12 patients who received treatment (with prednisone, intravenous immune globulin, or plasma exchange), 9 had improvement of their conditions, but 17 of 29 untreated patients (59%) with follow-up also eventually had improvement, albeit at a much slower rate. Improvement was usually incomplete. CONCLUSION: We suggest that the entity of subacute diabetic proximal neuropathy is an extensive and severe variant of bilateral lumbosacral radiculoplexopathy, with some features suggestive of an immune-mediated cause. It differs from chronic inflammatory demyelinating polyradiculoneuropathy in that most cases have a more restricted distribution and seem to be monophasic and self-limiting. The efficacy of immunotherapy is unproved

[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.

Symptom Management and End-of-Life Care in Amyotrophic Lateral Sclerosis.

PubMed

Jackson, Carlayne E; McVey, April L; Rudnicki, Stacy; Dimachkie, Mazen M; Barohn, Richard J

2015-11-01

The number of available symptomatic treatments has markedly enhanced the care of patients with amyotrophic lateral sclerosis (ALS). Once thought to be untreatable, patients with ALS today clearly benefit from multidisciplinary care. The impact of such care on the disease course, including rate of progression and mortality, has surpassed the treatment effects commonly sought in clinical drug trials. Unfortunately, there are few randomized controlled trials of medications or interventions addressing symptom management. In this review, the authors provide the level of evidence, when available, for each intervention that is currently considered standard of care by consensus opinion. Copyright © 2015 Elsevier Inc. All rights reserved.

Intracoronary stent implantation: new approach using a monorail system and new large-lumen 7F catheters from the brachial route.

PubMed

Jenny, D B; Robert, G P; Fajadet, J C; Cassagneau, B G; Marco, J

1992-04-01

In this brief report we describe a case of successful multivessel PTCA with intracoronary stent implantation using a new large-lumen 7F catheter from the left brachial approach. The application of this technique should be considered for intravascular stent implantation when anticoagulation ideally should not be interrupted or in anatomical situations limiting femoral vascular access.

Ankle brachial index for the diagnosis of lower limb peripheral arterial disease.

PubMed

Crawford, Fay; Welch, Karen; Andras, Alina; Chappell, Francesca M

2016-09-14

Peripheral arterial disease (PAD) of the lower limb is common, with prevalence of both symptomatic and asymptomatic disease estimated at 13% in the over 50 age group. Symptomatic PAD affects about 5% of individuals in Western populations between the ages of 55 and 74 years. The most common initial symptom of PAD is muscle pain on exercise that is relieved by rest and is attributed to reduced lower limb blood flow due to atherosclerotic disease (intermittent claudication). The ankle brachial index (ABI) is widely used by a variety of healthcare professionals, including specialist nurses, physicians, surgeons and podiatrists working in primary and secondary care settings, to assess signs and symptoms of PAD. As the ABI test is non-invasive and inexpensive and is in widespread clinical use, a systematic review of its diagnostic accuracy in people presenting with leg pain suggestive of PAD is highly relevant to routine clinical practice. To estimate the diagnostic accuracy of the ankle brachial index (ABI) - also known as the ankle brachial pressure index (ABPI) - for the diagnosis of peripheral arterial disease in people who experience leg pain on walking that is alleviated by rest. We carried out searches of the following databases in August 2013: MEDLINE (Ovid SP),Embase (Ovid SP), the Cumulative Index to Nursing and Allied Health Literature (CINAHL) (EBSCO), Latin American and Caribbean Health Sciences (LILACS) (Bireme), Database of Abstracts of Reviews of Effects and the Health Technology Assessment Database in The Cochrane Library, the Institute for Scientific Information (ISI) Conference Proceedings Citation Index - Science, the British Library Zetoc Conference search and Medion. We included cross-sectional studies of ABI in which duplex ultrasonography or angiography was used as the reference standard. We also included cross-sectional or diagnostic test accuracy (DTA) cohort studies consisting of both prospective and retrospective studies.Participants were

Pathogenesis of amyotrophic lateral sclerosis.

PubMed

Morgan, Sarah; Orrell, Richard W

2016-09-01

Amyotrophic lateral sclerosis (ALS) or motor neuron disease is a rapidly progressive neurodegenerative disorder. The primary involvement is of motor neurons in the brain, spinal cord and peripherally. There is secondary weakness of muscles and primary involvement of other brain regions, especially involving cognition. Peer-reviewed journal articles and reviews. PubMed.gov The pathogenesis of ALS remains largely unknown. There are a wide range of potential mechanisms related to neurodegeneration. An increasing number of genetic factors are recognized. There remains controversy, or lack of knowledge, in explaining how cellular events manifest as the complex human disease. There is controversy as to how well cellular and animal models of disease relate to the human disease. Large-scale international collaborative genetic epidemiological studies are replacing local studies. Therapies related to pathogenesis remain elusive, with the greatest advances to date relating to provision of care (including multidisciplinary management) and supportive care (nutrition and respiratory support). The identification of C9orf72 hexanucleotide repeats as the most frequent genetic background to ALS, and the association with frontotemporal dementia, gives the potential of a genetic background against which to study other risk factors, triggers and pathogenic mechanisms, and to develop potential therapies. © The Author 2016. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Oxidized/misfolded superoxide dismutase-1: the cause of all amyotrophic lateral sclerosis?

PubMed

Kabashi, Edor; Valdmanis, Paul N; Dion, Patrick; Rouleau, Guy A

2007-12-01

The identification in 1993 of superoxide dismutase-1 (SOD1) mutations as the cause of 10 to 20% of familial amyotrophic lateral sclerosis cases, which represents 1 to 2% of all amyotrophic lateral sclerosis (ALS) cases, prompted a substantial amount of research into the mechanisms of SOD1-mediated toxicity. Recent experiments have demonstrated that oxidation of wild-type SOD1 leads to its misfolding, causing it to gain many of the same toxic properties as mutant SOD1. In vitro studies of oxidized/misfolded SOD1 and in vivo studies of misfolded SOD1 have indicated that these protein species are selectively toxic to motor neurons, suggesting that oxidized/misfolded SOD1 could lead to ALS even in individuals who do not carry an SOD1 mutation. It has also been reported that glial cells secrete oxidized/misfolded mutant SOD1 to the extracellular environment, where it can trigger the selective death of motor neurons, offering a possible explanation for the noncell autonomous nature of mutant SOD1 toxicity and the rapid progression of disease once the first symptoms develop. Therefore, considering that sporadic (SALS) and familial ALS (FALS) cases are clinically indistinguishable, the toxic properties of mutated SOD1 are similar to that of oxidized/misfolded wild-type SOD1 (wtSOD1), and secreted/extracellular misfolded SOD1 is selectively toxic to motor neurons, we propose that oxidized/misfolded SOD1 is the cause of most forms of classic ALS and should be a prime target for the design of ALS treatments.

Modeling tunneling for the unconventional superconducting proximity effect

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

Zareapour, Parisa; Xu, Jianwei; Zhao, Shu Yang F.

Recently there has been reinvigorated interest in the superconducting proximity effect, driven by predictions of the emergence of Majorana fermions. To help guide this search, we have developed a phenomenological model for the tunneling spectra in anisotropic superconductor-normal metal proximity devices. We combine successful approaches used in s-wave proximity and standard d-wave tunneling to reproduce tunneling spectra in d-wave proximity devices, and clarify the origin of various features. Different variations of the pair potential are considered, resulting from the proximity-induced superconductivity. Furthermore, the effective pair potential felt by the quasiparticles is momentum-dependent in contrast to s-wave superconductors. The probabilities ofmore » reflection and transmission are calculated by solving the Bogoliubov equations. Our results are consistent with experimental observations of the unconventional proximity effect and provide important experimental parameters such as the size and length scale of the proximity induced gap, as well as the conditions needed to observe the reduced and full superconducting gaps.« less

Modeling tunneling for the unconventional superconducting proximity effect

DOE PAGES

Zareapour, Parisa; Xu, Jianwei; Zhao, Shu Yang F.; ...

2016-10-12

Recently there has been reinvigorated interest in the superconducting proximity effect, driven by predictions of the emergence of Majorana fermions. To help guide this search, we have developed a phenomenological model for the tunneling spectra in anisotropic superconductor-normal metal proximity devices. We combine successful approaches used in s-wave proximity and standard d-wave tunneling to reproduce tunneling spectra in d-wave proximity devices, and clarify the origin of various features. Different variations of the pair potential are considered, resulting from the proximity-induced superconductivity. Furthermore, the effective pair potential felt by the quasiparticles is momentum-dependent in contrast to s-wave superconductors. The probabilities ofmore » reflection and transmission are calculated by solving the Bogoliubov equations. Our results are consistent with experimental observations of the unconventional proximity effect and provide important experimental parameters such as the size and length scale of the proximity induced gap, as well as the conditions needed to observe the reduced and full superconducting gaps.« less

Non-invasive ventilation in amyotrophic lateral sclerosis.

PubMed

Vrijsen, Bart; Testelmans, Dries; Belge, Catharina; Robberecht, Wim; Van Damme, Philip; Buyse, Bertien

2013-03-01

Abstract Non-invasive ventilation (NIV) is widely used to improve alveolar hypoventilation in amyotrophic lateral sclerosis. Several studies indicate a better survival when NIV is used, certainly in patients with none to moderate bulbar dysfunction. Data on quality of life (QoL) are rather disputable. Overall QoL is shown to be equivalent in patients with or without NIV, although health-related QoL is shown to be increased in patients with none to moderate bulbar dysfunction. NIV improves sleep quality, although patient-ventilator asynchronies are demonstrated. FVC < 50%, seated or supine, has been widely applied as threshold to initiate NIV. Today, measurements of respiratory muscle strength, nocturnal gas exchange and symptomatic complaints are used as indicators to start NIV. Being compliant with NIV therapy increases QoL and survival. Cough augmentation has an important role in appropriate NIV. Patients have today more technical options and patients with benefit from these advances are growing in number. Tracheal ventilation needs to be discussed when NIV seems impossible or becomes insufficient.

Narrative discourse deficits in amyotrophic lateral sclerosis.

PubMed

Ash, Sharon; Menaged, Anna; Olm, Christopher; McMillan, Corey T; Boller, Ashley; Irwin, David J; McCluskey, Leo; Elman, Lauren; Grossman, Murray

2014-08-05

We examined narrative discourse in amyotrophic lateral sclerosis (ALS) to assess the role of executive functioning in support of language and the neuroanatomical basis for such support. We analyzed a semistructured speech sample in 26 patients with ALS and 19 healthy seniors for narrative discourse features of coherence. Regression analyses related a measure of discourse coherence ("local connectedness") to gray matter atrophy and reduced white matter fractional anisotropy. Patients with ALS were impaired relative to controls on measures of discourse adequacy, including local connectedness and maintenance of the theme. These discourse measures were related to measures of executive functioning but not to motor functioning. Regressions related local connectedness to gray matter atrophy in ventral and dorsal prefrontal regions and to reduced fractional anisotropy in white matter tracts mediating projections between prefrontal regions. Patients with ALS exhibit deficits in their ability to organize narrative discourse. These deficits appear to be related in part to executive limitations. Consistent with the hypothesis that ALS is a multisystem disorder, this deficit is related to disease in prefrontal regions. © 2014 American Academy of Neurology.

Absence of airway secretion accumulation predicts tolerance of noninvasive ventilation in subjects with amyotrophic lateral sclerosis.

PubMed

Vandenberghe, Nadia; Vallet, Anne-Evelyne; Petitjean, Thierry; Le Cam, Pierre; Peysson, Stéphane; Guérin, Claude; Dailler, Frédéric; Jay, Sylvie; Cadiergue, Vincent; Bouhour, Françoise; Court-Fortune, Isabelle; Camdessanche, Jean-Philippe; Antoine, Jean-Christophe; Philit, François; Beuret, Pascal; Bin-Dorel, Sylvie; Vial, Christophe; Broussolle, Emmanuel

2013-09-01

To assess factors that predict good tolerance of noninvasive ventilation (NIV), in order to improve survival and quality of life in subjects with amyotrophic lateral sclerosis. We conducted a prospective study in subjects with amyotrophic lateral sclerosis and requiring NIV. The primary end point was NIV tolerance at 1 month. Subjects, several of whom failed to complete the study, were classified as "tolerant" or "poorly tolerant," according to the number of hours of NIV use (more or less than 4 h per night, respectively). Eighty-one subjects, 73 of whom also attended the 1-month follow-up visit, participated over 34 months. NIV tolerance after the first day of utilization predicted tolerance at 1 month (77.6% and 75.3% of subjects, respectively). Multivariate analysis disclosed 3 factors predicting good NIV tolerance: absence of airway secretions accumulation prior to NIV onset (odds ratio 11.5); normal bulbar function at initiation of NIV (odds ratio 8.5); and older age (weakly significant, odds ratio 1.1). Our study reveals 3 factors that are predictive of good NIV tolerance, in particular the absence of airway secretion accumulation, which should prompt NIV initiation before its appearance.

Effect of virtual reality versus conventional physiotherapy on upper extremity function in children with obstetric brachial plexus injury

PubMed Central

El-Shamy, Shamekh; Alsharif, Rabab

2017-01-01

Objectives: The objective was to evaluate the effects of virtual reality versus conventional physiotherapy on upper extremity function in children with obstetric brachial plexus injury. Methods: Forty children with Erb’s palsy were selected for this randomized controlled study. They were assigned randomly to either group A (conventional physiotherapy program) or group B (virtual reality program using Armeo® spring for 45 min three times/week for 12 successive weeks). Mallet system scores for shoulder function and shoulder abduction, and external rotation range of motion (ROM) were obtained; shoulder abductor, and external rotators isometric strength were evaluated pre-and post-treatment using Mallet scoring system, standard universal goniometer, and handheld dynamometer. Results: The results of this study indicate that the children in both groups showed improvement in shoulder functions post-treatment with greater improvements in group B. The abduction muscle strength after treatment was 8.53 and 11.3 Nm for group A and group B, respectively. The external rotation muscle strength after treatment was 5.88 and 7.45 Nm for group A and group B, respectively. Conclusions: The virtual reality program is a significantly more effective than conventional physiotherapy program in improving the upper extremity functions in children with obstetric brachial plexus injury. PMID:29199193

The expanding syndrome of amyotrophic lateral sclerosis: a clinical and molecular odyssey

PubMed Central

Turner, Martin R; Swash, Michael

2015-01-01

Recent advances in understanding amyotrophic lateral sclerosis (ALS) have delivered new questions. Disappointingly, the initial enthusiasm for transgenic mouse models of the disease has not been followed by rapid advances in therapy or prevention. Monogenic models may have inadvertently masked the true complexity of the human disease. ALS has evolved into a multisystem disorder, involving a final common pathway accessible via multiple upstream aetiological tributaries. Nonetheless, there is a common clinical core to ALS, as clear today as it was to Charcot and others. We stress the continuing relevance of clinical observations amid the increasing molecular complexity of ALS. PMID:25644224

Autoimmune-like hepatitis during masitinib therapy in an amyotrophic lateral sclerosis patient

PubMed Central

Salvado, Maria; Vargas, Victor; Vidal, Marta; Simon-Talero, Macarena; Camacho, Jessica; Gamez, Josep

2015-01-01

We report a case of acute severe hepatitis resulting from masitinib in a young amyotrophic lateral sclerosis patient. Hepatotoxicity induced by masitinib, a tyrosine kinase inhibitor, is usually transient with mild elevation of transaminases, although acute hepatitis has been not reported to date. The hepatitis was resolved after masitinib was discontinued and a combination of prednisone and azathioprine was started. The transaminases returned to baseline normal values five months later. This is the first case in the hepatitis literature associated with masitinib. The autoimmune role of this drug-induced liver injury is discussed. Physicians should be aware of this potential complication. PMID:26420975

Brachial artery vasomotion and transducer pressure effect on measurements by active contour segmentation on ultrasound

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

Cary, Theodore W.; Sultan, Laith R.; Sehgal, Chandra M., E-mail: sehgalc@uphs.upenn.edu

Purpose: To use feed-forward active contours (snakes) to track and measure brachial artery vasomotion on ultrasound images recorded in both transverse and longitudinal views; and to compare the algorithm's performance in each view. Methods: Longitudinal and transverse view ultrasound image sequences of 45 brachial arteries were segmented by feed-forward active contour (FFAC). The segmented regions were used to measure vasomotion artery diameter, cross-sectional area, and distention both as peak-to-peak diameter and as area. ECG waveforms were also simultaneously extracted frame-by-frame by thresholding a running finite-difference image between consecutive images. The arterial and ECG waveforms were compared as they traced eachmore » phase of the cardiac cycle. Results: FFAC successfully segmented arteries in longitudinal and transverse views in all 45 cases. The automated analysis took significantly less time than manual tracing, but produced superior, well-behaved arterial waveforms. Automated arterial measurements also had lower interobserver variability as measured by correlation, difference in mean values, and coefficient of variation. Although FFAC successfully segmented both the longitudinal and transverse images, transverse measurements were less variable. The cross-sectional area computed from the longitudinal images was 27% lower than the area measured from transverse images, possibly due to the compression of the artery along the image depth by transducer pressure. Conclusions: FFAC is a robust and sensitive vasomotion segmentation algorithm in both transverse and longitudinal views. Transverse imaging may offer advantages over longitudinal imaging: transverse measurements are more consistent, possibly because the method is less sensitive to variations in transducer pressure during imaging.« less

Brachial artery vasomotion and transducer pressure effect on measurements by active contour segmentation on ultrasound.

PubMed

Cary, Theodore W; Reamer, Courtney B; Sultan, Laith R; Mohler, Emile R; Sehgal, Chandra M

2014-02-01

To use feed-forward active contours (snakes) to track and measure brachial artery vasomotion on ultrasound images recorded in both transverse and longitudinal views; and to compare the algorithm's performance in each view. Longitudinal and transverse view ultrasound image sequences of 45 brachial arteries were segmented by feed-forward active contour (FFAC). The segmented regions were used to measure vasomotion artery diameter, cross-sectional area, and distention both as peak-to-peak diameter and as area. ECG waveforms were also simultaneously extracted frame-by-frame by thresholding a running finite-difference image between consecutive images. The arterial and ECG waveforms were compared as they traced each phase of the cardiac cycle. FFAC successfully segmented arteries in longitudinal and transverse views in all 45 cases. The automated analysis took significantly less time than manual tracing, but produced superior, well-behaved arterial waveforms. Automated arterial measurements also had lower interobserver variability as measured by correlation, difference in mean values, and coefficient of variation. Although FFAC successfully segmented both the longitudinal and transverse images, transverse measurements were less variable. The cross-sectional area computed from the longitudinal images was 27% lower than the area measured from transverse images, possibly due to the compression of the artery along the image depth by transducer pressure. FFAC is a robust and sensitive vasomotion segmentation algorithm in both transverse and longitudinal views. Transverse imaging may offer advantages over longitudinal imaging: transverse measurements are more consistent, possibly because the method is less sensitive to variations in transducer pressure during imaging.

Brachial artery vasomotion and transducer pressure effect on measurements by active contour segmentation on ultrasound

PubMed Central

Cary, Theodore W.; Reamer, Courtney B.; Sultan, Laith R.; Mohler, Emile R.; Sehgal, Chandra M.

2014-01-01

Purpose: To use feed-forward active contours (snakes) to track and measure brachial artery vasomotion on ultrasound images recorded in both transverse and longitudinal views; and to compare the algorithm's performance in each view. Methods: Longitudinal and transverse view ultrasound image sequences of 45 brachial arteries were segmented by feed-forward active contour (FFAC). The segmented regions were used to measure vasomotion artery diameter, cross-sectional area, and distention both as peak-to-peak diameter and as area. ECG waveforms were also simultaneously extracted frame-by-frame by thresholding a running finite-difference image between consecutive images. The arterial and ECG waveforms were compared as they traced each phase of the cardiac cycle. Results: FFAC successfully segmented arteries in longitudinal and transverse views in all 45 cases. The automated analysis took significantly less time than manual tracing, but produced superior, well-behaved arterial waveforms. Automated arterial measurements also had lower interobserver variability as measured by correlation, difference in mean values, and coefficient of variation. Although FFAC successfully segmented both the longitudinal and transverse images, transverse measurements were less variable. The cross-sectional area computed from the longitudinal images was 27% lower than the area measured from transverse images, possibly due to the compression of the artery along the image depth by transducer pressure. Conclusions: FFAC is a robust and sensitive vasomotion segmentation algorithm in both transverse and longitudinal views. Transverse imaging may offer advantages over longitudinal imaging: transverse measurements are more consistent, possibly because the method is less sensitive to variations in transducer pressure during imaging. PMID:24506648

Strategies for clinical approach to neurodegeneration in Amyotrophic lateral sclerosis.

PubMed

Carlesi, Cecilia; Pasquali, Livia; Piazza, Selina; Lo Gerfo, Annalisa; Caldarazzo Ienco, Elena; Alessi, Rosaria; Fornai, Francesco; Siciliano, Gabriele

2011-03-01

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and ultimately fatal neurodegenerative disorder of unknown aetiology that involves the loss of upper and lower motor neurons in the cerebral cortex, brainstem and spinal cord. Significant progress in understanding the cellular mechanisms of motor neuron degeneration in ALS has not been matched with the development of therapeutic strategies to prevent disease progression, and riluzole remains the only available therapy, with only marginal effects on disease survival. More recently alterations of mRNA processing in genetically defined forms of ALS, as those related to TDP-43 and FUS-TLS gene mutations have provided important insights into the molecular networks implicated in the disease pathogenesis. Here we review some of the recent progress in promoting therapeutic strategies for neurodegeneration.

[Palsy of the upper limb: Obstetrical brachial plexus palsy, arthrogryposis, cerebral palsy].

PubMed

Salazard, B; Philandrianos, C; Tekpa, B

2016-10-01

"Palsy of the upper limb" in children includes various diseases which leads to hypomobility of the member: cerebral palsy, arthrogryposis and obstetrical brachial plexus palsy. These pathologies which differ on brain damage or not, have the same consequences due to the early achievement: negligence, stiffness and deformities. Regular entire clinical examination of the member, an assessment of needs in daily life, knowledge of the social and family environment, are key points for management. In these pathologies, the rehabilitation is an emergency, which began at birth and intensively. Splints and physiotherapy are part of the treatment. Surgery may have a functional goal, hygienic or aesthetic in different situations. The main goals of surgery are to treat: joints stiffness, bones deformities, muscles contractures and spasticity, paresis, ligamentous laxity. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Dexpramipexole versus placebo for patients with amyotrophic lateral sclerosis (EMPOWER): a randomised, double-blind, phase 3 trial.

PubMed

Cudkowicz, Merit E; van den Berg, Leonard H; Shefner, Jeremy M; Mitsumoto, Hiroshi; Mora, Jesus S; Ludolph, Albert; Hardiman, Orla; Bozik, Michael E; Ingersoll, Evan W; Archibald, Donald; Meyers, Adam L; Dong, Yingwen; Farwell, Wildon R; Kerr, Douglas A

2013-11-01

In a phase 2 study, dexpramipexole (25-150 mg twice daily) was well tolerated for up to 9 months and showed a significant benefit at the high dose in a combined assessment of function and mortality in patients with amyotrophic lateral sclerosis. We aimed to assess efficacy and safety of dexpramipexole in a phase 3 trial of patients with familial or sporadic disease. In our randomised, double-blind, placebo-controlled phase 3 trial (EMPOWER), we enrolled participants aged 18-80 years (with first amyotrophic lateral sclerosis symptom onset 24 months or less before baseline) at 81 academic medical centres in 11 countries. We randomly allocated eligible participants (1:1) with a centralised voice-interactive online system to twice-daily dexpramipexole 150 mg or matched placebo for 12-18 months, stratified by trial site, area of disease onset (bulbar vs other areas), and previous use of riluzole. The primary endpoint was the combined assessment of function and survival (CAFS) score, based on changes in amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R) total scores and time to death up to 12 months. We assessed the primary endpoint in all participants who received at least one dose and had at least one post-dose ALSFRS-R measurement or died. We monitored adverse events in all participants. This study is registered with ClinicalTrials.gov, number NCT01281189. Between March 28, 2011, and Sept 30, 2011, we enrolled 943 participants (474 randomly allocated dexpramipexole, 468 randomly allocated placebo, and one withdrew). Least-square mean CAFS scores at 12 months did not differ between participants in the dexpramipexole group (score 441·76, 95% CI 415·43-468·08) and those in the placebo group (438·84, 412·81-464·88; p=0·86). At 12 months, we noted no differences in mean change from baseline in ALSFRS-R total score (-13·34 in the dexpramipexole group vs -13·42 in the placebo group; p=0·90) or time to death (74 [16%] vs 79 [17%]; hazard ratio

Endocannabinoids in Multiple Sclerosis and Amyotrophic Lateral Sclerosis.

PubMed

Pryce, Gareth; Baker, David

2015-01-01

There are numerous reports that people with multiple sclerosis (MS) have for many years been self-medicating with illegal street cannabis or more recently medicinal cannabis to alleviate the symptoms associated with MS and also amyotrophic lateral sclerosis (ALS). These anecdotal reports have been confirmed by data from animal models and more recently clinical trials on the ability of cannabinoids to alleviate limb spasticity, a common feature of progressive MS (and also ALS) and neurodegeneration. Experimental studies into the biology of the endocannabinoid system have revealed that cannabinoids have efficacy, not only in symptom relief but also as neuroprotective agents which may slow disease progression and thus delay the onset of symptoms. This review discusses what we now know about the endocannabinoid system as it relates to MS and ALS and also the therapeutic potential of cannabinoid therapeutics as disease-modifying or symptom control agents, as well as future therapeutic strategies including the potential for slowing disease progression in MS and ALS.

Genetic correlation between amyotrophic lateral sclerosis and schizophrenia

NASA Astrophysics Data System (ADS)

McLaughlin, Russell L.; Schijven, Dick; van Rheenen, Wouter; van Eijk, Kristel R.; O'Brien, Margaret; Kahn, René S.; Ophoff, Roel A.; Goris, An; Bradley, Daniel G.; Al-Chalabi, Ammar; van den Berg, Leonard H.; Luykx, Jurjen J.; Hardiman, Orla; Veldink, Jan H.; Shatunov, Aleksey; Dekker, Annelot M.; Diekstra, Frank P.; Pulit, Sara L.; van der Spek, Rick A. A.; van Doormaal, Perry T. C.; Sproviero, William; Jones, Ashley R.; Nicholson, Garth A.; Rowe, Dominic B.; Pamphlett, Roger; Kiernan, Matthew C.; Bauer, Denis; Kahlke, Tim; Williams, Kelly; Eftimov, Filip; Fogh, Isabella; Ticozzi, Nicola; Lin, Kuang; Millecamps, Stéphanie; Salachas, François; Meininger, Vincent; de Carvalho, Mamede; Pinto, Susana; Mora, Jesus S.; Rojas-García, Ricardo; Polak, Meraida; Chandran, Siddharthan; Colville, Shuna; Swingler, Robert; Morrison, Karen E.; Shaw, Pamela J.; Hardy, John; Orrell, Richard W.; Pittman, Alan; Sidle, Katie; Fratta, Pietro; Malaspina, Andrea; Petri, Susanne; Abdulla, Susanna; Drepper, Carsten; Sendtner, Michael; Meyer, Thomas; Wiedau-Pazos, Martina; Lomen-Hoerth, Catherine; van Deerlin, Vivianna M.; Trojanowski, John Q.; Elman, Lauren; McCluskey, Leo; Basak, Nazli; Meitinger, Thomas; Lichtner, Peter; Blagojevic-Radivojkov, Milena; Andres, Christian R.; Maurel, Cindy; Bensimon, Gilbert; Landwehrmeyer, Bernhard; Brice, Alexis; Payan, Christine A. M.; Saker-Delye, Safa; Dürr, Alexandra; Wood, Nicholas; Tittmann, Lukas; Lieb, Wolfgang; Franke, Andre; Rietschel, Marcella; Cichon, Sven; Nöuthen, Markus M.; Amouyel, Philippe; Tzourio, Christophe; Dartigues, Jean-François; Uitterlinden, Andre G.; Rivadeneira, Fernando; Estrada, Karol; Hofman, Albert; Curtis, Charles; van der Kooi, Anneke J.; de Visser, Marianne; Weber, Markus; Shaw, Christopher E.; Smith, Bradley N.; Pansarasa, Orietta; Cereda, Cristina; Del Bo, Roberto; Comi, Giacomo P.; D'Alfonso, Sandra; Bertolin, Cinzia; Sorarù, Gianni; Mazzini, Letizia; Pensato, Viviana; Gellera, Cinzia; Tiloca, Cinzia; Ratti, Antonia; Calvo, Andrea; Moglia, Cristina; Brunetti, Maura; Arcuti, Simon; Capozzo, Rosa; Zecca, Chiara; Lunetta, Christian; Penco, Silvana; Riva, Nilo; Padovani, Alessandro; Filosto, Massimiliano; Blair, Ian; Leigh, P. Nigel; Casale, Federico; Chio, Adriano; Beghi, Ettore; Pupillo, Elisabetta; Tortelli, Rosanna; Logroscino, Giancarlo; Powell, John; Ludolph, Albert C.; Weishaupt, Jochen H.; Robberecht, Wim; van Damme, Philip; Brown, Robert H.; Glass, Jonathan; Landers, John E.; Andersen, Peter M.; Corcia, Philippe; Vourc'h, Patrick; Silani, Vincenzo; van Es, Michael A.; Pasterkamp, R. Jeroen; Lewis, Cathryn M.; Breen, Gerome; Ripke, Stephan; Neale, Benjamin M.; Corvin, Aiden; Walters, James T. R.; Farh, Kai-How; Holmans, Peter A.; Lee, Phil; Bulik-Sullivan, Brendan; Collier, David A.; Huang, Hailiang; Pers, Tune H.; Agartz, Ingrid; Agerbo, Esben; Albus, Margot; Alexander, Madeline; Amin, Farooq; Bacanu, Silviu A.; Begemann, Martin; Belliveau, Richard A.; Bene, Judit; Bergen, Sarah E.; Bevilacqua, Elizabeth; Bigdeli, Tim B.; Black, Donald W.; Bruggeman, Richard; Buccola, Nancy G.; Buckner, Randy L.; Byerley, William; Cahn, Wiepke; Cai, Guiqing; Campion, Dominique; Cantor, Rita M.; Carr, Vaughan J.; Carrera, Noa; Catts, Stanley V.; Chambert, Kimberley D.; Chan, Raymond C. K.; Chan, Ronald Y. L.; Chen, Eric Y. H.; Cheng, Wei; Cheung, Eric F. C.; Chong, Siow Ann; Cloninger, C. Robert; Cohen, David; Cohen, Nadine; Cormican, Paul; Craddock, Nick; Crowley, James J.; Curtis, David; Davidson, Michael; Davis, Kenneth L.; Degenhardt, Franziska; Del Favero, Jurgen; Demontis, Ditte; Dikeos, Dimitris; Dinan, Timothy; Djurovic, Srdjan; Donohoe, Gary; Drapeau, Elodie; Duan, Jubao; Dudbridge, Frank; Durmishi, Naser; Eichhammer, Peter; Eriksson, Johan; Escott-Price, Valentina; Essioux, Laurent; Fanous, Ayman H.; Farrell, Martilias S.; Frank, Josef; Franke, Lude; Freedman, Robert; Freimer, Nelson B.; Friedl, Marion; Friedman, Joseph I.; Fromer, Menachem; Genovese, Giulio; Georgieva, Lyudmila; Giegling, Ina; Giusti-Rodríguez, Paola; Godard, Stephanie; Goldstein, Jacqueline I.; Golimbet, Vera; Gopal, Srihari; Gratten, Jacob; de Haan, Lieuwe; Hammer, Christian; Hamshere, Marian L.; Hansen, Mark; Hansen, Thomas; Haroutunian, Vahram; Hartmann, Annette M.; Henskens, Frans A.; Herms, Stefan; Hirschhorn, Joel N.; Hoffmann, Per; Hofman, Andrea; Hollegaard, Mads V.; Hougaard, David M.; Ikeda, Masashi; Joa, Inge; Julià, Antonio; Kalaydjieva, Luba; Karachanak-Yankova, Sena; Karjalainen, Juha; Kavanagh, David; Keller, Matthew C.; Kennedy, James L.; Khrunin, Andrey; Kim, Yunjung; Klovins, Janis; Knowles, James A.; Konte, Bettina; Kucinskas, Vaidutis; Kucinskiene, Zita Ausrele; Kuzelova-Ptackova, Hana; Kähler, Anna K.; Laurent, Claudine; Lee, Jimmy; Lee, S. Hong; Legge, Sophie E.; Lerer, Bernard; Li, Miaoxin; Li, Tao; Liang, Kung-Yee; Lieberman, Jeffrey; Limborska, Svetlana; Loughland, Carmel M.; Lubinski, Jan; Lönnqvist, Jouko; Macek, Milan; Magnusson, Patrik K. E.; Maher, Brion S.; Maier, Wolfgang; Mallet, Jacques; Marsal, Sara; Mattheisen, Manuel; Mattingsdal, Morten; McCarley, Robert W.; McDonald, Colm; McIntosh, Andrew M.; Meier, Sandra; Meijer, Carin J.; Melegh, Bela; Melle, Ingrid; Mesholam-Gately, Raquelle I.; Metspalu, Andres; Michie, Patricia T.; Milani, Lili; Milanova, Vihra; Mokrab, Younes; Morris, Derek W.; Mors, Ole; Murphy, Kieran C.; Murray, Robin M.; Myin-Germeys, Inez; Müller-Myhsok, Bertram; Nelis, Mari; Nenadic, Igor; Nertney, Deborah A.; Nestadt, Gerald; Nicodemus, Kristin K.; Nikitina-Zake, Liene; Nisenbaum, Laura; Nordin, Annelie; O'Callaghan, Eadbhard; O'Dushlaine, Colm; O'Neill, F. Anthony; Oh, Sang-Yun; Olincy, Ann; Olsen, Line; van Os, Jim; Pantelis, Christos; Papadimitriou, George N.; Papiol, Sergi; Parkhomenko, Elena; Pato, Michele T.; Paunio, Tiina; Pejovic-Milovancevic, Milica; Perkins, Diana O.; Pietiläinen, Olli; Pimm, Jonathan; Pocklington, Andrew J.; Price, Alkes; Pulver, Ann E.; Purcell, Shaun M.; Quested, Digby; Rasmussen, Henrik B.; Reichenberg, Abraham; Reimers, Mark A.; Richards, Alexander L.; Roffman, Joshua L.; Roussos, Panos; Ruderfer, Douglas M.; Salomaa, Veikko; Sanders, Alan R.; Schall, Ulrich; Schubert, Christian R.; Schulze, Thomas G.; Schwab, Sibylle G.; Scolnick, Edward M.; Scott, Rodney J.; Seidman, Larry J.; Shi, Jianxin; Sigurdsson, Engilbert; Silagadze, Teimuraz; Silverman, Jeremy M.; Sim, Kang; Slominsky, Petr; Smoller, Jordan W.; So, Hon-Cheong; Spencer, Chris C. A.; Stahl, Eli A.; Stefansson, Hreinn; Steinberg, Stacy; Stogmann, Elisabeth; Straub, Richard E.; Strengman, Eric; Strohmaier, Jana; Stroup, T. Scott; Subramaniam, Mythily; Suvisaari, Jaana; Svrakic, Dragan M.; Szatkiewicz, Jin P.; Söderman, Erik; Thirumalai, Srinivas; Toncheva, Draga; Tosato, Sarah; Veijola, Juha; Waddington, John; Walsh, Dermot; Wang, Dai; Wang, Qiang; Webb, Bradley T.; Weiser, Mark; Wildenauer, Dieter B.; Williams, Nigel M.; Williams, Stephanie; Witt, Stephanie H.; Wolen, Aaron R.; Wong, Emily H. M.; Wormley, Brandon K.; Xi, Hualin Simon; Zai, Clement C.; Zheng, Xuebin; Zimprich, Fritz; Wray, Naomi R.; Stefansson, Kari; Visscher, Peter M.; Adolfsson, Rolf; Andreassen, Ole A.; Blackwood, Douglas H. R.; Bramon, Elvira; Buxbaum, Joseph D.; Børglum, Anders D.; Darvasi, Ariel; Domenici, Enrico; Ehrenreich, Hannelore; Esko, Tõnu; Gejman, Pablo V.; Gill, Michael; Gurling, Hugh; Hultman, Christina M.; Iwata, Nakao; Jablensky, Assen V.; Jönsson, Erik G.; Kendler, Kenneth S.; Kirov, George; Knight, Jo; Lencz, Todd; Levinson, Douglas F.; Li, Qingqin S.; Liu, Jianjun; Malhotra, Anil K.; McCarroll, Steven A.; McQuillin, Andrew; Moran, Jennifer L.; Mortensen, Preben B.; Mowry, Bryan J.; Owen, Michael J.; Palotie, Aarno; Pato, Carlos N.; Petryshen, Tracey L.; Posthuma, Danielle; Riley, Brien P.; Rujescu, Dan; Sham, Pak C.; Sklar, Pamela; St Clair, David; Weinberger, Daniel R.; Wendland, Jens R.; Werge, Thomas; Daly, Mark J.; Sullivan, Patrick F.; O'Donovan, Michael C.

2017-03-01

We have previously shown higher-than-expected rates of schizophrenia in relatives of patients with amyotrophic lateral sclerosis (ALS), suggesting an aetiological relationship between the diseases. Here, we investigate the genetic relationship between ALS and schizophrenia using genome-wide association study data from over 100,000 unique individuals. Using linkage disequilibrium score regression, we estimate the genetic correlation between ALS and schizophrenia to be 14.3% (7.05-21.6 P=1 × 10-4) with schizophrenia polygenic risk scores explaining up to 0.12% of the variance in ALS (P=8.4 × 10-7). A modest increase in comorbidity of ALS and schizophrenia is expected given these findings (odds ratio 1.08-1.26) but this would require very large studies to observe epidemiologically. We identify five potential novel ALS-associated loci using conditional false discovery rate analysis. It is likely that shared neurobiological mechanisms between these two disorders will engender novel hypotheses in future preclinical and clinical studies.

Genetic correlation between amyotrophic lateral sclerosis and schizophrenia.

PubMed

McLaughlin, Russell L; Schijven, Dick; van Rheenen, Wouter; van Eijk, Kristel R; O'Brien, Margaret; Kahn, René S; Ophoff, Roel A; Goris, An; Bradley, Daniel G; Al-Chalabi, Ammar; van den Berg, Leonard H; Luykx, Jurjen J; Hardiman, Orla; Veldink, Jan H

2017-03-21

We have previously shown higher-than-expected rates of schizophrenia in relatives of patients with amyotrophic lateral sclerosis (ALS), suggesting an aetiological relationship between the diseases. Here, we investigate the genetic relationship between ALS and schizophrenia using genome-wide association study data from over 100,000 unique individuals. Using linkage disequilibrium score regression, we estimate the genetic correlation between ALS and schizophrenia to be 14.3% (7.05-21.6; P=1 × 10 -4 ) with schizophrenia polygenic risk scores explaining up to 0.12% of the variance in ALS (P=8.4 × 10 -7 ). A modest increase in comorbidity of ALS and schizophrenia is expected given these findings (odds ratio 1.08-1.26) but this would require very large studies to observe epidemiologically. We identify five potential novel ALS-associated loci using conditional false discovery rate analysis. It is likely that shared neurobiological mechanisms between these two disorders will engender novel hypotheses in future preclinical and clinical studies.

Amyotrophic lateral sclerosis: A 40-year personal perspective.

PubMed

Eisen, Andrew

2009-04-01

Amyotrophic lateral sclerosis (ALS) or motor neuron disease (MND) shares with other neurodegenetrative disorders of the aging nervous system a polygenic, multifactorial aetiology. Less than 10% are familial and these too probably are associated with several interactive genes. The onset of ALS predates development of clinical symptoms by an unknown interval which may extend several years. The cause of neurodegeneration remains unknown but a common end-point is protein misfolding which in turn causes cell function failure. The complex nature of ALS has hindered therapeutic advances. In recent years longer survival is attributable largely to institution of non-invasive ventilation with BiPAP and timely implementation of percutaneous endoscopic gastrostomy (PEG) feeding. Symptomatic treatment has advanced improving quality of life. Several encouraging avenues of therapy for ALS are beginning to be emerge raising hope for real benefit. They include protective autoimmunity, vaccines against misfolded protein epitopes and other deleterious species, new drug delivery systems employing nanotechnology and the potential of stem cell therapy.

Kinematic Features of Jaw and Lips Distinguish Symptomatic from Presymptomatic Stages of Bulbar Decline in Amyotrophic Lateral Sclerosis

ERIC Educational Resources Information Center

Bandini, Andrea; Green, Jordan R.; Wang, Jun; Campbell, Thomas F.; Zinman, Lorne; Yunusova, Yana

2018-01-01

Purpose: The goals of this study were to (a) classify speech movements of patients with amyotrophic lateral sclerosis (ALS) in presymptomatic and symptomatic phases of bulbar function decline relying solely on kinematic features of lips and jaw and (b) identify the most important measures that detect the transition between early and late bulbar…

Cellular Therapy for Chronic Traumatic Brachial Plexus Injury

PubMed Central

Sharma, Alok; Sane, Hemangi; Gokulchandran, Nandini; Badhe, Prerna; Pai, Suhasini; Kulkarni, Pooja; Yadav, Jayanti; Inamdar, Sanket

2018-01-01

Cellular therapy is being actively pursued as a therapeutic modality in many of the neurological diseases. A variety of stem cells from diverse sources have been studied in detail and have been shown to exhibit angiogenetic and immunomodulatory properties in addition to other neuroprotective effects. Published clinical data have shown bone marrow mononuclear cell (BMMNC) injection in neurological disorders is safe and possesses regenerative potential. We illustrate a case of 27-year-old male with traumatic brachial plexus injury, administered with autologous BMMNCs intrathecally and intramuscularly, followed by multidisciplinary rehabilitation. At the follow-up assessment of 3 and 7 months after first cell transplantation, improvements were recorded in muscle strength and movements. Electromyography (EMG) performed after the intervention showed a response in biceps and deltoid muscles suggesting the process of reinnervation at the site of injury. In view of the improvements observed after the treatment, the patient underwent second cell transplantation 8 months after the first transplantation. Muscle wasting had completely stopped with an increase in the muscle girth. No adverse effects were noted. Improvements were maintained for 4 years. A comprehensive randomized study for this type of injury is needed to establish the therapeutic benefits of cellular therapy. PMID:29657936

Sphingolipid Metabolism Is Dysregulated at Transcriptomic and Metabolic Levels in the Spinal Cord of an Animal Model of Amyotrophic Lateral Sclerosis

PubMed Central

Henriques, Alexandre; Croixmarie, Vincent; Bouscary, Alexandra; Mosbach, Althéa; Keime, Céline; Boursier-Neyret, Claire; Walter, Bernard; Spedding, Michael; Loeffler, Jean-Philippe

2018-01-01

Lipid metabolism is drastically dysregulated in amyotrophic lateral sclerosis and impacts prognosis of patients. Animal models recapitulate alterations in the energy metabolism, including hypermetabolism and severe loss of adipose tissue. To gain insight into the molecular mechanisms underlying disease progression in amyotrophic lateral sclerosis, we have performed RNA-sequencing and lipidomic profiling in spinal cord of symptomatic SOD1G86R mice. Spinal transcriptome of SOD1G86R mice was characterized by differential expression of genes related to immune system, extracellular exosome, and lysosome. Hypothesis-driven identification of metabolites showed that lipids, including sphingomyelin(d18:0/26:1), ceramide(d18:1/22:0), and phosphatidylcholine(o-22:1/20:4) showed profound altered levels. A correlation between disease severity and gene expression or metabolite levels was found for sphingosine, ceramide(d18:1/26:0), Sgpp2, Sphk1, and Ugt8a. Joint-analysis revealed a significant enrichment of glycosphingolipid metabolism in SOD1G86R mice, due to the down-regulation of ceramide, glucosylceramide, and lactosylceramide and the overexpression of genes involved in their recycling in the lysosome. A drug-gene interaction database was interrogated to identify potential drugs able to modulate the dysregulated genes from the signaling pathway. Our results suggest that complex lipids are pivotally changed during the first phase of motor symptoms in an animal model of amyotrophic lateral sclerosis. PMID:29354030

Impact of a systolic parameter, defined as the ratio of right brachial pre-ejection period to ejection time, on the relationship between brachial-ankle pulse wave velocity and left ventricular diastolic function.

PubMed

Hsu, Po-Chao; Lin, Tsung-Hsien; Lee, Chee-Siong; Chu, Chun-Yuan; Su, Ho-Ming; Voon, Wen-Chol; Lai, Wen-Ter; Sheu, Sheng-Hsiung

2011-04-01

Arterial stiffness is correlated with left ventricular (LV) diastolic function as well as susceptibility to LV systolic function. Therefore, if LV systolic function is not known, the relationship between arterial stiffness and LV diastolic function is difficult to determine. A total of 260 patients were included in the study. The brachial-ankle pulse wave velocity (baPWV) and the ratio of right brachial pre-ejection period to ejection time (rbPEP/rbET) were measured using an ABI-form device. Patients were classified into four groups. Groups 1, 2, 3 and 4 were patients with rbPEP/rbET and baPWV below the median, rbPEP/rbET above but baPWV below the median, rbPET/rbET below but baPWV above the median, and rbPET/rbET and baPWV above the median, respectively. The LV ejection fractions in groups 1 and 3 were higher than those in groups 2 and 4 (P<0.001 for all). Patients in group 1 had a lower left atrial volume index (LAVI) and higher early diastolic mitral annular velocity (Ea) than patients in the other groups (P≤0.002). Patients in group 2 had a LAVI and ratio of transmitral E wave velocity to Ea that were comparable to those in groups 3 and 4. In conclusion, rbPEP/rbET had an impact on the relationship between baPWV and LV diastolic function. In patients with high rbPEP/rbET but low baPWV, low baPWV may not indicate good LV diastolic function but implies that cardiac dysfunction may precede vascular dysfunction in such patients. When interpreting the relationship between baPWV and LV diastolic function, the rbPEP/rbET value obtained from the same examination should be considered.

Clinical aspects of patients with traumatic lesions of the brachial plexus following surgical treatment☆

PubMed Central

de Moraes, Frederico Barra; Kwae, Mário Yoshihide; da Silva, Ricardo Pereira; Porto, Celmo Celeno; Magalhães, Daniel de Paiva; Paulino, Matheus Veloso

2015-01-01

Objective To evaluate sociodemographic and clinical aspects of patients undergoing operations due to traumatic lesions of the brachial plexus. Method This was a retrospective study in which the medical files of a convenience sample of 48 patients operated between 2000 and 2010 were reviewed. The following were evaluated: (1) range of motion (ROM) of the shoulder, elbow and wrist/hand, in degrees; (2) grade of strength of the shoulder, elbow and wrist/hand; (3) sensitivity; and (4) visual analogue scale (VAS) (from 0 to 10). The Student's t, chi-square, Friedman, Wilcoxon and Kruskal–Wallis tests were used (p < 0.05). Results The patients’ mean age was 30.6 years; 60.4% of them had suffered motorcycle accidents and 52.1%, multiple trauma. The mean length of time until surgery was 8.7 months (range: 2–48). Thirty-one patients (64.6%) presented complete rupture of the plexus. The frequent operation was neurosurgery in 39 cases (81.3%). The ROM achieved was ≥30° in 20 patients (41.6%), with a range from 30° to 90° and mean of 73° (p = 0.001). Thirteen (27.1%) already had shoulder strength ≥M3 (p = 0.001). Twenty-seven patients (56.2%) had elbow flexion ≥80°, with a range from 30° to 160° and mean of 80.6° (p < 0.001). Twenty-two had strength ≥M3 (p < 0.001). Twenty-two patients (45.8%) had wrist extension ≥30° starting from flexion of 45°, with a range from 30° to 90° and mean of 70° (p = 0.003). Twenty-seven (56.3%) presented wrist/hand extension strength ≥M3 (p = 0.002). Forty-five (93.8%) had hypoesthesia and three (6.2%) had anesthesia (p = 0.006). The initial VAS was 4.5 (range: 1.0–9.0) and the final VAS was 3.0 (range: 1.0–7.0) (p < 0.001). Conclusion Traumatic lesions of the brachial plexus were more prevalent among young adults (21–40 years), men, people living in urban areas, manual workers and motorcycle accidents, with multiple trauma and total rupture of the plexus. Neurosurgery, with a second

Experiences of burden, needs, rewards and resilience in family caregivers of people living with Motor Neurone Disease/Amyotrophic Lateral Sclerosis: A secondary thematic analysis of qualitative interviews.

PubMed

Weisser, Fabia B; Bristowe, Katherine; Jackson, Diana

2015-09-01

Family caregivers of people with Motor Neurone Disease/Amyotrophic Lateral Sclerosis, an incurable, mostly rapidly fatal neurodegenerative disease, face many challenges. Although there is considerable research on caregiver burden in Motor Neurone Disease/Amyotrophic Lateral Sclerosis, there is less knowledge of the positive aspects of caring. To explore the experiences of family caregivers of people with Motor Neurone Disease/Amyotrophic Lateral Sclerosis, specifically the relationship between positive and negative experiences of caring, and to identify possible ways to better support these caregivers. Secondary thematic analysis of 24 semi-structured qualitative interviews conducted longitudinally with 10 family caregivers. Interviews explored rewarding and unrewarding aspects of caring. Themes emerged around burden, needs, rewards and resilience. Resilience included getting active, retaining perspective and living for the moment. Burden was multifaceted, including social burden, responsibility, advocacy, ambivalence, guilt and struggling with acceptance. Rewards included being helped and 'ticking along'. Needs were multifaceted, including social, practical and psychological needs. The four main themes were interrelated. A model of coping was developed, integrating resilience (active/positive), burden (active/negative), needs (passive/negative) and reward (passive/positive). Burden, resilience, needs and rewards are interrelated. Caregivers' ability to cope with caring for a person with Motor Neurone Disease/Amyotrophic Lateral Sclerosis oscillates between positive and negative aspects of caring, being at times active, at times passive. Coping is a non-linear process, oscillating between different states of mind. The proposed model could enable clinicians to better understand the caregiver experience, help family caregivers foster resilience and identify rewards, and develop appropriate individualised caregiver support plans. © The Author(s) 2015.

Emerging understanding of the genotype-phenotype relationship in amyotrophic lateral sclerosis.

PubMed

Goutman, Stephen A; Chen, Kevin S; Paez-Colasante, Ximena; Feldman, Eva L

2018-01-01

Amyotrophic lateral sclerosis (ALS) is a progressive, noncurable neurodegenerative disorder of the upper and lower motor neurons causing weakness and death within a few years of symptom onset. About 10% of patients with ALS have a family history of the disease; however, ALS-associated genetic mutations are also found in sporadic cases. There are over 100 ALS-associated mutations, and importantly, several genetic mutations, including C9ORF72, SOD1, and TARDBP, have led to mechanistic insight into this complex disease. In the clinical realm, knowledge of ALS genetics can also help explain phenotypic heterogeneity, aid in genetic counseling, and in the future may help direct treatment efforts. Copyright © 2018 Elsevier B.V. All rights reserved.

[Types of ventilatory support and their indications in amyotrophic lateral sclerosis].

PubMed

Perrin, C

2006-06-01

Respiratory muscle weakness represents the major cause of mortality in patients with amyotrophic lateral sclerosis (ALS). As a result, ventilatory assistance is an important part of disease management. Nowadays, noninvasive ventilation (NIV) has become the first choice modality for most patients and represents an alternative to tracheostomy intermittent positive-pressure ventilation. Although, some consensus guidelines have been proposed to initiate NIV in patients with restrictive chronic respiratory failure, these criteria are discussed regarding ALS. While the current consensus recommends that NIV may be used in symptomatic patients with hypercapnia or forced vital capacity<50p.cent of predicted value, early use of NIV is proposed in the literature and reported in this paper.

Amyotrophic lateral sclerosis: recent genetic highlights.

PubMed

White, Matthew A; Sreedharan, Jemeen

2016-10-01

Amyotrophic lateral sclerosis (ALS), like other neurodegenerative diseases, remains incurable, but gene mutations linked to ALS are providing clues as to how to target therapies. It is important for researchers to keep abreast of the rapid influx of new data in ALS, and we aim to summarize the major genetic advances made in the field over the past 2 years. Significant variation in seven genes has recently been found in ALS: TBK1, CCNF, GLE1, MATR3, TUBA4A, CHCHD10 and NEK1. These have mostly been identified through large exome screening studies, though traditional linkage approaches and candidate gene screening remain important. We briefly update C9orf72 research, noting in particular the development of reagents to better understand the normal role of C9orf72 protein. Striking advances in our understanding of the genetic heterogeneity of ALS continue to be made, year on year. These implicate proteostasis, RNA export, nuclear transport, the cytoskeleton, mitochondrial function, the cell cycle and DNA repair. Functional studies to integrate these hits are needed. By building a web of knowledge with interlinked genes and mechanisms, it is hoped we can better understand ALS and work toward effective therapies.

A Rare Form of Guillan Barre Syndrome: A Child Diagnosed with Anti-GD1a and Anti-GD1b Positive Pharyngeal-Cervical-Brachial Variant.

PubMed

Uysalol, Metin; Tatlı, Burak; Uzel, Nedret; Cıtak, Agop; Aygün, Erhan; Kayaoğlu, Semra

2013-09-01

Pharyngeal-cervical-brachial (PCB) variant is a rare form of Guillan-Barre Syndrome (GBS). Antibodies against other membrane proteins like GM1b and GD1a have been found only in a small number of patients with Guillan Barre syndrome variant. Here, we report a 5.5 year-old boy diagnosed early with positive GD1a and GD1b gangliosides of Guillan-Barre syndrome pharyngeal cervical-Brachial variant, who improved and recovered fully in a short period. This is in contrast to those whose recovery period prolongs in spite of early diagnosis and appropriate treatment and/or those who experience incomplete recovery. In summary, diagnosis of PCB variant of GBS should be considered in infants with sudden onset bulbar symptoms and muscle weakness, and it should be kept in mind that early diagnosis and appropriate treatment can give successful outcomes.

Proximal opening wedge osteotomy with wedge-plate fixation compared with proximal chevron osteotomy for the treatment of hallux valgus: a prospective, randomized study.

PubMed

Glazebrook, Mark; Copithorne, Peter; Boyd, Gordon; Daniels, Timothy; Lalonde, Karl-André; Francis, Patricia; Hickey, Michael

2014-10-01

Hallux valgus with an increased intermetatarsal angle is usually treated with a proximal metatarsal osteotomy. The proximal chevron osteotomy is commonly used but is technically difficult. This study compares the proximal opening wedge osteotomy of the first metatarsal with the proximal chevron osteotomy for the treatment of hallux valgus with an increased intermetatarsal angle. This prospective, randomized multicenter (three-center) study was based on the clinical outcome scores of the Short Form-36, the American Orthopaedic Foot & Ankle Society forefoot questionnaire, and the visual analog scale for pain, activity, and patient satisfaction. Subjects were assessed prior to surgery and at three, six, and twelve months postoperatively. Surgeon preference was evaluated based on questionnaires and the operative times required for each procedure. No significant differences were found for any of the patients' clinical outcome measurements between the two procedures. The proximal opening wedge osteotomy was found to lengthen, and the proximal chevron osteotomy was found to shorten, the first metatarsal. The intermetatarsal angles improved (decreased) significantly, from 14.8° ± 3.2° to 9.1° ± 2.9 (mean and standard deviation) after a proximal opening wedge osteotomy and from 14.6° ± 3.9° to 11.3° ± 4.0° after a proximal chevron osteotomy (p < 0.05 for both). Operative time required for performing a proximal opening wedge osteotomy is similar to that required for performing a proximal chevron osteotomy (mean and standard deviation, 67.1 ± 16.5 minutes compared with 69.9 ± 18.6 minutes; p = 0.510). Opening wedge and proximal chevron osteotomies have comparable radiographic outcomes and comparable clinical outcomes for pain, satisfaction, and function. The proximal opening wedge osteotomy lengthens, and the proximal chevron osteotomy shortens, the first metatarsal. The proximal opening wedge osteotomy was subjectively less technically demanding and was

A prospective study of quality of life in amyotrophic lateral sclerosis patients.

PubMed

Jakobsson Larsson, B; Ozanne, A G; Nordin, K; Nygren, I

2017-12-01

The aim of this prospective and longitudinal study was to describe individual quality of life in patients with amyotrophic lateral sclerosis (ALS) and its correlations with physical function and emotional well-being from diagnosis and over time. Thirty-six patients were included in the study. Individual quality of life was measured with the Schedule of Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW), illness severity was assessed using the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALS FRS-R), and emotional distress was measured using the Hospital Anxiety and Depression Scale (HADS). Data were collected from diagnosis and thereafter, every six months for a period of two years. Twelve patients completed the 24-month follow-up. Family, friends and own physical health were important for overall quality of life, from diagnosis and during the disease progression. Most patients had good quality of life, which remained stable, despite changed physical functions. Several patients scored above the cut-off score for doubtful and clinical anxiety and depression early on after diagnosis, and there was a significant decrease in anxiety over time. Soon after diagnosis, there was a correlation between depression and quality of life. The family, social relations and own physical health are important for overall quality of life in patients with ALS. Thus, supporting the family and facilitating so that patients can continue to stay in contact with friends are important aspects during the disease. Conducting an early screening for depression can be important for preventing decreased quality of life. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Military service, deployments, and exposures in relation to amyotrophic lateral sclerosis survival.

PubMed

Beard, John D; Engel, Lawrence S; Richardson, David B; Gammon, Marilie D; Baird, Coleen; Umbach, David M; Allen, Kelli D; Stanwyck, Catherine L; Keller, Jean; Sandler, Dale P; Schmidt, Silke; Kamel, Freya

2017-01-01

Military veterans may have higher rates of amyotrophic lateral sclerosis (ALS) mortality than non-veterans. Few studies, with sparse exposure information and mixed results, have studied relationships between military-related factors and ALS survival. We evaluated associations between military-related factors and ALS survival among U.S. military veteran cases. We followed 616 medical record-confirmed cases from enrollment (2005-2010) in the Genes and Environmental Exposures in Veterans with Amyotrophic Lateral Sclerosis study until death or July 25, 2013, whichever came first. We ascertained vital status information from several sources within the Department of Veterans Affairs. We obtained information regarding military service, deployments, and 39 related exposures via standardized telephone interviews. We used Cox proportional hazards regression models to estimate hazard ratios (HRs) and 95% confidence intervals. We adjusted for potential confounding and missing covariate data biases via inverse probability weights. We also used inverse probability weights to adjust for potential selection bias among a case group that included a disproportionate number of long-term survivors at enrollment. We observed 446 deaths during 24,267 person-months of follow-up (median follow-up: 28 months). Survival was shorter for cases who served before 1950, were deployed to World War II, or mixed and applied burning agents, with HRs between 1.58 and 2.57. Longer survival was associated with exposure to: paint, solvents, or petrochemical substances; local food not provided by the Armed Forces; or burning agents or Agent Orange in the field with HRs between 0.56 and 0.73. Although most military-related factors were not associated with survival, associations we observed with shorter survival are potentially important because of the large number of military veterans.

Early results for treatment of two- and three-part fractures of the proximal humerus using Contours PHP (proximal humeral plate).

PubMed

Biazzo, Alessio; Cardile, Carlo; Brunelli, Luca; Ragni, Paolo; Clementi, Daniele

2017-04-28

The management of displaced 2- and 3-part fractures of the proximal humerus is controversial, both in younger and in elderly patients. The purpose of this paper is to evaluate the functional results of the Contours Proximal Humerus Plate (OrthofixR, Bussolengo,Verona, Italy), for the treatment of displaced 2- and 3-part fractures of the proximal humerus. We retrospectively reviewed 55 patients with proximal humerus fractures, who underwent osteosynthesis with Contours Proximal Humerus Plate from December 2011 to March 2015. We had 21 patients with 2-part fractures and with an average age of 67.1 years and 34 patients with 3-part fractures, with average age of 63.6 years. The average union time was 3 months. The mean Constant score was 67 for 2-part fracture group and 64.9 for 3-part fracture group. The difference was not statistically significant (p = 0.18). The overall complication rate was 14.5 %. Six patients underwent additional surgery (10.9%). The most frequent major complication was secondary loss of reduction following varus collapse of the fracture (2 cases). In these patients, there was loss of medial hinge integrity due to impaction and osteoporosis. The placement of the main locking screw in the calcar area to provide inferomedial support is the rational of the Contours Proximal Humerus Plate. Osteosynthesis with Contours Proximal Humerus Plate is a safe system for treating displaced 2- and 3-part fractures of the proximal humerus, with good functional results and complication rates comparable to those reported in the literature.

Can we use lower volume of local anesthetic for infraclavicular brachial plexus nerve block under ultrasound guidance in children?

PubMed

Ince, Ilker; Aksoy, Mehmet; Dostbil, Aysenur; Tuncer, Kutsi

2017-09-01

To determine if the infraclavicular brachial plexus block can be applied with lower volume of local anesthetic. Randomised, double-blinded clinical trial. 60 patients aged 5-15years with ASA I-II who underwent emergent or elective arm, forearm or hand operations were included in the study. Patients were divided into two groups randomly; standard volume local anesthetic administered group (Group S, n=30) and low volume anesthetic administered group (Group L, n=30). Postoperative pain scores, sensory and motor block durations were noted. Pain scores (Wong-Baker Face Scale) were evaluated and the results were detected to be similar at all times (30min, 1, 2, 4, 8, 12, 24h). Durations of motor block were 168(±16) minutes and 268(±15) minutes in Group L and Group S respectively and the difference was statistically significant (p<0.001). Durations of sensory block were 385(±26) and 402(±39) in Group L and Group S respectively and no statistically significant difference was detected (p=0.064). Similar block success, postoperative sensory block durations and pain scores could be obtained during infraclavicular brachial plexus in pediatric patients with lower local anesthetic volumes. Copyright © 2016 Elsevier Inc. All rights reserved.

Incidence of amyotrophic lateral sclerosis in three counties in western Washington state.

PubMed

McGuire, V; Longstreth, W T; Koepsell, T D; van Belle, G

1996-08-01

We conducted a population-based study of amyotrophic lateral sclerosis (ALS) in King, Pierce, and Snohomish counties in western Washington state. Between April 1, 1990 and March 31, 1995, neurologists diagnosed 235 patients with ALS, including 127 men (54%) and 108 women (46%). The incidence rate, age-adjusted to the 1990 total U.S. population, was higher for men at 2.1 per 100,000 per year (95% CI, 1.3 to 2.9) than for women at 1.9 (95% CI, 1.1, 2.7) and increased with age for both men and women. These incidence rates are consistent with other studies from northern latitudes.

Metabolic Dysregulation in Amyotrophic Lateral Sclerosis: Challenges and Opportunities.

PubMed

Joardar, Archi; Manzo, Ernesto; Zarnescu, Daniela C

2017-06-01

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease for which there is no cure and treatments are at best palliative. Several genes have been linked to ALS, which highlight defects in multiple cellular processes including RNA processing, proteostasis and metabolism. Clinical observations have identified glucose intolerance and dyslipidemia as key features of ALS however the causes of these metabolic alterations remain elusive. Recent studies reveal that motor neurons and muscle cells may undergo cell type specific metabolic changes that lead to utilization of alternate fuels. For example, ALS patients' muscles exhibit reduced glycolysis and increased reliance on fatty acids. In contrast, ALS motor neurons contain damaged mitochondria and exhibit impaired lipid beta oxidation, potentially leading to increased glycolysis as a compensatory mechanism. These findings highlight the complexities of metabolic alterations in ALS and provide new opportunities for designing therapeutic strategies based on restoring cellular energetics.

Proximal Hamstring Tendinosis and Partial Ruptures.

PubMed

Startzman, Ashley N; Fowler, Oliver; Carreira, Dominic

2017-07-01

Proximal hamstring tendinosis and partial hamstring origin ruptures are painful conditions of the proximal thigh and hip that may occur in the acute, chronic, or acute on chronic setting. Few publications exist related to their diagnosis and management. This systematic review discusses the incidence, treatment, and prognosis of proximal hamstring tendinosis and partial hamstring ruptures. Conservative treatment measures include nonsteroidal anti-inflammatory drugs, physical therapy, rest, and ice. If these measures fail, platelet-rich plasma or shockwave therapy may be considered. When refractory to conservative management, these injuries may be treated with surgical debridement and hamstring reattachment. [Orthopedics. 2017; 40(4):e574-e582.]. Copyright 2017, SLACK Incorporated.

14 CFR 135.153 - Ground proximity warning system.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 14 Aeronautics and Space 3 2011-01-01 2011-01-01 false Ground proximity warning system. 135.153... Equipment § 135.153 Ground proximity warning system. (a) No person may operate a turbine-powered airplane... equipped with an approved ground proximity warning system. (b) [Reserved] (c) For a system required by this...

14 CFR 135.153 - Ground proximity warning system.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 14 Aeronautics and Space 3 2010-01-01 2010-01-01 false Ground proximity warning system. 135.153... Equipment § 135.153 Ground proximity warning system. (a) No person may operate a turbine-powered airplane... equipped with an approved ground proximity warning system. (b) [Reserved] (c) For a system required by this...

Military service, deployments, and exposures in relation to amyotrophic lateral sclerosis etiology and survival.

PubMed

Beard, John D; Kamel, Freya

2015-01-01

Rates of amyotrophic lateral sclerosis (ALS) have been reported to be higher among US military veterans, who currently number more than 21 million, but the causal factor(s) has not been identified. We conducted a review to examine the weight of evidence for associations between military service, deployments, and exposures and ALS etiology and survival. Thirty articles or abstracts published through 2013 were reviewed. Although the current evidence suggests a positive association with ALS etiology, it is too limited to draw firm conclusions regarding associations between military service and ALS etiology or survival. Some evidence suggests that deployment to the 1990-1991 Persian Gulf War may be associated with ALS etiology, but there is currently no strong evidence that any particular military exposure is associated with ALS etiology. Future studies should address the limitations of previous ones, such as reliance on mortality as a surrogate for incidence, a dearth of survival analyses, lack of clinical data, low statistical power, and limited exposure assessment. The Genes and Environmental Exposures in Veterans with Amyotrophic Lateral Sclerosis (GENEVA) Study is one such study, but additional research is needed to determine whether military-related factors are associated with ALS and to assess potential prevention strategies. Published by Oxford University Press on behalf of the Johns Hopkins Bloomberg School of Public Health 2014. This work is written by (a) US Government employee(s) and is in the public domain in the US.

Loss of tubular creatinine secretion as the only sign of tubular proximal cell dysfunction in light chain proximal tubulopathy: A case report.

PubMed

Stehlé, Thomas; Vignon, Marguerite; Flamant, Martin; Figueres, Marie-Lucile; Rabant, Marion; Rodenas, Anita; Noël, Laure-Hélène; Arnulf, Bertrand; Vidal-Petiot, Emmanuelle

2016-06-01

Light chain proximal tubulopathy (LCPT) is a rare disease, characterized by cytoplasmic inclusions of light chain (usually kappa) immunoglobulins. Clinical presentation is usually a Fanconi syndrome. The proximal tubular dysfunction can be incomplete, and exceptional cases of LCPT without any tubular dysfunction have even been described. Here, we report a case of LCPT in which the only sign of proximal tubulopathy is the absence of secretion of creatinine, as assessed by the simultaneous measurement of renal clearance of creatinine and CrEDTA. The loss of tubular creatinine secretion as a sign of tubular proximal cell dysfunction ought to be identified in patients with light chain proximal tubulopathy as it leads to a clinically relevant underestimation of GFR by the creatinine-derived equations. The prevalence and prognostic significance of this particular proximal tubular damage in LCPT remain to be determined.

Successful outcome of modified quad surgical procedure in preteen and teen patients with brachial plexus birth palsy.

PubMed

Nath, Rahul K; Somasundaram, Chandra

2012-01-01

To evaluate the outcome of modified Quad procedure in preteen and teen patients with brachial plexus birth palsy. We have previously demonstrated a significant improvement in shoulder abduction, resulting from the modified Quad procedure in children (mean age 2.5 years; range, 0.5-9 years) with obstetric brachial plexus injury. We describe in this report the outcome of 16 patients (6 girls and 10 boys; 7 preteen and 9 teen) who have undergone the modified Quad procedure for the correction of the shoulder function, specifically abduction. The patients underwent transfer of the latissimus dorsi and teres major muscles, release of contractures of subscapularis pectoralis major and minor, and axillary nerve decompression and neurolysis (the modified Quad procedure). Mean age of these patients at surgery was 13.5 years (range, 10.1-17.9 years). The mean preoperative total Mallet score was 14.8 (range, 10-20), and active abduction was 84° (range, 20°-140°). At a mean follow-up of 1.5 years, the mean postoperative total Mallet score increased to 19.7 (range, 13-25, P < .0001), and the mean active abduction improved to 132° (range, 40°-180°, P < .0003). The modified Quad procedure greatly improves not only the active abduction but also other shoulder functions in preteen and teen patients, as this outcome is the combined result of decompression and neurolysis of the axillary nerve and the release of the contracted internal rotators of the shoulder.

New therapeutic targets for amyotrophic lateral sclerosis.

PubMed

Kuzma-Kozakiewicz, Magdalena; Kwiecinski, Hubert

2011-02-01

Amyotrophic lateral sclerosis (ALS) is one of the most devastating neurological disorders, affecting approximately half a million people worldwide. Currently there is no cure or prevention for ALS. Although ALS is a rare condition, it places a tremendous socioeconomic burden on patients, family members, caregivers and health systems. The review examines the mechanisms that may contribute to motor neuron degeneration in ALS, among which oxidative damage, glutatamate excitoxicity, mitochondrial dysfunction, impaired axonal transport, apoptotic cell death, growth factor deficiency, glial cell pathology and abnormal RNA metabolism are potential targets for ALS treatment. The article provides an overview of clinical trials performed to date in attempts to treat ALS with regard to molecular mechanisms and pathways they act on. It also discusses new trials based on recently developed molecular biology techniques. Despite significant effectiveness of several potential therapeutics observed in preclinical trials, the results were not translatable to patients with ALS. The development of effective treatments of ALS strictly depends on understanding the primary cause of the disease. This goal will only be achieved when we identify the trigger point for motor neuron death in ALS.

Preventing Proximal Adjacent Level Kyphosis With Strap Stabilization.

PubMed

Zaghloul, Khaled M; Matoian, Brett J; Denardin, Nicholas B; Patel, Vikas V

2016-07-01

A substantial proportion of patients develop proximal junctional kyphosis following spinal surgery. To combat this postoperative change, several techniques have focused on maintaining the structural integrity of adjacent spinal levels and adapting the proximal end of the fusion construct to accommodate the increased mechanical stressors produced by long spinal fusion. The use of Mersilene tape (Ethicon, Somerville, New Jersey) for spine and orthopedic surgery is well documented, although considerably less is known about its use for preventing proximal junctional kyphosis. This article describes a proposed technique using Mersilene tape to provide a check-rein strap stabilization at the proximal end of fusion constructs. Initial data suggest that use of this technique may prevent formation of proximal junctional kyphosis. [Orthopedics. 2016; 39(4):e794-e799.]. Copyright 2016, SLACK Incorporated.

Age of onset of amyotrophic lateral sclerosis is modulated by a locus on 1p34.1.

PubMed

Ahmeti, Kreshnik B; Ajroud-Driss, Senda; Al-Chalabi, Ammar; Andersen, Peter M; Armstrong, Jennifer; Birve, Anne; Blauw, Hylke M; Brown, Robert H; Bruijn, Lucie; Chen, Wenjie; Chio, Adriano; Comeau, Mary C; Cronin, Simon; Diekstra, Frank P; Soraya Gkazi, Athina; Glass, Jonathan D; Grab, Josh D; Groen, Ewout J; Haines, Jonathan L; Hardiman, Orla; Heller, Scott; Huang, Jie; Hung, Wu-Yen; Jaworski, James M; Jones, Ashley; Khan, Humaira; Landers, John E; Langefeld, Carl D; Leigh, P Nigel; Marion, Miranda C; McLaughlin, Russell L; Meininger, Vincent; Melki, Judith; Miller, Jack W; Mora, Gabriele; Pericak-Vance, Margaret A; Rampersaud, Evadnie; Robberecht, Wim; Russell, Laurie P; Salachas, Francois; Saris, Christiaan G; Shatunov, Aleksey; Shaw, Christopher E; Siddique, Nailah; Siddique, Teepu; Smith, Bradley N; Sufit, Robert; Topp, Simon; Traynor, Bryan J; Vance, Caroline; van Damme, Philip; van den Berg, Leonard H; van Es, Michael A; van Vught, Paul W; Veldink, Jan H; Yang, Yi; Zheng, J G

2013-01-01

Amyotrophic lateral sclerosis (ALS) is the third most common adult-onset neurodegenerative disease. Individuals with ALS rapidly progress to paralysis and die from respiratory failure within 3 to 5 years after symptom onset. Epidemiological factors explain only a modest amount of the risk for ALS. However, there is growing evidence of a strong genetic component to both familial and sporadic ALS risk. The International Consortium on Amyotrophic Lateral Sclerosis Genetics was established to bring together existing genome-wide association cohorts and identify sporadic ALS susceptibility and age at symptom onset loci. Here, we report the results of a meta-analysis of the International Consortium on Amyotrophic Lateral Sclerosis Genetics genome-wide association samples, consisting of 4243 ALS cases and 5112 controls from 13 European ancestry cohorts from across the United States and Europe. Eight genomic regions provided evidence of association with ALS, including 9p21.2 (rs3849942, odds ratio [OR] = 1.21; p = 4.41 × 10(-7)), 17p11.2 (rs7477, OR = 1.30; p = 2.89 × 10(-7)), and 19p13 (rs12608932, OR = 1.37, p = 1.29 × 10(-7)). Six genomic regions were associated with age at onset of ALS. The strongest evidence for an age of onset locus was observed at 1p34.1, with comparable evidence at rs3011225 (R(2)(partial) = 0.0061; p = 6.59 × 10(-8)) and rs803675 (R(2)(partial) = 0.0060; p = 6.96 × 10(-8)). These associations were consistent across all 13 cohorts. For rs3011225, individuals with at least 1 copy of the minor allele had an earlier average age of onset of over 2 years. Identifying the underlying pathways influencing susceptibility to and age at onset of ALS may provide insight into the pathogenic mechanisms and motivate new pharmacologic targets for this fatal neurodegenerative disease. Copyright © 2013 Elsevier Inc. All rights reserved.

Proximal Participation: A Pathway into Work

ERIC Educational Resources Information Center

Chan, Selena

2013-01-01

In a longitudinal case study of apprentices, the term proximal participation was coined to describe the entry process of young people, with unclear career destinations, into the trade of baking. This article unravels the significance of proximal participation in the decision-making processes of young people who enter a trade through initial…

Refinement of myotome values in the upper limb: Evidence from brachial plexus injuries.

PubMed

Bell, S W; Brown, M J C; Hems, T J

2017-02-01

We reviewed patients with partial supraclavicular brachial plexus injuries in order to refine the myotome values of the upper limb. Forty-two patients with defined partial injuries to the supraclavicular brachial plexus were reviewed from a prospective database. The injuries patterns covered C5, C5-6, C5-7, C5-8, C7-T1 and C8-T1 roots. Upper plexus injuries were classified on the basis of surgical exploration and intraoperative stimulation and lower plexus injuries from MRI. Flexor Carpi Radialis (FCR) was paralyzed in C5-7 injuries, in addition to paralysis of deltoid, supraspinatus, infraspinatus and biceps, when compared to C5-6 injuries. Complete paralysis of Flexor Digitorum Profundus (FDP) and Flexor Digitorum Superficialis (FDS) to all digits was identified in C7-T1 injuries. In C5-8 injuries weakness was noted in FDP of ulnar digits and intrinsics innervated by the ulnar nerve, while in C8-T1 injuries paralysis was noted in the FDP to the radial digits. All patients with C8-T1 injuries had paralysis of FDS and the thenar muscles. In upper plexus injuries paralysis of FCR indicated involvement of C7 root in addition to C5 and C6 roots. The results provide new detail of innervation of muscles acting on the hand. Flexor muscles and intrinsic muscles of the thumb and radial fingers (median nerve) have an important contribution from T1, while for those acting on the ulnar digits (ulnar nerve) the main contribution is from C8 with some input from C7. T1 also gives consistent innervation to extensor pollicis longus. A revised myotome chart for the upper limb is proposed. Copyright © 2015 Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. Published by Elsevier Ltd. All rights reserved.

A Rare Form of Guillan Barre Syndrome: A Child Diagnosed with Anti-GD1a and Anti-GD1b Positive Pharyngeal-Cervical-Brachial Variant

PubMed Central

Uysalol, Metin; Tatlı, Burak; Uzel, Nedret; Çıtak, Agop; Aygün, Erhan; Kayaoğlu, Semra

2013-01-01

Background: Pharyngeal-cervical-brachial (PCB) variant is a rare form of Guillan-Barre Syndrome (GBS). Antibodies against other membrane proteins like GM1b and GD1a have been found only in a small number of patients with Guillan Barre syndrome variant. Case Report: Here, we report a 5.5 year-old boy diagnosed early with positive GD1a and GD1b gangliosides of Guillan-Barre syndrome pharyngeal cervical-Brachial variant, who improved and recovered fully in a short period. This is in contrast to those whose recovery period prolongs in spite of early diagnosis and appropriate treatment and/or those who experience incomplete recovery. Conclusion: In summary, diagnosis of PCB variant of GBS should be considered in infants with sudden onset bulbar symptoms and muscle weakness, and it should be kept in mind that early diagnosis and appropriate treatment can give successful outcomes. PMID:25207134

Prosthetic replacement for proximal humeral fractures.

PubMed

Kontakis, George; Tosounidis, Theodoros; Galanakis, Ioannis; Megas, Panagiotis

2008-12-01

The ideal management of complex proximal humeral fractures continues to be debatable. Evolution of proximal humeral fracture management, during the past decade, led to the implementation of many innovations in surgical treatment. Even though the pendulum of treatment seems to swing towards new trends such as locked plating, hemiarthroplasty remains a valid and reliable option that serves the patient's needs well. Hemiarthroplasty is indicated for complex proximal humeral fractures in elderly patients with poor bone stock and when internal fixation is difficult or unreliable. Hemiarthroplasty provides a better result when it is performed early post-injury. Stem height, retroversion and tuberosity positioning are technical aspects of utmost importance. Additionally reverse total shoulder arthroplasty is an alternative new modality that can be used as a primary solution in selected patients with proximal humeral fracture treatment. Failed hemiarthroplasty and fracture sequelae can be successfully managed with reverse total shoulder arthroplasty. Individual decision-making and tailored treatment that takes into consideration the personality of the fracture and the patient's characteristics should be used.

Proximal tibial osteotomy. A survivorship analysis.

PubMed

Ritter, M A; Fechtman, R A

1988-01-01

Proximal tibial osteotomy is generally accepted as a treatment for the patient with unicompartmental arthritis. However, a few reports of the long-term results of this procedure are available in the literature, and none have used the technique known as survivorship analysis. This technique has an advantage over conventional analysis because it does not exclude patients for inadequate follow-up, loss to follow-up, or patient death. In this study, survivorship analysis was applied to 78 proximal tibial osteotomies, performed exclusively by the senior author for the correction of a preoperative varus deformity, and a survival curve was constructed. It was concluded that the reliable longevity of the proximal tibial osteotomy is approximately 6 years.

Interaction between PON1 and population density in amyotrophic lateral sclerosis.

PubMed

Diekstra, Frank P; Beleza-Meireles, Ana; Leigh, Nigel P; Shaw, Christopher E; Al-Chalabi, Ammar

2009-01-28

Paraoxonase polymorphisms have been associated with amyotrophic lateral sclerosis (ALS). Paraoxonases are detoxifying enzymes involved in the metabolism of organophosphates. We tested the hypothesis that genetic variation within paraoxonase genes would interact with the environmental exposure to paraoxonase substrates. We used population density in the location of residence of ALS patients as a surrogate marker for environmental exposure. Paraoxonase genotypes at previously associated single nucleotide polymorphisms rs662, rs854560, rs6954345, and rs11981433 were studied in 98 patients from the South East England ALS population-based register. A case-only analysis was carried out and median population density was used to categorize patients into rural or urban environments. We found a significant interaction with population density for marker rs854560 (L55M) in ALS.

Debates to personal conclusion in peripheral nerve injury and reconstruction: A 30-year experience at Chang Gung Memorial Hospital

PubMed Central

Chuang, David Chwei-Chin

2016-01-01

Significant progress has been achieved in the science and management of peripheral nerve injuries over the past 40 years. Yet there are many questions and few answers. The author, with 30 years of experience in treating them at the Chang Gung Memorial Hospital, addresses debates on various issues with personal conclusions. These include: (1) Degree of peripheral nerve injury, (2) Timing of nerve repair, (3)Technique of nerve repair, (4) Level of brachial plexus injury,(5) Level of radial nerve injury,(6) Traction avulsion amputation of major limb, (7) Proximal Vs distal nerve transfers in brachial plexus injuries and (8) Post paralysis facial synkinesis. PMID:27833273

The theoretical simulation on electrostatic distribution of 1st proximity region in proximity focusing low-light-level image intensifier

NASA Astrophysics Data System (ADS)

Zhang, Liandong; Bai, Xiaofeng; Song, De; Fu, Shencheng; Li, Ye; Duanmu, Qingduo

2015-03-01

Low-light-level night vision technology is magnifying low light level signal large enough to be seen by naked eye, which uses the photons - photoelectron as information carrier. Until the micro-channel plate was invented, it has been possibility for the realization of high performance and miniaturization of low-light-level night vision device. The device is double-proximity focusing low-light-level image intensifier which places a micro-channel plate close to photocathode and phosphor screen. The advantages of proximity focusing low-light-level night vision are small size, light weight, small power consumption, no distortion, fast response speed, wide dynamic range and so on. It is placed parallel to each other for Micro-channel plate (both sides of it with metal electrode), the photocathode and the phosphor screen are placed parallel to each other. The voltage is applied between photocathode and the input of micro-channel plate when image intensifier works. The emission electron excited by photo on the photocathode move towards to micro-channel plate under the electric field in 1st proximity focusing region, and then it is multiplied through the micro-channel. The movement locus of emission electrons can be calculated and simulated when the distributions of electrostatic field equipotential lines are determined in the 1st proximity focusing region. Furthermore the resolution of image tube can be determined. However the distributions of electrostatic fields and equipotential lines are complex due to a lot of micro-channel existing in the micro channel plate. This paper simulates electrostatic distribution of 1st proximity region in double-proximity focusing low-light-level image intensifier with the finite element simulation analysis software Ansoft maxwell 3D. The electrostatic field distributions of 1st proximity region are compared when the micro-channel plates' pore size, spacing and inclination angle ranged. We believe that the electron beam movement

25 years of neuroimaging in amyotrophic lateral sclerosis.

PubMed

Foerster, Bradley R; Welsh, Robert C; Feldman, Eva L

2013-09-01

Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease for which a precise cause has not yet been identified. Standard CT or MRI evaluation does not demonstrate gross structural nervous system changes in ALS, so conventional neuroimaging techniques have provided little insight into the pathophysiology of this disease. Advanced neuroimaging techniques--such as structural MRI, diffusion tensor imaging and proton magnetic resonance spectroscopy--allow evaluation of alterations of the nervous system in ALS. These alterations include focal loss of grey and white matter and reductions in white matter tract integrity, as well as changes in neural networks and in the chemistry, metabolism and receptor distribution in the brain. Given their potential for investigation of both brain structure and function, advanced neuroimaging methods offer important opportunities to improve diagnosis, guide prognosis, and direct future treatment strategies in ALS. In this article, we review the contributions made by various advanced neuroimaging techniques to our understanding of the impact of ALS on different brain regions, and the potential role of such measures in biomarker development.

Metal-deficient SOD1 in amyotrophic lateral sclerosis.

PubMed

Hilton, James B; White, Anthony R; Crouch, Peter J

2015-05-01

Mutations to the ubiquitous antioxidant enzyme Cu/Zn superoxide dismutase (SOD1) were the first established genetic cause of the fatal, adult-onset neurodegenerative disease amyotrophic lateral sclerosis (ALS). It is widely accepted that these mutations do not cause ALS via a loss of antioxidant function, but elucidating the alternate toxic gain of function has proven to be elusive. Under physiological conditions, SOD1 binds one copper ion and one zinc ion per monomer to form a highly stable and functional homodimer, but there is now ample evidence to indicate aberrant persistence of SOD1 in an intermediate metal-deficient state may contribute to the protein's involvement in ALS. This review briefly discusses some of the data to support a role for metal-deficient SOD1 in the development of ALS and some of the outcomes from drug development studies that have aimed to modify the symptoms of ALS by targeting the metal state of SOD1. The implications for the metal state of SOD1 in cases of sporadic ALS that do not involve mutant SOD1 are also discussed.

A(a)LS: Ammonia-induced amyotrophic lateral sclerosis

PubMed Central

Parekh, Bhavin

2015-01-01

Amyotrophic lateral sclerosis (ALS) is a dreadful, devastating and incurable motor neuron disease. Aetiologically, it is a multigenic, multifactorial and multiorgan disease. Despite intense research, ALS pathology remains unexplained. Following extensive literature review, this paper posits a new integrative explanation. This framework proposes that ammonia neurotoxicity is a main player in ALS pathogenesis. According to this explanation, a combination of impaired ammonia removal— mainly because of impaired hepatic urea cycle dysfunction—and increased ammoniagenesis— mainly because of impaired glycolytic metabolism in fast twitch skeletal muscle—causes chronic hyperammonia in ALS. In the absence of neuroprotective calcium binding proteins (calbindin, calreticulin and parvalbumin), elevated ammonia—a neurotoxin—damages motor neurons. Ammonia-induced motor neuron damage occurs through multiple mechanisms such as macroautophagy-endolysosomal impairment, endoplasmic reticulum (ER) stress, CDK5 activation, oxidative/nitrosative stress, neuronal hyperexcitability and neuroinflammation. Furthermore, the regional pattern of calcium binding proteins’ loss, owing to either ER stress and/or impaired oxidative metabolism, determines clinical variability of ALS. Most importantly, this new framework can be generalised to explain other neurodegenerative disorders such as Huntington’s disease and Parkinsonism. PMID:27785351

25 years of neuroimaging in amyotrophic lateral sclerosis

PubMed Central

Foerster, Bradley R.; Welsh, Robert C.; Feldman, Eva L.

2014-01-01

Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease for which a precise cause has not yet been identified. Standard CT or MRI evaluation does not demonstrate gross structural nervous system changes in ALS, so conventional neuroimaging techniques have provided little insight into the pathophysiology of this disease. Advanced neuroimaging techniques—such as structural MRI, diffusion tensor imaging and proton magnetic resonance spectroscopy—allow evaluation of alterations of the nervous system in ALS. These alterations include focal loss of grey and white matter and reductions in white matter tract integrity, as well as changes in neural networks and in the chemistry, metabolism and receptor distribution in the brain. Given their potential for investigation of both brain structure and function, advanced neuroimaging methods offer important opportunities to improve diagnosis, guide prognosis, and direct future treatment strategies in ALS. In this article, we review the contributions made by various advanced neuroimaging techniques to our understanding of the impact of ALS on different brain regions, and the potential role of such measures in biomarker development. PMID:23917850

Cardiometabolic health and risk of amyotrophic lateral sclerosis.

PubMed

Timmins, Hannah C; Saw, Wilfred; Cheah, Benjamin C; Lin, Cindy S Y; Vucic, Steve; Ahmed, Rebekah M; Kiernan, Matthew C; Park, Susanna B

2017-10-01

Patients diagnosed with amyotrophic lateral sclerosis (ALS) generally have a limited medical history and a normal body mass index, raising the possibility of a premorbid ALS phenotype. The prevalence of cardiometabolic factors was analyzed in 58 ALS patients via comprehensive cardiovascular assessments and compared with Australian population norms. ALS patients had good cardiac fitness and no reported cardiovascular events. Average blood pressure, heart rate, PR interval, and corrected QT interval were in the normal range. There were significantly fewer obese women in the ALS cohort (13.6%, P < 0.05) and more men with a normal body mass index than in the general population (47.2%, P < 0.001). The percentage of individuals who had never smoked was greater for the ALS cohort (55.8%, P ≤ 0.001), and the prevalence of dyslipidemia was lower (38.7%) compared with the general population (74.4%, P < 0.001). ALS patients had good cardiometabolic health, with evidence of a reduced vascular risk profile. Muscle Nerve 56: 721-725, 2017. © 2016 Wiley Periodicals, Inc.

Intraspinal Stem Cell Transplantation for Amyotrophic Lateral Sclerosis

PubMed Central

Chen, Kevin S.; Sakowski, Stacey A.; Feldman, Eva L.

2015-01-01

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder in which the loss of upper and lower motor neurons produces progressive weakness and eventually death. In the decades since the approval of riluzole, the only FDA approved medication to moderately slow progression of ALS, no new therapeutics have arisen to alter the course of the disease. This is partly due to our incomplete understanding of the complex pathogenesis of motor neuron degeneration. Stem cells have emerged as an attractive option in treating ALS since they come armed with equally complex cellular machinery and may modulate the local microenvironment in many ways to rescue diseased motor neurons. While various stem cell types are being evaluated in preclinical and early clinical applications, here we review the preclinical strategies and advances supporting the recent clinical translation of neural progenitor cell therapy for ALS. Specifically, we focus on the use of spinal cord neural progenitor cells and the pipeline starting from preclinical studies to the designs of the Phase I and IIa clinical trials involving direct intraspinal transplantation in humans. PMID:26696091

Ankle brachial index values, leg symptoms, and functional performance among community-dwelling older men and women in the lifestyle interventions and independence for elders study

USDA-ARS?s Scientific Manuscript database

The prevalence and significance of low normal and abnormal ankle brachial index (ABI) values in a community dwelling population of sedentary, older individuals is unknown. We describe the prevalence of categories of definite peripheral artery disease (PAD), borderline ABI, low-normal ABI and no PAD...

Restoration of Elbow Flexion in Patients With Complete Traumatic and Obstetric Brachial Plexus Injury After Functional Free Gracilis Muscle Transfer: Our Experience and Management.

PubMed

Nath, Rahul K; Boutros, Sean G; Somasundaram, Chandra

2017-01-01

Background: Functional free gracilis muscle transfer is an operative procedure for elbow reconstruction in patients with complete brachial plexus nerve and avulsion injuries and in delayed or prolonged nerve denervation, as well as in patients with inadequate upper extremity function after primary nerve reconstruction. Methods: We retrospectively reviewed our patient records and identified 24 patients with complete brachial plexus nerve injury (13 obstetric, 11 males and 2 females; 11 traumatic, 9 males and 2 females) whose affected arm and shoulder were totally paralyzed and their voluntary elbow flexion or the biceps function was poor preoperatively (mean M0-1/5 in MRC grade). These patients had undergone the functional free gracilis muscle transfer procedure at our clinic since 2005. Results: Ninety-two percent of all patients showed recovery and improvement. Successful free gracilis muscle transfer is defined as antigravity biceps muscle strength of M3-4/5 and higher, which was observed in 16 (8 obstetric and 8 traumatic) of our 24 patients (67%) in this study at least 1 year after functional free gracilis muscle transfer. This is statistically significant ( P < .000001) in comparison with their mean preoperative score (M0-1/5). There was no improvement in motor level of the biceps muscle (M0/5) in 2 patients (1 from each group). The donor site of these 24 patients showed no deficit in motor and sensory functions. Conclusions: Taken together, a significant number (92%) of patients in both obstetric and traumatic brachial plexus injury groups had recovery and improvement and most of these patients (64%) achieved antigravity and elbow flexion at least 1 year after free gracilis muscle transfer at our clinic.

Restoration of Elbow Flexion in Patients With Complete Traumatic and Obstetric Brachial Plexus Injury After Functional Free Gracilis Muscle Transfer: Our Experience and Management

PubMed Central

Boutros, Sean G.; Somasundaram, Chandra

2017-01-01

Background: Functional free gracilis muscle transfer is an operative procedure for elbow reconstruction in patients with complete brachial plexus nerve and avulsion injuries and in delayed or prolonged nerve denervation, as well as in patients with inadequate upper extremity function after primary nerve reconstruction. Methods: We retrospectively reviewed our patient records and identified 24 patients with complete brachial plexus nerve injury (13 obstetric, 11 males and 2 females; 11 traumatic, 9 males and 2 females) whose affected arm and shoulder were totally paralyzed and their voluntary elbow flexion or the biceps function was poor preoperatively (mean M0-1/5 in MRC grade). These patients had undergone the functional free gracilis muscle transfer procedure at our clinic since 2005. Results: Ninety-two percent of all patients showed recovery and improvement. Successful free gracilis muscle transfer is defined as antigravity biceps muscle strength of M3-4/5 and higher, which was observed in 16 (8 obstetric and 8 traumatic) of our 24 patients (67%) in this study at least 1 year after functional free gracilis muscle transfer. This is statistically significant (P < .000001) in comparison with their mean preoperative score (M0-1/5). There was no improvement in motor level of the biceps muscle (M0/5) in 2 patients (1 from each group). The donor site of these 24 patients showed no deficit in motor and sensory functions. Conclusions: Taken together, a significant number (92%) of patients in both obstetric and traumatic brachial plexus injury groups had recovery and improvement and most of these patients (64%) achieved antigravity and elbow flexion at least 1 year after free gracilis muscle transfer at our clinic. PMID:29213347

Military service, deployments, and exposures in relation to amyotrophic lateral sclerosis survival

PubMed Central

Beard, John D.; Engel, Lawrence S.; Richardson, David B.; Gammon, Marilie D.; Baird, Coleen; Umbach, David M.; Allen, Kelli D.; Stanwyck, Catherine L.; Keller, Jean; Sandler, Dale P.; Schmidt, Silke; Kamel, Freya

2017-01-01

Background Military veterans may have higher rates of amyotrophic lateral sclerosis (ALS) mortality than non-veterans. Few studies, with sparse exposure information and mixed results, have studied relationships between military-related factors and ALS survival. We evaluated associations between military-related factors and ALS survival among U.S. military veteran cases. Methods We followed 616 medical record-confirmed cases from enrollment (2005–2010) in the Genes and Environmental Exposures in Veterans with Amyotrophic Lateral Sclerosis study until death or July 25, 2013, whichever came first. We ascertained vital status information from several sources within the Department of Veterans Affairs. We obtained information regarding military service, deployments, and 39 related exposures via standardized telephone interviews. We used Cox proportional hazards regression models to estimate hazard ratios (HRs) and 95% confidence intervals. We adjusted for potential confounding and missing covariate data biases via inverse probability weights. We also used inverse probability weights to adjust for potential selection bias among a case group that included a disproportionate number of long-term survivors at enrollment. Results We observed 446 deaths during 24,267 person-months of follow-up (median follow-up: 28 months). Survival was shorter for cases who served before 1950, were deployed to World War II, or mixed and applied burning agents, with HRs between 1.58 and 2.57. Longer survival was associated with exposure to: paint, solvents, or petrochemical substances; local food not provided by the Armed Forces; or burning agents or Agent Orange in the field with HRs between 0.56 and 0.73. Conclusions Although most military-related factors were not associated with survival, associations we observed with shorter survival are potentially important because of the large number of military veterans. PMID:29016608

Genetic epidemiology of amyotrophic lateral sclerosis: a systematic review and meta-analysis.

PubMed

Zou, Zhang-Yu; Zhou, Zhi-Rui; Che, Chun-Hui; Liu, Chang-Yun; He, Rao-Li; Huang, Hua-Pin

2017-07-01

Genetic studies have shown that C9orf72 , SOD1 , TARDBP and FUS are the most common mutated genes in amyotrophic lateral sclerosis (ALS). Here, we performed a meta-analysis to determine the mutation frequencies of these major ALS-related genes in patients with ALS. We performed an extensive literature research to identify all original articles reporting frequencies of C9orf72 , SOD1 , TARDBP and FUS mutations in ALS. The mutation frequency and effect size of each study were combined. Possible sources of heterogeneity across studies were determined by meta-regression, sensitivity analysis and subgroup analysis. 111 studies were included in the meta-analysis. The overall pooled mutation frequencies of these major ALS-related genes were 47.7% in familial amyotrophic lateral sclerosis (FALS) and 5.2% in sporadic ALS (SALS). A significant difference was identified regarding the frequencies of mutations in major ALS genes between European and Asian patients. In European populations, the most common mutations were the C9orf72 repeat expansions (FALS 33.7%, SALS 5.1%), followed by SOD1 (FALS 14.8%, SALS 1.2%), TARDBP (FALS 4.2%, SALS 0.8%) and FUS mutations (FALS 2.8%, SALS 0.3%), while in Asian populations the most common mutations were SOD1 mutations (FALS 30.0%, SALS 1.5%), followed by FUS (FALS 6.4%, SALS 0.9%), C9orf72 (FALS 2.3%, SALS 0.3%) and TARDBP (FALS 1.5%, SALS 0.2%) mutations. These findings demonstrated that the genetic architecture of ALS in Asian populations is distinct from that in European populations, which need to be given appropriate consideration when performing genetic testing of patients with ALS. 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/.

Promoting proximal formative assessment with relational discourse

NASA Astrophysics Data System (ADS)

Scherr, Rachel E.; Close, Hunter G.; McKagan, Sarah B.

2012-02-01

The practice of proximal formative assessment - the continual, responsive attention to students' developing understanding as it is expressed in real time - depends on students' sharing their ideas with instructors and on teachers' attending to them. Rogerian psychology presents an account of the conditions under which proximal formative assessment may be promoted or inhibited: (1) Normal classroom conditions, characterized by evaluation and attention to learning targets, may present threats to students' sense of their own competence and value, causing them to conceal their ideas and reducing the potential for proximal formative assessment. (2) In contrast, discourse patterns characterized by positive anticipation and attention to learner ideas increase the potential for proximal formative assessment and promote self-directed learning. We present an analysis methodology based on these principles and demonstrate its utility for understanding episodes of university physics instruction.

Technology-Aided Programs for Assisting Communication and Leisure Engagement of Persons with Amyotrophic Lateral Sclerosis: Two Single-Case Studies

ERIC Educational Resources Information Center

Lancioni, Giulio E.; Singh, Nirbhay N.; O'Reilly, Mark F.; Sigafoos, Jeff; Ferlisi, Gabriele; Ferrarese, Giacomina; Zullo, Valeria; Addante, Luigi M.; Spica, Antonella; Oliva, Doretta

2012-01-01

Technology-aided programs for assisting communication and leisure engagement were assessed in single-case studies involving two men with amyotrophic lateral sclerosis (ALS). Study I involved a 51-year-old man with a virtually total loss of his motor repertoire and assessed a technology-aided program aimed at enabling him to (a) write and send out…

Comprehensive care of amyotrophic lateral sclerosis patients: a care model.

PubMed

Güell, Maria Rosa; Antón, Antonio; Rojas-García, Ricardo; Puy, Carmen; Pradas, Jesus

2013-12-01

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease that presents with muscle weakness, causing progressive difficulty in movement, communication, eating and ultimately, breathing, creating a growing dependence on family members and other carers. The ideal way to address the problems associated with the disease, and the decisions that must be taken, is through multidisciplinary teams. The key objectives of these teams are to optimise medical care, facilitate communication between team members, and thus to improve the quality of care. In our centre, we have extensive experience in the care of patients with ALS through an interdisciplinary team whose aim is to ensure proper patient care from the hospital to the home setting. In this article, we describe the components of the team, their roles and our way of working. Copyright © 2012 SEPAR. Published by Elsevier Espana. All rights reserved.

Common Molecular Pathways in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia.

PubMed

Weishaupt, Jochen H; Hyman, Tony; Dikic, Ivan

2016-09-01

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are age-related neurodegenerative diseases in which predominantly motor neurons and cerebral cortex neurons, respectively, are affected. Several novel ALS and FTD disease genes have been recently discovered, pointing toward a few overarching pathways in ALS/FTD pathogenesis. Nevertheless, a precise picture of how various cellular processes cause neuronal death, or how different routes leading to ALS and FTD are functionally connected is just emerging. Moreover, how the most recent milestone findings in the ALS/FTD field might lead to improved diagnosis and treatment is actively being explored. We highlight some of the most exciting recent topics in the field, which could potentially facilitate the identification of further links between the pathogenic ALS/FTD pathways related to autophagy, vesicle trafficking, and RNA metabolism. Copyright © 2016 Elsevier Ltd. All rights reserved.

FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations.

PubMed

Neumann, Manuela; Bentmann, Eva; Dormann, Dorothee; Jawaid, Ali; DeJesus-Hernandez, Mariely; Ansorge, Olaf; Roeber, Sigrun; Kretzschmar, Hans A; Munoz, David G; Kusaka, Hirofumi; Yokota, Osamu; Ang, Lee-Cyn; Bilbao, Juan; Rademakers, Rosa; Haass, Christian; Mackenzie, Ian R A

2011-09-01

Accumulation of the DNA/RNA binding protein fused in sarcoma as cytoplasmic inclusions in neurons and glial cells is the pathological hallmark of all patients with amyotrophic lateral sclerosis with mutations in FUS as well as in several subtypes of frontotemporal lobar degeneration, which are not associated with FUS mutations. The mechanisms leading to inclusion formation and fused in sarcoma-associated neurodegeneration are only poorly understood. Because fused in sarcoma belongs to a family of proteins known as FET, which also includes Ewing's sarcoma and TATA-binding protein-associated factor 15, we investigated the potential involvement of these other FET protein family members in the pathogenesis of fused in sarcoma proteinopathies. Immunohistochemical analysis of FET proteins revealed a striking difference among the various conditions, with pathology in amyotrophic lateral sclerosis with FUS mutations being labelled exclusively for fused in sarcoma, whereas fused in sarcoma-positive inclusions in subtypes of frontotemporal lobar degeneration also consistently immunostained for TATA-binding protein-associated factor 15 and variably for Ewing's sarcoma. Immunoblot analysis of proteins extracted from post-mortem tissue of frontotemporal lobar degeneration with fused in sarcoma pathology demonstrated a relative shift of all FET proteins towards insoluble protein fractions, while genetic analysis of the TATA-binding protein-associated factor 15 and Ewing's sarcoma gene did not identify any pathogenic variants. Cell culture experiments replicated the findings of amyotrophic lateral sclerosis with FUS mutations by confirming the absence of TATA-binding protein-associated factor 15 and Ewing's sarcoma alterations upon expression of mutant fused in sarcoma. In contrast, all endogenous FET proteins were recruited into cytoplasmic stress granules upon general inhibition of Transportin-mediated nuclear import, mimicking the findings in frontotemporal lobar degeneration

Impact of Expiratory Strength Training in Amyotrophic Lateral Sclerosis

PubMed Central

Plowman, Emily K.; Watts, Stephanie A.; Tabor, Lauren; Robison, Raele; Gaziano, Joy; Domer, Amanda S.; Richter, Joel; Vu, Tuan; Gooch, Clifton

2016-01-01

Introduction We evaluated the feasibility and impact of Expiratory Muscle Strength Training (EMST) on respiratory and bulbar function in persons with amyotrophic lateral sclerosis (ALS). Methods 25 ALS patients participated in this delayed intervention open-label clinical trial. Following a lead-in period, patients completed a 5-week EMST protocol. Outcome measures included: maximum expiratory pressure (MEP), physiologic measures of swallow and cough, and Penetration-Aspiration Scale (PAS) scores. Results Of those participants who entered the active phase of the study (n=15), EMST was well tolerated and led to significant increases in MEPs and maximum hyoid displacement during swallowing post-EMST (P<0.05). No significant differences were observed for PAS scores or cough spirometry measures. Discussion EMST was feasible and well tolerated in this small cohort of ALS patients and led to improvements in expiratory force-generating pressures and swallow kinematics. Further investigation is warranted to confirm these preliminary findings. PMID:26599236

Disease-modifying and symptomatic treatment of amyotrophic lateral sclerosis

PubMed Central

Dorst, Johannes; Ludolph, Albert C.; Huebers, Annemarie

2017-01-01

In this review, we summarize the most important recent developments in the treatment of amyotrophic lateral sclerosis (ALS). In terms of disease-modifying treatment options, several drugs such as dexpramipexole, pioglitazone, lithium, and many others have been tested in large multicenter trials, albeit with disappointing results. Therefore, riluzole remains the only directly disease-modifying drug. In addition, we discuss antisense oligonucleotides (ASOs) as a new and potentially causal treatment option. Progress in symptomatic treatments has been more important. Nutrition and ventilation are now an important focus of ALS therapy. Several studies have firmly established that noninvasive ventilation improves patients’ quality of life and prolongs survival. On the other hand, there is still no consensus regarding best nutritional management, but big multicenter trials addressing this issue are currently ongoing. Evidence regarding secondary symptoms like spasticity, muscle cramps or sialorrhea remains generally scarce, but some new insights will also be discussed. Growing evidence suggests that multidisciplinary care in specialized clinics improves survival. PMID:29399045

Spatiotemporal coupling of the tongue in amyotrophic lateral sclerosis.

PubMed

Kuruvilla, Mili S; Green, Jordan R; Yunusova, Yana; Hanford, Kathy

2012-12-01

The primary aim of the investigation was to identify deficits in spatiotemporal coupling between tongue regions in amyotrophic lateral sclerosis (ALS). The relations between disease-related changes in tongue movement patterns and speech intelligibility were also determined. Methods The authors recorded word productions from 11 individuals with ALS with mild, moderate, and severe dysarthria using an x-ray microbeam during word productions. A coupling index based on sliding window covariance was used to determine disease-related changes in the coupling between the tongue regions across each word. The results indicated decreased spatiotemporal coupling of mid-posterior tongue regions and reduced tongue speed in the ALS-moderate subgroup. Changes in the range of tongue coupling relations and speed of movement were highly correlated with speech intelligibility. These results provide new insights into the loss of lingual motor control due to ALS and suggest that measures of tongue performance may provide useful indicators of bulbar disease severity and progression.

Myasthenia gravis and amyotrophic lateral sclerosis: A pathogenic overlap.

PubMed

Gotaas, Håvard Torvik; Skeie, Geir Olve; Gilhus, Nils Erik

2016-06-01

The aim was to examine potential joint disease mechanisms for myasthenia gravis (MG) and amyotrophic lateral sclerosis (ALS) through the examination of long-term patient cohorts for comorbidity. Recent studies support early involvement of the neuromuscular junction in ALS patients with subsequent degeneration of motor neurons. Medical records at Haukeland University Hospital from 1987 to 2012 were examined for International Classification of Diseases diagnostic codes for MG and ALS. Sera were re-tested for antibodies to acetylcholine receptor, titin, MuSK and GM1. We report one patient with both MG and ALS, and another 3 patients with suggestive evidence of both conditions. This is far more than expected from prevalence and incidence figures in this area if the disorders were unrelated. Our data suggest that immunological mechanisms in the neuromuscular junction are relevant in ALS pathogenesis. Attention should be given to possible therapeutic targets in the neuromuscular junction and muscle in ALS patients. Copyright © 2016 Elsevier B.V. All rights reserved.

Metabolic Dysregulation in Amyotrophic Lateral Sclerosis: Challenges and Opportunities

PubMed Central

Joardar, Archi; Manzo, Ernesto

2017-01-01

Purpose of Review Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease for which there is no cure and treatments are at best palliative. Several genes have been linked to ALS, which highlight defects in multiple cellular processes including RNA processing, proteostasis and metabolism. Clinical observations have identified glucose intolerance and dyslipidemia as key features of ALS however the causes of these metabolic alterations remain elusive. Recent Findings Recent studies reveal that motor neurons and muscle cells may undergo cell type specific metabolic changes that lead to utilization of alternate fuels. For example, ALS patients’ muscles exhibit reduced glycolysis and increased reliance on fatty acids. In contrast, ALS motor neurons contain damaged mitochondria and exhibit impaired lipid beta oxidation, potentially leading to increased glycolysis as a compensatory mechanism. Summary These findings highlight the complexities of metabolic alterations in ALS and provide new opportunities for designing therapeutic strategies based on restoring cellular energetics. PMID:29057168

Simple animal models for amyotrophic lateral sclerosis drug discovery.

PubMed

Patten, Shunmoogum A; Parker, J Alex; Wen, Xiao-Yan; Drapeau, Pierre

2016-08-01

Simple animal models have enabled great progress in uncovering the disease mechanisms of amyotrophic lateral sclerosis (ALS) and are helping in the selection of therapeutic compounds through chemical genetic approaches. Within this article, the authors provide a concise overview of simple model organisms, C. elegans, Drosophila and zebrafish, which have been employed to study ALS and discuss their value to ALS drug discovery. In particular, the authors focus on innovative chemical screens that have established simple organisms as important models for ALS drug discovery. There are several advantages of using simple animal model organisms to accelerate drug discovery for ALS. It is the authors' particular belief that the amenability of simple animal models to various genetic manipulations, the availability of a wide range of transgenic strains for labelling motoneurons and other cell types, combined with live imaging and chemical screens should allow for new detailed studies elucidating early pathological processes in ALS and subsequent drug and target discovery.

NEK1 variants confer susceptibility to amyotrophic lateral sclerosis

PubMed Central

Kenna, Kevin P; van Doormaal, Perry T C; Dekker, Annelot M; Ticozzi, Nicola; Kenna, Brendan J; Diekstra, Frank P; van Rheenen, Wouter; van Eijk, Kristel R; Jones, Ashley R; Keagle, Pamela; Shatunov, Aleksey; Sproviero, William; Smith, Bradley N; van Es, Michael A; Topp, Simon D; Kenna, Aoife; Miller, Jack W; Fallini, Claudia; Tiloca, Cinzia; McLaughlin, Russell L; Vance, Caroline; Troakes, Claire; Colombrita, Claudia; Mora, Gabriele; Calvo, Andrea; Verde, Federico; Al-Sarraj, Safa; King, Andrew; Calini, Daniela; de Belleroche, Jacqueline; Baas, Frank; van der Kooi, Anneke J; de Visser, Marianne; Asbroek, Anneloor L M A ten; Sapp, Peter C; McKenna-Yasek, Diane; Polak, Meraida; Asress, Seneshaw; Muñoz-Blanco, José Luis; Strom, Tim M; Meitinger, Thomas; Morrison, Karen E; Lauria, Giuseppe; Williams, Kelly L; Leigh, P Nigel; Nicholson, Garth A; Blair, Ian P; Leblond, Claire S; Dion, Patrick A; Rouleau, Guy A; Pall, Hardev; Shaw, Pamela J; Turner, Martin R; Talbot, Kevin; Taroni, Franco; Boylan, Kevin B; Van Blitterswijk, Marka; Rademakers, Rosa; Esteban-Pérez, Jesús; García-Redondo, Alberto; Van Damme, Phillip; Robberecht, Wim; Chio, Adriano; Gellera, Cinzia; Drepper, Carsten; Sendtner, Michael; Ratti, Antonia; Glass, Jonathan D; Mora, Jesús S; Basak, Nazli A; Hardiman, Orla; Ludolph, Albert C; Andersen, Peter M; Weishaupt, Jochen H; Brown, Robert H; Al-Chalabi, Ammar; Silani, Vincenzo; Shaw, Christopher E; van den Berg, Leonard H; Veldink, Jan H; Landers, John E

2017-01-01

To identify genetic factors contributing to amyotrophic lateral sclerosis (ALS), we conducted whole-exome analyses of 1,022 index familial ALS (FALS) cases and 7,315 controls. In a new screening strategy, we performed gene-burden analyses trained with established ALS genes and identified a significant association between loss-of-function (LOF) NEK1 variants and FALS risk. Independently, autozygosity mapping for an isolated community in the Netherlands identified a NEK1 p.Arg261His variant as a candidate risk factor. Replication analyses of sporadic ALS (SALS) cases and independent control cohorts confirmed significant disease association for both p.Arg261His (10,589 samples analyzed) and NEK1 LOF variants (3,362 samples analyzed). In total, we observed NEK1 risk variants in nearly 3% of ALS cases. NEK1 has been linked to several cellular functions, including cilia formation, DNA-damage response, microtubule stability, neuronal morphology and axonal polarity. Our results provide new and important insights into ALS etiopathogenesis and genetic etiology. PMID:27455347

Psychological responses to the proximity of climate change

NASA Astrophysics Data System (ADS)

Brügger, Adrian; Dessai, Suraje; Devine-Wright, Patrick; Morton, Thomas A.; Pidgeon, Nicholas F.

2015-12-01

A frequent suggestion to increase individuals' willingness to take action on climate change and to support relevant policies is to highlight its proximal consequences, that is, those that are close in space and time. But previous studies that have tested this proximizing approach have not revealed the expected positive effects on individual action and support for addressing climate change. We present three lines of psychological reasoning that provide compelling arguments as to why highlighting proximal impacts of climate change might not be as effective a way to increase individual mitigation and adaptation efforts as is often assumed. Our contextualization of the proximizing approach within established psychological research suggests that, depending on the particular theoretical perspective one takes on this issue, and on specific individual characteristics suggested by these perspectives, proximizing can bring about the intended positive effects, can have no (visible) effect or can even backfire. Thus, the effects of proximizing are much more complex than is commonly assumed. Revealing this complexity contributes to a refined theoretical understanding of the role that psychological distance plays in the context of climate change and opens up further avenues for future research and for interventions.

A Mutation in the Vesicle-Trafficking Protein VAPB Causes Late-Onset Spinal Muscular Atrophy and Amyotrophic Lateral Sclerosis

PubMed Central

Nishimura, Agnes L.; Mitne-Neto, Miguel; Silva, Helga C. A.; Richieri-Costa, Antônio; Middleton, Susan; Cascio, Duilio; Kok, Fernando; Oliveira, João R. M.; Gillingwater, Tom; Webb, Jeanette; Skehel, Paul; Zatz, Mayana

2004-01-01

Motor neuron diseases (MNDs) are a group of neurodegenerative disorders with involvement of upper and/or lower motor neurons, such as amyotrophic lateral sclerosis (ALS), spinal muscular atrophy (SMA), progressive bulbar palsy, and primary lateral sclerosis. Recently, we have mapped a new locus for an atypical form of ALS/MND (atypical amyotrophic lateral sclerosis [ALS8]) at 20q13.3 in a large white Brazilian family. Here, we report the finding of a novel missense mutation in the vesicle-associated membrane protein/synaptobrevin-associated membrane protein B (VAPB) gene in patients from this family. Subsequently, the same mutation was identified in patients from six additional kindreds but with different clinical courses, such as ALS8, late-onset SMA, and typical severe ALS with rapid progression. Although it was not possible to link all these families, haplotype analysis suggests a founder effect. Members of the vesicle-associated proteins are intracellular membrane proteins that can associate with microtubules and that have been shown to have a function in membrane transport. These data suggest that clinically variable MNDs may be caused by a dysfunction in intracellular membrane trafficking. PMID:15372378

Elastin cross-linking in the skin from patients with amyotrophic lateral sclerosis

NASA Technical Reports Server (NTRS)

Ono, S.; Yamauchi, M.

1994-01-01

Two cross-links unique to elastin, desmosine and isodesmosine were measured and compared in skin tissue (left upper arm) from 10 patients with amyotrophic lateral sclerosis (ALS) and from seven age-matched controls. The contents of desmosine and isodesmosine were significantly decreased (p < 0.01 and p < 0.01, respectively) in patients with ALS compared with those of controls, and were negatively and significantly associated with duration of illness in ALS patients (r = -0.77, p < 0.01 and r = -0.65, p < 0.05, respectively). The decline in skin desmosine and isodesmosine is more rapid in ALS than in normal ageing. Thus cross-linking of skin elastin is affected in ALS.

Glutamate and aspartate are decreased in the skin in amyotrophic lateral sclerosis

NASA Technical Reports Server (NTRS)

Ono, S.; Yamauchi, M.

1992-01-01

We measured the levels of amino acids in biopsied skin from eight patients with amyotrophic lateral sclerosis (ALS) and seven controls. The most conspicuous changes in ALS patients were as follows. First, the contents of the acidic amino acids glutamate and aspartate were significantly decreased in ALS, and were negatively and significantly associated with the duration of illness. Second, the levels of the collagen-associated amino acids hydroxyproline, proline, glycine, alanine, and hydroxylysine were significantly decreased in ALS, and correlated inversely with the duration of illness. These results suggest that there are abnormalities of acidic amino acids and collagen-associated amino acids in the skin of patients with ALS. These changes may underlie the pathogenesis of ALS.

Glutamate release and uptake processes are altered in a new mouse model of amyotrophic lateral sclerosis.

PubMed

Grigoriev, V V; Efimova, A D; Ustyugov, A A; Shevchenko, V P; Bachurin, S O; Myasoedov, N F

2016-05-01

In this paper, we showed that in the cortex of mice expressing an abberant form of FUS protein that model amyotrophic lateral sclerosis (ALS), the processes of KCl-induced and basal [(3)H]glutamate release and uptake are altered at the presymptomatic stage as compared to the non-transgenic littermates. The change in these three parameters in transgenic animals causes excitotoxicity, which, in turn, may lead to massive loss of motor neurons and the onset of ALS symptoms.

Neurogenic regulation of proximal bicarbonate and chloride reabsorption.

PubMed

Cogan, M G

1986-01-01

Although a change in renal nerve activity is known to alter proximal reabsorption, it is unclear whether reabsorption of NaHCO3 or NaCl or both are affected. Sprague-Dawley rats (n = 10) were studied using free-flow micropuncture techniques during euvolemia and following acute ipsilateral denervation. Glomerular filtration rate and single nephron glomerular filtration rate were stable. Absolute proximal bicarbonate reabsorption fell following denervation (933 +/- 40 to 817 +/- 30 pmol/min) with a parallel reduction in chloride reabsorption (1,643 +/- 116 to 1,341 +/- 129 peq/min). Urinary sodium, potassium, bicarbonate, and chloride excretion all increased significantly. To further assess the physiological significance of neurogenic modulation of proximal transport, other rats (n = 6) were subjected to acute unilateral nephrectomy (AUN). There is evidence that AUN induces a contralateral natriuresis (renorenal reflex) at least partially by causing inhibition of efferent renal nerve traffic. AUN caused significant changes in proximal NaHCO3 and NaCl reabsorption as well as in whole kidney electrolyte excretion in the same pattern as had denervation. Prior denervation of the remaining kidney prevented the proximal and whole kidney response to AUN (n = 6). In conclusion, depression of renal nerve activity inhibits both NaHCO3 and NaCl reabsorption in the rat superficial proximal convoluted tubule. The data are consistent with the hypothesis that changes in renal nerve activity modify whole kidney electrolyte excretion under physiological conditions at least partially by regulating proximal transport.