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Sample records for proximal brachial amyotrophic

  1. True Aneurysm of the Proximal Brachial Artery

    PubMed Central

    Ramakrishna, Pinjala; Mahapatra, Sandeep; Rajesh, Ratna

    2013-01-01

    A 35-year-old farmer presented with complaints of pain in the right upper limb for 1 month and bluish discoloration of the right-hand finger tips with tingling and numbness. He sustained injury to the right upper limb while lifting a heavy object 1 mo previously. There was an ovoid swelling of 4 × 2 cm on the medial aspect of the right arm, 12 cm above the medial epicondyle at the level of the armpit with visible pulsations. There was distal neurovascular deficit. Duplex scan of the right upper limb arterial system revealed a pseudoaneurysm of the proximal right brachial artery, with dampened monophasic flow in the ulnar artery and no flow in the radial artery. Spiral computed tomography angiogram showed the presence of an echogenic periarterial lesion in the proximal brachial artery suggestive of pseudoaneurysm or an extrinsic compression by hematoma. Distal brachial artery was found to have filled with thrombus, with non-opacification of the radial and the distal ulnar artery. The patient was posted for excision of the aneurysmal arterial segment. A 5-cm-long reversed segment of vein graft was interposed in between the cut ends of the brachial artery. Histopathology: Specimen shows a part of the vessel wall composed of intimal, medial, and adventitial layers with intraluminal thrombus showing evidence of recanalization suggestive of true aneurysm of the brachial artery. PMID:26798692

  2. Electrodiagnosis of brachial plexopathies and proximal upper extremity neuropathies.

    PubMed

    Simmons, Zachary

    2013-02-01

    This article describes the normal anatomy of the brachial plexus and its major terminal branches, as well as the major causes and clinical presentations of lesions of these structures. An approach to electrodiagnosis of brachial plexopathies and proximal upper extremity neuropathies is provided, with an emphasis on those nerve conduction studies and portions of the needle examination, which permit localization of lesions to specific trunks, cords, and terminal branches. The importance of specific sensory nerve conduction studies for differentiating plexopathies from radiculopathies and mononeuropathies is emphasized. PMID:23177028

  3. MR Imaging of Brachial Plexus and Limb-Girdle Muscles in Patients with Amyotrophic Lateral Sclerosis.

    PubMed

    Gerevini, Simonetta; Agosta, Federica; Riva, Nilo; Spinelli, Edoardo G; Pagani, Elisabetta; Caliendo, Giandomenico; Chaabane, Linda; Copetti, Massimiliano; Quattrini, Angelo; Comi, Giancarlo; Falini, Andrea; Filippi, Massimo

    2016-05-01

    Purpose To assess brachial plexus magnetic resonance (MR) imaging features and limb-girdle muscle abnormalities as signs of muscle denervation in patients with amyotrophic lateral sclerosis (ALS). Materials and Methods This study was approved by the local ethical committees on human studies, and written informed consent was obtained from all subjects before enrollment. By using an optimized protocol of brachial plexus MR imaging, brachial plexus and limb-girdle muscle abnormalities were evaluated in 23 patients with ALS and clinical and neurophysiologically active involvement of the upper limbs and were compared with MR images in 12 age-matched healthy individuals. Nerve root and limb-girdle muscle abnormalities were visually evaluated by two experienced observers. A region of interest-based analysis was performed to measure nerve root volume and T2 signal intensity. Measures obtained at visual inspection were analyzed by using the Wald χ(2) test. Mean T2 signal intensity and volume values of the regions of interest were compared between groups by using a hierarchical linear model, accounting for the repeated measurement design. Results The level of interrater agreement was very strong (κ = 0.77-1). T2 hyperintensity and volume alterations of C5, C6, and C7 nerve roots were observed in patients with ALS (P < .001 to .03). Increased T2 signal intensity of nerve roots was associated with faster disease progression (upper-limb Medical Research Council scale progression rate, r = 0.40; 95% confidence interval: 0.001, 0.73). Limb-girdle muscle alterations (ie, T2 signal intensity alteration, edema, atrophy) and fat infiltration also were found, in particular, in the supraspinatus muscle, showing more frequent T2 signal intensity alterations and edema (P = .01) relative to the subscapularis and infraspinatus muscles. Conclusion Increased T2 signal intensity and volume of brachial nerve roots do not exclude a diagnosis of ALS and suggest involvement of the peripheral

  4. Brachial plexopathy

    MedlinePlus

    Neuropathy - brachial plexus; Brachial plexus dysfunction; Parsonage Turner syndrome; Pancoast syndrome ... dysfunction (brachial plexopathy) is a form of peripheral neuropathy . It occurs when there is damage to the ...

  5. Consideration of Dose Limits for Organs at Risk of Thoracic Radiotherapy: Atlas for Lung, Proximal Bronchial Tree, Esophagus, Spinal Cord, Ribs, and Brachial Plexus

    SciTech Connect

    Kong, Feng-Ming; Ritter, Timothy; Quint, Douglas J.; Senan, Suresh; Gaspar, Laurie E.; Komaki, Ritsuko U.; Hurkmans, Coen W.; Timmerman, Robert; Bezjak, Andrea; Bradley, Jeffrey D.; Movsas, Benjamin; Marsh, Lon; Okunieff, Paul; Choy, Hak; Curran, Walter J.

    2011-12-01

    Purpose: To review the dose limits and standardize the three-dimenional (3D) radiographic definition for the organs at risk (OARs) for thoracic radiotherapy (RT), including the lung, proximal bronchial tree, esophagus, spinal cord, ribs, and brachial plexus. Methods and Materials: The present study was performed by representatives from the Radiation Therapy Oncology Group, European Organization for Research and Treatment of Cancer, and Soutwestern Oncology Group lung cancer committees. The dosimetric constraints of major multicenter trials of 3D-conformal RT and stereotactic body RT were reviewed and the challenges of 3D delineation of these OARs described. Using knowledge of the human anatomy and 3D radiographic correlation, draft atlases were generated by a radiation oncologist, medical physicist, dosimetrist, and radiologist from the United States and reviewed by a radiation oncologist and medical physicist from Europe. The atlases were then critically reviewed, discussed, and edited by another 10 radiation oncologists. Results: Three-dimensional descriptions of the lung, proximal bronchial tree, esophagus, spinal cord, ribs, and brachial plexus are presented. Two computed tomography atlases were developed: one for the middle and lower thoracic OARs (except for the heart) and one focusing on the brachial plexus for a patient positioned supine with their arms up for thoracic RT. The dosimetric limits of the key OARs are discussed. Conclusions: We believe these atlases will allow us to define OARs with less variation and generate dosimetric data in a more consistent manner. This could help us study the effect of radiation on these OARs and guide high-quality clinical trials and individualized practice in 3D-conformal RT and stereotactic body RT.

  6. CONSIDERATION OF DOSE LIMITS FOR ORGANS AT RISK OF THORACIC RADIOTHERAPY: ATLAS FOR LUNG, PROXIMAL BRONCHIAL TREE, ESOPHAGUS, SPINAL CORD, RIBS, AND BRACHIAL PLEXUS

    PubMed Central

    Kong, Feng-Ming (Spring); Ritter, Timothy; Quint, Douglas J.; Senan, Suresh; Gaspar, Laurie E.; Komaki, Ritsuko U.; Hurkmans, Coen W.; Timmerman, Robert; Bezjak, Andrea; Bradley, Jeffrey D.; Movsas, Benjamin; Marsh, Lon; Okunieff, Paul; Choy, Hak; Curran, Walter J.

    2012-01-01

    Purpose To review the dose limits and standardize the three-dimenional (3D) radiographic definition for the organs at risk (OARs) for thoracic radiotherapy (RT), including the lung, proximal bronchial tree, esophagus, spinal cord, ribs, and brachial plexus. Methods and Materials The present study was performed by representatives from the Radiation Therapy Oncology Group, European Organization for Research and Treatment of Cancer, and Soutwestern Oncology Group lung cancer committees. The dosimetric constraints of major multicenter trials of 3D-conformal RT and stereotactic body RT were reviewed and the challenges of 3D delineation of these OARs described. Using knowledge of the human anatomy and 3D radiographic correlation, draft atlases were generated by a radiation oncologist, medical physicist, dosimetrist, and radiologist from the United States and reviewed by a radiation oncologist and medical physicist from Europe. The atlases were then critically reviewed, discussed, and edited by another 10 radiation oncologists. Results Three-dimensional descriptions of the lung, proximal bronchial tree, esophagus, spinal cord, ribs, and brachial plexus are presented. Two computed tomography atlases were developed: one for the middle and lower thoracic OARs (except for the heart) and one focusing on the brachial plexus for a patient positioned supine with their arms up for thoracic RT. The dosimetric limits of the key OARs are discussed. Conclusions We believe these atlases will allow us to define OARs with less variation and generate dosimetric data in a more consistent manner. This could help us study the effect of radiation on these OARs and guide high-quality clinical trials and individualized practice in 3D-conformal RT and stereotactic body RT. PMID:20934273

  7. Brachial plexopathy

    MedlinePlus

    Neuropathy - brachial plexus; Brachial plexus dysfunction; Parsonage Turner syndrome; Pancoast syndrome ... or post-viral brachial plexus disease called Parsonage Turner syndrome. Tests that may be done to diagnose ...

  8. Brachial plexus

    MedlinePlus

    The brachial plexus is a group of nerves that run from the lower neck through the upper shoulder area. These ... Damage to the brachial plexus nerves can cause muscle and sensation problems that are often associated with pain in the same area. Symptoms may ...

  9. Brachial plexopathy.

    PubMed

    Khadilkar, Satish V; Khade, Snehaldatta S

    2013-01-01

    Brachial plexus injury can occur as a result of trauma, inflammation or malignancies, and associated complications. The current topic is concerned with various forms of brachial plexopathy, its clinical features, pathophysiology, imaging findings, and management. Idiopathic brachial neuritis (IBN), often preceded with antecedent events such as infection, commonly present with abruptonset painful asymmetric upper limb weakness with associated wasting around the shoulder girdle and arm muscles. Idiopathic hypertrophic brachial neuritis, a rare condition, is usually painless to begin with, unlike IBN. Hereditary neuralgic amyotrophy is an autosomal-dominant disorder characterized by repeated episodes of paralysis and sensory disturbances in an affected limb, which is preceded by severe pain. While the frequency of the episodes tends to decrease with age, affected individuals suffer from residual deficits. Neurogenic thoracic outlet syndrome affects the lower trunk of the brachial plexus. It is diagnosed on the basis of electrophysiology and is amenable to surgical intervention. Cancer-related brachial plexopathy may occur secondary to metastatic infiltration or radiation therapy. Traumatic brachial plexus injury is commonly encountered in neurology, orthopedic, and plastic surgery set-ups. Trauma may be a direct blow or traction or stretch injury. The prognosis depends on the extent and site of injury as well as the surgical expertise. PMID:23661957

  10. Brachial plexopathy

    PubMed Central

    Khadilkar, Satish V.; Khade, Snehaldatta S.

    2013-01-01

    Brachial plexus injury can occur as a result of trauma, inflammation or malignancies, and associated complications. The current topic is concerned with various forms of brachial plexopathy, its clinical features, pathophysiology, imaging findings, and management. Idiopathic brachial neuritis (IBN), often preceded with antecedent events such as infection, commonly present with abruptonset painful asymmetric upper limb weakness with associated wasting around the shoulder girdle and arm muscles. Idiopathic hypertrophic brachial neuritis, a rare condition, is usually painless to begin with, unlike IBN. Hereditary neuralgic amyotrophy is an autosomal-dominant disorder characterized by repeated episodes of paralysis and sensory disturbances in an affected limb, which is preceded by severe pain. While the frequency of the episodes tends to decrease with age, affected individuals suffer from residual deficits. Neurogenic thoracic outlet syndrome affects the lower trunk of the brachial plexus. It is diagnosed on the basis of electrophysiology and is amenable to surgical intervention. Cancer-related brachial plexopathy may occur secondary to metastatic infiltration or radiation therapy. Traumatic brachial plexus injury is commonly encountered in neurology, orthopedic, and plastic surgery set-ups. Trauma may be a direct blow or traction or stretch injury. The prognosis depends on the extent and site of injury as well as the surgical expertise. PMID:23661957

  11. Brachial Plexus Injuries

    MedlinePlus

    ... Diversity Find People About NINDS NINDS Brachial Plexus Injuries Information Page Synonym(s): Erb's Palsy Table of Contents ( ... done? Clinical Trials Organizations What are Brachial Plexus Injuries? The brachial plexus is a network of nerves ...

  12. Brachial plexus (image)

    MedlinePlus

    The brachial plexus is a group of nerves that originate from the neck region and branch off to give rise ... movement in the upper limb. Injuries to the brachial plexus are common and can be debilitating. If the ...

  13. Brachial Plexus Injuries

    MedlinePlus

    ... to the shoulder, arm, and hand. Brachial plexus injuries are caused by damage to those nerves. Symptoms ... sensation in the arm or hand Brachial plexus injuries can occur as a result of shoulder trauma, ...

  14. Amyotrophic lateral sclerosis

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/000688.htm Amyotrophic lateral sclerosis To use the sharing features on this page, please enable JavaScript. Amyotrophic lateral sclerosis, or ALS, is a disease of the nerve ...

  15. Brachial plexus variations during the fetal period.

    PubMed

    Woźniak, Jowita; Kędzia, Alicja; Dudek, Krzysztof

    2012-12-01

    The brachial plexus is an important nervous system structure. It can be injured during the perinatal period and by postnatal damage. The goal of this study was to assess human fetal brachial plexus variability. A total of 220 brachial plexuses were surgically prepared from 110 human fetuses aged 14-32 weeks of fetal life (50 females and 60 males) ranging in CRL from 80 to 233 mm. The study incorporated the following methods: dissectional and anthropological, digital image acquisition, digital image processing using Image J and GIMP software, and statistical methods (Statistica 9.0). Symmetry and sexual dimorphism were examined. Anomalies of the brachial plexuses were observed in 117 (53.18 %) cases. No sexual dimorphism was found. It was observed that cord variations occurred more often on the left side. Division variants (33.64 %) occurred most often, but also cords (18.18 %) as well as root nerves and terminal ramifications (15.90 %) were found. Trunk anomalies were rare and occurred in only 5.45 % of plexuses. Three height types of median nerve roots in combination with the nerve were distinguished. In one-third of cases, median nerve root connections were found below the axillary fossa and even half in the proximal part of the humerus. In conclusion, the brachial plexus was characterized for anatomical structural variability. Most often division and cord variations were observed. Anomalies occurred regardless of sex or body side except for cord variants. Brachial plexus variation recognition is significant from the neurosurgical and traumatological point of view. PMID:22945314

  16. Amyotrophic Lateral Sclerosis

    MedlinePlus

    Amyotrophic lateral sclerosis (ALS) is a nervous system disease that attacks nerve cells called neurons in your ... people with ALS die from respiratory failure. The disease usually strikes between age 40 and 60. More ...

  17. 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 ...

  18. 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

  19. Brachial artery pseudoaneurysm

    PubMed Central

    Kemp, Katie; Radwan, Rami; Shingler, Guy; Davies, Chris

    2014-01-01

    We describe a case of an elderly man who presented with an upper arm swelling that had developed following a humeral fracture 8 months previously. The swelling was painless but associated with significantly diminished motor function of his right hand and concurrent paraesthaesia. On examination, a large pulsatile mass was identified and CT angiography confirmed the presence of an 11×7 cm brachial artery pseudoaneurysm. The patient underwent surgical repair in which a fragment of the humerus was found to have punctured the brachial artery resulting in a pseudoaneurysm. The patient had an uncomplicated postoperative period and was discharged 2 days later having regained some motor function in his right hand. PMID:24859555

  20. Conjugal amyotrophic lateral sclerosis

    PubMed Central

    Dewitt, John D.; Kwon, Julia; Burton, Rebecca

    2012-01-01

    Amyotrophic lateral sclerosis (ALS) is a disease characterized by progressive degeneration of motor neurons in the motor cortex, brainstem, and spinal cord. The incidence of sporadic ALS is 1.5 to 2.7 in 100,000, and the prevalence is 5.2 to 6.0 in 100,000. Conjugal ALS is even rarer than sporadic ALS. We report a case of conjugal ALS encountered in our outpatient neurology clinic. PMID:22275781

  1. Brachial plexus injury in newborns

    MedlinePlus

    ... and vascular disorders. In: Fenichel GM, ed. Neonatal Neurology . 4th ed. Philadelphia, PA: Elsevier Churchill Livingstone; 2006: ... CB, Kratz JR, Jelin AC, Gelfand AA. Child neurology: brachial plexus birth injury: what every neurologist needs ...

  2. True aneurysm of brachial artery.

    PubMed

    Hudorović, Narcis; Lovričević, Ivo; Franjić, Dario Bjorn; Brkić, Petar; Tomas, Davor

    2010-10-01

    True upper extremity peripheral artery aneurysms are a rarely encountered arterial disorder. Following computer-tomography angiographic (CT-a) imaging examination, true saccular aneurysm, originating from the left brachial artery was diagnosed in the 77-year-old female without history of trauma. The aneurysm was resected by surgical intervention, and primary repair of the brachial artery was performed by interposition of a part of great saphenous vein harvested from the left groin and creation of two end-to-end anastomoses between interposition graft and previously resected part of brachial artery. No complication was observed during the follow-up. Surgical intervention for upper extremity aneurysms should be initiated without delay. Factors combined with minimal morbidity associated with repair suggest that surgical repair should be performed routinely for true upper extremity arterial aneurysms. PMID:20865459

  3. Changes in Spinal Cord Architecture after Brachial Plexus Injury in the Newborn

    ERIC Educational Resources Information Center

    Korak, Klaus J.; Tam, Siu Lin; Gordon, Tessa; Frey, Manfred; Aszmann, Oskar C.

    2004-01-01

    Obstetric brachial plexus palsy is a devastating birth injury. While many children recover spontaneously, 20-25% are left with a permanent impairment of the affected limb. So far, concepts of pathology and recovery have focused on the injury of the peripheral nerve. Proximal nerve injury at birth, however, leads to massive injury-induced…

  4. [Amyotrophic neuralgia associated with bilateral phrenic paralysis treated with non-invasive mechanical ventilation].

    PubMed

    García García, María Del Carmen; Hernández Borge, Jacinto; Antona Rodríguez, María José; Pires Gonçalves, Pedro; García García, Gema

    2015-09-01

    Amyotrophic neuralgia is an uncommon neuropathy characterized by severe unilateral shoulder pain. Isolated or concomitant involvement of other peripheral motor nerves depending on the brachial plexus such as phrenic or laryngeal nerves is unusual(1). Its etiology is unknown, yet several explanatory factors have been proposed. Phrenic nerve involvement, either unilateral or bilateral, is exceedingly rare. Diagnosis relies on anamnesis, functional and imaging investigations and electromyogram. We report the case of a 48-year-old woman with a past history of renal transplantation due to proliferative glomerulonephritis with subsequent transplant rejection, who was eventually diagnosed with amyotrophic neuralgia with bilateral phrenic involvement, and who required sustained non-invasive mechanical ventilation. PMID:26049960

  5. Proximity of arteries to the anterior ulna with changing flexion.

    PubMed

    Enad, Jerome G; Douglas, Thomas J; Ruland, Robert T

    2015-04-01

    During surgery for elbow fracture, wires and screws crossing the elbow from posterior to anterior place the brachial and ulnar arteries at risk for inadvertent penetration. The authors' goal was to define the sagittal proximity of the brachial and ulnar arteries to the proximal ulna throughout an arc of elbow motion using dynamic fluoroscopy. The brachial artery was injected with barium in 10 fresh-frozen cadaveric elbows. Sagittal fluoroscopic images were obtained at elbow flexion angles of 0°, 30°, 60°, 90°, and 120°. Two measurements were obtained at each flexion angle: (1) the distance between the coronoid tip and the brachial artery and (2) the distance between the coronoid base and the ulnar artery. One-way analysis of variance was used to compare mean distances for each flexion angle within each measurement group. A coronal image identified the mediolateral course of the brachial artery. The distance from the coronoid tip to the brachial artery significantly increased with increasing flexion from 0° to 60° (P<.001). The distance from the ulnar artery to the coronoid base significantly increased with increasing flexion from 0° to 120° (P<.002). The brachial artery traversed lateral to the coronoid in 9 of 10 specimens. The brachial and ulnar arteries are located further from the coronoid with increasing elbow flexion to at least 60°, and the brachial artery is typically located lateral to the coronoid in the coronal plane. These measurements can be used as surgical guides to reduce the risk of arterial injury during olecranon fracture surgery. PMID:25901616

  6. Amyotrophic lateral sclerosis mimic syndromes

    PubMed Central

    Ghasemi, Majid

    2016-01-01

    Amyotrophic lateral sclerosis (ALS) misdiagnosis has many broad implications for the patient and the neurologist. Potentially curative treatments exist for certain ALS mimic syndromes, but delay in starting these therapies may have an unfavorable effect on outcome. Hence, it is important to exclude similar conditions. In this review, we discuss some of the important mimics of ALS. PMID:27326363

  7. Brachial neuritis following a corticosteroid injection.

    PubMed

    Robinson, Matthew; Fulcher, Mark

    2014-01-01

    This report presents a case of brachial neuritis following a subacromial corticosteroid injection. The patient developed an anterior interosseous neuropathy shortly after the injection, with no other trigger being identified. This neuropathy has unfortunately not shown any sign of recovery at 2 years. The authors propose that corticosteroid injection be added to the list of possible triggering events of brachial neuritis and highlight the frequent use of oral corticosteroids in its treatment. (1) The injection of local anaesthetic and corticosteroid should be considered as a potential trigger for brachial neuritis. (2) Brachial neuritis should be considered in the differential diagnosis for patients presenting with severe arm pain and weakness. (3) The nerves originating from the upper trunk of the brachial plexus are most commonly affected. (4) The anterior interosseous nerve is involved in one-third of cases. PMID:24596414

  8. 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. PMID:23550891

  9. A cadaveric microanatomical study of the fascicular topography of the brachial plexus.

    PubMed

    Sinha, Sumit; Prasad, G Lakshmi; Lalwani, Sanjeev

    2016-08-01

    OBJECT Mapping of the fascicular anatomy of the brachial plexus could provide the nerve surgeon with knowledge of fascicular orientation in spinal nerves of the brachial plexus. This knowledge might improve the surgical outcome of nerve grafting in brachial plexus injuries by anastomosing related fascicles and avoiding possible axonal misrouting. The objective of this study was to map the fascicular topography in the spinal nerves of the brachial plexus. METHODS The entire right-sided brachial plexus of 25 adult male cadavers was dissected, including all 5 spinal nerves (C5-T1), from approximately 5 mm distal to their exit from the intervertebral foramina, to proximal 1 cm of distal branches. All spinal nerves were tagged on the cranial aspect of their circumference using 10-0 nylon suture for orientation. The fascicular dissection of the C5-T1 spinal nerves was performed under microscopic magnification. The area occupied by different nerve fascicles was then expressed as a percentage of the total cross-sectional area of a spinal nerve. RESULTS The localization of fascicular groups was fairly consistent in all spinal nerves. Overall, 4% of the plexus supplies the suprascapular nerve, 31% supplies the medial cord (comprising the ulnar nerve and medial root of the median nerve [MN]), 27.2% supplies the lateral cord (comprising the musculocutaneous nerve and lateral root of the MN), and 37.8% supplies the posterior cord (comprising the axillary and radial nerves). CONCLUSIONS The fascicular dissection and definitive anatomical localization of fascicular groups is feasible in plexal spinal nerves. The knowledge of exact fascicular location might be translatable to the operating room and can be used to anastomose related fascicles in brachial plexus surgery, thereby avoiding the possibility of axonal misrouting and improving the results of plexal reconstruction. PMID:26654179

  10. 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

  11. Acromioclavicular joint dislocation with associated brachial plexus injury

    PubMed Central

    Gallagher, Charles Alexander; Blakeney, William; Zellweger, René

    2014-01-01

    We present the case of a 32-year-old female who sustained a left acromioclavicular (AC) joint type V injury and brachial plexus injury. The patient's AC joint injury was identified 6 days after she was involved in a motorbike accident where she sustained multiple other injuries. She required operative fixation of the AC joint using a locking compression medial proximal tibial plate. At 3 months post operatively, the patient was found to have a subluxed left shoulder as a result of an axonal injury to the upper trunk of the brachial plexus. In addition, the tibial plate had cut out. The plate was subsequently removed. At 8 months the glenohumeral articulation had been restored and the patient had clinically regained significant shoulder function. After 15 months the patient was pain free and could complete all her activities of daily living without impediment. She returned to playing competitive pool after 24 months. PMID:24855076

  12. Neurinomas of the brachial plexus: case report.

    PubMed

    Forte, A; Gallinaro, L S; Bertagni, A; Montesano, G; Prece, V; Illuminati, G

    1999-01-01

    Neurinomas, also referred to as neurilemmomas and schwannomas, are rare benign tumours of the peripheral nerves, a low proportion of which arise from the brachial plexus. Authors report a case of an ancient schwannoma arising from the brachial plexus. The tumour, usually asymptomatic, may cause sensory radicular symptoms, or rarely motor deficits in the involved arm. Enucleation of the tumour from the nerve without damage to any of the fascicles is the correct treatment. PMID:10710825

  13. In situ cephalic vein bypasses from axillary to the brachial artery after catheterization injuries.

    PubMed

    Hudorovic, Narcis; Lovricevic, Ivo; Ahel, Zaky

    2010-07-01

    The need to bypass to the brachial artery is rare. Over a five-year period, 16 patients had suffered iatrogenic post-catheterization injuries of the upper extremity. We have performed 16 bypasses, in 16 patients, mean age was 65 years (range 47-75), to the brachial artery originating from an artery proximal to the shoulder joint. In all cases, the axillary artery was the donor artery. All bypasses were created by using the cephalic vein with the in situ technique and distal anastomoses were made to a distance-free section of brachial artery. No operative mortality, neurological complications or major upper-extremity amputation was associated with the procedure. Life-long-conduit analysis showed 75% patency in the five-year period. After iatrogenic post-catheterization trauma of arterial system of upper extremity, bypasses from axillary to brachial artery with the cephalic vein with the in situ technique is a safe operation with satisfactory long-term patency. PMID:20395248

  14. Herpetic Brachial Plexopathy: Application of Brachial Plexus Magnetic Resonance Imaging and Ultrasound-Guided Corticosteroid Injection.

    PubMed

    Kim, Jeong-Gil; Chung, Sun G

    2016-05-01

    Herpes zoster, commonly known as shingles, is an infectious viral disease characterized by painful, unilateral skin blisters occurring in specific sensory dermatomes. Motor paresis is reported in 0.5% to 5% of patients. Although the mechanism of zoster paresis is still unclear, the virus can spread from the dorsal root ganglia to the anterior horn cell or anterior spinal nerve roots. It rarely involves the brachial plexus. We report a case of brachial plexitis following herpes zoster infection in which pathological lesions were diagnosed using brachial plexus magnetic resonance imaging and treated with ultrasound-guided perineural corticosteroid injection. PMID:26829085

  15. Brachial Artery Injury Accompanying Closed Elbow Dislocations

    PubMed Central

    Harnarayan, Patrick; Cawich, Shamir O.; Harnanan, Dave; Budhooram, Steve

    2014-01-01

    Introduction Brachial artery injuries from elbow dislocations are uncommon, but they may lead to disastrous consequences if the diagnosis is delayed. Presentation of case We report a case of a patient who sustained a fall onto the elbow, with dislocation and brachial artery injury, despite an ipsilateral radial pulse being palpable. Discussion Clinicians should maintain a high index of suspicion for brachial injury when patients present with a fall onto the elbow coupled with signs suggestive of fracture-dislocation, nerve injury and/or signs of limb ischemia. Frank ischamia, however, is uncommon as there is a rich collateral anastomosis in the upper limb. Conclusion A high index of suspicion should be maintained in order to make the diagnosis early. Exploration with excision of the injured segment and reverse vein interposition grafting is the treatment of choice in these cases. PMID:25644552

  16. Magnetic resonance neurography of the brachial plexus

    PubMed Central

    Upadhyaya, Vaishali; Upadhyaya, Divya Narain; Kumar, Adarsh; Pandey, Ashok Kumar; Gujral, Ratni; Singh, Arun Kumar

    2015-01-01

    Magnetic Resonance Imaging (MRI) is being increasingly recognised all over the world as the imaging modality of choice for brachial plexus and peripheral nerve lesions. Recent refinements in MRI protocols have helped in imaging nerve tissue with greater clarity thereby helping in the identification, localisation and classification of nerve lesions with greater confidence than was possible till now. This article on Magnetic Resonance Neurography (MRN) is based on the authors’ experience of imaging the brachial plexus and peripheral nerves using these protocols over the last several years. PMID:26424974

  17. Magnetic resonance imaging in brachial plexus injury.

    PubMed

    Caranci, F; Briganti, F; La Porta, M; Antinolfi, G; Cesarano, E; Fonio, P; Brunese, L; Coppolino, F

    2013-08-01

    Brachial plexus injury represents the most severe nerve injury of the extremities. While obstetric brachial plexus injury has showed a reduction in the number of cases due to the improvements in obstetric care, brachial plexus injury in the adult is an increasingly common clinical problem. The therapeutic measures depend on the pathologic condition and the location of the injury: Preganglionic avulsions are usually not amenable to surgical repair; function of some denervated muscles can be restored with nerve transfers from intercostals or accessory nerves and contralateral C7 transfer. Postganglionic avulsions are repaired with excision of the damaged segment and nerve autograft between nerve ends or followed up conservatively. Magnetic resonance imaging is the modality of choice for depicting the anatomy and pathology of the brachial plexus: It demonstrates the location of the nerve damage (crucial for optimal treatment planning), depicts the nerve continuity (with or without neuroma formation), or may show a completely disrupted/avulsed nerve, thereby aiding in nerve-injury grading for preoperative planning. Computed tomography myelography has the advantage of a higher spatial resolution in demonstration of nerve roots compared with MR myelography; however, it is invasive and shows some difficulties in the depiction of some pseudomeningoceles with little or no communication with the dural sac. PMID:23949940

  18. What has changed in brachial plexus surgery?

    PubMed Central

    de Rezende, Marcelo Rosa; Silva, Gustavo Bersani; de Paula, Emygdio José Leomil; Junior, Rames Mattar; de Camargo, Olavo Pires

    2013-01-01

    Brachial plexus injuries, in all their severity and complexity, have been extensively studied. Although brachial plexus injuries are associated with serious and often definitive sequelae, many concepts have changed since the 1950s, when this pathological condition began to be treated more aggressively. Looking back over the last 20 years, it can be seen that the entire approach, from diagnosis to treatment, has changed significantly. Some concepts have become better established, while others have been introduced; thus, it can be said that currently, something can always be offered in terms of functional recovery, regardless of the degree of injury. Advances in microsurgical techniques have enabled improved results after neurolysis and have made it possible to perform neurotization, which has undoubtedly become the greatest differential in treating brachial plexus injuries. Improvements in imaging devices and electrical studies have allowed quick decisions that are reflected in better surgical outcomes. In this review, we intend to show the many developments in brachial plexus surgery that have significantly changed the results and have provided hope to the victims of this serious injury. PMID:23644864

  19. Amyotrophic Lateral Sclerosis: A Historical Perspective.

    PubMed

    Katz, Jonathan S; Dimachkie, Mazen M; Barohn, Richard J

    2015-11-01

    This article looks back in time to see where the foundational basis for the understanding of amyotrophic lateral sclerosis originated. This foundation was created primarily in France by Jean-Martin Charcot and his fellow countrymen and disciples, along with key contributions from early clinicians in England and Germany. The early work on amyotrophic lateral sclerosis provides a useful foundation for today's clinicians with respect to tying together genetic and biologic aspects of the disorder that have been discovered over the past few decades. PMID:26515617

  20. Retroviruses and amyotrophic lateral sclerosis

    PubMed Central

    Alfahad, Tariq; Nath, Avindra

    2013-01-01

    Amyotrophic lateral sclerosis (ALS) is a progressive, invariably fatal neurologic disorder resulting from upper and lower motor neuron degeneration, which typically develops during the sixth or seventh decade of life, and is diagnosed based on standard clinical criteria. Its underlying cause remains undetermined. The disease may occur with increased frequency within certain families, often in association with specific genomic mutations, while some sporadic cases have been linked to environmental toxins or trauma. Another possibility, first proposed in the 1970s, is that retroviruses play a role in pathogenesis. In this paper, we review the published literature for evidence that ALS is associated either with infection by an exogenous retrovirus or with the expression of human endogenous retroviral (HERV) sequences in cells of the central nervous system. A small percentage of persons infected with the human immunodeficiency virus-1 (HIV-1) or human T cell leukemia virus-1 (HTLV-1) develop ALS-like syndromes. While HTLV-1 associated ALS-like syndrome has several features that may distinguish it from classical ALS, HIV-infected patients may develop neurological manifestations that resemble classical ALS although it occurs at a younger age and they may show a dramatic improvement following the initiation of antiretroviral therapy. However, most patients with probable or definite ALS show no evidence of HIV-1 or HTLV-1 infection. In contrast, recent reports have shown a stronger association with HERV, as analysis of serum samples, and postmortem brain tissue from a number of patients with a classical ALS has revealed significantly increased expression of HERV-K, compared to controls. These findings suggest that endogenous retroviral elements are involved in the pathophysiology of ALS, but there is no evidence that they are the primary cause of the syndrome. PMID:23707220

  1. Proximity fuze

    DOEpatents

    Harrison, Thomas R.

    1989-08-22

    A proximity fuze system includes an optical ranging apparatus, a detonation circuit controlled by the optical ranging apparatus, and an explosive charge detonated by the detonation cirtcuit. The optical ranging apparatus includes a pulsed laser light source for generating target ranging light pulses and optical reference light pulses. A single lens directs ranging pulses to a target and collects reflected light from the target. An optical fiber bundle is used for delaying the optical reference pulses to correspond to a predetermined distance from the target. The optical ranging apparatus includes circuitry for providing a first signal depending upon the light pulses reflected from the target, a second signal depending upon the light pulses from the optical delay fiber bundle, and an output signal when the first and second signals coincide with each other. The output signal occurs when the distance from the target is equal to the predetermined distance form the target. Additional circuitry distinguishes pulses reflected from the target from background solar radiation.

  2. Proximity fuze

    DOEpatents

    Harrison, T.R.

    1987-07-10

    A proximity fuze system includes an optical ranging apparatus, a detonation circuit controlled by the optical ranging apparatus, and an explosive charge detonated by the detonation circuit. The optical ranging apparatus includes a pulsed laser light source for generating target ranging light pulses and optical reference light pulses. A single lens directs ranging pulses to a target and collects reflected light from the target. An optical fiber bundle is used for delaying the optical reference pulses to correspond to a predetermined distance from the target. The optical ranging apparatus includes circuitry for providing a first signal depending upon the light pulses reflected from the target, a second signal depending upon the light pulses from the optical delay fiber bundle, and an output signal when the first and second signals coincide with each other. The output signal occurs when the distance from the target is equal to the predetermined distance from the target. Additional circuitry distinguishes pulses reflected from the target from background solar radiation. 3 figs.

  3. [Sporadic amyotrophic lateral sclerosis. Diagnostic criteria].

    PubMed

    Godoy, J M; de Oliveira, M A; de Moraes Neto, J B; Balassiano, S L; Montagna, N; Pinto, J R; Skacel, M

    1993-06-01

    The authors report two cases of amyotrophic lateral sclerosis (ALS) misdiagnosis (a craniocervical junction disorder, and a cervical spinal cord ependymoma). They review some causes of ALS-like syndrome and propose a protocol to be adopted for the study of all patients who present clinical abnormalities suggesting ALS. PMID:8274087

  4. Double cortical stimulation in amyotrophic lateral sclerosis.

    PubMed Central

    Yokota, T; Yoshino, A; Inaba, A; Saito, Y

    1996-01-01

    OBJECTIVE: Transcranial double magnetic stimulation on the motor cortex was used to investigate central motor tract function in 16 patients with amyotrophic lateral sclerosis, five with spinal muscular atrophy, and 16 age matched normal controls. METHODS: Surface EMG responses were recorded from the relaxed abductor pollicis brevis (APB) muscle. RESULTS: Responses to test stimuli were markedly attenuated by a subthreshold conditioning stimulus given at a condition-test (C-T) interval of 1-4 ms in normal controls and patients with spinal muscular atrophy, but attenuation was mild in patients with amyotrophic lateral sclerosis. In the normal controls this suppression was caused by activation of the intracortical inhibitory mechanism because responses to electrical test stimuli and the H wave were not suppressed by the same magnetic subthreshold conditioning stimulus. In amyotrophic lateral sclerosis the effect of the conditioning cortical stimulus on the H wave was also in the normal range. CONCLUSION: The intracortical inhibitory mechanism may be impaired in patients with amyotrophic lateral sclerosis. PMID:8971106

  5. Axillary brachial plexus blockade in moyamoya disease?

    PubMed Central

    Yalcin, Saban; Cece, Hasan; Nacar, Halil; Karahan, Mahmut Alp

    2011-01-01

    Moyamoya disease is characterized by steno-occlusive changes of the intracranial internal carotid arteries. Cerebral blood flow and metabolism are strictly impaired. The goal in perioperative anaesthetic management is to preserve the stability between oxygen supply and demand in the brain. Peripheral nerve blockade allows excellent neurological status monitoring and maintains haemodynamic stability which is very important in this patient group. Herein, we present an axillary brachial plexus blockade in a moyamoya patient operated for radius fracture. PMID:21712873

  6. Axillary brachial plexus blockade in moyamoya disease?

    PubMed

    Yalcin, Saban; Cece, Hasan; Nacar, Halil; Karahan, Mahmut Alp

    2011-03-01

    Moyamoya disease is characterized by steno-occlusive changes of the intracranial internal carotid arteries. Cerebral blood flow and metabolism are strictly impaired. The goal in perioperative anaesthetic management is to preserve the stability between oxygen supply and demand in the brain. Peripheral nerve blockade allows excellent neurological status monitoring and maintains haemodynamic stability which is very important in this patient group. Herein, we present an axillary brachial plexus blockade in a moyamoya patient operated for radius fracture. PMID:21712873

  7. Proximal humerus fractures in children and adolescents.

    PubMed

    Lefèvre, Y; Journeau, P; Angelliaume, A; Bouty, A; Dobremez, E

    2014-02-01

    Proximal humerus fractures are rare in paediatric traumatology. Metaphyseal fractures account for about 70% of cases and epiphyseal separation for the remaining 30%. The development and anatomy of the proximal humerus explain the various fracture types, displacements, and potential complications; and also help in interpreting the radiographic findings, most notably in young children. Physicians should be alert to the possibility of an underlying lesion or pathological fracture requiring appropriate diagnostic investigations, and they should consider child abuse in very young paediatric patients. Although the management of proximal humerus fractures remains controversial, the extraordinary remodelling potential of the proximal humerus in skeletally immature patients often allows non-operative treatment without prior reduction. When the displacement exceeds the remodelling potential suggested by the extent of impaction, angulation, and patient age, retrograde elastic stable intramedullary nailing (ESIN) provides effective stabilisation. As a result, the thoraco-brachial abduction cast is less often used, although this method remains a valid option. Retrograde ESIN must be performed by a surgeon who is thoroughly conversant with the fundamental underlying principles. Direct percutaneous pinning is a fall-back option when the surgeon's experience with ESIN is insufficient. Finally, open reduction is very rarely required and should be reserved for severely displaced fractures after failure of closed reduction. When these indications are followed, long-term outcomes are usually excellent, with prompt resumption of previous activities and a low rate of residual abnormalities. PMID:24394917

  8. Proximal renal tubular acidosis

    MedlinePlus

    Renal tubular acidosis - proximal; Type II RTA; RTA - proximal; Renal tubular acidosis type II ... by alkaline substances, mainly bicarbonate. Proximal renal tubular acidosis (Type II RTA) occurs when bicarbonate is not ...

  9. Idiopathic brachial plexus neuritis after laparoscopic treatment of endometriosis: a complication that may mimic position-related brachial plexus injury.

    PubMed

    Minas, Vasileios; Aust, Thomas

    2013-01-01

    We report the case of a 37-year-old woman who developed idiopathic brachial plexus neuritis, also referred to as Parsonage-Turner syndrome, after laparoscopic excision of endometriosis. The differential diagnosis between this non-position-related neuritis and brachial plexus injury is discussed. The aim of this report was to raise awareness on this distressing postoperative complication. PMID:24183278

  10. Lightning strike-induced brachial plexopathy

    PubMed Central

    Bhargava, Amita N.; Kasundra, Gaurav M.; Khichar, Subhakaran; Bhushan, Bharat S. K.

    2014-01-01

    We describe a patient who presented with a history of lightning strike injury. Following the injury, he sustained acute right upper limb weakness with pain. Clinically, the lesion was located to the upper and middle trunk of the right brachial plexus, and the same confirmed with electrophysiological studies. Nerve damage due to lightning injuries is considered very rare, and a plexus damage has been described infrequently, if ever. Thus, the proposed hypothesis that lightning rarely causes neuropathy, as against high-voltage electric current, due to its shorter duration of exposure not causing severe burns which lead to nerve damage, needs to be reconsidered. PMID:25288846

  11. Central Adaptation following Brachial Plexus Injury.

    PubMed

    Simon, Neil G; Franz, Colin K; Gupta, Nalin; Alden, Tord; Kliot, Michel

    2016-01-01

    Brachial plexus trauma (BPT) often affects young patients and may result in lasting functional deficits. Standard care following BPT involves monitoring for clinical and electrophysiological evidence of muscle reinnervation, with surgical treatment decisions based on the presence or absence of spontaneous recovery. Data are emerging to suggest that central and peripheral adaptation may play a role in recovery following BPT. The present review highlights adaptive and maladaptive mechanisms of central and peripheral nervous system changes following BPT that may contribute to functional outcomes. Rehabilitation and other treatment strategies that harness or modulate these intrinsic adaptive mechanisms may improve functional outcomes following BPT. PMID:26409073

  12. Shoulder pain and isolated brachial plexopathy.

    PubMed

    Kishan, Amar U; Syed, Sana; Fiorito-Torres, Franchesca; Thakore-James, Manisha

    2012-01-01

    Pancoast syndrome, classically considered as a constellation of (1) pain along the C8-T2 dermatomes, (2) weakness and atrophy of the hand and (3) Horner's syndrome, often presents a diagnostic challenge. In fact, it may manifest as a singular orthopaedic complaint, prompting a futile barrage of tests and referrals. The authors present the case of an elderly man who initially presented with severe shoulder pain. Due to progressive pain and weakness, he was referred to rheumatology and was treated with corticosteroid injections for a presumed musculoskeletal lesion. Ultimately, he manifested gross muscular atrophy and worsening pain, prompting a referral to neurology. An electromyogram (EMG) suggested a lower brachial plexopathy, and a follow-up brachial plexus MRI identified a large Pancoast tumour. Unfortunately, his disease was rapidly progressive, and he passed away within 2 months. While the MRI remains the gold standard for diagnosing Pancoast syndrome, an EMG can facilitate diagnosis in difficult cases such as this one. PMID:22744250

  13. [Measurement ofthe ankle-brachial pressure index (ABPI)].

    PubMed

    Kulisić, Sandra Marinović

    2012-10-01

    Measurement of the ankle-brachial pressure index, also known as ankle-brachial index or ankle-arm index is a ratio of the ankle blood pressure and brachial blood pressure. It is easy to perform and allows for diagnosis and further definition of the severity of peripheral arterial disease with sensitivity 90% and specificity 98%. The test is not appropriate for mild arterial changes as in case of comorbidity. Its further objectives are to identify patients at an higher risk of cardiovascular events. PMID:23193828

  14. Brachial plexus injuries in neonates: an osteopathic approach.

    PubMed

    Mason, David C; Ciervo, Carman A

    2009-02-01

    Neonates and infants with brachial plexus injuries are typically treated using splinting, range-of-motion exercise, and, in more severe cases, nerve reconstruction. However, myofascial release--a common osteopathic manipulative treatment technique that has been used to manage thoracic outlet syndrome in adults--may provide effective, noninvasive management of brachial plexus injuries in neonates and infants. While emphasizing the importance of good communication with parents of affected patients, the authors review brachial plexus anatomy, describe diagnostic examinations, and outline a comprehensive treatment strategy. PMID:19269939

  15. 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

  16. 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

  17. Birth brachial plexus palsy: a race against time.

    PubMed

    Patra, Sambeet; Narayana Kurup, Jayakrishnan K; Acharya, Ashwath M; Bhat, Anil K

    2016-01-01

    A 5-year-old child presented to us with weakness of the left upper limb since birth. With the given history of obstetric trauma and limb examination, a diagnosis of birth brachial plexus palsy was made. Brachial plexus exploration along with microsurgery was performed at the same time which included extrinsic neurolysis of the roots and trunks and nerve transfer for better shoulder external rotation and elbow flexion. Both the movements were severely restricted previously due to co-contractures with the shoulder internal rotators and triceps. The problem of birth brachial plexus palsy is proving to be a global health burden both in developed countries and in developing countries such as India. The lack of awareness among the general public and primary healthcare providers and inadequate orthopaedic and neurosurgeons trained to treat the condition have worsened the prognosis. This case lays stress on the delayed complications in birth brachial palsy and its effective management. PMID:27402656

  18. [Myxoid/round cell liposarcoma of the brachial plexus].

    PubMed

    Giner, Javier; Isla, Alberto; Hernández, Borja; Nistal, Manuel

    2014-01-01

    Myxoid/round cell liposarcoma is a soft tissue sarcoma that is extremely rare in the brachial plexus. We report a case of a myxoid/round cell liposarcoma originating in the brachial plexus that was surgically resected and evolved well, with no deficit or recurrence after 2 years of follow-up. To date, there has been no other case of this sarcoma in the literature. PMID:25126709

  19. Proximal Tibial Bone Graft

    MedlinePlus

    ... Complications Potential problems after a PTBG include infection, fracture of the proximal tibia and pain related to the procedure. Frequently Asked Questions If proximal tibial bone graft is taken from my knee, will this prevent me from being able to ...

  20. Limb preference in children with obstetric brachial plexus palsy.

    PubMed

    Yang, Lynda J-S; Anand, Praveen; Birch, Rolfe

    2005-07-01

    Brachial plexus palsy affects children differently than adults. In children with obstetric brachial plexus palsy, motor development must depend on nervous system adaptation. Previous studies report sensory plasticity in these children. This noninvasive study provides support for neural plasticity (the general ability of the brain to reorganize neural pathways based on new experiences) in children with obstetric brachial plexus palsy by considering upper limb preference. As in the general population, we expect that 90% of children would prefer their right upper limb. However, only 17% of children affected by right obstetric brachial plexus palsy prefer the right upper limb for overall movement; children with left obstetric brachial plexus palsy did not significantly differ from the general population in upper limb preference. This study also provides the first evidence of a significant correlation between actual task performance and select obstetric brachial plexus palsy outcome measurement systems, thereby justifying the routine use of these outcome measurement systems as a reflection of the practical utility of the affected limb to the patient. PMID:15876521

  1. Amyotrophic lateral sclerosis: one or multiple causes?

    PubMed Central

    Bastos, Aline Furtado; Orsini, Marco; Machado, Dionis; Mello, Mariana Pimentel; Nader, Sergio; Silva, Júlio Guilherme; da Silva Catharino, Antonio M.; de Freitas, Marcos R.G.; Pereira, Alessandra; Pessoa, Luciane Lacerda; Sztajnbok, Flavio R.; Leite, Marco Araújo; Nascimento, Osvaldo J.M.; Bastos, Victor Hugo

    2011-01-01

    The Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease in the adulthood, and it is characterized by rapid and progressive compromise of the upper and lower motor neurons. The majority of the cases of ALS are classified as sporadic and, until now, a specific cause for these cases still is unknown. To present the different hypotheses on the etiology of ALS. It was carried out a search in the databases: Bireme, Scielo and Pubmed, in the period of 1987 to 2011, using the following keywords: Amyotrophic lateral sclerosis, motor neuron disease, etiology, causes and epidemiology and its similar in Portuguese and Spanish. It did not have consensus as regards the etiology of ALS. Researches demonstrates evidences as regards intoxication by heavy metals, environmental and occupational causes, genetic mutations (superoxide dismutase 1), certain viral infections and the accomplishment of vigorous physical activity for the development of the disease. There is still no consensus regarding the involved factors in the etiology of ALS. In this way, new research about these etiologies are necessary, for a better approach of the patients, promoting preventive programs for the disease and improving the quality of life of the patients. PMID:21785676

  2. Brachial plexus neuropathy secondary to septic arthritis and osteomyelitis: report of two cases.

    PubMed

    Wang, Y C; Lin, F K; Hung, K L; Wu, D Y

    1994-01-01

    Two infants, delivered uneventfully, later developed right brachial plexus palsy secondary to pyogenic osteomyelitis and arthritis of the right shoulder joint. Weakness of right arms occurred at the sixth and tenth days of age respectively. Both had right arm tenderness on palpation and passive movement. Roentgenograms of their right shoulder joints showed irregular radiolucency of the proximal margin of right humerus head. In both cases, electromyography revealed various degrees of significant denervation pattern for the C5-C7 innervated muscles. Pus culture from right shoulder joints grew Streptococcus viridans and Staphylococcus aureus, respectively. After antibiotic therapy and arthrotomy with drainage, weakness improved gradually following continuous rehabilitation. Follow-up at six months of age showed almost complete recovery of right upper extremity function in one patient, but mild residual weakness in the other. Follow-up electromyography studies showed continued improvement. The possible mechanism of this rare occurrence is discussed. PMID:7942033

  3. Autogenous side-to-side brachial-basilic fistulas without vein transposition: a valuable option?

    PubMed

    Lomonte, Carlo; Casucci, Francesco; Antonelli, Maurizio; Losurdo, Nicola; Marchio, Giovanni; Teutonico, Annalisa; Libutti, Pasquale; Basile, Carlo

    2009-01-01

    An autogenous brachial-basilic arteriovenous fistula (BBAVF) in the upper arm must be considered before placing prosthetic grafts in hemodialysis patients with multiple failures of forearm AVFs. The aim of this observational study was to compare technical and clinical outcomes of a new construction technique for BBAVF (n-BBAVF) with that of the standard one-stage side-artery to end-vein transposed BBAVF (t-BBAVF). A n-BBAVF is constructed in the following way: basilic vein and brachial artery are isolated. Patency of the proximal and distal vein is verified by injecting warmed (37 degrees C) saline solution. A venotomy and an arterotomy of 4-5 mm are performed. The two vessels are prepared for a side-to-side anastomosis without transposition of the vein. The latter allows both an antegrade and retrograde flow along the basilic vein, both proximally and distally to the anastomosis with more sites available for the venipunctures of the dialysis. Thirty BBAVFs were constructed as the secondary or tertiary vascular access in 30 patients over a 4-year period: 17 patients with adequate forearm basilic vein underwent the construction of a n-BBAVF; 13 underwent the construction of a t-BBAVF. The construction of a n-BBAVF requires a significantly lesser surgical time (55.0 +/- 9.0 minutes vs. 115.0 +/- 18.0, p < 0.0001), has fewer surgical complications (5.9% vs. 46.2%, p < 0.0001), and a reduced time to first use (24.5 +/- 6.3 vs. 37.7 +/- 9.1 days, p < 0.0001) than that of a t-BBAVF. n-BBAVFs showed a relatively low rate of thrombosis per patient-year at risk (0.067 at 1 year and 0.099 at 2 years). The latter was significantly lower at 1 year when compared with t-BBAVFs (0.067 vs. 0.285; p < 0.004). Our policy of "all AVFs should be autogenous" led us to the construction of a vascular access which is based on a side-to-side anastomosis between the brachial artery and the basilic vein without transposition of the vein allowing both antegrade and retrograde flow into the

  4. 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

  5. Amyotrophic lateral sclerosis and environmental factors.

    PubMed

    Bozzoni, V; Pansarasa, Orietta; Diamanti, L; Nosari, G; Cereda, C; Ceroni, M

    2016-01-01

    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

  6. Amyotrophic lateral sclerosis associated with pregnancy.

    PubMed

    Tyagi, A; Sweeney, B J; Connolly, S

    2001-12-01

    Amyotrophic lateral sclerosis (ALS) is the most common, progressive motor neurone disease but is rare in the obstetric population. Only 4 cases have been described in the English literature since 1975. We describe a 29 year old woman who presented with ataxia, lower limb weakness and dysarthria 4 weeks after the birth of her first child. The symptoms had onset during the pregnancy but had not been considered remarkable. There were clinical features of upper and lower motor neurone involvement without any sensory loss. MRI of brain and spine was normal. CSF analysis was negative. EMG studies confirmed the presence of widespread anterior horn cell dysfunction compatible with ALS. The patient was commenced on Riluzole and has progressed clinically, at 12 months post diagnosis. PMID:11799421

  7. 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

  8. Toward precision medicine in amyotrophic lateral sclerosis

    PubMed Central

    Liu, Chang-Yun; Che, Chun-Hui

    2016-01-01

    Precision medicine is an innovative approach that uses emerging biomedical technologies to deliver optimally targeted and timed interventions, customized to the molecular drivers of an individual’s disease. This approach is only just beginning to be considered for treating amyotrophic lateral sclerosis (ALS). The clinical and biological complexities of ALS have hindered development of effective therapeutic strategies. In this review we consider applying the key elements of precision medicine to ALS: phenotypic classification, comprehensive risk assessment, presymptomatic period detection, potential molecular pathways, disease model development, biomarker discovery and molecularly tailored interventions. Together, these would embody a precision medicine approach, which may provide strategies for optimal targeting and timing of efforts to prevent, stop or slow progression of ALS. PMID:26889480

  9. 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 lowerPomcbut higherAgrpmRNA 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 lateral

  10. Linking β-methylamino-L-alanine exposure to sporadic amyotrophic lateral sclerosis in Annapolis, MD.

    PubMed

    Field, Nicholas C; Metcalf, James S; Caller, Tracie A; Banack, Sandra A; Cox, Paul A; Stommel, Elijah W

    2013-08-01

    Most amyotrophic lateral sclerosis (ALS) cases occur sporadically. Some environmental triggers have been implicated, including beta-methylamino-L-alanine (BMAA), a cyanobacteria produced neurotoxin. This study aimed to identify environmental risk factors common to three sporadic ALS patients who lived in Annapolis, Maryland, USA and developed the disease within a relatively short time and within close proximity to each other. A questionnaire was used to identify potential risk factors for ALS among the cohort of patients. One common factor among the ALS patients was the frequent consumption of blue crab. Samples of blue crab from the patients' local fish market were tested for BMAA using LC-MS/MS. BMAA was identified in these Chesapeake Bay blue crabs. We conclude that the presence of BMAA in the Chesapeake Bay food web and the lifetime consumption of blue crab contaminated with BMAA may be a common risk factor for sporadic ALS in all three patients. PMID:23660330

  11. Respiratory arrest in patients undergoing arteriovenous graft placement with supraclavicular brachial plexus block: a case series.

    PubMed

    Afonso, Anoushka; Beilin, Yaakov

    2013-06-01

    Supraclavicular brachial plexus block is commonly used for upper extremity surgery. Respiratory arrest in three patients with end-stage renal disease after ultrasound-guided supraclavicular brachial plexus block for creation of an arteriovenous graft over a 6-month period is presented. Patients with renal failure may represent a group at particular risk for respiratory failure following supraclavicular brachial plexus block. PMID:23830847

  12. Permanent upper trunk plexopathy after interscalene brachial plexus block.

    PubMed

    Avellanet, Merce; Sala-Blanch, Xavier; Rodrigo, Lidia; Gonzalez-Viejo, Miguel A

    2016-02-01

    Interscalene brachial plexus block (IBPB) has been widely used in shoulder surgical procedures. The incidence of postoperative neural injury has been estimated to be as high as 3 %. We report a long-term neurologic deficit after a nerve stimulator assisted brachial plexus block. A 55 year-old male, with right shoulder impingement syndrome was scheduled for elective surgery. The patient was given an oral dose of 10 mg of diazepam prior to the nerve stimulator assisted brachial plexus block. The patient immediately complained, as soon as the needle was placed in the interscalene area, of a sharp pain in his right arm and he was sedated further. Twenty-four hours later, the patient complained of severe shoulder and arm pain that required an increased dose of analgesics. Severe peri-scapular atrophy developed over the following days. Electromyography studies revealed an upper trunk plexus injury with severe denervation of the supraspinatus, infraspinatus and deltoid muscles together with a moderate denervation of the biceps brachii muscle. Chest X-rays showed a diaphragmatic palsy which was not present post operatively. Pulmonary function tests were also affected. Phrenic nerve paralysis was still present 18 months after the block as was dysfunction of the brachial plexus resulting in an inability to perform flexion, abduction and external rotation of the right shoulder. Severe brachial plexopathy was probably due to a local anesthetic having been administrated through the perineurium and into the nerve fascicles. Severe brachial plexopathy is an uncommon but catastrophic complication of IBPB. We propose a clinical algorithm using ultrasound guidance during nerve blocks as a safer technique of regional anesthesia. PMID:25744163

  13. Retinal Vascular Caliber and Brachial Flow-Mediated Dilation

    PubMed Central

    Nguyen, Thanh T.; Islam, F.M. Amirul; Farouque, H.M. Omar; Klein, Ronald; Klein, Barbara E.K.; Cotch, Mary Frances; Herrington, David M.; Wong, Tien Yin

    2010-01-01

    Background and Purpose Retinal vascular caliber changes have been shown to predict stroke, but the underlying mechanism of this association is unknown. We examined the relationship between retinal vascular caliber with brachial flow-mediated dilation (FMD), a measure of systemic endothelial function. Methods The Multi-Ethnic Study of Atherosclerosis (MESA) is a population-based study of persons 45 to 84 years of age residing in 6 US communities free of clinical cardiovascular disease at baseline. Brachial FMD data were collected at baseline (July 2000 to June 2002), and retinal vascular caliber was measured from digital retinal photographs at the second examination, immediately after the first (August 2002 to January 2004). Data were available for 2851 participants for analysis. Results The mean brachial FMD was 4.39±2.79%. After adjusting for age and gender, brachial FMD was reduced in persons with wider retinal venular caliber (changes in FMD −0.25, 95% CI, −0.36, − 0.13; P<0.001, per SD increase in venular caliber). This relationship persists after adjusting for systolic blood pressure, serum total cholesterol, use of lipid-lowering and antihypertensive medication, body mass index, current smoking status, and hemoglobinA1C (−0.18; 95% CI −0.30, − 0.06; P=0.004, per SD increase in venular caliber). Brachial FMD was not associated with retinal arteriolar caliber. Conclusions Persons with wider retinal venules have reduced brachial FMD, independent of other vascular risk factors. This suggests that retinal venular caliber, previously shown to predict stroke, may be a marker of underlying systemic endothelial dysfunction. PMID:20508189

  14. Pediatric Stinger Syndrome: Acute Brachial Plexopathy After Minor Trauma.

    PubMed

    Quong, Whitney L; Hynes, Sally L; Arneja, Jugpal S

    2015-11-01

    The "stinger" or "burner" is a form of transient brachial plexopathy termed for its characteristic knife-like pain extending from the neck to the fingertips. Muscle weakness and paresthesia are oftentimes associated symptoms and are similarly temporary. Commonly observed in athletes of contact sports, the stinger results from high force trauma causing either traction/direct compression to the brachial plexus or extension/compression of the cervical nerve roots. We describe a pediatric case of a stinger in a 14-year-old boy, which was caused by a relatively low force trauma accident. Our management strategy and recommendations are discussed. PMID:26893985

  15. [Brachial plexus. Long lasting neurological deficit following interscalene blockade of the brachial plexus].

    PubMed

    Funk, W; Angerer, M; Sauer, K; Altmeppen, J

    2000-07-01

    An interscalene block of the brachial plexus was combined with general anaesthesia for repair of a complex chronic lesion of the shoulder. The localisation of the plexus with electro-stimulation and the injection of Bupivacain 0.5% were accomplished easily and without painful sensations. 48 hours later the block was still partially present. Paraesthesia and a sensory and motor innervation deficit affected mainly the dorsal fascicle, but also areas innervated by the median and lateral fascicles. The deficit did not completely disappear for 18 month. The cause could have been due to direct traumatisation during blockade or operation, toxic action of the injected substance (Bupivacain 0.5%, 30 ml), distension of the plexus, a cervical syndrome or an aseptic plexitis, although a definite determination is not possible. However, the pattern of the lesion and the lack of pain during localisation of the plexus and injection favour traumatisation during the acromioplasty. PMID:10969388

  16. Motoneuron afterhyperpolarisation duration in amyotrophic lateral sclerosis

    PubMed Central

    Piotrkiewicz, Maria; Hausmanowa-Petrusewicz, Irena

    2011-01-01

    Abstract Motor unit (MU) potentials were registered from 20 ALS patients and 13 age-matched control individuals during isometric constant force contractions of brachial biceps (BB). The registered signals were decomposed into single MU potential trains. The estimates of duration of the afterhyperpolarisation (AHP) in MNs, derived from the interspike interval variability, was compared between ALS patients (124 MNs) and control subjects (111 MNs) and no significant differences were encountered. However, the relationship between TI and age for patients appeared to be qualitatively different from that of the control group. The dependence of patients’ AHPs on relative force deficit (RFD), which quantified muscle involvement, was more specific. For RFDs below 30%, the AHP estimate was significantly lower than control values and then increased thereafter with increasing RFDs. Moreover, firing rates of patients with the smallest RFDs were significantly higher while firing rates of patients with the greatest RFDs were significantly lower than control values. The AHP shortening in the early stages of muscle impairment is consistent with the decrease in firing threshold of ‘fast’ MNs found in spinal cord slices from neonatal SOD1 mice. The later elongation of the AHP may be caused by the higher vulnerability of ‘fast’ MNs to degeneration and by the influence of reinnervation. Our results are comparable to what has been observed in acute experiments in animal models, providing a bridge between animal and clinical research that may be relevant for identification of mechanism(s) underlying neurodegeneration in ALS. PMID:21486815

  17. Effects of Handgrip Training With Venous Restriction on Brachial Artery Vasodilation

    PubMed Central

    Credeur, Daniel P.; Hollis, Brandon C.; Welsch, Michael A.

    2010-01-01

    Previous studies have shown that resistance training with restricted venous blood flow (Kaatsu) results in significant strength gains and muscle hypertrophy. However, few studies have examined the concurrent vascular responses following restrictive venous blood flow training protocols. Purpose To examine the effects of 4 weeks of handgrip exercise training, with and without venous restriction, on handgrip strength and brachial artery flow mediated dilation (BAFMD). Methods Twelve participants (age=22±1yr; male = 5, female = 7), completed 4 weeks of bilateral handgrip exercise training (Duration: 20 min; Intensity: 60% of the MVC; Cadence: 15 grips*min−1; Frequency: 3 sessions*week−1). During each session venous blood flow was restricted in one arm (Experimental arm = EXP) using a pneumatic cuff placed 4 cm proximal to the antecubital fossa, and inflated to 80 mmHg for the duration of each exercise session. The EXP and control (CON) arm were randomly selected. Handgrip strength was measured using a hydraulic hand dynamometer. Brachial diameters and blood velocity profiles were assessed, using Doppler ultrasonography, before and after 5 min of forearm occlusion (200 mmHg), prior to and at the end of 4 weeks exercise. Results Following exercise training, handgrip strength increased 8.32% (p=0.05) in the CON arm and 16.17% (p=0.05) in the EXP arm. BAFMD increased 24.19% (p=0.0001) in the CON arm, and decreased 30.36% (p=0.0001) in the EXP arm. Conclusion The data indicate handgrip training combined with venous restriction results in superior strength gains, but reduced BAFMD compared to the non-restricted arm. PMID:20019641

  18. 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

  19. Altered Cortical Communication in Amyotrophic Lateral Sclerosis

    PubMed Central

    Blain-Moraes, Stefanie; Mashour, George A.; Lee, Heonsoo; Huggins, Jane E.; Lee, UnCheol

    2013-01-01

    Amyotrophic lateral sclerosis (ALS) is a disorder associated primarily with the degeneration of the motor system. More recently, functional connectivity studies have demonstrated potentially adaptive changes in ALS brain organization, but disease-related changes in cortical communication remain unknown. We recruited individuals with ALS and age-matched controls to operate a brain-computer interface while electroencephalography was recorded over three sessions. Using normalized symbolic transfer entropy, we measured directed functional connectivity from frontal to parietal (feedback connectivity) and parietal to frontal (feedforward connectivity) regions. Feedback connectivity was not significantly different between groups, but feedforward connectivity was significantly higher in individuals with ALS. This result was consistent across a broad electroencephalographic spectrum (4 – 35 Hz), and in theta, alpha and beta frequency bands. Feedback connectivity has been associated with conscious state and was found to be independent of ALS symptom severity in this study, which may have significant implications for the detection of consciousness in individuals with advanced ALS. We suggest that increases in feedforward connectivity represent a compensatory response to the ALS-related loss of input such that sensory stimuli have sufficient strength to cross the threshold necessary for conscious processing in the global neuronal workspace. PMID:23567743

  20. Characteristics of pain in amyotrophic lateral sclerosis

    PubMed Central

    Hanisch, Frank; Skudlarek, Anika; Berndt, Janine; Kornhuber, Malte E

    2015-01-01

    Background Pain is an often underestimated and neglected symptom in amyotrophic lateral sclerosis (ALS). Methods In a cross-sectional survey, 46 patients with ALS, 46 age- and gender matched population-based controls, and 23 diseased controls with myotonic dystrophy type 2 (DM2) were screened for occurrence, type, distribution, and treatment of pain and cramps. Data were collected with the use of the short form brief pain inventory (BPI). Results Pain was reported in 78% of ALS patients,79% of DM2 patients, and 54% of controls (P < 0.05). More ALS patients than controls reported moderate to severe pain (42% vs. 20%). Pain in ALS patients interfered significantly more with daily activities than in controls (median pain interference score: 3.0 vs. 1.2, P < 0.05), especially enjoyment of life (5.0 vs. 1.0) and mood (3.0 vs. 1.0). There was no correlation between the duration of the disease and the severity of pain. Movement-induced cramps were reported in 63% of ALS patients, mostly in the distal extremities. There was no difference in the duration of ALS disease between patients reporting cramps and those who did not. Discussion Our study showed that pain was a relatively frequent symptom which had an important impact on the quality of life. Pain that requires treatment can occur at every stage of ALS. PMID:25642388

  1. Epidemiologic correlates of sporadic amyotrophic lateral sclerosis

    SciTech Connect

    Armon, C.; Kurland, L.T.; Daube, J.R.; O'Brien, P.C. )

    1991-07-01

    The authors evaluated 74 selected patients with amyotrophic lateral sclerosis (ALS) and 201 matched controls for risk factors for ALS by a case-control design and a sequential questionnaire/interview technique to quantitate biographic data. They analyzed occupational and recreational data only for 47 male patients and 47 corresponding patient controls; data for women were insufficient. They used nonparametric analyses to evaluate five primary comparisons of ALS patients with controls: (1) more hard physical labor, p not significant (NS); (2) greater frequency of neurodegenerative disease in family members, p NS; (3) greater exposure to lead, p less than 0.05; (4) more years lived in a rural community, p NS; and (5) more trauma or major surgery, p NS. Men with ALS had worked more frequently at blue-collar jobs (although not a statistically significant difference, p = 0.10) and at welding or soldering (p less than 0.01). These results suggest that there may be an association between ALS in men and exposure to lead vapor. The limited nature of the association favors a multifactorial etiologic mechanism of ALS.

  2. Selective attention impairment in amyotrophic lateral sclerosis.

    PubMed

    Volpato, Chiara; Prats Sedano, Maria Angeles; Silvoni, Stefano; Segato, Nicoletta; Cavinato, Marianna; Merico, Antonio; Piccione, Francesco; Palmieri, Arianna; Birbaumer, Niels

    2016-01-01

    Objective of this study was to evaluate attentional control mechanisms in amyotrophic lateral sclerosis (ALS) using an auditory event-related potentials (ERPs) paradigm. Fifteen mild to moderate ALS patients and 15 healthy controls were administered a brief neuropsychological test battery and an ERPs paradigm assessing selective attention. Four types of auditory stimuli were presented in random order: short standard (200 Hz, 200 ms), long standard (200 Hz, 500 ms), short deviant (1000 Hz, 200 ms) and long deviant (1000 Hz, 500 ms). Participants had to respond to the long deviant stimuli only. During the task the electroencephalogram (EEG) was recorded. The N200, P300 and re-orienting negativity (RON) ERP components were analysed. Compared to controls ALS patients showed reduced amplitudes and delayed latencies of N200, P300 and RON. These results could be attributable to both an alteration in change detection resulting in a reduction of the allocation and re-orientation of attentional resources or a general slowing or reduction of neural processing efficiency in the same system. The ERPs results support the hypothesis that ALS involves extramotor cognitive functions including auditory attentional processing at all processing stages, early (200 ms) and late (300-600 ms). These data prove the usefulness and sensitivity of the auditory ERPs in detection of cognitive functions in ALS patients. PMID:26889872

  3. Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis

    PubMed Central

    Shi, Ping; Gal, Jozsef; Kwinter, David M.; Liu, Xiaoyan; Zhu, Haining

    2009-01-01

    The etiology of motor neuron degeneration in amyotrophic lateral sclerosis (ALS) remains to be better understood. Based on the studies from ALS patients and transgenic animal models, it is believed that ALS is likely to be a multifactorial and multisystem disease. Many mechanisms have been postulated to be involved in the pathology of ALS, such as oxidative stress, glutamate excitotoxicity, mitochondrial damage, defective axonal transport, glia cell pathology and aberrant RNA metabolism. Mitochondria, which play crucial roles in excitotoxicity, apoptosis and cell survival, have shown to be an early target in ALS pathogenesis and contribute to the disease progression. Morphological and functional defects in mitochondria were found in both human patients and ALS mice overexpressing mutant SOD1. Mutant SOD1 was found to be preferentially associated with mitochondria and subsequently impair mitochondrial function. Recent studies suggest that axonal transport of mitochondria along microtubules and mitochondrial dynamics may also be disrupted in ALS. These results also illustrate the critical importance of maintaining proper mitochondrial function in axons and neuromuscular junctions, supporting the emerging “dying-back” axonopathy model of ALS. In this review, we will discuss how mitochondrial dysfunction has been linked to the ALS variants of SOD1 and the mechanisms by which mitochondrial damage contributes to the disease etiology. PMID:19715760

  4. Miro1 deficiency in amyotrophic lateral sclerosis

    PubMed Central

    Zhang, Fan; Wang, Wenzhang; Siedlak, Sandra L.; Liu, Yingchao; Liu, Jun; Jiang, Keji; Perry, George; Zhu, Xiongwei; Wang, Xinglong

    2015-01-01

    Proper transportation of mitochondria to sites with high energy demands is critical for neuronal function and survival. Impaired mitochondrial movement has been repeatedly reported in motor neurons of amyotrophic lateral sclerosis (ALS) patients and indicated as an important mechanism contributing to motor neuron degeneration in ALS. Miro1, a RhoGTPase also referred to as Rhot1, is a key regulator of mitochondrial movement linking mitochondria and motor proteins. In this study, we investigated whether the expression of Miro1 was altered in ALS patients and ALS animal models. Immunoblot analysis revealed that Miro1 was significantly reduced in the spinal cord tissue of ALS patients. Consistently, the decreased expression of Miro1 was also noted only in the spinal cord, and not in the brain tissue of transgenic mice expressing ALS-associated SOD1 G93A or TDP-43 M337V. Glutamate excitotoxicity is one of the major pathophysiological mechanisms implicated in the pathogenesis of ALS, and we found that excessive glutamate challenge lead to significant reduction of Miro1 expression in spinal cord motor neurons both in vitro and in mice. Taken together, these findings show Miro1 deficiency in ALS patients and ALS animal models and suggest glutamate excitotoxicity as a likely cause of Miro1 deficiency. PMID:26074815

  5. Controversies and priorities in amyotrophic lateral sclerosis

    PubMed Central

    Turner, Martin R; Hardiman, Orla; Benatar, Michael; Brooks, Benjamin R; Chio, Adriano; de Carvalho, Mamede; Ince, Paul G; Lin, Cindy; Miller, Robert G; Mitsumoto, Hiroshi; Nicholson, Garth; Ravits, John; Shaw, Pamela J; Swash, Michael; Talbot, Kevin; Traynor, Bryan J; den Berg, Leonard H Van; Veldink, Jan H; Vucic, Steve; Kiernan, Matthew C

    2015-01-01

    Summary Two decades after the discovery that 20% of familial amyotrophic lateral sclerosis (ALS) cases were linked to mutations in the superoxide dismutase-1 (SOD1) gene, a substantial proportion of the remainder of cases of familial ALS have now been traced to an expansion of the intronic hexanucleotide repeat sequence in C9orf72. This breakthrough provides an opportunity to re-evaluate longstanding concepts regarding the cause and natural history of ALS, coming soon after the pathological unification of ALS with frontotemporal dementia through a shared pathological signature of cytoplasmic inclusions of the ubiquitinated protein TDP-43. However, with profound clinical, prognostic, neuropathological, and now genetic heterogeneity, the concept of ALS as one disease appears increasingly untenable. This background calls for the development of a more sophisticated taxonomy, and an appreciation of ALS as the breakdown of a wider network rather than a discrete vulnerable population of specialised motor neurons. Identification of C9orf72 repeat expansions in patients without a family history of ALS challenges the traditional division between familial and sporadic disease. By contrast, the 90% of apparently sporadic cases and incomplete penetrance of several genes linked to familial cases suggest that at least some forms of ALS arise from the interplay of multiple genes, poorly understood developmental, environmental, and age-related factors, as well as stochastic events. PMID:23415570

  6. Amyotrophic Lateral Sclerosis: New Perpectives and Update

    PubMed Central

    Orsini, Marco; Oliveira, Acary Bulle; Nascimento, Osvaldo J.M.; Reis, Carlos Henrique Melo; Leite, Marco Antonio Araujo; de Souza, Jano Alves; Pupe, Camila; de Souza, Olivia Gameiro; Bastos, Victor Hugo; de Freitas, Marcos R.G.; Teixeira, Silmar; Bruno, Carlos; Davidovich, Eduardo; Smidt, Benny

    2015-01-01

    Amyotrophic lateral sclerosis (ALS), Charcot’s disease or Lou Gehrig’s disease, is a term used to cover the spetrum of syndromes caracterized by progressive degeneration of motor neurons, a paralytic disorder caused by motor neuron degeneration. Currently, there are approximately 25,000 patients with ALS in the USA, with an average age of onset of 55 years. The incidence and prevalence of ALS are 1-2 and 4-6 per 100,000 each year, respectively, with a lifetime ALS risk of 1/600 to 1/1000. It causes progressive and cumulative physical disabilities, and leads to eventual death due to respiratory muscle failure. ALS is diverse in its presentation, course, and progression. We do not yet fully understand the causes of the disease, nor the mechanisms for its progression; thus, we lack effective means for treating this disease. In this chapter, we will discuss the diagnosis, treatment, and how to cope with impaired function and end of life based on of our experience, guidelines, and clinical trials. Nowadays ALS seems to be a more complex disease than it did two decades – or even one decade – ago, but new insights have been plentiful. Clinical trials should be seen more as experiments on pathogenic mechanisms. A medication or combination of medications that targets more than one pathogenic pathway may slow disease progression in an additive or synergistic fashion. PMID:26487927

  7. Altered cortical communication in amyotrophic lateral sclerosis.

    PubMed

    Blain-Moraes, Stefanie; Mashour, George A; Lee, Heonsoo; Huggins, Jane E; Lee, Uncheol

    2013-05-24

    Amyotrophic lateral sclerosis (ALS) is a disorder associated primarily with the degeneration of the motor system. More recently, functional connectivity studies have demonstrated potentially adaptive changes in ALS brain organization, but disease-related changes in cortical communication remain unknown. We recruited individuals with ALS and age-matched controls to operate a brain-computer interface while electroencephalography was recorded over three sessions. Using normalized symbolic transfer entropy, we measured directed functional connectivity from frontal to parietal (feedback connectivity) and parietal to frontal (feedforward connectivity) regions. Feedback connectivity was not significantly different between groups, but feedforward connectivity was significantly higher in individuals with ALS. This result was consistent across a broad electroencephalographic spectrum (4-35 Hz), and in theta, alpha and beta frequency bands. Feedback connectivity has been associated with conscious state and was found to be independent of ALS symptom severity in this study, which may have significant implications for the detection of consciousness in individuals with advanced ALS. We suggest that increases in feedforward connectivity represent a compensatory response to the ALS-related loss of input such that sensory stimuli have sufficient strength to cross the threshold necessary for conscious processing in the global neuronal workspace. PMID:23567743

  8. Lockhart Clarke's contribution to the description of amyotrophic lateral sclerosis.

    PubMed

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

    2010-11-01

    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

  9. 38 CFR 3.318 - Presumptive service connection for amyotrophic lateral sclerosis.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 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...

  10. 38 CFR 3.318 - Presumptive service connection for amyotrophic lateral sclerosis.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 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...

  11. 38 CFR 3.318 - Presumptive service connection for amyotrophic lateral sclerosis.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 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...

  12. 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...

  13. 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)

  14. General intravenous anesthesia for brachial plexus surgery in the rabbit.

    PubMed

    Reichert, P; Rutowski, R; Kielbowicz, Z; Kuryszko, J; Kielbowicz, M

    2013-01-01

    The rabbit is a good experimental model for brachial plexus surgery. The risks of death during anesthesia were significantly greater in rabbits than cats or dogs. This article presents the protocol of injectable anesthesia for a short surgical procedure, safe for the rabbit patient and convenient for the surgeon. PMID:24597314

  15. Proximal tibiofibular synostosis.

    PubMed

    Wong, K; Weiner, D S

    1978-09-01

    The occurrence of a proximal tibiofibular synostosis is indeed a rare condition with only 2 cases unassociated with other diseases reported to our knowledge to date. Two skeletally immature patients presented with a synostosis of the proximal tibiofibular region associated with shortening of the limb in the affected segments. Although the shortening and the synostosis seem interrelated no explanation of their relationship is evident from these 2 cases. PMID:709951

  16. Suicide among patients with amyotrophic lateral sclerosis.

    PubMed

    Fang, Fang; Valdimarsdóttir, Unnur; Fürst, Carl Johan; Hultman, Christina; Fall, Katja; Sparén, Pär; Ye, Weimin

    2008-10-01

    Studies on the suicide risk among patients with amyotrophic lateral sclerosis (ALS) in countries without legalized euthanasia or assisted suicide are important additions to data on the wish to die of these patients. We conducted a population-based cohort study in Sweden between 1965 and 2004, which comprised of 6,642 patients with incident ALS identified from the Swedish Inpatient Register. We calculated the standardized mortality ratios (SMRs) of suicide among the patients using the suicide rates of the general Swedish population as a reference. In total, 21 patients committed suicide during follow-up, compared to the predicted 3.6 suicides. Thus, we noted an almost 6-fold increased risk for suicide among ALS patients [SMR 5.8, 95% confidence interval (CI) 3.6-8.8]. Patients who committed suicide were, on average, around 7 years younger at the time of their first period of hospitalization than patients who did not commit suicide. The highest relative risk for suicide was observed within the first year after the patient's first period of hospitalization (SMR 11.2, 95% CI 5.8-19.6). After that, the relative risks decreased with time after hospitalization (P-value for trend = 0.006), but remained elevated 3 years later. The relative risks of suicide among ALS patients did not show a clear trend over time in contrast to the decreasing trend of relative risks for suicide among patients with cancer during the same period. Patients with ALS are at excess risk of suicide in Sweden and the relative risk is higher during the earlier stage of the disease. PMID:18669498

  17. Rodent Models of Amyotrophic Lateral Sclerosis

    PubMed Central

    Philips, Thomas; Rothstein, Jeffrey D.

    2015-01-01

    Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease affecting upper and lower motor neurons in the CNS. Patients with ALS develop extensive muscle wasting and atrophy leading to paralysis and death 3-5 years after disease onset. ALS may be familial (fALS 10%) or sporadic ALS (sALS, 90%). The large majority of fALS) cases are due to genetic mutations in the Superoxide dismutase 1 gene (SOD1, 15% of fALS) and repeat nucleotide expansions in the gene encoding C9ORF72 (around 40-50% of fALS and ~10% of sALS). From a wide range of pathological studies the general conclusion is that ALS disease is mediated through aberrant protein homeostasis (ie ER stress and autophagy) and/or changes in RNA processing (as seen in all non-SOD1-mediated ALS). In all of these cases, animal models suggest that the disease is mediated non-cell-autonomously, i.e. not only motor neurons are involved, but glial cells including microglia, astrocytes and oligodendrocytes and other neuronal subpopulations are also implicated in disease pathogenesis. This overview will give a chronological overview of a wide range of different ALS rodent models generated so far with a thorough description of their intrinsic advantages and disadvantages. We will focus on their respective correlation with disease as seen in humans and their potential for understanding basic disease biology. As RNA processing has more recently come to the foreground of ALS research, we will mainly focus on a thorough description of the most recently generated ALS rodent models. PMID:26344214

  18. PESTICIDE EXPOSURE AND AMYOTROPHIC LATERAL SCLEROSIS

    PubMed Central

    Kamel, Freya; Umbach, David M; Bedlack, Richard S; Richards, Marie; Watson, Mary; Alavanja, Michael CR; Blair, Aaron; Hoppin, Jane A; Schmidt, Silke; Sandler, Dale P

    2012-01-01

    Our objectives were to summarize literature on the association of amyotrophic lateral sclerosis (ALS) with pesticides as a group and to evaluate associations of ALS with specific pesticides. We conducted a meta-analysis of published studies of ALS and pesticides as a group and investigated the association of ALS with specific pesticides, using data from the Agricultural Health Study (AHS), a cohort including 84,739 private pesticide applicators and spouses. AHS participants provided information on pesticide use at enrollment in 1993-1997. In mortality data collected through February, 2010, ALS was recorded on death certificates of 41 individuals whom we compared to the remaining cohort (controls), using unconditional logistic regression adjusted for age and gender to calculate odds ratios (ORs) and 95% confidence intervals. In the meta-analysis, ALS was associated with use of pesticides as a group (1.9, 1.1-3.1). In the AHS, ALS was not associated with pesticides as a group, but was associated with use of organochlorine insecticides (OCs) (1.6, 0.8-3.5), pyrethroids (1.4, 0.6-3.4), herbicides (1.6, 0.7-3.7), and fumigants (1.8, 0.8-3.9). ORs were elevated for ever use of the specific OCs aldrin (2.1, 0.8-5.1), dieldrin (2.6, 0.9-7.3), DDT (2.1, 0.9-5.0), and toxaphene (2.0, 0.8-4.9). None of these associations was statistically significant. Similar results were observed in an analysis restricted to men. In conclusion, the meta-analysis suggests that ALS risk is associated with use of pesticides as a group, and our analysis of AHS data points to OC use in particular. The latter results are novel but based on a small number of cases and require replication in other populations. PMID:22521219

  19. Unusual Origin of a Double Upper Subscapular Nerve from the Suprascapular Nerve and the Posterior Division of the Upper Trunk of the Brachial Plexus: A Case Report

    PubMed Central

    Koutsouflianiotis, Konstantinos; Iliou, Kalliopi; Bitsis, Theodosios; Kitsoulis, Panagiotis

    2016-01-01

    A double upper subscapular nerve on the right side was detected in a male cadaver, with the proximal one arising from the suprascapular nerve and the distal one from the posterior division of the upper trunk of the brachial plexus. Both of them penetrated and supplied the uppermost portion of the right subscapularis muscle. That anatomic variation was associated with a median nerve formed by two lateral roots. The origin and pattern of the upper subscapular nerve displays high variability, however the presented combination of the variable origin of a double upper subscapular nerve has rarely been described in the literature. The knowledge of such an anatomic variation is essential for the surgeon operating in the region especially in instances of brachial plexus’ repair after any traumatic injury. Moreover, the awareness of the precise origin and topography of these nerves is important for the physician attempting to block these nerves or utilizing these nerves as grafts for neurotization of adjacent damaged nerves of the brachial plexus. PMID:27504272

  20. Unusual Origin of a Double Upper Subscapular Nerve from the Suprascapular Nerve and the Posterior Division of the Upper Trunk of the Brachial Plexus: A Case Report.

    PubMed

    Paraskevas, George; Koutsouflianiotis, Konstantinos; Iliou, Kalliopi; Bitsis, Theodosios; Kitsoulis, Panagiotis

    2016-06-01

    A double upper subscapular nerve on the right side was detected in a male cadaver, with the proximal one arising from the suprascapular nerve and the distal one from the posterior division of the upper trunk of the brachial plexus. Both of them penetrated and supplied the uppermost portion of the right subscapularis muscle. That anatomic variation was associated with a median nerve formed by two lateral roots. The origin and pattern of the upper subscapular nerve displays high variability, however the presented combination of the variable origin of a double upper subscapular nerve has rarely been described in the literature. The knowledge of such an anatomic variation is essential for the surgeon operating in the region especially in instances of brachial plexus' repair after any traumatic injury. Moreover, the awareness of the precise origin and topography of these nerves is important for the physician attempting to block these nerves or utilizing these nerves as grafts for neurotization of adjacent damaged nerves of the brachial plexus. PMID:27504272

  1. 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.

  2. 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.

  3. Unusual proximal tibiofibular synostosis.

    PubMed

    Takai, S; Yoshino, N; Hirasawa, Y

    1999-01-01

    Proximal tibiofibular synostosis without multiple hereditary exostosis is extremely rare and only 7 cases have been reported in the literature. All of the previously reported cases accompanied deformities such as distal positioning of the proximal tibiofibular joint, leg length discrepancy, bowing of the fibula, and valgus deformity of the knee. The present case of a 24-year-old man had neither a history of trauma nor deformity around the knee. Therefore, it was suggested that this type of synostosis occurred after epiphyseal plate closure. PMID:10741527

  4. Close proximity gunshot residues.

    PubMed

    Thornton, J I

    1986-04-01

    Intuitively, a hand held in close proximity to a firearm at the instant of discharge will intercept a significant amount of gunshot residue, even though the hand did not actually come into contact with the weapon. There is, however, little information specifically described in the forensic science literature concerning the residue levels which might be encountered in such an instance. The present work confirms that antimony levels consistent with an individual having fired or handled a firearm may be intercepted by a hand held in close proximity. PMID:3711843

  5. Transplantation of human amniotic epithelial cells repairs brachial plexus injury: pathological and biomechanical analyses

    PubMed Central

    Yang, Qi; Luo, Min; Li, Peng; Jin, Hai

    2014-01-01

    A brachial plexus injury model was established in rabbits by stretching the C6 nerve root. Immediately after the stretching, a suspension of human amniotic epithelial cells was injected into the injured brachial plexus. The results of tensile mechanical testing of the brachial plexus showed that the tensile elastic limit strain, elastic limit stress, maximum stress, and maximum strain of the injured brachial plexuses were significantly increased at 24 weeks after the injection. The treatment clearly improved the pathological morphology of the injured brachial plexus nerve, as seen by hematoxylin eosin staining, and the functions of the rabbit forepaw were restored. These data indicate that the injection of human amniotic epithelial cells contributed to the repair of brachial plexus injury, and that this technique may transform into current clinical treatment strategies. PMID:25657737

  6. 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…

  7. 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…

  8. 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…

  9. Chapter 15 Juvenile amyotrophic lateral sclerosis.

    PubMed

    Orban, Paul; Devon, Rebecca S; Hayden, Michael R; Leavitt, Blair R

    2007-01-01

    Several forms of genetically defined juvenile amy-otrophic lateral sclerosis (ALS) have now been charac-terized and discussion of these conditions will form the basis for this chapter. ALS2 is an autosomal recessive form of ALS with a juvenile onset and very slow progression that mapped to chromosome 2q33. Nine different mutations have been identified in the ALS2 gene that result in premature stop codons, suggesting a loss of function in the gene product, alsin. The alsin protein is thought to function as a guanine-nucleotide exchange factor for GTPases and may play a role in vesicle transport or membrane trafficking processes. ALS4 is an autosomal dominant form of juvenile onset ALS associated with slow progression, severe muscle weakness and pyramidal signs, in the absence of bulbar and sensory abnormalities. Mutations in the SETX gene cause ALS4, and the SETX gene product senataxin may have DNA and RNA helicase activity and play a role in the regulation of RNA and/or DNA in the cell. A third form of juvenile-onset ALS (ALS5) is associated with slowly progressing lower motor neuron signs (weak-ness and atrophy) initially of the hands and feet, with eventual bulbar involvement. Progressive upper motor neuron disease becomes more obvious with time. ALS5 has been linked to a 6 cM region of chromosome 15q15.1-q21.1, but the causative gene mutation for ALS5 has yet to be identified. The high degree of clin-ical and genetic heterogeneity in the various forms of juvenile ALS can make differential diagnosis difficult, other genetic disorders that must be considered include: spinal muscular atrophy, hereditary spastic paraplegia, SBMA, GM2 gangliosidosis and the hereditary motor neuronopathies/motor forms of Charcot-Marie-Tooth disease. Acquired disorders that must also be consid-ered include heavy metal intoxications (especially lead), multifocal motor neuropathy, paraneoplastic syndromes, vitamin deficiencies (B12) and infections (HTLV-II, HIV and poliomyelitis). PMID

  10. XQL and Proximal Nodes.

    ERIC Educational Resources Information Center

    Baeza-Yates, Ricardo; Navarro, Gonzalo

    2002-01-01

    Discussion of models that have been developed to structure text documents for information retrieval focuses on XML and its proposed query language XQL. Considers efficiency of the query engine and shows that an already existing model, Proximal Nodes, can be used as an efficient query engine behind an XQL front-end. (Author/LRW)

  11. Proximal tibiofibular synostosis.

    PubMed

    Gamble, J G

    1984-03-01

    A case of proximal tibiofibular synostosis with a 10-year follow-up is presented. The lesion was documented roentgenographically when the patient was 3 years of age and when she became symptomatic at 13 years of age after vigorous running. The symptoms were successfully treated with custom-molded shoe orthotics. PMID:6699166

  12. Proximate Analysis of Coal

    ERIC Educational Resources Information Center

    Donahue, Craig J.; Rais, Elizabeth A.

    2009-01-01

    This lab experiment illustrates the use of thermogravimetric analysis (TGA) to perform proximate analysis on a series of coal samples of different rank. Peat and coke are also examined. A total of four exercises are described. These are dry exercises as students interpret previously recorded scans. The weight percent moisture, volatile matter,…

  13. Steerable Capacitive Proximity Sensor

    NASA Technical Reports Server (NTRS)

    Jenstrom, Del T.; Mcconnell, Robert L.

    1994-01-01

    Steerable capacitive proximity sensor of "capaciflector" type based partly on sensing units described in GSC-13377 and GSC-13475. Position of maximum sensitivity adjusted without moving sensor. Voltage of each driven shield adjusted separately to concentrate sensing electric field more toward one side or other.

  14. Idiopathic brachial neuritis in a child: A case report and review of the literature

    PubMed Central

    Jain, Shikha; Bhatt, Girish Chandra; Rai, Nirendra; Bhan, Bhavna Dhingra

    2014-01-01

    Brachial neuritis is a rare disease in children, affecting mainly the lower motor neurons of the brachial plexus and/or individual nerves or nerve branches. We report a case of idiopathic brachial plexus neuritis in a 2½-year-old female child admitted with acute respiratory distress and given antibiotic therapy following which she developed weakness of the left hand. She was diagnosed as a case of idiopathic brachial plexus neuritis and was given supportive care. Although, the association with antibiotic therapy in this case could be incidental, indeed it is intriguing and requires further studies. PMID:25624937

  15. Idiopathic brachial neuritis in a child: A case report and review of the literature.

    PubMed

    Jain, Shikha; Bhatt, Girish Chandra; Rai, Nirendra; Bhan, Bhavna Dhingra

    2014-01-01

    Brachial neuritis is a rare disease in children, affecting mainly the lower motor neurons of the brachial plexus and/or individual nerves or nerve branches. We report a case of idiopathic brachial plexus neuritis in a 2½-year-old female child admitted with acute respiratory distress and given antibiotic therapy following which she developed weakness of the left hand. She was diagnosed as a case of idiopathic brachial plexus neuritis and was given supportive care. Although, the association with antibiotic therapy in this case could be incidental, indeed it is intriguing and requires further studies. PMID:25624937

  16. Post-operative brachial plexus neuropraxia: A less recognised complication of combined plastic and laparoscopic surgeries

    PubMed Central

    Thomas, Jimmy

    2014-01-01

    This presentation is to increase awareness of the potential for brachial plexus injury during prolonged combined plastic surgery procedures. A case of brachial plexus neuropraxia in a 26-year-old obese patient following a prolonged combined plastic surgery procedure was encountered. Nerve palsy due to faulty positioning on the operating table is commonly seen over the elbow and popliteal fossa. However, injury to the brachial plexus has been a recently reported phenomenon due to the increasing number of laparoscopic and robotic procedures. Brachial plexus injury needs to be recognised as a potential complication of prolonged combined plastic surgery. Preventive measures are discussed. PMID:25593443

  17. Continuous shoulder analgesia via an indwelling axillary brachial plexus catheter.

    PubMed

    Reuben, S S; Steinberg, R B

    2000-09-01

    Continuous interscalene brachial plexus blockade can provide anesthesia and analgesia in the shoulder region. Difficulty accessing the interscalene space and premature displacement of interscalene catheters may preclude their use in certain situations. We present two case reports in which a catheter was advanced from the axilla along the brachial plexus sheath to the interscalene space to provide continuous cervicobrachial plexus analgesia. In the first case report, previous neck surgery made the anatomic landmarks for performing an interscalene block very difficult. An epidural catheter was advanced from the axillary brachial plexus sheath to the interscalene space under fluoroscopic guidance. This technique provided both intraoperative analgesia for shoulder surgery as well as 24-hour postoperative analgesia by an infusion of 0.125% bupivacaine. In the second case report, a catheter was inserted in a similar fashion from the axillary to the interscalene space to provide 14 days of continuous analgesia in the management of complex regional pain syndrome. We have found that this technique allows us to secure the catheter more easily than with the traditional interscalene approach and thus prevents premature dislodgment. This approach may be a suitable alternative when either an interscalene or an infraclavicular catheter may not be inserted. PMID:11090734

  18. 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

  19. Associations and clinical relevance of aortic-brachial artery stiffness mismatch, aortic reservoir function, and central pressure augmentation

    PubMed Central

    Schultz, Martin G.; Hughes, Alun D.; Davies, Justin E.; Sharman, James E.

    2015-01-01

    Central augmentation pressure (AP) and index (AIx) predict cardiovascular events and mortality, but underlying physiological mechanisms remain disputed. While traditionally believed to relate to wave reflections arising from proximal arterial impedance (and stiffness) mismatching, recent evidence suggests aortic reservoir function may be a more dominant contributor to AP and AIx. Our aim was therefore to determine relationships among aortic-brachial stiffness mismatching, AP, AIx, aortic reservoir function, and end-organ disease. Aortic (aPWV) and brachial (bPWV) pulse wave velocity were measured in 359 individuals (aged 61 ± 9, 49% male). Central AP, AIx, and aortic reservoir indexes were derived from radial tonometry. Participants were stratified by positive (bPWV > aPWV), negligible (bPWV ≈ aPWV), or negative stiffness mismatch (bPWV < aPWV). Left-ventricular mass index (LVMI) was measured by two-dimensional-echocardiography. Central AP and AIx were higher with negative stiffness mismatch vs. negligible or positive stiffness mismatch (11 ± 6 vs. 10 ± 6 vs. 8 ± 6 mmHg, P < 0.001 and 24 ± 10 vs. 24 ± 11 vs. 21 ± 13%, P = 0.042). Stiffness mismatch (bPWV -aPWV) was negatively associated with AP (r = −0.18, P = 0.001) but not AIx (r = −0.06, P = 0.27). Aortic reservoir pressure strongly correlated to AP (r = 0.81, P < 0.001) and AIx (r = 0.62, P < 0.001) independent of age, sex, heart rate, mean arterial pressure, and height (standardized β = 0.61 and 0.12, P ≤ 0.001). Aortic reservoir pressure independently predicted abnormal LVMI (β = 0.13, P = 0.024). Positive aortic-brachial stiffness mismatch does not result in higher AP or AIx. Aortic reservoir function, rather than discrete wave reflection from proximal arterial stiffness mismatching, provides a better model description of AP and AIx and also has clinical relevance as evidenced by an independent association of aortic reservoir pressure with LVMI. PMID:26276816

  20. Associations and clinical relevance of aortic-brachial artery stiffness mismatch, aortic reservoir function, and central pressure augmentation.

    PubMed

    Schultz, Martin G; Hughes, Alun D; Davies, Justin E; Sharman, James E

    2015-10-01

    Central augmentation pressure (AP) and index (AIx) predict cardiovascular events and mortality, but underlying physiological mechanisms remain disputed. While traditionally believed to relate to wave reflections arising from proximal arterial impedance (and stiffness) mismatching, recent evidence suggests aortic reservoir function may be a more dominant contributor to AP and AIx. Our aim was therefore to determine relationships among aortic-brachial stiffness mismatching, AP, AIx, aortic reservoir function, and end-organ disease. Aortic (aPWV) and brachial (bPWV) pulse wave velocity were measured in 359 individuals (aged 61 ± 9, 49% male). Central AP, AIx, and aortic reservoir indexes were derived from radial tonometry. Participants were stratified by positive (bPWV > aPWV), negligible (bPWV ≈ aPWV), or negative stiffness mismatch (bPWV < aPWV). Left-ventricular mass index (LVMI) was measured by two-dimensional-echocardiography. Central AP and AIx were higher with negative stiffness mismatch vs. negligible or positive stiffness mismatch (11 ± 6 vs. 10 ± 6 vs. 8 ± 6 mmHg, P < 0.001 and 24 ± 10 vs. 24 ± 11 vs. 21 ± 13%, P = 0.042). Stiffness mismatch (bPWV-aPWV) was negatively associated with AP (r = -0.18, P = 0.001) but not AIx (r = -0.06, P = 0.27). Aortic reservoir pressure strongly correlated to AP (r = 0.81, P < 0.001) and AIx (r = 0.62, P < 0.001) independent of age, sex, heart rate, mean arterial pressure, and height (standardized β = 0.61 and 0.12, P ≤ 0.001). Aortic reservoir pressure independently predicted abnormal LVMI (β = 0.13, P = 0.024). Positive aortic-brachial stiffness mismatch does not result in higher AP or AIx. Aortic reservoir function, rather than discrete wave reflection from proximal arterial stiffness mismatching, provides a better model description of AP and AIx and also has clinical relevance as evidenced by an independent association of aortic reservoir pressure with LVMI. PMID:26276816

  1. Proximity Networks and Epidemics

    NASA Astrophysics Data System (ADS)

    Guclu, Hasan; Toroczkai, Zoltán

    2007-03-01

    We presented the basis of a framework to account for the dynamics of contacts in epidemic processes, through the notion of dynamic proximity graphs. By varying the integration time-parameter T, which is the period of infectivity one can give a simple account for some of the differences in the observed contact networks for different diseases, such as smallpox, or AIDS. Our simplistic model also seems to shed some light on the shape of the degree distribution of the measured people-people contact network from the EPISIM data. We certainly do not claim that the simplistic graph integration model above is a good model for dynamic contact graphs. It only contains the essential ingredients for such processes to produce a qualitative agreement with some observations. We expect that further refinements and extensions to this picture, in particular deriving the link-probabilities in the dynamic proximity graph from more realistic contact dynamics should improve the agreement between models and data.

  2. Proximal Point Methods Revisited

    NASA Astrophysics Data System (ADS)

    Boikanyo, Oganeditse A.; Moroşanu, Gheorghe

    2011-09-01

    The proximal point methods have been widely used in the last decades to approximate the solutions of nonlinear equations associated with monotone operators. Inspired by the iterative procedure defined by B. Martinet (1970), R.T. Rockafellar introduced in 1976 the so-called proximal point algorithm (PPA) for a general maximal monotone operator. The sequence generated by this iterative method is weakly convergent under appropriate conditions, but not necessarily strongly convergent, as proved by O. Güler (1991). This fact explains the introduction of different modified versions of the PPA which generate strongly convergent sequences under appropriate conditions, including the contraction-PPA defined by H.K. Xu in 2002. Here we discuss Xu's modified PPA as well as some of its generalizations. Special attention is paid to the computational errors, in particular the original Rockafellar summability assumption is replaced by the condition that the error sequence converges to zero strongly.

  3. 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. PMID:26984092

  4. Distributed proximity sensor system

    NASA Technical Reports Server (NTRS)

    Lee, Sukhan (Inventor)

    1988-01-01

    The invention relates to sensors embedded on the surface of a robot hand, or other moving member. By distributing proximity sensors capable of detecting distances and angles to points on the surface of an object, information is obtained for achieving noncontacting shape and distance perception, i.e., for automatic determination of the object's shape, direction, and distance, as well as the orientation of the object relative to the robot hand or other moving member.

  5. Natural compounds used as therapies targeting to amyotrophic lateral sclerosis.

    PubMed

    Nabavi, Seyed F; Daglia, Maria; D'Antona, Giuseppe; Sobarzo-Sánchez, Eduardo; Talas, Zeliha S; Nabavi, Seyed M

    2015-01-01

    Amyotrophic lateral sclerosis (ALS) is a neuromuscular disease that occurs throughout the world with no racial, ethnic or socioeconomic boundaries. Despite its high morbidity and mortality, there are limited medications available for ALS that may increase survival in patients with amyotrophic lateral sclerosis by approximately 2-3 months. Inasmuch as negative effects of riluzole on muscle atrophy and wasting, weakness, muscle spasticity, dysarthria, dysphagia, and overall patient quality of life and its different adverse effects, much attention has been paid to natural products and herbal medicines. Overall scientific reports indicate that natural products have beneficial effects on patients with ALS low side effects and multiple targets. In the present paper, we review the scientific reports on beneficial role of natural polyphenolic compounds in treatment of ALS. PMID:25601606

  6. 3T MR tomography of the brachial plexus: structural and microstructural evaluation.

    PubMed

    Mallouhi, Ammar; Marik, Wolfgang; Prayer, Daniela; Kainberger, Franz; Bodner, Gerd; Kasprian, Gregor

    2012-09-01

    Magnetic resonance (MR) neurography comprises an evolving group of techniques with the potential to allow optimal noninvasive evaluation of many abnormalities of the brachial plexus. MR neurography is clinically useful in the evaluation of suspected brachial plexus traumatic injuries, intrinsic and extrinsic tumors, and post-radiogenic inflammation, and can be particularly beneficial in pediatric patients with obstetric trauma to the brachial plexus. The most common MR neurographic techniques for displaying the brachial plexus can be divided into two categories: structural MR neurography; and microstructural MR neurography. Structural MR neurography uses mainly the STIR sequence to image the nerves of the brachial plexus, can be performed in 2D or 3D mode, and the 2D sequence can be repeated in different planes. Microstructural MR neurography depends on the diffusion tensor imaging that provides quantitative information about the degree and direction of water diffusion within the nerves of the brachial plexus, as well as on tractography to visualize the white matter tracts and to characterize their integrity. The successful evaluation of the brachial plexus requires the implementation of appropriate techniques and familiarity with the pathologies that might involve the brachial plexus. PMID:21763092

  7. Penile erectile dysfunction after brachial plexus root avulsion injury in rats

    PubMed Central

    Fu, Guo; Qin, Bengang; Jiang, Li; Huang, Xijun; Lu, Qinsen; Zhang, Dechun; Liu, Xiaolin; Zhu, Jiakai; Zheng, Jianwen; Li, Xuejia; Gu, Liqiang

    2014-01-01

    Our previous studies have demonstrated that some male patients suffering from brachial plexus injury, particularly brachial plexus root avulsion, show erectile dysfunction to varying degrees. However, the underlying mechanism remains poorly understood. In this study, we evaluated the erectile function after establishing brachial plexus root avulsion models with or without spinal cord injury in rats. After these models were established, we administered apomorphine (via a subcutaneous injection in the neck) to observe changes in erectile function. Rats subjected to simple brachial plexus root avulsion or those subjected to brachial plexus root avulsion combined with spinal cord injury had significantly fewer erections than those subjected to the sham operation. Expression of neuronal nitric oxide synthase did not change in brachial plexus root avulsion rats. However, neuronal nitric oxide synthase expression was significantly decreased in brachial plexus root avulsion + spinal cord injury rats. These findings suggest that a decrease in neuronal nitric oxide synthase expression in the penis may play a role in erectile dysfunction caused by the combination of brachial plexus root avulsion and spinal cord injury. PMID:25422647

  8. 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. PMID:26860655

  9. Brachial Plexus Involvement of Myeloid Sarcoma Detected by Reconstruction Magnetic Resonance Neurography.

    PubMed

    Sugiyama, Atsuhiko; Ito, Shoichi; Sugita, Yasumasa; Shimada, Jun-Ichiro; Takeuchi, Masahiro; Hirano, Shigeki; Kuwabara, Satoshi

    2015-01-01

    Myeloid sarcoma is a rare hematological disorder that presents as an extramedullary mass of immature myeloid precursors. We herein present the case of a 57-year-old man with a seven-month history of progressive weakness in the right upper extremity. Reconstruction magnetic resonance neurography showed a marked enlargement of the right brachial plexus. Fluorodeoxyglucose positron emission tomography revealed a radioactive lesion in the sacrum, in addition to the right brachial plexus, and a biopsy of the sacrum revealed myeloid sarcoma. The brachial plexus lesion was also regarded as myeloid sarcoma because of the treatment response. Isolated myeloid sarcoma involving the brachial plexus is very rare and its diagnosis is difficult as there was neither a history of leukemia nor bone marrow involvement in this patient. In this case, reconstructed magnetic resonance neurography was useful for detecting the brachial plexus mass lesion which led to an early diagnosis and good recovery. PMID:26328656

  10. Obesity in children with brachial plexus birth palsy.

    PubMed

    Singh, Avreeta K; Mills, Janith; Bauer, Andrea S; Ezaki, Marybeth

    2015-11-01

    Fetal macrosomia is associated with a 14-fold increased risk of brachial plexus birth palsy (BPBP), and is a predictor of childhood obesity. The purpose of this study was to identify the relationships between BPBP, fetal macrosomia, and childhood obesity. We retrospectively reviewed 214 children with BPBP. The average age was 8 years and 53% had a Narakas 1 grade BPBP. Overall, 49% of children were normal weight, 22% overweight, and 29% obese. Of the children with a history of fetal macrosomia, 41% were obese; a statistically significant difference. Overall quality of life scores, however, were not correlated with obesity. PMID:26163865

  11. 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.

  12. Boston Children's Hospital approach to brachial plexus birth palsy.

    PubMed

    Vuillermin, Carley; Bauer, Andrea S

    2016-07-01

    The treatment of infants with brachial plexus birth palsy (BPBP) continues to be a focus at Boston Children's Hospital. Over the last 15 years, there have been many developments in the treatment of infants with BPBP. Some of the greatest changes have emerged through technical advances such as the advent of distal nerve transfers to allow targeted reinnervation as well as through research to understand the pathoanatomical changes that lead to glenohumeral dysplasia and how this dysplasia can be remodeled. This review will discuss our current practice of evaluation of the infant with BPBP, techniques for microsurgical reconstruction, and prevention and treatment of secondary glenohumeral dysplasia. PMID:27137763

  13. Proximity networks and epidemics

    NASA Astrophysics Data System (ADS)

    Toroczkai, Zoltán; Guclu, Hasan

    2007-05-01

    Disease spread in most biological populations requires the proximity of agents. In populations where the individuals have spatial mobility, the contact graph is generated by the “collision dynamics” of the agents, and thus the evolution of epidemics couples directly to the spatial dynamics of the population. We first briefly review the properties and the methodology of an agent-based simulation (EPISIMS) to model disease spread in realistic urban dynamic contact networks. Using the data generated by this simulation, we introduce the notion of dynamic proximity networks which takes into account the relevant time-scales for disease spread: contact duration, infectivity period, and rate of contact creation. This approach promises to be a good candidate for a unified treatment of epidemic types that are driven by agent collision dynamics. In particular, using a simple model, we show that it can account for the observed qualitative differences between the degree distributions of contact graphs of diseases with short infectivity period (such as air-transmitted diseases) or long infectivity periods (such as HIV).

  14. Echosonography with proximity sensors

    NASA Astrophysics Data System (ADS)

    Thaisiam, W.; Laithong, T.; Meekhun, S.; Chaiwathyothin, N.; Thanlarp, P.; Danworaphong, S.

    2013-03-01

    We propose the use of a commercial ultrasonic proximity sensor kit for profiling an altitude-varying surface by employing echosonography. The proximity sensor kit, two identical transducers together with its dedicated operating circuit, is used as a profiler for the construction of an image. Ultrasonic pulses are emitted from one of the transducers and received by the other. The time duration between the pulses allows us to determine the traveling distance of each pulse. In the experiment, the circuit is used with the addition of two copper wires for directing the outgoing and incoming signals to an oscilloscope. The time of flight of ultrasonic pulses can thus be determined. Square grids of 5 × 5 cm2 are made from fishing lines, forming pixels in the image. The grids are designed to hold the detection unit in place, about 30 cm above a flat surface. The surface to be imaged is constructed to be height varying and placed on the flat surface underneath the grids. Our result shows that an image of the profiled surface can be created by varying the location of the detection unit along the grid. We also investigate the deviation in relation to the time of flight of the ultrasonic pulse. Such an experiment should be valuable for conveying the concept of ultrasonic imaging to physical and medical science undergraduate students. Due to its simplicity, the setup could be made in any undergraduate laboratory relatively inexpensively and it requires no complex parts. The results illustrate the concept of echosonography.

  15. Injury mechanisms in supraclavicular stretch injuries of the brachial plexus.

    PubMed

    Soldado, Francisco; Ghizoni, Marcos F; Bertelli, Jayme

    2016-02-01

    The aim of this study was to describe the mechanisms involved in stretch injuries of the brachial plexus. One hundred and fifty consecutive patients with supraclavicular brachial plexus injuries (BPI) were asked about the mechanism of injury during the actual injury event, particularly about the type of trauma to their shoulder, shoulder girdle and head. Fifty-seven of the patients provided enough information about their accident to allow for analysis of the shoulder trauma. The injury mechanism for all patients having upper root or total palsy (n=46) was described as a direct vertical impact to the shoulder. In 44 of these patients, the trauma followed a motorcycle accident and, in most of them, the patient hit a fixed vertical structure before falling to the ground. The injury mechanism for the lower root palsy cases (n=11) was variable. The most frequent mechanism was forceful anterior shoulder compression by a car seat belt. We found that injury mechanisms differed significantly from the ones commonly discussed in published studies. PMID:27117025

  16. 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

  17. Traumatic Pseudoaneurysm of Axillary Artery Combined with Brachial Plexus Injury

    PubMed Central

    Chen, Lin; Peng, Feng; Wang, Tao; Chen, Desong; Yang, Jianyun

    2014-01-01

    Traumatic pseudoaneurysm of the axillary artery combined with brachial plexus injury is extremely rare. The factors that influence the symptoms and functional recovery related to this condition are unclear. Nine patients who had sustained this trauma were surgically treated at our unit between June 1999 and November 2010. The cause of trauma, symptoms, signs and examinations of neurological and vascular deficits, and the surgical findings of the involved nerves and vessels were recorded in detail. The functional recovery of vessels and nerves, as well as the extent of pain, were evaluated, respectively. The average length of patient follow-up was 4.5 years (range, 24 months to 11.3 years). After vessel repair, whether by endovascular or operative treatment, the distending, constant, and pulsating pain was relieved in all patients. Furthermore, examination of the radial artery pulse on the repaired side appeared normal at last follow-up. All patients showed satisfactory sensory recovery, with motor recovery rated as good in five patients and fair in four patients. The symptom characteristics varied with the location of the damage to the axillary artery. Ultrasound examination and computed tomography angiography are useful to evaluate vascular injury and provide valuable information for operative planning. Surgical exploration is an effective therapy with results related to the nerve injury condition of the brachial plexus. PMID:25412426

  18. Effect of Collateral Sprouting on Donor Nerve Function After Nerve Coaptation: A Study of the Brachial Plexus

    PubMed Central

    Reichert, Paweł; Kiełbowicz, Zdzisław; Dzięgiel, Piotr; Puła, Bartosz; Wrzosek, Marcin; Bocheńska, Aneta; Gosk, Jerzy

    2016-01-01

    Background The aim of the present study was to evaluate the donor nerve from the C7 spinal nerve of the rabbit brachial plexus after a coaptation procedure. Assessment was performed of avulsion of the C5 and C6 spinal nerves treated by coaptation of these nerves to the C7 spinal nerve. Material/Methods After nerve injury, fourteen rabbits were treated by end-to-side coaptation (ETS), and fourteen animals were treated by side-to-side coaptation (STS) on the right brachial plexus. Electrophysiological and histomorphometric analyses and the skin pinch test were used to evaluate the outcomes. Results There was no statistically significant difference in the G-ratio proximal and distal to the coaptation in the ETS group, but the differences in the axon, myelin sheath and fiber diameters were statistically significant. The comparison of the ETS and STS groups distal to the coaptation with the controls demonstrated statistically significant differences in the fiber, axon, and myelin sheath diameters. With respect to the G-ratio, the ETS group exhibited no significant differences relative to the control, whereas the G-ratio in the STS group and the controls differed significantly. In the electrophysiological study, the ETS and STS groups exhibited major changes in the biceps and subscapularis muscles. Conclusions The coaptation procedure affects the histological structure of the nerve donor, but it does not translate into changes in nerve conduction or the sensory function of the limb. The donor nerve lesion in the ETS group is transient and has minimal clinical relevance. PMID:26848925

  19. Effect of Collateral Sprouting on Donor Nerve Function After Nerve Coaptation: A Study of the Brachial Plexus.

    PubMed

    Reichert, Pawel; Kiełbowicz, Zdzisław; Dzięgiel, Piotr; Puła, Bartosz; Wrzosek, Marcin; Bocheńska, Aneta; Gosk, Jerzy

    2016-01-01

    BACKGROUND The aim of the present study was to evaluate the donor nerve from the C7 spinal nerve of the rabbit brachial plexus after a coaptation procedure. Assessment was performed of avulsion of the C5 and C6 spinal nerves treated by coaptation of these nerves to the C7 spinal nerve. MATERIAL AND METHODS After nerve injury, fourteen rabbits were treated by end-to-side coaptation (ETS), and fourteen animals were treated by side-to-side coaptation (STS) on the right brachial plexus. Electrophysiological and histomorphometric analyses and the skin pinch test were used to evaluate the outcomes. RESULTS There was no statistically significant difference in the G-ratio proximal and distal to the coaptation in the ETS group, but the differences in the axon, myelin sheath and fiber diameters were statistically significant. The comparison of the ETS and STS groups distal to the coaptation with the controls demonstrated statistically significant differences in the fiber, axon, and myelin sheath diameters. With respect to the G-ratio, the ETS group exhibited no significant differences relative to the control, whereas the G-ratio in the STS group and the controls differed significantly. In the electrophysiological study, the ETS and STS groups exhibited major changes in the biceps and subscapularis muscles. CONCLUSIONS The coaptation procedure affects the histological structure of the nerve donor, but it does not translate into changes in nerve conduction or the sensory function of the limb. The donor nerve lesion in the ETS group is transient and has minimal clinical relevance. PMID:26848925

  20. Complications of Lower-Extremity Outpatient Arteriography via Low Brachial Artery

    SciTech Connect

    Chatziioannou, A.; Ladopoulos, C.; Mourikis, D.; Katsenis, K.; Spanomihos, G.; Vlachos, L.

    2004-01-15

    We retrospectively evaluated low brachial artery puncture for arteriography and its complications as an alternative approach route for bilateral lower extremity run-off. Using the Seldinger technique and catheterization with a sheathless 4-F multiple side-hole pigtail catheter, we performed 2250 low brachial artery punctures in outpatients.The right brachial artery (RBA) was successfully punctured in 2039 patients; the left brachial artery (LBA) in 200. The transfemoral approach was used in 11 patients when catheterizing either of brachial arteries failed. Ten major or moderate complications (2 pseudoaneurysms, 2 thrombosis, 1 dissection and 5 hematomas) were encountered. Surgical intervention was necessary in three cases. There were no transient ischemic attacks. Twenty-one patients suffered temporary loss of radial pulse which returned spontaneously in less than 1 hour. One patient demonstrated prolonged loss of pulse which required heparin. Low brachial artery puncture and catheterization at the antecubital fossa is a very safe and cost-effective alternative to the femoral artery approach for lower extremity intra-arterial arteriography in the hands of experienced operators. The success rate in catheterizing one of the brachial arteries was 99.52% with a low significant complications rate of 0.44%. The transbrachial approach should be used as a standard method for lower extremity IA - DSA in an outpatient setting.

  1. Some Properties of Fuzzy Soft Proximity Spaces

    PubMed Central

    Demir, İzzettin; Özbakır, Oya Bedre

    2015-01-01

    We study the fuzzy soft proximity spaces in Katsaras's sense. First, we show how a fuzzy soft topology is derived from a fuzzy soft proximity. Also, we define the notion of fuzzy soft δ-neighborhood in the fuzzy soft proximity space which offers an alternative approach to the study of fuzzy soft proximity spaces. Later, we obtain the initial fuzzy soft proximity determined by a family of fuzzy soft proximities. Finally, we investigate relationship between fuzzy soft proximities and proximities. PMID:25793224

  2. 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.

  3. [Ipsilateral brachial plexus C7 root transfer. Presentation of a case and a literature review].

    PubMed

    Vergara-Amador, Enrique; Ramírez, Alejandro

    2014-01-01

    The C7 root in brachial plexus injuries has been used since 1986, since the first description by Gu at that time. This root can be used completely or partially in ipsilateral or contralateral lesions of the brachial plexus. A review of the literature and the case report of a 21-month-old girl with stab wounds to the neck and section of the C5 root of the right brachial plexus are presented. A transfer of the anterior fibres of the ipsilateral C7 root was performed. At 9 months there was complete recovery of abduction and external rotation of the shoulder. PMID:23474130

  4. Frontotemporal Dysfunction and Dementia in Amyotrophic Lateral Sclerosis.

    PubMed

    Woolley, Susan C; Strong, Michael J

    2015-11-01

    Although amyotrophic lateral sclerosis (ALS) is classically considered a disorder exclusively affecting motor neurons, there is substantial clinical, neuroimaging, and neuropathologic evidence that more than half of patients have an associated syndrome of frontotemporal dysfunction. These syndromes range from frontotemporal dementia to behavioral or cognitive syndromes. Neuroimaging and neuropathologic findings are consistent with frontotemporal lobar degeneration that underpins alterations in network connectivity. Future clinical trials need to be stratified based on the presence or absence of frontotemporal dysfunction on the disease course of ALS. PMID:26515622

  5. Complementary and Alternative Therapies in Amyotrophic Lateral Sclerosis.

    PubMed

    Bedlack, Richard S; Joyce, Nanette; Carter, Gregory T; Paganoni, Sabrina; Karam, Chafic

    2015-11-01

    Given the severity of their illness and lack of effective disease-modifying agents, it is not surprising that most patients with amyotrophic lateral sclerosis (ALS) consider trying complementary and alternative therapies. Some of the most commonly considered alternative therapies include special diets, nutritional supplements, cannabis, acupuncture, chelation, and energy healing. This article reviews these in detail. The authors also describe 3 models by which physicians may frame discussions about alternative therapies: paternalism, autonomy, and shared decision making. Finally, the authors review a program called ALSUntangled, which uses shared decision making to review alternative therapies for ALS. PMID:26515629

  6. Respiratory failure as the presenting manifestation of amyotrophic lateral sclerosis.

    PubMed

    Srivali, Narat; Ryu, Jay H; Rabatin, Jeffrey T

    2016-07-01

    Although amyotrophic lateral sclerosis (ALS) does not directly affect the lung parenchyma, it can jeopardize the mechanical function of the respiratory system. About one-quarter of ALS patients have had at least one prior misdiagnosis. Therefore, a high clinical suspicion, and careful correlation of physical examination and electromyography (EMG) are needed to reach the correct diagnosis. We report a 65-year-old man who presented with a progressive exertional dyspnea. He was subsequently found to have a diaphragmatic paralysis that was felt to be secondary to spinal cord stenosis. However, his subsequent EMG showed evidence of muscle fasciculation and he was ultimately diagnosed with ALS. PMID:26899358

  7. [Dysfunction of mitochondrial dynamic and distribution in Amyotrophic Lateral Sclerosis].

    PubMed

    Walczak, Jarosław; Szczepanowska, Joanna

    2015-01-01

    Amyotrophic lateral sclerosis (ALS) is a complex disease leading to degradation of motor neurons. One of the early symptoms of many neurodegenerative disorders are mitochondrial dysfunctions. Since few decades mitochondrial morphology changes have been observed in tissues of patients with ALS. Mitochondria are highly dynamic organelles which constantly undergo continuous process of fusion and fission and are actively transported within the cell. Proper functioning of mitochondrial dynamics and distribution is crucial for cell survival, especially neuronal cells that have long axons. This article summarizes the current knowledge about the role of mitochondrial dynamics and distribution in pathophysiology of familial and sporadic form of ALS. PMID:26689011

  8. Tracheomegaly Secondary to Tracheotomy Tube Cuff in Amyotrophic Lateral Sclerosis

    PubMed Central

    Lee, Dong Hoon; Yoon, Tae Mi; Lee, Joon Kyoo; Lim, Sang Chul

    2015-01-01

    Abstract Tracheomegaly has not been reported in amyotrophic lateral sclerosis (ALS). Herein, the authors report a case of tracheomegaly secondary to tracheotomy tube cuff in a patient with ALS. To our knowledge, this is the first report of an ALS patient with tracheomegaly and of tracheomegaly being associated with tracheotomy tube cuff and home tracheotomy mechanical ventilator. The clinician should consider the possibility of tracheomegaly in the differential diagnosis, if a patient with ALS develops repeat air leakage around the tracheotomy tube or rupture of tracheotomy tube cuff. PMID:26496301

  9. Ultrasonography of the brachial plexus, normal appearance and practical applications.

    PubMed

    Lapegue, F; Faruch-Bilfeld, M; Demondion, X; Apredoaei, C; Bayol, M A; Artico, H; Chiavassa-Gandois, H; Railhac, J-J; Sans, N

    2014-03-01

    Ultrasound examination of the brachial plexus, although at first sight difficult, is perfectly feasible with fairly rapid practical and theoretical training. The roots are accurately identified due to the shape (a single tubercle) of the transverse process of C7 in the paravertebral space, and the superficial position of C5 in the interscalene groove. The téléphérique technique allows the roots, trunks and cords to be followed easily into the supraclavicular fossa. In just a few years, ultrasound imaging of the plexus has become a routine anesthesia examination for guiding nerve blocks. In trained hands, it also provides information in thoracic outlet syndromes, traumatic conditions (particularly for postganglionic lesions) and tumoral diseases. Even if MRI remains the standard examination in these indications, ultrasound, with its higher definition and dynamic character, is an excellent additional method which is still under-exploited. PMID:24603038

  10. Brachial-Ankle Pulse Wave Velocity: Myths, Misconceptions, and Realities

    PubMed Central

    Sugawara, Jun; Tanaka, Hirofumi

    2015-01-01

    A variety of techniques to evaluate central arterial stiffness have been developed and introduced. None of these techniques, however, have been implemented widely in regular clinical settings, except for brachial-ankle pulse wave velocity (baPWV). The most prominent procedural advantage of baPWV is its ease of use, since it only requires the wrapping of blood pressure cuffs on the 4 extremities. There is mounting evidence indicating the ability of baPWV to predict the risk of future cardiovascular events and total mortality. Additionally, the guidelines for the management of hypertension in Japan recommended the measurement of baPWV be included in the assessment of subclinical target organ damage. However, baPWV has not been fully accepted worldwide due to perceived theoretical and methodological issues. In this review, we address the most frequently mentioned questions and concerns regarding baPWV to shed some light on this simple and easy arterial stiffness measurement. PMID:26587459

  11. Temporal pattern of pulse wave velocity during brachial hyperemia reactivity

    NASA Astrophysics Data System (ADS)

    Graf, S.; Valero, M. J.; Craiem, D.; Torrado, J.; Farro, I.; Zócalo, Y.; Valls, G.; Bía, D.; Armentano, R. L.

    2011-09-01

    Endothelial function can be assessed non-invasively with ultrasound, analyzing the change of brachial diameter in response to transient forearm ischemia. We propose a new technique based in the same principle, but analyzing a continuous recording of carotid-radial pulse wave velocity (PWV) instead of diameter. PWV was measured on 10 healthy subjects of 22±2 years before and after 5 minutes forearm occlusion. After 59 ± 31 seconds of cuff release PWV decreased 21 ± 9% compared to baseline, reestablishing the same after 533 ± 65 seconds. There were no significant changes observed in blood pressure. When repeating the study one hour later in 5 subjects, we obtained a coefficient of repeatability of 4.8%. In conclusion, through analysis of beat to beat carotid-radial PWV it was possible to characterize the temporal profiles and analyze the acute changes in response to a reactive hyperemia. The results show that the technique has a high sensitivity and repeatability.

  12. Resting Doppler ankle brachial pressure index measurement: a literature review.

    PubMed

    Sihlangu, Dorcus; Bliss, Julie

    2012-07-01

    Peripheral vascular disease (PVD) is under-diagnosed in primary and acute settings. The use of Doppler ankle brachial pressure index (ABPI) is effective in diagnosing PVD , aid in determining aetiology of leg ulcers and is cost efficient in reducing the effects of atherosclerosis and cardiovascular events. The aim of this literature review was to review practitioners' experience in using Doppler ABPI, different skills used to measure ABPI and to examine practitioners' confidence in ABPI. The findings identified variation in method for Doppler measurement: including position of the artery, arm measurement, resting period and type of equipment for measuring blood pressure, variations in practitioners' training and experience have demonstrated variability in ABPI results. Although limited in number, the studies have demonstrated knowledge gap, and the need for training among health professionals. PMID:22875182

  13. Ankle brachial pressure index of normal, healthy, younger adults.

    PubMed

    Niblo, Jane; Coull, Alison

    Doppler ultrasound and ankle brachial pressure index (ABPI) calculations are used in the assessment of lower limb vascularity, specifically to determine arterial deficiency. ABPI is important as it is used as an indicator when deciding management options for the treatment of leg ulceration. This study aimed to investigate the range of ABPI measurement, using Doppler ultrasound and sphygmomanometry in 36 young healthy adults aged 18-55 years. The findings show a mean ABPI in the left leg of 1.19 and a mean ABPI of 1.17 in the right leg which, while within the normal range, are consistently in the upper range and significantly higher than the acknowledged 'normal' midpoint of 1.0. It would appear that younger people will have ABPIs within the upper aspect of the normal range and well above the established norm of 1.0. PMID:24151719

  14. Interscalenic approach to the cervico-brachial plexus.

    PubMed

    Evenepoel, M C; Blomme, A

    1981-12-01

    The concept of a closed peri-neurovascular space surrounding the cervicobrachial plexus, introduced by A. Winnie, allows the blockade of the cervical and brachial plexuses by means of a single puncture technique. The single puncture has positive advantages: 1. The rapidity of the blockade; 2. The simplicity of the blockade; 3. Comfort for the patient. The landmarks are easy to make. As with epidural blockade, the injection level and the volume of local anesthetic determine the quality and extent of the block. The traditional indication is surgery of the shoulder and of the supraclavicular area. A new indication seems to be the implantation of a cardiac pacemaker. Complications often quoted in literature are Horner syndrome-a minor complication-and blockade of the ascending branches of the recurrent laryngeal nerve and of the phrenic nerve. The risk of a pneumothorax is almost nil. PMID:7324853

  15. Supraclavicular Brachial Plexus Block for Arteriovenous Hemodialysis Access Procedures.

    PubMed

    Hull, Jeffrey; Heath, Jean; Bishop, Wendy

    2016-05-01

    Ultrasound-guided supraclavicular brachial plexus block using 1% and 2% lidocaine in 21 procedures is reported. Average procedure time was 5.1 minutes (± 1.2 min; range, 2-8 min). Average time of onset and duration were 4.8 minutes (± 3.7 min; range, 0-10 min) and 77.9 minutes (± 26.7 min; range, 44-133 min), respectively, for sensory block and 8.4 minutes (± 5.7 min; range, 3-23 min) and 99 minutes (± 40.5 min; range, 45-171 min), respectively, for motor block. The pain scale assessment averaged 0.4 (± 1.1; range, 0-4). There were no complications. PMID:27106648

  16. Brachial artery waveforms for automatic blood pressure measurement.

    PubMed

    Al-Jumaily, A M; Lan, H; Stergiopulos, N

    2013-02-01

    Theoretically the auscultatory method using Korotkoff sounds is more related to the maximum artery closure status, while the oscillometric method is more related to the overall artery closure status under the cuff. Therefore, the latter is less accurate than the former. This work introduces a new method, which is more accurate than the oscillometric method and suitable for automatic devices. To monitor the maximum artery closure status, a piezoelectric film sensor is attached to the skin just above the brachial artery and under the central section of the cuff where maximum cuff pressure is transferred to the arm. Using the waveform features obtained by this sensor, measurement errors of 0.7±2.5 and 1.27±4.53 mmHg were obtained for the systolic and diastolic pressure, respectively. These reflect small deviations from auscultatory clinical data. PMID:23149078

  17. Brachial Plexus Neuritis Associated With Streptococcus agalactiae Infection: A Case Report.

    PubMed

    Seo, Yu Jung; Lee, Yu Jin; Kim, Joon Sung; Lim, Seong Hoon; Hong, Bo Young

    2014-08-01

    Brachial plexus neuritis is reportedly caused by various factors; however, it has not been described in association with Streptococcus agalactiae. This is a case report of a patient diagnosed with brachial plexus neuritis associated with pyogenic arthritis of the shoulder. A 57-year-old man visited the hospital complaining of sudden weakness and painful swelling of the left arm. The diagnosis was pyogenic arthritis of the left shoulder, and the patient was treated with open irrigation and debridement accompanied by intravenous antibiotic therapy. S. agalactiae was isolated from a wound culture, and an electrodiagnostic study showed brachial plexopathy involving the left upper and middle trunk. Nine weeks after onset, muscle strength improved in most of the affected muscles, and an electrodiagnostic study showed signs of reinnervation. In conclusion, S. agalactiae infection can lead to various complications including brachial plexus neuritis. PMID:25229037

  18. Brachial Plexus Neuritis Associated With Streptococcus agalactiae Infection: A Case Report

    PubMed Central

    Seo, Yu Jung; Lee, Yu Jin; Kim, Joon Sung; Lim, Seong Hoon

    2014-01-01

    Brachial plexus neuritis is reportedly caused by various factors; however, it has not been described in association with Streptococcus agalactiae. This is a case report of a patient diagnosed with brachial plexus neuritis associated with pyogenic arthritis of the shoulder. A 57-year-old man visited the hospital complaining of sudden weakness and painful swelling of the left arm. The diagnosis was pyogenic arthritis of the left shoulder, and the patient was treated with open irrigation and debridement accompanied by intravenous antibiotic therapy. S. agalactiae was isolated from a wound culture, and an electrodiagnostic study showed brachial plexopathy involving the left upper and middle trunk. Nine weeks after onset, muscle strength improved in most of the affected muscles, and an electrodiagnostic study showed signs of reinnervation. In conclusion, S. agalactiae infection can lead to various complications including brachial plexus neuritis. PMID:25229037

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

    SciTech Connect

    Shankar, Sridhar Sonnenberg, Eric van; Silverman, Stuart G.; Tuncali, Kemal; Flanagan, Hugh L.; Whang, Edward E.

    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 plexus 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.

  20. Pulsed radiofrequency treatment within brachial plexus for the management of intractable neoplastic plexopathic pain.

    PubMed

    Arai, Young-Chang P; Nishihara, Makoto; Aono, Shuichi; Ikemoto, Tatsunori; Suzuki, Chiharu; Kinoshita, Akiko; Ushida, Takahiro

    2013-04-01

    We report on the use of pulsed radiofrequency (RF) within the plexus for the management of intractable pain in three patients with metastatic or invasive plexopathy. The patients were a 38-year-old woman with a history of breast cancer 6 years earlier whose computed tomography (CT) scans revealed a mass lesion at the infraclavicular part of the right brachial plexus, a 68-year-old man diagnosed with advanced lung cancer whose CT scans revealed a bone metastasis in the right humerus invading the axillary region of the right brachial plexus, and a 67-year-old woman diagnosed with advanced lung cancer whose CT scans revealed a bone metastasis in the left humerus invading the axillary region of the left brachial plexus. Ultrasound-guided pulsed RF was performed within the interscalene brachial plexus. During the follow-up period, their intractable pain was moderately controlled. PMID:23070568

  1. Large cervicothoracic myxoinflammatory fibroblastic sarcoma with brachial plexus invasion: A case report and literature review

    PubMed Central

    Jia, Xiaotian; Yang, Jianyun; Chen, Lin; Yu, Cong

    2016-01-01

    Myxoinflammatory fibroblastic sarcoma is a rare sarcoma that develops in patients of all ages, which usually presents as a slow-growing painless mass in the distal extremities. To date, myxoinflammatory fibroblastic sarcoma with invasion of the brachial plexus has rarely been reported in the literature. In this study, a case of large cervicothoracic sarcoma, which invaded the brachial plexus, is presented. The patient reported no sensory disturbance or dyskinesia. The tumor was completely resected without injury of the brachial plexus. The postoperative histological diagnosis was myxoinflammatory fibroblastic sarcoma. Follow-up examination performed 24 months after surgery revealed no tumor recurrence and no sensory disturbance or dyskinesia was reported. This study presents a rare case of large myxoinflammatory fibroblastic sarcoma with brachial plexus invasion that was successfully managed by surgery. PMID:27588121

  2. Upright MRI of glenohumeral dysplasia following obstetric brachial plexus injury.

    PubMed

    Nath, Rahul K; Paizi, Melia; Melcher, Sonya E; Farina, Kim L

    2007-11-01

    The purpose of this study was to evaluate the role of upright magnetic resonance imaging (MRI) shoulder scanning in the diagnosis of glenohumeral deformity following obstetric brachial plexus injury (OBPI). Eighty-nine children (ages 0.4 to 17.9 years) with OBPI who have medial rotation contracture and reduced passive and active lateral rotation of the shoulder were evaluated via upright MRI of the affected glenohumeral joint. Qualitative impressions of glenoid form were recorded, and quantitative measurements were made of glenoid version and posterior subluxation. Glenoid version of the affected shoulder averaged -16.8 +/- 11.0 degrees (range, -55 degrees to 1 degrees ), and percentage of the humeral head anterior to the glenoid fossa (PHHA) averaged 32.6 +/- 16.5% (range, -17.8% to 52.4%). The glenoid form was normal in 43 children, convex in 19 children and biconcave in 27 children. Standard MRI protocols were used to obtain bilateral images from 14 of these patients. Among the patients with bilateral MR images, glenoid version and PHHA were significantly different between the involved and uninvolved shoulders (P<.000). Glenoid version in the involved shoulder averaged -19.0 +/- 13.1 degrees (range, -52 degrees to -3 degrees ), and PHHA averaged 29.7 +/- 18.4% (range, -16.2% to 48.7%). In the uninvolved shoulder, the average glenoid version and PHHA were -5.2 +/- 3.7 degrees (range, -12 degrees to -1 degrees ) and 47.7 +/- 3.0% (range, 43% to 54%), respectively. The relative beneficial aspects of upright MRI include lack of need for sedation, low claustrophobic potential and, most important, natural, gravity-influenced position, enabling the surgeon to visualize the true preoperative picture of the shoulder. It is an effective tool for demonstrating glenohumeral abnormalities resulting from brachial plexus injury worthy of surgical exploration. PMID:17448618

  3. 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.

  4. Morphometric Atlas Selection for Automatic Brachial Plexus Segmentation

    SciTech Connect

    Van de Velde, Joris; Wouters, Johan; Vercauteren, Tom; De Gersem, Werner; Duprez, Fréderic; De Neve, Wilfried; Van Hoof, Tom

    2015-07-01

    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. This 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.

  5. Chitotriosidase - a putative biomarker for sporadic amyotrophic lateral sclerosis

    PubMed Central

    2013-01-01

    Background Potential biomarkers to aid diagnosis and therapy need to be identified for Amyotrophic Lateral Sclerosis, a progressive motor neuronal degenerative disorder. The present study was designed to identify the factor(s) which are differentially expressed in the cerebrospinal fluid (CSF) of patients with sporadic amyotrophic lateral sclerosis (SALS; ALS-CSF), and could be associated with the pathogenesis of this disease. Results Quantitative mass spectrometry of ALS-CSF and control-CSF (from orthopaedic surgical patients undergoing spinal anaesthesia) samples showed upregulation of 31 proteins in the ALS-CSF, amongst which a ten-fold increase in the levels of chitotriosidase-1 (CHIT-1) was seen compared to the controls. A seventeen-fold increase in the CHIT-1 levels was detected by ELISA, while a ten-fold elevated enzyme activity was also observed. Both these results confirmed the finding of LC-MS/MS. CHIT-1 was found to be expressed by the Iba-1 immunopositive microglia. Conclusion Elevated CHIT-1 levels in the ALS-CSF suggest a definitive role for the enzyme in the disease pathogenesis. Its synthesis and release from microglia into the CSF may be an aligned event of neurodegeneration. Thus, high levels of CHIT-1 signify enhanced microglial activity which may exacerbate the process of neurodegeneration. In view of the multifold increase observed in ALS-CSF, it can serve as a potential CSF biomarker for the diagnosis of SALS. PMID:24295388

  6. Case-control study of amyotrophic lateral sclerosis

    SciTech Connect

    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, suggesting 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.

  7. Anthropometry of Arm: Nutritional Risk Indicator in Amyotrophic Lateral Sclerosis.

    PubMed

    Salvioni, Cristina Cleide Dos Santos; Stanich, Patricia; Oliveira, Acary Souza Bulle; Orsini, Marco

    2015-12-29

    The aim of the paper is to examine the correlation between clinical data, nutritional, respiratory and functional parameters in amyotrophic lateral sclerosis (ALS). This is a descriptive study of 111 ALS patients [91 spinal onset (GS) and 20 bulbar onset (GB)] carried on using nutritional and respiratory parameters and amyotrophic lateral sclerosis functional rating scale (ALSFRS). ALSFRS was analyzed in the main domains (D1, D2 and D3). Forced vital capacity and anthropometric measurements, there was significant association for GS and GB, and in GS there was positive correlation with midarm circumference (MAC) (r=0.30; P=0.020), midarm muscle circumference (r=0.29; P=0.026), arm muscle area (r=0.28; P=0.033) and protein-caloric malnutrition score (r=0.27; P=0.039), while for GB only with body weight (r=0.64; P=0.024). On correlation of nutritional parameters and ALSFRS for GS patients we observed that MAC and %MAC presented positive association with both issues of D1 and D2. For GB, the total score in addition to correlate positively with anthropometric parameters related to lean body mass also presented negative association with a parameter associated with body fat. In summary, it is suggested that the application of anthropometry of arm could be useful in routine monitoring of ALS patients. PMID:26788263

  8. Anthropometry of Arm: Nutritional Risk Indicator in Amyotrophic Lateral Sclerosis

    PubMed Central

    Stanich, Patricia; Oliveira, Acary Souza Bulle; Orsini, Marco

    2015-01-01

    The aim of the paper is to examine the correlation between clinical data, nutritional, respiratory and functional parameters in amyotrophic lateral sclerosis (ALS). This is a descriptive study of 111 ALS patients [91 spinal onset (GS) and 20 bulbar onset (GB)] carried on using nutritional and respiratory parameters and amyotrophic lateral sclerosis functional rating scale (ALSFRS). ALSFRS was analyzed in the main domains (D1, D2 and D3). Forced vital capacity and anthropometric measurements, there was significant association for GS and GB, and in GS there was positive correlation with midarm circumference (MAC) (r=0.30; P=0.020), midarm muscle circumference (r=0.29; P=0.026), arm muscle area (r=0.28; P=0.033) and protein-caloric malnutrition score (r=0.27; P=0.039), while for GB only with body weight (r=0.64; P=0.024). On correlation of nutritional parameters and ALSFRS for GS patients we observed that MAC and %MAC presented positive association with both issues of D1 and D2. For GB, the total score in addition to correlate positively with anthropometric parameters related to lean body mass also presented negative association with a parameter associated with body fat. In summary, it is suggested that the application of anthropometry of arm could be useful in routine monitoring of ALS patients. PMID:26788263

  9. Association of hemoglobin with ankle-brachial index in general population

    PubMed Central

    Chenglong, Zhang; Jing, Lei; Xia, Ke; Yang, Tianlun

    2016-01-01

    OBJECTIVES: Previous studies have demonstrated that both low and high hemoglobin concentrations are predictive of adverse cardiovascular outcomes in various populations. However, an association of hemoglobin with the ankle-brachial index, which is widely used as a screening test for peripheral arterial disease, has not yet been identified. METHODS: We examined 786 subjects (236 women and 550 men) who received routine physical check-ups. The ankle-brachial index and several hematological parameters, including the hemoglobin level, hematocrit and red blood cell count and other demographic and biochemical characteristics were collected. Univariate and multivariate linear regression analyses were performed to assess the relationships between the ankle-brachial index and the independent determinants. Receiver operating characteristic curve analysis was conducted to calculate the cut-off level of hemoglobin for a relatively low ankle-brachial index (less than 20% of all subjects, which was 1.02). RESULTS: The hemoglobin level, hematocrit and red blood cell count were correlated with the ankle-brachial index in the males (r=-0.274, r=-0.224 and r=-0.273, respectively, p<0.001 for all), but these associations were not significant in the females. Multivariate linear regression analysis revealed that the independent determinants of the ankle-brachial index included age, total cholesterol, high-density lipoprotein cholesterol and the white blood cell count for the females and age, hypertension, total cholesterol and hemoglobin (β=-0.001, p<0.001) for the males after adjusting for confounding factors. Receiver operating characteristic curve analysis revealed that the cut-off level of hemoglobin for predicting a low ankle-brachial index was 156.5 g/L in the males. CONCLUSIONS: A high hemoglobin concentration was independently correlated with a low ankle-brachial index in the healthy males, indicating that an elevation in this level may be associated with an increased

  10. Brachial plexus injury as an unusual complication of coronary artery bypass graft surgery

    PubMed Central

    Chong, A; Clarke, C; Dimitri, W; Lip, G

    2003-01-01

    Brachial plexus injury is an unusual and under-recognised complication of coronary artery bypass grafting especially when internal mammary artery harvesting takes place. It is believed to be due to sternal retraction resulting in compression of the brachial plexus. Although the majority of cases are transient, there are cases where the injury is permanent and may have severe implications as illustrated in the accompanying case history. PMID:12612322

  11. Nerve Transfers in Birth Related Brachial Plexus Injuries: Where Do We Stand?

    PubMed

    Davidge, Kristen M; Clarke, Howard M; Borschel, Gregory H

    2016-05-01

    This article reviews the assessment and management of obstetrical brachial plexus palsy. The potential role of distal nerve transfers in the treatment of infants with Erb's palsy is discussed. Current evidence for motor outcomes after traditional reconstruction via interpositional nerve grafting and extraplexal nerve transfers is reviewed and compared with the recent literature on intraplexal distal nerve transfers in obstetrical brachial plexus injury. PMID:27094890

  12. Axillary nerve neurotization with the anterior deltopectoral approach in brachial plexus injuries.

    PubMed

    Jerome, J Terrence Jose; Rajmohan, Bennet

    2012-09-01

    Combined neurotization of both axillary and suprascapular nerves in shoulder reanimation has been widely accepted in brachial plexus injuries, and the functional outcome is much superior to single nerve transfer. This study describes the surgical anatomy for axillary nerve relative to the available donor nerves and emphasize the salient technical aspects of anterior deltopectoral approach in brachial plexus injuries. Fifteen patients with brachial plexus injury who had axillary nerve neurotizations were evaluated. Five patients had complete avulsion, 9 patients had C5, six patients had brachial plexus injury pattern, and one patient had combined axillary and suprascapular nerve injury. The long head of triceps branch was the donor in C5,6 injuries; nerve to brachialis in combined nerve injury and intercostals for C5-T1 avulsion injuries. All these donors were identified through the anterior approach, and the nerve transfer was done. The recovery of deltoid was found excellent (M5) in C5,6 brachial plexus injuries with an average of 134.4° abduction at follow up of average 34.6 months. The shoulder recovery was good with 130° abduction in a case of combined axillary and suprascapular nerve injury. The deltoid recovery was good (M3) in C5-T1 avulsion injuries patients with an average of 64° shoulder abduction at follow up of 35 months. We believe that anterior approach is simple and easy for all axillary nerve transfers in brachial plexus injuries. PMID:22434572

  13. Analysis of the neurofilament heavy subunit (NFH) gene in familial amyotrophic lateral sclerosis

    SciTech Connect

    Rooke, K.; Rouleau, G.A.; Figlewicz, D.A.

    1994-09-01

    Amyotrophic lateral sclerosis (ALS) is a fatal, adult-onset, degenerative disorder of the motor neurons in the cortex, brainstem and spinal cord. Approximately 10% of ALS cases are familial (FALS) and are inherited as an age-dependent autosomal dominant trait. Mutations in the Cu/Zn superoxide dismutase (SOD-1) gene on chromosome 21 have been found in a subset of cases. However, for the remaining FALS cases, the etiology is unknown. The abnormal accumulation of neurofilaments in the cell body and proximal axon of motor neurons is a characteristic pathological finding in ALS. Furthermore, aberrant neuronal swellings that closely resemble those found in ALS have been reported in transgenic mice overexpressing NFH. The C-terminal region of NFH contains a unique functional domain with multiple repeats of the amino acids (Lys-Ser-Pro) (KSP) and forms the side-arms which appear, at the level of electron microscopy, to cross-link neurofilaments. Recently, deletions in the DSP repeat domain have been identified in five ALS patients diagnosed as sporadic cases of the disease. Based on these findings, we propose to analyze all 4 exons of the NFH gene for variation in FALS. DNA from 110 FALS cases has been amplified by the polymerase chain reaction (PCR) and analyzed by single strand conformation polymorphism (SSCP) analysis. Exon 2, exon 3 and the KSP repeat domain (part of exon 4) appear normal in all our FALS individuals under several different SSCP conditions. The analysis of exon 1 and the remainder of exon 4 has yet to be completed.

  14. Tools for proximal soil sensing

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Proximal soil sensing (i.e. near-surface geophysical methods) are used to study soil phenomena across spatial scales. Geophysical methods exploit contrasts in physical properties (dielectric permittivity, apparent electrical conductivity or resistivity, magnetic susceptibility) to indirectly measur...

  15. Hirayama Disease with Proximal Involvement.

    PubMed

    Kim, Jinil; Kim, Yuntae; Kim, Sooa; Oh, Kiyoung

    2016-10-01

    Hirayama disease is a slowly progressing benign motor neuron disease that affects the distal upper limb. A 29-year-old man visited the hospital with a 1-year history of weakened left proximal upper limb. He was diagnosed with Hirayama disease 9 years ago, while there was no further progression of the muscle weakness afterward. Atrophy and weakness was detected in proximal upper limb muscles. Magnetic resonance imaging and somatosensory evoked potentials were normal. Needle electromyography showed abnormal findings in proximal upper limb muscles. Our patient had Hirayama disease involving the proximal portion through secondary progression. Clinical manifestation and accurate electromyography may be useful for diagnosis. Rare cases with progression patterns as described here are helpful and have clinical meaning for clinicians. PMID:27550499

  16. Case of Young-Onset Sporadic Amyotrophic Lateral Sclerosis.

    PubMed

    Artemiadis, Artemios K; Peppas, Christos; Giannopoulos, Sotiris; Zouvelou, Vasiliki; Triantafyllou, Nikos

    2016-06-01

    Amyotrophic lateral sclerosis (ALS) constitutes the main type of motor neuron disease. Familial ALS is characterized by the presence of positive family history and accounts for 10% of ALS cases. Although familial ALS is the main culprit for early-onset disease, there are rare cases of early- or young-onset ALS with negative family history or sporadic ALS. We describe a 23-year-old man with clinical and electrophysiological evidence of probable sporadic ALS according to the revised EI Escorial criteria. Interestingly, brain neuroimaging revealed bilaterally increased T2 signals across corona radiata, posterior limb of the internal capsule, and descending motor tracts in the brainstem and hypointensity rim of the motor cortex on T2-weighted images. Young-onset sporadic ALS may be a distinct nosological entity. The topic is shortly discussed in the light of its genetic and clinical characteristics. PMID:27224438

  17. 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. PMID:27102003

  18. Pain in Amyotrophic Lateral Sclerosis: A Neglected Aspect of Disease

    PubMed Central

    Handy, Chalonda R.; Krudy, Christina; Boulis, Nicholas; Federici, Thais

    2011-01-01

    Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder marked by progressive loss of motor neurons, muscle wasting, and respiratory dysfunction. With disease progression, secondary symptoms arise creating new problematic conditions for ALS patients. Amongst these is pain. Although not a primary consequence of disease, pain occurs in a substantial number of individuals. Yet, studies investigating its pathomechanistic properties in the ALS patient are lacking. Therefore, more exploratory efforts into its scope, severity, impact, and treatment should be initiated. Several studies investigating the use of Clostridial neurotoxins for the reduction of pain in ALS patients suggest the potential for a neural specific approach involving focal drug delivery. Gene therapy represents a way to accomplish this. Therefore, the use of viral vectors to express transgenes that modulate the nociceptive cascade could prove to be an effective way to achieve meaningful benefit in conditions of pain in ALS. PMID:21766021

  19. Amyotrophic Lateral Sclerosis with an Acute Hypertensive Crises

    PubMed Central

    Lee, Ha Lim

    2012-01-01

    Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder involving the systemic motor neurons, but autonomic nervous function is relatively well preserved. A few studies related to autonomic dysfunction have been reported, but autonomic dysfunction is rare in ALS. Moreover, dysautonomia symptoms are not prominent in patients with ALS. We present a 55-year-old male patient with ALS, who had acute severe hypertension and tachycardia crises, as well as sudden falls in his blood pressure. After he was diagnosed with ALS, he suddenly collapsed and was placed under mechanical ventilation. Several hypertensive attacks and dysautonomic signs then occurred. We successfully controlled the dysautonomia using diazepam and doxazocin mesylate, an alpha receptor antagonist. PMID:22837981

  20. Is erythropoietin gene a modifier factor in amyotrophic lateral sclerosis?

    PubMed

    Ghezzi, Serena; Del Bo, Roberto; Scarlato, Marina; Nardini, Martina; Carlesi, Cecilia; Prelle, Alessandro; Corti, Stefania; Mancuso, Michelangelo; Briani, Chiara; Siciliano, Gabriele; Murri, Luigi; Bresolin, Nereo; Comi, Giacomo Pietro

    2009-05-01

    To investigate the role of erythropoietin (EPO) as genetic determinant in the susceptibility to sporadic amyotrophic lateral sclerosis (SALS). We sequenced a 259-bp region spanning the 3'hypoxia-responsive element of the EPO gene in 222 Italian SALS patients and 204 healthy subjects, matched for age and ethnic origin. No potentially causative variation was detected in SALS subjects; in addition, two polymorphic variants (namely C3434T and G3544T) showed the same genotype and haplotype frequencies in patients and controls. Conversely, a weak but significant association between G3544T and age of disease onset was observed (p=0.04). Overall, our data argue against the hypothesis of EPO as a genetic risk factor for motor neuron dysfunction, at least in Italian population. However, further studies on larger cohort of patients are needed to confirm the evidence of EPO gene as modifier factor. PMID:17888545

  1. 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. PMID:21412722

  2. [BIPAP-mask-ventilation in terminal amyotrophic lateral sclerosis (ALS)].

    PubMed

    Sellner-Pogány, Theresa; Lahrmann, Heinz

    2009-12-01

    Amyotrophic lateral sclerosis (ALS) is a progressive, neuromuscular disease without any curative therapy at the moment. Non-invasive BIPAP-ventilation has proven to be helpful to cope with the increasing hypoventilation due to weakness in ventilatory muscles in ALS. If BIPAP-ventilation is well tolerated by the patient, it can be very helpful for symptom control in palliative homecare. In this case presentation we discuss how non-invasive ventilation can influence perception of dying at home and thereby quality of life of patients, caregivers and members of palliative teams. We will see that especially during this sensible terminal phase various questions and fears concerning the mechanically assisted ventilation may arise. These issues should be addressed in advance with careful attention and information. Members of palliative teams should therefore be provided with good training and regular supervision. PMID:20151350

  3. Projected increase in amyotrophic lateral sclerosis from 2015 to 2040

    PubMed Central

    Arthur, Karissa C.; Calvo, Andrea; Price, T. Ryan; Geiger, Joshua T.; Chiò, Adriano; Traynor, Bryan J.

    2016-01-01

    Although amyotrophic lateral sclerosis (ALS) is relatively rare, the socioeconomic significance of the disease is extensive. It is therefore vital to project the epidemiologic trend of ALS. To date, there have been few published studies attempting to estimate the number and distribution of ALS cases in the upcoming years. Here we show that the number of ALS cases across the globe will increase from 222,801 in 2015 to 376,674 in 2040, representing an increase of 69%. This increase is predominantly due to ageing of the population, particularly among developing nations. This projection is likely an underestimate due to improving healthcare and economic conditions. The results should be used to inform healthcare policy to more efficiently allocate healthcare resources. PMID:27510634

  4. Assessment of the upper motor neuron in amyotrophic lateral sclerosis.

    PubMed

    Huynh, William; Simon, Neil G; Grosskreutz, Julian; Turner, Martin R; Vucic, Steve; Kiernan, Matthew C

    2016-07-01

    Clinical signs of upper motor neuron (UMN) involvement are an important component in supporting the diagnosis of amyotrophic lateral sclerosis (ALS), but are often not easily appreciated in a limb that is concurrently affected by muscle wasting and lower motor neuron degeneration, particularly in the early symptomatic stages of ALS. Whilst recent criteria have been proposed to facilitate improved detection of lower motor neuron impairment through electrophysiological features that have improved diagnostic sensitivity, assessment of upper motor neuron involvement remains essentially clinical. As a result, there is often a significant diagnostic delay that in turn may impact institution of disease-modifying therapy and access to other optimal patient management. Biomarkers of pathological UMN involvement are also required to ensure patients with suspected ALS have timely access to appropriate therapeutic trials. The present review provides an analysis of current and recently developed assessment techniques, including novel imaging and electrophysiological approaches used to study corticomotoneuronal pathology in ALS. PMID:27291884

  5. 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.

  6. Amyotrophic Lateral Sclerosis and Metabolomics: Clinical Implication and Therapeutic Approach

    PubMed Central

    Kumar, Alok; Ghosh, Devlina; Singh, R. L.

    2013-01-01

    Amyotrophic lateral sclerosis (ALS) is one of the most common motor neurodegenerative disorders, primarily affecting upper and lower motor neurons in the brain, brainstem, and spinal cord, resulting in paralysis due to muscle weakness and atrophy. The majority of patients die within 3–5 years of symptom onset as a consequence of respiratory failure. Due to relatively fast progression of the disease, early diagnosis is essential. Metabolomics offer a unique opportunity to understand the spatiotemporal metabolic crosstalks through the assessment of body fluids and tissue. So far, one of the most challenging issues related to ALS is to understand the variation of metabolites in body fluids and CNS with the progression of disease. In this paper we will review the changes in metabolic profile in response to disease progression condition and also see the therapeutic implication of various drugs in ALS patients. PMID:26317018

  7. 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

  8. 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

  9. Amyotrophic lateral sclerosis and motor neuron syndromes in Asia.

    PubMed

    Shahrizaila, N; Sobue, G; Kuwabara, S; Kim, S H; Birks, Carol; Fan, D S; Bae, J S; Hu, C J; Gourie-Devi, M; Noto, Y; Shibuya, K; Goh, K J; Kaji, R; Tsai, C P; Cui, L; Talman, P; Henderson, R D; Vucic, S; Kiernan, M C

    2016-08-01

    While the past 2 decades have witnessed an increasing understanding of amyotrophic lateral sclerosis (ALS) arising from East Asia, particularly Japan, South Korea, Taiwan and China, knowledge of ALS throughout the whole of Asia remains limited. Asia represents >50% of the world population, making it host to the largest patient cohort of ALS. Furthermore, Asia represents a diverse population in terms of ethnic, social and cultural backgrounds. In this review, an overview is presented that covers what is currently known of ALS in Asia from basic epidemiology and genetic influences, through to disease characteristics including atypical phenotypes which manifest a predilection for Asians. With the recent establishment of the Pan-Asian Consortium for Treatment and Research in ALS to facilitate collaborations between clinicians and researchers across the region, it is anticipated that Asia and the Pacific will contribute to unravelling the uncertainties in ALS. PMID:27093948

  10. REHABILITATION IN AMYOTROPHIC LATERAL SCLEROSIS: WHY IT MATTERS

    PubMed Central

    MAJMUDAR, SALONY; WU, JASON; PAGANONI, SABRINA

    2015-01-01

    Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that results in a constellation of problematic symptoms and a high patient and caregiver burden. Multidisciplinary care includes rehabilitation interventions that have the goal of assisting people to teach their fullest potential despite the presence of a disabling disease. Given the progressive nature of ALS, the clinician must be aware of the expected disease trajectory and apply appropriate interventions at each stage. This review will present rehabilitation strategies that can be utilized to maximize patient independence, function, safety, and quality of life, and to minimize disease-related symptoms. The role of bracing, exercise, assistive devices, and adaptive equipment will be discussed. At each disease stage, an experienced rehabilitation team is well positioned to make a significant impact on the life of ALS patients. PMID:24510737

  11. 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. PMID:23540596

  12. Projected increase in amyotrophic lateral sclerosis from 2015 to 2040.

    PubMed

    Arthur, Karissa C; Calvo, Andrea; Price, T Ryan; Geiger, Joshua T; Chiò, Adriano; Traynor, Bryan J

    2016-01-01

    Although amyotrophic lateral sclerosis (ALS) is relatively rare, the socioeconomic significance of the disease is extensive. It is therefore vital to project the epidemiologic trend of ALS. To date, there have been few published studies attempting to estimate the number and distribution of ALS cases in the upcoming years. Here we show that the number of ALS cases across the globe will increase from 222,801 in 2015 to 376,674 in 2040, representing an increase of 69%. This increase is predominantly due to ageing of the population, particularly among developing nations. This projection is likely an underestimate due to improving healthcare and economic conditions. The results should be used to inform healthcare policy to more efficiently allocate healthcare resources. PMID:27510634

  13. Compromising abnormalities of the brachial plexus as displayed by magnetic resonance imaging.

    PubMed

    Collins, J D; Shaver, M L; Disher, A C; Miller, T Q

    1995-01-01

    Magnetic resonance images (MRI) of brachial plexus anatomy bilaterally, not possible by plain radiographs or CT, were presented to the Vascular Surgery, Neurology, and the Neurosurgery departments. Patients were requested for MRI of their brachial plexus. They were referred for imaging and the imaging results were presented to the faculty and housestaff. Our technique was accepted and adopted to begin referrals for MRI evaluation of brachial plexopathy. Over 175 patients have been studied. Eighty-five patients were imaged with the 1.5 Tesla magnet (Signa; General Electric Medical Systems, Milwaukee, WI) 3-D reconstruction MRI. Coronal, transverse (axial), oblique transverse, and sagittal plane T1-weighted and selected T2-weighted pulse sequences were obtained at 4-5 mm slice thickness, 40-45 full field of view, and a 512 x 256 size matrix. Saline water bags were used to enhance the signal between the neck and the thorax. Sites of brachial plexus compromise were demonstrated. Our technique with 3-D reconstruction increased the definition of brachial plexus pathology. The increased anatomical definition enabled the vascular surgeons and neurosurgeons to improve patient care. Brachial plexus in vivo anatomy as displayed by MRI, magnetic resonance angiography (MRA), and 3-D reconstruction offered an opportunity to augment the teaching of clinical anatomy to medical students and health professionals. Selected case presentations (bodybuilder, anomalous muscle, fractured clavicle, thyroid goiter, silicone breast implant rupture, and cervical rib) demonstrated compromise of the brachial plexus displayed by MRI. The MRI and 3-D reconstruction techniques, demonstrating the bilateral landmark anatomy, increased the definition of the clinical anatomy and resulted in greater knowledge of patient care management. PMID:7697507

  14. Postfixed Brachial Plexus Radiculopathy Due to Thoracic Disc Herniation in a Collegiate Wrestler: A Case Report

    PubMed Central

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

    2013-01-01

    Objective: To present the unique case of a collegiate wrestler with C7 neurologic symptoms due to T1–T2 disc herniation. Background: 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. Differential Diagnosis: Cervical spine injury, stinger/burner, peripheral nerve injury, spinal cord injury, thoracic outlet syndrome, brachial plexus radiculopathy. Treatment: 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. Uniqueness: 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. Conclusions: 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

  15. Racing against the clock: recognizing, differentiating, diagnosing, and referring the amyotrophic lateral sclerosis patient.

    PubMed

    Shook, Steven J; Pioro, Erik P

    2009-01-01

    Recognition of the early symptoms and signs in amyotrophic lateral sclerosis, exclusion of alternative diagnoses, and referral to a tertiary center can have a significant positive impact on the lives of patients and their caregivers. This article provides the most current amyotrophic lateral sclerosis criteria, as well as helpful clinical clues to the diagnosis. An approach to laboratory testing, electrodiagnostic testing, and imaging to exclude diseases that mimic ALS also are discussed, as are atypical presentations that can confound timely diagnosis. PMID:19191305

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

    PubMed Central

    Liu, Kevin X.; Edwards, Benjamin; Lee, Sheena; Finelli, Mattéa J.; Davies, Ben

    2015-01-01

    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 SOD1G93A 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 SOD1G93A 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 SOD1G93A 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. PMID:25753484

  17. 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

  18. [Differential diagnostic criteria in cervico-brachial psychalgia].

    PubMed

    Tröltzsch, M; Fischer, P

    1979-11-15

    In 100 patients with only for a short time existing pains in the region of neck, shoulder and arm and inconspicuous laboratory and X-ray findings by means of the galvanic test of the muscular function and Janda's test of the muscular function a vastly intact nerve-muscle-apparatus was proved. In the ENR-test after Brengelmann and Brengelmann clear introversion values, significantly high neurodizism values and slightly increased rigidity values were shown. Also the VELA-values were significantly higher than in the normal comparative group. In an additional inquiry predisposing biographical references for the existence of actual conflicts were found. As to the inclusion of further biographical data, 68% of intense affect reactions, 16% of functionally fixed neurotic reactions, 9% of primary and 7% of secondary neurotic maldevelopments are concerned. After a 4-week- hard-and-fast date (15 mg/a day) the complaints improved without a clear retrogression of the neurotic constellations. At the same time a change of the initially slightly increased conducting values of the skin and of the vegetative complaints appeared. A cervico-brachial psychalgia is present, when an organic muscle disease was excluded, when in the ENR-test high values to neuroticism, to introversion and rigidity are found and when a temporary connection to actual conflicts are the result. PMID:549300

  19. Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome.

    PubMed

    Wakerley, Benjamin R; Yuki, Nobuhiro

    2014-03-01

    The pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome is defined by rapidly progressive oropharyngeal and cervicobrachial weakness associated with areflexia in the upper limbs. Serial nerve conduction studies suggest that PCB represents a localised subtype of Guillain-Barré syndrome characterised by axonal rather than demyelinating neuropathy. Many neurologists are unfamiliar with PCB, which is often misdiagnosed as brainstem stroke, myasthenia gravis or botulism. The presence of additional ophthalmoplegia and ataxia indicates overlap with Fisher syndrome. Half of patients with PCB carry IgG anti-GT1a antibodies which often cross-react with GQ1b, whereas most patients with Fisher syndrome carry IgG anti-GQ1b antibodies which always cross-react with GT1a. Significant overlap between the clinical and serological profiles of these patients supports the view that PCB and Fisher syndrome form a continuous spectrum. In this review, we highlight the clinical features of PCB and outline new diagnostic criteria. PMID:23804237

  20. Arterial function of carotid and brachial arteries in postmenopausal vegetarians

    PubMed Central

    Su, Ta-Chen; Torng, Pao-Ling; Jeng, Jiann-Shing; Chen, Ming-Fong; Liau, Chiau-Suong

    2011-01-01

    Background: Vegetarianism is associated with a lower risk of cardiovascular disease. However, studies of arterial function in vegetarians are limited. Methods: This study investigated arterial function in vegetarianism by comparing 49 healthy postmenopausal vegetarians with 41 age-matched omnivores. The arterial function of the common carotid artery was assessed by carotid duplex, while the pulse dynamics method was used to measure brachial artery distensibility (BAD), compliance (BAC), and resistance (BAR). Fasting blood levels of glucose, lipids, lipoprotein (a), high-sensitivity C-reactive protein, homocysteine, and vitamin B12 were also measured. Results: Vegetarians had significantly lower serum cholesterol, high-density and low-density lipoprotein, and glucose compared with omnivores. They also had lower vitamin B12 but higher homocysteine levels. Serum levels of lipoprotein (a) and high-sensitivity C-reactive protein were no different between the two groups. There were no significant differences in carotid beta stiffness index, BAC, and BAD between the two groups even after adjustment for associated covariates. However, BAR was significantly lower in vegetarians than in omnivores. Multiple linear regression analysis revealed that age and pulse pressure were two important determinants of carotid beta stiffness index and BAD. Vegetarianism is not associated with better arterial elasticity. Conclusion: Apparently healthy postmenopausal vegetarians are not significantly better in terms of carotid beta stiffness index, BAC, and BAD, but have significantly decreased BAR than omnivores. Prevention of vitamin B12 deficiency might be beneficial for cardiovascular health in vegetarians. PMID:21915169

  1. Clinical usefulness of ankle brachial index and brachial-ankle pulse wave velocity in patients with ischemic stroke

    PubMed Central

    Lee, Hyung-Suk; Lee, Hye Lim; Han, Ho-seong; Yeo, Minju; Kim, Ji Seon; Lee, Sung-Hyun; Lee, Sang-Soo; Shin, Dong-Ick

    2016-01-01

    Abstract Ankle brachial index (ABI) and brachial-ankle pulse wave velocity (baPWV) are widely used noninvasive modalities to evaluate atherosclerosis. Recently, evidence has increased supporting the use of ABI and baPWV as markers of cerebrovascular disease. This study sought to examine the relationship between ABI and baPWV with ischemic stroke. This study also aimed to determine which pathogenic mechanism, large artery disease (LAD) or small vessel disease (SVD), is related to ABI or baPWV. Retrospectively, 121 patients with ischemic stroke and 38 subjects with no obvious ischemic stroke history were recruited. First, ABI and baPWV were compared between the groups. Then, within the stroke group, the relevance of ABI and baPWV with regard to SVD and LAD, which were classified by brain magnetic resonance image (MRI) and magnetic resonance angiography (MRA) or computed tomography angiography (CTA) findings, was assessed. The baPWV was higher in the stroke group than non-stroke group (1,944.18±416.6 cm/s vs. 1,749.76±669.6 cm/s, P<0.01). Regarding LAD, we found that mean ABI value was lower in the group with extracranial large artery stenosis (P<0.01), and there was an inverse linear correlation between ABI and the grade of extracranial large artery stenosis (P<0.01). For SVD, there was a significant correlation between SVD and baPWV (2,057.6±456.57 cm/s in the SVD (+) group vs. 1,491±271.62 cm/s in the SVD (-) group; P<0.01). However, the grade of abnormalities detected in SVD did not correlate linearly with baPWV. These findings show that baPWV is a reliable surrogate marker of ischemic stroke. Furthermore, baPWV and ABI can be used to indicate the presence of small vessel disease and large arterial disease, respectively. PMID:27533937

  2. Brachial-to-radial systolic blood pressure amplification in patients with type 2 diabetes mellitus.

    PubMed

    Climie, R E D; Picone, D S; Keske, M A; Sharman, J E

    2016-06-01

    Brachial-to-radial-systolic blood pressure amplification (Bra-Rad-SBPAmp) can affect central SBP estimated by radial tonometry. Patients with type 2 diabetes mellitus (T2DM) have vascular irregularities that may alter Bra-Rad-SBPAmp. By comparing T2DM with non-diabetic controls, we aimed to determine the (1) magnitude of Bra-Rad-SBPAmp; (2) haemodynamic factors related to Bra-Rad-SBPAmp; and (3) effect of Bra-Rad-SBPAmp on estimated central SBP. Twenty T2DM (64±8 years) and 20 non-diabetic controls (60±8 years; 50% male both) underwent simultaneous cuff deflation and two-dimensional ultrasound imaging of the brachial and radial arteries. The first Korotkoff sound (denoting SBP) was identified from the first inflection point of Doppler flow during cuff deflation. Bra-Rad-SBPAmp was calculated by radial minus brachial SBP. Upper limb and systemic haemodynamics were recorded by tonometry and ultrasound. Radial SBP was higher than brachial SBP for T2DM (136±19 vs 127±17 mm Hg; P<0.001) and non-diabetic controls (135±12 vs 121±11 mm Hg; P<0.001), but Bra-Rad-SBPAmp was significantly lower in T2DM (9±8 vs 14±7 mm Hg; P=0.042). The product of brachial mean flow velocity × brachial diameter was inversely and independently correlated with Bra-Rad-SBPAmp in T2DM (β=-0.033 95% confidence interval -0.063 to -0.004, P=0.030). When radial waveforms were calibrated using radial, compared with brachial SBP, central SBP was significantly higher in both groups (T2DM, 116±13 vs 125±15 mm Hg; and controls, 112±10 vs 124±11 mm Hg; P<0.001 both) and there was a significant increase in the number of participants classified with 'central hypertension' (SBP⩾130 mm Hg; P=0.004). Compared with non-diabetic controls, Bra-Rad-SBPAmp is significantly lower in T2DM. Regardless of disease status, radial SBP is higher than brachial SBP and this results in underestimation of central SBP using brachial-BP-calibrated radial tonometry. PMID:26446391

  3. A case of relapsing-remitting facial palsy and ipsilateral brachial plexopathy caused by HSV-1.

    PubMed

    Alstadhaug, Karl B; Kvarenes, Hanne W; Prytz, Jan; Vedeler, Christian

    2016-05-01

    The etiologies of Bell's palsy and brachial neuritis remain uncertain, and the conditions rarely co-occur or reoccur. Here we present a woman in her twenties who had several relapsing-remitting episodes with left-sided facial palsy and brachial neuropathy. The episodes always started with painful left-sided oral blisters. Repeat PCRs HSV-1 DNA from oral vesicular lesions were positive. Extensive screening did not reveal any other underlying cause. Findings on MRI T2-weighted brachial plexus STIR images, using a 3.0-Tesla scanner during an episode, were compatible with brachial plexus neuritis. Except a mannose-binding lectin deficiency, a congenital complement deficiency that is frequently found in the general Caucasian population, no other immunodeficiency was demonstrated in our patient. In vitro resistance to acyclovir was tested negative, but despite prophylactic treatment with the drug in high doses, relapses recurred. To our knowledge, this is the first ever reported documentation of relapsing-remitting facial and brachial plexus neuritis caused by HSV-1. PMID:26991053

  4. The Extent of Blockade Following Axillary and Infraclavicular Approaches of Brachial Plexus Block in Uremic Patients

    PubMed Central

    Sariguney, Damla; Mahli, Ahmet; Coskun, Demet

    2012-01-01

    Introduction This study was aimed to compare the axillary approach performed through multiple injection method and vertical infraclavicular approach performed through single injection method in terms of the sensory and motor block onset, quality, and extent of blocks of brachial plexus in uremic patients who underwent arteriovenous fistula surgery. Methods Forty patients scheduled for creation of arteriovenous fistula with axillary brachial plexus block (group AX, n = 20) or infraclavicular brachial plexus block (IC group, n = 20) were examined. The median, radial, ulnar, and musculocutaneous nerves were selectively localized by nerve stimulation. The volume of the local anesthetics was calculated based on the height of each patient, and the volume determined was prepared by mixing 2% lidocaine and 0.5% bupivacaine in equal proportions. Sensory and motor block were assessed at 3, 6, 9, 12, 15, 18, and 30th min and their durations were measured. Results While the adequate sensory and motor block rate with axillary approach was 100% in musculocutaneous, median, radial, ulnar and medial antebrachial cutaneous nerves, it was 65% in axillary nerve, 80% in intercostobrachial nerve and 95% in medial brachial cutaneous nerve. This rate was found to be 100% for all the nerves with infraclavicular approach. Conclusion For arteriovenous fistula surgeries in uremic patients, both axillary approach performed through multiple injection method and vertical infraclavicular approach performed through single injection method can be used successfully; however, for the short performance of the procedure, infraclavicular block may be preferred. Keywords Brachial plexus block; Axillary; Infraclavicular; Uremic patients PMID:22383924

  5. Use of a Collagen-Based Device for Closure of Low Brachial Artery Punctures

    SciTech Connect

    Belenky, A. Aranovich, D.; Greif, F.; Bachar, G.; Bartal, G.; Atar, E.

    2007-04-15

    Purpose. To report our experience with the Angioseal vascular closure device for hemostasis of distal brachial artery puncture. Methods. Between September 2003 and August 2005, 64 Angioseal vascular closure devices were inserted in 64 patients (40 men, 24 women; mean age 65 years) immediately after diagnostic or therapeutic arterial angiographies performed through a 5 Fr to 7 Fr sheath via the distal brachial artery. Ultrasound examination of the brachial artery preceded the angiography in all cases and only arteries wider than 4 mm were closed by the Angioseal. In cases of a sonographically evident thin subcutaneous space of the cubital fossa, tissue tumescence, using 1% Lidocaine, was performed prior to the arterial closure. Results. The deployment success rate was 100%. No major complications were encountered; only 2 patients developed puncture site hematoma, and these were followed conservatively. Conclusions. Closure of low brachial artery punctures with the Angioseal is simple and safe. No additional manual compression is required. We recommend its use after brachial artery access interventions, through appropriately wide arteries, to improve early patient ambulation and potentially reduce possible puncture site complications.

  6. Early transient radiation-induced brachial plexopathy in locally advanced head and neck cancer

    PubMed Central

    Etiz, Durmus

    2016-01-01

    Aim of the study Early transient brachial plexopathy following radiotherapy (RT) in patients with head and neck cancer may be underreported and associated with a dose-response. Our purpose was to determine the incidence of early transient radiation-ınduced brachial plexopathy (RIBP) in patients receiving primary RT (± chemotherapy) for locally advanced head and neck cancer (HNC). Material and methods Twenty-seven locally advanced HNC patients who have no finding of brachial plexopathy at the diagnosis were evaluated 3 times by a specifically developed 13-item questionnaire for determining early transient RIBP. The 54 brachial plexus in 27 patients were delineated and dose volume histograms were calculated. Results Median follow-up period was 28 (range: 15–40) months. The mean BP volume was 7.9 ±3.6 cm3, and the mean and maximum doses to the BP were 45.3 (range: 32.3–59.3) Gy, and 59.4 (range: 41.4–70.3) Gy, respectively. Maximum dose to the BP was ≥ 70 Gy only in 2 nasopharyngeal cancer patients. Two (7%) early transient RIBP were reported at 7th and 8th month after RT under maximum 67.17 and 55.37 Gy, and mean 52.95 and 38.60 Gy RT doses. Conclusions Two (7%) early RIBP were seen in the patient group, although brachial plexus maximum doses were ≥ 66 Gy in 75% of patients. PMID:27095943

  7. Above Elbow Amputation Under Brachial Plexus Block at Supraclavicular and Interscalene Levels

    PubMed Central

    Ahmad, Hassan; Yadagiri, Manjula; Macrosson, Duncan; Majeed, Amer

    2015-01-01

    Introduction: The brachial plexus block is a commonly performed procedure in the anesthetic practice today. It is performed for analgesia as well as anesthesia for upper limb procedures. It has been used for amputation and replantation surgeries of the upper limb. Case presentation: We present the case of a 68-year-old gentleman who had brachial plexus block at supraclavicular and interscalene levels as the sole anesthetic for undergoing above elbow amputation. He was deemed to be very high risk for a general anesthetic as he suffered from severe chronic obstructive pulmonary disease (COPD) and a very poor exercise tolerance (NYHA Class III). The supraclavicular brachial plexus block was supplemented with an interscalene brachial plexus block due to inadequate surgical anesthesia encountered with the former. The procedure was successfully completed under regional anesthesia. Conclusions: The brachial plexus block can be performed at different levels in the same patient to achieve desired results, while employing sound anatomical knowledge and adhering to the maximum safe dose limit of the local anesthetic. PMID:26705518

  8. 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. PMID:25175852

  9. 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…

  10. 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…

  11. Cubesat Proximity Operations Demonstration (CPOD)

    NASA Technical Reports Server (NTRS)

    Villa, Marco; Martinez, Andres; Petro, Andrew

    2015-01-01

    The CubeSat Proximity Operations Demonstration (CPOD) project will demonstrate rendezvous, proximity operations and docking (RPOD) using two 3-unit (3U) CubeSats. Each CubeSat is a satellite with the dimensions 4 inches x 4 inches x 13 inches (10 centimeters x 10 centimeters x 33 centimeters) and weighing approximately 11 pounds (5 kilograms). This flight demonstration will validate and characterize many new miniature low-power proximity operations technologies applicable to future missions. This mission will advance the state of the art in nanosatellite attitude determination,navigation and control systems, in addition to demonstrating relative navigation capabilities.The two CPOD satellites are scheduled to be launched together to low-Earth orbit no earlier than Dec. 1, 2015.

  12. Does C5 or C6 Radiculopathy Affect the Signal Intensity of the Brachial Plexus on Magnetic Resonance Neurography?

    PubMed Central

    Seo, Tae Gyu; Kim, In-Soo; Son, Eun Seok

    2016-01-01

    Patients with C5 or C6 radiculopathy complain of shoulder area pain or shoulder girdle weakness. Typical idiopathic neuralgic amyotrophy (INA) is also characterized by severe shoulder pain, followed by paresis of shoulder girdle muscles. Recent studies have demonstrated that magnetic resonance neurography (MRN) of the brachial plexus and magnetic resonance imaging (MRI) of the shoulder in patients with INA show high signal intensity (HSI) or thickening of the brachial plexus and changes in intramuscular denervation of the shoulder girdle. We evaluated the value of brachial plexus MRN and shoulder MRI in four patients with typical C5 or C6 radiculopathy. HSI of the brachial plexus was noted in all patients and intramuscular changes were observed in two patients who had symptoms over 4 weeks. Our results suggest that HSI or thickening of the brachial plexus and changes in intramuscular denervation of the shoulder girdle on MRN and MRI may not be specific for INA. PMID:27152289

  13. Does C5 or C6 Radiculopathy Affect the Signal Intensity of the Brachial Plexus on Magnetic Resonance Neurography?

    PubMed

    Seo, Tae Gyu; Kim, Du Hwan; Kim, In-Soo; Son, Eun Seok

    2016-04-01

    Patients with C5 or C6 radiculopathy complain of shoulder area pain or shoulder girdle weakness. Typical idiopathic neuralgic amyotrophy (INA) is also characterized by severe shoulder pain, followed by paresis of shoulder girdle muscles. Recent studies have demonstrated that magnetic resonance neurography (MRN) of the brachial plexus and magnetic resonance imaging (MRI) of the shoulder in patients with INA show high signal intensity (HSI) or thickening of the brachial plexus and changes in intramuscular denervation of the shoulder girdle. We evaluated the value of brachial plexus MRN and shoulder MRI in four patients with typical C5 or C6 radiculopathy. HSI of the brachial plexus was noted in all patients and intramuscular changes were observed in two patients who had symptoms over 4 weeks. Our results suggest that HSI or thickening of the brachial plexus and changes in intramuscular denervation of the shoulder girdle on MRN and MRI may not be specific for INA. PMID:27152289

  14. Double free gracilis muscle transfer after complete brachial plexus injury: First Canadian experience

    PubMed Central

    Elzinga, Kate; Zuo, Kevin J; Olson, Jaret L; Morhart, Michael; Babicki, Sasha; Chan, K Ming

    2014-01-01

    Traumatic brachial plexus root avulsions are devastating injuries, and are complex and challenging to reconstruct. Double free muscle transfer using the gracilis muscles is a potentially effective method of restoring upper extremity function. The authors report on the first two patients treated using this technique in Canada. Both sustained traumatic brachial plexus root avulsion injuries resulting in a flail arm. In the first step of this two-stage procedure, a gracilis muscle was transferred to restore elbow flexion, and wrist and digit extension. Months later, the transfer of the second gracilis muscle was performed to enhance elbow flexion and to enable wrist and digit flexion. Postoperatively, both patients achieved Medical Research Council grade 4 elbow flexion, functional handgrip and were able to return to gainful employment. Patient satisfaction was high and active range of motion improved substantially. The authors’ experience supports the use of this technique following severe brachial plexus injury. PMID:25152644

  15. Primary brachial vein transposition for hemodialysis access: report of a case and review of the literature.

    PubMed

    Lambidis, Constantinos; Galanopoulos, Georgios

    2013-07-01

    The superiority of autogenous fistulae in patients with end-stage renal disease, performing hemodialysis, is well established and largely accepted. However, in case that superficial veins in the upper arm are not available for fistula construction, brachial vein transposition may be a viable alternative prior to graft placement. This transposition could be done as a primary or staged procedure, depending on the vein size. We present the case of a 63-year-old male patient with a thrombosed arteriovenous graft in the forearm and a large brachial vein in the ipsilateral upper arm. A one-stage (primary) brachial vein transposition was performed. The fistula, 10 months after its construction, is still patent. No complications have occurred. PMID:23134151

  16. A novel technique of ultrasound-guided brachial plexus block in calves.

    PubMed

    Iwamoto, Jiro; Yamagishi, Norio; Sasaki, Kouya; Kim, Danil; Devkota, Bhuminand; Furuhama, Kazuhisa

    2012-12-01

    An interventional ultrasound technique to increase the safety of surgical treatment of the calf forelimb was tested. First, the brachial plexus was evaluated using ultrasonography and then 2% lidocaine was injected under ultrasound guidance. Ultrasonically, the brachial plexus appeared as multiple hypoechoic areas surrounded by a hyperechoic rim or a hyperechoic structure characterised by multiple discontinuous lines. It was located between the omotransverse muscle and axillary artery and vein. The sensitive effect in the forelimb was seen mainly in the area supplied by the musculocutaneous nerve, indicating successful blockage in the nerve plexus. Out of the eight forelimbs, the motor effect was observed in seven forelimbs. These results suggest the clinical feasibility of ultrasound-guided brachial plexus block in bovine medicine, although further studies are needed to examine various approaches, including the sites of needle insertion and the appropriate volume and dosage of anaesthetic. PMID:22682007

  17. Brachial Plexopathy/Nerve Root Avulsion in a Football Player: The Role of Electrodiagnostics

    PubMed Central

    Radecki, Jeffrey; Wolfe, Scott W.; Strauss, Helene L.; Mintz, Douglas N.

    2008-01-01

    Electromyography (EMG) studies are a useful tool in anatomical localization of peripheral nerve and brachial plexus injuries. They are especially helpful in distinguishing between brachial plexopathy and nerve root injuries where surgical intervention may be indicated. EMG can also assist in providing prognostic information after nerve injury as well as after nerve repair. In this case report, a football player presented with weakness in his right upper limb after a traction/traumatic injury to the right brachial plexus. EMG studies revealed evidence of both pre- and postganglionic injury to multiple cervical roots. The injury was substantial enough to cause nerve root avulsions involving the C6 and C7 levels. Surgical referral led to nerve grafts targeted at regaining function in shoulder abduction and elbow flexion. After surgery, the patient’s progress was monitored utilizing EMG to assist in identifying true axonal regeneration. PMID:18751870

  18. Brachial Plexus Tumors in a Consecutive Series of Twenty One Patients

    PubMed Central

    Go, Myeong Hoon; Cho, Ki Hong

    2012-01-01

    Objective This is a retrospective review of 22 surgically treated benign and malignant tumors of brachial plexus region to describe clinical presentation, the characteristics of brachial plexus tumor and clinical outcomes with a literature review. Methods Twenty-one patients with consecutive 22 surgeries for primary brachial plexus tumors were enrolled between February 2002 and November 2011 were included in this study. The medical records of all patients were reviewed. Results Eleven male and 10 female patients were enrolled. Mean age was 39 years. Three patients had brachial plexus tumor associated with neurofibromatosis (13.6%). Presenting signs and symptoms included parenthesis and numbness (54.5%), radiating pain (22.7%), direct tenderness and pain (27.2%), palpable mass (77.3%). Twelve patients presented preoperative sensory deficit (54.5%) and 9 patients presented preoperative motor deficit (40.9%). Twenty tumors (90.9%) were benign and 2 tumors (9.1%) were malignant. Benign tumors included 15 schwannomas (68.2%), 4 neurofibromas (18.2%) and 1 granular cell tumor (4.5%). There were 1 malignant peripheral nerve sheath tumor (MPNST) and 1 malignant granular cell tumor. Gross total resection was achieved in 16 patients (72.7%), including all schwannomas, 1 neurofibroma. Subtotal resection was performed in 6 tumors (27.3%), including 3 neurofibromatosis associated with brachial plexus neurofibromas, 1 MPNST and 2 granular cell tumor in one patient. Conclusion Resection of tumor is the choice of tumor in the most of benign and malignant brachial plexus tumors. Postoperative outcomes are related to grade of resection at surgery and pathological features of tumor. PMID:23091673

  19. Outcome in adolescence of brachial plexus birth palsy

    PubMed Central

    Hulleberg, Gunn; Elvrum, Ann-Kristin G; Brandal, Merethe; Vik, Torstein

    2014-01-01

    Background and purpose — The frequency and severity of a permanent lesion after brachial plexus birth palsy (BPBP) and its impact on activities of daily living are not well documented. We therefore investigated the outcome of BPBP in adolescents, regarding arm function and consequences for activity and participation. Participants and methods — Of 30,574 babies born at St. Olavs University Hospital in 1991–2000, 91 had BPBP (prevalence 3 per 1,000), and 69 of these individuals were examined at a median age of 14 (10–20) years. The examination included the modified Mallet classification, range of motion, shoulder rotation and grip strength, Assisting Hand Assessment, and Canadian Occupational Performance Measure. Of the 22 subjects who were not examined, 3 could not be traced and 19 reported having no problems in the affected arm. Results — At follow-up, 17 adolescents had a permanent lesion (i.e. individual Mallet subscore below 4) with a median Mallet total score of 15 (9–19), while 52 had good or normal shoulder function (median Mallet total score 25 (23–25)). All participants with a permanent lesion had reduced active shoulder rotation (≤ 15°), 16 had elbow extension deficit, and 10 had subnormal grip strength. External rotation was considerably weaker in the affected shoulder. In addition, they had ineffective use of the affected arm in bimanual activities. Even so, all except 1 were independent in activities of daily living, although 15 experienced minor difficulties. Interpretation — Every fourth to fifth child with BPBP had a permanent lesion as an adolescent. External rotation was the most impaired movement. Despite ineffective use of the affected arm in bimanual activities, all of the participants except one were independent in activities of daily living. PMID:25238434

  20. Quality of life following traumatic brachial plexus injury: A questionnaire study.

    PubMed

    Gray, Beverley

    2016-08-01

    There is limited qualitative research available that explores the impact of a traumatic brachial plexus injury on patients and their quality of life experiences. This paper builds upon previous work on this subject by this author. Patients were selected from those who were on the database for the Scottish National Brachial Plexus Injury Service between 2011 and 2013. The World Health Organization (WHO) Quality of Life (QoL) - BREF questionnaire was used and 47 questionnaires were distributed with 22 returned. Findings included patients' ratings of their quality of life, physical and psychological health along with their perceived satisfaction with social relationships. PMID:27091305

  1. Hereditary Neuropathy with Liability to Pressure Palsy Presenting as an Acute Brachial Plexopathy: A Lover's Palsy

    PubMed Central

    Wedderburn, Sarah; Pateria, Puraskar; Panegyres, Peter K.

    2014-01-01

    It is generally regarded that patients with hereditary neuropathy to pressure palsies, due to a deletion in the PMP22 gene, show recurrent pressure palsy and generalised peripheral neuropathy (pes cavus and hammer toes sometimes develop). Brachial plexopathy is rarely identified as a first presentation of hereditary neuropathy to pressure palsies. We describe a young man who developed a painless flail upper limb with a clinical diagnosis of a brachial plexopathy after his partner slept on his arm – a PMP22 deletion was found. His father, who had a symmetrical polyneuropathy without recurrent mononeuropathies, shared the PMP22 deletion. PMID:25685136

  2. Brachial plexus injury in two red-tailed hawks (Buteo jamaicensis).

    PubMed

    Shell, L; Richards, M; Saunders, G

    1993-01-01

    Two red-tailed hawks (Buteo jamaicensis), found near Deltaville, Virginia (USA), were evaluated because of inability to use a wing. Results of needle electromyographic studies of the affected wing muscles in both hawks were compatible with denervation. On euthanasia, one hawk had extensive axon and myelin loss with multifocal perivascular lymphocytic inflammation of its brachial plexus and radial nerve. Demyelination and axon loss in the dorsal white matter of the spinal cord on the affected side also were found at the origin of the brachial plexus. The other hawk's wing had not returned to functional status > 2 yr after injury. PMID:8383253

  3. Brachial Artery Aneurysm in a 7-Month-Old Infant: Case Report and Literature Review

    PubMed Central

    Gangopadhyay, Noopur; Chong, Tae; Chhabra, Avneesh

    2016-01-01

    Summary: Congenital upper extremity aneurysms are very rare and can be challenging to diagnose and treat. Although they can present as an isolated finding, they are often associated with other systemic conditions. We present a rare case of brachial artery aneurysm in a 7-month-old boy. The patient was evaluated with ultrasound, magnetic resonance angiography, and vein mapping before surgical reconstruction. After excision of the aneurysm, the brachial artery was reconstructed with an interposition saphenous vein graft. Because of potentially associated diagnoses and the possibility of concurrent aneurysms, this condition requires multidisciplinary management. PMID:27014554

  4. 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. PMID:25042285

  5. Geographic Proximity and Enrollment Competition.

    ERIC Educational Resources Information Center

    Zammuto, Raymond F.

    The use of a measure of geographic proximity to help explain enrollment competition among postsecondary institutions was investigated. The measure, the number of miles between institutions, was obtained by determining the longitude and latitude coordinates for about 99% of the schools in the Higher Education General Information System universe.…

  6. Driven shielding capacitive proximity sensor

    NASA Technical Reports Server (NTRS)

    Vranish, John M. (Inventor); McConnell, Robert L. (Inventor)

    2000-01-01

    A capacitive proximity sensing element, backed by a reflector driven at the same voltage as and in phase with the sensor, is used to reflect the field lines away from a grounded robot arm towards an intruding object, thus dramatically increasing the sensor's range and sensitivity.

  7. Fiber-optic proximity sensor

    NASA Technical Reports Server (NTRS)

    Bejczy, A. K.; Hermann, W. A.; Primus, H. C.

    1980-01-01

    Proximity sensor for mechanical hand of remote manipulator incorporates fiber optics to conduct signals between light source and light detector. Fiber optics are not prone to noise from electromagnetic interference and radio-frequency interference as are sensors using long electrical cables.

  8. Origin of Medial and Lateral Pectoral Nerves from the Supraclavicular Part of Brachial Plexus and its Clinical Importance – A Case Report

    PubMed Central

    Shetty, Prakashchandra; Nayak, Satheesha B; Kumar, Naveen; Thangarajan, Rajesh; D’Souza, Melanie Rose

    2014-01-01

    Knowledge of normal and anomalous formation of brachial plexus and its branches is of utmost importance to anatomists, clinicians, anesthesiologists and surgeons. Possibility of variations in the origin, course and distribution of branches of brachial plexus must be kept in mind during anesthetizing the brachial plexus, mastectomy and plastic surgery procedures. In the current case, the medial pectoral nerve arose directly from the middle trunk of the brachial plexus and the lateral pectoral nerve arose from the anterior division of the upper trunk of the brachial plexus. The lateral pectoral nerve supplied the pectoralis major and the medial pectoral nerve supplied pectoralis major and pectoralis minor muscles through two separate branches. PMID:24701504

  9. Glycosphingolipids are modulators of disease pathogenesis in amyotrophic lateral sclerosis

    PubMed Central

    Dodge, James C.; Treleaven, Christopher M.; Pacheco, Joshua; Cooper, Samantha; Bao, Channa; Abraham, Marissa; Cromwell, Mandy; Sardi, S. Pablo; Chuang, Wei-Lien; Sidman, Richard L.; Cheng, Seng H.; Shihabuddin, Lamya S.

    2015-01-01

    Recent genetic evidence suggests that aberrant glycosphingolipid metabolism plays an important role in several neuromuscular diseases including hereditary spastic paraplegia, hereditary sensory neuropathy type 1, and non-5q spinal muscular atrophy. Here, we investigated whether altered glycosphingolipid metabolism is a modulator of disease course in amyotrophic lateral sclerosis (ALS). Levels of ceramide, glucosylceramide, galactocerebroside, lactosylceramide, globotriaosylceramide, and the gangliosides GM3 and GM1 were significantly elevated in spinal cords of ALS patients. Moreover, enzyme activities (glucocerebrosidase-1, glucocerebrosidase-2, hexosaminidase, galactosylceramidase, α-galactosidase, and β-galactosidase) mediating glycosphingolipid hydrolysis were also elevated up to threefold. Increased ceramide, glucosylceramide, GM3, and hexosaminidase activity were also found in SOD1G93A mice, a familial model of ALS. Inhibition of glucosylceramide synthesis accelerated disease course in SOD1G93A mice, whereas infusion of exogenous GM3 significantly slowed the onset of paralysis and increased survival. Our results suggest that glycosphingolipids are likely important participants in pathogenesis of ALS and merit further analysis as potential drug targets. PMID:26056266

  10. Amyotrophic Lateral Sclerosis: An Emerging Era of Collaborative Gene Discovery

    PubMed Central

    Gwinn, Katrina; Corriveau, Roderick A.; Mitsumoto, Hiroshi; Bednarz, Kate; Brown, Robert H.; Cudkowicz, Merit; Gordon, Paul H.; Hardy, John; Kasarskis, Edward J.; Kaufmann, Petra; Miller, Robert; Sorenson, Eric; Tandan, Rup; Traynor, Bryan J.; Nash, Josefina; Sherman, Alex; Mailman, Matthew D.; Ostell, James; Bruijn, Lucie; Cwik, Valerie; Rich, Stephen S.; Singleton, Andrew; Refolo, Larry; Andrews, Jaime; Zhang, Ran; Conwit, Robin; Keller, Margaret A.

    2007-01-01

    Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease (MND). It is currently incurable and treatment is largely limited to supportive care. Family history is associated with an increased risk of ALS, and many Mendelian causes have been discovered. However, most forms of the disease are not obviously familial. Recent advances in human genetics have enabled genome-wide analyses of single nucleotide polymorphisms (SNPs) that make it possible to study complex genetic contributions to human disease. Genome-wide SNP analyses require a large sample size and thus depend upon collaborative efforts to collect and manage the biological samples and corresponding data. Public availability of biological samples (such as DNA), phenotypic and genotypic data further enhances research endeavors. Here we discuss a large collaboration among academic investigators, government, and non-government organizations which has created a public repository of human DNA, immortalized cell lines, and clinical data to further gene discovery in ALS. This resource currently maintains samples and associated phenotypic data from 2332 MND subjects and 4692 controls. This resource should facilitate genetic discoveries which we anticipate will ultimately provide a better understanding of the biological mechanisms of neurodegeneration in ALS. PMID:18060051

  11. The Puzzling Case of Hyperexcitability in Amyotrophic Lateral Sclerosis

    PubMed Central

    Bae, Jong Seok; Simon, Neil G.; Menon, Parvathi; Vucic, Steve

    2013-01-01

    The development of hyperexcitability in amyotrophic lateral sclerosis (ALS) is a well-known phenomenon. Despite controversy as to the underlying mechanisms, cortical hyperexcitability appears to be closely related to the interplay between excitatory corticomotoneurons and inhibitory interneurons. Hyperexcitability is not a static phenomenon but rather shows a pattern of progression in a spatiotemporal aspect. Cortical hyperexcitability may serve as a trigger to the development of anterior horn cell degeneration through a 'dying forward' process. Hyperexcitability appears to develop during the early disease stages and gradually disappears in the advanced stages of the disease, linked to the destruction of corticomotorneuronal pathways. As such, a more precise interpretation of these unique processes may provide new insight regarding the pathophysiology of ALS and its clinical features. Recently developed technologies such as threshold tracking transcranial magnetic stimulation and automated nerve excitability tests have provided some clues about underlying pathophysiological processes linked to hyperexcitability. Additionally, these novel techniques have enabled clinicians to use the specific finding of hyperexcitability as a useful diagnostic biomarker, enabling clarification of various ALS-mimic syndromes, and the prediction of disease development in pre-symptomatic carriers of familial ALS. In terms of nerve excitability tests for peripheral nerves, an increase in persistent Na+ conductances has been identified as a major determinant of peripheral hyperexcitability in ALS, inversely correlated with the survival in ALS. As such, the present Review will focus primarily on the puzzling theory of hyperexcitability in ALS and summarize clinical and pathophysiological implications for current and future ALS research. PMID:23626643

  12. Dissociated lower limb muscle involvement in amyotrophic lateral sclerosis.

    PubMed

    Simon, Neil G; Lee, Michael; Bae, Jong Seok; Mioshi, Eneida; Lin, Cindy S-Y; Pfluger, Casey M; Henderson, Robert D; Vucic, Steve; Swash, Michael; Burke, David; Kiernan, Matthew C

    2015-06-01

    It has been suggested that corticomotoneuronal drive to ankle dorsiflexors is greater than to ankle plantar flexor muscles, despite the finding that plantar flexors are no less active than TA during walking and standing. The present study was undertaken to determine whether there was differential involvement of distal lower limb muscles in amyotrophic lateral sclerosis (ALS), to elucidate pathophysiological mechanisms of selective muscle involvement. Prospective studies were undertaken in 52 ALS patients, including clinical assessment, disease staging (revised ALS functional rating scale), Medical Research Council sum score, and a scale of upper motor neurone (UMN) dysfunction. Motor unit number estimates (MUNE) and compound muscle action potentials (CMAP) from ankle dorsiflexors and plantar flexors were used to provide objective measures. A novel 'split leg index' was calculated as follows: SLI = CMAPDF ÷ CMAPPF. In ALS, there was significantly greater reduction of MUNE and CMAP amplitude recorded from plantar flexors when compared to dorsiflexors, suggesting preferential involvement of plantar flexor muscles, underpinning a 'split leg' appearance. The SLI correlated with clinical plantar flexor strength (R= -0.56, p < 0.001). In no patient did the SLI suggest preferential dorsiflexor involvement. In subgroup analyses, mean SLI was greatest in lower limb-onset ALS. In conclusion, the present study has established dissociated involvement of muscles acting around the ankle in ALS. We suggest this reflects underlying differences in cortical, descending or local spinal modulation of these muscles. PMID:25845764

  13. Canine degenerative myelopathy: a model of human amyotrophic lateral sclerosis.

    PubMed

    Nardone, Raffaele; Höller, Yvonne; Taylor, Alexandra C; Lochner, Piergiorgio; Tezzon, Frediano; Golaszewski, Stefan; Brigo, Francesco; Trinka, Eugen

    2016-02-01

    Canine degenerative myelopathy (CDM) represents a unique naturally occurring animal model for human amyotrophic lateral sclerosis (ALS) because of similar clinical signs, neuropathologic findings, and involvement of the superoxide dismutase 1 (SOD1) mutation. A definitive diagnosis can only be made postmortem through microscopic detection of axonal degeneration, demyelination and astroglial proliferation, which is more severe in the dorsal columns of the thoracic spinal cord and in the dorsal portion of the lateral funiculus. Interestingly, the muscle acetylcholine receptor complexes are intact in CDM prior to functional impairment, thus suggesting that muscle atrophy in CDM does not result from physical denervation. Moreover, since sensory involvement seems to play an important role in CDM progression, a more careful investigation of the sensory pathology in ALS is also warranted. The importance of SOD1 expression remains unclear, while oxidative stress and denatured ubiquinated proteins appear to play a crucial role in the pathogenesis of CDM. In this updated narrative review we performed a systematic search of the published studies on CDM that may shed light on the pathophysiological mechanisms of human ALS. A better understanding of the factors that determine the disease progression in CDM may be beneficial for the development of effective treatments for ALS. PMID:26432396

  14. Executive dysfunction predicts social cognition impairment in amyotrophic lateral sclerosis.

    PubMed

    Watermeyer, Tamlyn J; Brown, Richard G; Sidle, Katie C L; Oliver, David J; Allen, Christopher; Karlsson, Joanna; Ellis, Catherine M; Shaw, Christopher E; Al-Chalabi, Ammar; Goldstein, Laura H

    2015-07-01

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder of the motor system with recognised extra-motor and cognitive involvement. This cross-sectional study examined ALS patients' performance on measures requiring social inference, and determined the relationship between such changes and variations in mood, behaviour, personality, empathy and executive function. Fifty-five ALS patients and 49 healthy controls were compared on tasks measuring social cognition and executive function. ALS patients also completed measures examining mood, behaviour and personality. Regression analyses explored the contribution of executive function, mood, behaviour and personality to social cognition scores within the ALS sample. A between-group MANOVA revealed that, the ALS group was impaired relative to controls on two composite scores for social cognition and executive function. Patients also performed worse on individual tests of executive function measuring cognitive flexibility, response inhibition and concept formation, and on individual aspects of social cognition assessing the attribution of emotional and mental states. Regression analyses indicated that ALS-related executive dysfunction was the main predictor of social cognition performance, above and beyond demographic variables, behaviour, mood and personality. On at least some aspects of social cognition, impaired performance in ALS appears to be secondary to executive dysfunction. The profile of cognitive impairment in ALS supports a cognitive continuum between ALS and frontotemporal dementia. PMID:25957636

  15. Amyotrophic lateral sclerosis in an Italian professional soccer player.

    PubMed

    Vanacore, Nicola; Binazzi, Alessandra; Bottazzi, Marco; Belli, Stefano

    2006-06-01

    Amyotrophic lateral sclerosis (ALS) is a rare devastating neurodegenerative disease of unknown etiology. Two recent epidemiological studies showed a high risk for ALS among Italian male soccer players. We present the clinical and occupational history of an Italian professional soccer player affected by sporadic ALS. The early onset of ALS (45 years), the bulbar form, the playing position (midfielder) and the duration of the job as professional soccer (17 years) are four characteristics of this patient that are in good agreement with the findings in the previous epidemiological studies. This patient reports the frequent consumption of fructose 1,6 biphosphate, extracts of suprarenal cortex, crotetamide and cropropamide, and dietary supplements (branched chain amino acids and creatine) during his playing career. Some hypotheses have been proposed to explain this high excess of deaths for ALS among soccer players: (a) vigorous physical activity; (b) soccer specific trauma or microtrauma; (c) use of illegal toxic substances or chronic misuse of drugs (most often anti-inflammatory) and dietary supplements; and (d) exposure to pesticides used on playing fields. The overall available clinical and epidemiological evidence supports the possible relation between the specific occupational environment (soccer) and the occurrence of ALS in this patient. PMID:16459125

  16. CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia.

    PubMed

    Williams, Kelly L; Topp, Simon; Yang, Shu; Smith, Bradley; Fifita, Jennifer A; Warraich, Sadaf T; Zhang, Katharine Y; Farrawell, Natalie; Vance, Caroline; Hu, Xun; Chesi, Alessandra; Leblond, Claire S; Lee, Albert; Rayner, Stephanie L; Sundaramoorthy, Vinod; Dobson-Stone, Carol; Molloy, Mark P; van Blitterswijk, Marka; Dickson, Dennis W; Petersen, Ronald C; Graff-Radford, Neill R; Boeve, Bradley F; Murray, Melissa E; Pottier, Cyril; Don, Emily; Winnick, Claire; McCann, Emily P; Hogan, Alison; Daoud, Hussein; Levert, Annie; Dion, Patrick A; Mitsui, Jun; Ishiura, Hiroyuki; Takahashi, Yuji; Goto, Jun; Kost, Jason; Gellera, Cinzia; Gkazi, Athina Soragia; Miller, Jack; Stockton, Joanne; Brooks, William S; Boundy, Karyn; Polak, Meraida; Muñoz-Blanco, José Luis; Esteban-Pérez, Jesús; Rábano, Alberto; Hardiman, Orla; Morrison, Karen E; Ticozzi, Nicola; Silani, Vincenzo; de Belleroche, Jacqueline; Glass, Jonathan D; Kwok, John B J; Guillemin, Gilles J; Chung, Roger S; Tsuji, Shoji; Brown, Robert H; García-Redondo, Alberto; Rademakers, Rosa; Landers, John E; Gitler, Aaron D; Rouleau, Guy A; Cole, Nicholas J; Yerbury, Justin J; Atkin, Julie D; Shaw, Christopher E; Nicholson, Garth A; Blair, Ian P

    2016-01-01

    Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are overlapping, fatal neurodegenerative disorders in which the molecular and pathogenic basis remains poorly understood. Ubiquitinated protein aggregates, of which TDP-43 is a major component, are a characteristic pathological feature of most ALS and FTD patients. Here we use genome-wide linkage analysis in a large ALS/FTD kindred to identify a novel disease locus on chromosome 16p13.3. Whole-exome sequencing identified a CCNF missense mutation at this locus. Interrogation of international cohorts identified additional novel CCNF variants in familial and sporadic ALS and FTD. Enrichment of rare protein-altering CCNF variants was evident in a large sporadic ALS replication cohort. CCNF encodes cyclin F, a component of an E3 ubiquitin-protein ligase complex (SCF(Cyclin F)). Expression of mutant CCNF in neuronal cells caused abnormal ubiquitination and accumulation of ubiquitinated proteins, including TDP-43 and a SCF(Cyclin F) substrate. This implicates common mechanisms, linked to protein homeostasis, underlying neuronal degeneration. PMID:27080313

  17. The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis.

    PubMed

    Loeffler, Jean-Philippe; Picchiarelli, Gina; Dupuis, Luc; Gonzalez De Aguilar, Jose-Luis

    2016-03-01

    Amyotrophic lateral sclerosis (ALS) is a fatal adult-onset disease primarily characterized by upper and lower motor neuron degeneration, muscle wasting and paralysis. It is increasingly accepted that the pathological process leading to ALS is the result of multiple disease mechanisms that operate within motor neurons and other cell types both inside and outside the central nervous system. The implication of skeletal muscle has been the subject of a number of studies conducted on patients and related animal models. In this review, we describe the features of ALS muscle pathology and discuss on the contribution of muscle to the pathological process. We also give an overview of the therapeutic strategies proposed to alleviate muscle pathology or to deliver curative agents to motor neurons. ALS muscle mainly suffers from oxidative stress, mitochondrial dysfunction and bioenergetic disturbances. However, the way by which the disease affects different types of myofibers depends on their contractile and metabolic features. Although the implication of muscle in nourishing the degenerative process is still debated, there is compelling evidence suggesting that it may play a critical role. Detailed understanding of the muscle pathology in ALS could, therefore, lead to the identification of new therapeutic targets. PMID:26780251

  18. Communication and pragmatic breakdowns in amyotrophic lateral sclerosis patients.

    PubMed

    Bambini, Valentina; Arcara, Giorgio; Martinelli, Ilaria; Bernini, Sara; Alvisi, Elena; Moro, Andrea; Cappa, Stefano F; Ceroni, Mauro

    2016-02-01

    While there is increasing attention toward cognitive changes in amyotrophic lateral sclerosis (ALS), the domain of pragmatics, defined as the ability to integrate language and context to engage in successful communication, remains unexplored. Here we tested pragmatic abilities in 33 non-demented ALS patients and 33 healthy controls matched for age and education through 6 different tasks, ranging from discourse organization to the comprehension of figurative language, further grouped in three composite measures for pragmatic production, pragmatic comprehension and global pragmatic abilities. For a subgroup of patients, assessment included executive functions and social cognition skills. ALS patients were impaired on all pragmatic tasks relative to controls, with 45% of the patients performing below cut-off in at least one pragmatic task, and 36% impaired on the global pragmatic score. Pragmatic breakdowns were more common than executive deficit as defined by the consensus criteria, and approximately as prevalent as deficits in social cognition. Multiple regression analyses support the idea of an interplay of executive and social cognition abilities in determining the pragmatic performance, although all these domains show some degree of independence. These findings shed light on pragmatic impairment as a relevant dimension of ALS, which deserves further consideration in defining the cognitive profile of the disease, given its vital role for communication and social interaction in daily life. PMID:26799425

  19. Syntactic Comprehension in Patients with Amyotrophic Lateral Sclerosis

    PubMed Central

    Yoshizawa, Kentarou; Yasuda, Nao; Fukuda, Michinari; Yukimoto, Yumi; Ogino, Mieko; Hata, Wakana; Ishizaka, Ikuyo; Higashikawa, Mari

    2014-01-01

    Recent neuropsychological studies of patients with amyotrophic lateral sclerosis (ALS) have demonstrated that some patients have aphasic symptoms, including impaired syntactic comprehension. However, it is not known if syntactic comprehension disorder is related to executive and visuospatial dysfunction. In this study, we evaluated syntactic comprehension using the Syntax Test for Aphasia (STA) auditory comprehension task, frontal executive function using the Frontal Assessment Battery (FAB), visuospatial function using Raven's Coloured Progressive Matrices (RCPM), and dementia using the Hasegawa Dementia Scale-Revised (HDS-R) in 25 patients with ALS. Of the 25 patients, 18 (72%) had syntactic comprehension disorder (STA score < IV), nine (36%) had frontal executive dysfunction (FAB score < 14), six (24%) had visuospatial dysfunction (RCPM score < 24), and none had dementia (HDS-R score < 20). Nine of the 18 patients with syntactic comprehension disorder (50%) passed the FAB and RCPM. Although sample size was small, these patients had a low STA score but normal FAB and RCPM score. All patients with bulbar onset ALS had syntactic comprehension disorder. These results indicate that it might be necessary to assess syntactic comprehension in patients with bulbar onset ALS. The implications of these findings are discussed in relation to the pathological continuum of ALS. PMID:25161339

  20. Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis.

    PubMed

    Manfredi, Giovanni; Kawamata, Hibiki

    2016-06-01

    Physical and functional interactions between mitochondria and the endoplasmic reticulum (ER) are crucial for cell life. These two organelles are intimately connected and collaborate to essential processes, such as calcium homeostasis and phospholipid biosynthesis. The connections between mitochondria and endoplasmic reticulum occur through structures named mitochondria associated membranes (MAMs), which contain lipid rafts and a large number of proteins, many of which serve multiple functions at different cellular sites. Growing evidence strongly suggests that alterations of ER-mitochondria interactions are involved in neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS), a devastating and rapidly fatal motor neuron disease. Mutations in proteins that participate in ER-mitochondria interactions and MAM functions are increasingly being associated with genetic forms of ALS and other neurodegenerative diseases. This evidence strongly suggests that, rather than considering the two organelles separately, a better understanding of the disease process can derive from studying the alterations in their crosstalk. In this review we discuss normal and pathological ER-mitochondria interactions and the evidence that link them to ALS. PMID:26282323

  1. Deficits in sentence expression in amyotrophic lateral sclerosis.

    PubMed

    Ash, Sharon; Olm, Christopher; McMillan, Corey T; Boller, Ashley; Irwin, David J; McCluskey, Leo; Elman, Lauren; Grossman, Murray

    2015-03-01

    Quantitative examinations of speech production in amyotrophic lateral sclerosis (ALS) are rare. To identify language features minimally confounded by a motor disorder, we investigated linguistic and motor sources of impaired sentence expression in ALS, and we related deficits to gray matter (GM) and white matter (WM) MRI abnormalities. We analyzed a semi-structured speech sample in 26 ALS patients and 19 healthy seniors for motor- and language-related deficits. Regression analyses related grammaticality to GM atrophy and reduced WM fractional anisotropy (FA). Results demonstrated that ALS patients were impaired relative to controls on quantity of speech, speech rate, speech articulation errors, and grammaticality. Speech rate and articulation errors were related to the patients' motor impairment, while grammatical difficulty was independent of motor difficulty. This was confirmed in subgroups without dysarthria and without executive deficits. Regressions related grammatical expression to GM atrophy in left inferior frontal and anterior temporal regions and to reduced FA in superior longitudinal and inferior frontal-occipital fasciculi. In conclusion, patients with ALS exhibit multifactorial deficits in sentence expression. They demonstrate a deficit in grammatical expression that is independent of their motor disorder. Impaired grammatical expression is related to disease in a network of brain regions associated with syntactic processing. PMID:25482157

  2. Transcranial magnetic stimulation and amyotrophic lateral sclerosis: pathophysiological insights

    PubMed Central

    Vucic, Steve; Ziemann, Ulf; Eisen, Andrew; Hallett, Mark; Kiernan, Matthew C

    2013-01-01

    Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder of the motor neurons in the motor cortex, brainstem and spinal cord. A combination of upper and lower motor neuron dysfunction comprises the clinical ALS phenotype. Although the ALS phenotype was first observed by Charcot over 100 years ago, the site of ALS onset and the pathophysiological mechanisms underlying the development of motor neuron degeneration remain to be elucidated. Transcranial magnetic stimulation (TMS) enables non-invasive assessment of the functional integrity of the motor cortex and its corticomotoneuronal projections. To date, TMS studies have established motor cortical and corticospinal dysfunction in ALS, with cortical hyperexcitability being an early feature in sporadic forms of ALS and preceding the clinical onset of familial ALS. Taken together, a central origin of ALS is supported by TMS studies, with an anterograde transsynaptic mechanism implicated in ALS pathogenesis. Of further relevance, TMS techniques reliably distinguish ALS from mimic disorders, despite a compatible peripheral disease burden, thereby suggesting a potential diagnostic utility of TMS in ALS. This review will focus on the mechanisms underlying the generation of TMS measures used in assessment of cortical excitability, the contribution of TMS in enhancing the understanding of ALS pathophysiology and the potential diagnostic utility of TMS techniques in ALS. PMID:23264687

  3. 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. PMID:26408162

  4. Diagnostic timelines and delays in diagnosing amyotrophic lateral sclerosis (ALS).

    PubMed

    Paganoni, Sabrina; Macklin, Eric A; Lee, Alexandra; Murphy, Alyssa; Chang, Judith; Zipf, Amanda; Cudkowicz, Merit; Atassi, Nazem

    2014-09-01

    The objective of this study was to characterize the diagnostic timelines and their predictors in people with amyotrophic lateral sclerosis (ALS). Patients were identified through ALS billing codes. Time from presenting symptom to first doctor visit, first doctor visit to suspected ALS diagnosis, suspected to confirmed ALS diagnosis, and presenting symptom to confirmed ALS diagnosis (total diagnostic time) were collected. Regression models were used to analyze the predictors of diagnostic delay. Three hundred and four ALS patients were included in the analysis. Median total diagnostic time was 11.5 months. Diagnostic timelines were longer in patients with age > 60 years (p < 0.001), sporadic ALS (p = 0.043), and limb onset (p = 0.010). The presence of fasciculations, slurred speech, and lower extremity weakness when symptoms were first noted were independent predictors of shorter time to ALS diagnosis (p = 0.04, p = 0.02, and p = 0.04, respectively). About half of the patients (52%) received an alternative diagnosis and each patient saw an average of three different physicians before ALS diagnosis was confirmed. In conclusion, diagnostic timelines in ALS are long, and patients see many physicians and receive multiple alternative diagnoses before the diagnosis of ALS is confirmed. Older age, sporadic disease, and limb onset can delay ALS diagnosis. PMID:24981792

  5. Identification of new mutations in familial amyotrophic lateral sclerosis

    SciTech Connect

    Siddique, T.; Deng, H.X.; Hentati, A.

    1994-09-01

    Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease due to motor neuron death in the cortex, brain stem and spinal cord. Ten percent of ALS cases are familial (FALS). Previously a subset of FALS families have been mapped to chromosome 21 and mutations in the Cu,Zn superoxide dismutase gene have been identified in those families. Nineteen different mutations at 16 distinct codons have been documented, of which 12 different mutations were identified in our 29 FALS families. These mutations account for about twenty percent of all FALS families screened. The mutations identified in our FALS families are A4V, A4T, G37R, G41D, H43R, G85R, G93A, E100G, L106V, I113T, L144F, and V148G. Mutation A4V is the most frequent one which occurred in 14 out of our 29 FALS families. In further screening of our FALS families, two new mutations, V14M and L84V, have been identified. Thus a total of 21 different mutations at 18 distinct codon sites have been identified in SOD1.

  6. Dynamic markers of altered gait rhythm in amyotrophic lateral sclerosis

    NASA Technical Reports Server (NTRS)

    Hausdorff, J. M.; Lertratanakul, A.; Cudkowicz, M. E.; Peterson, A. L.; Kaliton, D.; Goldberger, A. L.

    2000-01-01

    Amyotrophic lateral sclerosis (ALS) is a disorder marked by loss of motoneurons. We hypothesized that subjects with ALS would have an altered gait rhythm, with an increase in both the magnitude of the stride-to-stride fluctuations and perturbations in the fluctuation dynamics. To test for this locomotor instability, we quantitatively compared the gait rhythm of subjects with ALS with that of normal controls and with that of subjects with Parkinson's disease (PD) and Huntington's disease (HD), pathologies of the basal ganglia. Subjects walked for 5 min at their usual pace wearing an ankle-worn recorder that enabled determination of the duration of each stride and of stride-to-stride fluctuations. We found that the gait of patients with ALS is less steady and more temporally disorganized compared with that of healthy controls. In addition, advanced ALS, HD, and PD were associated with certain common, as well as apparently distinct, features of altered stride dynamics. Thus stride-to-stride control of gait rhythm is apparently compromised with ALS. Moreover, a matrix of markers based on gait dynamics may be useful in characterizing certain pathologies of motor control and, possibly, in quantitatively monitoring disease progression and evaluating therapeutic interventions.

  7. CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia

    PubMed Central

    Williams, Kelly L.; Topp, Simon; Yang, Shu; Smith, Bradley; Fifita, Jennifer A.; Warraich, Sadaf T.; Zhang, Katharine Y.; Farrawell, Natalie; Vance, Caroline; Hu, Xun; Chesi, Alessandra; Leblond, Claire S.; Lee, Albert; Rayner, Stephanie L.; Sundaramoorthy, Vinod; Dobson-Stone, Carol; Molloy, Mark P.; van Blitterswijk, Marka; Dickson, Dennis W.; Petersen, Ronald C.; Graff-Radford, Neill R.; Boeve, Bradley F.; Murray, Melissa E.; Pottier, Cyril; Don, Emily; Winnick, Claire; McCann, Emily P.; Hogan, Alison; Daoud, Hussein; Levert, Annie; Dion, Patrick A.; Mitsui, Jun; Ishiura, Hiroyuki; Takahashi, Yuji; Goto, Jun; Kost, Jason; Gellera, Cinzia; Gkazi, Athina Soragia; Miller, Jack; Stockton, Joanne; Brooks, William S.; Boundy, Karyn; Polak, Meraida; Muñoz-Blanco, José Luis; Esteban-Pérez, Jesús; Rábano, Alberto; Hardiman, Orla; Morrison, Karen E.; Ticozzi, Nicola; Silani, Vincenzo; de Belleroche, Jacqueline; Glass, Jonathan D.; Kwok, John B. J.; Guillemin, Gilles J.; Chung, Roger S.; Tsuji, Shoji; Brown, Robert H.; García-Redondo, Alberto; Rademakers, Rosa; Landers, John E.; Gitler, Aaron D.; Rouleau, Guy A.; Cole, Nicholas J.; Yerbury, Justin J.; Atkin, Julie D.; Shaw, Christopher E.; Nicholson, Garth A.; Blair, Ian P.

    2016-01-01

    Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are overlapping, fatal neurodegenerative disorders in which the molecular and pathogenic basis remains poorly understood. Ubiquitinated protein aggregates, of which TDP-43 is a major component, are a characteristic pathological feature of most ALS and FTD patients. Here we use genome-wide linkage analysis in a large ALS/FTD kindred to identify a novel disease locus on chromosome 16p13.3. Whole-exome sequencing identified a CCNF missense mutation at this locus. Interrogation of international cohorts identified additional novel CCNF variants in familial and sporadic ALS and FTD. Enrichment of rare protein-altering CCNF variants was evident in a large sporadic ALS replication cohort. CCNF encodes cyclin F, a component of an E3 ubiquitin–protein ligase complex (SCFCyclin F). Expression of mutant CCNF in neuronal cells caused abnormal ubiquitination and accumulation of ubiquitinated proteins, including TDP-43 and a SCFCyclin F substrate. This implicates common mechanisms, linked to protein homeostasis, underlying neuronal degeneration. PMID:27080313

  8. Deficits in sentence expression in amyotrophic lateral sclerosis

    PubMed Central

    Ash, Sharon; Olm, Christopher; Mcmillan, Corey T.; Boller, Ashley; Irwin, David J; Mccluskey, Leo; Elman, Lauren; Grossman, Murray

    2015-01-01

    Quantitative examinations of speech production in amyotrophic lateral sclerosis (ALS) are rare. To identify language features minimally confounded by a motor disorder, we investigated linguistic and motor sources of impaired sentence expression in ALS, and we related deficits to gray matter (GM) and white matter (WM) MRI abnormalities. We analyzed a semi-structured speech sample in 26 ALS patients and 19 healthy seniors for motor- and language-related deficits. Regression analyses related grammaticality to GM atrophy and reduced WM fractional anisotropy (FA). Results demonstrated that ALS patients were impaired relative to controls on quantity of speech, speech rate, speech articulation errors, and grammaticality. Speech rate and articulation errors were related to the patients’ motor impairment, while grammatical difficulty was independent of motor difficulty. This was confirmed in subgroups without dysarthria and without executive deficits. Regressions related grammatical expression to GM atrophy in left inferior frontal and anterior temporal regions and to reduced FA in superior longitudinal and inferior frontal-occipital fasciculi. In conclusion, patients with ALS exhibit multifactorial deficits in sentence expression. They demonstrate a deficit in grammatical expression that is independent of their motor disorder. Impaired grammatical expression is related to disease in a network of brain regions associated with syntactic processing. PMID:25482157

  9. Black hairy tongue in a patient with amyotrophic lateral sclerosis.

    PubMed

    Erriu, Matteo; Pili, Francesca Maria Giovanna; Denotti, Gloria; Garau, Valentino

    2016-01-01

    Black hairy tongue (BHT) is a condition characterized by the elongation of filiform papillae associated with a marked discoloration, from yellowish-brown to black, and a thick lingual coating. BHT is usually observed in the elderly and in patients with limited self-sufficiency, as a consequence of poor oral hygiene. In this perspective, the patients affected by amyotrophic lateral sclerosis (ALS) represent a high-risk category for the occurrence of BHT. The fast and inexorable loss of their self-sufficiency due to progressive muscle atrophy as well as the impropriate education of healthcare assistants have demonstrated to have significant reflection on the maintenance of an adequate standard of oral hygiene. This paper firstly described a case of BHT in a patient affected by ALS. A case of BHT in a patient (Caucasic, male, 63 years old) affected by ALS was described. The primary goal of the work was to teach and motivate the patient to the use of the tongue cleaner in association with the local application of chlorexidine 0.20%. Furthermore, in order to support the patient with accurate domiciliary oral hygiene, a proper training for his health-care assistant was provided. The maintenance of the oral health of ALS patient is fundamental to prevent systemic complications that could jeopardize the already fragile physical balance of these patients. The dedicated monitoring by a dentist or a dental hygienist would seem essential in order to achieve this objective. PMID:27011938

  10. How common are behavioural changes in amyotrophic lateral sclerosis?

    PubMed

    Lillo, Patricia; Mioshi, Eneida; Zoing, Margaret C; Kiernan, Matthew C; Hodges, John R

    2011-01-01

    Our objectives were to assess the frequency of behavioural changes in patients with amyotrophic lateral sclerosis (ALS) and to compare the clinical profile of ALS patients with those with behavioural variant frontotemporal dementia (bvFTD). Ninety-two patients with ALS and their carers participated in a postal survey. ALS patients completed self-report measures of motor function and mood. Eighty-one carers of ALS patients and 25 carers of bvFTD patients completed the revised version of the Cambridge Behavioural Inventory (CBI-R). Results showed that reduced motivation was reported in more than 80% of the ALS cases, with almost 41% of them having moderate-severe apathy. Depression was present in 30% of ALS patients and did not contribute significantly to the presence of behavioural symptoms. Bulbar and limb onset ALS patients did not differ. Abnormal behaviour and stereotypical and motor behaviours were present to a moderate-severe degree in around 20%, and 11% reached the criteria for FTD. The rate of behavioural symptoms was significantly higher in the bvFTD group than ALS in all behavioural domains (p <0.001). In conclusion, apathy was the most prominent feature in ALS patients. A substantial proportion of ALS patients manifested behavioural changes of the type seen in FTD, with 11% fulfilling the criteria for FTD. PMID:20849323