Neuromuscular Medicine Core Competencies Outline
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An Improved Technique for Radial Nerve Conduction Studies
J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.30.4.332 on 1 August 1967. Downloaded from J. Neurol. Neurosurg. Psychiat., 1967, 30, 332 An improved technique for radial nerve conduction studies ALLAN W. DOWNIE1 AND THOMAS R. SCOTT From the Division of Neurology, Department of Medicine, School of Medicine, University ofNorth Carolina, Chapel Hill, North Carolina, U.S.A. A technique for recording evoked sensory potentials MATERIALS AND METHOD from the radial nerve has already been reported by us (Downie and Scott, 1964). This technique, The apparatus used was a TECA two-channel electro- myograph. The recording electrodes consisted of a pair although reliable, is time consuming and occasion- of chlorided silver discs 1 cm. in diameter, mounted ally difficult and the amplitude of potentials may be 2 5 cm. apart on a plastic base. The active recording as low as 1 to 2 microvolts. The purpose of this electrode was placed over the largest palpable branch communication is to describe a simpler technique by of the radial nerve as it crossed the tendon of the extensor which potentials of greater amplitude can be pollicis longus. The distal recording electrode was placed obtained. In addition, the segment of nerve tested is over the first dorsal interosseous muscle but not neces- Protected by copyright. one which can be readily identified and biopsied if sarily over the nerve, of which the position in this area so desired without causing unpleasant or disturbing cannot be precisely determined (Fig. 1). An experiment sensory loss to the subject. made to assess the importance of the position of this electrode showed no significant difference in latency to The location of a nerve which is to be tested is peak when it was placed in turn on three points along usually determined by stimulating its motor fibres a line between the tendons of the extensor pollicis and finding the stimulus site from which maximal longus and extensor indicis provided the interelectrode muscle contraction is obtained. -
Neuropathology of Dementia in Patients with Parkinson's Disease: a Systematic Review of Autopsy Studies
Neurodegeneration J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp-2019-321111 on 23 August 2019. Downloaded from REVIEW Neuropathology of dementia in patients with Parkinson’s disease: a systematic review of autopsy studies Callum Smith ,1 Naveed Malek,2 Katherine Grosset,1 Breda Cullen ,3 Steve Gentleman,4 Donald G Grosset1 ► Additional material is ABSTRact have other causes of cognitive impairment and published online only. To view Background Dementia is a common, debilitating dementia, such as comorbid Alzheimer’s disease please visit the journal online (http:// dx. doi. org/ 10. 1136/ feature of late Parkinson’s disease (PD). PD dementia (AD) or cerebrovascular disease. These pathologies jnnp- 2019- 321111). (PDD) is associated with α-synuclein propagation, but may be difficult to identify in vivo, as the clinical coexistent Alzheimer’s disease (AD) pathology may features often overlap with those of PDD, and there 1 Department of Neurology, coexist. Other pathologies (cerebrovascular, transactive are no definitive biomarkers. The differentiation Institute of Neurosciences, response DNA-binding protein 43 (TDP-43)) may of dementia type is an important consideration in Queen Elizabeth University Hospital, Glasgow, UK also influence cognition.W e aimed to describe the clinical research, as treatments under development 2Department of Neurology, neuropathology underlying dementia in PD. are specifically targeted against abnormal accumu- Ipswich Hospital NHS Trust, Methods Systematic review of autopsy studies lation of α-synuclein, which is the pathological Ipswich, UK 3 3 published in English involving PD cases with dementia. hallmark of PDD and DLB, or tau or amyloid-β, Institute of Health and 4 5 Wellbeing, College of Medical, Comparison groups included PD without dementia, AD, which underlie AD. -
A Guide to Transthyretin Amyloidosis
A Guide to Transthyretin Amyloidosis Authored by Teresa Coelho, Bo-Goran Ericzon, Rodney Falk, Donna Grogan, Shu-ichi Ikeda, Mathew Maurer, Violaine Plante-Bordeneuve, Ole Suhr, Pedro Trigo 2016 Edition Edited by Merrill Benson, Mathew Maurer What is amyloidosis? Amyloidosis is a systemic disorder characterized by extra cellular deposition of a protein-derived material, known as amyloid, in multiple organs. Amyloidosis occurs when native or mutant poly- peptides misfold and aggregate as fibrils. The amyloid deposits cause local damage to the cells around which they are deposited leading to a variety of clinical symptoms. There are at least 23 different proteins associated with the amyloidoses. The most well-known type of amyloidosis is associated with a hematological disorder, in which amyloid fibrils are derived from monoclonal immunoglobulin light-chains (AL amyloidosis). This is associated with a clonal plasma cell disorder, closely related to and not uncommonly co-existing with multiple myeloma. Chronic inflammatory conditions such as rheumatoid arthritis or chronic infections such as bronchiectasis are associated with chronically elevated levels of the inflammatory protein, serum amyloid A, which may misfold and cause AA amyloidosis. The hereditary forms of amyloidosis are autosomal dominant diseases characterized by deposition of variant proteins, in dis- tinctive tissues. The most common hereditary form is transthyretin amyloidosis (ATTR) caused by the misfolding of protein monomers derived from the tetrameric protein transthyretin (TTR). Mutations in the gene for TTR frequently re- sult in instability of TTR and subsequent fibril formation. Closely related is wild-type TTR in which the native TTR protein, particu- larly in the elderly, can destabilize and re-aggregate causing non- familial cases of TTR amyloidosis. -
Expert Consensus Recommendations to Improve Diagnosis of ATTR Amyloidosis with Polyneuropathy
Journal of Neurology https://doi.org/10.1007/s00415-019-09688-0 REVIEW Expert consensus recommendations to improve diagnosis of ATTR amyloidosis with polyneuropathy David Adams1 · Yukio Ando2 · João Melo Beirão3 · Teresa Coelho4 · Morie A. Gertz5 · Julian D. Gillmore6 · Philip N. Hawkins6 · Isabelle Lousada7 · Ole B. Suhr8 · Giampaolo Merlini9,10 Received: 10 December 2019 / Revised: 20 December 2019 / Accepted: 23 December 2019 © The Author(s) 2020 Abstract Amyloid transthyretin (ATTR) amyloidosis with polyneuropathy (PN) is a progressive, debilitating, systemic disease wherein transthyretin protein misfolds to form amyloid, which is deposited in the endoneurium. ATTR amyloidosis with PN is the most serious hereditary polyneuropathy of adult onset. It arises from a hereditary mutation in the TTR gene and may involve the heart as well as other organs. It is critical to identify and diagnose the disease earlier because treatments are available to help slow the progression of neuropathy. Early diagnosis is complicated, however, because presentation may vary and family history is not always known. Symptoms may be mistakenly attributed to other diseases such as chronic infammatory demyelinating polyradiculoneuropathy (CIDP), idiopathic axonal polyneuropathy, lumbar spinal stenosis, and, more rarely, diabetic neuropathy and AL amyloidosis. In endemic countries (e.g., Portugal, Japan, Sweden, Brazil), ATTR amyloidosis with PN should be suspected in any patient who has length-dependent small-fber PN with autonomic dysfunction and a family history of ATTR amyloidosis, unexplained weight loss, heart rhythm disorders, vitreous opacities, or renal abnormali- ties. In nonendemic countries, the disease may present as idiopathic rapidly progressive sensory motor axonal neuropathy or atypical CIDP with any of the above symptoms or with bilateral carpal tunnel syndrome, gait disorders, or cardiac hypertro- phy. -
Practical Neurology: Peripheral Neuropathy for the Internist
Practical Neurology: Polyneuropathy for the Non-Neurologist Steven A. Day, MD Providence Neurological Specialties Definitions Foundational Principle: With neurological problems think of LOCALIZATION before SYNDROME Definitions • Neuronopathy – Motor neuronopathy – Sensory neuronopathy • Radiculopathy • Plexopathy • Neuropathy – Mononeuropathy – Polyneuropathy The Netter Collection of Medical Illustrations, Volume 1, Nervous System, 2002 “ROOTS” C4 TRUNKS C5 Dorsal scapular n. C6 TRUNKS C7 Suprascapular n. T1 DIVISIONS Musculocut- aneous n. CCF CORDS 2002 Long thoracic n. TERMINAL NERVES CCF ©2002 Axillary n. Radial n. Median n. Ulnar n. Definitions ‘Neuropathy’ is a diagnosis which specifies the location of pathology, not a symptom Definitions • Axonal = axon loss pathology • Demyelinating = myelin loss pathology Topical Diagnosis in Neurology, 3rd ed. 1998 Topical Diagnosis in Neurology, 3rd ed. 1998 Duss’ Topical Diagnosis in Neurology, 4th ed. 2005 Polyneuropathy Polyneuropathy: Typical Presentation • Insidious onset • Distal (toes, pads of feet) • Gradual progression • Complaints are primarily sensory Polyneuropathy: Key Exam Features • Sensory – Distal gradient of sensory loss • Pin prick or cold • Monofilament • Cotton wisp • Vibration at toes and ankles • Proprioception: toe movements Polyneuropathy: Key Exam Features • Motor – Is there intrinsic foot or hand muscle atrophy? – Weakness pattern • Distal • Proximal and distal • Asymmetric – Able to stand/elevate on toes and heels? Polyneuropathy: Key Exam Features • Reflexes – Distal -
Neuromuscular Disorders Neurology in Practice: Series Editors: Robert A
Neuromuscular Disorders neurology in practice: series editors: robert a. gross, department of neurology, university of rochester medical center, rochester, ny, usa jonathan w. mink, department of neurology, university of rochester medical center,rochester, ny, usa Neuromuscular Disorders edited by Rabi N. Tawil, MD Professor of Neurology University of Rochester Medical Center Rochester, NY, USA Shannon Venance, MD, PhD, FRCPCP Associate Professor of Neurology The University of Western Ontario London, Ontario, Canada A John Wiley & Sons, Ltd., Publication This edition fi rst published 2011, ® 2011 by Blackwell Publishing Ltd Blackwell Publishing was acquired by John Wiley & Sons in February 2007. Blackwell’s publishing program has been merged with Wiley’s global Scientifi c, Technical and Medical business to form Wiley-Blackwell. Registered offi ce: John Wiley & Sons Ltd, The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK Editorial offi ces: 9600 Garsington Road, Oxford, OX4 2DQ, UK The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK 111 River Street, Hoboken, NJ 07030-5774, USA For details of our global editorial offi ces, for customer services and for information about how to apply for permission to reuse the copyright material in this book please see our website at www.wiley.com/wiley-blackwell The right of the author to be identifi ed as the author of this work has been asserted in accordance with the UK Copyright, Designs and Patents Act 1988. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the publisher. -
CAP-1002 for Advanced DMD: a New Treatment Option
CAP-1002 for Advanced DMD: A New Treatment Option October 24, 2019 NASDAQ: CAPR Forward-Looking Statements Statements in this press release regarding the efficacy, safety, and intended utilization of Capricor's product candidates; the initiation, conduct, size, timing and results of discovery efforts and clinical trials; the pace of enrollment of clinical trials; plans regarding regulatory filings, future research and clinical trials; regulatory developments involving products, including the ability to obtain regulatory approvals or otherwise bring products to market; plans regarding current and future collaborative activities and the ownership of commercial rights; scope, duration, validity and enforceability of intellectual property rights; future royalty streams, revenue projections; expectations with respect to the expected use of proceeds from the recently completed offerings and the anticipated effects of the offerings, and any other statements about Capricor's management team's future expectations, beliefs, goals, plans or prospects constitute forward-looking statements within the meaning of the Private Securities Litigation Reform Act of 1995. Any statements that are not statements of historical fact (including statements containing the words "believes," "plans," "could," "anticipates," "expects," "estimates," "should," "target," "will," "would" and similar expressions) should also be considered to be forward- looking statements. There are a number of important factors that could cause actual results or events to differ materially from those indicated by such forward-looking statements. More information about these and other risks that may impact Capricor's business is set forth in Capricor's Annual Report on Form 10-K for the year ended December 31, 2018 as filed with the Securities and Exchange Commission on March 29, 2019, and as amended by its Amendment No. -
The Usefulness of Proximal Radial Motor Conduction in Acute Compressive Radial Neuropathy
JCN Open Access ORIGINAL ARTICLE pISSN 1738-6586 / eISSN 2005-5013 / J Clin Neurol 2015;11(2):178-182 / http://dx.doi.org/10.3988/jcn.2015.11.2.178 The Usefulness of Proximal Radial Motor Conduction in Acute Compressive Radial Neuropathy Kun Hyun Kima b Background and PurposezzThe objective of this study was to determine diagnostic and Kee-Duk Park a prognostic values of proximal radial motor conduction in acute compressive radial neuropathy. Pil-Wook Chung a MethodszzThirty-nine consecutive cases of acute compressive radial neuropathy with radial Heui-Soo Moon conduction studies–including stimulation at Erb’s point–performed within 14 days from a Yong Bum Kim clinical onset were reviewed. The radial conduction data of 39 control subjects were used as a Won Tae Yoon reference data. b Hyung Jun Park ResultszzThirty-one men and eight women (age, 45.2±12.7 years, mean±SD) were enrolled. Bum Chun Suha All 33 patients in whom clinical follow-up data were available experienced complete recov- ery, with a recovery time of 46.8±34.3 days. Partial conduction block was found frequently a Department of Neurology, Kangbuk Samsung Hospital, (17 patients) on radial conduction studies. The decrease in the compound muscle action po- Sungkyunkwan University tential area between the arm and Erb’s point was an independent predictor for recovery time. School of Medicine, Seoul, Korea zzProximal radial motor conduction appears to be a useful method for the early b Conclusions Department of Neurology, detection and prediction of prognosis of acute compressive radial neuropathy. Mokdong Hospital, Ewha Womans University Key Wordszz radial neuropathy, nerve conduction study, conduction block, diagnosis, School of Medicine, Seoul, Korea prognosis. -
Internet Resources for Neurosurgeons and Neuropathologists S Thomson, N Phillips
154 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.74.2.154 on 1 February 2003. Downloaded from REVIEW Internet resources for neurosurgeons and neuropathologists S Thomson, N Phillips ............................................................................................................................. J Neurol Neurosurg Psychiatry 2003;74:154–157 Neurosurgical and neuropathological resources on the Neurosurgery://On-call has an “image bank” internet are rapidly developing. Some excellent clinical, section, which has an excellent downloadable collection of radiographs. There are also some patient information, professional, academic, and photographic images. This is a rapidly developing teaching web sites are available. This review database, easy to search, and likely to have images summarises the most useful online sites for relevant to most neurosurgical and neuropatho- logical conditions. neurosurgeons and neuropathologists in the United Neuroanatomy and Neuropathology on the Kingdom and beyond. More general internet resources Internet provides a very comprehensive collection of pathological images within the online neu- have been covered in the first article in this series. ropathology atlas. The neuroanatomy structures .......................................................................... subsection shows the locations of anatomical structures within normal pathological specimens. ver the past 10 years the internet has The functional neuroanatomy tables are also expanded rapidly. While potential ben- highly -
A Case of Bickerstaff S Brainstem Encephalitis in Childhood '
View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Directory of Open Access Journals Korean Journal of Pediatrics Vol. 53, No. 4, 2010 DOI : 10.3345/kjp.2010.53.4.607 Case report 1)jtj A case of Bickerstaff’s brainstem encephalitis in childhood Ji Youn Kim, M.D., Young Ok Kim, M.D., Young Jun Son, M.D. and Young Jong Woo, M.D. Department of Pediatrics, Chonnam National University Medical School, Gwangju, Korea = Abstract = Bickerstaff's brainstem encephalitis (BBE) is a rare disease diagnosed by specific clinical features such as 'progressive, relatively symmetric external ophthalmoplegia and ataxia by 4 weeks' and 'disturbance of consciousness or hyperreflexia' after the exclusion of other diseases involving the brain stem. Anti-ganglioside antibodies (GM, GD and GQ) in the serum or cerebrospinal fluid (CSF) are sometimes informative for the diagnosis of BBE because of the rarity of positive findings in other diagnositic methods: brain magnetic resonance imaging (MRI), routine CSF examination, motor nerve conduction study, and needle electromyography. We report a rare case of childhood BBE with elevated anti-GM1 antibodies in the serum, who had specific clinical symptoms such as a cranial polyneuropathy presenting as ophthalmoplegia, dysarthria, dysphagia, and facial weakness; progressive motor weakness; altered mental status; and ataxia. However, the brain MRI, routine CSF examination, nerve conduction studies, electromyography, somatosensory evoked potentials, and brainstem auditory evoked potentials were normal. BBE was suspected and the patient was successfully treated with intravenous immunoglobulins. (Korean J Pediatr 2010;53:607-611) Key Words : Encephalitis, Brain stem, Child bulins (IVIG) for the treatment of BBE suggests an auto- Introduction immune etiology5, 6). -
Neuromuscular Disorders of Infancy, Childhood, and Adolescence
Chapter 1 Introduction: Historical Perspectives Darryl C. De Vivo, Basil T. Darras, Monique M. Ryan, and H. Royden Jones, Jr. INTRODUCTION infancy, childhood, and adolescence. Nevertheless, these traditional tests remain useful in certain situations: to The role of the clinician in the diagnosis and treatment of evaluate patients in whom molecular genetic analyses fail a weak child is as important today as it was in the 19th to confirm the initial clinical impression, to facilitate the century, when pediatric neuromuscular diseases were first 1 rapid electrophysiologic diagnosis of a neuropathy or being recognized by such luminaries as Meryon (1852), spinal muscular atrophy, or to focus more precisely on Duchenne (1861),2 Werdnig (1891),3 and Hoffmann 4 5 molecular mechanisms before ordering relatively expen- (1893), and later by Batten (1903). The clinician needs sive molecular genetic tests. The dramatic advances in to react to the concerns of the patient and their parents at DNA diagnostics have added to the complexity of a chal- the first encounter. A detailed medical history and care- lenging clinical field and have left physicians occasion- fully performed physical examination remain the funda- ally uncertain about the relative indications for traditional mental tools and starting point for assessing various tests such as EMG and muscle biopsy. Clearly, all these symptoms and signs. This initial clinical assessment has diagnostic tests remain useful, and it is up to the modern three possible immediate outcomes: (1) the concerns of clinician to make informed decisions, after the initial clin- the patient and family appear to be unfounded, and the ical evaluation, to facilitate an accurate biomolecular clinician can be reassuring. -
Peripheral Polyneuropathy in Patients Receiving Long-Term Statin Therapy
552 Turk Kardiyol Dern Ars 2019;47(7):552-553 doi: 10.5543/tkda.2019.52735 Invited Editorial / Davetli Editöryal Yorum Peripheral polyneuropathy in patients receiving long-term statin therapy Uzun dönem statin kullanan hastalarda periferik polinöropati gelişimi Öner Özdoğan, M.D. Department of Cardiology, Tepecik Training and Research Hospital, İzmir, Turkey Although drug-induced neuropathies (DIN) are not an enzyme which could Abbreviation: very common, they are one of the main reasons of pe- alter the neurons’ energy DIN Drug-induced neuropathies ripheral neuropathies.[1] DIN cause to sensory, motor, utilization.[6] On the con- ENMG Electroneuromyography and autonomic dysfunctions depending on the type of SAMs Statin-associated muscle trary; some animal stu- symptoms the peripheral nerve involvement. As significant re- dies have reported that covery could be observed after discontinuation of the statins provided a neuroprotective effect against pe- causal agent drug, early diagnosis is important. How- ripheral nerve injury.[7] In a recent Danish case-control ever, symptoms of DIN are usually seen after months study, use of statins in 370 cases, was not associated [2] or years of exposure. Therefore, defining the causal with an elevated risk of polyneuropathy. Similarly, no relationship between the drugs and long term side ef- association was observed between polyneuropathy fects like drug-induced peripheral neuropathies is not risk and long-term high-intensity statin.[8] easy always, and commonly missed. Electrodiagnos- tic tests are the most important methods to confirm In this issue of the Archives of Turkish Society tthe peripheral neuropathy.[3] We classify the type of of Cardiology, Ozdemir et al.