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Basics with the Experts Basics With The Experts John D. England, MD Jun Kimura, MD Vern C. Juel, MD Bassam A. Bassam, MD AANEM 59th Annual Meeting Orlando, Florida Copyright © October 2012 American Association of Neuromuscular & Electrodiagnostic Medicine 2621 Superior Drive NW Rochester, MN 55901 Printed by Johnson Printing Company, Inc. 1 Please be aware that some of the medical devices or pharmaceuticals discussed in this handout may not be cleared by the FDA or cleared by the FDA for the specific use described by the authors and are “off-label” (i.e., a use not described on the product’s label). “Off-label” devices or pharmaceuticals may be used if, in the judgment of the treating physician, such use is medically indicated to treat a patient’s condition. Information regarding the FDA clearance status of a particular device or pharmaceutical may be obtained by reading the product’s package labeling, by contacting a sales representative or legal counsel of the manufacturer of the device or pharmaceutical, or by contacting the FDA at 1-800-638-2041. 2 Basics With The Experts Table of Contents Course Committees & Course Objectives 4 Faculty 5 The Evaluation of Polyneuropathy 7 John D. England, MD Long and Short of Nerve Conduction Studies for Neuropathy 13 Jun Kimura, MD Repetitive Nerve Stimulation Testing 19 Vern C. Juel, MD Muscle Cramps and Hyperactivity Syndromes 25 Bassam A. Bassam, MD CME Questions 31 No one involved in the planning of this CME activity had any relevant financial relationships to disclose. Authors/faculty have nothing to disclose Chair: John D. England, MD The ideas and opinions expressed in this publication are solely those of the specific authors and do not necessarily represent those of the AANEM. 3 Objectives Objectives - Participants will acquire skills to: (1) indicate the laboratory tests which have the highest yield in the evaluation of peripheral neuropathy, (2) identify the NCSs which are most useful in the evaluation of peripheral neuropathy, (3) describe the technique of repetitive nerve stimulation and the differences between pre-synaptic and post-synaptic disorders of NM transmission, and (4) recognize the EMG characteristics, CLINICAL USEFULNESS AND SYNDROMES of nerve and muscle hyperactivity. Target Audience: • Neurologists, physical medicine and rehabilitation and other physicians interested in neuromuscular and electrodiagnostic medicine • Health care professionals involved in the management of patients with neuromuscular diseases • Researchers who are actively involved in the neuromuscular and/or electrodiagnostic research Accreditation Statement - The AANEM is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education (CME) for physicians. CME Credit - The AANEM designates this live activity for a maximum of 3.25 AMA PRA Category 1 CreditsTM. If purchased, the AANEM designates this enduring material for a maximum of 5.75 AMA PRA Category 1 CreditsTM. This educational event is approved as an Accredited Group Learning Activity under Section 1 of the Framework of Continuing Professional Development (CPD) options for the Maintenance of Certification Program of the Royal College of Physicians and Surgeons of Canada. Physicians should claim only the credit commensurate with the extent of their participation in the activity. CME for this course is available 10/2012 - 10/2015. CEUs Credit - The AANEM has designated this live activity for a maximum of 3.25 AANEM CEUs. If purchased, the AANEM designates this enduring material for a maximum of 5.75 CEUs. 2011-2012 Course Committee Shawn J. Bird, MD, Chair Shashi B. Kumar, MD Marcy C. Schlinger, DO Philadelphia, PA Tacoma, WA Bath, MI Lawrence W. Frank, MD A. Arturo Leis, MD Nizar Souayah, MD Elmhurst, IL Jackson, MS Westfield, NJ Taylor B. Harrison, MD Benjamin S. Warfel, II, MD Atlanta, GA Lancaster, PA 2011-2012 AANEM President John C. Kincaid, MD Indianapolis, IN Basics With The Experts Faculty John D. England, MD Vern C. Juel, MD Department of Neurology Division of Neurology LSUHSC School of Medicine Duke University School of Medicine New Orleans, Louisiana Durham, North Carolina Dr. England is the Grace Benson Professor and Chairman of the Dr. Juel completed neurology residency and neuromuscular Department of Neurology at the LSUHSC School of Medicine fellowship training at Duke University Medical Center. He is in New Orleans. He is an author or co-author of more than 200 currently an Associate Professor in the Division of Neurology at publications. He has served on many AANEM committees and Duke where he directs the Neuromuscular Medicine Fellowship was the Secretary-Treasurer from 2005-2008 and the President Program. Dr. Juel is board certified in neurology, clinical from 2009-2010. He is currently the Chairman of the Guidelines neurophysiology, neuromuscular medicine, and electrodiagnostic Development Subcommittee of the American Academy of medicine. He has served on several AANEM committees and Neurology. His major interests are the pathophysiology and has chaired the Graduate Medical Education and Neuromuscular clinical assessment of peripheral neuropathies. Medicine Self-Examination Committees. His areas of academic interest include neuromuscular junction disorders, Jun Kimura, MD chemodenervation for focal dystonia, and neurological education. Department of Neurology University of Iowa Health Care Bassam A. Bassam, MD Iowa City, Iowa Neuromuscular Program and EMG Laboratory University of South Alabama Dr. Kimura received his medical degree from Kyoto University Mobile, Alabama in Japan. He moved to the United States as a Fulbright scholar for residency training in neurology and a fellowship in Dr. Bassam completed residency training in neurology and a electrophysiology at the University of Iowa, where he now serves fellowship in neuromuscular disease at Wayne State University, as Professor of Neurology. He also has taught at the University with additional fellowship training at Mayo Clinic. He is board of Manitoba in Canada, Kyoto University in Japan, and Tiantan certified by the American Board of Psychiatry and Neurology and Hospital in China. Dr. Kimura has more than 500 original the American Board of Electrodiagnostic Medicine (ABEM) and publications, including four editions of his book, Electrodiagnosis is a diplomate in the neuromuscular medicine sub-specialty. Dr. in Diseases of Nerve and Muscle. Dr. Kimura has received Bassam has served on various AANEM committees, including honorary membership from 25 national societies of neurology, chair of the Workshop Committee; he current serves on the ABEM neurophysiology, and rehabilitation medicine. Examination Committee. His academic interests and achievements focus on neuromuscular disorders and electromyography. 5 6 BASICS WITH THE EXPERTS The Evaluation of Polyneuropathy John D. England, MD The Grace Benson Professor and Head Department of Neurology Louisiana State University Health Sciences Center School of Medicine New Orleans, Louisiana Amparo Gutierrez, MD Associate Professor Department of Neurology Louisiana State University Health Sciences Center School of Medicine New Orleans, Louisiana INTRODUCTION The physician should explore for the presence of other medical conditions, medications which the patient takes (including Peripheral neuropathies are common in neurological practice, nonprescription dietary supplements and vitamins), and and there are a variety of clinical presentations. Through a exposure to toxins such as alcohol, heavy metals, and solvents. combination of clinical findings, electrodiagnostic (EDX) It is important to determine the rate of symptom progression, the studies, and laboratory investigations tailored to the individual pattern of symptoms/signs, and the type of fibers (large versus patients’ circumstances, most neuropathies can be categorized small fibers) involved. The most common clinical phenotype is by subtype and etiology. Such classification is important for that of slowly evolving distal sensory loss with varying degrees determining prognosis and treatment. of muscle weakness, and occasionally autonomic symptoms. The most common variety of polyneuropathy is a chronic distal EPIDEMIOLOGY symmetric polyneuropathy.13-16,29 In this type of polyneuropathy, nerve fibers are involved in a “length-dependent” fashion; as The overall prevalence of polyneuropathy is approximately 2,400 such, symptoms usually begin in the toes/distal feet and spread per 100,000 (2.4%) people, but for those older than 55 years centripetally. Sensory symptoms usually precede motor weakness. the prevalence rises to at least 8,000 per 100,000 (8%).13,19,26 Myotatic reflexes are also affected in a length-dependent manner In the developed, world the most common cause of peripheral such that ankle reflexes are depressed or absent prior to the loss neuropathy is diabetes mellitus (DM) and prediabetes.6 A of other reflexes. The clinical picture is usually one of a stocking- community-based study estimated the prevalence of peripheral glove sensory loss, distal wasting and weakness, and depressed neuropathy in patients with type 2 DM to be 26.4%.9 Although or absent myotatic reflexes. This is an easily recognizable entity. not commonly seen in the United States, in some parts of the world, such as Southeast Asia, India, and Africa, leprosy is still a Another form of neuropathy that is frequently encountered major cause of peripheral neuropathy. is a painful small fiber neuropathy.13 These patients typically complain of burning pain, lancinating
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