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MND Pathophysiology and Genetics Rehab Aspects of Treatment, CIDP, and Related Disorders

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MND Pathophysiology and Genetics Rehab Aspects of Treatment, CIDP, and Related Disorders NM Update I: MND Pathophysiology and Genetics Rehab Aspects of Treatment, CIDP, and Related Disorders Mark B. Bromberg, MD, PhD Nanette C. Joyce, DO Thomas H. Brannagan, MD Richard A. Lewis, MD Dianna Quan, MD W. David Arnold, MD Eduardo A. DeSousa, MD AANEM 59th Annual Meeting Orlando, Florida Copyright © September 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 NM Update I: MND Pathophysiology and Genetics Rehab Aspects of Treatment, CIDP, and Related Disorders Table of Contents Course Committees & Course Objectives 4 Faculty 5 Motor Neuron Disease Pathophysiology and Genetics Update: Rehabilitative Aspects of Treatment 7 Mark B. Bromberg, MD, PhD Nanette C. Joyce, DO Chronic Inflammatory Demyelinating Polyradiculoneuropathy 17 Thomas H. Brannagan, III, MD Multifocal Variants of Chronic Inflammatory Demyelinating Polyradiculoneuropathy, Lewis-Sumner Syndrome, and Multifocal Motor Neuropathy 21 Richard A. Lewis MD Neuromuscular Vignettes 25 Dianna Quan, MD W. David Arnold, MD Eduardo De Sousa,MD No one involved in the planning of this CME activity had any relevant financial relationships to disclose. Dr. Lewis is a consultant for CLS Behring, MNS Pharmaceuticals, and Axelacare and received a grant from Baxter. Any conflict of interest has been resolved according to ACCME standards. Chair: Dianna Quan, 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) utilize a pattern recognition approach elucidated through clinical vignettes in the diagnosis and management of patients with MNDs, (2) recognize clinical and electrophysiological features of CIDP and related disorders and become familiar with treatment options, and (3) practice the vignette-based format used for many questions on the NM medicine board examination. 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 4 NM Update I: MND Pathophysiology and Genetics Rehab Aspects of Treatment, CIDP, and Related Disorders Faculty Mark B Bromberg, MD, PhD at Columbia-Presbyterian. He is the medical advisor for the Department of Neurology Neuropathy Association, on the Muscle & Nerve editorial board, University of Utah associate editor of BMC Neurology, and ad hoc reviewer for 30- Salt Lake City, Utah plus publications, including the New England Journal of Medicine and Neurology. Dr. Brannagan has authored or co-authored Dr. Bromberg received a doctorate in neurophysiology at the almost 60 peer-reviewed articles and more than 70 abstracts and University of Vermont and was on the faculty at the University of contributed to more than 20 books. His major interest is the Michigan, where he then attended medical school and completed evaluation and treatment of peripheral neuropathies, particularly his neurology residency and a neuromuscular-EMG fellowship. immune-mediated neuropathies. He now is a professor in the Department of Neurology at the University of Utah and serves as the director of the MDA Clinics Richard A. Lewis, MD there. Dr. Bromberg’s clinical interests focus on nerve and muscle Department of Neurology disorders, in particular ALS. His research interests are in ALS Wayne State University clinical trials and quality of life assessments. He also is interested Detroit, Michigan in new EMG techniques to facilitate the diagnosis of ALS and in following the course of the disease. Dr. Lewis is professor and associate chair of neurology at Wayne State University, moving soon to a position at Cedars-Sinai Nanette C. Joyce, DO Medical Center in Los Angeles. He received his neurology Department of Physical Medicine and Rehabilitation training at the University of Pennsylvania. Dr. Lewis’ interests are University of California-Davis in the clinical and electrophysiologic consequences of peripheral Sacramento, CA. nerve demyelination as it relates to inflammatory and inherited neuropathies. Multifocal sensorimotor demyelinating neuropathy Dr. Joyce is an assistant professor in the Department of Physical with persistent conduction block now is known as the Lewis- Medicine and Rehabilitation at the University of California, Sumner Syndrome, due to his paper on this topic written with Davis (UC-Davis) School of Medicine. She is board certified Drs. Austin Sumner, Mark Brown, and Arthur Asbury. Dr. Lewis in both physical medicine and rehabilitation and neuromuscular has been involved in multiple investigations of CIDP, multifocal medicine. She completed her medical degree at Touro University motor neuropathy, and other immune-mediated neuromuscular College of Osteopathic Medicine, her PMR residency at Michigan disorders and was the principal investigator on the first multicenter State University, and her fellowships at UC Davis. Dr. Joyce has North American trial in CMT-1A. authored or co-authored 12 publications. Her research focuses on developing stem cell therapies for treatment of ALS, for which W. David Arnold, MD she is supported by a K12 early career development award Department of Neurology through the Association of Academic Physiatrist and the National The Ohio State University Institutes of Health. Columbus, Ohio Thomas H. Brannagan III, MD Dr. Arnold is an assistant professor of neurology in the Department of Neurology Neuromuscular Medicine Division at Ohio State University. He Columbia University received his medical degree from the University of Louisville and New York, New York continued his training there with a residency in physical medicine and rehabilitation. He completed fellowship training in clinical Dr. Brannagan is a professor of neurology, director of the neuromuscular medicine in the Neurology Department at The Peripheral Neuropathy Center at Columbia University, and Ohio State University. His main clinical and research interests co-director of the EMG Laboratory at Columbia’s New York- include channelopathies, neuromuscular junction disorders, spinal Presbyterian Hospital. He earned his medical degree at the muscular atrophy, and less commonly used neurophysiological University of Virginia and completed his neurology residency and techniques such as single fiber and quantitative EMG. EMG, neuromuscular disease, and neuroimmunology fellowships 5 Dianna Quan, MD Department of Neurology University of Colorado Denver Aurora, Colorado Dr. Quan received her medical degree from Columbia University and her neurology residency and neuromuscular disorders and electrodiagnosis fellowship training at the University of Pennsylvania. She is currently an associate professor of neurology at the University of Colorado Denver School of Medicine in Aurora, where she is program director for the neuromuscular medicine fellowship and director of the EMG laboratory. She has been an active member of the AANEM and the American Academy of Neurology and serves as a medical editor for the online e-Medicine Textbook of Neurology and a frequent ad hoc reviewer for Muscle & Nerve. Her interests include peripheral neuropathy, amyotrophic lateral sclerosis, and postherpetic neuralgia.
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