Neuromuscular Update I Kerry H. Levin MD Steven J. Shook MD Greg T. Carter, MD, MS Dianna Quan, MD James W. Teener, MD Mohammad K. Salajegheh, MD Anthony A. Amato, MD 2009 Neuromuscular Update Course C AANEM 56th Annual Meeting San Diego, California Copyright © October 2009 American Association of Neuromuscular & Electrodiagnostic Medicine 2621 Superior Drive NW Rochester, MN 55901 Printed by Johnson Printing Company, Inc. ii Neuromuscular Update I Faculty Anthony A. Amato, MD Kerry H. Levin, MD Department of Neurology Chairman, Department of Neurology Brigham and Women’s Hospital Director, Neuromuscular Center Harvard Medical School Cleveland Clinic Boston, Massachusetts Cleveland, Ohio Dr. Amato is the vice-chairman of the department of neurology and the Dr. Levin received his bachelor’s degree and his medical degree from Johns director of the neuromuscular division and clinical neurophysiology labo- Hopkins University in Baltimore, Maryland. He then performed a resi- ratory at Brigham and Women’s Hospital (BWH/MGH) in Boston. He is dency in internal medicine at University Hospitals of Cleveland, followed also professor of neurology at Harvard Medical School. He is the director by a neurology residency at the University of Chicago Hospitals, where he of the Partners Neuromuscular Medicine fellowship program. Dr. Amato served as chief resident. He is currently chairman of the Department of is an author or co-author on over 150 published articles, chapters, and Neurology and director of the Neuromuscular Center at Cleveland Clinic. books. He co-wrote the textbook Neuromuscular Disorders with Dr. James Dr. Levin is also professor of medicine at the Cleveland Clinic College Russell. He has been involved in clinical research trials involving patients of Medicine of Case Western Reserve University. His current research with amyotrophic lateral sclerosis, peripheral neuropathies, neuromuscular interests include electromyography, radiculopathy, myasthenia gravis, and junction disorders, and myopathies. polyneuropathy. Gregory T. Carter, MD, MS Dianna Quan, MD Professor of Rehabilitation Medicine Associate Professor University of Washington School of Medicine Department of Neurology Seattle, Washington University of Colorado Denver School of Medicine Dr. Carter is professor of rehabilitation medicine at the University of Aurora, Colorado Washington, where he helped found and now co-directs the Muscular Dr. Quan received her undergraduate degree from the University of Dystrophy Association/Amyotrophic Lateral Sclerosis Center. His clinical Chicago and her medical degree from Columbia University College of research focuses on improving rehabilitative and palliative management of Physicians and Surgeons. She completed her neurology residency and patients with neuromuscular disease (NMD), emphasizing function and fellowship training in neuromuscular disorders and electrodiagnosis at the quality of life. His pre-clinical research involves characterizing the electro- Hospital of the University of Pennsylvania. She is currently an associate physiology of mutant and transgenic murine models of NMD. He has professor of neurology at the University of Colorado Denver School of over 100 peer-reviewed publications in these areas. Medicine located in Aurora, Colorado, where she is program director of the neuromuscular medicine fellowship and director of the EMG labora- tory. She has been an active member of the AAN and AANEM and serves as a medical editor for the online e-Medicine Textbook of Neurology and a frequent ad hoc reviewer for Muscle & Nerve, among other journals. Her interests include peripheral neuropathy, ALS, and postherpetic neuralgia. No one involved in the planning of this CME activity had any relevant financial relationships to disclose. Authors/faculty have nothing to disclose. Course Chair: Gil I. Wolfe, 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. iii Mohammad K. Salajegheh, MD James W. Teener, MD Department of Neurology Director Brigham and Women’s Hospital Neuromuscular Program Harvard Medical School Department of Neurology Boston, Massachusetts University of Michigan Health System Dr. Salajegheh received his medical degree from Tehran University of Ann Arbor, Michigan Medical Sciences in 1995 and completed his training in neurology at Dr. Teener is director of the neuromuscular program, co-director of the the University of Massachusetts Medical School in 2003. He finished EMG laboratory, and associate professor at the University of Michigan. four years of fellowship training in neuromuscular disease, two years He completed his residency in neurology and a neuromuscular fellowship at the National Institutes of Health and another two years at Brigham at the University of Pennsylvania. His research interests include neuro- and Women's hospital, and is board certified by the American Board of muscular disorders associated with critical illness and intensive care treat- Medical Specialties in both Neurology and Neuromuscular Medicine. He ment of severe neuromuscular disease. His clinical interests include the full is currently a member of the faculty at Harvard Medical School and a spectrum of neuromuscular disease. He collaborates widely and provides staff member in the Department of Neurology at Brigham and Women's electrodiagnostic expertise to a wide variety of research projects. Hospital. He is actively involved in clinical research on muscle channelo- pathies as well as both clinical and laboratory based research focused on studying the mechanisms of inflammatory myopathies. Steven J. Shook MD Staff Neuromuscular Center Cleveland Clinic Cleveland, Ohio Dr. Shook graduated summa cum laude from Ohio State University College of Medicine and Public Health in 2002. He completed an intern- ship in internal medicine at MetroHealth Medical Center in Cleveland, Ohio, followed by a neurology residency at the Cleveland Clinic, where he served as chief resident. After completing a fellowship in neuromuscular medicine he joined the Neuromuscular Center at Cleveland Clinic as staff in 2007. His specialty interests include electromyography, ultrasound of peripheral nerve and muscle, magnetic resonance neurography, peripheral nerve injury, entrapment neuropathies, and peripheral nerve tumors. iv 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 judgement of the treating physician, such use is medically indi- cated 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. v Neuromuscular Update I Contents Faculty ii Objectives v Course Committee vi Mononeuropathy: Postoperative Foot Drop 1 Kerry H. Levin MD; Steven J. Shook MD; Greg T. Carter, MD, MS Systemic Disease and Neuromuscular Disorders 7 Dianna Quan, MD; James W. Teener, MD Myotonic Disorders: Patient With Muscle Stiffness and Episodic Weakness 17 Mohammad K. Salajegheh, MD; Anthony A. Amato, MD O BJECTIVES — The overall objective of this two-part neuromuscular update course is to present participants with clinical cases in neuromuscular diseases. After attending both sessions, participants will learn how to diagnose, evaluate, and in some instances treat neuromuscular diseases. This course is an excellent review of neuromuscular medicine. Update I covers focal mononeuropathy, systemic disease and neuromuscular disorders, and exercise-induced cramps. P REREQUISITE —This course is designed as an educational opportunity for physicians. A CCREDIT A TI O N ST A TEMENT —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 activity for a maximum of 3.25 AMA PRA Category 1 Credit(s).TM If purchased, the AANEM designates this activity for 2 AMA PRA Category 1 Credit(s).TM 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. Each physician should claim only those hours of credit he or she actually spent in the educational activity. CME for this course is available 10/09 - 10/12. vi Neuromuscular Update I AANEM Course 2008-2009 AANEM COURSE COMMITTEE Anthony E. Chiodo, MD, Chair Ann Arbor, Michigan Shawn J. Bird, MD Mazen M. Dimachkie, MD Benjamin S. Warfel, II, MD Philadelphia, Pennsylvania Kansas City, Kansas Lancaster, Pennsylvania Gary L. Branch, DO John E. Hartmann, MD Dewitt, Michigan Augusta, Georgia John E. Chapin, MD David Bryan Shuster, MD Walla Walla, Washington Dayton, Ohio 2008-2009 AANEM PRESIDENT Michael T. Andary, MD, MS East Lansing, Michigan 1 Mononeuropathy: Postoperative Foot Drop Kerry H. Levin MD Greg T. Carter, MD, MS Chairman, Department of Neurology Professor of Rehabilitation Medicine Director, Neuromuscular Center University of Washington School of Medicine Cleveland Clinic