Motor Neuron Disease Lewis P. Rowland, MD Robert H. Brown, MD, DPhil Eric J. Sorenson, MD Jeremy M. Shefner, MD, PhD Lisa S. Krivickas, MD Robert G. Miller, MD 2008 Plenary AANEM 55th Annual Meeting Providence, Rhode Island Copyright © September 2008 American Association of Neuromuscular & Electrodiagnostic Medicine 2621 Superior Drive NW Rochester, MN 55901 Printed by Johnson Printing Company, Inc. ii Motor Neuron Disease Faculty Lewis P. Rowland, MD Eric James J. Sorenson, MD Professor of Neurology Associate Professor Columbia University Medical Center Department of Neurology New York, New York Mayo Clinic Dr. Rowland is Professor of Neurology at Columbia University. Dr. Rochester, Minnesota Rowland received both his bachelor of science and his medical degree from Dr. Sorenson received his medical degree and completed his residency in Yale University. He was trained in biochemistry and is known for research neurology at Mayo Clinic, Rochester, Minnesota. A fellowship in neuro- in neuromuscular diseases and age-related neurodegenerative diseases, muscular diseases was completed in 1996 at the University of Michigan especially amyotrophic lateral sclerosis (ALS). He has written many papers Medical Center and he also received a master’s degree in clinical research on ALS and edited two books on that disease. He was the Editor-in Chief from the Mayo Graduate School of Medicine. He is currently a consultant of Neurology, 1977-1987, and has been a member of the editorial boards in the Department of Neurology at Mayo Clinic and an associate professor of other journals, including the New England Journal of Medicine, Medical at the Mayo Clinic College of Medicine. Dr. Sorenson is board certified by Letter and the Journal of Neurological Sciences. Since 2001, he has been chief the American Board of Electrodiagnostic Medicine and American Board editor of another official publication of the American Academy of Neurology of Psychiatry and Neurology. His interests include neuromuscular diseases (AAN), a bi-weekly newspaper called Neurology Today. He has been editor with an emphasis on amyotrophic lateral sclerosis. of Merritt’s Textbook of Neurology editions 7-11 and is now, with Timothy A. Pedley, co-editor of the forthcoming 12th edition. Jeremy Shefner Dr. Rowland has been President of the Association of University Professors of Professor and Chair Neurology (1977-1978), President of the American Neurological Association Department of Neurology (1980-1981), President of the AAN (1989-1991), and Chairman of the Academy’s Education and Research Foundation (1996-1999). As Chairman SUNY Upstate Medical University of two departments of neurology, as an officer of these organizations, and as a Syracuse, New York member of committees of the NINDS, Dr. Rowland has focused on training Dr. Shefner received a PhD in experimental psychology from the University in clinical neurology and research. He is currently President of the Parkinson’s of Illinois in 1976, and was a National Reasearch Service Award Research Disease Foundation and a member of the Institute of Medicine. Fellow from 1977 to 1979. He then pursued medical training; following graduation from medical school at Northwestern University, he completed neurology training at the Harvard Longwood Neurological Training Robert H. Brown, Jr, MD, DPhil program, and a fellowship in clinical neurophysiology and neuromuscular Professor disease at the Brigham and Women’s Hospital. He spent 6 years on the faculty of Harvard Medical School and the Brigham and Women’s Hospital, Department of Neurology and moved to State University of New York Upstate Medical University in Harvard Medical School 1996, where he is now Professor and Chair of Neurology. Boston, Massachusetts Dr. Brown graduated from Harvard Medical School and completed his doctoral Dr. Shefner founded the Northeast ALS Clinical Trials Consortium training in neurophysiology at Oxford University. Dr. Brown trained in neurol- (NEALS) in 1995, and now serves as co-chair. NEALS is the largest ogy at Massachusetts General Hospital (MGH) in Boston, Massachusetts. In academic clinical trials consortium in the United States, and has completed 1984, he founded The Day Laboratory for Neuromuscular Research at MGH 6 multicenter ALS trials since 2003. Upon moving to SUNY, he established to investigate neuromuscular diseases, including Miyoshi myopathy and amyo- the multidisciplinary ALS clinic there, and has held leadership positions in trophic lateral sclerosis (ALS). many National Institute of Health and industry-supported clinical trials. He has published more than 100 peer reviewed scientific articles, recently Dr. Brown is currently Professor of Neurology at Harvard Medical School completed a term as Associate Editor of Muscle & Nerve, is Neuromuscular and Director of the Day Neuromuscular Research Laboratory. He is Section Editor of UpToDate, and serves on the editorial board of Clinical renowned for his work on the inherited basis of neurodegenerative and Neurophysiology and the ALS Journal. He is also Chair of the ALS neuromuscular diseases and has a record of significant discoveries in ALS Research Group. and neuromuscular disorders, like hyperkalemic periodic paralysis and some types of muscular dystrophy. In 1993, Dr. Brown and colleagues defined mutations in the gene encoding SOD1 as one cause of familial ALS. Authors had nothing to disclose. Plenary Chair: John E. Chapin, 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. Reference to commercial products herein are for illustrative purposes only and should not be interpreted as an AANEM endorsement. Lisa S. Krivickas, MD Robert G. Miller, MD Director of Electrodiagnostic Medicine California Pacific Medical Center Spaulding Rehabilitation Hospital San Francisco, California Associate Chief of PM&R Dr. Miller is a Clinical Professor of Neurology at the University of Massachusetts General Hospital California, San Francisco (UCSF), Clinical Professor of Neurology and Associate Professor of PM&R Neurological Sciences at Stanford University Medical School, the Director Associate Chair of Academic Affairs of the Forbes Norris Amyotrophic Lateral Sclerosis (ALS) Research Center, and Chairman of the Department of Neurosciences at the California Harvard Medical School Pacific Medical Center in San Francisco. Dr. Miller received his medical Dr. Krivickas is Associate Professor of Physical Medicine and Rehabilitation degree from Weill Medical College of Cornell University, completed his (PMR) at Harvard Medical School, Associate Chair of Academic Affairs, residency at UCSF Medical Center & University of California San Diego Associate Chief of PMR at Massachusetts General Hospital, Director Medical Center, and completed a fellowship at the National Hospital of the Muscle Cell Physiology Laboratory at Spaulding Rehabilitation Queen’s Square. Hospital, and Director of EMG at Spaulding Rehabilitation Hospital. She received a bachelor of science in mechanical engineering from Cornell Dr. Miller chairs the medical advisory board of the ALS Care Program University and her medical degree from Harvard Medical School. She designed to improve database outcomes for North American patients with completed a residency in PMR at University of Medicine and Dentistry ALS. He was the lead author of the American Academy of Neurology of New Jersey, New Jersey Medical School and Kessler Institute of evidence-based practice parameters for managing ALS and he is leading Rehabilitation, and a fellowship in electromyography and neuromuscular an effort to revise and update the practice guidelines. He has been active disease in the Department of Neurology at the Cleveland Clinic. She has in ALS clinical trials and is the Chair of the Western ALS Study Group, authored over 70 publications and serves on the editorial board of several an organization that has carried out numerous clinical trials in ALS. He scientific journals. Dr. Krivickas’ current research involves the study of chairs the World Federation of Neurology ALS Research Group, and is muscle physiology in neuromuscular disorders and aging, investigation of involved with several collaborative efforts to find better markers for disease new electrophysiologic techniques for the assessment of neuromuscular progression and better treatments for ALS. disorders, and clinical trials for ALS. She is a director for the American Board of Electrodiagnostic Medicine and a member of the Neuromuscular Dr. Miller was President of the American Association of Neuromuscular & Medicine Examination Committee of the American Board of Psychiatry Electrodiagnostic Medicine (AANEM) from 1986-1987 and has served on and Neurology. She also served a term on the Board of Trustees of the numerous committees, and task forces for the association American College of Sports Medicine 2003-2006. She received the 2006 Young Academician Award from the Association of Academic Physiatrists. 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