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Content
Myasthenia Gravis and Myasthenic Disorders, Second Edition is a thoroughly re-written and updated version of the highly successful first edition published in 1999. The current edition begins with an overview of the anatomy and molecular architecture of the neuromuscular junction and the electrophysiologic diagnosis of its disorders. The introductory chapters are followed by a detailed exposition of the pathogenesis, natural history, diagnosis and therapy of the autoimmune myasthenias, the Lambert-Eaton myasthenic syndrome, and the increasingly complex and fascinating diseases collectively referred to as congenital myasthenic syndromes. The acetylcholine receptor is a major target of both autoimmune and inherited myasthenias and a separate chapter reviews its structural and functional properties. The book also describes disorders that often target terminal nerve segment near the neuromuscular junction- the syndrome of peripheral nerve hyperexcitability and the Guillain-Barre syndrome. Comprehensively written by leaders at the forefront of research, not to mention thoroughly referenced throughout and gorgeously illustrated, this new edition of the classic 1999 text will cement its place as the text on Myasthenia Gravis and related disorders for years to come. Table of Contents CHAPTER 1: THE ANATOMY AND MOLECULAR ARCHITECTURE OF THE NEUROMUSCULAR, ANDREW G. ENGEL, MD ; CHAPTER 2: ELECTRODIAGNOSIS OF MYASTHENIC DISORDERS, C. MICHEL HARPER, MD ; CHAPTER 3: THE IMMUNOPATHOGENESIS OF MYASTHENIA GRAVIS, REINHARD HOHLFELD, HARTMUT WEKERLE, AND ALEXANDER MARX ; CHAPTER 4: NATURAL HISTORY OF MYASTHENIA GRAVIS, VLADIMIR LUCHANOK, MD, PHD, AND HENRY J. KAMINSKI, MD ; CHAPTER 5: THE DIAGNOSIS OF MYASTHENIA GRAVIS AND OTHER DISORDERS OF NEUROMUSCULAR TRANSMISSION, JAMES F. HOWARD, JR., M.D. ; CHAPTER 6: THERAPY OF MYASTHENIA GRAVIS, DANIEL B. DRACHMAN, M.D. ; CHAPTER 7: THE LAMBERT-EATON MYASTHENIC SYNDROME, VERN C. JUEL, M.D., AND DONALD B. SANDERS, M.D. ; CHAPTER 8: CONGENITAL MYASTHENIC SYNDROMES, ANDREW G. ENGEL, M.D., XIN-MING SHEN, PH.D., KINJI OHNO, M.D., PH.D, STEVEN M. SINE, PH.D. ; CHAPTER 9: END-PLATE ACETYLCHOLINE RECEPTOR, A TARGET FOR MYASTHENIC DISORDERS: STRUCTURAL AND MECHANISTIC ESSENTIALS, STEVEN M. SINE AND ANDREW G. ENGEL ; CHAPTER 10: PERIPHERAL NERVE HYPEREXCITABILITY SYNDROMES, SRIKANTH MUPPIDI, M.D. AND STEVEN VERNINO, M.D., PH.D. ; CHAPTER 11: EFFECTS OF ANTIGANGLIOSIDE ANTIBODIES AT THE NEUROMUSCULAR JUNCTION, JAAP J. PLOMP AND HUGH J. WILLISON Review "Dr. Engel is a consummate investigator and master of the multiple sciences involved in understanding the causes and pathogenesis of myasthenia gravis and related syndromes. As editor of this encyclopedic book, he has gathered a stellar group of co-authors to cover every conceivable aspect of these diseases. The discussions will help students, clinicians and scientists who seek the details they need to understand the diseases and to deliver the best possible treatments. These diseases are among the best understood human autoimmune conditions. The antigens and antibodies are known, the immune system has been dissected, and treatment has improved to the point that almost no one now dies of what was once a lethal disease." -- Lewis P. Rowland MD, Columbia University Medical Center, New York, NY "The second edition of Engel's Myasthenia Gravis and Myasthenic Disorders will be warmly welcomed by neurologists and the neuromuscular fraternity. This magnum opus is a masterwork, beautifully orchestrated by Andrew Engel, and covering the whole range of major advances in recent years. In addition to his own contribution in several chapters, he has assembled an impressive array of experts in the field and covered topics ranging from anatomy and molecular architecture of the neuromuscular junction in his own opening chapter, through diagnostic techniques and special chapters on Congenital Myasthenic Syndromes and Lambert-Eaton syndrome, to advances in therapy. It should continue to maintain its position as a pre-eminent guide and reference for this important field." -- Victor Dubowitz, Neuromuscular Disorders About the Author Dr. Andrew Engel is the McKnight-3M Professor of Neuroscience and Professor of Neurology, Mayo Clinic College of Medicine as well as the Director of the Neuromuscular Disease laboratory at the Mayo Clinic, Rochester, MN campus. He earned his medical degree at McGill University, was trained in Neurology at the NIH and the Mayo Clinic and in Neuropathology at Columbia University and has spent his entire professional carrier at the Mayo Clinic. Dr Engel has had a longstanding interest in neuromuscular diseases and especially those affecting neuromuscular transmission.
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