Myasthenia Gravis and Myasthenic Disorders
Andrew G. Engel
Oxford University Press, USA, Apr 3, 2012 - Medical - 286 pages
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-Barré 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.
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1 THE ANATOMY AND MOLECULAR ARCHITECTURE OF THE NEUROMUSCULAR JUNCTION
2 ELECTRODIAGNOSIS OF MYASTHENIC DISORDERS
3 THE IMMUNOPATHOGENESIS OF MYASTHENIA GRAVIS
4 NATURAL HISTORY OF MYASTHENIA GRAVIS
5 THE DIAGNOSIS OF MYASTHENIA GRAVIS AND OTHER DISORDERS OF NEUROMUSCULAR TRANSMISSION
6 THERAPY OF MYASTHENIA GRAVIS
7 THE LAMBERTEATON MYASTHENIC SYNDROME
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abnormal Acad Sci acetylcholine receptor AChR AChR antibodies action potential active zones agonist agrin amplitude Ann Neurol Ann NY Acad anti anti-AChR antibodies antigen associated autoantibodies axonal binding Ca2+ calcium caused Cell Biol channel opening choline Clin clinical CMAP congenital myasthenic syndrome decreased decrement defect deficiency diagnosis domain dose drome drugs edrophonium effects endplate Engel exocytosis expression Figure gangliosides gene genetic immune immunosuppressive increased inhibitors jitter junctional folds kinase kinetic Lambert-Eaton myasthenic syndrome Lambert-Eaton syndrome LEMS loop mechanism MG patients mice molecular muscle fiber Muscle Nerve MuSK mutations myas myasthenia gravis myasthenic disorders myopathy Neurology neuromuscular junction neuromuscular transmission neuromyotonia Neurosci Newsom-Davis nicotinic normal Ohno onset Physiol presynaptic membrane protein ptosis quantal rapsyn rate constant release repetitive stimulation respiratory response severe Sine SM single-fiber EMG slow-channel structure studies subunit symptoms synaptic vesicles synaptotagmin therapy thymectomy thymic thymoma thymus tion treatment VGCC voltage-gated