Myasthenia Gravis and Myasthenic Disorders

Front Cover
Andrew Engel
Oxford University Press, 1999 - Medical - 310 pages
This volume of the Contemporary Neurology Series presents an in-depth survey of presently recognized myasthenic disorders. The book begins with a description of the approach to diseases of the neuromuscular junction, surveying the anatomy and molecular architecture of the neuromuscular junction, the structure and kinetic properties of the acetylcholine receptor, and the electromyographic investigation of defects of neuromuscular transmission. The second section reviews the immunopathogenesis, experimental models, natural history, diagnosis, and treatment of autoimmune myasthenia gravis. The third section focuses on the clinical electrophysiologic, and, where relevant, the molecular genetic features of the Lambert-Eaton syndrome, the presynaptic, synaptic, and postsynaptic varieties of the congenital myasthenic syndromes, the neuromyotonias, and the anti-ganglioside antibody associated diseases. Many disease entities here are novel. Throughout the book, the aim is to guide the reader to meet the challenge of diagnosis and therapy of a unique group of disorders. To this end, classical clinical observations, basic science data, and recent discoveries are presented in a balanced manner.
 

Contents

STRUCTURE AND KINETIC PROPERTIES
40
EXTRAOCULAR MUSCLES EXPRESS ADULT
45
Intracellular Segment Between M3 and M4 Is the Major Determinant
51
THE SAFETY FACTOR FOR NEUROMUSCULAR
57
ELECTRODIAGNOSIS OF ENDPLATE DISEASE
65
THE IMMUNOPATHOGENESIS OF MYASTHENIA GRAVIS
87
THE ROLE OF THE THYMUS IN MYASTHENIA GRAVIS
96
TCELL RESPONSES IN MYASTHENIA GRAVIS
102
TREATMENT OF MYASTHENIA GRAVIS
167
Seronegative
193
Myasthenic Syndromes and Related Disorders
203
DIAGNOSIS
221
16
223
NEUROMYOTONIA AND ANTIGANGLIOSIDE
229
CONGENITAL MYASTHENIC SYNDROMES
251
PRESYNAPTIC CONGENITAL MYASTHENIC SYNDROMES
259

SPECIFICITIES OF T HELPER CELLS IN EAMG
122
NATURAL HISTORY OF MYASTHENIA GRAVIS
131
Acetylcholine Receptor Pregnancy Mortality
140
DIAGNOSIS OF MYASTHENIA GRAVIS
146
CRITERIA PROPOSED FOR THE DIAGNOSIS
158
POSTSYNAPTIC CONGENITAL MYASTHENIC SYNDROMES
273
SlowChannel Syndromes Decreased
280
INCOMPLETELY CHARACTERIZED SYNDROMES
290
INDEX
299
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About the author (1999)

Andrew G. Engel is at Mayo Medical School and Director, The Neuromuscular Disease Laboratory, Mayo Clinic/ Mayo Foundation, Rochester, Minnesota.

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