Myasthenia Gravis and Myasthenic DisordersAndrew Engel 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 |
Other editions - View all
Common terms and phrases
abnormal acetyl acetylcholine receptor AChR AChR antibodies AChR-specific action potential activity amino acids amplitude Ann Neurol Ann NY Acad anti antigen autoantibodies autoimmune myasthenia gravis azathioprine binding Biol Ca2+ calcium cells channel choline choline receptor clinical CMAP congenital myasthenic syndrome corticosteroids decrement detected diagnosis disease disorders dose drug EAMG edrophonium endplate potential Engel epitopes gene immune immunoglobulin improvement junctional folds Lambert-Eaton myasthenic syndrome LEMS IgG Lindstrom membrane ment MG patients mice molecules motor muscle fibers Muscle Nerve mutations myas myasthenia gravis Natl Acad Sci nerve terminal Neurology neuromuscular junction neuromuscular transmission neuromyotonia Newsom-Davis normal ocular onset peptide Physiol plasma plasmapheresis postsynaptic presynaptic Proc Natl Acad protein ptosis quantal region repetitive stimulation respiratory response SCLC seronegative serum SFEMG skeletal muscle specific studies symptoms synaptic vesicles T-cell thenia thenia gravis therapy thymectomy thymic thymoma thymus tients tion treatment VGCCs weakness