Myasthenia gravis


Authors: Peter Špalek
Authors‘ workplace: Neurologická klinika SZU FNsP Bratislava-Ružinov
Published in: Cesk Slov Neurol N 2008; 71/104(1): 7-24
Category: Minimonography

Overview

Myasthenia gravis (MG) is defined as a disorder of the neuromuscular junction with fluctuating weakness of voluntary muscles associated with exhaustibility. The incidence of MG is 7,0 to14,8 cases per million population per year. The prevalence rate ranges from 80,0 to191,6 per million population. The most common age at onset of MG is the second and third decades in women, the sixth and seventh decades in men. MG is a heterogenous disorder with 4 distinct subgroups which differ immunologically, clinically and in responses to various treatments: A. 75 % MG patients have seropositive MG (SPMG) with autoantibodies against nicotinic acetylcholine receptor (AChR). B. 15 % MG patients have SPMG with autoantibodies to AChR associated with thymoma and antibodies against titine. C. 7 % MG patients have seronegative MG (SNMG) to AChR and positive antibodies against MuSK (muscle specific kinase). D. 8 % MG patients have „pure“ SNMG (no autoantibodies to AChR/MuSK). Diagnostic procedures in MG are clinical evaluation, intravenous administration of neostigmin/edrophonium, EMG (repetitive nerve stimulation, single fibre electromyography), autoantibodies to AChR, MuSK, titine; chest CT or chest MR (thymoma diagnosis). Treatment focuses on anticholinesterases, immunosuppressive agents, thymectomy, and short-term interventions such as plasmapheresis, immunoadsorption and intravenous immunoglobulin (IVIg). These treatments, usually in combination, produce remission or improvement in about 90 % of MG patients. Refractory cases to immunosuppressive therapy may improve with plasmapheresis or IVIg on chronic intermittent basis. Treatment should be individualized and there is no single regimen that is appropriate for all patients.

Key words:
myasthenia gravis – neuromuscular junction – pathophysiology – imunopathogenesis – diagnosis – management – prognosis


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