Téma: Original Works

Myasthenia gravis – autoimmune spectrum and immunopathogenetic classification

Peter Špalek

Myasthenia gravis (MG) is defined as a disorder of the neuromuscular junction with fluctuating weakness of voluntary muscles associated with exhaustibility. MG is a heterogenous disorder with 4 distinct subgroups which differ immunologically, clinically and in responses to various treatments: 1. Seropositive MG (SPMG). 70-75 % MG patients have positive autoantibodies against nicotinic acetylcholine receptor (AChR). According to the different role of the human thymus for the immunopathogesis there are two forms of SPMG: A. SPMG associated with thymic lymphofollicular hyperplasia and young onset (< 45 years). B. SPMG associated with thymic atrophy and old onset (> 45 years). 2. Thymoma associated myasthenia gravis (TAMG). 10-15 % MG patients have SPMG with autoantibodies to AChR associated with thymoma and antibodies against titine. In TAMG there is an evidence of both central (thymic) and peripheral (extrathymic) immunopathogenesis. 3. Seronegative MG (SNMG) to AChR and positive antibodies against MuSK (muscle specific kinase) has 7 % of MG patients. 4. „Pure“ SNMG (no autoantibodies to AchR and to MuSK) has 8 % of MG patients. Treatment focuses on anticholinesterases, immunosuppressive agents, thymectomy, and short-term interventions such as plasmapheresis and intravenous immunoglobulin. These treatments, usually in combination, produce remission or marked improvement in about 90 % of MG patients. Treatment should be individualized and there is no single regimen that is appropriate for all patients.

Ročník 2009  Témy časopisu Neurology 1 / 2009

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