Melzer, Nico and Ruck, Tobias and Fuhr, Peter and Gold, Ralf and Hohlfeld, Reinhard and Marx, Alexander and Melms, Arthur and Tackenberg, Bjoern and Schalke, Berthold and Schneider-Gold, Christiane and Zimprich, Fritz and Meuth, Sven G. and Wiendl, Heinz (2016) Clinical features, pathogenesis, and treatment of myasthenia gravis: a supplement to the Guidelines of the German Neurological Society. JOURNAL OF NEUROLOGY, 263 (8). pp. 1473-1494. ISSN 0340-5354, 1432-1459
Full text not available from this repository. (Request a copy)Abstract
Myasthenia gravis (MG) is an autoimmune antibody-mediated disorder of neuromuscular synaptic transmission. The clinical hallmark of MG consists of fluctuating fatigability and weakness affecting ocular, bulbar and (proximal) limb skeletal muscle groups. MG may either occur as an autoimmune disease with distinct immunogenetic characteristics or as a paraneoplastic syndrome associated with tumors of the thymus. Impairment of central thymic and peripheral self-tolerance mechanisms in both cases is thought to favor an autoimmune CD4(+) T cell-mediated B cell activation and synthesis of pathogenic high-affinity autoantibodies of either the IgG1 and 3 or IgG4 subclass. These autoantibodies bind to the nicotinic acetylcholine receptor (AchR) itself, or muscle-specific tyrosine-kinase (MuSK), lipoprotein receptor-related protein 4 (LRP4) and agrin involved in clustering of AchRs within the postsynaptic membrane and structural maintenance of the neuromuscular synapse. This results in disturbance of neuromuscular transmission and thus clinical manifestation of the disease. Emphasizing evidence from clinical trials, we provide an updated overview on immunopathogenesis, and derived current and future treatment strategies for MG divided into: (a) symptomatic treatments facilitating neuromuscular transmission, (b) antibody-depleting treatments, and (c) immunotherapeutic treatment strategies.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | HIGH-DOSE CYCLOPHOSPHAMIDE; REMITTING MULTIPLE-SCLEROSIS; MAIN IMMUNOGENIC REGION; LONG-TERM AZATHIOPRINE; THYMIC MYOID CELLS; NICOTINIC ACETYLCHOLINE-RECEPTORS; SINGLE-FIBER ELECTROMYOGRAPHY; CONTROLLED PHASE-3 TRIAL; MUSCLE-SPECIFIC KINASE; ANTI-MUSK ANTIBODIES; Myasthenia gravis; Pathogenesis; Treatment guidelines |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Neurologie |
| Depositing User: | Petra Gürster |
| Date Deposited: | 28 Aug 2020 08:42 |
| Last Modified: | 28 Aug 2020 08:42 |
| URI: | https://pred.uni-regensburg.de/id/eprint/3580 |
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