Clinical features, pathogenesis, and treatment of myasthenia gravis: a supplement to the Guidelines of the German Neurological Society
Melzer N, Ruck T, Fuhr P, Gold R, Hohlfeld R, Marx A, Melms A, Tackenberg B, Schalke B, Schneider-Gold C, Zimprich F, Meuth SG, Wiendl H (2016)
Publication Type: Journal article
Publication year: 2016
Journal
Book Volume: 263
Pages Range: 1473-94
Journal Issue: 8
DOI: 10.1007/s00415-016-8045-z
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
Involved external institutions
Westfälische Wilhelms-Universität (WWU) Münster
Germany (DE)
Ruhr-Universität Bochum (RUB)
Germany (DE)
Universität Basel
Switzerland (CH)
Ludwig-Maximilians-Universität (LMU)
Germany (DE)
Ruprecht-Karls-Universität Heidelberg
Germany (DE)
Philipps-Universität Marburg
Germany (DE)
Universität Regensburg
Germany (DE)
Medizinische Universität Wien
Austria (AT)
Germany (DE)
Ruhr-Universität Bochum (RUB)
Germany (DE)
Universität Basel
Switzerland (CH)
Ludwig-Maximilians-Universität (LMU)
Germany (DE)
Ruprecht-Karls-Universität Heidelberg
Germany (DE)
Philipps-Universität Marburg
Germany (DE)
Universität Regensburg
Germany (DE)
Medizinische Universität Wien
Austria (AT)
How to cite
APA:
Melzer, N., Ruck, T., Fuhr, P., Gold, R., Hohlfeld, R., Marx, A.,... Wiendl, H. (2016). Clinical features, pathogenesis, and treatment of myasthenia gravis: a supplement to the Guidelines of the German Neurological Society. Journal of Neurology, 263(8), 1473-94. https://dx.doi.org/10.1007/s00415-016-8045-z
MLA:
Melzer, Nico, et al. "Clinical features, pathogenesis, and treatment of myasthenia gravis: a supplement to the Guidelines of the German Neurological Society." Journal of Neurology 263.8 (2016): 1473-94.
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