title: Clinical features, pathogenesis, and treatment of myasthenia gravis: a supplement to the Guidelines of the German Neurological Society.
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
authors.affiliation: Department of Neurology, University of Münster, Albert-Schweitzer-Campus 1, 48149, Münster, Germany. nico.melzer@ukmuenster.de.
authors.forename: Nico
authors.initials: N
authors.lastname: Melzer
authors.affiliation: Department of Neurology, University of Münster, Albert-Schweitzer-Campus 1, 48149, Münster, Germany.
authors.forename: Tobias
authors.initials: T
authors.lastname: Ruck
authors.affiliation: Department of Neurology, University of Basel, Basel, Switzerland.
authors.forename: Peter
authors.initials: P
authors.lastname: Fuhr
authors.affiliation: Department of Neurology, University of Bochum, Bochum, Germany.
authors.forename: Ralf
authors.initials: R
authors.lastname: Gold
authors.affiliation: Institute of Clinical Neuroimmunology, Ludwig-Maximilians-University Munich, Munich, Germany.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Hohlfeld
authors.affiliation: Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg, Mannheim, Germany.
authors.forename: Alexander
authors.initials: A
authors.lastname: Marx
authors.affiliation: Department of Neurology, University of Erlangen, Erlangen, Germany.
authors.forename: Arthur
authors.initials: A
authors.lastname: Melms
authors.affiliation: Department of Neurology, University of Marburg, Marburg, Germany.
authors.forename: Björn
authors.initials: B
authors.lastname: Tackenberg
authors.affiliation: Department of Neurology, University of Regensburg, Regensburg, Germany.
authors.forename: Berthold
authors.initials: B
authors.lastname: Schalke
authors.affiliation: Department of Neurology, University of Bochum, Bochum, Germany.
authors.forename: Christiane
authors.initials: C
authors.lastname: Schneider-Gold
authors.affiliation: Department of Neurology, Medical University of Vienna, Vienna, Austria.
authors.forename: Fritz
authors.initials: F
authors.lastname: Zimprich
authors.affiliation: Department of Neurology, University of Münster, Albert-Schweitzer-Campus 1, 48149, Münster, Germany.
authors.forename: Sven G
authors.initials: SG
authors.lastname: Meuth
authors.affiliation: Department of Neurology, University of Münster, Albert-Schweitzer-Campus 1, 48149, Münster, Germany.
authors.forename: Heinz
authors.initials: H
authors.lastname: Wiendl
mesh_terms: D019468:Disease Management; D005858:Germany; D006801:Humans; D009157:Myasthenia Gravis / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D012955:Societies, Medical / Q000592:standards*
keywords: Myasthenia gravis; Pathogenesis; Treatment guidelines