| Literature DB >> 31538208 |
Wolfgang Müllges1, Guido Stoll2.
Abstract
Myasthenia gravis (MG) is an autoimmune disorder caused by antibodies against acetylcholine receptors (AChR) or other structural proteins of the neuromuscular junction. This diminishes cholinergic transmission, thus leading to exercise-induced fatigue and sometimes manifest muscle weakness, including the bulbar and ocular musculature. Whereas ocular MG is as a rule initially symptomatically treated with acetylcholine esterase inhibitors, generalized MG requires long-term immunosuppression. The thymus plays a particular role in the pathophysiology of AChR antibody-positive MG, which can also manifest as a paraneoplastic disorder in the context of a thymoma. This article reviews the basic and advanced treatment options of the different disease subtypes including plasma exchange and immunoglobulins for treatment in a myasthenic crisis. Recently, clinical approval of eculizumab, a complement inhibitor, enriched the pharmacological armamentarium for AChR antibody-positive MG patients not appropriately responding to immunosuppression alone.Entities:
Keywords: Eculizumab; Immunotherapy; Myasthenic crisis; Plasma exchange; Thymus
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Year: 2019 PMID: 31538208 DOI: 10.1007/s00115-019-00798-8
Source DB: PubMed Journal: Nervenarzt ISSN: 0028-2804 Impact factor: 1.214