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.
Keywords: Eculizumab; Immunotherapy; Myasthenic crisis; Plasma exchange; Thymus.