Highlights & Basics
- Myasthenia gravis (MG) is a chronic autoimmune disorder of the postsynaptic membrane at the neuromuscular junction in skeletal muscle.
- MG is characterized by muscle weakness that increases with exercise (fatigue) and improves on rest. It commonly presents with drooping eyelids, double vision, oropharyngeal and/or appendicular weakness, and shortness of breath.
- Elevated serum levels of antibodies against the acetylcholine receptor (AChR) or muscle-specific tyrosine kinase (MuSK) are usually present. Antibodies have also been identified to proteins located at the neuromuscular junction: low-density lipoprotein receptor-related protein 4 (LRP4), agrin, collagen Q, and cortactin. Patients with MG may have elevated levels of one or more of these antibodies.
- Clinical electrophysiology shows decremental response on repetitive nerve stimulation or increased jitter on single-fiber study.
- Treatments include the anticholinesterase inhibitor pyridostigmine and immunotherapy (a corticosteroid or other immunosuppressant). Thymectomy is required if thymoma is present. Thymectomy has also been shown to be effective in people with generalized MG without thymoma who are AChR antibody positive.
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Wang L, Huan X, Xi JY, et al. Immunosuppressive and monoclonal antibody treatment for myasthenia gravis: a network meta-analysis. CNS Neurosci Ther. 2019 May;25(5):647-58.[Abstract][Full Text]
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