Myasthenia gravis.

Nils Erik Gilhus, Socrates Tzartos, Amelia Evoli, Jacqueline Palace, Ted M Burns, Jan J G M Verschuuren

Journal: Nature reviews. Disease primers 2019;5(1):30

PMID: 31048702

Full Text on Nature Publishing Group View on PubMed

Abstract

Myasthenia gravis (MG) is an autoimmune disease caused by antibodies against the acetylcholine receptor (AChR), muscle-specific kinase (MuSK) or other AChR-related proteins in the postsynaptic muscle membrane. Localized or general muscle weakness is the predominant symptom and is induced by the antibodies. Patients are grouped according to the presence of antibodies, symptoms, age at onset and thymus pathology. Diagnosis is straightforward in most patients with typical symptoms and a positive antibody test, although a detailed clinical and neurophysiological examination is important in antibody-negative patients. MG therapy should be ambitious and aim for clinical remission or only mild symptoms with near-normal function and quality of life. Treatment should be based on MG subgroup and includes symptomatic treatment using acetylcholinesterase inhibitors, thymectomy and immunotherapy. Intravenous immunoglobulin and plasma exchange are fast-acting treatments used for disease exacerbations, and intensive care is necessary during exacerbations with respiratory failure. Comorbidity is frequent, particularly in elderly patients. Active physical training should be encouraged.

Address: Department of Clinical Medicine, University of Bergen, Bergen, Norway. [email protected].; Department of Neurology, Haukeland University Hospital, Bergen, Norway. [email protected].; Department of Neurobiology, Hellenic Pasteur Institute and Tzartos NeuroDiagnostics, Athens, Greece.; Istituto di Neurologia, Fondazione Policlinico A. Gemelli, IRCCS, Rome, Italy.; Università Cattolica del Sacro Cuore, Rome, Italy.; Nuffield Department of Clinical Neurosciences, University of Oxford, Hospitals Trust, Oxford, UK.; Department of Neurology, University of Virginia, Charlottesville, VA, USA.; Department of Neurology, Leiden University Medical Centre, Leiden, Netherlands.

DOI: 10.1038/s41572-019-0079-y

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https://doi.org/10.1038/s41572-019-0079-y

MeSH Terms: Acetylcholinesterase, Adrenal Cortex Hormones, Agrin, Anti-Inflammatory Agents, Non-Steroidal, Autoantibodies, Biomarkers, Blepharoptosis, Collagen, Cortactin, Electromyography, Humans, Kv1.4 Potassium Channel, LDL-Receptor Related Proteins, Muscle Proteins, Myasthenia Gravis, Receptor Protein-Tyrosine Kinases, Receptors, Cholinergic, Receptors, Nicotinic, Risk Factors, Ryanodine Receptor Calcium Release Channel