重症肌无力
医学
胸腺切除术
抗体
乙酰胆碱受体
自身抗体
肌肉无力
弱点
胸腺瘤
乙酰胆碱酯酶
神经肌肉接头
内科学
免疫学
神经肌肉疾病
疾病
外科
受体
神经科学
酶
化学
生物
生物化学
作者
Nils Erik Gilhus,Socrates J. Tzartos,Amelia Evoli,Jacqueline Palace,Ted M. Burns,Jan J.G.M. Verschuuren
标识
DOI:10.1038/s41572-019-0079-y
摘要
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.
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