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重症肌无力。

Myasthenia gravis.

机构信息

Department of Clinical Medicine, University of Bergen, Bergen, Norway.

Department of Neurology, Haukeland University Hospital, Bergen, Norway.

出版信息

Nat Rev Dis Primers. 2019 May 2;5(1):30. doi: 10.1038/s41572-019-0079-y.

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.

摘要

重症肌无力(MG)是一种由乙酰胆碱受体(AChR)、肌肉特异性激酶(MuSK)或突触后肌膜中其他 AChR 相关蛋白的抗体引起的自身免疫性疾病。局部或全身肌肉无力是主要症状,由抗体引起。根据抗体的存在、症状、发病年龄和胸腺病理,将患者分为不同的组别。大多数具有典型症状和阳性抗体试验的患者的诊断较为直接,但对于抗体阴性的患者,详细的临床和神经生理学检查很重要。MG 的治疗应该积极,旨在实现临床缓解或仅轻度症状,同时保持接近正常的功能和生活质量。治疗应基于 MG 亚组,并包括使用乙酰胆碱酯酶抑制剂的对症治疗、胸腺切除术和免疫疗法。静脉注射免疫球蛋白和血浆置换是用于疾病恶化的快速治疗方法,在伴有呼吸衰竭的恶化期间需要重症监护。合并症很常见,尤其是在老年患者中。应鼓励积极的身体训练。

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