重症肌无力

最后审阅: 4 六月 2022
最后更新: 01 四月 2021

小结

定义

病史和体格检查

关键诊断因素

  • 肌肉易疲劳
  • 睑下垂
  • 复视
  • 吞咽困难
  • 构音障碍
  • 面神经麻痹
  • 四肢近端无力
  • 呼吸短促
更多 关键诊断因素

危险因素

  • 自身免疫性疾病家族史
  • 遗传标记
  • 癌症靶向治疗
更多 危险因素

诊断性检查

首要检查

  • 血清乙酰胆碱受体 (AChR) 抗体分析
  • 肌肉特异性受体酪氨酸激酶 (MuSK) 抗体
  • 系列肺功能试验
更多 首要检查

需考虑的检查

  • 抗横纹肌受体抗体检测
  • 重复神经刺激
  • 单纤维 EMG
  • 胸部 CT
更多 需考虑的检查

治疗流程

急症处理

严重疾病(IV 类和 V 类、肌无力危象)

持续性治疗

轻度疾病(I 类和 II 类)

中度疾病(III 类)

严重疾病(肌无力危象后):无免疫抑制剂治疗耐受不良或禁忌证

严重疾病(肌无力危象后):有免疫抑制剂治疗耐受不良或禁忌证

撰稿人

作者

Robert P. Lisak, MD, FRCP (E), FAAN, FANA

Parker Webber Chair in Neurology

Professor of Neurology

Professor of Immunology and Microbiology

Wayne State University School of Medicine

Detroit

MI

利益声明

RPL is an Advisory Board member for Argenx (developing a clinical trial for chronic inflammatory demyelinating polyneuropathy [CIDP]). RPL has worked with Alexion (testing a treatment for myasthenia gravis); Novartis (design of research related to primary progressive MS vs. secondary progressive MS); GLG Consulting, Putnam Consulting, Slingshot Consulting, Haven Consulting, Clearview Consulting, Alpha Sites Consulting, and Insights Consulting (providing insights on the current and future treatments of several neuroimmunologic diseases including MS, MG, Guillain-Barré syndrome, CIDP, and neuromyelitis optica spectrum disorder); and Teva Pharmaceuticals (two non-branded talks to patient groups about MS). RPL has presented several CME talks for companies with multiple sources of support (no direct payments to RBL). RPL has received clinical trial grants (no direct payments) for studies in myasthenia gravis (Alexion, Argenx, Ra Pharmaceuticals) and MS (Novartis, Teva, Genentech/Roche, Chugai). He has received royalties from Blackwell Press as an editors of a textbook International Neurology. RBL is an author of several papers cited in this topic.

鸣谢

Dr Robert Lisak would like to gratefully acknowledge Dr Andrea Corse and Dr Ami Mankodi, previous contributors to this topic. AC and AM declare that they have no competing interests.

同行评议者

Vern C. Juel, MD

Associate Professor of Medicine (Neurology)

Duke University

Durham

NC

利益声明

VCJ declares that he has no competing interests.

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