重症肌无力

最后审阅: 6 十一月 2022
最后更新: 30 八月 2022

小结

定义

病史和体格检查

关键诊断因素

  • 肌力易疲劳
  • 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

肌无力危象

持续性治疗

轻至中度疾病(I-III 类)

重度(IV 类或 V 类)或难治性疾病

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 currently serves on an Advisory Board for Argenx for myasthenia gravis (MG), and was an Advisory Board member for Argenx (developing a clinical trial for chronic inflammatory demyelinating polyneuropathy [CIDP]). RPL has been reimbursed by Argenx the manufacturer of efartigimod for serving on a clinical advisory board. RPL has worked with Alexion (testing a treatment for MG); Novartis (design of research related to primary progressive multiple sclerosis [MS] vs. secondary progressive MS); Horizon Pharmaceuticals (advising on MG and neuromyelitis optica spectrum disorder); Takeda (advising on autoimmune diseases); 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, chronic inflammatory demyelinating polyradiculoneuropathy, and neuromyelitis optica spectrum disorder). RPL has presented several CME talks for companies with multiple sources of support (no direct payments to RPL). RPL has received clinical trial grants (no direct payments) for studies in MG (Alexion, Argenx, Ra Pharmaceuticals) and MS (Novartis, Teva, Genentech/Roche, Chugai). He has received royalties from Blackwell Press as an editor of the textbook International Neurology, and from Oxford University Press as an editor of a book, Neuroimmunology. RPL 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.

利益声明

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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