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慢性炎性脱髓鞘性多发性神经根神经病

最后审阅: 23 Jun 2024
最后更新: 31 May 2023

小结

定义

病史和体格检查

关键诊断因素

  • 疾病进展
  • 虚弱
  • 感觉改变
  • 深部腱反射减弱
完整详情

其他诊断因素

  • 共济失调
  • 40 至 60 岁
  • 近期感染
  • 未接触引发神经病的药物
  • 呼吸困难
  • 面部无力
  • 构音障碍
  • 吞咽困难
  • 尿失禁
  • 尿急或排尿踌躇
  • 阳痿
  • 体位性低血压
  • 视神经乳头水肿
  • 视力丧失
  • 痉挛状态
完整详情

危险因素

  • 男性
  • 自身免疫性疾病
  • 糖尿病
  • 感染
  • 未定性的单克隆丙种球蛋白病 (MGUS)
完整详情

诊断性检查

首要检查

  • 神经传导检查
完整详情

需考虑的检查

  • 脑脊液 (CSF) 评估
  • 神经超声
  • 脊柱和神经丛 MRI(平扫和增强)
  • 临床治疗尝试
  • 神经活检
  • 酶联免疫吸附测定 (ELISA) 或蛋白质印迹法检测自身抗体
  • 其他检查
完整详情

治疗流程

急症处理

对功能和生活质量未产生重大影响

对功能和生活质量产生重大影响

对初步单药治疗局部反应或无反应

对 2 种初始药物的联合治疗无反应

持续性治疗

对治疗产生反应

对治疗无反应

撰稿人

作者

Karissa L. Gable, MD

Associate Professor of Neurology

Department of Neurology

Duke University School of Medicine

Durham

NC

利益声明

KLG has taken part in CIDP education events for Medscape/Web MD. KLG is an author of references cited in this topic.

Thapat Wannarong, MD

Fellow of Neurology

Department of Neurology

Duke University School of Medicine

Durham

NC

利益声明

TW is an author of references cited in this topic.

鸣谢

Dr Karissa L. Gable and Dr Thapat Wannarong would like to gratefully acknowledge Professor Glenn Lopate, the previous contributor to this topic.

利益声明

GL is on the medical advisory board for Alnylam Pharmaceuticals and a consultant for Gerson Lehrman Group. GL is an author of references cited in this topic.

同行评议者

Anza B. Memon, MD

Senior Staff Neurologist

Henry Ford Health System

Clinical Assistant Professor

Wayne State University School of Medicine

Detroit

MI

利益声明

ABM declares that she has no competing interests.

Richard A.C. Hughes, MD, FRCP, FMedSci

Professor

Cochrane Neuromuscular Disease Group

MRC Centre for Neuromuscular Disease

National Hospital for Neurology and Neurosurgery

London

UK

利益声明

RACH has held or holds consultancies with companies that produce intravenous immunoglobulin (CSL Behring, LFB, Octapharma, Kedrion, and Talecris) and with other companies that produce immunomodulatory drugs (Biogen-Idec and Ares-Serono). RACH has received grants from the UK Department of Health for the Cochrane Neuromuscular Disease Review Group, Sandoz (now Novartis), and Ares-Serono. RACH is an author of a number of references cited in this topic.

Jean-Michel Vallat, MD

Professor

CHU Dupuytren

Service et Laboratoire de Neurologie

Limoges

France

利益声明

Not disclosed.

  • 鉴别诊断

    • 吉兰-巴雷综合征 (GBS)
    • 腓骨肌萎缩症 (CMT)
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    更多 鉴别诊断
  • 指南

    • 欧洲神经病学学会/周围神经学会关于慢性炎症性脱髓鞘性多发性神经根神经病的诊断和治疗指南:联合工作组的报告(第二次修订)
    更多 指南
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