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朊病毒病

最后审阅: 5 Oct 2024
最后更新: 31 Aug 2023

小结

定义

病史和体格检查

关键诊断因素

  • 认知障碍
  • 四肢和/或共济失调步态
  • 肌阵挛
  • 帕金森氏病
  • 精神症状
  • 视觉变化
  • 20 多岁或 65 岁到 70 岁之间
  • 失眠,自主神经功能异常
  • 阳性家族史
  • 非特异度或全身症状
完整详情

其他诊断因素

  • 疼痛感觉症状
  • 运动障碍
完整详情

危险因素

  • 遗传易感性
  • 被朊病毒污染的手术器械
  • 输入血液或血液制品(变异型克雅氏病)
  • 从 1980 年到 1996 年英国的牛肉消费情况
  • 美国的牛肉消费情况
  • 在美国和加拿大疫区捕猎的鹿、麋鹿和驼鹿
  • 使用人生长激素
完整详情

诊断性检查

首要检查

  • 脑部 MRI
  • 脑电图 (EEG)
完整详情

需考虑的检查

  • 振动诱导转化 (QuIC)
  • CSF 生物标志物
  • 朊蛋白基因检测
  • 活检(脑部,扁桃体)
完整详情

治疗流程

持续性治疗

所有患者

撰稿人

作者

Valerie Sim, MD, FRCPC

Associate Professor

Department of Medicine

Division of Neurology

Centre for Prions and Protein Folding Diseases

University of Alberta

Edmonton

Alberta

Canada

利益声明

VS works as an academic neurologist, providing medical consultation on cases of dementia, including prion disease. This falls within the scope of her role so no extra specialised payments are received. VS also receives grants, held by the University of Alberta, to research prion disease. The grants support equipment, personnel salaries and lab supplies, but provide no personal financial support.

鸣谢

Dr Valerie Sim would like to gratefully acknowledge Dr Michael D. Geschwind, Dr Amy Kuo, and Dr R. Ronald Finley, the previous contributors to this topic. MDG participates in the speakers' bureau for Pfizer, Forest, and Novartis; is consultant for MedaCorp, Gerson-Lehman Group, and Clinical Advisors Incorporated; and is an author of a number of references cited in this topic. RRF participates in the speakers' bureau for Pfizer, Forest, and Novartis. AK declares that she has no competing interests.

同行评议者

Ali Hassoun, MD, FACP, FIDSA, AAHIVS

Infectious Disease Specialist

Alabama Infectious Diseases Center

Huntsville

AL

利益声明

AH declares that he has no competing interests.

Robert A. Larsen, MD

Associate Professor of Medicine

University of Southern California

Keck School of Medicine

Los Angeles

CA

利益声明

RAL declares that he has no competing interests.

William A. Petri, Jr., MD, PhD, FACP

Chief and Professor of Medicine

Division of Infectious Diseases and International Health

University of Virginia Health System

Charlottesville

VA

利益声明

WAP declares that he has no competing interests.

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