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
其他诊断因素
- 疼痛感觉症状
- 运动障碍
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
诊断性检查
首要检查
- 脑部 MRI
- 脑电图 (EEG)
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)
治疗流程
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.
鉴别诊断
- 阿尔茨海默氏痴呆 (AD)
- 路易体痴呆
- 额颞叶痴呆
更多 鉴别诊断指南
- 胃肠道内镜检查设备消毒指南
- 内镜消毒更新:资源敏感型再处理指南
更多 指南- 登录或订阅即可浏览 BMJ Best Practice 临床实践完整内容
内容使用需遵循免责声明