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Heidenhain型克-雅病

Heidenhain's variant of Creutzfeldt-Jakob's disease (Fren)

作     者:Fauquembergue M. Tilikete C. Perret-Liaudet A. 杨秀梅 

作者机构:Unit 'e de Neuro-Ophtalmologie Hospices Civils de Lyon H? opital Neurologique Pierre -Wertheimer 59 boulevard Pinel 69677 Bron Cedex France 

出 版 物:《世界核心医学期刊文摘(眼科学分册)》 (Digest of the World Core Medical Journals:Ophthalmology)

年 卷 期:2005年第1卷第10期

页      面:7-8页

学科分类:1002[医学-临床医学] 100204[医学-神经病学] 10[医学] 

主  题:克-雅病 Heidenhain 朊病毒蛋白 神经症状 免疫电泳 眼部检查 医源性损伤 临床解剖 

摘      要:***-Jakob’s disease has various anatomoclinical present ations including a rare form with preponderant visual signs described by Heidenh ain. In this form, the visual symptoms may be isolated for a few weeks, leading to multiple ophthalmological examinations. Observation. We report the case of a 75-year-old woman who developed isolated visual disorders which rapidly increa sed over a period of two months. Addition of neurological symptoms, abnormalitie s of EEG and positivity of 14-3-3 protein led to the diagnosis of Creutzfeldt -Jakob’s disease. The patient died 14 months after the first neuroophthalmolog ic signs. The diagnosis was established by post-mortem examination and immuno- electrophoretic demonstration of type 1 prion protein. Conclusion. Heidenhain’s form of Creut zfeldt Jakob’s disease highlights the importance of general rules for preventio n of iatrogenic hazard during ophthalmological examinations.

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