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 

机构地区：[1]Unit 'e de Neuro-Ophtalmologie, Hospices Civils de Lyon, H? opital Neurologique Pierre -Wertheimer, 59, boulevard Pinel, 69677 Bron Cedex, France

出　　处：《世界核心医学期刊文摘（眼科学分册）》2005年第10期7-8,共2页Digest of the World Core Medical Journals:Ophthalmology

摘　　要：Introduction.Creutzfeldt-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.Introduction.Creutzfeldt-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.

关 键 词：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 

分 类 号：R742.89[医药卫生—神经病学与精神病学]