慢性进展性散发型克-雅病一例  

Sporadic Creutzfeldt-Jakob Disease With Slow Progression:Report of One Case

作　　者：赵家华 武雷 晋薇 桂秋萍 张家堂 黄德晖 ZHAO Jiahua;WU Lei;JIN Wei;GUI Qiuping;ZHANG Jiatang;HUANG Dehui(Department of Neurology,The First Medical Centre of Chinese PLA General Hospital,Beijing 100853,China;Department of Pathology,The First Medical Centre of Chinese PLA General Hospital,Beijing 100853,China)

机构地区：[1]中国人民解放军总医院第一医学中心神经内科医学部,北京100853 [2]中国人民解放军总医院第一医学中心病理科,北京100853

出　　处：《中国医学科学院学报》2023年第5期859-862,共4页Acta Academiae Medicinae Sinicae

摘　　要：散发型克-雅病(sCJD)是一种由朊病毒导致的中枢神经系统退行性疾病,典型的临床表现为快速进展性痴呆,大部分患者病程小于1年,仅2%~3%患者大于2年。现报道1例以言语表达障碍起病、进展缓慢的sCJD病例,总结其临床表现、脑电图特点、影像及病理特征,以加强对慢性进展性sCJD的认识。Sporadic Creutzfeldt-Jakob disease(sCJD)is a prion-caused degenerative disease of the central nervous system,with the typical clinical manifestation of rapidly progressive dementia.The course of disease is less than 1 year in most patients and more than 2 years in only 2%to 3%patients.We reported a case of sCJD with expressive language disorder and slow progression in this paper.By summarizing the clinical manifestations and the electroencephalograhpy,MRI,and pathological features,we aimed to enrich the knowledge about the sCJD with slow progression.

关 键 词：散发型克-雅病 朊蛋白 脑活检 

分 类 号：R742[医药卫生—神经病学与精神病学]