亚急性海绵样脑病临床分析

目的描述Creutzfeldt-Jakob病(CJD)的临床特点.方法报道5例CJD患者并结合文献对本病的病因、临床表现、实验室检查、临床分类及预后进行探讨.结果5例患者均有认知功能障碍、锥体束损害及肌阵挛发作,其中3例伴小脑性共济失调,1例伴有震颤,1例有抑郁表现.脑电图(EEG)检查均有弥漫性异常,为周期性高伏尖波.结论CJD是一种可传播性疾病,临床特征为迅速进展的痴呆伴神经系统多系统损害.EEG有特征性改变.病程短、进展快、病死率达100%.对其尽早诊断可减少疾病的医源性传播.

The clinic analysis of Creutzfeldt-Jakob disease

Objective To describe the clinical features of Creutzfeldt-Jakob disease(CJD). Methods According to literature,combined with five cases we reported,the cause,clinical features,laboratory examination,clinical classification and prognosis of CJD were studied. Results In the five cases,the mean age of five cases was61.4,the mean course was10.8months,and the mean course of dementia was3.5months.All patients had dementia,pyramidal signs and myoclonus.Among them,3cases had cerebellar ataxia.1case had tremor and1case had depression.The electroen-cephalography(EEG)showed diffuse abnormality with periodic sharp wave,sharp slowwave and spike wave. Conclusion CJD is a spread disease.The clinical features of the disease is to lead to rapid dementia with multisystem lesion.EEG has typical change.The duration of the disease is shortand its mortality is100%.It is imprtant to make an early diagnosis of this disease and minimize the possibility of iatrogenesis.