国内散发性Creutzfeldt-Jakob病236例临床分析  

Retrospective analysis of 236 patients with sporadic Creutzfeldt-Jakob disease in China

作　　者：曹笃[1] 魏有东[1] 李琦[1] 任高平[1] 张刚[1] 谢鹏[1] 

机构地区：[1]重庆医科大学附属第一医院神经内科,重庆400016

出　　处：《第三军医大学学报》2013年第17期1858-1861,共4页Journal of Third Military Medical University

摘　　要：目的分析国内散发性Creutzfeldt-Jakob病(sporadic creutzfeldt-Jakob disease,sCJD)的临床特点,提高临床诊断的准确性。方法借鉴循证医学系统评价方法,对1990-2012年国内文献报道的临床病例资料及我院神经内科2006-2012年临床诊断为sCJD的住院病历资料进行综合分析。结果剔除可能重复的病例后,共收集到236例,其中完整病例资料93例,7例来自我院病历记录,86例来自文献报道。236例资料中,男性127例,女性109例,平均发病年龄57.5岁。主要症状依次有进行性痴呆93.3%,肌阵挛76.1%,锥体外系表现70.6%等。195例行头部MRI检查,DWI检查93例,其中92例表现为皮质或基底节区高信号。190例行脑电图检查,171例出现典型周期波。52例检测脑脊液14-3-3蛋白,41例(+),11例(-)。83例脑组织病理检查,均符合sCJD病理改变。结论 sCJD首发症状多样,主要临床症状以进行性痴呆、肌阵挛及锥体外系表现为主,病程晚期多出现无动性缄默,平均病程7.4个月。头部磁共振DWI早期敏感,绝大多数早于脑电图改变。根据临床症状结合DWI、EEG及14-3-3蛋白等检查,sCJD的临床诊断并不困难。Objective To study the clinical characteristics of sporadic Creutzfeldt-Jakob disease （sCJD） in China. Methods The records of sCJD patients in our hospital from 2006 to 2012 and the literatures in China Biological Medicine Database （CBM） and China National Knowledge Infrastructure （CNKI） from 1990 to 2012 were collected. The demographic data, clinical manifestations and laboratory findings of patients with sCJD were analyzed. Results Of the total 236 cases, 93 had integral data, including 7 from our hospital and 86 from literature. There were 127 males and 109 females, with a mean age of onsetting in 57.5, The cardinal symptoms included dementia （93.3%）, myoclonus （76.1%）, extrapyramidal symptoms （70.6%）, etc. Head magnetic resonance imaging （MRI） was performed in 195 cases. Diffusion-weighted imaging （DWI） of 92 cases showed abnormal hyperintensity in cerebral cortex and basal ganglia. A total of 190 cases had electroencephalogram （EEG） examination. Periodic sharp wave complexes （PSWC） were observed in 171 cases. 14-3-3 protein was found positive in cerebrospinal fluid （CSF） in 41 cases. Pathological examination of the brain tissues showed pathological changes of sCJD in 83 cases. Conclusion Consistent with dementia, myoclonus and extrapyramidal performance are the main symptoms of sCJD. The clinical diagnosis of sCJD is not difficult by combining cardinal symptoms with DWI, EEG and 14-3-3 protein.

关 键 词：散发性Creutzfeldt—Jakob病 磁共振 脑电图 

分 类 号：R181.23[医药卫生—流行病学] R742[医药卫生—公共卫生与预防医学]