摘要
目的了解2014—2017年北京市西城区克-雅病(Creutzfeldt-Jakob Disease,CJD)监测病例的流行病学特征、临床特点及转归情况。方法对2014—2017年北京市西城区医疗机构报告的可疑CJD病例的临床信息、流行病学、实验室检测结果以及随访资料进行分析。结果 2014—2017年北京市西城区共报告监测病例261例,支持CJD诊断的有80例,其中散发型克-雅病(sCJD)疑似诊断病例35例,sCJD临床诊断病例44例,遗传型克-雅病(gCJD)确诊病例1例,另外还有5例诊断为致死性家族型失眠症(FFI)。病例首发症状主要为快速进行性痴呆,临床表现根据出现频率高低依次进行性痴呆(100%)、锥体/锥体外系症状(76.47%)、视觉或小脑功能障碍(48.23%)、肌阵挛(43.53%)、无动性缄默(21.18%)和其他(31.76%)。辅助检查以头颅核磁共振(MRI)异常征象为主,实验室检测脑脊液14-3-3蛋白阳性率较高(51.76%,44/85),人类朊蛋白基因(PRNP)检测发现6例基因突变。病例流行病学特征分析显示发病时间、地区和职业均呈散在性。对所有诊断病例进行随访,有35例失访,失访率41.18%。结论 2014—2017年北京市西城区监测到的CJD病例以散发型为主,流行病学特征与2008—2013年的监测情况大致相同,定期随访有助于CJD正确诊断。
[Objective]To investigate the epidemiological characteristics,clinical features and outcomes of Crestzfeldt-Jakob Disease(CJD)network surveillance in Xicheng District of Beijing from 2014-2017.[Methods]The clinical information,epidemiology,laboratory results and follow-up data of suspected CJD cases reported by medical institutions in Xicheng District of Beijing from2014-2017 were analyzed.[Results]From 2014-2017,a total of 261 cases were monitored in Xicheng District of Beijing,80 cases were finally diagnosed as CJD,including 35 cases of suspected sporadic CJD( s CJD)and 44 diagnosed sCJD cases,one diagnosed case of genetic CJD(g CJD),and 5 cases were diagnosed as fatal familial insomnia(FFI).The first symptom of the case was rapid progressive dementia.The clinical manifestations according to the frequency of occurrence were as follows: progressive dementia(100%),pyramidal/extrapyramidal symptoms(76.47%),visual or cerebellar dysfunction(48.23%),myoclonus(43.53%),inactive silence(21.18%)and others(31.76%).Auxiliary examination was based on abnormal MRI signs of the head.The positive rate of 14-3-3 protein in cerebrospinal fluid was higher in the laboratory(51.76%,44/85),and 6 cases were found in prion protein in humans( PRNP)gene detection.The epidem iological characteristics of the cases showed the onset time,region and occupation were scattered.All the diagnosed cases were followed up,and 35 cases were lost to follow-up with the loss rate of41.18%.[Conclusion]The CJD surveillance cases in Xicheng District of Beijing from 2014-2017 are sporadic.The epidemiological characteristics are similar to the monitoring situation in 2008-2013.The regular follow-up will help the correct diagnosis of CJD.
作者
黄艳红
乔富宇
孙景異
王月仲
任剑
HUANG Yan-hong;QIAO Fu-yu;SUN Jing-yi;WANG Yue-zhong;REN Jian(Department of Prevention and Control of Infectious and Endemic Diseases,Xicheng District Center for Disease Control and Prevention,Beijing,100120,China)
出处
《职业与健康》
CAS
2019年第1期72-75,共4页
Occupation and Health
