Shi Qi, Gao Chen, Zhou Wei, Zhang Bao-Yun, Chen Jian-Ming, Tian Chan, Jiang Hui-Ying, Han Jun, Xiang Ni-Juan, Wang Xiao-Fang, Gao Yong-Jun, Dong Xiao-Ping
State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, 100 Ying-Xin Rd, Beijing 100052, PR China.
BMC Public Health. 2008 Oct 18;8:360. doi: 10.1186/1471-2458-8-360.
Human transmissible spongiform encephalopathies (HTSE), or Creutzfeldt-Jakob disease (CJD), is a group of rare and fatal diseases in central nervous system. Since outbreak of bovine spongiform encephalopathy (BSE) and variant CJD, a worldwide CJD surveillance network has been established under the proposition of WHO. In China, a national CJD surveillance system has started since 2002. The data of CJD surveillance from 2006 to 2007 was analyzed.
Total 12 provinces are included in CJD surveillance system. The surveillance unit in each province consists of one or two sentinel hospitals and the provincial CDC. All suspected CJD cases reported from CJD surveillance were diagnosed and subtyped based on the diagnostic criteria for CJD issued by WHO.
Total 192 suspected CJD cases were reported and 5 genetic CJD, 51 probable and 30 possible sporadic CJD (sCJD) cases were diagnosed. The collected sCJD cases distribute sporadically without geographical clustering and seasonal relativity and the highest incidences in both probable and possible sCJD cases appeared in the group of 60-69 year. The most common three foremost symptoms were progressive dementia, cerebellum and mental-related symptoms. The probable sCJD patients owning both typical EEG alteration and CSF protein 14-3-3 positive have more characteristic clinical syndromes than the ones having only one positive. The polymorphisms of codon 129 of all tested reported cases shows typical patterns of Han Chinese as previous reports, that M129M are predominant whereas M129V are seldom.
Chinese CJD patients possessed similar epidemiological and clinical characteristics as worldwide.
人类可传播性海绵状脑病(HTSE),即克雅氏病(CJD),是一组罕见的中枢神经系统致命疾病。自牛海绵状脑病(BSE)和变异型克雅氏病爆发以来,在世卫组织的提议下建立了全球克雅氏病监测网络。在中国,自2002年起启动了全国克雅氏病监测系统。对2006年至2007年克雅氏病监测数据进行了分析。
克雅氏病监测系统纳入了12个省份。每个省份的监测单位由一两家哨点医院和省级疾控中心组成。所有从克雅氏病监测中报告的疑似克雅氏病病例均根据世卫组织发布的克雅氏病诊断标准进行诊断和分型。
共报告192例疑似克雅氏病病例,诊断出5例遗传性克雅氏病、51例很可能的和30例可能的散发性克雅氏病(sCJD)病例。所收集的散发性克雅氏病病例呈散发性分布,无地域聚集性和季节性相关性,很可能的和可能的散发性克雅氏病病例发病率最高的均出现在60 - 69岁年龄组。最常见的三个主要症状是进行性痴呆、小脑和精神相关症状。脑电图典型改变且脑脊液蛋白14 - 3 - 3阳性的很可能的散发性克雅氏病患者比仅一项阳性的患者具有更典型的临床综合征。所有检测报告病例的密码子129多态性显示出与既往报道的中国汉族典型模式相同,即M129M为主,而M129V很少见。
中国克雅氏病患者具有与全球相似的流行病学和临床特征。