Tian Chan, Gao Chen, Shi Qi, Han Jun, Zhou Wei, Zhang Bao-Yun, 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, Beijing, China.
Zhonghua Liu Xing Bing Xue Za Zhi. 2009 Jul;30(7):713-5.
To describe the epidemiological and clinical characteristics of Creutzfeldt-Jakob disease (CJD) in China.
Clinical and epidemical data on patients from China CJD surveillance network was analyzed. Blood and cerebral spinal fluid (CSF) specimens from these patients were collected. Western blot assay was used to detect 14-3-3 protein in CSF, PCR and sequencing assay were used for analyzing the polymorphism of 129 amino acid and mutation of PRNP gene.
A total number of 31 probable and 11 possible sporadic CJD patients were identified. Additionally,one patient with Gerstmann-Straussler-Scheinker syndrome (GSS) and 2 familial CJD cases were identified. No geographic- or occupational-related events were observed among these cases. The mean age of onset on the probable or possible CJD patients were 56.7 and 57.4 years old, with sex ratios of the probable CJD patients as 8:9 and the possible one as 5:6 respectively. Rapid progressive dementia was the main foremost symptom, presenting in 33.3% of the CJD patients. Probable CJD patients showed more clinical manifestations than those possible ones.
Geography distribution, occupation, ratio of gender and the mean onset age of the CJD cases in 2008 were consistent with the characteristics of the sporadic CJD.
描述中国克雅氏病（CJD）的流行病学和临床特征。
分析来自中国CJD监测网络患者的临床和流行病学数据。收集这些患者的血液和脑脊液（CSF）样本。采用免疫印迹法检测脑脊液中的14-3-3蛋白，采用聚合酶链反应（PCR）和测序分析法分析129位氨基酸的多态性及PRNP基因的突变情况。
共确诊31例可能的散发性CJD患者和11例疑似散发性CJD患者。另外，确诊1例格斯特曼综合征（GSS）患者和2例家族性CJD病例。这些病例中未观察到与地理或职业相关的情况。可能或疑似CJD患者的平均发病年龄分别为56.7岁和57.4岁，可能的CJD患者性别比为8:9，疑似患者为5:6。快速进行性痴呆是主要症状，在33.3%的CJD患者中出现。可能的CJD患者比疑似患者表现出更多的临床表现。
2008年CJD病例的地理分布、职业、性别比例和平均发病年龄与散发性CJD的特征相符。
