Surveillance of Creutzfeldt-Jakob disease in China,2010

Objective To understand the incidence, epidemiological and clinical characteristics of Creutzfeldt-Jakob disease (CJD) in China. Methods The analysis was conducted on the clinical and epidemiological data of suspected CJD cases collected through the surveillance in China in 2010. Cerebrospinal fluid (CSF) and blood samples were taken from the patients to detect PrPSc in brain tissue and 14-3-3 protein in CSF by using Western blot assay and analyze the polymorphism of 129 amino acid and mutation of PRNP gene extracted from blood samples by using PCR and sequencing assay. Results Totally 1 laboratory confirmed cases, 46 clinical diagnosed and 25 suspected cases of CJD, 3 fatal familial insomnia cases (FFI) and 5 genetic CJD cases were detected, including 1 R208H, 1 P102L and 3 T188K mutation cases No seasonal, geographic and population clustering of the disease were observed. The mean age of clinical diagnosed patients was 62 years old, the male to female ratio was 1.30∶1, the mean age of suspected patients was 57 years old, the male to female ratio was 2.14∶1. Rapidly progressive dementia was the mostly common symptom, occurring firstly in 54.93% of the CJD patients. More typical clinical manifestations were presented in clinical diagnosed patients than in suspected patients. Conclusion CJD occurred sporadically in China in 2010, which was demonstrated by the time, geographic, population, gender and age distributions of the cases.