Study on the characteristics of the surveiled Creutzfeldt-Jakob disease patients in 2009 in China

Objective To describe epidemiological and clinical characteristics of Creutzfeldt-Jakob disease (CJD) patients in China. Methods The clinical and epidemical information of 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 for detecting PrPSc in brain tissue and 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. Results Totally 1 definite CJD patient, 31 probable and 31 possible sporadic CJD patients were identified. Additionally, 3 FFI cases and 3 genetic CJD cases including G114V, E196K, T188K were definitely diagnosed. No period-, geographic-or occupational-related events were observed among these cases. The mean onset age of probably and possibly diagnosed CJD patients were 60 and 52 years old, respectively. The male to female ratio of the probable CJD patients was 12 ∶ 19 while that of the possible CJD patients was 14 ∶ 17. Rapidly progressive dementia was the main foremost symptom, presenting in 48.4% of the CJD patients. Probable CJD patients showed more clinical manifestations than that of the possible one. Conclusion The period and geography distribution, occupation, the ratio of gender and the mean onset age of the CJD cases in 2009 were consistent with the general characteristics of sporadic CJD in the world.