Surveillance for Creutzfeldt-Jakob disease in China,2015

Objective To understand epidemiological and clinical characteristics of Creutzfeldt-Jakob disease (CJD) in China. Methods The surveillance data from China CJD surveillance network were analyzed. Blood and cerebral spinal fluid (CSF) specimens from CJD patients were collected. Western Blot assay was used for detecting 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 A total of 134 probable and 29 possible sporadic CJD patients were identified. Additionally, 4 FFI cases, 1 GSS cases and 17 genetic CJD cases, including 11 T188K, 4 E200K, 1 E196K and 1 E196A mutations, were definitely diagnosed. No period, geographic or occupational related clustering was observed among these cases. The mean onset age of probable and possible CJD patients were 63 and 60 years, respectively. The male to female ratio was 1.13:1 in the probable CJD patients and 1.64:1 in the possible CJD patients. Rapidly progressive dementia was the main foremost symptom, accounting for 36.81% in the CJD patients. Probable CJD patients showed more clinical manifestations than that of the possible CJD patients. Conclusion The CJD surveillance in 2015 indicated that the period, geographic and occupation distributions, gender ratio and mean onset age of the CJD patients in China were consistent with the general characteristics of sporadic CJD in the world.