Objective  To describe epidemiological and clinical characteristics of the patients with Creutzfeldt-Jakob disease (CJD) in 12 provinces in China.

  Methods  The clinical and epidemiological information of CJD patients obtained from China CJD surveillance network were analyzed. Blood and cerebral spinal fluid (CSF) specimens were collected from these patients. Western blot assay was conducted for detecting 14-3-3 protein in CSF, and Polymerase Chain Reaction (PCR) and sequencing were performed by using DNA extracted from whole blood genome for the analyses on polymorphism of 129 and 219 amino acid and mutation of PRNP gene.

  Results  In 2019, a total of 520 CJD cases were detected in the surveillance, including 190 (36.54%) probable CJD cases, 8 (1.54%) possible sporadic CJD cases, 25 (4.81%) genetic CJD cases, 5 (0.96%) fatal familial insomnia (FFI) cases and 1 (0.19%) cases of Gerstmann-Straussler-Scheinker (GSS) syndrome. The cases occurred sporadically without clustering in time, place and population. The median age of diagnosed and probable CJD cases was 62 years (40–84 years old), and the male to female ratio of the cases was 0.96∶1. The median age of possible CJD cases was 64.5 years (45–76 years old), and the male to female ratio of the cases was 1∶1. Rapidly progressive dementia was the major initial symptom. Among 508 cases, 491 were M/M in allele 129, and 497 were E/E in allele 219.

  Conclusion  CJD occurred sporadically in China in 2019. The time, place and population distributions, gender ratio and average onset age of the CJD cases were consistent with the general characteristics of sporadic CJD cases in the world.