Objective To investigate the incidence, epidemiology and clinical characteristics of Creutzfeldt-Jakob disease (CJD) in China in 2020.
Methods Clinical and epidemiological data of CJD patients obtained from the CJD surveillance network in China in 2020 were analyzed. Blood and cerebrospinal fluid of the patients were collected. The amino acid polymorphisms at 129 and 219 sites of PRNP were analyzed by PCR and DNA sequencing using extracted from whole blood genome. The 14-3-3 protein in cerebrospinal fluid was detected by Western Blot.
Results A total of 458 CJD cases were reported by the CJD surveillance network in 2020, in which 179 (39.08%) were probable sporadic CJD cases, 12 (2.62%) were possible cases, 18 (3.93%) were genetic CJD cases, and 1 (0.22%) was Gerstmann-Straussler-Scheinker syndrome case. Case reporting showed no seasonality, the long-term residence of the cases showed sporadic distribution, and the occupation distribution showed no clustering The median age of probable cases was 63.14 (43, 87) years, and the male-to-female ratio of the cases was 0.97∶1.The median age of possible cases was 68.08 (54, 77) years, and the male-to-female ratio of the cases was 1:1. Rapid-progressive dementia was the most common initial symptom. For the three test results of 14-3-3 protein in CSF, electroencephalogram and cranial magnetic resonance imaging, the patients with more positive results also had more typical symptoms. PRNP was detected in 439 blood samples. There were 434 cases of M/M homozygous amino acids, 3 cases of M/V heterozygous amino acids, and 2 cases of V/V homozygous amino acids at 129 position. There were 435 cases of E/E homozygous amino acids at 219 position, and 4 E/K cases of homozygous amino acids.
Conclusion The reporting time, distribution of long-term residence, occupation, sex ratio and age distribution of CJD cases detected in China in 2020 were all consistent with the characteristics of CJD.
Home
DownLoad: