title: The epidemiological, clinical, and laboratory features of sporadic Creutzfeldt-Jakob disease patients in China: surveillance data from 2006 to 2010.
abstract: BACKGROUND
  Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive fatal central nervous system disorder, which consists of three main catalogues: sporadic, familial, and iatrogenic CJD.
  
  
  RESULTS
  In China, the surveillance for CJD started in 2006, covering 12 provincial Centers for Disease Control and Prevention (CDCs) and 15 hospitals. From 2006 to 2010, 624 suspected patients were referred to China CJD surveillance. The epidemiological, clinical and laboratory features of sporadic CJD (sCJD) were analysed. Both groups of probable and possible sCJD showed highest incidences in the population of 60 to 69 year-olds. The most common presenting symptoms were progressive dementia and mental-related symptoms (neurological symptoms including sleeping turbulence, depression, anxiety and stress). Among the four main clinical manifestations, myoclonus was more frequently observed in the probable sCJD patients. About 2/3 of probable sCJD cases showed positive 14-3-3 in CSF and/or periodic sharp wave complexes (PSWC) in electroencephalography (EEG). The presence of myoclonus was significantly closely related with the appearance of PSWC in EEG. Polymorphisms of codon 129 in PRNP of the notified cases revealed a highly predominant M129M genotype in Han Chinese. Among 23 genetic human prion diseases, ten were D178N/M129M Fatal familial insomnia (FFI) and five were T188K genetic CJD (gCJD), possibly indicating a special distribution of gCJD-related mutations in Han Chinese.
  
  
  CONCLUSIONS
  From the period of 2006 to 2010, 261 patients were diagnosed as sCJD and 23 patients were diagnosed as genetic human prion diseases in China. The epidemiological, clinical and laboratory analysis data were consistent with the characteristics of sporadic CJD, which provide insight into the features of CJD in China.
authors: State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing, People's Republic of China.
authors: Chen
authors: C
authors: Gao
authors: Qi
authors: Q
authors: Shi
authors: Chan
authors: C
authors: Tian
authors: Cao
authors: C
authors: Chen
authors: Jun
authors: J
authors: Han
authors: Wei
authors: W
authors: Zhou
authors: Bao-Yun
authors: BY
authors: Zhang
authors: Hui-Ying
authors: HY
authors: Jiang
authors: Jin
authors: J
authors: Zhang
authors: Xiao-Ping
authors: XP
authors: Dong
mesh_terms: D048948:14-3-3 Proteins / Q000378:metabolism; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002681:China / Q000453:epidemiology; D003062:Codon / Q000235:genetics; D007562:Creutzfeldt-Jakob Syndrome / Q000453:epidemiology* / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D004569:Electroencephalography; D005260:Female; D005838:Genotype; D005843:Geography; D006801:Humans; D034062:Insomnia, Fatal Familial / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009207:Myoclonus / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D011110:Polymorphism, Genetic / Q000235:genetics; D055815:Young Adult
chemical_list: D048948:14-3-3 Proteins; D003062:Codon