title: Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada.
abstract: BACKGROUND
  An international study of the epidemiologic characteristics of Creutzfeldt-Jakob disease (CJD) was established in 1993 and included national registries in France, Germany, Italy, the Netherlands, Slovakia, and the United Kingdom. In 1997, the study was extended to Australia, Austria, Canada, Spain, and Switzerland.
  
  
  METHODS
  Data were pooled from all participating countries for the years 1993 to 2002 and included deaths from definite or probable CJD of all etiologic subtypes.
  
  
  RESULTS
  Four thousand four hundred forty-one cases were available for analysis and included 3,720 cases of sporadic CJD, 455 genetic cases, 138 iatrogenic cases, and 128 variant cases. The overall annual mortality rate between 1999 and 2002 was 1.67 per million for all cases and 1.39 per million for sporadic CJD. Mortality rates were similar in all countries. There was heterogeneity in the distribution of cases by etiologic subtype with an excess of genetic cases in Italy and Slovakia, of iatrogenic cases in France and the UK, and of variant CJD in the UK.
  
  
  CONCLUSIONS
  This study has established overall epidemiologic characteristics for Creutzfeldt-Jakob disease (CJD) of all types in a multinational population-based study. Intercountry comparisons did not suggest any relative change in the characteristics of sporadic CJD in the United Kingdom, and the evidence in this study does not suggest the occurrence of a novel form of human bovine spongiform encephalopathy infection other than variant CJD. However, this remains a possibility, and countries currently unaffected by variant CJD may yet have cases.
authors.affiliation: Istituto Superiore di Sanità, Department of Cell Biology and Neurosciences-ISS, Rome, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Ladogana
authors.forename: M
authors.initials: M
authors.lastname: Puopolo
authors.forename: E A
authors.initials: EA
authors.lastname: Croes
authors.forename: H
authors.initials: H
authors.lastname: Budka
authors.forename: C
authors.initials: C
authors.lastname: Jarius
authors.forename: S
authors.initials: S
authors.lastname: Collins
authors.forename: G M
authors.initials: GM
authors.lastname: Klug
authors.forename: T
authors.initials: T
authors.lastname: Sutcliffe
authors.forename: A
authors.initials: A
authors.lastname: Giulivi
authors.forename: A
authors.initials: A
authors.lastname: Alperovitch
authors.forename: N
authors.initials: N
authors.lastname: Delasnerie-Laupretre
authors.forename: J-P
authors.initials: JP
authors.lastname: Brandel
authors.forename: S
authors.initials: S
authors.lastname: Poser
authors.forename: H
authors.initials: H
authors.lastname: Kretzschmar
authors.forename: I
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authors.lastname: Rietveld
authors.forename: E
authors.initials: E
authors.lastname: Mitrova
authors.forename: J de Pedro
authors.initials: Jde P
authors.lastname: Cuesta
authors.forename: P
authors.initials: P
authors.lastname: Martinez-Martin
authors.forename: M
authors.initials: M
authors.lastname: Glatzel
authors.forename: A
authors.initials: A
authors.lastname: Aguzzi
authors.forename: R
authors.initials: R
authors.lastname: Knight
authors.forename: H
authors.initials: H
authors.lastname: Ward
authors.forename: M
authors.initials: M
authors.lastname: Pocchiari
authors.forename: C M
authors.initials: CM
authors.lastname: van Duijn
authors.forename: R G
authors.initials: RG
authors.lastname: Will
authors.forename: I
authors.initials: I
authors.lastname: Zerr
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001315:Australia / Q000453:epidemiology; D002170:Canada / Q000453:epidemiology; D015984:Causality; D002648:Child; D007562:Creutzfeldt-Jakob Syndrome / Q000145:classification / Q000401:mortality*; D005060:Europe / Q000453:epidemiology; D005260:Female; D020022:Genetic Predisposition to Disease / Q000453:epidemiology; D005843:Geography; D014943:Global Health; D006801:Humans; D007049:Iatrogenic Disease / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009026:Mortality / Q000639:trends; D011159:Population Surveillance / Q000379:methods; D017096:Prion Diseases / Q000209:etiology / Q000401:mortality; D012737:Sex Factors; D015047:Zoonoses / Q000453:epidemiology / Q000635:transmission