title: Human transmissible spongiform encephalopathies in eleven countries: diagnostic pattern across time, 1993-2002.
abstract: BACKGROUND
  The objective of this study was to describe the diagnostic panorama of human transmissible spongiform encephalopathies across 11 countries.
  
  
  METHODS
  From data collected for surveillance purposes, we describe annual proportions of deaths due to different human transmissible spongiform encephalopathies in eleven EUROCJD-consortium countries over the period 1993-2002, as well as variations in the use of diagnostic tests. Using logistic models we quantified international differences and changes across time.
  
  
  RESULTS
  In general, pre-mortem use of diagnostic investigations increased with time. International differences in pathological confirmation of sporadic Creutzfeldt-Jakob disease, stable over time, were evident. Compared to their counterparts, some countries displayed remarkable patterns, such as: 1) the high proportion, increasing with time, of variant Creutzfeldt-Jakob disease in the United Kingdom, (OR 607.99 95% CI 84.72-4363.40), and France (OR 18.35, 95% CI 2.20-152.83); 2) high, decreasing proportions of iatrogenic Creutzfeldt-Jakob disease in France, (OR 5.81 95% CI 4.09-8.24), and the United Kingdom, (OR 1.54 95% CI 1.03-2.30); and, 3) high and stable ratios of genetic forms in Slovakia (OR 21.82 95% CI 12.42-38.33) and Italy (OR 2.12 95% CI 1.69-2.68).
  
  
  CONCLUSIONS
  Considerable international variation in aetiological subtypes of human transmissible spongiform encephalopathies was evident over the observation period. With the exception of variant Creutzfeldt-Jakob disease and iatrogenic Creutzfeldt-Jakob disease in France and the United Kingdom, these differences persisted across time.
authors.affiliation: Instituto de Salud Carlos III, Centro Nacional de Epidemiologia, Departamento de Epidemiologia Aplicada, Calle Sinesio Delgado 6, 28029, Madrid, Spain. jpedro@isciii.es
authors.forename: Jesús
authors.initials: J
authors.lastname: de Pedro-Cuesta
authors.forename: Markus
authors.initials: M
authors.lastname: Glatzel
authors.forename: Javier
authors.initials: J
authors.lastname: Almazán
authors.forename: Katharina
authors.initials: K
authors.lastname: Stoeck
authors.forename: Vittorio
authors.initials: V
authors.lastname: Mellina
authors.forename: Maria
authors.initials: M
authors.lastname: Puopolo
authors.forename: Maurizio
authors.initials: M
authors.lastname: Pocchiari
authors.forename: Inga
authors.initials: I
authors.lastname: Zerr
authors.forename: Hans A
authors.initials: HA
authors.lastname: Kretszchmar
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Brandel
authors.forename: Nicole
authors.initials: N
authors.lastname: Delasnerie-Lauprêtre
authors.forename: Annick
authors.initials: A
authors.lastname: Alpérovitch
authors.forename: Cornelia
authors.initials: C
authors.lastname: Van Duijn
authors.forename: Pascual
authors.initials: P
authors.lastname: Sanchez-Juan
authors.forename: Steven
authors.initials: S
authors.lastname: Collins
authors.forename: Victoria
authors.initials: V
authors.lastname: Lewis
authors.forename: Gerard H
authors.initials: GH
authors.lastname: Jansen
authors.forename: Michael B
authors.initials: MB
authors.lastname: Coulthart
authors.forename: Ellen
authors.initials: E
authors.lastname: Gelpi
authors.forename: Herbert
authors.initials: H
authors.lastname: Budka
authors.forename: Eva
authors.initials: E
authors.lastname: Mitrova
mesh_terms: D001315:Australia / Q000453:epidemiology; D002170:Canada / Q000453:epidemiology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000401:mortality* / Q000635:transmission; D003431:Cross-Cultural Comparison; D004569:Electroencephalography; D005060:Europe; D005602:France / Q000453:epidemiology; D005838:Genotype; D006801:Humans; D007049:Iatrogenic Disease / Q000453:epidemiology; D038622:Internationality; D007558:Italy / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D011159:Population Surveillance / Q000379:methods*; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics* / Q000472:pathogenicity; D012042:Registries; D018154:Slovakia / Q000453:epidemiology; D013995:Time; D006113:United Kingdom / Q000453:epidemiology
chemical_list: C512656:PRNP protein, human; D000072002:Prion Proteins; D011328:Prions