DESCRIPTIVE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB-DISEASE IN 6 EUROPEAN COUNTRIES, 1993-1995

After the occurrence of bovine spongiform encephalopathy (BSE), there has been concern that transmission of BSE to the human population migh t result in a change in the epidemiological characteristics of Creutzf eldt-Jakob disease (CJD). A collaborative study of CJD in the European Union was performed from 1993 to 1995, to compare data from national registries for CJD in France, Germany, Italy, The Netherlands, Slovaki a, and the United Kingdom. Five hundred seventy-five patients with def inite or probable CJD died in the study period with an overall annual mortality rate of 0.71 cases per million. The incidence rates for CJD were similar in all participating countries despite variations in post mortem rates, and age-specific incidence rates were also relatively co nsistent, with the exception of an increased incidence of CJD in patie nts younger than 39 years of age in the United Kingdom. In relation to etiological subtypes of CJD, 87% of cases were sporadic, 8% genetic, and 5% iatrogenic. Genetic forms of CJD comprised 80% of all cases in Slovakia, and iatrogenic forms of CJD occurred most frequently in Fran ce and the United Kingdom. The statistical data reported here do not p rovide evidence of a causal link between BSE and CJD in Europe as a wh ole. However, the study has established baseline epidemiological param eters for CJD in participating European countries, which may be import ant in the assessment of any future change in the characteristics of C JD as a result of the epidemic of BSE.