INCIDENCE OF CREUTZFELDT-JAKOB-DISEASE IN FRANCE, 1992-1995

Background: Recent developments in animal and humans transmissible spo ngiform encephalopathies have motivated a study on incidence and risk factors of Creutzfeldt-Jakob disease (CJD) in France and 4 other Europ ean countries. Methods: CJD cases were ascertained through a national network including 250 neurological departments or neuropathological la boratories. CJD cases were classified as definite, probable or possibl e. Overall incidence, ate and age-standardized incidence rates by depa rtment were computed. Standardized incidence ratios and their 95 % con fidence intervals were computed for comparing observed and expected nu mber of CJD cases in each department. Results: Between 1992 and 1995, 216 CJD cases were registered (mean incidence rate : 0.87 per million inhabitants). The distribution of CJD cases was heterogeneous (p < 0.0 07). Nevertheless, the distribution of standardized incidence ratios f itted quite well a Poisson distribution. The observed number of CJD ca ses was significantly higher than expected in 4 departments and lower in 1 department. Conclusion: Incidence of CJD in France is similar to that observed in other European countries. Analysis of distribution of CJD cases by department showed a few significant differences which ca n be due to random fluctuations.