A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970-1979. II: Epidemiology.

A comprehensive search yielded 121 confirmed cases of Creutzfeldt-Jakob disease who died in England and Wales in the decade 1970-1979, 31 probable cases and 10 possible cases. Descriptive epidemiological data are presented. The average annual incidence was 0.3 cases/million. An unexpected female excess was found. There was no evidence of space-time clustering of cases and no associations with occupation or past medical treatment were apparent. There was statistically significant variation in incidence rates in different parts of the country but no relationship was discovered between incidence and population density.