Creutzfeldt-Jakob Disease in Scotland and Northern Ireland 1980–1989

Abstract

The epidemiological and clinical features of Creutzfeldt-Jakob disease have never before been studied in Scotland and Northern Ireland. Case records for those dying with this diagnosis were obtained for the period 1980–89. Over the ten year period, 25 definite or probable cases were identified, giving an annual incidence of 0.37 cases/million. There were more cases in the second half of the decade, and this was most likely due to increased ascertainment. One pair of cases occurred in close proximity to each other. Sex distribution showed an excess of males (male female ratio = 1.8: 1). Mean age at onset was 65.2 years, and mean duration of disease was 5.3 months. The presenting symptoms and clinical features were similar to those noted in previous studies of other populations. There was no excess of cases in occupations linked to food, farming, or medical/paramedical work.