SPORADIC CREUTZFELDT-JAKOB-DISEASE IN THE UNITED-KINGDOM - ANALYSIS OF EPIDEMIOLOGIC SURVEILLANCE DATA FOR 1970-96

Objective: To identify changes in the occurrence of Creutzfeldt-Jakob disease that might be related to the epidemic of bovine spongiform enc ephalopathy. Design: Epidemiological surveillance of the United Kingdo m population for Creutzfeldt-Jakob disease based on (a) referral of su spected cases by neurologists, neuropathologists, and neurophysiologis ts and (b) death certificates. Setting: England and Wales during 1970- 84, and whole of the United Kingdom during 1985-96. Subjects: AU 662 p atients identified as sporadic cases of Creutzfeldt-Jakob disease. Mai n outcome measures: Age distribution of patients, age specific time tr ends of disease, occupational exposure to cattle, potential exposure t o causative agent of bovine spongiform encephalopathy. Results: During 1970-96 there was an increase in the number of sporadic cases of Creu tzfeldt-Jakob disease recorded yearly in England and Wales. The greate st increase was among people aged over 70. There was a statistically s ignificant excess of cases among dairy farm workers and their spouses and among people at increased risk of contact with live cattle infecte d with bovine spongiform encephalopathy. During 1994-6 there were six deaths from sporadic Creutzfeldt-Jakob disease in the United Kingdom i n patients aged under 30. Conclusions: The increase in the incidence o f sporadic Creutzfeldt-Jakob disease and the high incidence in dairy f armers in the United Kingdom may be unrelated to bovine spongiform enc ephalopathy. The most striking change in the pattern of Creutzfeldt-Ja kob disease in the United Kingdom after the epidemic of bovine spongif orm encephalopathy is provided by the incidence in a group of exceptio nally young patients with a consistent and unusual neuropathological p rofile. The outcome of mouse transmission studies and the future incid ence of the disease in the United Kingdom and elsewhere, will be impor tant in judging whether the agent causing bovine spongiform encephalop athy has infected humans.