DIAGNOSIS AND INCIDENCE OF PRION (CREUTZFELDT-JAKOB) DISEASE - A RETROSPECTIVE ARCHIVAL SURVEY WITH IMPLICATIONS FOR FUTURE-RESEARCH

Reliable identification of Creutzfeldt-Jakob disease (CJD) in the UX h as become essential following the suggestion that prion disease in cat tle (BSE) might transmit, accidentally, to humans who eat contaminated beef. Recent data suggest that some cases of CTD may be clinically un recognized; in order to examine this proposal we reviewed all cases of dementia (n = 1000+) collected in the Runwell Hospital Brain Archive between 1964 and 1990. We identified 19 cases of spongiform encephalop athy of which only 11 were diagnosed before death. These 11 individual s had a characteristic clinical history of CJD (relentless mental dete rioration, prominent motor signs and death within a year). Their brain s showed little or no external abnormality. In contrast, only two of t he eight clinically unrecognized cases had characteristic symptoms. Th e remaining six presented atypically; their illness lasted 3 years or more, motor signs were much less evident, and simple dementia was the most prominent feature. The brains showed moderate or severe cerebral atrophy. Our data indicate that only about 60% of prion disease cases with pathologically typical spongiform encephalopathy were identified clinically during life. This suggests that human prion disease may be more common than previously supposed and that a further review of the epidemiology of the disease is required (C) 1995 Academic Press Limite d.