Creutzfeldt-Jakob disease: the need for vigilance

Creutzfeldt-Jakob disease (CJD) is a rapidly fatal neurodegenerative disorder belonging to the group of diseases known as transmissible spongiform encephalopathies. It has a stable worldwide incidence of approximately one case per million per year. Case identification relies upon characteristic neurological and EEG features, however, neuropathological assessment is required for confirmation. We have identified four cases of sporadic CJD occurring on the Central Coast of NSW, Australia over a 7 month period during 1997. This represents approximately fifteen times the expected number of cases for the population of 275 000. Multiple co-morbid states particularly in elderly patients, the absence of a reliable diagnostic test and the increasing care of cognitively impaired patients in community institutions are all challenges to accurate case ascertainment. A high index of suspicion for CJD is required and a post-mortem examination should be requested on all patients dying from a consistent clinical illness. (C) 2000 Harcourt Publishers Ltd.