Creutzfeldt-Jakob disease in elderly people

Creutzfeldt–Jakob disease (CJD) is a rare neurodegenerative illness which was first described in 1921 [1]. The disease is invariably fatal and is characterized by progressive multifocal neurological dysfunction and dementia leading to death in a few months. Most cases occur sporadically, with mean age of onset at around 65 years. A small number of cases are familial, related to mutation of the prion protein gene, and there have been rare iatrogenic cases. In 1996, a new clinico-pathological variant was described, variant (v)CJD, which is caused by transmission of bovine spongiform encephalopathy from cattle to man [2]. This variant was hitherto thought to be confined to a younger age group, as the mean age at death was 29 years (range 14–53) [3]. However, the recent description of a case of vCJD confirmed at autopsy in a 74-year-old man raises the possibility that dementia in this age group may be caused by CJD, with implications for future surveillance [4].