Abstract

The diagnosis of Creutzfeldt-Jakob disease (CJD) can only be confirmed by brain biopsy or at necropsy, although a rapidly progressive dementia, myoclonus, other neurological signs, and a characteristic electroencephalogram allows confident ante-mortem diagnosis in typical cases, albeit at a relatively advanced clinical stage. A new clinicopathological type of CJD, new variant CJD (nvCJD) has been reported in the UK and putatively linked on epidemiological grounds with dietary exposure to bovine spongiform encephalopathy (BSE).