Creutzfeldt-Jakob disease is a neurodegenerative disorder characterised by a rapidly progressing dementia and early neurological symptoms. Recently it was established that exposure to meat contaminated with bovine spongiform encephalopathy (bse) may cause a new variant of Creutzfeldt-Jakob disease in which prominent psychiatric symptoms may be found, notably in the early phase. Recognition of this disease in an early stage is therefore of importance to psychiatric practice.