[Interesting not only as differential Alzheimer dementia diagnosis. Bovine spongiform encephalopathy and other prion diseases].

Prion diseases are rare neurodegenerative pathologies that are associated with dementia and various neurological deficits. They may present sporadically, as familial, or as infectious diseases. The most important differential diagnosis is Alzheimer's disease. Prion diseases have aroused particular interest ever since the appearance of BSE (mad cow disease), a prion disease of cattle that can apparently be transmitted to humans and which is the cause of a new variant of Creutzfeldt-Jakob disease.