[Transmissible spongiform encephalopathies: Creutzfeldt-Jakob disease]

Different diseases of the transmissible spongiform encephalopathy (TSE) group are known to affect humans and various animals. Owing to the bovine spongiform encephalopathy (BSE) epidemic and the description of the new variant of Creutzfeldt-Jakob disease (nvCJD), which is probably linked to BSE, TSE received much attention. The nature of the causative agent is still disputed; none of the proposed concepts (prions, viruses) can explain all features. It is clear, however, that there is a genetic component in susceptibility to infection and in development of disease and that transmission may cross the species barrier. This paper gives an overview of the first results and latest developments of basic TSE research that has focused on in vivo early diagnosis and the prevention of possible (also iatrogenic) transmission.