[Human diseases caused by prions].

This paper reviews current data on prions, their complex molecular biology, especially in relation to the actual discussions on bovine spongiform encephalopathy and it possibly being connected with development of certain diseases in humans. We discussed various forms of Creutzfeldt-Jakob disease (classical, iatrogenic, atypical forms) in detail, but also other diseases connected with pathogenic effects of prions (Gerstmann-Straussler-Scheinker Syndrome (GSS), fatal familial insomnia etc.). This report describes clinical characteristics and diagnostic possibilities discussing their epidemiology and numerous inexplicible problems which remain subjects of further intensive investigations.