In conjunction with the epidemic of bovine spongiform encephalopathy (BSE) in England a new variant of the human form of this disease, Creutzfeldt-Jakob disease (CJD), came to the foreground of medical interest. It was formerly rare and therefore not a well known fatal disease. The presented article is a brief review of contemporary knowledge on clinical aspects, pathogenesis, epidemiology, diagnosis and prevention of the disease incl. characterisation of the new variant of CJD and its differences from the classical sporadic form of the disease.

        Key words: Creutzfeldt-Jakob disease - bovine spongiform encephalopathy - prions - neurodegenerative diseases