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Prion diseases are rare degenerative diseases of the central nervous system in humans and some other mammals. Three different etiopatogenetical forms of these diseases are recognised in humans, being, as such, unique in medical praxis. Results of numerous researches show the existence of prion, proteinaceous infective particle having physical and chemical characteristics of amyloid, as the common causative agent. Prion originates either in central nervous system or is introduced to it from the outside. The occurrence of the new variant of Creutzfeldt-Jakob’s disease, the most researched form of the human prion diseases, recently described in Great Britain and France, causes great concern due to the eventual possibility of the transmission of bovine spongiform encephalopathy to humans. The above described put the prion diseases at the unique position, imposing the need of being continuously informed about the current status of research to medical students and staff concerned.