[Prion diseases: current views]. / Prionnye bolezni: sovremennyi vzgliad na problemu.

This is a review of the problem. The above diseases represent a peculiar type of fatal neurodegenerative diseases of man and animals provoker of which is prion--a low molecular anucleinic protein resistant to inactivation. Human prior diseases include fatal familial sleeplessness, Kreutzfeldt-Jakob disease, kuru, Gerstmann-Streussler-Sheinker's syndrome, etc. Prion proteins possess a characteristic capacity to change a tertiary conformation resulting in the formation of a pathological form of protein having infectious properties. Pathogenesis, morphological and clinical manifestations as well as principles of diagnosis and treatment of these diseases are discussed.