Prion disease: bovine spongiform disease and variant Creutzfeldt-Jakob disease. Clinical manifestations

Prion diseases are a group of rare but fatal neurodegenerative disorders caused by the abnormal form of the prion protein (PrP^Sc). These diseases affect both humans and animals. The most well-known example is bovine spongiform encephalopathy (BSE, "mad cow disease"), which in the 1990s led to an outbreak of a human variant - new variant Creutzfeldt-Jakob disease (vCJD). To prevent the spread of this disease, various measures were implemented.