Presentation 6 pryon diseasesbatsuuri nantsagPrion diseases are progressive neurodegenerative disorders caused by abnormal prion proteins that induce misfolding of normal prion proteins in the brain. They have long incubation periods and cause spongiform changes and neuronal loss without inflammation. Known prion diseases include Creutzfeldt-Jakob disease (CJD), variant CJD, bovine spongiform encephalopathy (BSE), chronic wasting disease, and scrapie in animals. BSE peaked in the UK in 1993 but has since declined due to control measures like banning high-risk materials from animal feed.