Prion diseases or transmissible spongiform encephalopathies are a family of rare progressive neurodegenerative disorders that affect both humans and animals. A recent confirmed case of bovine spongiform encephalopathy in Scotland has brought this devastating group of diseases, that are usually rapidly progressive and always fatal, back into focus.
Prion diseases
Tee BL, Longoria Ibarrola EM, Geschwind MD. Neurol Clin 2018; 36 (4): 865–97.
Prions diseases are uniformly fatal neurodegenerative diseases that occur in sporadic, genetic and acquired forms. Acquired prion diseases, caused by infectious transmission, are least common. Most prion diseases are not infectious, but occur spontaneously through misfolding of normal prion proteins, or genetic mutations in the prion protein gene. Although most prion diseases are not caused by infection, they can be transmitted accidentally. Certain infection control protocols should be applied when handling central nervous system and other high-risk tissues. New diagnostic methods are improving pre-mortem and earlier diagnosis. Treatment trials have not shown improved survival, but therapies may be available soon.
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