prion diseases

Last reviewed 01/2018

Prion diseases are a varied group of rare, fatal neurodegenerative diseases. They may be inherited or acquired.

The common pathological finding is the deposition of an insoluble form of a host-encoded protein, prion protein.

Public interest was been stimulated by the link between a British epidemic of bovine spongiform encephalopathy, a bovine prion disease, and a variant of Creutzfeldt-Jakob disease.