Prion diseases

Prion diseases, also known as transmissible spongiform encephalopathies (TSE’s) are a group of progressive neurodegenerative conditions.  These illnesses exist in both animals and humans.  Scrapie, a disease affecting sheep and goats, was the first prion disease to be identified in the 1730s.

Most cases of prion disease are sporadic; that is, they arise spontaneously for no known reason. More rarely prion disease is inherited due to a faulty gene, or acquired by medical procedures, transfusions, or contaminated food.  Sporadic and inherited prion disease occurs worldwide in all populations.  The incidence of sporadic CJD is around 1 per million of the population per annum; males and females are equally affected.  The incidence of the various acquired prion diseases, however, is more localised to specific groups and populations.

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