Prion Diseases

Abstract

Prion diseases are a group of diseases affecting humans and animals, which involve central nervous system and always lead to a fatal outcome. The unique nature of prion diseases includes their pathogenesis, mode of transmission and neuropathology. They are also named as transmissible spongioform encephalopathy. Within the past 15 years, three outbreaks of environmentally acquired Creutzfeldt Jacob disease were seen asthe results of using contaminated human growth hormone, dura mater grafts and beef products from cattle with bovine spongioform encephalopathy. Especially, the transmission of the disease, breaking the species barrier, from cattle with bovine spongioform encephalopathy to humans increased the interest to these disease andreinvigorated the attempts to develop effective means of treatment and prophylaxis.