ABSTRACT
The human prion diseases, also known as the transmissible spongiform encephalopathies, have been traditionally classified into Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler syndrome (GSS; also known as Gerstmann-SträusslerScheinker disease) and kuru. Remarkable attention has been recently focused on these diseases because of the unique biology of the transmissible agent or prion, and also because of the epidemic of bovine spongiform encephalopathy (BSE) and the evidence that BSE prions have infected humans, causing the new human prion disease known as variant CJD (vCJD). Human prion infection can be associated with extremely long incubation periods, which may span decades, and considerable uncertainty remains as to the eventual epidemic size of BSE-related human prion disease and the risks of its secondary transmission via medical and surgical procedures.
