The transmissible subacute spongiform encephalopathies, or prion diseases, have a similar noninflammatory spongiform pathology and are caused by a similar transmissible agent -- an abnormal (“scrapie-like”) protease-resistant conformation of the prion protein (PrP), which is designated PrPSc. Sporadic human prion diseases include Creutzfeldt-Jakob disease and fatal sporadic insomnia. Prion diseases acquired by infection include kuru, variant Creutzfeldt-Jakob disease, and iatrogenic Creutzfeldt-Jakob disease. Familial human prion diseases include familial Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome, and fatal familial insomnia.
Apr. 24, 2021