Surveillance of Prion Diseases in Humans

In contrast to animal prion diseases, the human forms of prion disease are rare and include Creutzfeldt-Jakob disease (CJD), kuru, Gerstmann-Str�ussler-Scheinker syndrome (GSS), and fatal familial insomnia (FFI). Meticulous epidemiological research has demonstrated that kuru was caused by cross-contamination in the course of ritual cannibalism (1 ), whereas advances in molecular biology have provided powerful evidence that familial forms of human Piion disease, including GSS and FFI, are linked to mutations within the priori protein (PrP) gene (2 -4 ). The cause of sporadic CJD remains unknown despite both extensive epidemiological research and the remarkable advances in the understanding of pathogenic mechanisms in priori diseases.