The transmission of prions to humans

The identification of new-variant Creutzfeldt-Jakob disease (nvCJD) in 1996 led to the proposal that this new disease was caused by the transmission o f bovine spongiform encephalopathy (BSE) to the human population. The ramif ications of such a proposal have been extensive and profound, both politica lly and on the general public in the UK and other countries. Patients with nvCJD exhibit a consistent set of clinicopathological features, and cases o f nvCJD continue to be reported almost exclusively in the UK, the country w ith by far the highest incidence of BSE. Laboratory studies, including tran smission experiments in mice, provide strong support for the hypothesis tha t nvCJD is caused by BSE.