Abstract: Objective Prion diseases, also known as transmissible spongiform encephalopathies, are a group of rapidly progressive neurodegenerative diseases that can affect different species. The first case was found in a farm in the UK in 1986, and then the huge outbreak reached the peak in 1992. While, the variant Creutzfeldt-jakob disease (vCJD) due to consumption of bovine spongiform encephalopathy (BSE) beef appeared in 1995 and reach a peak in 2000. Because of the strict ban, thorough culling and active surveillance, the BSE and vCJD in the past three years has not been completely reset, but the possibility of a mass rally in the short term is very limited. Global human prion disease began in 1993 in part of Europe countries and extended to most of countries soon including Europe, North America, Oceania and northeast Aisa and the surveillance system was established in China since 2006. Establishment of monitoring system is to prevent a catastrophic public health problem again.