The emergence of bovine spongiform encephalopathy and related diseases

Since 1986, approximately 170,000 cases of bovine spongiform encephalopathy (BSE) have occurred among approximately one million animals infected by contaminated feed in the United Kingdom. A ruminant feed ban in 1988 resulted in the rapid decline of the epidemic. Transmissible spongiform encephalopathies due to agents indistinguishable from BSE have appeared in small numbers of exotic zoo animals; a small outbreak among domestic cats is declining. Creutzfeldt-Jakob disease (CJD) has been intensively monitored since 1990 because of the risk BSE could pose to public health. In 1995, two adolescents in the United Kingdom died of CJD, and through the early part of 1996, other relatively young people had cases of what became known as new variant CJD, whose transmissible agent (indistinguishable from that of BSE) is responsible for 26 cases in the United Kingdom and one in France. Areas of concern include how many cases will appear in the future and whether or not use of human blood and blood products may cause a second cycle of human infections.