Mad cow disease and Creutzfeldt-Jakob disease--is there a link?

The report of the Creutzfeldt-Jakob Surveillance Unit from March 1996 regarding 10 cases of a new variant of Creutzfeldt-Jakob disease (CJD) in young adults caused a great deal of uproar when it was suggested that a possible link with bovine spongiform encephalopathy (BSE) could not be excluded. BSE was first noticed in 1986 after the introduction of modified rendering systems in the manufacture of meat and bone meal containing animal wastes contaminated with scraple-like agents. This article reviews available information on CJD, BSE and other diseases caused by prions, transmission studies and the report of the CJD Surveillance Unit and discusses possible links between BSE and the new variant of CJD.