New variant Creutzfeldt-Jakob disease

Surveillance of Creutzfeldt-Jakob disease (CJD) was initiated in the UK in May 1990 in order to identify any change in the characteristics of this condition following the occurrence of bovine spongiform encephalopathy (BSE) in British cattle. In 1996 a novel form of CJD, designated new variant CJD (nvCJD), was identified by the CJD Surveillance Unit and it was proposed that these cases might be causally linked to the occurrence of (BSE). Since the publication of an article describing the cases and the predominantly epidemiological evidence implying a causal link [1], there has been extensive comment and debate on the strength of this data. In this article the overall evidence in relation to the proposed causal link between BSE and nvCJD is reviewed.