The new variant of Creutzfeldt-Jakob disease

New variant Creutzfeldt-Jakob disease (nvCJD) is a novel human transmissible spongiform encephalopathy which was first identified in 1996 in the United Kingdom (UK). Subsequent scientific studies have revealed that the strain of the transmissible agent responsible for nvCJD is identical to that of the bovine spongiform encephalopathy (BSE) agent, and the disease has been considered as 'human BSE'. By 31 December 1999, 52 cases of nvCJD had been reported (49 cases in the UK, two cases in France and one case in the Republic of Ireland). All these individuals were under 53 years of age and all those tested were methionine homozygotes at codon 129 of the prion protein gene. The number of cases of nvCJD likely to occur in the future is impossible to estimate because of multiple uncertainties, in particular the disease incubation period, the degree of exposure to the infective agent and the susceptibility of other genetic subtypes. Continued surveillance of both BSE and CJD is required in the UK and in other countries, to ensure that the scale of this potential epidemic is adequately monitored and that all possible steps are taken to prevent further human exposure to the BSE agent.