The new variant of Creutzfeldt-Jakob disease

New variant Creutzfeldt-Jakob disease (nvCJD) is a novel human transmissibl e spongiform encephalopathy which was first identified in 1996 in the Unite d Kingdom (UK). Subsequent scientific studies have revealed that the strain of the transmissible agent responsible for nvCJD is identical to that of t he bovine spongiform encephalopathy (BSE) agent, and the disease has been c onsidered as 'human BSE', By 31 December 1999, 52 cases of nvCJD had been r eported (49 cases in the UK, two cases in France and one case in the Republ ic of Ireland). All these individuals were under 53 years of age and all th ose tested were methionine homozygotes at codon 129 of the prion protein ge ne. The number of cases of nvCJD likely to occur in the future is impossibl e to estimate because of multiple uncertainties, in particular the disease incubation period, the degree of exposure to the infective agent and the su sceptibility of other genetic subtypes. Continued surveillance of both BSE and CJD is required in the UK and in other countries, to ensure that the sc ale of this potential epidemic is adequately monitored and that all possibl e steps are taken to prevent further human exposure to the BSE agent.