WHAT WENT WRONG IN BSE - FROM PRION DISEASE TO PUBLIC DISASTER

The recent report of 10 cases of a new variant of Creutzfeldt-Jakob di sease (CJD) which could be related to bovine spongiform encephalopathy (BSE) has precipitated alarm throughout Europe, The beef trade in the UK has collapsed and the European beef market has been seriously dama ged, What went wrong? Much of the difficulty of handling the BSE epide mic arose from the 4-5 year incubation period which made it difficult to ascertain whether measures taken to contain the epidemic had been e ffective, Public consternation and scientific equivocation arose becau se these prion diseases are unlike any other group of infectious disea ses, Rather than being caused by a conventional micro-organism, the pr imary pathogenic event consists of the transformation of a normal prot ein (the prion protein) into an abnormal form, which can transmit dise ase, Prion disease is endemic in humans and sheep where it is associat ed with polymorphisms or mutations within the prion protein gene, Alth ough the disease in these cases arises spontaneously, it produces an i nfectious prion protein, Under certain circumstances, abnormal prion p rotein contaminates other animals or humans resulting in epidemics of acquired prion disease, This review describes the events of the BSE ep idemic and considers the difficulties in assessing the current risk to human health.