This brief note is designed to help you respond. It usually presents in late middle-age with progressive dementia, and is usually fatal within 6 months. It is characterised by spongiform changes in the brain, but this can only readily be diagnosed at post mortem. CJD occurs worldwide at an annual incidence rate of 0.5-I per million per annum. To date, the UK annual incidence remains within expected limits - in 1994 the incidence was 0.92 per million and the provisional figure for 1995 is 0.64 per million.
Brain pathology shows marked spongiform change and extensive florid plaques throughout the brain.
As with typical sporadic CJD, symptoms in the early stages may be fairly non specific. An iatrogenic factor has not been identified, and in the absence of any other more credible alternative, a possible explanation at present is that these cases are linked to exposure from cattle infected with BSE before the SBO ban in 1989. It is not possible to predict how many further cases there will be. Even with the new variant the incidence last year was less than one in a million. It is therefore probable that most enquiries will come from those who are well but anxious about the risks to them and to their families.