CJD occurs worldwide at an annual incidence rate of 0.5-1 per million population per annum. To date, the UK annual incidence remains within the worldwide range - in 1994 the incidence was 0.92 per million and the provisional figure for 1995 is 0.64 per million. The CJD Surveillance Unit analysis of cases includes detailed clinical and neuropathological investigation and full dietary, medicinal and occupational history. In addition, the Unit is carrying out a case control study, and has retrospectively analysed cases presenting before 1990. The Unit's findings are published annually.
Brain pathology shows marked spongiform change and extensive amyloid plaques throughout the brain.
As with classic Sporadic CJD, symptoms in the early stages may be fairly non-specific. Other tests are currently under investigation and are likely to prove useful adjuncts to diagnosis. There is no cure. However, all neurologists, including paediatric neurologists, have been advised of the new findings in detail by the CJD Surveillance Unit. Total CJD figures for the first quarter of 1996 do not show an increase compared with the same period in 1995.