1996 - Ireland

21 March 1996

Disease Outbreak Reported

The UK has analyzed cases of CJD that have occurred in 10 adults below 42 years of age during the past year. The disease in these cases differs from classical CJD in several respects:

• early age (CJD is usually a disease of older people)
• differences in pathological changes in the brain
• absence of any hereditary factors (which are involved in typical CJD)

There is no direct evidence of a link between these cases and bovine spongiform encephalitis (BSE). However, in the absence of any apparent cause, the most likely explanation at present is that these cases are linked to exposure to BSE before control measures were instituted in the UK in 1989. The measures now in force in the UK are considered to have minimized any possible risk from eating beef.

No similar pattern of disease has been recognized in humans in any other country although BSE has also occurred in animals in Ireland, France, Portugal, and Switzerland. Precautions have been taken in these countries to avoid any possible risk to people from beef consumption.

WHO recommends that national health authorities be notified if any unusual occurrence of CJD is recognized in any other country.