A LINK with BSE was "perhaps the most plausible explanation" for a new strain of the fatal brain disease CJD, according to a scientific paper detailing findings which caused the beef scare. The paper was published in the Lancet medical journal yesterday.
Scientists monitoring cases of CJD said that the findings were "a cause for great concern" and warned that, if the diseases were linked, more cases of the new strain were likely to emerge.
It was the discovery of 10 unusual cases of CJD (Creutzfeldt Jakob Disease) which led the scientists to announce that BSE had probably been passed on to humans through infected beef.
CJD is the human equivalent of BSE (Bovine Spongiform Encephalopathy). Both destroy the brain, leaving it riddled with holes.
A team led by Dr Robert Will, head of the National CJD Surveillance Unit in Edinburgh, raised the alarm after learning of the new cases. All were much younger than most previous victims, had unusual symptoms at the start of their illness and took twice as long to die as other patients. "Plaques" of the abnormal prion protein - believed to cause the disease - appeared in their brains.
Because CJD is thought to have an incubation period of five to 15 years, it was assumed that they had been infected before 1989, when new controls on BSE infected offal came into force in Britain.
The paper revealed that the 10 cases were unlike 197 others investigated since May 1990.
Eight of the victims died at ages ranging from 19 to 41; two patients, aged 18 and 31, survived.
Information on potential risk factors was available for nine of the 10. None had a history of possible exposure to CJD through neurosurgery or treatment with human growth hormone. One had worked as a butcher for two years in the 1980s, and another had visited an abattoir for two days.
None had ever worked on live stock farms, although one had spent a week's holiday on a dairy farm which had no record of BSE in its herd. All nine had eaten beef or beef products in the past 10 years, but none was reported to have eaten brains, the most likely site of BSE infection. One victim had been a strict vegetarian since 1991.