'Human mad cow disease' deaths double

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The Independent Online

Science Editor

Twice as many people died from Creutzfeldt-Jakob Disease (CJD) - thought to be the human equivalent of mad cow disease - in Britain last year, compared to 1985, according to a government report published yesterday.

A 19-year-old died from the incurable degenerative brain disorder this year, a case which the report notes is "exceptional, but not without precedent". Last week, a medical journal reported the death of a third dairy farmer from the disease.

The 1994 toll of 55 deaths represents the highest figure yet recorded in Britain since fears were first raised that mad cow disease - bovine spongiform encephalopathy (BSE) - might be transmissible to people either by contact with infected animals or by eating their meat.

But Dr Rob Will, the country's leading expert on the incidence of CJD and one of the authors of the report, said yesterday that the rise might be more apparent than real.

Cases which might have been overlooked in the past, may be showing up simply because greater efforts are being made to track them down and obtain a correct diagnosis.

Dr Will stressed that the reported incidence of the disease was increasing in other countries, ones which had little or no history of BSE among their cattle.

"The incidence in the UK is comparable to France, the Netherlands and to a lesser extent Italy," he said. He added that he was continuing to eat beef and sausages.

But Dr Will expressed surprise at a report in The Lancet last week highlighting a third case of CJD among dairy farmers. "The Lancet said it was a matter for concern and I agree with that," he said.

The report also notes that "it would be premature" to see the death of the 19-year-old as caused by BSE.

It said: "CJD has been described previously in two adolescents in the USA which is free of BSE and in one adolescent in France which was free of BSE at the time of the patient's clinical illness."

Statistically, venison-eaters proved more likely to develop the disease last year, but the report warns that the number of cases is so small that the statistics are unreliable. The link detected between regular veal eating and CJD in 1993 was not repeated in 1994.

The figures are contained in the fourth annual report of the Edinburgh- based CJD Surveillance Unit, set up to study any changes in the pattern of the disease since the emergence of BSE.

There are no known cases of recovery from CJD. The incubation period for the disease can be as long as 30 years, but no sufferers are known to have survived more than two years after symptoms develop.

The first symptoms are often a mild dizziness and clumsiness, which rapidly develop until the sufferer loses all control over movement and speech.

Seventeen people are known to have contracted CJD after receiving growth hormone treatment - using extracts from the pituitary glands of corpses - as children. The practice was ceased in 1985 after the link was spotted.