"Human Mad Cow" - CJD - Risk For Up To 7,000+

September 20, 2002

Scientists believe more than 7,000 people in Britain could be at increased risk of contracting the human form of mad cow disease.

A preliminary study has found possible "markers" of the disease in samples taken during routine appendix and tonsil operations.

A tonsil archive is now being set up to examine the prevalence of variant Creutzfeldt-Jakob Disease.

Researchers have estimated that 120 people per million could be at increased risk of developing the disease.

Increased risk

Based on a UK population of just under 60 million, this could mean 7,200 people are at increased risk of vCJD.

But the director of the National CJD Surveillance Unit has warned that the tests might be an under-representation of infection.

Professor James Ironside said the tests used were not sensitive enough to pick up all positives for vCJD.

And Professor John Collinge of the Institute of Neurology in London said he believed that if the latest techniques had been used there could have been four times as many positives.

The research, published in the British Medical Journal, is the first estimate of the number of people who could have pre-clinical vCJD.

Experts say the disease is almost certainly caused by the agent causing Bovine Spongiform Encephalopathy in cattle.

Accumulation

The study, led by David Hilton of Plymouth's Derriford Hospital, was looking for an accumulation of prion protein, a reliable marker of the disease in animals.

The researchers studied 8,318 appendix and tonsil samples and tissue removed during postmortems or surgery from patients later confirmed as having vCJD.

Of the samples removed in tonsil and appendix operations, they found prion protein in one of them.

Among specimens taken from patients who had vCJD but had not yet displayed any symptoms, two out of the three studied tested positive for prion protein.

For the samples removed during postmortems of people with vCJD who had died, the figure was positive for 19 of the 20 studied.

http://www.sky.com/skynews/article/0,,30000-1064878,00.html