New study revives fears of CJD epidemic

New study revives fears of CJD epidemic

May 15, 2001 Press Association Newsfile by John von Radowitz

New research into the human form of mad cow disease today revived fears that a devastating epidemic is about to explode on the population of Britain [The research was published in the 5/15/01 issue of the Proceedings of the National Academy of Science--BSE coordinator].

One expert said the possibility of millions of deaths could not be ruled out.

Relatives of victims of variant CJD (vCJD) were said to be living in fear after learning that they may share a genetic susceptibility to the disease.

The findings from Professor John Collinge's prion unit at Imperial College, London, indicate that the incubation period of the illness may vary greatly depending on the genetic make-up of its victims.

If this is true, the 99 cases that have surfaced so far could be genetically disposed to have the shortest incubation periods.

Others may have to wait much longer before succumbing to the dreadful brain disease which spread to humans in meat from cows infected with BSE.

What has emerged up to now may merely be the tip of a large and terrifying iceberg.

Furthermore, the epidemic may appear to subside, lulling people into a false sense of security, only to return with a vengeance as the disease takes hold of a new genetic group.

The head of the scientific body advising the Government on BSE and its human equivalent, vCJD, admitted that usual assumptions about the progress of epidemics might have to be abandoned.

Consultant microbiologist Dr Stephen Dealler said the new data were entirely compatible with his own prediction that the number of cases will very soon start to rise rapidly, peaking between 2010 and 2020.

He stands by his claim, first made in 1993, that BSE transmitted to humans in contaminated beef could eventually strike down as many as five million people.

His forecast came three years before vCJD, a new strain of the human brain disease triggered by infected beef, was officially recognised. At the time the Government was insisting there was no evidence of any link between BSE and CJD.

Dr Dealler said of the new research today: "In a way, it's not surprising to find this out.

"Frankly, I'm surprised the total (number of cases) is as high as it is at this stage. But the figures can be expected to get much, much higher by the year 2010, doubling or tripling each year."

Prof Collinge's team discovered the location of three new genes in mice that help determine the incubation periods of prion diseases such as scrapie, BSE, and variant CJD.

The scientists think it is almost certain that corresponding genes exist in humans, since mice and humans share so many genetic similarities.

All those who have developed the disease so far have been from a genetic group labelled MM.

Prof Collinge, whose findings are reported in the journal Proceedings of the National Academy of Sciences, said: "It is likely that within the MM group there are differing degrees of susceptibility to prion disease and that the human counterparts of the genes we are now locating in mice will be crucial to this. Those patients we have seen so far with vCJD may be those genetically disposed to have the shortest incubation periods."

While taking care not to speculate about figures, he warned there was no room for complacency.

"We are most familiar with epidemics that develop rapidly over weeks and months, like influenza and foot-and-mouth disease," he said.

"Prion diseases develop over a quite different time-scale and we cannot rule out an epidemic that evolves over decades. It will be very important to identify and characterise these new genes to help us understand the risks involved."

Frances Hall, secretary of the Human BSE Foundation, whose son Peter was one of the first victims of vCJD, said the findings had come as a shock.

She said from her home in Chester-le-Street, County Durham: "It's quite frightening. You tend to think if you have lost a member of your family five years ago that the danger period might be receding for you, but that doesn't necessarily follow.

"We are watching each other now. I have another son, so I worry very much that he ate what Peter ate. You try to push it to the back of your mind, but every time they discover something like this, it's right there again.

"I just hope they concentrate on research into cures that can halt this disease, because the damage is already done."

Peter died in 1996 aged 20. His parents received the post-mortem report the day before former Health Secretary Stephen Dorrell told the Commons that a new disease had been identified which was probably caused by infected beef.

Mrs Hall's other son John is now aged 30.

She stopped buying beef for her family in 1989 when concerns were first raised about BSE, but it was too late for Peter.

"I will never, ever eat beef again," she added.

Prof Smith said it was impossible to make meaningful predictions about the future size of the epidemic.

"I think the outlook is neither optimistic nor pessimistic," he said. "It's merely a case of saying that predicting the epidemiology of this disease is complicated."

He said identifying susceptible genetic subgroups might some time in the future provide a means of screening for vCJD. But there would be no point in that unless a treatment was available.

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