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US trials offer hope for victims of CJD

John von Radowitz
Tuesday 14 August 2001 00:00 BST
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British sufferers of the human variant of BSE are to be given the chance to try a radical new method of treatment from America that could ease their symptoms.

The news follows what researchers claim are remarkable results from US medicinal trials involving a British sufferer of variant Creuzfeldt-Jakob disease. Rachel Forber, 20, from Newton-le-Willows, Merseyside, was diagnosed with suspected vCJD in June. She was confined to a wheelchair, could not recognise her parents and was given a year to live.

But, after 19 days of treatment at the University of California School of Medicine in San Francisco, the former soldier was able to walk unaided, use a knife and fork and do co-ordination tests that she had previously found impossible.

The National CJD Surveillance Unit will write to the six British sufferers to inform them of the American treatment. Each patient would then need to judge, with their doctor, whether it was a treatment that could be useful to them, a Department of Health official said.

Miss Forber's mother, Jane Taylor, said the family used the treatment as a last resort. She told Channel 4 News: "We had nothing to lose because we were told that Rachel would die and we had everything to gain if this did work."

The mixture of drugs used are quinacrine, used to treat malaria and the tropical parasite infection giardiasis, and chlorpromazine, which is given to patients with schizophrenia and other psychotic conditions. Tests on infected mouse cells showed that using both drugs stopped prion molecules changing shape to become the "bad" form linked with vCJD.

One vitally important feature is that both are able to penetrate the blood-brain barrier – a natural "wall" put up by the body to stop toxic substances entering the brain.

A paper on the research will be published tomorrow in the US journal Proceedings of the National Academy of Sciences.

The research was conducted in the laboratory of Professor Stanley Prusiner, who won the 1997 Nobel prize for medicine for his work on prions – the infectious protein agents thought to cause brain diseases such as BSE and CJD.

Asked if the British Government might back similar studies, a Department of Health spokesman said: "That is certainly something that we would want to discuss with the researchers and the National CJD Surveillance Unit."

Since variant CJD was first identified in 1996, a total of 99 sufferers in the UK have died.

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