Scientists have come up with a possible new treatment which could halt the devastating effects of the human form of mad cow disease. A British woman with variant Creutzfeldt-Jakob disease, who was con fined to a wheelchair, is now able to walk and feed herself after 19 days of drug therapy.
The treatment uses two drugs commonly prescribed for malaria and certain psychotic illnesses. Researchers from the University of California, San Francisco, tested them using mouse cells and found they could block the type of protein changes that occurred in the disease.
The researchers, who publish their findings this morning in the Proceedings of the National Academy of Science, withdrew the treatment and found that the mouse cells remained free of the abnormal prion protein responsible for the disease.
These drugs - quinacrine, used against malaria, and giardiasis and chlorpromazine, approved to treat schizophrenia - were later used on Ms Rachel Forber (20), from Merseyside.
She was diagnosed with suspected variant CJD in June and was confined to a wheelchair, could not recognise her parents and was given a year to live.
After 19 days of treatment at the University of California school of medicine, she was able to walk unaided, use a fork and knife and complete co-ordination tests which she previously found impossible.
The pioneering research was conducted in the laboratory of Prof 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.
British Department of Health officials have spoken to the team leading the UCSF study and are considering whether to allow trials on other variant CJD patients in Britain.
A department official said yesterday: "There are a lot of technical and ethical questions to get around, but we're determined to develop both a diagnostic test and a treatment for variant CJD."
Ms Forber's mother, Ms Jane Taylor, said the family decided to go ahead 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."
Since variant CJD was first identified in 1996, 99 people in the UK have died from the disease, including one in Northern Ireland, and there are seven more suspected sufferers. There has been one fatal case in the Republic in a woman who had lived in Britain for many years.
The drugs are apparently able to block the abnormal prion proteins that go on to cause variant CJD. The body produces its own prion proteins, which carry out a number of functions. The disease occurs when an abnormal variant of the prion protein forms, adopting a different shape and different chemical characteristics.
This prion converts the normal prion protein into the aberrant form, in time causing the brain damage and certain death typified by the disease.
Variant CJD is directly linked to the consumption of BSE-infected beef; it is thought that prions in the beef transferred in to humans to cause the disease.
-- Additional reporting, PA
