http://news.bbc.co.uk/hi/english/health/newsid_250000/250522.stm
January 8, 1999
CJD discovery in mice
Mice were cured of scrapie
A chemical that experts believe may be able to treat the brain disease
CJD has produced spectacularly successful results in mice.
A team from the Institute for Animal Health in Edinburgh inoculated mice
that had been infected with scrapie - a disease of the brain similar to
CJD - with the chemical pentosan polysulphate (PS).
Animals injected with one milligram of PS appeared to be completely
protected against the scrapie. They died from unrelated causes between
500 and 707 days after they were "inoculated" with the drug.
Mice who received no PS died of scrapie within 428 days.
The incubation period for scrapie was increased by up to 66% for doses
of PS as small as 250 micrograms.
The researchers say further studies are now needed to test whether PS
can reduce the risk of CJD.
Dr. Moira Bruce, head of pathology at the institute, said the previous
research had shown PS to be effective in treating scrapie, but not in
such small, single doses.
The Edinburgh research is also the first time that animals have been
successfully treated after being infected. Previously, they were
inoculated before being infected with the disease.
Dr. Bruce said: "This research shows that a very small dose can produce
really quite a dramatic effect if you get the time right."
Dr. Bruce warned, however, that much more work was needed before the
drug was used on humans. She said there would be problems identifying
which people were carrying the CJD infection soon enough to ensure PS
could work.
Thirty-five people in the UK have been diagnosed as suffering from new
variant CJD since scientists said in March 1996 that they had evidence
it could be contracted by eating beef from animals suffering from BSE,
or mad cow disease.
CJD, like BSE and scrapie, causes the brain to develop a spongy texture
known as spongiform encephalopathy.
At present, there is no treatment to slow or halt nv-CJD, and the
diagnosis is usually made when patients are terminally ill.
During the long incubation period when there are no symptoms, there is a
risk that the infection may be transferred by blood transfusion,
treatment with blood products, transplantation or reuse of surgical
instruments.
PS is already licensed in the US for the treatment of a form of
cystitis.
Microbiologist Dr. Stephen Dealler, of Burnley General Hospital, is
working on a treatment for CJD in humans. He said the Edinburgh research
was "very exciting".
"Pentosan does not just prevent the disease from progressing in animals,
it actually gets rid of it," he said.
"The potential importance of this drug to humans cannot be
underestimated."
--
Judith Richards, London, Ontario, Canada
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