The WA woman suffering symptoms consistent with a new strain of
Creutzfeldt-Jacob disease has died in Royal Perth Hospital.
RPH confirmed that the woman, in her early 20's, died early yesterday, most
likely from a complication of her immobility such as pneumonia or blood clots.
She had been seriously ill for more than a month.
Neurologist Graeme Hankey said it would take some weeks to establish whether
the woman had, in fact, suffered from Creutzfeldt-Jakob disease, or CJD.
A diagnosis of the extremely rare disease is only possible from an autopsy.
The woman's illness confused experts at the hospital because her symptoms
were consistent with two strains of CJD.
The first strain affects one in a million people. Most victims are elderly
and apart from about 10 per cent of hereditary cases, are randomly struck down.
But the second strain, identified last year in Britain and known as nvCJD,
has been linked to mad cow disease, or bovine spongiform encephalopathy.
Scientists in London
and Edinburgh claimed this week they had new evidence that nvCJD was the
equivalent of mad cow disease and was almost certainly contracted by eating
contaminated beef.
Despite the scientists' findings, there is still considerable scientific
debate about the link, and how it was possible to be "infected" with a
non-infectious disease.
Dr Hankey said it was extremely unlikely the woman had nvCJD. She had not
been overseas or eaten British beef, which was embargoed by the European
Union in March last year. However, it could not be ruled out.
Tim D'Arcy , a WA member of the Australian Beef Association, said health
standards
in the local beef industry were amongst the highest in the world.
"Our meatworks have to meet rigorous quality inspection criteria and high
standards are set for food retail outlets. Beef is a safe Australian food
product," Mr D'Arcy said.
He said it was misleading to link mad cow disease to the Royal Perth
Hospital patient.
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A WA woman is in Royal Perth Hospital with symptoms of a new strain of
Creutzfeldt-Jakob disease that has been linked to mad cow disease.
RPH neurologist Graeme Hankey said yesterday it was unlikely her condition
had been caused by eating infected beef or lamb.
Australia has no recorded cases of mad cow disease and no cases of the new
human strain.
"I would be quite stunned if it was", Dr Hankey said. "But I think we need
to be on the look-out."
The disease came to prominence in Britain. In March 1996, the European Union
put an embargo on British beef.
The WA woman, in her early 20's had not been overseas or eaten British beef.
She had symptoms of both strains of the disease. Steps had been taken to
rule out the possibility she had an unrelated condition that mimicked the
systems.
Diagnosis of either form of Creutzfeldt-Jacob disease,or CJD, was only
possible from an autopsy.
The first type of CJD disease occurs in one person per one million. The
average age of victims is 60. Symptoms include visual and mental deterioraton.
The second variant - known as nvCJD-was identified in Britain last year. Of
about 16 cases worldwide there are no known Australian victims.
The nvCJD hits younger victims and there are additional symptoms of
behavioural and psychological disturbances slurred speech and lack of
coordination.
Peter Buckman, Agriculture WA's acting chief veterinary officer, said there
were no animals infected with mad cow disease in Australia.
End.