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10 people killed by new CJD-like disease 7-9-08
A NEW form of fatal dementia has been discovered in 16 Americans, 10 of whom
have already died of the condition. It resembles Creutzfeldt-Jakob disease -
with patients gradually losing their ability to think, speak and move - but has
features that make it distinct from known forms of CJD.
No one yet knows how the disease originates, or under what conditions it might
spread. Nor is it clear how many people have the condition. "I believe the
disease has been around for many years, unnoticed," says Pierluigi Gambetti,
director of the US National Prion Disease Pathology Surveillance Center at Case
Western Reserve University in Cleveland, Ohio. Cases may previously have been
mistaken for other forms of dementia.
Since Gambetti's team wrote a paper describing an initial 11 cases referred to
his centre between 2002 and 2006 ( Annals of Neurology, vol 63, p 697), another
five have come to light. "So it is possible that it could be just the tip of the
iceberg," Gambetti says.
As in other spongiform encephalopathies, such as CJD and mad cow disease (BSE),
the brain tissue of victims is full of tiny holes. This damage is thought to be
caused by the accumulation of prions, misfolded versions of a brain protein
called PrP that can convert normal PrP molecules into their own misshapen form.
Some features of the new disease are different, however. All known
disease-causing prions resist degradation by proteases - enzymes which digest
the normal form of PrP. But prions from patients with the new disease are broken
down by the enzymes.
Some very rare forms of CJD run in families and are caused by mutations in the
gene for PrP. Six of the cases described in Gambetti's paper were from families
with a history of dementia, suggesting a genetic cause. However, these people
had no mutations in their PrP genes. "Maybe there are other genes that have an
influence on the disease," suggests James Ironside of the UK's National CJD
Surveillance Unit in Edinburgh.
Most forms of CJD develop spontaneously, for unknown reasons, but can be spread
if someone is exposed to brain material from people with CJD, for instance, by
neurosurgery using inadequately sterilized instruments.
One variant of CJD has been linked to the consumption of contaminated meat from
cattle with mad cow disease. If the new condition is similarly caused by
something in the victims' diet, or another environmental cause, new measures
might be needed to protect public health.
Gambetti is now conducting experiments in mice to see how the disease is
transmitted. He suspects that there is no cause for alarm. "I believe the
disease occurs naturally, and is not due to environmental causes," he says.
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