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From: TSS (
Subject: Patient with mad-cow-like brain ailment dies Gambetti said, he's pretty sure it's a prion disease
Date: August 20, 2004 at 6:34 am PST

Friday, August 20, 2004 - Page updated at 12:00 A.M.

Patient with mad-cow-like brain ailment dies

By Sandi Doughton
Seattle Times staff reporter

The woman treated at Harborview Medical Center this summer for a
mysterious brain ailment related to mad-cow disease has died.

An autopsy was performed, and should help national experts in their
quest to identify the disease, said epidemiologist Dr. Jo Hofmann, of
the Washington Department of Health.

"There will be brain tissue obtained from multiple parts of the brain,
that will definitely provide more information," Hofmann said.

The tissue will be sent to the National Prion Disease Pathology
Surveillance Center at Case Western Reserve University in Cleveland.

The woman, who Hofmann said was under the age of 60, was not a resident
of Washington and did not die here. The woman has not been identified to
protect her family's privacy.

She was treated at Harborview, where doctors performed a brain biopsy,
collecting a tiny sample of brain tissue they hoped would help them
diagnose the baffling illness, characterized by dementia.

When pathologists examined the brain tissue, they saw evidence that the
woman was suffering from a prion disease, a class of fatal brain
ailments that include mad-cow disease and its human form.

Experts ruled out mad-cow disease, and a similar disorder called
Creutzfeldt-Jakob disease, but they were not able to make a definitive
diagnosis, partly because the tissue sample was so tiny.

"We did all we could, but you can't carry out all the tests on a
biopsy," said Dr. Pierluigi Gambetti, director of the national prion

So far, Gambetti said, he's pretty sure it's a prion disease, named for
the misshapen proteins that cause the formation of holes in victims'
brains. But the tissues didn't match any of the known prion diseases.

"It could be something new," Gambetti said. "This is always a possibility."

In general, larger tissue samples allow scientists to conduct tests on
different brain regions," said Dr. Tom Montine, chief of neuropathology
at Harborview.

"The brain is unlike any other organ in the body in that function is
highly localized," he said in an e-mail. "This means that small lesions
in different parts of the brain can have very different clinical outcomes."

It probably will be several weeks or longer before all the tests are

Harborview is waiting for the results before deciding whether to notify
approximately 12 patients who had brain surgery after the sick woman 
but before the hospital super-sterilized the surgical instruments used
on her.

Laboratory tests have shown that ordinary sterilization is not always
enough to destroy prions.

In very rare cases, prion diseases have been transmitted by contaminated
medical equipment.

Sandi Doughton: 206-464-2491 or

Copyright © 2004 The Seattle Times Company


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