Unknown Prion Disease Killing Seattle Woman

[CVX]

"... neurosurgeons used some of the same instruments to operate on 12 other patients."
By Tracy Vedder
KOMO-TV Seattle
8-19-4

SEATTLE - Doctors at Harborview Medical Center are some of the best in the country, but now they are stumped. They've got a patient with a mystery disease.

The illness is not mad cow disease, even though it looks like it. And doctors are turning to scientists at the Centers for Disease Control for help.

More:

http://www.komotv.com/news/printstory.asp?id=32675

[Warsai]

If the disease is that horrible they better do everything they can to contain it. I hope they can back up all that talk about the chance of transmission being zero.

[plazul]

CDC will figure it out. They're pretty good at getting a handle on exotic diseases.

[illuminati]

WA State - Woman Dies Of Mad Cow Type Disease

By Sandi Doughton Seattle Times 8-20-4

A woman treated at Harborview Medical Center this summer for a mysterious brain ailment related to mad cow disease [bovine spongiform encephalopathy, or the human form known as variant Creutzfeldt-Jakob 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. She 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 [neuropathology, immunoreactivity, presence of prions?] that the woman was suffering from a prion disease, a class of fatal brain ailments that include mad cow disease and its human form [variant Creutzfeldt-Jakob disease, abbreviated in ProMED-mail as CJD (new var.) or vCJD].

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 center.

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 email. "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 conducted.

Harborview is waiting for the results before deciding whether to notify 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.

http://seattletimes.nwsource.com/cgi...ment_id=200201
0596&zsection_id=2001780260&slug=braindeath20m&dat e=20040820