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WA State - Woman Dies
Of Mad Cow Type Disease

From Patricia Doyle, PhD
dr_p_doyle@hotmail.com
8-22-4
 
Jeff -
 
According to the "confusing" information below, it appears that the brain disease was a prion disease however, it appears to be a novel or mutated disease. I expect to hear nvCJD and BSE soon to be suffixed with LIKE.
 
It was unclear if the woman lived in Canada or the US. I have to wonder how the funeral home will handle fluids, tissues etc etc. Will any of the fludis, tissues be flushed into area sewer system, as is the case with funeral parlors.
 
We know that mad cow disease has become atypical or mutated in bovine, therefore one can expect to hear a similar situation arise in humans.
 
It would be very helpful to know what meats the woman consumed, such as deer or elk meat, or where the woman lived. Could she have consumed the US/Canadian mad cow found in the US? We also do not know about her travel background, i.e. had she visited Europe, the UK or other BSE/nvCJD infected country.
 
Hopefully, results will become available soon. I do think that the autopsy should give us a clue as to area of the brain that became spongified which would give us a clue as to which prion disease was closely resembled.
 
In any event, this news is not good.
 
Patricia Doyle
 
From ProMED-mail
 
 
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/cgibin/PrintStory.pl?document_id=200201
0596&zsection_id=2001780260&slug=braindeath20m&date=20040820
 
 
ProMED-mail promed@promedmail.org
 
[This report is too vague and preliminary to evaluate. The basis for the conclusion that the disease is vCJD/BSE-like, but neither vCJD nor (sporadic) CJD, is unclear to the moderator. Examination of the biopsy material may establish whether a prion is present which resembles any of the 4 common PRP/Sc types found in humans to date: types 1-3 in sporadic and iatrogenic CJD, and type 4 associated exclusively with vCJD. A proper scientific account of this case is awaited. - Mod.CP]
 
Patricia A. Doyle, PhD
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Zhan le Devlesa tai sastimasa
Go with God and in Good Health





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