Medical experts are confused about the death of a woman who was recently admitted to Harborview Medical Center in Seattle, with severe dementia. Blood
testing is currently underway to find out why the woman from Alaska, died of a mysterious brain-wasting disease, but had tested negative for
Creutzfeldt-Jakob disease (CJD) in two preliminary tests. They have also said that it was clearly not mad-cow, also known as Bovine Spongiform
Prion disease experts study woman's death
A Harborview physician who cared for the patient said they found no test evidence of the proteins indicating either Creutzfeldt-Jakob disease or mad
"We know what it's not, but it's way too early to say what it is," said Dr. Thomas Montine, director of neuropathology at Harborview and the
University of Washington.
Montine said there were signs the dementia in this patient could have been caused by an extremely rare form of prion disease known as
Gerstmann-StraŘssler-Scheinker (GSS), but even that was not a close match. GSS typically occurs in families, indicating a genetic cause. Please visit the link provided for the complete story.
The disease seems to have caused this woman to lose portions of her brain, which just disappear, resulting in dementia and worse. What is interesting
is that most testing has so far, failed to identify what exactly killed the middle-aged woman. It certainly seems to be a prion type of disease just
like mad-cow or CJD, but yet doesn't show up in testing. Doctors did not realize the contition until making a biopsy of her brain.
It was clearly not the human form of mad cow, nor does it appear to have been Creutzfeldt-Jakob Disease, a closely related condition, said Dr.
Pierluigi Gambetti, director of the National Prion Disease Pathology Surveillance Center.
"We really are puzzled at this time," he told The Seattle Times. "This is unusual."Please visit the link provided for the complete story.
The biopsy of the woman's brain had indicated that a prion disease was probable, and now the hospital is checking it's procedures of instrument
sterilizing, since those same instruments were also used on as many as 12 more patients. Prion diseases are known to resist standard sterilization
procedures, so now the hospital is supersterilizing the instruments according to CDC's guidelines.
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