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From: TSS (
Subject: Nation's experts work to identify mystery disease (NEW STRAIN TSE?)
Date: August 18, 2004 at 7:00 am PST

-------- Original Message --------
Subject: Nation's experts work to identify mystery disease
Date: Wed, 18 Aug 2004 09:06:26 -0500
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy

Wednesday, August 18, 2004 - Page updated at 12:00 A.M.

Nation's experts work to identify mystery disease

By Sandi Doughton
Seattle Times staff reporter

The nation's top experts in mad-cow-like diseases are trying to identify
a strain discovered this summer in a patient at Harborview Medical
Center in Seattle.

The disease is clearly not the human form of mad-cow, nor does it appear
to be Creutzfeldt-Jakob Disease (CJD), 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 said yesterday. "This is unusual."

The condition of the patient was not available.

Blood tests conducted over the next two to three weeks should provide
more information on the mysterious ailment, said Gambetti, whose
Cleveland lab analyzes most of the so-called prion diseases diagnosed in
the United States.

Prions are the misshapen proteins believed to cause a family of deadly,
brain-wasting diseases that include human forms such as CJD and animal
versions such as mad-cow and scrapie. Scientists think the human form of
mad-cow disease, also called variant CJD, is probably caused by eating
infected beef. But the causes of other prion diseases are not
well-understood, though genetic factors play a role in some cases.

Because they are proteins, not microbes, prions are resistant to most
ordinary sterilization techniques. Surgical instruments used to take a
biopsy from the Seattle patient's brain were used subsequently in up to
12 other brain surgeries before being subjected to the supercleaning
prescribed for prion diseases, hospital officials said.

Human prion disease

There are five known human prion diseases:

Sporadic Creutzfeldt-Jakob disease is believed to arise spontaneously in
one person in a million and is more common in the elderly. Some cases
also have been caused by contaminated surgical instruments or
contaminated brain implants, corneal transplants and blood transfusion.

Variant Creutzfeldt-Jakob disease is believed to be caused by eating
beef infected with mad-cow disease and mainly strikes young people.

Kuru was discovered in New Guinea, where it was spread by the practice
of cannibalizing human brains.

Fatal Familial Insomnia, marked by progressive sleeplessness, is
genetically linked and has been found only in nine extended families.


Scheinker also is caused by genetic mutations and occurs in only about
50 extended families.

Sources: CDC; Medscape

But Gambetti said the risk to those patients is slight.

"The chances that this disease is transmissible are very, very low," he

The hospital is reviewing its records and will decide whether the
patients should be notified once the disease is positively identified,
said Johnese Spisso, Harborview's chief operating officer.

"We need to sit down and review all the facts so we know what to tell
people," she said. "We need to make sure we give them the best
information possible so we don't unduly alarm people."

There are only six cases on record of a prion disease  specifically CJD
 being transmitted through contaminated surgical instruments or brain
electrodes, said Dr. Ermias Belay of the U.S. Centers for Disease
Control and Prevention. And all of those cases occurred before 1976,
when common sterilization techniques were improved, said Belay, who
helped write international guidelines for prion sterilization.

It's possible the strain from Harborview is a type that can't be passed
from one person to another, even through a direct dose to the brain,
Gambetti said.

Preliminary tests show that it most closely resembles an extremely rare
prion disease named GSS for the three German scientists who discovered
it (Gerstmann-Straüssler-Scheinker). GSS can be triggered by a dozen
different genetic mutations, Gambetti said, and only one of those forms
has been shown to be transmissible in laboratory animals.

But even if the risk is minuscule, the diseases are so horrible that
hospitals always should err on the side of caution when it comes to
sterilizing instruments, said Florence Kranitz, president of the CJD
Foundation, a support and advocacy group.

Invariably fatal, CJD and its close cousins progressively destroy the
brains of their victims, robbing them of their memories and their
ability to move or speak.

"I sure don't want them doing surgery on anyone I know and love with
instruments that have been contaminated by prions," Kranitz said.

The CDC recommends that hospitals superclean instruments used for brain
surgery on all patients where prion diseases haven't been ruled out,
Belay said. Another option is to "quarantine" the instruments, not using
them again until a final diagnosis eliminates the possibility of prion

But Harborview officials say two preliminary tests on the patient were
negative for CJD, which, though it strikes only about 300 Americans a
year, is the most common human prion disease.

"In the opinion of the neurologist, this was not CJD," said Dr. Tom
Montine, the hospital's director of neuropathology.

So the hospital used its normal sterilization techniques: Soaking the
instruments in an enzyme solution, followed by a high-pressure wash and
a 272-degree vacuum autoclave.

Two weeks later, when the patient's biopsy results hinted at the
possibility of a prion disease, the hospital supersterilized the
instruments according to CDC's guidelines, which include an hourlong
soak in sodium hydroxide  the powerful caustic also called lye  before
the high-temperature autoclave.

"We feel confident we followed the recommended national sterilization
standards based on the patient's clinical information," said Harborview
spokeswoman Susan Gregg-Hanson.

She said the hospital is reviewing the case and may change its
procedures if the review finds flaws.

Similar cases over the past few years have raised concern about
sterilization techniques in the U.S., Canada and the United Kingdom.

Doctors at a Denver hospital operated on six people in 2001 with the
same instruments used to take a brain biopsy from a woman who later died
of CJD. Two of the patients are suing the hospital.

Several patients settled lawsuits filed against Tulane University
Hospital in New Orleans after a similar occurrence in 2000 may have
exposed eight people to CJD.

In 2001, Madigan Army Medical Center in Pierce County closed its
operating rooms two days for extra sterilization after discovering a
woman who had undergone surgery there four months before had CJD. The
hospital notified five people who might have been exposed.

Britain, where a mad-cow epidemic wiped out herds and infected more than
100 people, is spending more than $350 million to upgrade sterilization

Sandi Doughton: 206-464-2491 or

Copyright © 2004 The Seattle Times Company


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