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From: TSS ()
Subject: French woman may have had vCJD in 1971
Date: March 24, 2005 at 6:33 am PST

-------- Original Message --------
Subject: French woman may have had vCJD in 1971
Date: Thu, 24 Mar 2005 08:25:55 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@KALIV.UNI-KARLSRUHE.DE


##################### Bovine Spongiform Encephalopathy #####################


French woman may have had vCJD in 1971

By Steve Mitchell
Medical Correspondent
Published 3/24/2005 8:53 AM

WASHINGTON, March 24 (UPI) -- The brain of a French woman who died in
1971 shows evidence consistent with human mad cow disease, United Press
International has learned, a finding that if confirmed would indicate
the deadly disease began infecting people more than 20 years earlier
than previously thought.

A former National Institutes of Health scientist said he tested the
woman's brain in 2000 and it showed a pattern that looked like variant
Creutzfeldt-Jakob disease -- a fatal, brain-wasting illness humans can
contract from eating beef products infected with the pathogen that
causes mad cow disease, also known as bovine spongiform encephalopathy
or BSE.

VCJD was unheard of in 1971. The first recognized case was detected in
the United Kingdom in 1995, so if the French woman did indeed suffer
from vCJD, the case would shift the origins of the disease back more
than two decades and possibly to a different country. The woman's brain
is held at the National Institutes of Health in Bethesda, Md.

"Variant CJD could've been around for donkey's years, who knows?" said
Bruce Johnson, a former researcher at the NIH's Laboratory for Central
Nervous System Studies, who examined the woman's brain. The CNSS lab
received brains from CJD patients from all over the world and has
samples dating back to 1963. The woman's identity could not be revealed
for confidentiality reasons, but it is known she was French and
approximately 40 to 50 years old when she died in 1971, Johnson said.

Johnson told UPI he tested the woman's brain using a technique called
Western blot, which detects prions -- infectious agents thought to play
a role in causing vCJD and similar diseases.

At the time of her death, the woman was thought to be suffering from
sporadic CJD, a condition with no known cause that appears to arise
spontaneously. However, Johnson said, the prions he detected looked
different from those associated with CJD and instead were consistent
with the prion strain associated with vCJD.

The pattern on the test "was more like BSE than CJD," Johnson said,
noting he never saw a pattern like that in the hundreds of other brains
from CJD patients he had tested.

A sample of the woman's brain had been injected into a chimpanzee
sometime around 1977, and when Johnson examined the chimpanzee's brain,
it, too, showed a pattern consistent with vCJD -- not sporadic CJD.

"So she may have been an early case of BSE in France before it ever got
to England," he said.

Johnson said he never published his finding because he wanted to confirm
it, but he never had an opportunity to do so before he retired in 2003.
The CNSS lab was officially closed in April 2004.

He said he hopes to conduct further examinations of the woman's brain
when he starts a new position with the Food and Drug Administration.

"If we've still got her brain, we can look and see if it's BSE," he
said. One possible way is to inject some of the woman's brain into mice.

Mad cow first showed up in humans in the United Kingdom beginning around
1995. In all, 154 people in that country have been infected with the
human equivalent of mad cow disease.

France runs a distant second in vCJD cases with nine. A recent report
published in the journal Veterinary Research estimated that from 1980 to
2000 more than 300,000 cattle were infected with BSE in France, yet went
undetected.

Stephen Dealler, a microbiologist at Lancaster Royal infirmary, recently
proposed a hypothesis that some of the people who developed vCJD in the
United Kingdom may have been exposed to BSE in baby food beginning as
early as 1970.

Johnson subscribes to the hypothesis put out by his NIH colleague Joe
Gibbs, who thought it was possible that all mammals, including cows,
spontaneously develop a mad cow-like disease at the rate of one per
million. If that is true, Johnson said, the French woman may have
developed her condition from being unfortunate enough to have eaten
infected meat from that one in a million animal.

Dr. Paul Brown, former medical director of the CNSS lab and an expert on
CJD and BSE, worked with Johnson. He told UPI he remembered Johnson
mentioning the French woman's brain, but the information did not sound
conclusive.

He said more research would need to be done to determine if the woman's
disease was variant CJD, including injecting it into laboratory animals
and having CJD experts examine the brain tissue.

Patient advocacy representatives had mixed reactions.

"I would be looking to get the opinion of more than one CJD neurologist
before making any further comment," Graham Steel, vice-chair of the
patient advocacy group Human BSE Foundation in the United Kingdom, told UPI.

"It doesn't surprise me at all that you can find a vCJD case in the
NIH's brain collection," said Terry Singeltary, who is associated with
several CJD patient groups and closely monitors developments about these
diseases.

"It wouldn't surprise me for it to go back that far," Singeltary, whose
mother died of a type of CJD called Heidenhain variant in 1997, told
UPI. "A lot of scientists believe this BSE epidemic started way before
1984."

Johnson said it was possible there could be other vCJD cases in the
NIH's collection, which consists of brain samples from hundreds of
patients thought to have CJD.

That may never be known, however. The brains have never been screened
for vCJD and the NIH may destroy part or all of the collection.

--

E-mail: sciencemail@upi.com

Copyright © 2001-2005 United Press International

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Copyright 2005 United Press International


http://www.upi.com/view.cfm?StoryID=20050323-061733-6847r

TSS


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