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03-24-2005, 04:30 PM
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.
--
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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
Get Copyright Clearance <http://vcs.valeoip.com/3.5981?icx_id=20050323-061733-6847r> Want to use this article? Click here for options! <http://vcs.valeoip.com/3.5981?icx_id=20050323-061733-6847r>
Copyright 2005 United Press International
http://www.upi.com/view.cfm?StoryID=20050323-061733-6847r
http://www.upi.com/view.cfm?StoryID=20050323-061733-6847r
http://www.washtimes.com/upi-breaking/20050323-061733-6847r.htm
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