- WASHINGTON (UPI) -- Brain
samples from a California man whose neurologist suspected may have been
the first person to acquire human mad cow disease in the United States
have been sent to France to be re-analyzed, United Press International
has learned.
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- Patrick Hicks, 49, of Riverside, Calif., died late last
year. U.S. authorities in January ruled out variant Creutzfeldt Jakob disease,
or vCJD, which humans can contract from eating beef products contaminated
with the mad cow pathogen.
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- Both Hicks' family and his neurologist, Dr. Ron Bailey
of Riverside Medical Center in Riverside, Calif., thought there still were
unanswered questions about the final diagnosis and recently arranged for
brain samples to be sent to experts in France.
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- Bailey said Hicks had symptoms consistent with vCJD,
and his relatively young age also made him a possible candidate for the
disease, which typically affects those under the age of 55.
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- If Hicks did in fact suffer from vCJD, he could be the
first case of the disease due to consumption of domestic beef since his
family said he was a heavy eater of beef, never traveled outside of the
country and had not undergone any medical procedures that would put him
at risk of contracting the disease.
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- Only one confirmed case of mad cow has been detected
among U.S. herds and the only U.S. citizen to contract vCJD is thought
to have gotten it in the United Kingdom, where she grew up. More than 150
people around the world have contracted vCJD.
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- The tissue will be examined by Dr. Jean Jacques Hauw
at the Laboratoire De Neuropathologie at the Groupe Hospitalier Pitie-Salpetriere
in Paris, Debbi Hicks, Patrick's sister, told UPI.
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- Debbi Hicks lives in France and helped arrange for the
samples to be transferred to Hauw, who plans to inject Patrick's brain
tissue into experimental animals. Scientists say this is a definitive way
to determine whether Hicks suffered from vCJD or a similar condition called
sporadic CJD that has no known cause.
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- "It'd be nice to see if there's anything better
we can find out about Pat and if it could help other people who are in
the same situation," Debbi Hicks said.
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- Bailey said he thought the National Prion Disease Pathology
Surveillance Center in Cleveland, which conducted the initial examination
of Hicks' brain, ruled it sporadic CJD without conclusive proof.
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- "The question is does he have variant or does he
have sporadic?" Bailey told UPI. "We haven't successfully answered
that question."
-
- He added that even if Hicks had the sporadic form of
CJD, it doesn't necessarily mean he didn't get it from eating contaminated
beef. Some studies have suggested sporadic CJD could be caused by mad cow
disease.
-
- Laura Manuelidis, a CJD expert and section chief of surgery
in the neuropathology department at Yale University, agreed the NPDPSC
diagnosis was not conclusive.
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- "They'll never know unless they inject it into animals,"
Manuelidis told UPI.
-
- Tests done by the NPDPSC are interesting and suggestive,
Manuelidis said, "but without looking at how it behaves in an animal,
they don't know."
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- Carrie Harris of the NPDPSC declined to comment on the
case due to patient confidentiality restrictions. "I'm afraid I'm
not able to comment on any cases in particular," Harris told UPI.
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- Pierluigi Gambetti, director of NPDPSC, did not return
a phone call from UPI.
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- Bailey said the decision to re-examine Patrick's brain
tissue was spurred by problems with the initial autopsy and questions about
the final diagnosis from NPDPSC.
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- "We had a family discussion about the issues and
what caused this development was the debacle of the autopsy in this patient,"
Bailey said.
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- The NPDPSC arranged for a company called 1-800-AUTOPSY
to collect Patrick's brain upon his death. However, the company failed
to follow the proper protocol required by NPDPSC and did not freeze any
samples from the brain. Instead, the autopsy company fixed the entire organ
in formalin, making it difficult to conduct definitive tests that would
help distinguish whether Hicks had vCJD or sporadic CJD.
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- The company previously told UPI they did not have the
equipment necessary to freeze the tissue and would have made NPDPSC aware
of this when they were first contacted.
-
- Bailey also said he was uncomfortable with what he termed
"inconsistencies" in the final lab report from NPDPSC.
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- "I think there was enough ambiguity there that I
certainly didn't mind a second opinion," he said.
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- The pathology report from the NPDPSC, obtained by UPI,
stated the brain tissue showed evidence consistent with sporadic CJD and
that vCJD was "unlikely."
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- The report, which is signed by Gambetti, noted, "Unfortunately,
due to the lack of frozen tissue, the prion protein gene and protein analyses
could not be carried out. These analyses would have been useful to support
the present diagnosis."
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- Hicks' family also was troubled by the failure to collect
frozen tissue.
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- "Why would they put it in formalin when it's known
you shouldn't do that?" Debbi Hicks said. "There's no reason
that should've been done ... because that makes it look like a cover-up."
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- Markus Moser, a molecular biologist and chief executive
officer of Prionics, a Switzerland firm that manufactures test kits for
detecting mad cow disease, told UPI it still would be possible to carry
out animal injection studies and other tests without frozen tissue, but
he found it strange frozen tissue was not collected.
-
- "It is particularly odd that no frozen tissue was
stored and the explanation of missing freezing equipment is unsatisfactory
at best," Moser said.
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- He noted freezing would not even have been necessary
for a type of test called Western blot. The tissue still would have been
suitable for conducting that test even if it had been stored for days at
room temperature.
-
- He also said animal injection studies still could be
undertaken, even with the formalin-fixed tissue because that process does
not destroy the infectivity of the pathogen.
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- "The case could actually be further investigated,
if there is a willingness to do so," he said.
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- Copyright 2005 by United Press International. All Rights
Reserved.
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