US Man To Be Retested
For Human Mad Cow
By Steve Mitchell
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.
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.
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.
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.
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.
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.
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.
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.
"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.
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.
"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.
"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."
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.
Pierluigi Gambetti, director of NPDPSC, did not return a phone call from UPI.
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.
"We had a family discussion about the issues and what caused this development was the debacle of the autopsy in this patient," Bailey said.
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.
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.
"I think there was enough ambiguity there that I certainly didn't mind a second opinion," he said.
The pathology report from the NPDPSC, obtained by UPI, stated the brain tissue showed evidence consistent with sporadic CJD and that vCJD was "unlikely."
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."
Hicks' family also was troubled by the failure to collect frozen tissue.
"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."
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.
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.
"The case could actually be further investigated, if there is a willingness to do so," he said.
Copyright 2005 by United Press International. All Rights Reserved.



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