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vCJD in the US?

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Feb 10, 2005
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Southern Manitoba
Testing of human mad cow suspect delayed

WASHINGTON, Jun 02, 2005 (United Press International via COMTEX) -- U.S. officials have delayed sending brain samples from a deceased California man to France to be screened for human mad cow disease, leading the man's family and his neurologist to question the reasons for the holdup.

The National Prion Disease Pathology Surveillance Center in Cleveland diagnosed Patrick Hicks, 49, of Riverside, Calif., with a brain-wasting illness called sporadic Creutzfeldt Jakob disease after his death last November.

Hicks's family, and Dr. Ron Bailey, a neurologist at Riverside Medical Center, wanted a second opinion, however, because the initial autopsy had been botched and they thought his symptoms were consistent with a related disease called variant CJD, which humans can contract from eating beef products contaminated with the mad cow pathogen.

In a March 31 letter, Ronele Hicks, Patrick's wife, requested that NPDPSC send samples of Patrick's brain to Dr. Jean-Jacques Hauw at Laboratoire De Neuropathologie at the Groupe Hospitalier Pitie-Salpetriere in Paris. Hauw had agreed to conduct further tests that could help provide a more conclusive diagnosis.

"It sounds like they didn't send the tissue and I don't know why," a distraught Ronele told United Press International.

"I don't known what is going on here," Bailey told UPI. "The NPDPSC should be embracing the opportunity to have a second opinion on the tissue."

If Hicks's diagnosis turned out to be vCJD, his would be the first case linked to consumption of U.S. beef, because he never traveled outside of the country, except for a week spent in Canada, and did not have any risk factors for contracting the disease, other than being a meat-eater.

Both vCJD and mad cow disease are politically sensitive issues, because they can have international trade ramifications. After a lone U.S. cow tested positive for the disease in 2003, dozens of nations closed their borders to U.S. beef, resulting in up to $4.7 billion in losses for the industry.

The NPDPSC initially appeared to comply with Ronele's request to send brain tissue to Hauw. NPDPSC employee Carrie Harris stated in an April 18 e-mail to Ronele, "I have already submitted your request for processing and no follow up should be necessary from you at this time."

The tissue samples never arrived in France, however, so Ronele sent another e-mail last week to Harris asking about the status of her request. There was no reply, but two days later she received a letter from Pierluigi Gambetti, the NPDPSC's director, sent by FEDEX stating, "We can definitely rule out the diagnosis of variant CJD."

Gambetti added, "We will be glad to send tissue to Dr. JJ. Hauw, whom I know well, if you wish."

This communication arrived nearly two months after Ronele had sent her letter, via certified mail, addressed to Gambetti requesting her husband's brain tissue be sent to Hauw.

Bailey also received a similar letter from Gambetti last week.

Monday, Ronele received a response from Harris to her initial e-mail, stating: "The samples are currently in transit. I apologize for the delay, but as you might expect, it takes a lot of legwork to send tissue samples overseas."

The seemingly conflicting messages have left Ronele confused.

"It (the e-mail) appears to contradict the FEDEX letter, but what am I to do?" she said.

Ronele said she worries she also could develop the disease.

"If it's from beef, am I next?" she asked, and noted she and Patrick had consumed essentially the same meals over their 22-year marriage. She also said she is concerned her sons could develop the disease as well, because the NPDPSC had told her the sporadic CJD they diagnosed in Patrick could be genetically linked.

"I have two kids and I'm wondering are they going to come down with this?" Ronele asked, and at times cried at the memory of her husband.

Neither Harris nor Gambetti responded to UPI's request for comment.

The ordeal over sending Patrick's sample to France has rendered both Bailey and Ronele skeptical about whether they will obtain a conclusive diagnosis of his illness, and Ronele said she now wonders if NPDPSC will send the correct tissue sample to France.

"That's been a concern all along," she said. "How do we know it's not some other person's tissue instead of Pat's?"

Bailey questioned how the NPDPSC could have ruled out vCJD, because, he said, they did not take such a strong position in January and have not indicated they have conducted any further tests that would enable them to make a definitive diagnosis.

"Am I missing something here?" Bailey asked.

Other researchers agreed further tests would be needed to rule out vCJD. Dr. Laura Manuelidis a CJD expert and section chief of surgery in the neuropathology department at Yale University, told UPI previously that a type of test called Western blot, as well as injecting the sample into animals (which the French researchers plan to do), would help establish whether Patrick died of vCJD or the sporadic form of the disease.

Gambetti noted in his letter that the Western blot test and genetic sequencing could not be done because 1-800-AUTOPSY -- the company contracted by the NPDPSC to conduct the initial autopsy -- did not freeze any of the brain tissue, which is necessary to conduct such tests.

"It doesn't make sense to me they wouldn't do all the things they do all over the world when they have a vCJD suspect," such as freezing brain tissue, 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.

"If the only thing you have is (the tests conducted by NPDPSC), I would also say there is no clear way to absolutely rule out vCJD, once symptoms have been more usual for variant and unusual for sporadic," Moser said.

He agreed animal-injection studies would help clarify the diagnosis. "So if somebody is willing to do that, they should do it," he said.

Gambetti's letter stated that Patrick's brain shows evidence that "most resembles" sporadic CJD. Nevertheless, that does not rule out vCJD. Recent research in mice suggests the mad cow pathogen can cause both forms of the disease.

The research, led by John Collinge, director of the Medical Research Council Prion Unit in London, also suggested that the mad cow pathogen, known as a prion, could generate novel forms of itself with properties that mimic the type normally associated with sporadic CJD.

"Our findings do suggest we should be taking steps to draw up a more sophisticated system of categorizing the disease, so that we don't mistake (mad cow)-related infection for a version of sporadic CJD," Collinge said.

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