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Questions linger in U.S. CJD cases

By STEVE MITCHELL, Senior Medical Correspondent   |   Oct. 21, 2005 at 9:49 PM   |   Comments

WASHINGTON, Oct. 21 (UPI) -- French researchers have ruled out the human form of mad cow disease in a deceased California man, even though they did not conduct the critical test widely regarded as the only way to determine precisely the nature of his disease, United Press International has learned.

The case of Patrick Hicks, who died last November from his condition, has remained murky from the beginning. Dr. Ron Bailey, of Riverside, Calif., the man's neurologist, had suspected the 49-year-old Hicks of having contracted variant Creutzfeldt Jakob disease -- a fatal, brain-wasting illness humans can contract from eating beef products contaminated with the mad cow pathogen -- and both he and the family wanted an autopsy conducted to determine if Hicks had succumbed to the disorder.

Bailey became concerned that Hicks might have contracted vCJD because he initially had exhibited psychiatric symptoms, his illness appears to have lasted for more than one year and he showed normal brain-wave patterns via EEGs until the late stages -- all consistent with the disease. In addition, Hicks's relatively young age raised concerns, because nearly all of the more than 150 cases of vCJD detected worldwide have occurred in people under age 55.

The first hint of oddness began when, according to both Hicks's brother and mother, a team of six doctors, who they suspect were with the Centers for Disease Control and Prevention in Atlanta, visited Patrick last October while he was still alive and under care at Loma Linda University Medical Center in Loma Linda, Calif.

They said they were asked to leave when the doctors arrived to examine Patrick.

CDC officials would not confirm to UPI whether they had investigated the case, but the agency's policy does require examining all suspected cases of vCJD in anyone under 55.

The family also said Loma Linda refused to released Hicks's medical records to them.

The oddities continued after Hicks's death. Bailey found it almost impossible to get an autopsy conducted on Hicks, the only way to determine conclusively whether he had variant or sporadic CJD -- a version of the disease not related to mad cow. One county coroner's office referred him to another and both refused to conduct the procedure, he said.

Then, the National Prion Disease Pathology Surveillance Center in Cleveland, Ohio -- which was established by the CDC to investigate potential vCJD cases in the United States -- dispatched a mobile autopsy company called 1-800-Autopsy, but the company failed to follow the center's protocol and did not collect frozen sections of brain, which are required for tests to determine whether the disease is vCJD or sCJD. Instead, the autopsy company fixed the entire brain in formalin.

The NPDPSC, however, considers the collection of frozen brain tissue essential to distinguishing vCJD from other forms of CJD.

"Only frozen brain tissue examination definitely confirms or excludes the diagnosis of prion disease and provides the information to identify the type of prion disease," the center's Web site says. Prions are abnormal proteins thought to play a role in causing vCJD and sCJD.

The problem raised enough concern that both Bailey and Hicks's family sought a second opinion.

Experts had told them that animal-injection studies could be done with formalin-fixed tissue, so the family arranged to have a sample of Patrick's brain sent to Dr. Jean Jacques Hauw at the Laboratoire De Neuropathologie at the Groupe Hospitalier Pitie-Salpetriere in Paris, who they thought had agreed to do the studies.

The NPDPSC, however, delayed sending the sample to France for two months after the family's request last March. During the delay, Pierluigi Gambetti, the NPDPSC's director, sent a letter to Hicks's wife.

"We can definitely rule out the diagnosis of variant CJD," the letter stated.

Gambetti's strong conclusion sounded strange to Bailey, because the NPDPSC had not conducted further tests since January, when they had said vCJD was unlikely but that they were unable to rule it out entirely.

After examining the brain tissue, Hauw's team told the family the disease was consistent with sCJD, but to date they have not explained why they did not conduct the animal-injection studies -- the family's reason for sending samples of his brain to France.

Asked the reasons for not following the family's wishes and conducting the animal studies, Hauw told UPI, "I cannot answer your question," citing French regulations that prohibited him from providing information about a specific patient.

He did say, however, that "animal injection is not needed for the routine diagnosis of Creutzfeldt-Jakob disease and its various variants, at least in France and in the United Kingdom."

That may be true, but it remains unclear why he accepted the case in the first place, knowing that is what the family wanted.

Moreover, this was not a "routine diagnosis." If Hicks suffered from vCJD, he potentially would have been the first person in the United States to have acquired the disease domestically, a development with significant domestic and international ramifications.

In addition, other experts, such as Dr. Laura Manuelidis, section chief of surgery in the neuropathology department at Yale University, have said the only way to know conclusively whether the disease is due to sCJD or vCJD is through animal-injection studies.

"From what I gather, the result was merely rubber stamped," Bailey told UPI. "I guess we will never really know for sure."

The handling of the case is noteworthy, because the NPDPSC currently is investigating nine potential sCJD cases in Idaho. Experts suspect some of those cases could be vCJD.

Bailey and some patient advocates said they are now skeptical of the NPDPSC's behavior.

"How could my experience with the Hicks case ... and the interaction with NPDPSC not lessen my confidence?" Bailey asked. "I anticipate that all of the Idaho cluster of CJD patients will turn out to have sCJD. I cannot for a minute see their results indicating anything but this. After all, if any patient were to have vCJD, it would have been Patrick Hicks. The results of NPDPSC are not definitive in excluding Hicks as not having vCJD. There certainly will always be that question in my mind."

Terry Singletary, a patient advocate whose mother died of a form of the disease called Heidenhain variant, told UPI he likewise had lost confidence in the NPDPSC.

"I do not trust them," Singletary said. "It's all going to be sporadic. This is the way they want it. They do not want to find out all the routes and sources of this agent."

Both vCJD and mad cow disease are politically sensitive issues because they can impact international trade. Dozens of nations closed their borders to American beef after a lone U.S. cow tested positive for the disease in 2003, resulting in more than $4.7 billion in losses for the industry, and the U.S. Department of Agriculture delayed doing confirmatory tests for seven months on what turned out to be a second case of mad cow.

The NPDPSC did not respond to UPI's phone call requesting comment about the Idaho cases. The CDC referred UPI to Idaho officials.

Of the nine Idaho cases, three people have tested positive for a CJD-like illness, but officials are conducting further tests to determine whether the disease is sCJD. Two others tested negative and four were buried without autopsies.

The cases could just be a statistical fluke, but the state averages about 1.2 sCJD cases per year and has never had more than three in a single year. The disease is rare and generally is thought to occur at the rate of one case per million people.

Several CJD clusters in other states have far exceeded that rate, however. These included:

--southern New Jersey (2000-2003),

--Lehigh, Pa. (1986-90),

--Allentown, Pa. (1989-92),

--Tampa, Fla. (1996-97),

--Oregon (2001-02), and

--Nassau County, N.Y. (1999-2000).

Some of the clusters involved as many as 18 deaths, and ranged from a rate of four to eight cases per million people.

A group of J.P. Morgan analysts issued an advisory last year on the impact the clusters could have on the beef industry, and said that some of the cases could be due to vCJD.

"The existence of clusters raises the question of 'contamination' or 'infection,' and also raises the hypothesis that rather than cases of sCJD, these might have been cases of vCJD," the advisory said. "Given that sCJD occurs randomly in one out of 1 million cases, it is a statistical rarity to find an sCJD cluster -- let alone six."

If that assessment is accurate, another cluster in Idaho would be even more unlikely.

Another possibility is some of the Idaho cases could be due to chronic wasting disease, which is similar to mad cow disease and currently is epidemic among deer and elk in several states, including Idaho's neighbors Wyoming and Utah.

No human cases of CWD have ever been confirmed, but the disease has been shown to infect human cells in a lab dish. Also, a team of researchers led by Jason Bartz of Creighton University in Omaha, Neb., report in the November issue of the Journal of Virology they had experimentally transmitted CWD to squirrel monkeys --the first reported transmission of CWD to primates.

If CWD is capable of infecting humans, it is unknown whether the resulting disease would resemble sCJD, vCJD or a novel disorder. If the disease looks like sCJD, cases could be going undetected or misdiagnosed.

--

E-mail: healthbiz@upi.com

© 2005 United Press International, Inc. All Rights Reserved. Any reproduction, republication, redistribution and/or modification of any UPI content is expressly prohibited without UPI's prior written consent.
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