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Calif. man had human mad cow symptoms

By STEVE MITCHELL, Medical Correspondent   |   Jan. 6, 2005 at 9:15 AM   |   Comments

WASHINGTON, Jan. 5 (UPI) -- Public health officials have ruled out the human version of mad cow disease as the cause of death for a California man, but the man's neurologist told United Press International the man had several symptoms of the fatal disease and questions remain about the case.

Patrick Hicks, 49, died late last year at Reche Canyon Health Care Center in Colton, Calif., as first reported by UPI in November.

Upon Hicks' death, Dr. Ron Bailey, a neurologist at Riverside Medical Center in Riverside, Calif., who treated him, arranged for a sample of his brain to be sent to the National Prion Disease Pathology Surveillance Center in Cleveland. NPDPSC is funded by the Centers for Disease Control and Prevention to analyze brain specimens for possible variant Creutztfeldt Jakob disease, a fatal condition humans can contract from eating beef products infected with the mad cow pathogen.

"Clinically, the case did look like it was variant CJD -- no question about that," Bailey told UPI.

Hicks initially had psychiatric symptoms, his illness appears to have lasted for more than one year and he had normal EEGs or brain-wave patterns until the late stages -- all consistent with vCJD, Bailey said. In addition, Hicks' 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.

Bailey said, however, the NPDPSC concluded in late December that Hicks did not have vCJD, but rather a similar disease called sporadic CJD, which has no known cause and is not thought to be due to contaminated beef.

Allison Marsh of the NPDPSC told UPI the center could not comment on Hicks' final diagnosis.

Hicks' wife, Ronele, and his brother, Thomas Hicks, recently met with Bailey to discuss the results. Thomas told UPI he was left with the impression that the information NPDPSC provided about the case "wasn't acceptable, as far as Bailey was concerned."

"He (Bailey) feels they're hiding something," Thomas added.

In an interview with UPI, Bailey said he harbored no conspiracy theories about the case and he would defer to NPDPSC's diagnosis, but he said "some questions remain as far as I'm concerned."

If Hicks had vCJD, it would have been the first domestically borne case of the disease, because his family said he had never traveled to England or Europe, which have experienced epidemics of mad cow disease and where nearly all cases of vCJD have occurred.

Concerns about mad cow in North America recently were heightened due to a second case reported in Canada the day after the U.S. Department of Agriculture announced its intention to reopen the border to Canadian beef. The border was closed in May 2003, when Canada reported its first domestic case of the deadly disease.

Seeking further information about the Hicks case, Bailey requested the NPDPSC do a genetic analysis of Patrick's brain that could aid in determining whether he had contracted was vCJD or CJD.

Bailey said an NPDPSC employee, Carrie Harris, told him the lab did not hold any frozen brain tissue from Hicks, which is required to do the analysis. Bailey said he found Harris's explanation troubling, because the protocol on the NPDPSC Web site stipulates the collection of frozen tissue.

Harris did not return phone calls from UPI.

Bailey questioned how NPDPSC arrived at its conclusion that Hicks did not suffer from vCJD, because he thinks it is impossible to distinguish CJD from vCJD by examining the brain using the type of tests they did. This is a view shared by other experts in the field and it is supported by evidence from scientific studies. For example, recent research by John Collinge and colleagues at University College in London demonstrated mice injected with mad cow disease can develop brain damage that looks like both vCJD and CJD.

CDC spokesman Llelwyn Grant told UPI the genetic analysis Bailey wanted conducted would have provided some added information, but it would not have ruled out either vCJD or sporadic CJD.

"If a lab gets a good specimen, that lab should be able to with (the type of testing NPDPSC did and) tell whether or not they're dealing with sporadic or variant CJD," Grant said.

Laura Manuelidis, an expert on these diseases and section chief of surgery in the neuropathology department at Yale University, told UPI the frozen tissue would have been invaluable in clearing up any discrepancy over whether Hicks had vCJD or CJD.

Some evidence suggests "that vCJD can look like sporadic CJD, so how do you know which it is?" Manuelidis asked. "The answer is that you don't because they have no other tissue with which to explore this further and this is where the frozen tissue gets to be an issue."

Frozen tissue is required to conduct a type of test called Western blot that can aid in distinguishing vCJD from CJD, Manuelidis explained. Frozen specimens also are a requisite for animal-injection experiments, which are time consuming but come close to being definitive about whether a disease is vCJD or CJD, she added.

Marsh confirmed that for unknown reasons whoever removed Hicks' brain failed to follow protocol and fixed the entire organ in formalin, making it impossible to do the genetic analysis or other tests. This was done by somebody in California, however, not an employee of NPDPSC, Marsh said.

Bailey said the NPDPSC hired the autopsy company, 1-800-Autopsy of Los Angeles, to perform the procedure and "from what I gathered, the NPDPSC had worked with these people in the past and they were versed in the protocol they had."

A spokeswoman for 1-800-Autopsy, who did not identify herself, confirmed that the company worked on the case. Asked why they did not collect frozen brain tissue, as the NPDPSC protocol stipulates, she said, "We don't have the capability to freeze it," because the required refrigeration equipment is too expensive.

The spokeswoman added that the company would have informed NPDPSC of this, "up front."

Asked why NPDPSC would hire a company that does not have the capability to follow their protocol, Marsh referred UPI to Harris, who was out of the office Wednesday and did not respond to an e-mail and another voicemail message.

Another issue Bailey found troubling is what he described as the refusal of the Riverside County coroner's office to conduct an autopsy on Hicks, which is required to get the brain tissue to send to NPDPSC.

"It was beyond belief. It was like pulling teeth," to get the autopsy performed, Bailey said. "If you have a condition that is this rare with the potential to be the first variant CJD case in the state, it raises questions in my mind why they would be so reluctant to do an autopsy on him," he said.

Bailey said the county coroner's office at first did not return his call and even after NPDPSC's Harris called them, they still refused to conduct an autopsy on Hicks. Ultimately, the brain tissue was removed by 1-800-Autopsy at the crematorium prior to cremating Hicks' body.

Earl Quinata, spokesman for the Riverside County coroner's office, told UPI the case would have been the responsibility of the coroner in San Bernadino County, because that is where Hicks died.

Bailey said he never got the impression that was the reason the coroner's office refused to deal with Hicks' body and he sent a letter to NPDPSC apologizing for the lack of cooperation from the coroner's office.

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E-mail sciencemail@upi.com

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