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From: Terry S. Singeltary Sr. (216-119-132-25.ipset12.wt.net)
Subject: Calif. man had human mad cow symptoms -- "Clinically, the case did look like it was variant CJD -- no question about that,"
Date: January 6, 2005 at 9:45 am PST

-------- Original Message --------
Subject: Calif. man had human mad cow symptoms
Date: Thu, 6 Jan 2005 08:56:04 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@LISTSERV.KALIV.UNI-KARLSRUHE.DE


##################### Bovine Spongiform Encephalopathy #####################

Calif. man had human mad cow symptoms


By Steve Mitchell
Medical Correspondent

Washington, DC, 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.

--

E-mail sciencemail@upi.com

http://www.washtimes.com/upi-breaking/20050105-062458-5715r.htm

TSS

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