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From: TSS (
Subject: New CJD case identified, but autopsy casts doubt on another KINGSTON
Date: October 16, 2004 at 8:23 am PST

-------- Original Message --------
Subject: New CJD case identified, but autopsy casts doubt on another
Date: Sat, 16 Oct 2004 10:04:03 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

New CJD case identified, but autopsy casts doubt on another

By Jesse J. Smith , Freeman staff 10/16/2004

KINGSTON - An autopsy on one of five local residents believed to have
died from the rare brain disorder Creutzfeldt-Jakob's disease found
that, in fact, CJD was not present in the woman's brain, her father said
on Friday.

Colleen Staccio died Aug. 28 at Benedictine Hospital in Kingston after
being diagnosed with CJD at Albany Medical Center. But an autopsy report
from the National Prion Disease Research Center at Case Western
University in Cleveland said the disease was not present in the brain of
the 46-year-old Lake Katrine woman. Staccio's father, Don Genther, said
he received the report on Friday and that it did not cite a cause of
death, saying only that CJD was not found.

"We really don't know what it is now," Genther said.

The report noted, however, that the autopsy did not analyze Staccio's
tonsils or other areas of the body where CJD, an invariably fatal
disease, can exist.

The autopsy report arrived at Genther's home a day after Kingston
Hospital officials confirmed that a patient who died there last November
had CJD. That case brings to five the number of suspected CJD deaths
among local residents, including Staccio.

Hospital officials did not identify the person who died last November.
Besides Staccio, the only local CJD victim to be identified so far is
Kingston resident Richard Tobey, who died last week at Benedictine. The
other two CJD sufferers died in September.

CJD typically strikes only one person per million each year worldwide,
so the local cluster of deaths is considered unusual. The state Health
Department said this week it was investigating the local deaths, and
whether they could be linked in any way, but said there was no cause for
public concern.

On Tuesday, Kingston-based physician Dr. Joel Mandelbaum said he was
directly involved in treating three of the local CJD cases. All three,
Mandelbaum said, were diagnosed through analysis of spinal fluid or
brain tissue.

Tobey's CJD was confirmed through a brain biopsy shortly before his
death last week, according to Tobey's family.

According to the U.S. Centers for Disease Control and Prevention, about
85 percent of CJD cases are known as "sporadic CJD," meaning they are
caused by unknown factors.

The disease also can be inherited, and in a few cases, CJD has been
spread through exposure to contaminated neurosurgical instruments.
Family members have said both Staccio and Tobey had back surgery in the
mid-1990s at Kingston Hospital, but there has been no evidence linking
those surgeries to the eventual deaths of the two patients.

A variant form of CJD, commonly called Mad Cow disease, is contracted by
eating contaminated beef. Most cases of the Mad Cow variant have
occurred in the United Kingdom, and the only human case of Mad Cow
diagnosed in the United States occurred in a woman who was born and
raised in England.

CJD - believed to be caused by a malformation in certain proteins called
prions, which infect and destroy parts of the brain - remains poorly
understood, and experts say it often is misdiagnosed.

"It is the most enigmatic disease on the face of the Earth," said
Florence Kranitz, president of the Creutzfeldt-Jakob Disease Foundation.

She also said the true incidence of CJD is almost certainly higher than

"Doctors don't recognize it," she said. "They studied it for 10 minutes
in med school, thinking they would never see a case, so they don't."

Kranitz also said a spinal fluid analysis frequently used to diagnose
CJD is unreliable and frequently results in false negative or false
positive results. The only way to definitively diagnose the syndrome is
through a brain biopsy or autopsy, Kranitz said.

On Friday, officials at Kingston Hospital said that they were
cooperating with the Health Department investigation into the CJD
deaths, while Benedictine officials said they had not yet been contacted
by the department.

©Daily Freeman 2004tss


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