- WASHINGTON (United Press
International via COMTEX) -- A 62-year old man in Northern New Jersey has
died from a brain disorder that appears to be Creutzfeldt-Jakob disease,
which, if confirmed, would be the fifth case of the rare disease in a little
over a year in a two-county area, United Press International has learned.
-
- The death last Friday of Ronald Swartz, of Denville,
also would be the second CJD case in 2004 in New Jersey, where federal
and state health authorities are investigating a potential cluster of cases
of the rare brain disorder in the southern region of the state.
-
- UPI also has learned that officials have reopened the
case of a Philadelphia woman who died in 2000 and that case also is included
among those in the possible cluster. Carrie Mahan, 29, died from a brain
disorder that was never identified, but which physicians initially suspected
of being the nation's first case of variant CJD, the form of the disease
linked to mad cow disease.
-
- The possible CJD cluster is associated with the Garden
State Race Track in Cherry Hill, where as many as 13 CJD deaths might have
occurred among employees and patrons who ate at the now defunct track.
Both variant CJD and the spontaneously occurring form of the disease --
called sporadic CJD or simply CJD -- are incurable conditions that degenerate
the brain and ultimately cause death.
-
- Swartz's case does not appear linked to the racetrack,
but if his death turns out to be due to CJD, it would make five confirmed
or probable cases of the disease in the adjoining area of Somerset and
Morris counties within a span of only 15 months.
-
- Somerset already had recorded a probable CJD death this
year, as well as two confirmed cases last year, according to data from
the New Jersey Department of Health and Senior Services. Morris county,
where Swartz resided, recorded a probable case in 2003.
-
- This would be an unusually high number for a uncommon
disorder that is thought to occur at the rate of only one case per 1 million
population per year.
-
- The combined population of the two counties is approximately
789,000, so they would expect to see no more than one case of sporadic
CJD in that time frame. According to DHSS figures, which go back to 1979,
the two counties have never experienced two CJD cases in the same year
-- let alone five.
-
- Asked about the seemingly high rate, DHSS spokeswoman
Jennifer Sciortino said, "New Jersey's incident rate (for the entire
state) is approximately 8 per year and thus there is no indication that
we are exceeding the average case count per year." Sciortino added,
"In fact, over the last 25 years there have been instances where the
total number of cases topped out at around 14 per year."
-
- DHSS officials, who are looking into the racetrack cluster,
might be investigating Swartz's case.
-
- Carolyn Swartz, Ronald's wife, said through his brother
Wayne that infectious disease specialists from the New Jersey Health Department
had contacted her about the case while Ronald was in St. Clare's Hospital
in Sussex, the facility where he initially received treatment prior to
being transferred to New York-Presbyterian Hospital in New York City.
-
- Wayne Swartz said he was uncertain what information the
New Jersey officials were seeking, and the DHSS declined to comment on
the case, citing federal regulations that prohibit such disclosure.
-
- "We do not comment on individual CJD cases because
federal HIPAA (Health Insurance Portability and Accountability Act) laws
prevent us from disclosing any information that might help in ascertaining
a patient's identity," Sciortino told UPI.
-
- The Centers for Disease Control and Prevention in Atlanta,
which is assisting the DHSS in the investigation into the potential cluster,
also declined to comment on the Swartz case. CDC spokesman Tom Skinner
said last week he would look into it but did not return UPI's phone call
and an e-mail.
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- In an interview prior to Ronald Swartz's death, Wayne
told UPI, neurologists at Presbyterian had informed the family they were
nearly certain Ronald was suffering from CJD.
-
- "They are 100 percent certain that's what it is,"
Wayne said. The only way to conclusively diagnose the disease, however,
is via an autopsy, which Wayne said the family plans to have done on Ronald,
who would have turned 63 on Wednesday.
-
- Concerns about vCJD have been heightened since the discovery
of a case of mad cow disease in Washington last December. There have been
no confirmed cases of vCJD in the United States, except for a 22-year old
woman in Florida in 2002 who was a United Kingdom citizen and was thought
to have contracted the disease while in England.
-
- Ronald Swartz's age makes him an unlikely candidate for
vCJD because the disease typically strikes those under age 55. But vCJD
is a possibility because it previously has been detected in elderly people
in the United Kingdom -- including a 74-year-old man and a recently discovered
case of a 62-year-old man, who appears to have contracted the disease via
an infected blood transfusion.
-
- Janet Skarbek, a private citizen in Cinnaminson, N.J.,
who identified the CJD cases tied to the Garden State Race Track, thinks
they are due to the consumption of beef contaminated with mad cow disease
that might have been served at the track.
-
- Both the DHSS and the CDC doubt Skarbek's hypothesis
and downplay the possibility of a cluster related to either the racetrack
or mad-cow-tainted beef.
-
- Carrie Mahan, the subject of the newly reopened case,
worked at the racetrack and is included in the list of potential cluster
victims compiled by Skarbek.
-
- Mahan's physicians at the University of Pennsylvania
Medical Center in Philadelphia initially suspected she was suffering from
variant CJD due to her young age. Subsequent tests at the National Prion
Disease Pathology Surveillance Center at Case Western Reserve University
in Cleveland -- an institute set up by the CDC to autopsy possible vCJD
cases -- ruled out both vCJD and CJD.
-
- The case has baffled neurologists, such as Dr. Pierluigi
Gambetti, director of the Surveillance Center, because Mahan's condition
was never identified conclusively. However, many experts, including Dr.
Nicholas Gonatas -- the pathologist who performed the autopsy on Mahan
-- thought it was CJD.
-
- Now Gambetti plans to determine if newer, more sensitive
tests developed since 2000 can detect the presence of prions, the agents
thought to be responsible for both CJD and vCJD, in Mahan's brain tissue.
-
- Allen Mahan, Carrie's brother, said Gambetti requested
permission from him last week to retest Carrie's brain tissue.
-
- "Gambetti said they've developed new testing methods
and they want to try them out on her case," Allen told UPI.
-
- Gambetti declined to comment on the case due to patient
confidentiality restrictions, but he said CJD tests are now more sensitive
and offer "better detection ability" than in 2000.
-
- Another factor driving the decision to re-examine Mahan's
diagnosis could be the opinions of neurologists who observed the slides
of her brain when Gambetti recently presented them anonymously at a neurology
meeting. Allen said Gambetti told him most neurologists there had agreed
the condition looked like CJD.
-
- He added Gambetti said he expects to have the updated
results in about three months.
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