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Five CJD deaths in north N.J. in 15 months

By STEVE MITCHELL, United Press International   |   March 24, 2004 at 1:14 PM   |   Comments

WASHINGTON, March 24 (UPI) -- 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.

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.

--

Steve Mitchell is UPI's Medical Correspondent. E-mail sciencemail@upi.com

Topics: Tom Skinner
© 2004 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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