January 27, 2004 Courier-Post (Cherry Hill, NJ)  by RICHARD PEARSALL

A cluster is an unusually high number of cases in a given population that cannot be explained as coincidence.

Skarbek says her list of suspected Creutzfeldt-Jakob disease victims with links to the now-closed racetrack has grown to eight and is likely to keep growing as people continue coming to her with information.

"I'm doing the CDC's work for them until they decide to do the cases themselves,' Skarbek said, referring to the federal Centers for Disease Control in Atlanta.

The eighth case on her list is said by a relative to have been a season pass holder at the now-closed racetrack.

While a number of people on her list had casual contact with the track -- a single visit in some cases -- two on the list were white-collar employees and another two (if the eighth case pans out) were regular attendees.

Skarbek has heard people discount her theory by pointing out that hundreds of thousands of people visited the track.

But "what if there are two administrative employees out of 100 at the track, and two season pass holders out of 1,000?' she asks.

Creutzfeldt-Jakob disease is a rare brain disorder that, in its classic form, generally strikes the elderly, is inevitably fatal and has no known cause.

A spokesman for the CDC said Friday that any new cases would have to be investigated first by the state health department.

"The protocol is for the New Jersey health department to take the lead,' said the spokesman, Tom Skinner. "If they feel they need assistance, we will be happy to help them out.'

A spokeswoman for the state Department of Health and Human Services, Jennifer Sciortino, said Friday that "at the moment, we're not looking into any cases.'

"But she (Skarbek) is supposed to be reaching out to us,' Sciortino said.

Creutzfeldt-Jakob disease was discovered in the 1920s by two German scientists for whom it was named.

In the 1990s, a variant form of the disease emerged in England for which a cause was identified.

The "new variant,' as it is called, is contracted by eating beef or beef products from cattle infected with mad cow disease, which is the bovine version of the affliction.

There has never been a case of the new variant form identified among native-born Americans, and Skarbek, for one, does not think the first case is here.

What she does suspect is that eating beef could be behind the classic or sporadic form of the disease, the one for which no cause has been identified.

"With all that's going on, people cannot jump to the conclusion that sporadic is not related to eating beef,' she said last week.

Most scientists discount Skarbek's theory as highly unlikely.

"That it occurs all over, in every state,' Dr. Lawrence Schoneberger says of the classic or sporadic form of CJD, "is an indication of its randomness.'

Schoneberger is an epidemiologist for the CDC in Atlanta.

CJD occurs in roughly one in every 1 million Americans, the statistics show.

"One has to be very careful about calling seven cases a cluster,' Dr. Peter Crino, a neurologist at the University of Pennsylvania Medical School, said earlier this month when Skarbek's list was at that number.

If there were a proximate cause such as beef, Crino added, isn't it likely that a problem with cattle would have surfaced by now?

But a number of scientists, including Crino, believe there is a need for more investigation.

Crino treated one of the key cases in the alleged cluster, a 29-year-old Garden State employee, Carrie Mahan, who died in 2000.

Testing by the CDC's designated laboratory in Ohio concluded the woman did not die of CJD, either the variant or classic form.

Crino agrees it wasn't the variant form, but if it wasn't sporadic CJD, he said, he doesn't know what it was.

"It's extremely likely she contracted CJD,' Crino said, having discounted the other possibilities as extremely unlikely.

Both the CDC and the state health department have refused to consider the Mahan case in examining Skarbek's cluster allegation.

They discarded a number of other cases because no autopsies were done and no tissue was available for testing at the Ohio lab.

Not all the cases can be easily dismissed, however, and Skarbek is now focusing on four people whose contact with the track was substantial: two people who worked there and two who had season passes.

The employees were Mahan and Carol Olive, a 56-year-old Cinnaminson woman who worked as a press officer at the track. She died in May 2003 in the one case to date that the CDC's testing confirms as sporadic CJD.

The season pass holders were John Paul Weber, a retired radio newscaster from Pennsauken, who died in 2000 of what his doctor at Cooper University Hospital in Camden diagnosed as CJD; and a case the state health department expects to examine soon, John LaPaglia, 71, of Westville. He died Sept. 3, 2003, at Underwood-Memorial Hospital in Woodbury of what was diagnosed as CJD, according to his son, John Jr.

Reach Richard Pearsall at (856) 486-2465 or rpearsall@courierpostonline.com