However, the authorities deny congressional pressure influenced their decision to investigate the suspected cases of the illness, one form of which has been linked to mad cow disease.
The investigation was spurred by the persistence of Janet Skarbek, a private citizen in Cinnaminson, N.J., who has been collecting information about local CJD cases for nearly a year. Skarbek has compiled a list of at least nine -- and as many as 13 -- CJD cases with ties to the Garden State Race Track in Cherry Hill.
Skarbek became concerned about the disease after Carol Olive, a woman with whom her mother had worked at the Race Track, died last May of CJD.
As Skarbek's research turned up more cases of the rare disease, she began urging the Centers for Disease Control and Prevention in Atlanta, and the New Jersey Department of Health and Senior Services, to investigate. But both agencies dismissed her pleas, claiming there was no evidence of abnormally high occurrences of CJD in the state.
Undeterred, Skarbek recently contacted several members of congress and senators, some of whom showed particular interest. Rep. Rob Andrews, R-N.J., and aides to Sen. Richard Durbin, D-Ill. -- who has co-sponsored a Senate bill that would increase surveillance for CJD -- wrote letters to the health agencies, urging them to investigate.
Now, after weeks of denying there is a cluster or rise in the rate of CJD in southern New Jersey, both the CDC and the state health department appear at least to be willing to closely examine the cases identified by Skarbek.
Dr. Eddy A. Bresnitz, the New Jersey state epidemiologist, sent an e-mail on Feb. 25 to a Senate aide, saying his department along with the CDC was investigating the cases.
The agency "is in the process of verifying whether they are N.J. residents and whether the diagnosis was confirmed in all individuals, which is often not the case," Bresnitz wrote in the e-mail, which was obtained by UPI. "We are working with the CDC (Dr. Larry Schonberger) on this assessment as the CJD unit he heads coordinates surveillance efforts nationwide," the e-mail continued.
CDC spokesman Tom Skinner offered few details about the investigation, other than to say it was being spearheaded by the New Jersey health department. "They're kind of taking the lead and we're supporting them," Skinner told UPI.
DHSS spokeswoman Jennifer Sciortino said the agency had obtained the list of cases from Skarbek only a few weeks ago.
"We don't have a definitive timeframe right now when the whole investigation is going to be wrapped up," Sciortino told UPI. "Once they reach a definitive conclusion, it will be made public," she added.
Rep. Andrews, who sent a letter on Feb. 11 to both the CDC and the DHSS, said he was persuaded by the facts Skarbek presented to him that the cases warranted further investigation.
"I also thought she had been dismissed by the CDC and the New Jersey health Department far too quickly," he added.
Andrews said he will continue to monitor the situation to ensure the CDC and the DHSS take a thoughtful look at the situation. He called their decision to move ahead with gathering more information about the cases a step in the right direction, but he said he will not be satisfied until they deliver a thorough answer explaining these cases.
In January, both the DHSS and the CDC emphatically refuted the possibility there was an increased number of cases of CJD in New Jersey.
"There is no cluster of cases of CJD in New Jersey nor is there an increased incidence of CJD in the state," Bresnitz told UPI at the time.
However, as reported by UPI, the CDC in late January privately requested information about the cases from Skarbek.
Asked about the apparent shift in position, Sciortino said, "Our position has not changed since you spoke to Dr. Bresnitz in January. At this point, we are verifying the diagnoses and where the individual resided at the time of death."
CJD clusters, which have occurred in several states in recent years, received renewed attention following the discovery of a single case of mad cow disease in Washington state in December. The concern was prompted by the fact that more than 150 people have contracted variant CJD -- the human form of the illness linked to mad cow disease -- in the United Kingdom and Europe, following an outbreak of mad cow in British herds that began in the 1980s.
None of the U.S. clusters has been tied to tainted meat and all appear to consist of sporadic CJD -- which is not thought to be connected to mad cow. However, two recent studies raise the possibility that at least some sporadic cases could be due to mad cow, otherwise known as bovine spongiform encephalopathy or BSE.
Research in mice by John Collinge of the Imperial College of London indicated the mad cow pathogen can cause both variant CJD and sporadic CJD. Also, Italian scientists reported last month they had identified a new strain of mad cow disease that shows many similarities to sporadic CJD.
One racetrack employee, Carrie Mahan, 29, died from a mysterious condition in 2000 that initially appeared to be vCJD. Subsequent tests, however, ruled out both vCJD and sporadic CJD, and it remains unclear exactly what killed Mahan. But at least one expert, Dr. Omar Bagasra, a biologist at Claflin University in Orangeburg, S.C., suspected it could be a new strain of CJD related to mad cow-infected meat.
The uncertainty that often surrounds CJD cases is one reason why Sen. Durbin introduced his bill calling for increased surveillance of the disorder.
"Right now we're not tracking instances of BSE outbreaks or CJD," Durbin's spokesman Joe Shoemaker told UPI. "It's hard for us epidemiologically to say there's a cause and effect without some of those baseline comparisons," he said.
Not all of the victims Skarbek included on her list lived in New Jersey, but all had ties to the race track -- either as employees or attendees of events there. The race track is no longer in operation.
Skarbek said she thinks the racetrack cases are related to the consumption of meat infected with the mad cow pathogen because sporadic CJD is too rare -- scientists think it occurs in only one per million people -- to have happened by chance.
"Finding all of these cases related to the racetrack is statistically impossible, if it's one in a million and its supposed to be a coincidence," she told UPI.
Even if some or all of Skarbek's cases turn out to be coincidental, there still appears to be an unusual number of cases in a three-county area in the southern part of the state, according to the New Jersey Department of Health's own records.
Those records show seven people in Burlington, Camden and Gloucester counties have died from sporadic CJD since 2000. An eighth confirmed case -- a 70-year old man who died in Gloucester county in September -- does not yet appear to be included in the state's data.
Eight people over four years is double the expected number of cases for an area with a population of 1.2 million. Only about four people should have developed CJD over that time period.
It is not clear how many of the cases identified by Skarbek overlap with those included in the New Jersey Department of Health's records because the state data do not provide identifiable information.
Skarbek said she recently identified a possible ninth case in Burlington County: a 72-year-old man who died on March 2 and currently is being autopsied for CJD.
Dr. John Trojanowski, a brain researcher at the University of Pennsylvania in Philadelphia, said if that rate were sustained over time, it would indicate a true increase. But the rate may return to normal in future years, so it could turn out to be just a coincidence, he added.
Skarbek said she remains skeptical the CDC and New Jersey officials will release findings that support her claim of the CJD cases being tied to tainted meat.
"I think they're going to do their best to downplay it," she said. "I'd love for them to prove me wrong because then I can stop worrying about my mom, but I know in my heart I'm correct."
Steve Mitchell is UPI's Medical Correspondent. E-mail email@example.com
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