WASHINGTON, Jan. 21 (UPI) -- An eighth person has died from Creutzfeldt-Jakob disease in southern New Jersey and two other possible cases may be linked to the area where seven others have succumbed to the rare brain disorder -- one form of which is linked to mad cow, United Press International has learned.
The newly identified CJD death brings the number of confirmed cases among people who lived in the area to eight -- a very large number for a disease thought to occur in only about one in a million people.
CJD clusters, which have occurred in several states in recent years, have received renewed attention since the discovery of a cow with mad cow disease in Washington state last month. Humans can contract a form of the disorder known as variant CJD from eating infected meat. None of the clusters, however, has been tied to tainted meat and all appear to consist mainly of a spontaneous form of the disease called sporadic CJD.
Still, the unusually high number of cases in southern New Jersey has raised concerns, including those of Janet Skarbek, an accountant in private practice in Cinnaminson, N.J., who has monitored cases in the area closely since last summer.
Officials from the Centers for Disease Control and Prevention in Atlanta, and the New Jersey Department of Health and Senior Services in Trenton, remain adamant the cases do not represent an unusual increase. New Jersey officials, however, have not interviewed family members involved and have only investigated three of the cases.
The CDC also has refused to look into the cases and, for more than six months, has declined to provide documents requested by Skarbek, under the Freedom of Information Act, regarding CJD clusters and investigations.
"It's unbelievable just how uninterested they are in looking at this," Skarbek said.
The CDC employs CJD experts and has a special center at Case Western Reserve University to study the disease but chose not to investigate.
"There's this perception CDC is not responsive -- but it's very important to understand the protocol here," which calls for state health departments to take the lead, agency spokesman Tom Skinner told UPI.
The latest confirmed case was John LaPaglia, 70, who lived in Deptford, N.J., and died last September, his sister Natalie Depman told UPI.
Two other cases -- which have not yet been confirmed -- did not live in the area but attended events at the Garden State Race Track in Cherry Hill, also in southern New Jersey. George Dernoeden, who was in his 50s, died Monday in Maryland but lived in Philadelphia, close to the racetrack. The cause of death was unclear but his brother Peter sent the body to Johns Hopkins University in Baltimore for an autopsy to check for evidence of CJD.
Frank Roland, 72, who lived in Abingdon, Pa., died in 1995 and also ate at the racetrack. Roland initially was diagnosed with Alzheimer's, but died within a year of developing symptoms -- which his physician thought was more consistent with CJD, his daughter Rosemarie Sheehan told UPI. An autopsy to confirm the disease never was performed.
These cases join seven others Skarbek has identified that died of CJD, amounting to 10 people in recent years who either worked or ate at the racetrack.
Whether the racetrack played a role in the disease may be immaterial, however. Many of the victims lived in the same three-county region, which has a population of 1.2 million.
Approximately one case of CJD would be expected per year in a population that size, but the region has exceeded that rate by up to four times in some years, according to figures provided by the New Jersey Department of Health.
In 2000 four people in the three counties died from CJD and for the four-year period from 2000 to 2003, a total of eight people in the three counties died from the disease.
Despite these figures, Dr. Eddy A. Bresnitz, the New Jersey state epidemiologist, told UPI, "There is no cluster of cases of CJD in New Jersey nor is there an increased incidence of CJD in the state."
Steve Mitchell is UPI's Medical Correspondent. E-mail firstname.lastname@example.org