The Department of Health and Senior Services released a report today on an investigation of a suspected cluster of Creutzfeldt-Jakob Disease (CJD) associated with the Garden State Racetrack. The report summarizes an evaluation of the medical records of 17 individuals in six states suspected of having the rare, fatal brain disease.

“Today’s report indicates that there is no evidence of an increased incidence of CJD associated with the Garden State Racetrack or anywhere in this state,’’ said Commissioner of Health and Senior Services Clifton R. Lacy, M.D. “The number of cases is well within the range of expected cases for New Jersey and the United States.’’

CJD, which usually causes death within a year, is characterized by a rapidly progressive dementia, muscle twitching and a characteristic electroencephalogram (EEG) pattern. The disease is caused by a unique protein (prion) in the brain.

The report, “An Evaluation of a Suspected Cluster of Creutzfeldt-Jakob Disease (CJD) in New Jersey,’’ examines the records of 17 deceased individuals from six states: New Jersey, Pennsylvania, Maryland, Connecticut, Virginia and Delaware.

A South Jersey resident contacted the department about a number of suspected CJD cases she associated with beef consumption at the now-closed Garden State Racetrack in Cherry Hill.

Of the 17 cases examined in the report, 11 have a definite or probable diagnosis of sporadic CJD and 3 do not have CJD. Three cases are still under investigation pending receipt of patient records.

Six of the 11 definite or probable sporadic cases were residents of New Jersey. The mean age of the 11 definite/probable cases was 67.4 years. Analysis of the data did not reveal more cases than would be expected.

Dr. Eddy Bresnitz, State Epidemiologist and Senior Assistant Commissioner, was the lead author of the report. “The Department has worked closely with the U.S. Centers for Disease Control and Prevention, the National Prion Center, and other states to evaluate the records of these individuals. We are confident in our analysis and conclusions,’’ Dr. Bresnitz said.

The CDC established the National Prion Disease Pathology Surveillance Center in Cleveland, OH in the mid-1990s to provide enhanced neuropathologic and biochemical diagnostic services free of charge to U.S. physicians and state and local health departments.

There are 3 forms of CJD: Sporadic, inherited (or familial), and acquired by infection (which includes variant CJD). Variant CJD was linked to an outbreak of “Mad Cow Disease” (Bovine Spongiform Encephalopathy, BSE) in England in the mid-1980s. There have been no reported cases of variant CJD acquired in the U.S.

“The evidence does not support the existence of an outbreak of CJD among attendees at the Garden State Racetrack, nor does it suggest that case-patients with CJD were exposed to BSE-contaminated beef in the period from 1988 to 1992 at the Garden State Racetrack in New Jersey,’’ the report states.

CJD occurs at an overall rate of approximately one case per million people per year, but at a rate of 4.1 cases per million in individuals age 55 or older. Nationwide, there was an average of 253 CJD cases between 1985 and 2001, according to the report.

 An average of seven sporadic CJD deaths have been reported in New Jersey each year for the past 25 years. With a population of 8.5 million, the CJD incidence rate in New Jersey is within the expected incidence rate.

There has been no documented increased trend in the annual reported number of sporadic CJD cases in the U.S. or New Jersey.

New Jersey and about half of the states in the U.S. require physicians and others to report diagnosed or suspected cases of patients with CJD to local or state health departments.

The key to determining potential cases of CJD in the U.S. is through brain autopsies of all patients who die of suspected CJD. The DHSS will encourage physicians to obtain permission from family members for autopsies of suspected cases at the time of death and submit brain tissue and other laboratory data to the National Prion Center for expert analysis.

The department also will send a letter (prepared by the National Prion Center) to neurologists and pathologists, informing them of the need to consider CJD in diagnosing a patient with a rapidly progressive neurological disorder with dementia. The letter will point out that the National Prion Center offers its diagnostic services to evaluate brain autopsies.

The department will continue to review all suspected cases of CJD detected through reporting by health providers, and by reviewing hospital billing data and death certificate data.

The report is available at http://www.state.nj.us/health/eoh/cjd2004.pdf.

 The CDC will be releasing a companion report later today.

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