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.
Creutzfeldt-Jakob Disease (CJD) is a brain disorder characterized by memory loss, jerky movements, gait disorder, rigid posture, and seizures due to a rapid loss of cerebral cells caused by transmissible proteins called prions. The disease is correctly diagnosed in anywhere from one to two people per million and it usually appears in mid-life with an average disease onset age of 50.
The prion that is believed to cause Creutzfeldt-Jakob exhibits an amino acid sequence and configuration which makes it insoluble in water, while the normal protein is highly soluble. So, as the numbers of defective prion proteins propagate and increase exponentially, the process leads to a huge load of insoluble prions in affected cells. This load of proteins disrupts cell function and causes cell death. Once the prion is transmitted, the defective proteins invade the brain like a forest fire and the patient dies within a few months (a few patients live for about 1-2 years). The defective protein can be transmitted by human growth hormone products, corneal grafts or dural grafts (acquired form) or it can be inherited (hereditary form) or appear for the first time in the patient (sporadic form). In the latter two forms the defective protein is not transmitted from an external source but already exists in the genes of the individual.
Cannibalism has also been implicated as a transmission mechanism for abnormal prions, the disease being known as Kuru found primarily among women and children of the Fore tribe in Papua New Guinea. The disease has also been shown to result from usage of HGH drawn from the pituitary glands of cadavers who died from Creutzfeldt-Jakob Disease , though the known incidence of this cause is (as of April 2004) quite small. Infection through HGH usage is restricted to patients in the U.S. who were treated with HGH during or before 1977, when newer methods of HGH purification were adopted.
Diagnosis is usually established by clinical findings and certain characteristic atypical electroencephalography findings. Biopsy of living brain tissue is definitive. There is currently no treatment for the disease, though as of December 2002 the first test of a proposed treatment (injection of pentosan polysulphate directly into the brain) has been approved in Britain.
A new variant of the disease (usually called just variant Creutzfeldt-Jakob Disease (vCJD) but sometimes new variant Creutzfeldt-Jakob Disease (nvCJD)) is distinguished from the classical type by its early onset (usually in the 20s) and a predominance of psychiatric and sensory symptoms. The prions in this form are thought to be transmitted by consuming the meat of bovines with so-called mad cow disease (Bovine Spongiform Encephalopathy), although there is no definite proof of this association as yet. However over 95% of identified cases of vCJD are in Britain, which suffered a mad cow disease epidemic in the mid-80s.
On September 26, 2003, it was reported that an experimental treatment given to a Northern Irish teenager halted the progress of brain damage caused by variant Creutzfeldt-Jakob disease. The drug, called pentosan polysulphate and commonly used to treat cystitis, was injected into the patient's brain. The patient weight and heart rate returned to normal levels after receiving the treatment. Still, there is no cure for vCJD, a fatal disease. 
The two German neurologists who first described this disease are Hans Gerhard Creutzfeldt and Alfons Maria Jakob. Interestingly, most of the clinical findings described in their first papers do not match current criteria for Creutzfeldt-Jakob disease, and it is considered highly likely that the patients in their initial studies were suffering from a completely different disorder. http://en.wikipedia.org
The report is available at http://www.state.nj.us/health/eoh/cjd2004.pdf.