Fifth Victim In NY
County CJD Cluster
Health Department Will Probe Deaths

By Gabriel J. Wasserman
Poughkeepsie Journal
KINGSTON -- A fifth Ulster County death has been linked to Creutzfeldt-Jakob Disease, a rare and incurable brain illness.
According to published reports, an autopsy revealed the disease as the cause of death for a female who died in November at Kingston Hospital. That brought to five the total number of Ulster cases over the past year.
No cause for the cluster has been identified, which is under investigation by the state Department of Health. Creutzfeldt-Jakob is a mysterious illness, with 80 percent of the cases having no known cause.
"It's not a communicable disease," said Dean Palen, director of public health for Ulster County, meaning it cannot be spread "person-to-person, from casual contact."
The state sees approximately 20 cases of "sporadic CJD" annually, said state Department of Health spokeswoman Claire Pospisil, referring to cases for which the cause is unknown. Other cases are linked to genetics.
Kingston Hospital officials could not be reached for comment Friday.
"We are not discussing any specifics on any cases due to patient confidentiality," Pospisil said.
Brent Tobey, whose father Richard died of the disease Oct. 9, would like to see more effort put toward prevention of the disease.
"We need to find answers as to why and how my father contracted this disease. It's just horrible for anybody's family to have to go through," he said. "This disease takes away everything that someone holds dear to them, to the point where they can't do anything, they forget how to swallow. We need to find out a way to prevent it."
Abnormal proteins infect brain
The Centers for Disease Control said Creutzfeldt-Jakob Disease, which is caused by abnormal proteins that infect and destroy brain tissue, occurs annually in one per every 1 million people.
Only 250 cases have ever been credibly connected to specific, non-genetic causes such as consumption of human growth hormone or contamination from surgical equipment, spokeswoman Christine Pearson said. The agency is helping state health officials, and will send experts to Ulster County if the state requests that, she said.
National data does not exist on historical clusters of Creutzfeldt-Jakob Disease, Pearson said, but New York may map it. Pospisil said she would "have to check" if that data is available.
The most rare but well-known form of the illness is mad cow disease, which is contracted by eating the infected brain or spinal tissue of cattle. None of the Ulster County cases have been identified as mad cow. Pearson said mad cow and "classic CJD" do not have the same causes.
State and local health officials said there has been no evidence that the Ulster County deaths were caused by a single source, adding that there is no threat to the public's health.
On Friday, Pospisil issued a new "fact sheet" on Creutzfeldt-Jakob Disease symptoms and causes, saying it was generated over the past week. It's available through the state health department.
Investigating the Ulster cases, she said, is "a priority for the department," with conclusions to be made "as soon as we possibly can."
- The Associated Press contributed to this story.
Copyright © 2004, Poughkeepsie Journal




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