- KINGSTON -- A fifth Ulster
County death has been linked to Creutzfeldt-Jakob Disease, a rare and incurable
- 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
- 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
- "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.
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