Three cases of an extremely rare and fatal brain disease have appeared in a single Oregon county, defying statistical probability for an illness that typically affects just one or two people per million worldwide.
Hood River County Health Department reported one confirmed and two probable cases of Creutzfeldt-Jakob disease (CJD) within the past eight months. Two of the affected individuals have died, and officials are awaiting definitive test results on the remaining case. For a county of just 23,000 people, this cluster is remarkably unusual, given that the entire United States typically sees only 350 cases annually.
The disease creates sponge-like holes in the brain through misfolded proteins called prions. Dr. Brian Appleby, director of the National Prion Disease Pathology Surveillance Center, explained to The Oregonian that CJD is typically identified by examining brain samples under a microscope for specific lesions and prion protein deposits. The condition progresses rapidly, with symptoms including confusion, hallucinations, and loss of coordination.
Health officials are investigating possible connections while respecting family privacy. "We're trying to look at any common risk factors that might link these cases… but it's pretty hard in some cases to come up with what the real cause is," Trish Elliott, director of Hood River County Health Department, told The Oregonian. The department has alerted local healthcare providers to watch for patients showing rapidly progressing Alzheimer's symptoms, sudden behavioral changes, or quick cognitive decline.
"The risk of getting CJD is extremely low," the health department said in a statement. "It does not spread through the air, touch, social contact, or water."
Previously:
• When she was 23, a lab worker pricked her thumb in an experiment with prion-infected mice. She died 10 years later
• Man eats squirrel brains, gets brain disorder and dies
• Fear and Trembling: Prion diseases on Twitter
• Study tracking people who ate zombie deer meat found no ill-effects, so far
