Two people have died of a rare brain disorder called Creutzfeldt-Jakob disease in Hood River County, Oregon. Over the past eight months, three cases have been identified, with two confirmed and one presumptive diagnosis.
Creutzfeldt-Jakob is a progressive dementia caused by infectious proteins called prions, which can lead to rapid cognitive decline, movement disorders, and behavioral changes. The disease is incurable and universally fatal, with about 350 cases per year reported in the US.
Health officials say that transmission of the disease between people is rare, except through organ or tissue transplants or unusual exposure to contaminated tissue. Most cases are sporadic, meaning no clear cause has been identified, while hereditary cases are linked to a genetic mutation passed on from a parent.
The county’s health department is investigating the causes of the Hood River cases, but so far, there’s no evidence that they’re related to infected cattle. The department is advising local healthcare providers to be aware of Creutzfeldt-Jakob disease in patients with rapidly progressing Alzheimer’s symptoms or sudden behavioral changes.
Genetic testing can help identify hereditary cases, while brain samples and other tests can reveal the cause of a given case. Experts say that variant Creutzfeldt-Jakob disease linked to contaminated beef is typically identified through testing.
Source: https://www.oregonlive.com/health/2025/04/2-in-oregon-die-of-rare-brain-disease.html