Multiple Cases of Rare Brain Disease Reported in County in Oregon

Three rare fatal brain disease cases have emerged in rural Oregon, leaving health officials monitoring closely as two victims have already died from the illness.

Key Takeaways

Hood River County, Oregon has reported three cases of Creutzfeldt-Jakob Disease (CJD) in the past eight months, with two resulting in deaths.
CJD is an incurable fatal brain disorder caused by prions (misfolded proteins) that affects only about 1 in a million Americans annually.
Health officials are investigating potential common risk factors but stress that public risk remains “extremely low.”
The disease progresses rapidly with severe neurological symptoms and typically leads to death within a year of onset.
Most CJD cases occur spontaneously without known causes, though some are hereditary or linked to specific exposures.

Rare Brain Disease Cluster Emerges in Oregon Community

A cluster of Creutzfeldt-Jakob Disease (CJD) has appeared in Hood River County, Oregon, with three cases identified over the past eight months. Two of these individuals have already succumbed to the disease, according to local health officials. This development has drawn attention because CJD is exceptionally rare, typically affecting only about one person per million annually in the United States. The appearance of multiple cases in a single county has prompted health authorities to launch an investigation into possible connections between the cases.

CJD belongs to a category of brain disorders called prion diseases, which occur when normal proteins in the brain misfold into abnormal shapes. These misfolded proteins then cause other healthy proteins to also misfold, creating a cascade effect that progressively damages brain tissue. Unlike most infectious diseases, prions aren’t viruses or bacteria but instead are malformed proteins that essentially infect normal proteins. The disease progresses rapidly and is invariably fatal, with no known effective treatments or cures currently available.

Investigating Potential Links Between Cases

Health officials have emphasized that the risk to the general public remains minimal. CJD cannot be transmitted through casual contact or airborne routes. The disease can only be spread through direct exposure to infected brain or nervous system tissue, such as through certain medical procedures or, in rare variants, consumption of contaminated beef. Investigators are currently examining whether the three Hood River cases share any common exposures or risk factors that might explain their proximity.

“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.” Hood River County Health Department director Trish Elliot said.

Adding to the complexity of the investigation is the fact that most CJD cases (about 85%) occur spontaneously without any identifiable cause—these are known as sporadic CJD. Approximately 10-15% of cases are genetic, resulting from inherited mutations. A small fraction, known as variant CJD, has been linked to consuming infected beef. However, health officials do not currently believe the Oregon cases are linked to cattle exposure.

Two dead, another infected, as rare brain disease reported in one Oregon county https://t.co/4LJS2Tliyo pic.twitter.com/pDke7uWFwa

— The Independent (@Independent) April 14, 2025

Recognizing CJD’s Devastating Progression

CJD produces symptoms that can initially appear similar to dementia or Alzheimer’s disease but progress at a dramatically accelerated rate. Early signs include memory problems, personality changes, and coordination difficulties. As the disease advances, patients may experience impaired vision, involuntary movements, blindness, weakness, and eventually progress to a coma. According to the Mayo Clinic, the disease advances “much faster” than Alzheimer’s, with most patients dying within a year of symptom onset.

One of the most challenging aspects of CJD is that definitive diagnosis typically requires examination of brain tissue, usually obtained after death. While tests on spinal fluid and brain imaging can suggest CJD in living patients, these are not always conclusive. This diagnostic difficulty has hampered research and treatment development for the condition. Additionally, the rarity of the disease means many physicians may encounter only one case—or none at all—during their entire careers.

Public Health Response and Recommendations

Hood River County health officials have described the risk to the public as “extremely low” while continuing their investigation. The Oregon Health Authority is working with local health departments to monitor the situation and identify any additional cases or risk factors. Experts emphasize that while the appearance of multiple cases in a small geographic area is unusual, clusters of rare diseases can occur by chance. Oregon has approximately 5-12 cases of CJD annually across the entire state, with this cluster representing a notable concentration in a single county.

Health authorities recommend that concerned individuals speak with their healthcare providers about any neurological symptoms, particularly rapidly progressing cognitive decline or unusual neurological manifestations. However, they caution against undue alarm, noting that the baseline risk of CJD remains low for the general population. The investigation continues as officials work to determine whether environmental factors or other exposures might explain this unusual cluster of a devastating but rare brain disease.