3 cases of rare brain disease found in Oregon; 2 deaths reported

Two people have died and a third remains under investigation after health officials in Hood River County identified multiple cases of Creutzfeldt-Jakob disease (CJD), a rare but deadly brain disorder caused by abnormal infectious proteins known as prions.

Over the past eight months, one case has been confirmed through autopsy and two others are classified as probable, according to the Hood River County Health Department. The patients’ identities and ages have not been released out of respect for their families.

Officials say there is currently no evidence of a link between the cases and emphasized that CJD poses extremely low risk to the general public.

What is Creutzfeldt-Jakob disease and how does it spread?

The backstory:

Creutzfeldt-Jakob disease is a rapidly progressing neurodegenerative disorder caused by prions—abnormal proteins that cause brain damage. It leads to symptoms such as dementia, movement disorders and personality changes. It is considered universally fatal, and there is no cure.

The disease is extremely rare, with about 350 cases diagnosed annually in the United States, according to the National Institutes of Health. Around 85% of cases are classified as sporadic, meaning there is no known cause. Some are hereditary, linked to a genetic mutation, while others result from medical procedures like organ transplants involving contaminated tissue.

CJD is not spread through the air, water, touch, or social interaction, and is distinct from bovine spongiform encephalopathy (mad cow disease), though both are prion diseases. The Hood River cases are not believed to be related to infected beef.

What are health officials doing to investigate?

What's next:

Trish Elliott, director of the Hood River County Health Department, said an investigation is underway in coordination with the Oregon Health Authority (OHA) and the Centers for Disease Control and Prevention (CDC).

Autopsy and testing can take months, especially when brain tissue must be examined out of state. Elliott said health officials are looking for any shared risk factors between the patients, but such investigations can be inconclusive due to the complexity and mystery surrounding prion diseases.

Healthcare providers in the area have been alerted to consider CJD in cases involving rapid cognitive decline or unusual neurological symptoms.

What they're saying:

Trish Elliott told reporters that while officials are trying to identify any links between the cases, it’s often difficult to pinpoint a specific cause. "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," she said.

Dr. Brian Appleby, director of the National Prion Disease Pathology Surveillance Center, explained that a diagnosis is typically confirmed by looking at brain tissue for prion protein deposits and lesions under a microscope. Genetic testing can also determine if a case is hereditary.

Why you should care:

Though CJD is extremely rare, its symptoms resemble other forms of rapidly progressing dementia, and its incurable nature makes early identification important. Health departments are urging local doctors to be aware of the warning signs, though the general public faces no elevated risk.

Health officials are continuing to monitor the situation closely and advise anyone with concerns to speak with their primary healthcare provider.