Two people dead as HR County Health Department investigates Creutzfeldt-Jakob Disease; No link yet found in cases

By Tom Peterson

The Dalles, Ore., April 15, 2025 — The Hood River County Health Department is looking into the deaths of two individuals as officials have confirmed one case of Creutzfeldt-Jakob Disease (CJD) and suspect two others.

“Over the last 8 months, there have been 1 confirmed and 2 probable cases. Of these three cases, two people are deceased,” the department is reporting.

Creutzfeldt-Jakob Disease (CJD) is a rare, degenerative, and fatal brain disorder. It belongs to a group of diseases known as prion diseases, which also includes bovine spongiform encephalopathy (BSE or "mad cow disease") in cows and scrapie in sheep.

CJD is caused by abnormal prion proteins in the brain. Prions are proteins that normally exist in a harmless form, but when they misfold, they become infectious and cause other prion proteins to misfold as well. This chain reaction leads to brain damage and rapid neurological decline.

Creutzfeldt-Jakob Disease (CJD) comes in several forms, with sporadic CJD being the most common. It occurs due to spontaneous misfolding of prion proteins in the brain and accounts for the vast majority of cases, typically arising without any known cause. Familial CJD is much rarer and results from an inherited mutation in the PRNP gene, passed down through families. Iatrogenic CJD is very rare and is caused by exposure to contaminated tissue or medical instruments during certain procedures, such as transplants or older hormone treatments. The rarest form today is variant CJD, which is linked to eating beef from cattle infected with BSE ("mad cow disease"), and has become extremely uncommon due to modern food safety measures.

“Per state law, the health department investigates and monitors diseases that could be a risk to the public. At this time, there is no identifiable link between these three cases, the department reported.

Casandra Ware of The Dalles recently did some research into the probability of the three cases in a population of this size and wrote a letter to CCCNews.

“In a county the size of Hood River (~23,745 people), we would expect less than one CJD case every 30 years. To see three in just eight months is statistically extraordinary. The odds of three unrelated sporadic cases happening in that time and place are less than 1 in 193,000.”

“The risk of getting CJD is extremely low,” the health department stated. “It does not spread through the air, touch, social contact, or water. People with questions about their healthcare are encouraged to contact their primary medical team.”

This is an active and ongoing investigation in partnership with Oregon Health Authority (OHA) and Centers for Disease Control and Prevention (CDC). The health department will continue to monitor the situation and keep you informed of any risk to public health.

For more information on CJD, please visit CJD Foundation or the CDC’s website.