Normally occurring prions in the body appear as tiny spheres but when misfolded, they can cause other normal proteins to misfold, allowing the abnormal cells to build up and ultimately destroy brain tissue. (Shutterstock illustration),
Three cases of an always-fatal disease in humans that resembles “mad cow disease” have been found in the Hood River area of Oregon over the past eight months, according to health officials.
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Two people have died of extremely rare Creutzfeldt-Jakob disease, or CJD, and a third is showing symptoms consistent with the disease, according to public health officials in Hood River County.
In the United States, only about 350 people a year contract it, according to the National Institutes of Health. Globally, about one to two cases per 1 million people are diagnosed every year.
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That makes it statistically unusual that three people in a county of about 24,000 people have contracted it. Health investigators haven’t found a common link in the three cases beyond geography. Hood River County is located south of the Columbia River near the Oregon-Washington border.
The Hood River County Health Department said in mid-April there’s no need to panic.
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“The risk of getting CJD is extremely low. It does not spread through the air, touch, social contact, or water,” the health department said, adding it will continue to monitor the situation to ensure there is no risk to the public.
“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, the director of the Hood River County Health Department, told the Oregonian.
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Elliott declined to say whether the people who were infected were related, their names or their ages, the news outlet reported. CJD usually affects older adults, according to the Mayo Clinic.
Below are five things to know about Creutzfeldt-Jakob disease.
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What Is Creutzfeldt-Jakob Disease?
Creutzfeldt-Jakob disease, which is named for the two German doctors who first described the condition in the 1920s, is a rapidly progressive and always fatal neurodegenerative disorder.
It’s a prion disease, which scientists are still trying to decode. This much has been documented in various studies:
Prions, normally harmless proteins found in the body, can adopt an abnormal three-dimensional shape that in turn acts a bit like a template. That causes other normal proteins to misfold, allowing the abnormal cells to build up and ultimately destroy brain tissue.
Other aspects of prion diseases are less understood, such as their origin and development, transmission and the the underlying causes of different clinical presentations.
Is It Mad Cow Disease In People?
CJD and mad cow disease — bovine spongiform encephalopathy, or BSE — are both caused by prions, but they are distinct diseases.
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Humans can acquire a variant form of Creutzfeldt-Jakob disease by eating meat from cattle infected with mad cow disease, but those cases are even rarer. Only four cases ever have been reported to the CDC, and the agency said the evidence is strong that they were acquired in other countries outside the United States.
Elliott told the Oregonian that investigators don’t think the Hood River cases are linked to cattle with mad cow disease. The risk of contracting CJD by eating infected meat is generally low in the United States due to bans on the use of high-risk cattle parts in animal feed — a key factor in the spread of BSE in the United Kingdom.
Variant Creutzfeldt-Jakob disease, the type contracted from eating tainted beef, became prominent in the 1990s in the United Kingdom when dozens of people died after eating BSE-contaminated beef from cattle with BSE, delivering a multi-billion-dollar blow to the British beef industry. More than 4.5 million head of cattle were slaughtered before control measures similar to those in the United States were put in place.
If Not Mad Cow, Then What?
Eating infected meat isn’t the only way Creutzfeldt-Jakob disease can develop.
It may also be inherited through genetic mutations that inevitably lead to the emergence of prions. About 15 percent of those with CJD have a family history and may test positive for genetic changes associated with the disease, according to the Mayo Clinic.
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It can be transmitted in other rare scenarios as well.
A small number of people developed the disease after medical procedures, such as injections of pituitary human growth hormone from an infected source, and cornea and skin transplants from people who had CDJ, Mayo said, adding that clinics have changed procedures to eliminate those risks.
Also, a few people developed Creutzfeldt-Jakob disease after brain surgery with contaminated instruments, according to Mayo. That’s because standard cleaning methods don’t destroy the prions that cause the disease. Today, instruments that may have been contaminated with the disease-causing prions are destroyed.
Mostly — and again Creutzfeldt-Jakob disease is very rare — the disease is acquired sporadically and for no apparent reason, according to the Mayo Clinic. Most often, there is no identifiable cause of the disease.
The Hood River County Health Department said it is conducting an “active and ongoing investigation” in partnership with the Oregon Health Authority and the CDC.
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What Are The Symptoms?
Symptoms of Creutzfeldt-Jakob disease can be very similar to those of Alzheimer’s disease, although it progresses much faster, usually within several weeks to a few months after infection, and always leads to death.
Symptoms can include confusion, disorientation, hallucinations, seizures, psychological disturbances and loss of coordination.
Early symptoms include:
- Personality changes
- Memory loss
- Impaired thinking
- Blurry vision or blindness
- Insomnia
- Problems with coordination
- Trouble speaking
- Trouble swallowing.
- Sudden, jerky movements
Death typically occurs within a year of the onset of symptoms and is typically attributed to associated medical issues, such as falls, heart problems, lung failure, pneumonia and other infections, according to Mayo.
Link With ‘Zombie’ Deer Disease?
Last year, a team of researchers at the University of Texas Health Science Center at San Antonio caused a stir when they linked chronic wasting disease in deer to the CJD-attributed deaths of two hunters.
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“Zombie deer disease,” as it is sometimes called, is also caused by prions. It causes severe weight loss, lack of coordination, drooling and lack of fear of people among deer, elk and other animals First identified in deer at a Colorado research facility in the 1960s, CWD was discovered in wild deer in 1981. Since then, 36 states have reported CWD in wild deer, elk and moose in 33 states, according to the U.S. Geological Survey.
The University of Texas researchers’ study pointed to commonalities between the two hunters. They had both been at the same hunting lodge. Both ate venison from the same herd of deer known to have had chronic wasting disease.
The researchers emphasized their study, presented at the annual meeting of the American Academy of Neurology in 2024, did not prove chronic wasting disease jumped from deer to humans.
However, they said it “emphasizes the need for further investigation into the potential risks of consuming CWD-infected deer and its implications for public health.”
At the time, the CDC said it agreed more research is warranted on the potential for deer-to-human spread of chronic wasting disease, but said there is no evidence the two hunters’ shared activities contributed to their fatal diseases.
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“A history of hunting and/or eating venison does not mean that someone got CJD that way,” Ryan Maddox, a CDC epidemiologist, said in an email to USA Today. “Many Americans hunt and even more eat venison. Some will develop sporadic CJD by chance and others will not.”
Maddox pointed out that it’s unlikely the two hunters contracted CJD from eating the venison because symptoms don’t occur immediately.
“The men died from CJD either before or around the same time that CWD was found in the area where they hunted, leaving no time for a lengthy incubation period,” he said in the email to USA Today.
Also in response to the report, the Tennessee Wildlife Federation said the researchers went too far, stating that “attempting to link sporadic cases of CJD in humans to CWD with no documentation or evidence is incredibly irresponsible.”
