A Fatal Mad Cow-Like Brain Disease Has Hit Oregon
In Hood River County, Oregon, three cases of a fatal brain disease were reported. Hood River County is located south of the Columbia River on the border of Washington and Oregon.
The county’s health department has confirmed one case and identified two other cases of the rare brain disease within the last eight months.
Two of the people with the condition have died. It is very similar to mad cow disease and is called Creutzfeldt-Jakob disease (CJD). It was named after two German doctors who first described the condition during the 1920s.
Around one to two million people worldwide are affected by the disease. The United States sees about 350 cases per year. Between 23,000 and 24,000 people live in Hood River County, so it was odd that three cases of CJD emerged.
CJD is a prion disease caused by abnormally folded proteins that damage brain tissue. It leads to sponge-like holes forming in the brain. The prions also make normal proteins in the brain turn into prions that inflict further damage.
Symptoms of the disease include confusion, disorientation, lack of coordination, muscle stiffness, hallucinations, changes in personality, psychological problems, and seizures. The symptoms progress quickly, leaving people unable to communicate and unaware of their surroundings.
The disease is fatal—roughly 70 percent of those affected die within a year of receiving a diagnosis. The most common type of CJD is “sporadic,” which means that normal proteins in the brain transform into prions for unknown reasons. Next, there is the genetic type, where CJD is triggered by inherited mutations in a gene called PRNP.
The most well-known and rare form of CJD involves prions entering the body from external sources. For example, if a person eats beef from cows with bovine spongiform encephalopathy (BSE), also known as mad cow disease, then CJD can occur. This type accounts for just one percent of cases.
In the U.S., only seven cases of BSE have ever been detected among cows. Another way for prions to get into the body is through medical exposure, like when a person receives a transplant or blood transfusion from someone with CJD.
Sign up for Chip Chick’s newsletter and get stories like this delivered to your inbox.
Since the 1990s, the U.S. has established strict agricultural regulations to keep mad cow-affected beef out of the food supply and prevent cases of acquired CJD.
In addition, there are infection control guidelines in place to prevent the transmission of CJD through medical procedures.
The Hood River County Health Department has not revealed which type of CJD the recent cases are. However, experts don’t think that infected cattle are the cause. The three cases do not appear to be linked to one another.
“The risk of getting CJD is extremely low,” said the department’s statement. “It does not spread through the air, touch, social contact, or water.”
The department is still investigating the cases and will keep the public informed of any health risks.
More About:News