A rare, incurable brain disorder similar to "mad cow disease" has claimed two lives in Oregon, alarming health officials. Three cases of Creutzfeldt-Jakob Disease (CJD), a rapidly progressing illness that leads to dementia and death, have been reported in the state over the past eight months.
Health officials confirmed that the cases were reported in Hood River County, with one confirmed and two probable diagnoses of Creutzfeldt-Jakob Disease.
"At this time, there is no identifiable link between these three cases. The risk of getting CJD is extremely low. 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," the Hood River County health officials said in a news release.
Creutzfeldt-Jakob disease often begins subtly, with personality changes, memory lapses, or trouble focusing, but it quickly escalates into severe neurological decline. While its early symptoms may resemble those of Alzheimer's, CJD progresses far more rapidly, and unlike Alzheimer's, it is always fatal.
Within weeks or months, people may experience blurred vision, insomnia, slurred speech, and muscle jerks. As the disease progresses, coordination worsens, swallowing becomes difficult, memory fades, and patients die from complications, falls, heart issues, lung failure, pneumonia, or other infections.
The disease gained widespread attention in the 1990s when a variant form, vCJD, emerged in the U.K., linked to eating beef from cattle infected with mad cow disease. However, most CJD cases are not connected to consuming contaminated meat.
This devastating brain disorder is caused by misfolded proteins called prions that can trigger a chain reaction, causing healthy proteins in the brain to misfold as well, leading to rapid brain damage. CJD affects about 1 to 1.4 people per million each year, with roughly 500 new cases annually in the United States.
Most cases arise spontaneously, without any clear cause, and are classified as sporadic CJD. This spontaneous form accounts for the vast majority of diagnoses. In fewer than 15% of cases, the disease runs in families due to inherited genetic mutations, a type known as familial CJD. Despite decades of research, the exact trigger for sporadic prion misfolding remains a mystery.
