Health officials in Hood River County say that two people have died of a rare brain disease.
County health officials say they’ve identified three cases of Creutzfeldt-Jakob disease in the last eight months. One was confirmed by autopsy, while two are presumptive diagnoses.
Creutzfeldt-Jakob is a rare brain disorder caused by infectious proteins called prions, which causes rapid, progressive dementia, movement disorders and behavioral changes. It is considered incurable and universally fatal. There are about 350 cases per year in the United States, according to the National Institutes of Health.
There’s no evidence the disease can be spread from person to person except through organ or tissue transplants or other unusual exposure to contaminated tissue.
According to the Centers for Disease Control, about 85% of all cases of Creutzfeldt-Jakob disease are considered sporadic, meaning there’s no clear cause. Most of the remaining cases are hereditary, linked to a genetic mutation passed on from a parent.
More health
Trish Elliott, director of Hood River County Health Department, said presumptive cases can only be confirmed by examining brain tissue and cerebrospinal fluid after death, which can take months. The bodies must be examined out of state.
Prion diseases are not well understood, and in many cases a cause is never identified.
“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.
She declined to say whether the people infected were related or to identify their ages out of respect for their families.
Creutzfeldt-Jakob disease is distinct from bovine spongiform encephalopathy, known as mad cow disease, which infects cows and is also caused by prions. A variant of Creutzfeldt-Jakob disease has been linked to consuming meat from cattle infected with mad cow disease, but such cases are much rarer.
Elliott said the Hood River cases are not believed to be related to infected cattle.
Dr. Brian Appleby, director of the National Prion Disease Pathology Surveillance Center at Case Western Reserve University, said Creutzfeldt-Jakob disease is typically identified by looking at brain samples under a microscope for lesions and prion protein deposits.
That and other tests can help identify the cause of a given case of the disease. Genetic testing can also reveal a hereditary case.
Cases of variant Creutzfeldt-Jakob disease resulting from eating contaminated beef can also usually be definitively identified through testing, Appleby said.
The county health department sent a notice to local health providers advising them to keep Creutzfeldt-Jakob disease in mind if a patient has rapidly progressing Alzheimer’s symptoms, sudden behavioral changes or rapid loss of cognitive abilities.
-- Kristine de Leon covers consumer health, retail, small business and data enterprise stories. Reach her at kdeleon@oregonian.com.
CORRECTION: An earlier version of this story incorrectly reported the number of deaths tied to Creutzfeldt-Jakob disease in Hood River County. Health officials reported three cases of Creutzfeldt-Jakob disease. Two people have died.