Two people inOregonhave died of a rare brain disorder that causes dementia-like symptoms without a clear cause or possible link tying the deaths together.
Officials in Hood River County, home to about 24,000 people in northern Oregon, have identified three cases of Creutzfeldt-Jakob disease over the past eight months, two of which have been fatal.
Creutzfeldt-Jakob disease (CJD) is rare - infecting about 350 Americans per year - but it isalmost always fatal.The disease, which causes a rapid descent into dementia, is caused by faulty proteins in the brain called prions that corrupt healthy ones.
The underlying trigger of CJD remains unclear and most cases are sporadic with no known cause. However, some forms are genetic or linked to contaminated beef.
It is not spread from person-to-person unless in the case of infected transplanted organs, and Oregon officials do not know if the state's cases are related.
One patient was confirmed by autopsy to have died of CJD, while the other death is believed to be caused by CJD.State pathologists are examining brain tissue and cerebrospinal fluid, one of the two ways to diagnose CJD.
Rarely, a brain biopsy is performed to confirm the disease, which involves surgically removing brain tissue and often results in brain bleeding, infection, and death.
A third person suspected of having the disease is still alive.
Trish Elliott, director of Hood River County Health Department, said: ‘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.’
Panel B shows a section of the cerebral cortex where prions have killed white matter in the brain, creating lots of tiny holes, a pattern called “status spongiosis,” caused by severe nerve cell loss. Panel C shows severe damage in the deeper layer of white matter
Health officials declined to give personal details on the patients or say whether the three cases were from the same household or family.
A variant of CJD has been tied to eating meat from cattle infected with mad cow disease, another prion disorder, but Elliott said the cases in Hood River are not believed to be linked to cattle.
Eighty-five percent of CJD cases are ‘sporadic,’ meaning they have no apparent cause or recognizable pattern of spreading, according to the CDC.
Between 10 to 14 percent of CJD cases are caused by mutations in the PRNP gene, which encodes for the prion protein.
The remaining one percent are caused by external factors, such as eating infected beef or undergoing medical procedures with infected tools or receiving transplanted organs.
It typically affects people in their 50s and older and presents as rapid degeneration of brain tissue.
Patients usually die within four or five months.
There are outliers, though. A 22-year-old college student lived for seven months with progressively worsening dementia tied to CJD.
Prion diseases primarily affect people in their 50s and older and presents as rapid degeneration of brain tissue
A previously high-functioning college student, she returned home from school and began experiencing severe anxiety, prompting treatment with benzodiazepines prescribed by a doctor.
Still, her symptoms worsened. She experienced hallucinations, sleepwalking, and insomnia.
She also developed repetitive behaviors and became fixated on completing tasks like handwashing and toothbrushing in a precise manner.
She soon began to experience cognitive issues, including memory problems, trouble doing familiar tasks, and forgetting the names of close family and friends.
Doctors performed MRIs, cerebral spinal fluid tests, and genetic testing to determine the cause and suspected it to be CJD.
It wasn’t confirmed until the patient died, though, by examining brain tissue in the lab, spotting the sponge-like holes indicative of CJD.
Early signs of the disease include unexplained mood shifts, irritability, paranoia, delusions and other symptoms of psychosis, and social withdrawal.
Over the following weeks or months, CJD causes aggression, confusion, memory loss, inability to speak or eat, and incontinence.
Hood River County Health Department did not disclose when the three cases first became sick.
Health officials issued a warning to local health workers to keep CJD in mind when they treat patients exhibiting signs of rapid descent into dementia.
They maintain that the risk to the public is low.
