A Michigan woman died from a rare brain disease that kills 100 percent of patients and, in most cases, strikes at random.
Arlene VonMyhr, 55, woke up the night of Jan. 8 with stroke symptoms, which typically include confusion, difficulty walking and facial weakness.
Doctors sent her home and, over the next two weeks, she was rushed to the hospital four times for slurred speech and balance issues.
On January 26, the mother and grandmother went to the hospital and did not return home.
“It was five weeks of really rapid decline,” said Gary VonMyhr, her husband of 34 years. Michigan live.
Arlene VonMyhr, 55, of Michigan, died in February after being diagnosed with Creutzfeldt-Jakob disease, a degenerative disease that kills 100 percent of patients and usually strikes at random.
Five days later, tests revealed Creutzfeldt-Jakob disease (CJD), a degenerative brain disorder that has been compared to mad cow disease.
The disease mainly affects random patients, is always fatal and has no cure.
“Once CJD was definitively diagnosed, they stopped all treatments and the IV because there was nothing they could do for her,” Mr. VonMyhr said.
“At that time it was all about comfort and dignity.”
Mrs. VonMyhr died on February 19, one of five people at Corewell Health in Michigan to succumb to the disease in one year, prompting an “urgent investigation” last year.
CJD is an aggressive brain disorder caused by proteins called prions that misfold and perforate the brain.
This disrupts communication between cells and causes dementia and symptoms such as memory loss, slurred speech, balance problems and jerky movements.
About 85 percent of cases have no known cause, most of the remainder come from a genetic mutation of the prion protein.
Less than one percent is found in patients who ate diseased beef from animals with mad cow disease, clinically known as bovine spongiform encephalopathy (BSE).
Animals can acquire this protein by eating the meat of others that have the disease, the classic form of the disease, or it can occur spontaneously when a mutation causes the protein to fold incorrectly inside, the atypical form.
Animals with the atypical form may take years to start showing symptoms.
CJD is incredibly rare, occurring in only one or two per million people a year.
However, the risk increases with age and affects five in every million people aged 55 and over.
This means that one in every 6,000 deaths in the United States is due to CJD.
Although rare, Michigan reported five cases between June 2021 and June 2022including a 78-year-old woman who told doctors, “I don’t feel like my brain is working.”
The patients came from four counties in western Michigan, and doctors said this indicated a much higher rate of CJD: between 3.1 and 12.5 per million.
“Such a dense wave of temporal-spatial clusters of CJD in western Michigan is highly unusual and alarming,” the researchers wrote in the case report.
The CDC said it is aware of the Michigan report, noting that “several cases of sporadic CJD may occasionally be diagnosed in a particular area at approximately the same time due purely to chance,” according to epidemiologist Dr. Ryan Maddox.
VonMyhr is now trying to raise awareness in the hopes that CJD can be better researched.
“This obviously doesn’t affect that many people, but it is very aggressive, very debilitating and very impactful,” he said. “Without a doubt, the ultimate motivation would be to find a cure.”