Michigan woman, 55, dies from mad cow disease that is 100% fatal and has no known cause or cure

A Michigan woman died from a rare brain disease that kills 100 percent of patients and usually strikes randomly.

Arlene VonMyhr, 55, woke up the night of Jan. 8 with symptoms of a stroke, which typically include confusion, difficulty walking and facial weakness.

The doctors sent her home, and over the next two weeks she was rushed to the hospital four times for slurred speech and balance problems.

On January 26, the mother and grandmother went to the hospital and did not return home.

“It was a very rapid five weeks of decline,” said Gary VonMyhr, her husband of 34 years Michigan is alive.

Tests five days later revealed Creutzfeldt-Jakob disease (CJD), a degenerative brain disease that has been likened to mad cow disease.

The condition usually strikes patients randomly, is always fatal, and has no cure.

“Once CJD was finally diagnosed, they stopped all treatment and the IV at that point because there was nothing they could do for her,” Mr. VonMyhr said.

“It was all about comfort and dignity at that moment.”

Ms VonMyhr died on February 19, one of five at Corewell Health in Michigan to succumb to the condition within a year, prompting an “urgent investigation” last year.

CJD is an aggressive brain disorder caused by proteins called prions that misfold and punch holes in the brain.

This disrupts communication between cells and leads to dementia and symptoms such as memory loss, speech problems, balance problems and jerky movements.

About 85 percent of cases have no known cause, with most of the remaining cases coming 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 pick up this protein by eating the flesh of others who have the disease, the classic form of the disease, or it can occur spontaneously when a mutation causes the protein in them to misfold, the atypical form.

It can take years before animals with the atypical form begin to show symptoms.

CJD is incredibly rare, occurring in only one or two per million people per year.

However, the risk increases with age and affects five in a million people aged 55 and over.

This means that one in 6,000 deaths in the US 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 my brain working.”

The patients came from four West Michigan counties, and doctors said this indicated a much higher rate of CJD — between 3.1 and 12.5 per million.

“Such a wave of dense temporo-spatial clustering of CJD in West Michigan is very unusual and alarming,” researchers wrote in the case report.

The CDC said it was aware of the Michigan report and noted that “several cases of sporadic CJD may occasionally be diagnosed in a given area at about the same time based solely on chance,” according to epidemiologist Dr. .Ryan Maddox.

Mr. VonMyhr is now trying to raise awareness in the hope that CJD can be better researched.

“Obviously this doesn’t impact many people, but it is so aggressive, so debilitating and so impactful,” he said. “The ultimate motivation would certainly be to find a cure.”