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

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  • Arlene VonMyhr, 55, died after being diagnosed with Creutzfeldt-Jakob Disease
  • The condition kills 100 percent of patients and usually strikes at random 
  • READ MORE: US records 7th-ever case of mad cow disease in South Carolina

A Michigan woman died from a rare brain disease that kills 100 percent of patients and most often strikes at random.

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

Doctors sent her home, and over the next two weeks, she was rushed to the hospital four times over slurred speech and balance issues. 

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

‘It was a really rapid five weeks of decline,’ Gary VonMyhr, her husband of 34 years, told Michigan Live. 

Arlene VonMyhr, 55, of Michigan, died in February after being diagnosed with Creutzfeldt-Jakob Disease, a degenative disease that kills 100 percent of patients usually strikes at random

Tests five days later revealed Creutzfeldt-Jakob Disease (CJD), a degenerative brain disorder that has been compared to Mad Cow Disease. 

The condition mostly afflicts patients at random, is always fatal, and has no cure. 

‘Once a definitive diagnosis came back to CJD, then at that point they stopped all the treatments and the IV because there wasn’t anything they could do for her,’ Mr VonMyhr said.

‘It was all about comfort and dignity at that point.’ 

Mrs VonMyhr died on February 19, one of five at Michigan’s Corewell Health to succumb to the condition in 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 like memory loss, trouble speaking, balance issues, and jerky movements. 

About 85 percent of cases have no known cause, the most of the remaining ones come from a genetic mutation of the prion protein.

Less than one percent are found in patients who ate diseased beef from animals with mad cow disease, clinically known as bovine spongiform encephalopathy (BSE).

Animals can either pick up 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 misfold inside them, the atypical form.

It can take years for animals with the atypical form to start to show symptoms. 

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

However, risk increases with age, affecting five per one million people age 55 and older. 

This means that one in every 6,000 US deaths are from CJD. 

Though rare, Michigan reported five cases between June 2021 and June 2022, including a 78-year-old woman who told doctors, ‘I do not feel my brain work.’ 

The patients came from four counties in West Michigan, and doctors said that 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 is aware of the Michigan report, noting ‘several cases of sporadic CJD may occasionally be diagnosed in a particular area around the same time due purely to chance,’ according to epidemiologist Dr Ryan Maddox.

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

‘This obviously doesn’t impact as many people but it’s so aggressive, so debilitating, so impactful,’ he said. ‘The ultimate motivation would certainly be finding a cure.’

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