Arlene VonMyhr, Michigan woman, 55, dies from rare brain disease, Creutzfeldt-Jakob Disease a form of mad cow disease in quick descent over 5 weeks. Cause of disease remains unknown and is 100% fatal.
A Michigan woman has died from a rare brain disease which is known to be 100% fatal. Even more disconcerting, the 55 year old woman’s death is not the first to be reported in recent months.
Arlene VonMyhr, 55, on the evening of January 8 began to suddenly experience stroke symptoms, which typically include confusion, trouble walking, and facial weakness.
Doctors sent her home, and over the next two weeks, the mom and grandmom was rushed to the hospital four times over slurred speech and balance issues.
On January 26, VonMyhr went to the hospital only to never return home.
‘It was a really rapid five weeks of decline,’ Gary VonMyhr, her husband of 34 years, told Michigan Live.
Tests five days later revealed Creutzfeldt-Jakob Disease (CJD), a degenerative brain disorder that has been compared to Mad Cow Disease, the dailymail reported.
The condition mostly afflicts patients at random, is always fatal, and has no cure and despite previous instances when it first appeared in humans in the 90’s, as a byproduct of eating contaminated beef, almost no known cases are known to come from cattle today.
According to the National Institute of Health, Creutzfeldt-Jakob disease (CJD) is a rare, rapidly worsening brain disorder that causes unique changes in brain tissue and affects muscle coordination thinking, and memory. There are about 350 cases per year in the U.S.
The two main symptoms of CJD are:
- Severe mental deterioration and dementia
- Involuntary (unwanted) muscle jerks (myoclonus) or muscle movement
‘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 told MLive.
‘It was all about comfort and dignity at that point.’
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.
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.
Gary 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.’
