Grandmother dies suddenly of rare brain disease with 100% fatality rate and no cure
A Michigan grandmother has tragically passed away from a rare degenerative brain disorder similar to mad cow disease, which carries a 100% fatality rate and has no known cure.
Arlene VonMyhr, 55, and her husband, Gary, celebrated the University of Michigan winning the national football championship on Jan. 8 before she woke up with stroke symptoms, as reported by MLive.
After being initially sent home from Byron Center undiagnosed, VonMyhr was rushed to the ER four times over the following two weeks due to slurred speech and balance issues, according to the outlet. Sadly, on her fourth visit on Jan. 26, she passed away in the hospital on Feb. 19.
“It was a really rapid five weeks of decline,” shared Gary VonMyhr, her high school sweetheart and husband of 34 years, with MLive.
Doctors at Metro Health conducted a lumbar puncture that finally revealed her diagnosis of Creutzfeldt-Jakob Disease (CJD), a rare degenerative brain disorder that is invariably fatal and has no cure, as reported by the outlet.
“It was all about comfort and dignity at that point,” VonMyhr stated about his late wife, who was a mother of two and grandmother of three.
The disease, primarily affecting older adults, is caused by misfolded proteins that clump together and create holes in the brain, leading to dementia symptoms such as memory loss, speech difficulties, balance problems, and jerky movements.
“For almost everyone, unfortunately, it’s an extremely rapid course,” noted Brian Appleby, director of the National Prion Disease Pathology Surveillance Center.
About 85% of CJD cases are sporadic, occurring without a known cause, while the remaining cases result from a genetic mutation of a prion protein. Less than 1% of cases are variant CJD, caused by consuming tainted beef from animals with mad cow disease, although these are not the same diseases.
“In the US, CJD occurs in one to two people per one million a year, but risk increases with age,” said Appleby. “One in every 6,000 deaths in the country are due to the rare illness.”
While there is currently no cure for CJD, ongoing research and clinical trials offer hope for potential treatments in the future. Gary VonMyhr hopes to raise awareness about the disease to encourage further research and ultimately find a cure.
“This obviously doesn’t impact as many people but it’s so aggressive, so debilitating, so impactful,” he expressed to MLive. “The ultimate motivation would certainly be finding a cure.”
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