A 55-year-old grandmother from Michigan, Arlene VonMyhr, recently succumbed to a rare, incurable degenerative brain disorder akin to mad cow disease. The disease, known for its 100% fatality rate, struck VonMyhr suddenly after she celebrated the University of Michigan’s national football championship victory on January 8. Initially, she experienced symptoms similar to a stroke, which escalated over the following weeks into slurred speech and balance issues.
VonMyhr was admitted to the emergency room four times within two weeks. On her fourth visit on January 26, she remained in the hospital until her death on February 19. “It was a really rapid five weeks of decline,” said Gary VonMyhr, Arlene’s husband of 34 years and high school sweetheart.
Arlene was eventually diagnosed with Creutzfeldt-Jakob Disease (CJD) after a lumbar puncture was conducted by Metro Health doctors. CJD is a rare degenerative brain disease that is always fatal and has no known cure. The disease affects its victims randomly. “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,” Gary VonMyhr said. The focus then shifted to ensuring Arlene’s comfort and dignity.
The disease has prompted an “urgent investigation” after Corewell Health reported five cases within a year in West Michigan. CJD, which typically affects older adults, is caused by proteins that misfold, clump together, and create holes in the brain. This leads to dementia-like symptoms such as memory loss, speech problems, balance issues, and jerky movements.
The cause of these protein abnormalities, known as prion diseases, remains unknown. However, they are known to cause rapid deterioration and death within a few months. Approximately 85% of CJD cases are “sporadic,” meaning they occur without an apparent cause. The remaining cases are mostly due to a genetic mutation of a prion protein. Less than 1% of cases are variants where patients consumed tainted beef from animals with mad cow disease.
In the United States, CJD affects one to two people per million each year. However, the risk increases with age, affecting five per million people aged 55 and older. The disease accounts for one in every 6,000 deaths in the country.
While there is currently no cure for CJD, a treatment tested among British patients six years ago showed “promising early results,” and a clinical trial is underway in the US. The US Centers for Disease Control has acknowledged the Michigan case, noting that several cases of sporadic CJD may occasionally be diagnosed in a particular area due to chance.
Gary VonMyhr is now dedicated to raising awareness about the disease in hopes of spurring more research, treatment, and ultimately, a cure. “This obviously doesn’t impact as many people but it’s so aggressive, so debilitating, so impactful,” he said.