A grandmother in Michigan died from a rare degenerative brain disease similar to mad cow disease that has a 100% fatality rate and no cure.
Arlene Fonmire, 55, and her husband, Gary, celebrated the University of Michigan’s national football championship on Jan. 8, before they woke up with symptoms of a stroke. M Live reported.
The report said that after Vonmeyer went home from the Byron Center without being diagnosed, she developed slurred speech and problems with balance and was rushed to the hospital four times over the next two weeks.
During her fourth visit on January 26th, she did not leave the hospital until her death on February 19th.
“The five-week decline was really rapid,” Gary Fonmire, her high school sweetheart and husband of 34 years, told MLive.
Metro Health doctors performed a lumbar puncture and ultimately diagnosed her with Creutzfeldt-Jakob disease. The disease is a rare degenerative brain disease that is always fatal and has no cure, the newspaper reported.
Its victims are also mostly random.
“Once they got the confirmed diagnosis of CJD, at that point they stopped all treatment and IV fluids because there was nothing they could do for her,” Vonmaier, 55, told MLive.
“At that point, it was all about peace and dignity,” he added of his wife, a mother of two and grandmother of three.
Doctors called for an “urgent investigation” after Corewell Health confirmed five cases in West Michigan in a year, suspecting a cluster.
The disease, which usually affects older people, is caused when proteins misfold and clump together, creating holes in the brain and can cause symptoms such as memory loss, speech problems, balance problems, and jerky movements. It causes associated dementia.
Researchers don’t know why the protein acts the way it does, but the disorder is known as prion disease, and those affected rapidly deteriorate and die within a few months.
“Unfortunately, for almost everyone, this is a very rapid progression,” said Brian Appleby, director of the National Prion Disease Pathology Surveillance Center.
About 85% of cases occur without an obvious cause, and most of the rest are reported to be “sporadic” because they are caused by genetic mutations in the prion protein, according to MLive.
Although it is not the same disease, less than 1% of cases of the variant have been caused by patients eating contaminated beef from animals infected with mad cow disease, another prion disease.
“It damages the brain. It kills brain cells. We don’t necessarily know why,” Appleby said.
In the United States, CJD affects 1 to 2 people per million per year, but the risk increases with age, affecting 5 people per million over the age of 55.
One in every 6,000 deaths in the country is caused by this rare disease, he told the media.
There is no cure for CJ, but one treatment tested on patients in the UK six years ago showed “promising early results” and clinical trials are reportedly underway in the US. .
The U.S. Centers for Disease Control said it was aware of the Michigan case report, noting that “several cases of sporadic CJD may be diagnosed in a given area at about the same time, purely by chance.” , MLIve reported, citing epidemiologist Ryan Maddox.
The state Department of Health and Human Services said the findings were not surprising given the “broad geographic scope defined in the paper” and the size of Corewell’s footprint.
“Michigan’s CJD case numbers continue to remain within our expectations, although there is some variation from year to year,” the agency said in a statement.
According to federal data, the number of annual infections has jumped from 238 to 538 over 20 years, but the rate has remained the same after adjusting for age.
Von Mayer said he is trying to tell more people about the disease to allow for research, treatment and a cure.
“This obviously doesn’t affect a lot of people, but it’s very aggressive, very debilitating, very impactful,” he told MLive. “The ultimate motivation will certainly be to find a cure.”
