GRAND RAPIDS, Mich. (Michigan News Source) – A group of West Michigan doctors have filed a case report exploring localized clusters of a rare and lethal disease. The report cites five confirmed cases within a ninety-mile radius of Grand Rapids, calling the situation “a serious public health challenge [that] warrants urgent investigation.”
Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disease caused by an abnormal form of the brain prion protein, which can be inherited, spontaneous, or in extremely rare cases transmitted through contact with contaminated brain tissue. Dr. Nicholas Lannen, a neurologist with Corewell Health, said the disease is “catastrophic.”
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“Unfortunately, it’s uniformly fatal,” he said. “No one’s ever survived CJD.”
According to Michigan’s CJD fact sheet, the disease can incubate for up to 30 years before patients show symptoms. First signs include progressive dementia, confusion, behavioral changes, and muscle incoordination. Patients diagnosed with the disease usually do not survive beyond a year.
There are typically fewer than 1,000 cases annually in the United States, and the odds of contracting the disease are about one in a million. Dr. Ling Ling Rong, neurology division chief at Corewell Health, said she was surprised to treat two cases in a single month.
“That made my attention go, ‘What’s going on with this disease here in Michigan?’” she said.
Both Lannen and Rong helped author the case study, which has garnered attention in the medical community.
Actual rates of CJD in western Michigan may be higher than the confirmed case count. According to the case study, family members of three patients diagnosed with the disease reported several additional possible or probable cases of CJD among their friends or communities. The study concludes that this report warrants “urgent investigation,” followed by investigation into environmental factors and transmission events that may link the cases.
“I’m hoping that there’s no more cases, Lannen said, “but if there are we will be more aware and able to keep a better sense of the trend.”