Man dies from extremely rare disease after eating squirrel brains

Grant Boone
October 20, 2018

A NY man died after he developed a rare and fatal brain disorder from eating squirrel brains.

Only once his brain scan results and other tests were out the experts found out that he may have had a variant of Creutzfeldt-Jakob disease (vCJD). vCJD is a type of a rare neurodegenerative ailment that can be fatal. According to reports, only a few hundred cases of vCJD have ever been reported.

The 61-year-old man was taken to a hospital in Rochester, New York in 2015, after his ability to think, recognition and motor skills diminished.

According to the man's family, he liked to hunt and used to eat squirrel brains.

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative brain disorder that is caused by coming into contact with tissue that has been infected, such as eating contaminated meat.

It might be shocking for many but, indeed, there was an American who ate squirrel brains.

The non-variant Creutzfeldt-Jakob Disease is also rare, with only about 350 cases per year in the United States, according to the NIH.

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Most people who contract it only live around a year. There is no treatment or cure for the disease.

His case was one of several detailed by doctors from Rochester Regional Health, a major hospital network in the city.

First described in 1996 in the United Kingdom, this beef is already infected with a disease that is similar called bovine spongiform encephalopathy, or "mad cow disease". According to statistics, mad cow disease in 85 percent of cases, leads to death in a mild form and always heavy. This high number prompted Chen and colleagues to conduct a review of suspected CJD cases at the hospital from 2013 to 2018. Tests indicated that this was a "probable" case of vCJD because of the MRI finding and a test that showed specific proteins in the patient's cerebrospinal fluid, which often indicate the disease.

Of the five cases detailed in their report, however, two were eventually confirmed not to be CJD after all.

The authors note that CJD is only confirmed by testing the brain tissue during an autopsy.

The report highlights the need for doctors to keep CJD diagnosis in mind and for hospitals to have "policies for infection control when it comes to CJD", Hanna said.

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