Researchers believe that the death of a New York man infected with an extremely rare neurological disease was likely caused by eating squirrel brains.
The unidentified 61-year-old man arrived at a hospital in 2015 with symptoms of muddled thinking and difficulty walking.
A brain scan showed similarities with people suffering from variant Creutzfeldt-Jakob Disease (vCJD), a rare degenerative neurological condition in which the brain is infected with proteins called prions that form lesions. There’s strong evidence that the agent responsible for outbreaks of prion disease in cows, bovine spongiform encephalopathy (BSE, or “mad cow disease”), is the same agent responsible for vCJD in humans, according to the U.S. Centers for Disease Control and Prevention. The incurable, fatal disease can be transmitted through eating meat from infected cattle.
In the case of the New York man, he hunted and was known to eat squirrel brains, or at least squirrel meat that was likely contaminated by brain material, concluded Dr. Tara Chen of Rochester Regional Health and the lead author a research report on the case that was presented early this month at an Infectious Disease conference in San Francisco. “Intake of squirrel brains” was listed in the report as a possible risk factor.
The man died five months after he was diagnosed with vCJD. Researchers are seeking permission to test his brain tissue to determine what form of vCJD he contracted, Science Alert reported.
Squirrel brains, often mixed with other squirrel meat, are considered a delicacy in some regions of the U.S., noted a 1997 study in the Lancet that raised the possibility of a link to the disease.
To date, a total of only about 260 cases of vCJD worldwide have been linked to consuming beef, none of them in the U.S., according to the National Institute of Neurological Disorders and Stroke. Most cases have occurred in the U.K.
Besides BSE, other transmissible spongiform encephalopathies have been found in animals, including mink encephalopathy, feline encephalopathy and scrapie, which affects sheep and goats. Chronic wasting disease affects elk and deer.