Interlude: Mad Squirrel Disease

PlagueBlog apologizes for missing this 2018 account of a death from mad squirrel disease in Rochester, New York circa 2015. Mad squirrel disease (not its official name) has apparently been known for at least 35 years.

The risk of contracting Creutzfeldt-Jakob disease by eating squirrel brains was documented in the Lancet in 1997. It seems the skilled roadkill chefs of rural Kentucky are able to extract brains from squirrels (which is perhaps the most impressive part of the whole story), which they then scramble with eggs or add to a stew named "burgoo".

The Lancet article is still paywalled after a quarter of a century, but the New York Times reported on it at the time, giving away a good deal of paywalled information:

In the last four years, 11 cases of a human form of transmissible spongiform encephalopathy, called Creutzfeldt-Jakob disease, have been diagnosed in rural western Kentucky, said Dr. Erick Weisman, clinical director of the Neurobehavioral Institute in Hartford, Ky., where the patients were treated.

''All of them were squirrel-brain eaters,'' Dr. Weisman said. Of the 11 patients, at least 6 have died.

Within the small population of western Kentucky, the natural incidence of this disease should be one person getting it every 10 years or so, Dr. Weisman said. The appearance of this rare brain disease in so many people in just four years has taken scientists by surprise.

While the patients could have contracted the disease from eating beef and not squirrels, there has not been a single confirmed case of mad cow disease in the United States, Dr. Weisman said. Since every one of the 11 people with the disease ate squirrel brains, it seems prudent for people to avoid this practice until more is known, he said.

The journalists are also more specific about squirrel prep:

Families that eat brains follow only certain rituals. ''Someone comes by the house with just the head of a squirrel,'' Dr. Weisman said, ''and gives it to the matriarch of the family. She shaves the fur off the top of the head and fries the head whole. The skull is cracked open at the dinner table and the brains are sucked out.'' It is a gift-giving ritual. The second most popular way to prepare squirrel brains is to scramble them in white gravy, he said, or to scramble them with eggs. In each case, the walnut-sized skull is cracked open and the brains are scooped out for cooking.

These practices are not a matter of poverty, Dr. Berger said. People of all income levels eat squirrel brains in rural Kentucky and in other parts of the South. Dr. Frank Bastian, a neuropathologist at the University of South Alabama in Mobile, said he knew of similar cases of Creutzfeldt-Jakob disease in Alabama, Mississippi and West Virginia.

The self-styled "Official Mad Cow Disease Home Page" seems to have covered this story as well, even including a recipe for burgoo. Significantly for the timeline, they also included several references from the paper. Among them was a 1984 article, Creutzfeldt-Jakob Disease. Possible Transmission to Humans by Consumption of Wild Animal Brains, in which the wild animals considered were wild goats and squirrels.

And that's the smoking squirrel that takes us back 36 years, to a time long before mad cow disease. Needless to say, PlagueBlog recommends you remove and dispose of the brain and spinal cord before eating any mammal, whether it has been reported to transmit TSEs yet or not.