From 1997:
Doctors in Kentucky have issued a warning that people should not eat squirrel brains, a regional delicacy, because squirrels may carry a variant of mad cow disease that can be transmitted to humans and is fatal. The disease in humans, squirrels and cows produces holes in brain tissue. Human victims become demented, stagger and typically die in one or two years.
Although no squirrels have been tested for mad squirrel disease, there is reason to believe that they could be infected, said Dr. Joseph Berger, chairman of the neurology department at the University of Kentucky in Lexington. Elk, deer, mink, rodents and other wild animals are known to develop variants of mad cow disease that collectively are called transmissible spongiform encephalopathies.
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.
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, Dr. Weisman said. Transmissible spongiform encephalopathies have a long latency period, which means many people in the South may be at risk and not know it.
The cause of transmissible spongiform encephalopathies is hotly debated. Many scientists believe the infectious agent is a renegade protein. Others argue a more conventional infectious particle causes these diseases. In either case, the disease can be transmitted from one animal to another by eating infected brain tissue.
Such diseases were considered exotic and rare until 10 years ago, when an outbreak occurred among British cattle. Tens of thousands contracted mad cow disease, and their meat along with bits of brain tissue was sold as hamburger. Thus far 15 Britons have died of transmissible spongiform encephalopathy they seemed to have contracted from eating infected meat.
In rural Kentucky, where squirrels are a popular food, people eat either the meat or the brains but generally not both, Dr. Weisman said. Families tend to prefer one or the other depending on tradition. Those who eat only squirrel meat chop up the carcass and prepare it with vegetables in a stew called burgoo. Squirrels recently killed on the road are often thrown into the pot.
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.''
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.
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.
Squirrel season began last week and lasts through early December, Dr. Berger said. He and Dr. Weisman are asking hunters to send in brains for testing, including those taken from dead animals found on the roadside. A mad squirrel would be more likely to stagger into the road and be struck by vehicles, Dr. Berger said.
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