I don't know about an outbreak in Appalachia, but here's a report of one in western Kentucky in 1997.Wasn't there something published some years back about an "outbreak" or discovery of this ailment among Appalachian folks who ate squirrel brains?
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.
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.
"All of them were squirrel-brain eaters," Weisman said. Of the 11 patients,
at least six 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, Weisman said. The appearance of this rare brain
disease in so many people in just four years has taken scientists
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, 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 warning, describing the first five cases of Creutzfeldt-Jakob disease,
will appear in Saturday's issue of The Lancet, a British medical publication.
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. The people who died from Creutzfeldt-Jakob
disease in Kentucky were between 56 and 78, lived in different
towns and were not related, Weisman said.
The cause of transmissible spongiform encephalopathies is hotly
debated. Many scientists believe that the infectious agent is a
renegade protein, called a prion, which can infect cells and make
copies of itself. Others argue that a more conventional infectious
particle causes these diseases but that it has not yet been
identified. [It has now been established prions are the cause.]
In either case, the disease can be transmitted from one animal
to another by the eating of infected brain tissue.
Such diseases were considered exotic and rare until 10 years
ago, when an outbreak occurred among British cattle. Tens of
thousands of animals contracted a bovine variant called mad cow
disease, and their meat along with bits of brain tissue was sold as
hamburger. Thus far 15 people in Britain have died of a transmissible
spongiform encephalopathy that they seemed to have contracted from
eating infected meat.
Most people with Creutzfeldt-Jakob disease are elderly, but the
British victims were all young, which alarmed public-health
officials. The outbreak in western Kentucky has occurred in older
people, Weisman said, "which makes me think there may have been
an epidemic 30 years ago in the squirrel population."
Transmissible spongiform encephalopathies have a long latency
period, he said, which means many people in the South may be at
risk and not know it.