Fwd: [MC_USA] Articles on Squirrel Brains from University of KY

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Wayne Fugitt

Thu, 08 Jan 2004 23:50:18 -0600

[MC_USA] Articles on Squirrel Brains from University of KY

 

Creutzfeldt-Jakob Disease and eating squirrel brains

 

Lancet 350, Number 9078 - Saturday 30 August 1997

Joseph R Berger, Erick Weisman, Beverly Weisman

Department of Neurology, University of Kentucky, Lexington, KY 40536-0284, USA

 

Spongiform encephalopathies have been reported in a variety of large and small

mammals.1 While conducting a study of Creutzfeldt-Jakob disease (CJD) in south

Florida, one of us (JRB) observed an affected patient who was originally a

native of Kentucky and had a history of eating squirrel brains. Dietary

transmission of prion diseases has been documented experimentally in animals2

and in human beings who are cannibals.3 Several case reports have suggested the

possibility of transmission of CJD by consumption of brains of wild animals.4

 

These observations, together with recent concerns about the transmission of a

unique encephalopathy in man believed to be related to bovine spongiform

encephalopathy5 led us to examine the possible association of eating squirrel

brains with CJD in rural Kentucky, where eating squirrel and other small game is

not uncommon.

Culinary preparations include scrambling the brains with eggs or putting them in

a meat and vegetable stew referred to as " burgoo " . A history of eating squirrel

brains was obtained from family members of all five patients with probable or

definite CJD seen over 3,5 years in a neurocognitive clinic in western Kentucky.

 

Two women and three men aged from 56 to 78 years (mean 68.2 years) were

affected. None were related and each lived in a different town. Eating squirrel

brains was reported among 12 of 42 patients with Parkinson's disease seen in the

same clinic and 27 of 100 age-matched controls without neurological disease

living in western Kentucky. Ataxia early in the course of the disease was seen

in four of the patients with CJD and myoclonus and periodic complexes on the

electroencephalogram were seen in all.

 

Death occurred within 1 year in four, whereas, survival exceeded 3 years from

the onset of symptoms in one patient. Analysis of codon 129 of the prion protein

gene was not done. This observation will require confirmation by studies of

larger populations, and a search for a scrapie agent in the brains of squirrels,

which have not heretofore been reported as having spongiform encephalopathies.

In the meantime caution might be exercised in the ingestion of this arboreal

rodent.

1 Prusiner SB. Genetic and infectious prion diseases. Arch Neurol 1993; 50:

1129‚53.

2 Gibbs DJ Jr, Amyx HL, Bacote A, Masters C, Gajdusek DC. Oral transmission of

kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates. J Infect Dis

1980; 142: 205‚08.

3 Gajdusek DC. Unconventional viruses and the origin and disappearance of kuru.

Science 1977; 197: 943‚60.

4 Kamin M, Patten BM. Creutzfeldt-Jakob disease: possible transmission to humans

by consumption of wild animal brains. Am J Med 1984; 76: 142‚45. 5 Will RG,

Ironside JW, Ziedler M, et al. A new variant of Creutzfeldt-Jakob disease in the

UK. Lancet 1996; 347: 921‚25.

 

 

Kentucky Doctors Warn Against Regional Dish: Squirrels' Brains

 

By SANDRA BLAKESLEE August 29, 1997 NY Times

 

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 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, 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.

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.

 

Squirrels are a popular food in rural Kentucky, where people eat either the meat

or the brains but generally not both, 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, " 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 related to poverty, 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 that he knew of similar cases of Creutzfeldt-Jakob disease in

Alabama, Mississippi and West Virginia.

Squirrel-hunting season began last week, and it lasts through early December,

Berger said. He and Weisman are asking hunters to send in squirrel 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, Berger said.

 

 

Doctors probe squirrel-eaters

 

Thursday August 28 Reuter

LONDON - U.S. doctors are studying a possible link between eating squirrel

brains and catching the human variant of mad cow disease. Dr. Joseph Berger, a

neurologist at the University of Kentucky, said in a letter to the Lancet

medical journal that more research was needed but cautioned against eating

squirrels or similar rodents.

Berger said he had been conducting research into the fatal Creutzfeldt-Jakob

Disease (CJD) when he noticed that one patient, a Kentucky native, had a history

of eating squirrel brains. Consuming small rodents is not uncommon in the

southern state where squirrel brains are either scrambled with eggs or used in a

stew called " burgoo. "

 

Berger and his colleagues studied the eating habits of five patients in a

western Kentucky clinic with probable or definite CJD and found that all had a

history of eating squirrel brains. A new strain of CJD has been linked with

eating beef from cows infected with Bovine Spongiform Encephalopathy (BSE), or

mad cow disease in Britain, and several case reports suggest that CJD can be

transmitted by consuming the brains of wild animals.

Berger noted that patients with other diseases also had a fondness for the

rodent.

 

" Eating squirrel brains was reported in 12 of 42 patients with Parkinson's

disease seen in the same clinic and 27 or 100 age-matched controls without

neurological disease living in western Kentucky, " he told the Lancet.

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