Prion Deletion and Insertion Mutants

Squirrel brains and CJD in Kentucky
Kentucky Doctors: squirrels brain warning
Squirrel burgoo recipe (serves 25)
Doctors probe squirrel-eaters
World beef market set to recover after 1997 - WTO
Richard Rhodes: cure for E.coli is food irradiation

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: 112953.

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: 20508.

3 Gajdusek DC. Unconventional viruses and the origin and disappearance of kuru. Science 1977; 197: 94360.

4 Kamin M, Patten BM. Creutzfeldt-Jakob disease: possible transmission to humans by consumption of wild animal brains. Am J Med 1984; 76: 14245. 5 Will RG, Ironside JW, Ziedler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996; 347: 92125.

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.

World beef market set to recover after 1997 - WTO

Thursday August 28 1997
GENEVA, Aug 28 (Reuter) - After factors including the mad cow disease depressed global beef markets last year, 1997 looks to be a transition year for producers and traders worldwide, the World Trade Organisation (WTO) said on Thursday. Consumption is expected to recover from the dip caused by the suspected links between infected beef and Creutzfeldt-Jakob Disease (CJD), a dementing fatal illness in humans, the trade watchdog said in a report titled ``The International Markets for Meat.''

However, large and growing European Community (EC) stocks are overhanging the market, it said. ``This year may well be a transition period towards a better 1998,'' the report said. Referring to Bovine Spongiform Encephalopathy (BSE or mad cow disease), it said: ``As for the BSE impact which has dented beef consumption far beyond the EC, the worst may have passed and consumption may recover.''

The annual report by WTO economists pointed to signs that cattle liquidation in the United States is approaching its end and prices will recover as from late 1997. This promised higher imports and better returns for Australia and New Zealand, South American and other supplies to the U.S. market. The United States, which accounts for close to one-quarter of world beef imports, is set to import one million tonnes, a rise of nearly 10 percent. Second-place importer Japan will stabilise at 870 tonnes, followed by Russia (670 tonnes).

``As the U.S. supplies of cow beef decline, imports of beef (for processing) from Oceania are expected to pick up,'' it said. ``Next year, tightening supplies of grainfed beef may also dampen U.S. competitiveness on its overseas markets.''
In the 15-member EC, beef and veal production remained almost unchanged last year at 7.9 million tonnes. It is forecast to decrease by about one percent, with increases expected only in Ireland, France and Portugal, according to the WTO. EC consumption decreased by seven percent to 6.9 million tonnes last year and is expected to rise by two percent to 7.07 million tonnes this year, it added.

Australia is on track to remain the top beef exporter, with 1997 volume forecast at 1,080 tonnes, a rise of 6.5 percent. The EC is predicted to remain the second top beef exporter, but its export volume is forecast to drop five percent to 910 tonnes, followed by the United States at 860 tonnes, a rise of 1.5 percent.

In Asia, last year's import growth in Japan and South Korea was significantly below earlier expectations, the WTO said. ``In Japan, the impact of BSE, food poisoning due to E. coli bacteria, and the yen depreciation led to the first decline in imports after years of double-digit growth,'' it said. ``This year's prospects are highly uncertain, depending on factors such as consumer confidence in meat safety and further exchange rate developments,'' the report added. An outbreak of foot and mouth disease in Taiwan, which supplied 40 percent or 380,000 tonnes of Japan's pork imports last year, has left a big gap in the Japanese market, the WTO said.

Food Safety's Waiting Weapon

By RICHARD RHODES    August 28, 1997  NY Times Op Ed
Richard Rhodes is the author of ``Deadly Feasts: Tracking the Secrets of a Terrifying New Plague'' and ``The Making of the Atomic Bomb.''

MADISON, Conn. -- It's a good rule of thumb that technological solutions work better than increased regulation. Before 1920, thousands of babies died annually in New York and other large American cities from drinking contaminated milk. The solution wasn't more Federal dairy inspectors or a merger of Government agencies. It was pasteurization.

The solution to the problem of food poisoning -- whether the food involved is hamburger, strawberries, raspberries, cider or some other product susceptible to bacterial contamination -- has been sitting on the shelf for most of 40 years while hundreds of thousands of Americans have been sickened and thousands have died. It is the equivalent of pasteurization, and its neglect is a disgrace.

The technology is food irradiation. The Army pioneered its development beginning in 1943, and it has since passed into commercial application in some 40 countries, including limited use in the United States.

Irradiation uses gamma rays from a solid radioactive source to disrupt the DNA of, and thus to kill, noxious bacteria, parasites, mold and fungus in and on agricultural products. Gamma rays are similar to microwaves and X-rays.

Irradiation doesn't make food radioactive, nor does it noticeably change taste, texture or appearance. Depending on dose and on whether the food is packaged to prevent recontamination, irradiation can retard spoilage, kill germs or even completely preserve. The World Health Organization, the American Medical Association and the American Veterinary Medical Association all endorse the process.

The Food and Drug Administration has approved irradiation of pork, poultry, fruits, vegetables, spices and grains, although its use remains limited. Most imported spices are preserved with irradiation. Tropical fruits like mango and papaya from Hawaii are treated to kill exotic pests. Irradiated chicken is served in hospitals in the Southeast. Astronauts aboard the space shuttle eat irradiated food, including steak.

Food irradiation would have prevented the illnesses caused recently by contaminated hamburger from Hudson Foods and the several deaths linked to Jack in the Box restaurants in the Northwest in 1993. It could kill the salmonella that infects up to 60 percent of the poultry and eggs sold in the United States; the deadly mutant E. coli strain 0157:H7, which the Centers for Disease Control and Prevention have characterized as a major emerging infectious disease, and such ugly stowaways as beef tapeworms, fish parasites and the nematodes that cause trichinosis in pork.

Yet the new meat inspection system now being phased in by the United States Department of Agriculture does not even mention, much less mandate, irradiation. Neither Agriculture Secretary Dan Glickman nor the Food and Drug Administration invoked food irradiation as a solution to the Hudson Foods situation, preferring instead to press for destruction of 25 million pounds of meat that could have been made edible with the technique.

A petition for authorization to irradiate red meat has languished at the F.D.A. since 1994. Several states, including New York, have responded to pressure from citizen groups by either banning or imposing a moratorium on the sale of irradiated food without reviewing scientific evidence of the technology's safety and value.

Why the gap between promise and application? Because food irradiation -- like cancer treatment, medical diagnostics, sterilization of medical disposables, aircraft maintenance and many other technologies -- uses radioactivity, which Americans have been taught to fear. Commercial irradiators use metallic cesium-137 or cobalt-60 as sources of gamma radiation in heavily shielded processing plants; when the radioactive sources are not being used to sanitize food, they are stored safely underground.

Some anti-nuclear and environmental groups have campaigned against food irradiation, even imagining a conspiracy among the Food and Drug Administration, the World Health Organization and the nuclear power industry to use the process to dispose of nuclear waste.

Similarly fanatic resistance plagued the introduction of vaccination, water chlorination, pasteurization and fluoridation -- comparable technologies that have reduced disease and saved millions of lives. The unsupported fears of the Luddite opposition are making people suffer needlessly.

Mr. Glickman has said that the Hudson Foods case highlights the need to better educate the public on how to prepare food properly, but we can't all become sterile technicians at home. Thermometers won't protect us from E. coli-contaminated alfalfa sprouts.

Public health has been a primary responsibility of Government for more than a century. Inspection and testing alone, however responsibly applied, can never assure consumer safety where invisible pathogens are concerned. Pasteurization saved the babies. Irradiation can save our food.

Squirrel burgoo recipe (serves 25)

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Special Events Lawrenceburg Kentucky (Pop. 5,911)

Lawrenceburg Fair & Horse Show, Fairgrounds, June.
Fourth of July Celebration, July 4.
Kentucky Christian Horse Show, Fairgrounds, Sept.
Anderson County Burgoo Festival, late Sept.

INFORMATION: Contact Anderson Co. Chamber of Commerce, 205 E. Woodford St., Mon.-Fri.
8 am-4 pm or write P.O. Box 543, 40342. Phone (502)839-5564. Featuring Burgoo
Festival, historic Main St., Lawrenceburg. Antique shops, Boulevard Distillery, home of Wild
Turkey bourbon and Seagram Distilleries.

Kentucky Burgoo (Serves 25)

Makes 1200 gallons of stew; used for the Kentucky Derby Day celebration This recipe is from a very old southern cookbook entitled "322 Old Dixie Recipes"(1939)
Squirrels - 1 each 100 gals.
600 lbs. lean soup meat (no fat, no bacon) 
200 lbs. fat hens, plucked
2,000 lbs. potatoes, peeled and diced
200 lbs. onion, peeled and diced
5 bushels of cabbage, chopped
60 lbs.of tomatoes, deskinned & chopped
24 lbs. of tomatoes - pureed
24 lbs. of corn, cut from cob
Red pepper and salt to taste and Worcestershire by the pint.
Mix the ingredients, a little at a time and cook coutdoors in huge iron kettles over wood for 20 hour, stirring constantly.

The name burgoo literally means a soup composed of many vegetables and meats delectably fused together in an enormous caldron, over which, at the exact moment, a rabbit's foot at the end of a yarn string is properly waved by a black preacher whose salary has been paid to date. These are the good omens by which the burgoo is fortified.

What is burgoo?

If you are like most people you have never heard of much less eaten burgoo. This is one of those times its definately best to be in the minority. Burgoo is a savory stew made from a varrying array of ingredients. If is often cooked in enormous iron kettles outdoors over an open flame. Cooking can take as long as 30 hours and flavor improves as it ages.

It has been said that burgoo is more of a concept than a recipe. This is because there are as many different ways to prepare burgoo as there are people who prepare it. The meats could include any or all of the following meats: squirrel, mutton (sheep/lamb), beef, pork, chicken, veal or opossum. You will also find some combination of these vegetables: potatoes, corn, lima beans, tomatoes, or okra. Of course there are also many spices to choose from as well. As you might imagine there are many people who keep their recipes a closely guarded secret.

What does "BURGOO" mean?

It is believed that the word "burgoo" originated in the 17th century on the high seas. These sailors used to subsist on an oatmeal-like porridge made from the Middle-Eastern grain, bulgur (or bulghur) wheat. The term first appears in the 1650 book "Adventures by Sea" by Edward Coxere.

Where can I get some burgoo?

The center of the burgoo universe is Owensboro, Kentucky. Owensboro is knows as "The Bar-B-Q Capital of the World." This is due, in part, to the world renowned International Bar-B-Q Festival held on the Riverfront each May. As part of the festival teams from charitable organizations, churches or businesses compete to be named the best. During the festival over 1,500 GALLONS of burgoo will be served up. By the cup (with a slice of white bread) or by the gallon (wide-mouth glass or plastic jar). You will also find live entertainment, a midway with rides and games, arts and crafts as well as other foods.

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