Mad Cow Disease Fast Facts - CNN.com Mad Cow Disease Fast Facts CNN Library Updated 10:07 AM ET, Wed June 22, 2016 Chat with us in Facebook Messenger. Find out what's happening in the world as it unfolds. A cow is silhouetted on a pasture near the Trans-Canada Highway north of Calgary, Alberta on February 13,2015. The Canadian Food Inspection Agency (CFIA) confirmed February 13, 2015 that a case of mad cow disease has been found in Alberta, Canada .JOE KLAMAR/AFP/Getty Images) (CNN) Here's a look at Mad Cow Disease, a transmissible fatal brain disease found in cattle. Facts: It has been linked to a fatal brain disease in humans called variant Creutzfeldt-Jakob disease (vCJD). The official name of mad cow disease is bovine spongiform encephalopathy (BSE) . BSE lesions are characterized by sponge-like changes seen under an ordinary microscope. Eating contaminated meat or other products from cattle (excluding dairy products) with BSE is thought to be the cause of vCJD.

BSE is passed between cows through the practice of recycling bovine carcasses for meat and bone meal protein, which is fed back to other cattle. Read More Both mad cow disease and variant Creutzfeldt-Jakob Disease (vCJD) are fatal. Symptoms of vCJD involve psychiatric symptoms and behavioral changes, movement deficits, memory disturbances and cognitive impairments. BSE Statistics (Cattle): (source: CDC ) BSE cases in North America 1993-February 2015: 24 cases confirmed, 20 in Canada, and four in the United States. One of the infected cows that died in the United States was born in Canada. vCJD Statistics (Humans): (source: CDC ) 1996-2014 - 229 vCJD cases have been identified in 12 countries: United Kingdom - 177 Taiwan - 1 Timeline: 1986 - Mad cow disease is first discovered in the United Kingdom. From 1986 through 2001, a British outbreak affects about 180,000 cattle and devastates farming communities.

January 1993 - The BSE epidemic in Britain reaches its peak with almost 1,000 new cases being reported per week. 1996 - The first case of variant Creutzfeldt-Jakob Disease is reported. 1996-1999 - The European Union bans British beef. France continues the ban for three more years, on the grounds that British beef is not safe. May 20, 2003 - Canada's first case of mad cow disease is confirmed in an eight-year-old cow in Alberta. Canadian officials say the cow did not enter the food chain. May 21, 2003 - Mexico, Japan and South Korea join the United States in temporarily banning Canadian beef. The United States lifts the ban in March. December 23, 2003 - The U.S. Department of Agriculture confirms the first case of mad cow disease in the United States. The infected cow is discovered on a farm in Washington State in early December. Japan, China and South Korea stop the importation of U.S. beef.

The infected cow was born in Alberta, Canada, in April 1997 - just four months before the United States and Canada began banning the use of brain and spinal cord tissue in cattle feed. December 30, 2003 - The USDA announces that the beef from downer cattle will no longer be allowed in the human food chain. January 9, 2004 - The USDA says it will begin destroying about 130 cattle that were "herd mates" of the cow that tested positive for the first-ever U.S. case of mad cow disease - to make sure none of those cows enters the human food chain. January 26, 2004 - New safeguards against mad cow disease are announced by the Food and Drug Administration. They include banning chicken waste from cattle feed and barring restaurant meat scraps from being used in animal feed. January 28, 2004 - The Commodity Futures Trading Commission launches an investigation into whether some commodity futures market players may have known about the first U.S. case of mad cow disease before it was announced to the public.

June 20, 2004 - Charlene Singh, the first person known to live in the United States with variant Creutzfeldt-Jakob Disease, dies. January 2, 2005 - Canadian health authorities confirm that test results have identified a 10-year-old dairy cow in Alberta as having mad cow disease. This is Canada's second case of bovine spongiform encephalopathy (BSE) in two years. June 24, 2005 - The second U.S. case of BSE is confirmed by British tests. March 13, 2006 - The third U.S. case of BSE is confirmed after an Alabama cow tests positive. September 5, 2008 - Canadian scientists announce a discovery that paves the way for diagnostic testing of live cattle, rather than postmortem. September 13, 2008 - An Alabama research study shows that mad cow disease can sometimes be caused by genetic mutations.

March 10, 2009 - An anti-malaria drug known as quinacrine, which had reportedly shown promise against mad cow disease, is found to have no effect on the disease according to a British medical study. March 14, 2009 - U.S. government permanently bans the slaughter of cows too sick or weak to stand on their own, seeking to further minimize the contraction of mad cow disease. March 28, 2009 - Mad cow research pathologist Antonio Ruiz Villaescusa of Spain dies from mad cow disease. April 24, 2012 - The USDA confirms the fourth case of BSE, found in a dairy cow from central California . The announcement maintains that the cow was never presented for slaughter for human consumption and poses no risk. March 2014 - After 15 years, the United States lifts the ban on beef from the European Union, pending inspections. In the 1980s and 90s the brain disorder infected 180000 - ENG - speech View Full Document nightmare.

In the 1980s and '90s, the brain disorder infected 180,000 livestock in Europe and claimed dozens of human lives, devastating the British cattle industry. Britain, the United States and most other major cattle-producing nations have imposed stringent measures to control the disease. 1. HowStuffWorks.com Mad cow disease, or bovine spongiform encephalopathy (BSE), is a fatal brain disorder that occurs in cattle and is caused by some unknown agent. In BSE, the unknown agent causes the cow's brain cells to die, forming sponge-like holes in the brain. The cow behaves strangely and eventually dies. The connection between BSE and humans was uncovered in Great Britain in the 1990s when several young people died of a human brain disorder, a new variation of a rare brain disorder called Creutzfeldt-Jakob Disease (CJD), which typically strikes elderly people. The new variation was called new variant Creutzfeldt- Jakob Disease (nvCJD), was similar to BSE and its connection to BSE was based on the following findings: 2. The nvCJD victims had lived in areas where outbreaks of BSE had occurred in cattle years earlier.

No victims were found in areas without BSE outbreaks. The brains of nvCJD victims had proteins called prions (pronounced "pree-ahnz") that were similar to those from the brains of BSE-infected cows, but different from those found in victims of classic CJD. The time between the BSE outbreaks and the deaths of the victims This preview has intentionally blurred sections. Sign up to view the full version. This is the end of the preview. Sign up to access the rest of the document. TERM Center for Food Safety | Issues | Mad Cow Disease | Timeline: Mad Cow Disease Outbreaks Timeline: Mad Cow Disease Outbreaks Timeline of Mad Cow Disease Outbreaks 1986 Cattle in Britain begin to suffer from a condition similar to scrapie in sheep, nicknamed “mad cow disease” due to the behavior of the sick cows. The cause is unknown, though some suspect the feeding of rendered scrapie-infected sheep to cattle. 1988 421 cattle have been diagnosed with BSE in Britain. 1989 Britain bans human consumption of certain organ meats, including brain and spinal cord. The U.S.

prohibits the import of live cattle, sheep, bison, and goats from countries where BSE is known to exist in native cattle. Early 1990s The British government insists the disease poses no threat to humans. An early advisory committee states that cattle would be a “dead end host.” House cats begin dying from beef byproducts in their pet food. Five types of antelope die in British zoos from TSEs that had been fed commercial cattle feed. Through all of this, the British government continues to adamantly insist that British beef is perfectly safe, and BSE is no threat to humans. 1993 120,000 cattle have been diagnosed with BSE in Britain. Mid-1990s Britain bans the feeding of meat and bone meal to animals and its use as farm fertilizer, and begins tracking individual animals and testing any cow over 30 months old that is intended for human consumption. May 1995 Stephen Churchill, 19, becomes the first victim of a new version of Creutzfeldt-Jakob Disease (vCJD).

His is one of three vCJD deaths in 1995.[i] March 1996 British Health Secretary announces to the British House of Commons that mad cow disease is “the most likely explanation at present” for “10 cases of CJD in people aged under 42.” This is the first time the British government admits BSE could be transmitted to humans in a variant form of CJD. After this point, 4.5 million cattle are destroyed. Japan bans imports of meat-and-bone animal feed from Britain, while the EU announces a ban on British beef and beef products. August 1996 Britain’s agriculture ministry confirms that mad cow disease can be passed from cow to calf. British coroner rules that Peter Hall, a 20-year old vegetarian who died of vCJD, contracted it from eating beef burgers as a child. The verdict is the first to legally link a human death to mad cow disease. Some estimates are that three British farmers per week are killing themselves.[ii] July 1997 21 vCJD victims in Britain have been confirmed, many more unconfirmed cases. August 1997 The U.S.

Food and Drug Administration (FDA) bans protein made from cows, sheep, deer, and other so-called ruminants in feed for other ruminants. August 1999 The export ban of British beef is lifted after 3½ years.[iii] June 2000 Britain announces a cow born after measures were introduced to eradicate mad cow is found to have BSE.[iv] September 2001 The first outbreak of BSE occurs in Japan. April 2002 The first confirmed case of vCJD appears in the U.S., in a 22-year-old British woman living in Florida. August 2002 A Canadian man dies from vCJD, apparently after contracting the disease in Britain.[v] May 2003 A bull in Canada tests positive for BSE, the first confirmed case of a cow born in North America. December 23, 2003 A cow in Washington State tests positive for BSE. December 30, 2003 U.S.

Department of Agriculture (USDA) bans sick and injured (“downer”) cattle from human food supply, as well as specified risk material and tissues, such as brain and spinal cord, from cattle over 30 months old and mechanically separated meat. A new system of animal identification is also to be implemented.[vi] January 26, 2004 FDA bans feeding cow blood, chicken waste, and restaurant scraps to cattle.[vii] January 2004 143 people in Britain have been infected with vCJD, and 180,000 cattle have been diagnosed with BSE. March 15, 2004 USDA announces it will test at least 268,000 cattle a year. June 30, 2005 USDA confirms that it found a case of BSE in Texas the week before marking the first case of BSE from an animal that had lived its entire life in the U.S. March 13, 2006 USDA announces that on March 10 its inspectors found a cow with BSE in Alabama.

April 24, 2012 A routine inspection in California discovered a dairy cow with BSE. December 8, 2012 The Japanese government issued a ban on imports of raw beef from Brazil, based on reports that a cow which died in 2010 in southern Brazil carried disease-carrying proteins. December 20, 2012 USDA establishes rule requiring most livestock traveling across state lines to be tagged for traceability of Mad Cow disease. Cattle under 18 months are exempt, as are chicks moved across state lines directly from a hatchery. Mad Cow Disease and Your Pets | Little Big Cat Mad Cow Disease and Your Pets • Comments Off on Mad Cow Disease and Your Pets By Jean Hofve, DVM In December 23, 2003, the first official U.S. case of “mad cow disease” (Bovine Spongiform Encephalopathy or BSE) was reported in the news; a second followed shortly thereafter. However, astute researchers and even the pet food industry acknowledge that a BSE-like disease has been present in the U.S. for many years. Is this a threat to our pets?

Let’s look a little closer at this unusual disease. Around the end of 1985, the first cases of a bizarre bovine neurological disease, nicknamed “Mad Cow Disease,” were reported in England. British beef was quarantined and hundreds of thousands of cattle destroyed. As early as 1988, some experts thought that this disease might be transmissible to humans. However, a definitive link to a “new variant” of the human degenerative neurological disease, Creutzfeld-Jacob Disease (nvCJD), was not established until 1996. Consumption of infected beef meat was considered to be the cause. However, more recent research suggests it may not be the meat itself, but whole-body contamination with brain tissue during the slaughter process, which is to blame. The brain and spinal cord of infected animals carries a high concentration of infective particles, called “prions.” Captive bolt stunning, which is the usual way of knocking a cow unconscious just prior to slaughter, splatters brain matter into the bloodstream, where it is rapidly disseminated throughout the animal’s body, literally within a heartbeat.

This apparently happens faster than the animal can be hoisted and moved to the next stage of slaughter, where it is bled out, even though this normally occurs in less than 60 seconds. Subsequent removal of the brain and spinal cord may thus be inadequate to prevent infected material from directly contaminating edible tissues. Cattle presumably acquired BSE from eating food containing rendered material from sheep infected with scrapie, a common ovine disease. This feeding practice was banned in the U.K. about 18 months after the first BSE cases came to light. One European country after another subsequently discovered BSE within its borders. France, Ireland, Portugal, Switzerland, the Netherlands, Belgium and Denmark all reported cases of BSE in the 1990s. It has also leaped to far-off places as Japan, the Falkland Islands, the Azores, Oman, and Canada. In 2000, BSE was discovered in Germany, much to the consternation of the ministers who had prided themselves on strong regulations intended to keep the fearsome disease at bay.

One report stated that Italy and Spain are “likely to have been infected,” and that BSE “cannot be ruled out in four other European countries, as well as Australia, Canada, and the USA.” There have been some 180,000 infected cows identified worldwide since the outbreak began in Britain. More than 100 people have died from nvCJD related to BSE. In addition to human victims, more than 100 domestic and big cats, as well as several ruminant species in zoos, have been affected. In 1993 the epidemic reached its peak as the 100,000th BSE case was identified. Wrangling over which beef or sheep products could be used for what, and where and how they could or could not be exported, continued throughout the 1990s and into the new millennium. Until the end of 2003, the U.S. government kept itself busy congratulating itself on preventing such transmission in this country by virtue of its “active surveillance program.” However, it was never certain that we didn’t have BSE in American livestock. The U.S.

banned importation of British-origin beef in 1985 for other reasons, but by then the disease was entrenched in British cattle, and possibly present in imports prior to that time. About 500 live British cattle were imported into the U.S. between 1981 and 1989, when the USDA banned the importation of live cattle. While most of these cattle were traced and located, 34 could not be found. These were presumed dead, and most of the rest of the other cattle were also known to have died or been slaughtered. Of most interest, perhaps, is the disposition of the carcasses, but this is not known in all cases. It is possible, if not likely, that at least some of these animals were rendered. If any of them were infected, it is also possible that they may have become “downers,” and routed into the human and/or animal food chains. A similar scenario was blamed for several outbreaks of a BSE-like disease among mink in fur farms. We also have sheep scrapie in both the U.S. and Canada, which was recognized in the 1940s.

But not until 1989 did the American rendering industry initiate a “voluntary ban” on accepting potentially infected sheep parts for processing. And even then, a USDA survey three years later found that 6 of 11 inspected plants were still accepting the banned parts. The feeding of sheep and other ruminants back to ruminants was not officially banned in the U.S. until October 1997. In July 2000, a “foreign” spongiform encephalopathy was found in four imported sheep in Vermont. This prompted the USDA to declare an “extraordinary emergency.” More than 700 animals in three flocks of milking sheep were quarantined as a result. In March 2001, two flocks were seized for testing and destruction. USDA assumed the responsibility for ensuring that, as these sheep are destroyed, they do not enter the human or ruminant food chain; but there is nothing to prevent them being routed into pet food. In early 2001, USDA announced that inspections of feed mills and rendering facilities found almost 800 lacking required warning labels and/or systems in place to prevent mixing of banned products into ruminant feeds.

Thousands of facilities had yet to be inspected. In fact, USDA apparently has only a vague idea of how many such facilities actually exist. Just two weeks after that announcement, 1,200 cattle in Texas were quarantined because they may have been fed banned materials. Is BSE a threat to our companion animals? One industry analyst suggested early on that potentially infected materials should be excluded from cat foods, since cats were susceptible to the disease in Europe (about 100 domestic and exotic cats died from TSEs). Food-producing animals that die from unknown causes (which may include BSE or similar diseases) are typically routed into pet food and animal feeds. The presumed agent of transmission of BSE is not inactivated by rendering or most other types of processing, including cooking, formaldehyde, dry heat, autoclaving, or even incineration. There are a handful of methods that are known to destroy the prion, but they are highly impractical for most uses. BSE-like diseases (transmissible spongiform encephalopathies, or TSEs) have been present in the U.S. for decades. Chronic wasting disease of elk and deer is well characterized as a prion disease.

Sheep scrapie has been recognized since the 1940s in the US and Canada. Feeding of scrapie-infected sheep was blamed for the presence of a TSE disease among mink farms as early as 1947. However, in at least two instances, affected mink had not been fed sheep. A variant that caused an outbreak on two fur-farms in Wisconsin in 1985, where mink had been fed exclusively downer dairy cows, was shown to cause BSE lesions in experimentally infected cattle. Significantly, the infected steers did not develop symptoms of “madness” like British cattle, but instead became ataxic and went down. “What if the strain of BSE in American cattle produces more of a ‘downer cow syndrome’ than mad cow syndrome?” speculated the researchers. “This possibility would definitely complicate surveillance programmes for BSE in the United States, a country that has hundreds of thousands of downer cows each year and no means for routinely diagnosing their cause.” And in fact this exact scenario has played out in the U.S. Downer cows in the U.S.

were, until 2004, still slaughtered for human consumption; and to this day they are commonly used in pet food. Moreover, with non-ruminant proteins in high demand for ruminant feeds, ruminant proteins are now more likely to be processed into pet foods. Interestingly, although many species have been affected, cats seem to be especially susceptible. No canine cases were ever confirmed in the U.K., and it was presumed that dogs were somehow resistant. However, in 1997 a golden retriever who died in Norway had brain lesions consistent with a TSE-like disease, allegedly from dog food it consumed in the 1980s. Furthermore, a disease called “hound ataxia” has affected U.K. hunting dogs since the 1930s. Hunting hounds are commonly fed offal from downer cattle and sheep. Research in the early 1990s showed that microscopically, this disease shares pathological features with scrapie, and at the electron microscopic level, Scrapie Associated Fibrils (SAF) were detected. These facts imply the presence of BSE many years before its official debut in 1986.

However, both the initial study and further research to explore this possibility have been ignored or suppressed by the British government. The U.S. and Canada long believed they were free of bovine spongiform encephelopathy (mad cow disease). However, a significant portion of this confidence was based on the fact that BSE had not been found in any tested animals, including downer cows at slaughterhouses. Of the 37 million cattle slaughtered for meat each year in the U.S., about half a million cattle are downers. Canada tests fewer than 1,000 cattle a year. The U.S. currently tests about 7,500 downers per year (about 0.15%). Switzerland tested 20,000 cattle in a single year — and found five with BSE. Despite the lack of vigilance and appallingly inadequate testing, confirmed BSE cases were finally found in both Canada and the U.S. in 2003. One can only imagine how many cases continue to go unnoticed. UPDATE: 4/27/05 Whistleblowers claim Mad Cow cover-up by USDA (from Organic Consumers Association ).

“The USDA has been covering up cases of Mad Cow Disease, according to a USDA veterinarian in charge of monitoring cattle for the fatal disease. On the eve of retirement, Dr. Masua Doi confessed to sketchy testing since 1997. “I don’t want to carry on off to my retirement,” said Doi. “I want to hand it over to someone to continue, to find out. I think it’s very, very important. How many did we miss?” Doi’s concerns are shared by other USDA contracted scientists, like Dr. Karl Langheindrich who runs a test lab in Georgia and says the appropriate animal parts are not sent to him for accurate testing. In addition, two weeks ago, U.S. agriculture inspector Lester Friedlander offered testimony to a Canadian House of Commons committee revealing the U.S. has been covering up Mad Cow cases.” What can you do to prevent this disease in your home? The main consideration is to not buy commercial beef, particularly ground beef (which is plagued with many other contamination problems as well). However, organically grown beef from long-established herds, who have never been fed animal proteins, are most likely safe for human and feline consumption.

Commercial pet foods containing beef (and possibly lamb) may or may not be safe; they are probably best avoided. Pork is still considered safe, primarily because most hogs are slaughtered young; lambs, too, are slaughtered by 1 year of age–before they have time to develop the disease. Because birds’ metabolism is so different from mammals, poultry is still a safe choice, and game meats (except deer and elk, which can harbor a related prion disease), rabbits, and other non-commercially raised mammals are unlikely to harbor BSE. UPDATE: 10/10/11 New research from the University of Texas suggests at least some cases of “sporadic” Alzheimer’s disease “arise from an infectious process,” likely related to BSE. This may confirm the suspicions of those who believe that many (if not most) cases of Alzheimer’s are in fact a prion disease.

Consider this report published in 2004 (Could Mad Cow Disease Already be Killing Thousands of Americans Every Year?): The most frequent misdiagnosis of CJD among the elderly is Alzheimer’s disease.[55] Neither CJD nor Alzheimer’s can be conclusively diagnosed without a brain biopsy,[56] and the symptoms and pathology of both diseases overlap. There can be spongy changes in Alzheimer’s, for example, and senile Alzheimer’s plaques in CJD. Stanley Prusiner, the scientist who won the Nobel Prize for his discovery of prions, speculates that Alzheimer’s may even turn out to be a prion disease as well. In younger victims, CJD is more often misdiagnosed as multiple sclerosis or as a severe viral infection…Nobel Laureate [D. Carleton]Gajdusek, for example, estimates that 1% of people showing up in Alzheimer clinics actually have CJD. At Yale, out of a series of 46 patients clinically diagnosed with Alzheimer’s, six were proven to have CJD at autopsy.

Bovine Spongiform Encephalopathy (BSE) Questions and Answers Bovine Spongiform Encephalopathy (BSE) Questions and Answers Print What is BSE? BSE (bovine spongiform encephalopathy) is a progressive neurological disorder of cattle; its symptoms are similar to a disease of sheep, called scrapie. BSE has been called "mad cow disease." BSE and scrapie both result from infection with a very unusual infectious agent. As of January 2004, more than 180,000 cases of BSE were confirmed in Great Britain in more than 35,000 herds of cattle. The epidemic peaked in January 1993 at almost 1,000 new cases per week. Although the origin of the disease is uncertain it may have resulted from the feeding of scrapie containing meat and bone meal (MBM) to cattle or from feeding cattle MBM derived from a cow or other animal that developed the disease due to a spontaneous mutation. There is strong evidence and general agreement that the outbreak was amplified by feeding meat-and-bone meal prepared from cattle to young calves. What causes BSE?

The nature of the infectious agent that causes BSE and scrapie is unknown. Currently, the most accepted theory is that the agent is a modified form of a normal cell protein known as a prion. A prion is not a bacterium, parasite, or virus, and thus treatments usually used for treating or preventing bacterial infections (e.g. antibiotics) or viral infections are not effective against prions. Where is the BSE agent found in cattle? In cattle naturally infected with BSE, the BSE agent has been found in brain tissue, in the spinal cord, and in the retina of the eye. Additional experimental studies suggest that the BSE agent may also be present in the small intestine, tonsil, bone marrow, and dorsal root ganglia (lying along the vertebral column). Which countries have reported BSE? The vast majority of cases of BSE (more than 97% as of 2003) have been reported from the United Kingdom during an epidemic. However, endemic cases have also been reported in other European countries including: the Republic of Ireland, Switzerland, France, Liechtenstein, Luxembourg, Netherlands, Portugal and Denmark.

The numbers of reported cases by country are available on the web site of the Office International des Epizooties. These numbers should be interpreted with caution, however, because the intensity and methods of surveillance probably vary over time and by country. In 2003 one case was reported in Canada and one in the United States (in a cow born in Canada). How was BSE spread? It is thought that BSE was spread via meat-and-bone meal fed to cattle. The practice of using this material as a source of protein in cattle feed has been common for several decades. In the late 1970s there was a change in the production (rendering) process used to make this meat and bone meal. One hypothesis has been that this change permitted the infectious agent of scrapie (a transmissible spongiform encephalopathy, or TSE, of sheep) to survive the rendering process, and get transmitted to other animals, such as cows, that are fed meat-and-bone meal nutritional supplements. An inquiry by the British government has however, concluded that scrapie infected MBM was not the source of BSE nor was the change in the rendering practices responsible for survival of the BSE agent.

Rather, this inquiry has stated that BSE may have originated spontaneously as a result of a genetic mutation and was amplified by the feeding of contaminated MBM to cattle. What has the British government done in response to the BSE epidemic? In response to the BSE epidemic, the British Government instituted a series of measures to minimize the risk of disease transmission among both animals and humans. These included a ban on feeding ruminant protein (ruminants are animals, such as cows, sheep and goats) to ruminants (1988), removal of some "high risk" materials (such as brain, spinal cord and intestines) from cattle at slaughter (1989 and 1995), and a ban on cattle over 30 months of age from being used for food (1996). Following institution of these measures, Great Britain has seen a decrease in the number of cattle with BSE from a peak incidence of 36,682 confirmed cases in 1992 to 1044 confirmed in 2002. Does BSE occur in the US?

According to the Animal Health and Plant Inspection Service (APHIS) of the United States Department of Agriculture, BSE has been detected in one cow in the United States. Following complications of pregnancy this cow was slaughtered December 9, 2003. As part of the APHIS ongoing surveillance of downer animals a brain sample was taken to test for the BSE agent. On December 25, 2003 it was confirmed that this animal tested positive for the BSE agent (on December 23, 2003 this was a "presumptive" case). Following this discovery the USDA and FDA announced additional measures to enhance the US protections against BSE (see: What measures has the US government taken to ensure that people are not exposed to the BSE agent in foods?). What measures has the US government taken to ensure that people are not exposed to the BSE agent in foods? The USDA is responsible for the health of US livestock. To prevent BSE from entering the country, the USDA Animal and Plant Health Inspection Service (APHIS) has, since 1989, prohibited the importation of live ruminants from countries where BSE is known to exist in native cattle.

On December 12, 1997, APHIS stopped the importation of live ruminants and most ruminant products, including meat, meat-and-bone meal, offals, glands, etc. from all of Europe. FDA is responsible for animal feeds in the US. In August 1997, FDA prohibited the use of most mammalian protein in the manufacture of animal feeds given to ruminants. Following the discovery of one cow with BSE in the US, the USDA and FDA have announced additional measures to enhance protections against the spread of BSE in US cattle and to minimize human exposure to bovine materials that may contain the BSE agent. USDA has issued an interim final rule (Federal Register January 12, 2004 Vol.

69, Number 7) removing downer animals and specified risk materials and tissues from the human food chain; requiring additional process controls for establishments using advanced meat recovery (AMR); holding meat from cattle that have been targeted for BSE surveillance testing until the test has confirmed negative; and prohibiting the air injection stunning of cattle ( In January 2004, FDA proposed additional safeguards including: excluding brain, spinal cord, gut and eyes of older animals from human food and from rendered material in animal feeds, eliminating poultry litter, cow blood and processed plate waste as feed ingredients for cattle, labeling requirements for pet food, and additional control measures to prevent cross contamination of feed and feed ingredients at feed mills. In addition, since 1990, the USDA has led an interagency surveillance program for evidence of BSE in the US. USDA has tested 20,000 animals annually for each of the last 2 years, and approximately 75 percent of these were downers at slaughter.

A BSE risk assessment performed by Harvard University's Center for Risk Analysis at the School of Public Health concluded that even if BSE were to occur in the US the measures already taken would largely prevent its spread to animals or humans, and the disease would gradually disappear over a number of years. How is the BSE agent detected? The presence of the BSE agent in tissues is generally determined by injecting animals, usually mice, with samples, then observing the mice to see if they die and have characteristic brain tissue changes. Mouse inoculation studies take a long time (up to 700 days) to detect the agent, and a negative result (that is, lack of brain tissue changes in the injected mice) may only mean that there was too little of the infectious agent to cause symptoms, not that the material was completely free of the infectious agent. It is also possible to detect the presence of the abnormal prion protein in tissues (such as brain) using special staining procedures although these methods do not allow an accurate assessment of infectivity of the infected material. Does BSE or a similar disease occur in humans? BSE belongs to a group of progressive degenerative neurological diseases known as transmissible spongiform encephalopathies (TSEs).

TSE diseases are always fatal. The TSE diseases include scrapie, which affects sheep and goats; transmissible mink encephalopathy; feline (cat) spongiform encephalopathy; and chronic wasting disease of deer and elk. There are six TSE diseases that affect people: kuru, classical Creutzfeldt-Jakob disease (CJD) and variant Creutzfeldt-Jakob disease (vCJD), Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, and sporadic fatal insomnia. The human diseases are very rare; for example, classical CJD has been well studied and occurs sporadically worldwide at a rate of about one case per one million people each year. Questions and Answers on Variant CJD (vCJD) What is the new variant form of CJD that the experts in the United Kingdom believe might be related to the BSE outbreak in cattle? In contrast to the classic form of CJD, the new variant or variant form (vCJD) in the United Kingdom and France affects younger persons (average age at onset: 26 years), and has different clinical features from CJD.

People with vCJD begin with serious psychiatric problems or problems with their senses (ears, eyes or smell). This first set of symptoms is followed weeks or months later by poor muscle coordination, muscle spasms, and mental confusion. The illness lasts for at least 6 months, and on average people with vCJD die approximately 13 months after their symptoms begin. When patients' brains are examined by autopsy, there are clear changes in brain tissue structure, including many "spongiform," or open spongy-looking areas, abnormal clumps of prion protein called plaques, and other areas with less prominent accumulations of abnormal prion protein. Exactly how does this newly recognized variant of CJD differ from classical CJD? In 1996 the Spongiform Encephalopathy Advisory Committee (SEAC) of the UK announced the identification of 10 cases of variant CJD (vCJD, Lancet, 1996, 347: 921-25). Since then over 100 people have died of vCJD in the UK.

The following features describe how vCJD cases differ from the sporadic or classical form of CJD (Lancet, 1996, 347: 921-25, Rev.Med.Virol. 2002;12: 143-50): The affected individuals were much younger than the classical CJD patient. Typically, CJD patients are over 63 years old. The average patient with vCJD is 26 years old; patients ranged from 12-74 years old. The course of vCJD averaged 13 months (range 6-40 months). Classical CJD cases average a 4 month duration. In the vCJD cases, electroencephalographic (EEG) electrical activity in the brain, while abnormal (slowed), was not typical of classical CJD, which often has periodic bursts of increased electrical activity. Although changes in brain tissue structure of patients with vCJD were recognizable as CJD, the pattern was different from classical CJD, with large aggregates of prion protein plaques often surrounded by vacuoles. How did people get this new variant of CJD?

On March 20, 1996 a statement from the Spongiform Encephalopathy Advisory Committee (SEAC) of the United Kingdom indicated concern that before November 1989, when inclusion of certain cow and sheep by-products in human food was banned, the BSE agent may have been transmitted to people through contaminated food products. The SEAC said that food might account for the 10 vCJD cases described in 1996 (Lancet 1996; 347:921-5). The specific foods, if any that may be associated with the transmission of this agent from cattle to humans are unknown. However, the SEAC has indicated that milk and milk products are unlikely to pose any risk for human exposure to the BSE agent. What is the evidence directly linking this newly recognized variant of CJD to BSE exposure? There is strong epidemiologic and laboratory evidence suggesting that new variant CJD (vCJD) and BSE are caused by the same infectious agent. For instance, all cases of confirmed vCJD have occurred in people who have lived in geographic areas which have had BSE cases.

The majority of cases of vCJD have occurred in the UK, which has had the largest number of cases of BSE in cattle. In addition, the time interval or "incubation period" between the most likely period for the initial exposure of the population to potentially BSE-contaminated food (1984-1986) and onset of initial vCJD cases (1994-1996), about 10 years, is similar to the known time intervals between exposure to the classical CJD agent and the development of CJD. An experimental study reported in June 1996, showed that three cynomolgus macaque monkeys that were injected with brain tissue from cattle with BSE later developed symptoms and changes in brain tissue that were strikingly similar to vCJD (Nature 1996;381:743-4).

Another study published in 1996 showed that prion proteins obtained from 10 vCJD patients and BSE-infected animals had molecular characteristics that were similar to each other but distinct from prion proteins obtained from patients with classical CJD (Nature 1996;383:685-90). Furthermore, results of an ongoing experimental study involving injection of a panel of various strains of mice with the agent that causes BSE and vCJD suggested that these agents cause a similar disease in mice (Nature 1997;389:498-501). Histological analysis of mouse brains from this study showed no significant differences in the neuropathological changes observed in the BSE and vCJD-infected mice (Neuropathology and Applied Neurobiology 2003; 29:262-272). A study using transgenic mice (PNAS 1999; 96:15137-15242) also supports the hypothesis that the BSE agent from cattle causes vCJD. How many cases of variant CJD have occurred?

Cases of variant CJD are very rare, and most have occurred in the United Kingdom. Information provided by the UK Creutzfeldt-Jakob Disease Surveillance Unit (February, 2004) indicates that there have been 146 cases of definite and probable vCJD in the United Kingdom. These cases have all been diagnosed since 1995. France has reported six cases. The Republic of Ireland, Italy, Canada and the US have each reported one case. The cases in Ireland, Canada and the US each occurred in individuals who had spent several years in the UK. Could anyone in Europe diagnosed with the newly recognized variant of CJD (vCJD) have acquired this from vaccines? In the UK the majority of cases of vCJD were born before 1980, and it is very unlikely that they received vaccines contaminated with the BSE agent (Vaccine 2000; 19:409-410).

Surveillance of vCJD in the UK has identified three "risk factors," or characteristics common to most if not all of the people who had vCJD: i) residence in the UK; ii) a particular genetic susceptibility (met/met homozygosity at codon 129 of the PrP gene); and iii) age. Epidemiological evidence to date suggests that these cases of vCJD acquired the disease from eating beef products containing the BSE agent after 1980. To date (February 2004) there have been 156 reported cases of vCJD. Of these, 146 have occurred in the UK. Six cases of vCJD have been diagnosed in France, and one each in Canada, Ireland, Italy and the US. The cases in Canada, Ireland and the US each occurred in individuals who had spent several years in the UK. Related Links Cannibalism holdswarning of mad cow: By eating their dead, a remote Pacific tribe hoped to become immortal. Instead it cursed the living with a horrifying brain disease. Cannibalism holdswarning of mad cow: By eating their dead, a remote Pacific tribe hoped to become immortal.

Instead it cursed the living with a horrifying brain disease. June 3, 2001 The Ottawa Citizen by Mark Kennedy For generations, the Fore tribe in the remote mountains of Papua New Guinea held lavish mortuary feasts to commemorate the death of one of their relatives. But it was a feast with a twist: They ate the deceased. The ritual would begin as the Fore women scooped the brains from the skull of the cadaver. They then wiped the liquid and tissue all over their own bodies, leaving it in their hair and skin for weeks. Great fires were built to cook the body. The men of the villages -- who were accorded a higher status than women -- ate the best meat, the muscle. But there was plenty left over. Nothing was thrown away. The women and young children ate the brain and offal. The organs, even the feces, were all eaten. The bones were baked for weeks, hammered to a fine dust and sprinkled over vegetables. Everyone was eaten; only those with diseases like leprosy or dysentery were shunned. The dead lived on forever, quite literally consumed by their loving relatives, who themselves would later be eaten by the tribe.

It was a cannibalistic ritual born out of love and respect. But it spawned a killer disease -- called kuru -- that nearly brought the tribe to the brink of extinction. At some point, probably around 1915, someone in the tribe had developed a genetic mutation in their body. Their brain was rapidly wasted away by tiny, sponge-like holes. They died, and nobody in the Stone-Age tribe knew why. Nor could they know that in eating the remains of their tribe member, they were becoming infected with the same neurological disorder -- scientists now believe it was a random case of Creutzfeldt-Jakob Disease (vCJD). [CJD, not vCJD-- BSE coordinator ] But gradually, others began dying from the strange illness they called kuru, the "trembling" or "shivering" disease. Women were particularly vulnerable. Nobody knew why until scientists later figured it out: The women were eating the most infectious parts of a dead person's contaminated body -- the brain and nervous tissue. Before long, it was feared there wouldn't be enough of them left for the tribe to reproduce (men outnumbered women three-to-one in some villages).

In the late 1950s, the disease was killing some 200 people a year out of the 15,000-member tribe. By 1960, Westerners who had come to populate the island put a stop to the cannibalistic ritual they found so abhorrent. Within a few years, the number of Fore with kuru began diminishing slowly. Four decades later, the story of kuru is one that could hold the secret to many mysteries surrounding a more pressing public health scare, commonly known as mad-cow disease, which some fear could become a worldwide epidemic. Kuru foreshadowed the mad-cow disaster that killed at least 180,000 British cattle in the 1980s and '90s. It proved that, putting aside moral or philosophical arguments, cannibalism can be a disease's best friend. The Fore died of kuru because they ate the remains of other Fore who died of kuru. British cows died of bovine spongiform encephalopathy (BSE) because they were fed the protein-rich remains (known as meat and bonemeal) of other cows infected with BSE. Dr.

Michael Alpers, a recently retired scientist in Perth, Australia, is one of the world's foremost kuru experts. As director of the Papua New Guinea Institute of Medical Research from 1977 to last year, Dr. Alpers spent much of his career studying kuru. He was one of a handful of scientists who discovered the disease in the 1960s. Along with world-renowned scientist Dr. Carleton Gajdusek (who later won a Nobel Prize for his medical detective work on kuru), Dr. Alpers lived among the Fore in the early '60s. He watched their symptoms progress: the unsteadiness of gait, sometimes the uncontrolled laughter (early media reports dubbed it the "laughing death"), followed by an inability to walk, talk or eat."It's a terrible, slow death," says Dr. Alpers. "Eventually, the person just lies around the house in a moribund state and dies." Usually, death came within a year. But sometimes it could claim its victim within three months or the agony could last three years. Dr. Alpers and Dr.

Gajdusek collected the brains of some victims and sent them to a lab in the United States. In 1963, the infected brains were mashed in a blender and injected into the brains of three chimpanzees. Two years later, Dr. Alpers, who by then had moved to the U.S. to work in the lab, was the first scientist to witness a groundbreaking discovery. One of the chimps, named Georgette, began showing similar clinical symptoms to those among the Fore who died of kuru. Georgette shivered, her lip drooped, she constantly lost her balance and fell out of her cage, she grew steadily weaker. Eventually, she was euthanized and her brain was sent to London for analysis. The stunning telex that Dr. Alpers received: "PATHOLOGY OF GEORGETTE INDISTINGUISHABLE FROM HUMAN KURU." It was a remarkable discovery. The disease had crossed the species barrier, from humans to chimpanzees. Unfortunately, when BSE emerged among British cattle 20 years later, government scientists insisted there was no reason for alarm. That disease, they said, clearly posed a threat to cows.

But humans who ate contaminated beef, they said, were safe. BSE would not cross the species barrier. But it did. These days, Dr. Alpers doesn't skip a beat when asked to comment on another central issue about the mad-cow scandal: whether governments made a huge mistake in allowing cows to be fed to cows. "I think that's a valid view," he says. "If we used kuru as a model -- and I wasn't asked for my advice on this matter -- we could have said much earlier there are risks here." Dr. Alpers says kuru may hold the answer to another critical question now baffling the world's scientists. No one knows how any people may ultimately die from variant Creutzfeldt-Jakob Disease (vCJD), the strain of the neurological disease that humans got in recent years from eating BSE-contaminated beef. So far, 104 people have contracted the disease. Scientists disagree on how high the final toll will be. Part of the answer lies in how long vCJD incubates in humans before it emerges to kill all its victims, usually within 18 months. If the incubation period is 10 to 15 years, that's good news.

It means the cases of vCJD seen so far may represent a fair portion of the final tally. But if the disease has a much longer incubation period, it's bad news. It means the cases of vCJD that have emerged represent only the tip of an iceberg. The truth is, no one knows. No one's ever seen something quite like this before. The closest thing that comes to it is kuru. And with that disease, scientists have found a very long incubation. For the past 40 years, new cases have continued to emerge of kuru. To this day, scientists see one or two new cases a year of middle-aged Fore tribe members who would have been contaminated as children, before cannibalism was stopped. Dr. Alpers is not an optimist about the future numbers of vCJD victims. Like kuru, he suspects vCJD has an extended incubation period that could stretch for up to several decades. "If you use kuru as a model, this is what you can expect," says Dr. Alpers. "The picture is pretty bleak. It's not great news.

Everyone around the world is going to have to face this." GRAPHIC: Black & White Photo: A middle-aged female kuru victim. Women, were particularly vulnerable to kuru, the 'trembling' or 'shivering', disease.; Black & White Photo: Trevor Collens, The Ottawa Citizen ;, Australian medical researcher Michael Alpers was one of a handful of, scientists who discovered the kuru disease in the 1960s.

Question: What disease infected 180,000 British cows?
Answer: Mad Cow