Center for Food Safety | Issues |

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.

 
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[i] BBC News. “BSE Inquiry–The Final Stage: Chronology of Events.” http://news.bbc.co.uk/hi/english/static/special_report/
1999/06/99/bse_inquiry/default.stm

[ii] Rampton, Sheldon, and John Stauber.  Mad Cow U.S.A.–Could the Nightmare Happen Here? Maine: Common Courage Press, p. 13.

[iii] Reuters. “The spread of mad cow disease,” December 23, 2003. www.cnn.com/2003/HEALTH/12/23/madcow.chronology.reut

[iv] Reuters

[v] Reuters.

[vi] US Department of Agriculture.  “Veneman Announces Additional Protection Measures to Guard Against BSE,” December 30, 2003.   http://www.usda.gov/Newsroom/0449.03.html

[vii] U.S. Department of HHS. “Expanded ‘Mad Cow’ Safeguards Announced to Strengthen Existing Firewalls Against BSE Transmission,” January 26, 2004. http://www.cfsan.fda.gov/~lrd/hhsbse3.html

Mad Cow Disease Fast Facts

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.

Both mad cow disease and 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-August 2018: 26 cases confirmed, 20 in Canada, and six in the United States. One of the infected cows that died in the United States was born in Canada. One of the infected Canadian cows was imported from the United Kingdom.

vCJD Statistics (Humans)

(source: CDC)
Since 1996, 231 vCJD cases have been identified in 12 countries:
United Kingdom – 178
France – 27
Spain – 5
Ireland – 4
United States – 4
Italy – 3
The Netherlands – 3
Portugal – 2
Canada – 2
Japan – 1
Saudi Arabia – 1
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 vCJD is reported.

1996-1999 – The European Union bans British beef. France continues the ban for an additional three years.

May 20, 2003 – Canada’s first case of mad cow disease is confirmed in an 8-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.

December 23, 2003 – The US 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 US beef. The infected cow was born in 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.

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 US case of mad cow disease.

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 US 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 vCJD, 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 BSE in two years.

June 24, 2005 – The second US case of BSE is confirmed.

March 13, 2006 – The third US 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 – The US 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.

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.

June 11, 2014 – The USDA announces a recall of 4,000 pounds of beef; a spokesperson cites “an abundance of caution.” The meat comes out of the Fruitland American Meat processing plant in Jackson, Missouri.

March 24, 2016 – France confirms the first case of BSE since 2011.

October 18, 2018 – Scotland confirms an “isolated case” of mad cow disease has been discovered on a farm in Aberdeen.

All About BSE (Mad Cow Disease)

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The word BSE is short but it stands for a disease with a long name, bovine spongiform encephalopathy.  “Bovine” means that the disease affects cows, “spongiform” refers to the way the brain from a sick cow looks spongy under a microscope, and “encephalopathy” indicates that it is a disease of the brain. BSE is commonly called “mad cow disease.”

What is BSE?

BSE is a progressive neurologic disease of cows.   Progressive means that it gets worse over time.  Neurologic means that it damages a cow’s central nervous system (brain and spinal cord).

What Causes BSE?

Most scientists think that BSE is caused by a protein called a prion.  For reasons that are not completely understood, the normal prion protein changes into an abnormal prion protein that is harmful.  The body of a sick cow does not even know the abnormal prion is there.  Without knowing it is there, the cow’s body cannot fight off the disease.   

What are the Signs of BSE in Cows?

A common sign of BSE in cows is incoordination. A sick cow has trouble walking and getting up.  A sick cow may also act very nervous or violent, which is why BSE is often called “mad cow disease.”    

It usually takes four to six years from the time a cow is infected with the abnormal prion to when it first shows symptoms of BSE.  This is called the incubation period.  During the incubation period, there is no way to tell that a cow has BSE by looking at it.  Once a cow starts to show symptoms, it gets sicker and sicker until it dies, usually within two weeks to six months.  There is no treatment for BSE and no vaccine to prevent it. 

Currently, there is no reliable way to test for BSE in a live cow.  After a cow dies, scientists can tell if it had BSE by looking at its brain tissue under a microscope and seeing the spongy appearance.  Scientists can also tell if a cow had BSE by using test kits that can detect the abnormal prion in the brain.

Brain from a healthy cow, as seen under a microscope using special stains.

Photo courtesy of Dr. Katie Kelly, Johns Hopkins University

Brain from a cow sick with BSE, as seen under a microscope using special stains. The large white spaces are like the “holes” of a sponge.

Photo courtesy of the late Dr. Al Jenny, USDA

How Does a Cow Get BSE?

The parts of a cow that are not eaten by people are cooked, dried, and ground into a powder. The powder is then used for a variety of purposes, including as an ingredient in animal feed. A cow gets BSE by eating feed contaminated with parts that came from another cow that was sick with BSE. The contaminated feed contains the abnormal prion, and a cow becomes infected with the abnormal prion when it eats the feed.  If a cow gets BSE, it most likely ate the contaminated feed during its first year of life.  Remember, if a cow becomes infected with the abnormal prion when it is one-year-old, it usually will not show signs of BSE until it is five-years-old or older. 

Can People Get BSE?

People can get a version of BSE called variant Creutzfeldt-Jakob disease (vCJD).  As of 2019, 232 people worldwide are known to have become sick with vCJD, and unfortunately, they all have died. It is thought that they got the disease from eating food made from cows sick with BSE. Most of the people who have become sick with vCJD lived in the United Kingdom at some point in their lives. Only four lived in the U.S., and most likely, these four people became infected when they were living or traveling overseas.

Neither vCJD nor BSE is contagious. This means that it is not like catching a cold.  A person (or a cow) cannot catch it from being near a sick person or cow.  Also, research studies have shown that people cannot get BSE from drinking milk or eating dairy products, even if the milk came from a sick cow.

What is the FDA Doing to Keep Your Food Safe?

The U.S. Food and Drug Administration (FDA) is doing many things to keep the food in the U.S. safe for both people and cows.  Since August 1997, the FDA has not allowed most parts from cows and certain other animals to be used to make food that is fed to cows.  This protects healthy cows from getting BSE by making sure that the food they eat is not contaminated with the abnormal prion. 

In April 2009, the FDA took additional steps to make sure the food in the U. S. stays safe.  Certain high-risk cow parts are not allowed to be used to make any animal feed, including pet food.  This prevents all animal feed from being accidentally contaminated with the abnormal prion.  High-risk cow parts are those parts of the cow that have the highest chance of being infected with the abnormal prion, such as the brains and spinal cords from cows that are 30 months of age or older.  

By keeping the food that is fed to cows safe, the FDA is protecting people by making sure that the food they eat comes from healthy cows.

The FDA also works with the U.S. Department of Agriculture (USDA) to keep cows in the U.S. healthy and free of BSE. The USDA prevents high-risk cows and cow products from entering the U.S. from other countries.  The USDA also makes sure that high-risk cow parts, such as the brains and spinal cords, and cows that are unable to walk or that show other signs of disease are not used to make food for people.

The steps the FDA and USDA have taken to prevent cows in the U.S. from getting BSE are working very well.  Only six cows with BSE have been found in the U.S. The first case was reported in 2003 and the most recent case was found in August 2018.

It is worth noting that there are two types of BSE, classical and atypical. Classical is caused by contaminated feed fed to cows. Atypical is rarer and happens spontaneously, usually in cows 8-years-old or older. Of the six U.S. cows found with BSE, five were atypical. The only case of classical BSE in the U.S. was the first one, in 2003, in a cow imported from Canada.

Can Other Animals Get BSE?

Sheep, goats, mink, deer, and elk can get sick with their own versions of BSE.  Cats are the only common household pet known to have a version of BSE.  It is called feline spongiform encephalopathy, and the same things that are being done to protect people and cows are also protecting cats. No cat in the U.S. has ever been found to have this disease.

How Can I Get More Information?

Mad Cow Disease in Humans Symptoms, Causes, Treatment

What Is the History of Where Mad Cow Disease Came From?

In December 2003, the first case of BSE in the United States was detected in a dairy cow in the state of Washington. Two further cases were reported in 2005 and 2006. Before that, a devastating major outbreak occurred in the United Kingdom (England and Ireland) in the 1980s, peaking in 1993. Because the suspected cause was a prion transmitted in meat and bone meal products fed to cattle there, the government banned the practice of feeding such products that may contain diseased tissue to animals in 1988. By then, however, infected cattle had already entered the human food supply. At the peak of this outbreak in 1993, almost 1,000 cases per week of infected cattle were reported; the numbers have dropped dramatically since then, and currently there are only about 10 infected cattle identified each year in the U.K. The BSE-related condition vCJD was first described in the U.K. in 1996. By 1996, several people in the U.K. and others who had lived there were identified with a variant form of CJD (vCJD), and the cause was linked to eating meat from cattle infected with mad cow disease. In June 2014, over 4,000 pounds of beef were recalled by the U.S. Department of Agriculture due to improper processing that allowed cattle brain material (dorsal root ganglia) to be mixed with processed beef.

BSE and resulting cases of vCJD in humans have been diagnosed in other European countries such as Bosnia-Herzegovina, Liechtenstein, Macedonia, Norway, Sweden, and Yugoslavia. According to the World Health Organization (WHO), over 220 cases of vCJD have been reported worldwide, with the majority occurring in the UK (177 cases) and in France (27 cases). Only four cases have been reported in the U.S., and in all four of these cases, there is evidence that indicates that the infection was acquired while abroad in Europe or the Middle East.

Because there is no way to detect BSE in blood, people who have lived for long periods in areas where mad cow disease has been found are not allowed to donate blood in the U.S.

Prion diseases are also known as transmissible spongiform encephaolpathies (TSEs). Overall, prion diseases are a large group of related conditions affecting the nervous system, which affect both animals and humans. Included are Creutzfeldt-Jakob disease (CJD) and variant CJD discussed in detail here in relation to bovine spongiform encephalopathy (BSE, mad cow disease). Another human prion disease is Gerstmann-Sträussler-Scheinker (GSS) disease (see causes below). In animals, chronic wasting disease (CWD) is found in mule deer and elk in the U.S., and scrapie is a similar condition found in sheep. Cases have been reported in the U.S. These diseases all take a long time to develop but are typically rapidly progressive once symptoms begin.

All prion diseases are fatal. Animals and humans who develop a prion disease will die of it. There is no effective treatment. It is important to understand how these diseases are transmitted in order to prevent their spread.

Scientists reveal the possible origin of the “mad cow” disease

· A team of scientists demonstrates that bovine spongiform encephalopathy (BSE) could have originated from atypical scrapie prions, a neurodegenerative disease that appears spontaneously in sheep and goats.

· These infectious agents would have been transmitted to the cows when they were fed with meat meal and sheep bones infected with prions, a practice currently prohibited.

· 226 people suffered the variant of Creutzfeldt-Jackob disease after consuming beef contaminated with prions.

In the late 1990s, Europe suffered one of the worst food crises due to the disease of “crazy cows.” In the United Kingdom, about 200,000 cows presented symptoms such as tremor, changes in behavior and loss of balance, until death. In Spain, they were about a thousand. Some of the people who ingested products derived from “mad cow” suffered the variant of Creutzfeldt-Jackob disease, a neurodegenerative pathology without cure. The number of people affected worldwide by this disease was 226, five of which from Spain. The hypothesis that was raised was that the first cows had become ill because they had consumed feed made from sheepmeat and meat meal contaminated with classic scrapie prions, a fatal degenerative disease that affects the nervous system of sheep and goats and contagious among animals of the same flock. Until today, several studies have ruled out this initial hypothesis.

Now, a study led by the ENVT-INRA center of Tolouse, the Animal Health Research Center (IRTA-CReSA) and the Animal Health Research Center (INIA-CISA), with the participation of the Autonomous University of Barcelona (UAB) and of the University of Zaragoza (UNIZAR), reveals that the infectious agent responsible for bovine spongiform encephalopathy or “mad cow” disease could come from atypical scrapie, another type of prion involvement of sheep and goats. This prion disease was discovered in 2003 and unlike the classic scrapie appears spontaneously, in isolated cases and does not spread among the herd.

The study’s experiments, published in the Proceedings of the National Academy of Sciences of the United States of America (PNAS) journal, demonstrate for the first time that when atypical scrapie prions are transmitted from one species to another they change structure and their ability to infect evolves. “It is a relevant result because it gives us more clues about how a prion disease of sheep would have passed to cows and then to people, becoming a zoonotic disease,” says Enric Vidal, veterinarian and researcher of IRTA-CReSA.

PRIOCAT laboratory. /IRTA-CReSA (CC BY-NC 2.0)

Prions are proteins found in the body of animals and people naturally, mainly in the brain. The problem is that when they change their structure and cause other proteins to do so, they can become neurodegenerative. “This change of structure can be due to genetic causes, it can also happen spontaneously or by ingesting contaminated proteins. On the other hand, there have been some cases of accidental inoculation by manipulation of work tools, for example, in the laboratory or in animal surgeries”, explains Martí Pumarola, professor of the Department of Medicine and Animal Surgery of the Faculty of Veterinary Medicine of the UAB . Currently, there are still cases of people with Creutzfeldt-Jackob disease – different from the variant caused by crazy cows – as well as animals sick with prions spontaneously. These cases are no longer due to the intake of contaminated food because since the crisis of the “mad cows” the use of feed made from ruminant protein flours was prohibited.

So far there is no treatment or vaccines for human or animal prion diseases, but the study authors say that this finding will improve the risk assessment of these diseases in the field of animal health and public health. “Now we have verified that the atypical scrapie is capable of giving rise to bovine spongiform encephalopathy in cows, which we did not know until now. While the prohibition of these feeds made with sheep derivatives remains active we will not have new cases of” crazy cow “because intake,” explains Enric Vidal.

A decade of study with international collaboration

Experiments have been performed with transgenic mouse models – mice with cow genes – to see how prions of atypical scrapie affect them. The prions used in the study came from real cases diagnosed in sheep from Spain, France, Portugal and Norway. The tests have been carried out thanks to the PRIOCAT laboratory of the Biocontainment Unit of the IRTA-CReSA center. This biosafety level 3 infrastructure forms the Network of Hight Biosafety Laboratories (RLASB), a Singular Scientific and Technical Infrastructure of Spain (ICTS). PRIOCAT carries out the diagnosis of animal prion diseases of Catalonia within the program of control and eradication of transmissible spongiform encephalopathies (EETs), commissioned by the Department of Agriculture, Livestock, Fisheries and Food and the Department of Health of the Government of Catalonia.

The study has had the collaboration of other research centers in Norway, Portugal and the United Kingdom. It has had European FEDER funds from the INTERREG-POCTEFA cross-border program through the COTS project, with COTSA and Transprion, which currently make up the RedPRION network (EFA148/16), a group of different research groups for these diseases in Catalonia, Aragon, the Basque Country and southern France. It has also had funds from the La Marató de TV3 Foundation (Project 201821-30-31-32 Evaluation of the public health risk of atypical and emerging prions) of the edition of Infectious Diseases, in this project the zoonotic potential of ‘these sheep prions passed through the bovine species is being assessed by bioassays in humanized transgenic mice.

Article de referència: Alvina Huor*, Juan Carlos Espinosa*, Enric Vidal*, Hervé Cassard, Jean-Yves Douet, Séverine Lugan, Naima Aron, Alba Marín-Moreno, Patricia Lorenzo, Patricia Aguilar-Calvo, Juan Badiola, Rosa Bolea, Martí Pumarola, Sylvie L. Benestad, Leonore Orge, Alana M. Thackray, Raymond Bujdoso, Juan Maria Torres, and Olivier Andréoletti (2019). The emergence of classical BSE from atypical/Nor98 scrapie. Proceedings of the National Academy of Sciences of the United States of America (PNAS).

2. Mad-cow history

Symptoms, Causes and Treatments for vCJD

Mad cow disease has hit the U.S. and questions about this mysterious disease abound. Here’s what you need to know about mad cow disease.

What Is Mad Cow Disease?

Mad cow disease, or bovine spongiform encephalopathy (BSE), is a transmissible, slowly progressive, degenerative, and fatal disease affecting the central nervous system of adult cattle. The U.S. Department of Agriculture (USDA) has tested hundreds of thousands of cattle for BSE.

Researchers believe that the infectious agent that causes mad cow disease is an abnormal version of a protein normally found on cell surfaces, called a prion. For reasons still unknown, this protein becomes altered and destroys nervous system tissue — the brain and spinal cord.

Does Cooking Food Kill the Prion That Causes Mad Cow Disease?

Common methods to eliminate disease-causing organisms in food, like heat, do not affect prions. Also, prions only seem to live in nervous system tissue.

Does Mad Cow Disease Affect Humans?

A human version of mad cow disease called variant Creutzfeldt-Jakob disease (vCJD) is believed to be caused by eating beef products contaminated with central nervous system tissue, such as brain and spinal cord, from cattle infected with mad cow disease. For this reason, the USDA requires that all brain and spinal cord materials be removed from high-risk cattle — older cattle, animals that are unable to walk, and any animal that shows any signs of a neurological problem. These cow products do not enter the U.S. food supply. The USDA believes this practice effectively safeguards U.S. public health from vCJD.

Continued

According to the CDC, four deaths from vCJD have been identified in the U.S. However, it’s believed those cases were caused by consumption of meat outside the U.S.

It is important to clarify the differences between variant CJD and another form of the disease, referred to as classic or sporadic CJD. Classic CJD has no known cause and occurs each year at a rate of one to two cases per 1 million people throughout the world, including in the U.S. and countries where mad cow disease has never occurred. It is not linked to eating nerve tissue from mad cow disease-affected cattle — both vegetarians and meat eaters have died from classic CJD. CJD most commonly affects people over 65 and is usually fatal within six months from onset of symptoms.

What Are the Symptoms of vCJD?

The disease can affect all age groups and is very hard to diagnose until it has nearly run its course. In the early stages of vCJD, people have symptoms related to the nervous system, like depression and loss of coordination. Later in the illness, dementia develops. But only in advanced stages of the disease can brain abnormalities be detected by MRI (magnetic resonance imaging). vCJD is fatal, usually within 13 months of the onset of symptoms.

Is it Possible to Get vCJD From Eating Food Purchased in the U.

S.?

It is extremely unlikely that this would happen. To prevent mad cow disease from entering the country, since 1989 the federal government has prohibited the importation of certain types of live animals from countries where mad cow disease is known to exist. This ban includes meat products used in human, animal, and pet foods. In addition, prohibiting high-risk animals from entering the food supply and the removal of central nervous system tissue from the food supply helps assures that BSE is not a risk to consumers.

Can You Get vCJD From Drinking Milk From an Infected Cow?

Milk and milk products are not believed to pose any risk for transmitting mad cow disease to humans. Experiments have shown that milk from mad cow-infected cows has not caused infections.

What About Other Products Made From Cow By-Products?

The FDA stops the importation of cosmetic and dietary supplement ingredients containing bovine materials from animals originating in the 33 countries where mad cow disease has been found or from animals at risk of being infected.

What Is the Current Risk of vCJD to Americans Traveling Abroad?

According to the CDC, the current risk of acquiring vCJD from any specific country appears to be extremely small. But that cannot be precisely determined because cattle products from one country might be distributed and consumed in others.

How Long Have Health Officials Been Concerned About Mad Cow Disease?

Mad cow disease has been of great concern since 1986, when it was first reported among cattle in the U.K. At its peak in January 1993, almost 1,000 new cases per week were identified. Concern about this disease grew significantly in 1996 when an association between mad cow disease and vCJD in humans was discovered.

What Other Countries Have Reported Cases of Mad Cow Disease?

The disease also has been confirmed in cattle born in Austria, Belgium, Czech Republic, Denmark, Finland, France, Germany, Italy, Ireland, Israel, Japan, Liechtenstein, Luxembourg, the Netherlands, Poland, Portugal, Slovakia, Slovenia, Spain, Switzerland, and the U. K.

Canada has also been added to the list of countries from which imports are restricted, although that ban has been lifted recently. Importation of minimal-risk meat products is now allowed from Canada.

90,000 Stories about terrible diseases – Power – Kommersant

The h2N1 flu story is not the first medical horror story of our time. Over the past 15 years, doctors have predicted large-scale disasters for the world at least three times.

The first “threat of the century” was the already completely forgotten story with the spread among people of the virus of bovine spongiform encephalopathy (or, more simply, mad cow disease). This happened in the mid-1990s. The place from which the threat to peace then emanated was Great Britain.It was there that the epizootic of mad cow disease was recorded, and the first victims of this disease among people were also found there. According to some researchers, the disease was transmitted to humans with animal meat, causing an incurable brain disease in the infected – a new variant of Creutzfeldt-Jakob disease (nvCJD). The disease received this name because the world already existed, without attracting special attention, “ordinary” Creutzfeldt-Jakob disease. First in Great Britain, and then around the world, they began to slaughter cows and prohibit the import of foreign meat.Doctors with renewed vigor began to look for, but still have not found, an antidote for an incurable disease. Beef farmers suffered millions in losses. On the other hand, kangaroo meat and ostrich meat came into fashion, the producers of which had never even thought about conquering the European, American and Asian markets. Doctors specializing in nvCJD predicted tens of thousands of victims of the pandemic. According to them, over the past ten years, people have eaten 400 thousand cows with spongiform encephalopathy.But each cow could infect several people. However, 164 people have died from nvCJD to date. Interest in “mad cow” gradually faded, and almost the only memory of world hysteria remains kangaroo and ostrich meat, which can still be seen in some supermarkets outside Australia.

In 2003, the world was shocked by the appearance of a new disease, so mysterious and unknown that doctors did not immediately think of a name for it and at first spoke of it as “SARS”.Then it was finally given a scientific name, and now it is known as Severe Acute Respiratory Syndrome (SARS).

SARS made headlines after an American businessman died in Hong Kong. For several months the world has been talking only about the “killing microbe”, “invisible killer”, “death virus”. The World Health Organization, without malicious intent, but actively contributed to the spread of the most incredible rumors about the disease, openly admitting that the world is faced with an unknown disease, which is unknown how it originated, it is unknown what is caused and it is not known how it is transmitted.Not to mention the fact that it is not known how it is treated. Newspapers reprinted the famous phrase of one expert, who literally reported the following: “For now, we can say what this disease is not.” The “Silent Killer” turned out to be unusually playful. In one week, more than 150 people were diagnosed with SARS around the world – from Canada to the Philippines and from Switzerland to Israel. They said that no medicine could help against illness, it was almost impossible to protect oneself from it if one did not become a perfect recluse.And then the hysteria vanished. The secret of the fatal disease was revealed, it turned out to be not so fatal and completely banal. The causative agent of SARS was the plague virus of Chinese raccoon dogs and badgers. In total, 806 people died from the “plague of the XXI century”.

Finally, in 2005, humanity was once again under the threat of extermination by a new killer virus. This time he was identified quickly, but this only increased the panic. The H5N1 bird flu virus is actually unusually deadly – it killed millions of chickens, and even more were destroyed as part of measures to prevent the spread of the disease – it is difficult to treat, fraught with the most terrible complications. However, all this is compensated by the fact that a person has to work very hard to get infected with “bird flu”. It is almost not transmitted from person to person, and you can get sick with it either for a long time and closely communicating with the birds, or even longer and more closely with the sick person. Therefore, to date, only 248 people have died from the “bird flu”. However, world airlines still have not recovered from billions of dollars in losses caused by SARS and bird flu epidemics: the media then called civil aviation the fastest way to spread the virus.

Mad cow disease and prion diseases

I.A. Zavalishin
Professor, Doctor of Medical Sciences
I.E. Shitikova
Candidate of Medical Sciences
Scientific Center of Neurology, Russian Academy of Medical Sciences

Prion diseases are a group of neurodegenerative diseases in humans and animals, etiologically associated with a special infectious protein – prion. These diseases are characterized by a severe progressive course and inevitable death.

About 10 years ago, interest in prion diseases in the world sharply increased due to the registration of a new, especially severe and early variant of Creutzfeldt-Jakob disease, which appeared during the epidemic of the so-called bovine spongiform encephalopathy in England. The maximum incidence of animals – up to 37 thousand heads – fell on 1992. In total, over the period from 1985 to 1996, 170 thousand head of livestock were affected. In addition, in addition to cows, spongiform encephalopathy was reported in five species of London Zoo antelopes, domestic cats, other animals and cows outside Britain during the same period.

In the spring of 1996, the registration of a new variant of the previously known but extremely rare Creutzfeldt-Jakob disease (the incidence in the world is 1 case per 1 million people per year) and the associated “mad cow disease” literally shook the economic and political circles of England. This variant of the disease appeared in 1995, with individual cases reported in France and Italy. The disease made its debut at a young age, which is atypical for this disease, known since the 1920s, and in the process of morphological examination of the brain of deceased patients, changes were revealed similar to those in bovine spongiform encephalopathy.

And now a little history …
For the first time, the term “spongy (spongy, spongy) encephalopathy” was used in 1957 by the Icelandic scientist B. Sigurdsson when describing diseases of sheep on the island of Iceland, which differed from all known diseases by four signs: unusually long (up to several years) incubation period; slowly progressing (months and years) nature of the course; unusual damage to organs and tissues; inevitable death.B. Sigurdsson united such diseases under the general name “slow infections”.

Three years later, kuru was described for the first time – a previously unknown disease that occurs among savages with the habits of ritual cannibalism on the island of New Guinea, manifested by impaired coordination of movements and tremors. The clinical and pathological symptoms of kuru in humans were similar to those in sheep’s disease, known since 1700 – scrapie. In 1966, Gaidushek proved the infectious nature of kuru on monkeys (chimpanzees).In 1967, researchers published data according to which the following were found in the brain of chimpanzee chimpanzees infected with the kuru virus: “sponginess” of the gray matter of the brain, an increase in the number of auxiliary cells (astroglia), the death of neurons in several parts of the brain. These morphological signs (with certain variations) are characteristic of all prion diseases of humans and animals.

Subsequently, spongiform encephalopathy was discovered in people suffering from Creutzfeldt-Jakob disease, Gerstmann-Streussler syndrome, and fatal familial insomnia.At the end of the last century, WHO experts predicted the possibility of a significant epidemic of a new variant of Creutzfeldt-Jakob disease in the next 10 years. But in the last 5 years, fortunately, there has been a clear tendency towards a decrease in the increase in the disease.

By 2005, however, about 140 cases of the new variant of Creutzfeldt-Jakob disease had been reported in England, France and Italy. The disease is characterized by an earlier than usual onset. The age of the patients varied from 16 to 40 years, at the onset of the disease, mental disorders in the form of anxiety, depression, and behavioral changes were noted as clinical manifestations; after weeks and months, neurological disorders were added.In the later stages, as in other prion diseases, memory impairment, dementia, muscle twitching, increased muscle tone; the disease ends in death within 0.5-2 years. In Russia, there is no data on the emergence of a new variant of Creutzfeld-Jakob disease, although the usual Creutzfeld-Jakob disease with late onset of symptoms occurs with the same frequency as in other countries.

A big mystery and, accordingly, of interest is the fact that some variants of prion diseases can be infectious in nature (for example, caused by a dura mater transplant or the use of tissue extracts containing growth hormone and gonadotropin), others are hereditary, and still others are in no way related to either heredity or infectious transmission.

In all cases, the culprit of the disease is its own defective protein – prion protein, which turns into an infectious analogue – prion. The prion, as it were, “forces” the surrounding normal prion protein molecules to take a pathological form, and this process has the character of a chain reaction. As Professor Stanley Prusiner aptly put it, “Dr. Jekyll is turning into Mr. Hyde.” A defective prion protein has abnormal properties and is contagious and fatal to any nearby neurons.

Professor S. Pruziner received the 1997 Nobel Prize in Physiology or Medicine for his proof of the existence of a new biological infectious agent – a prion that does not contain DNA or RNA, capable of carrying information and crossing the species barrier.

Currently, the diagnostic criteria for prion diseases have been unified, and work is underway to identify and study them. The advances in molecular biology in recent years give hope for a deeper understanding of the cause of the disease and the mechanisms of pathological transformation of the prion protein and, most importantly, for finding effective remedies for the treatment of these deadly diseases.The preliminary results of experimental studies on cell cultures are very encouraging, and it is possible that some “anti-prion” compounds may become the object of the first clinical trials in the next 5 years.

© Journal “Nerves”, 2007, №2

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Russian officials admitted that there have been cases of death from mad cow disease

Doctors call this disease Creutzfeldt-Jakob disease.Over the past months, there have been statements at various levels that this deadly infection has not been noted in Russia. And now the chief infectious disease specialist of the Ministry of Health Mels Turyanov became the troublemaker. According to him, only in Moscow and only last year, the corresponding diagnosis was made to three patients. All of them have already passed away.

At the Burdenko Institute of Neurosurgery in the capital, which Turyanov mentioned, at first they refused to comment on his words. Then the deputy director Alexander Potapov made a denial.He said that the Burdenko clinic had never had such patients. Our neurosurgeons, – added Potapov, – know about this disease only from medical literature.

This is what is known about this disease, which in the West is compared to AIDS.

Mad Cow Disease is a new type of Creutzfeldt-Jakob disease known in medicine for a long time. The causative agent is not a bacterium or a virus, but a modified protein. American scientist Carlton Gaidushek received the Nobel Prize for this discovery.

For a long time it was believed that people cannot get infected with mad cow disease. But six years ago, 19-year-old Stephen Churchill died of spongiform encephalopathy in England. First known victim of mad cow disease. How many people in the world are infected with this infection is unknown. The incubation period is from five to forty years. As soon as the first symptoms appear, the disease begins to develop rapidly. In a few months, an absolutely healthy person turns into a weak-minded disabled person.And – inevitable death.

Scientists cannot yet determine the cause of the infection in cows. The most common opinion is that bone meal, which is added to livestock feed, is to blame. The disease cannot be detected at an early stage. The diagnosis – “mad cow disease” can be made only after death. Doctors are now comparing spongiform encephalopathy to AIDS. One hundred percent fatal, the same rate of spread. In Britain, in the homeland of mad cow disease, it is already being said that in the next 50 years, 136 thousand British people may die from this disease.

So, officially the history of mad cow disease began in Great Britain. And it is in this country that the greatest experience has been accumulated in monitoring the disease, which is not yet amenable not only to treatment, but even to early diagnosis.

Today, when the whole of Britain is captured by the fight against foot and mouth disease, few people remembered that exactly five years ago, a group of Scottish scientists discovered a connection between the consumption of meat from cows with rabies and the occurrence of an incurable brain disease in humans – the so-called Creutzfeldt-Jakob disease …

What a panic then began! In one day, prices for famous steaks fell tenfold, and most importantly, the European Union banned the export of British beef. It took five billion pounds of taxpayers to fight the crisis alone. Nobody counted the losses of the farmers. 177 thousand 780 cows suffering from spongiform encephalospyathy or, in common parlance, “mad cow disease”, were destroyed. In England, a computer database has been created that tracks the offspring and movement of each cow. The so-called cow passports were introduced.

The disease is believed to be caused by the use of mixed fodder from poorly processed bone tissue of sheep, which, in turn, are sick, in turn, by a specific shaking. And to this day in England it has not been completely eliminated. This year alone, the Ministry of Agriculture informed us, 88 more cases of mad cow disease have been identified. However, the government prefers to report that excellent work has been done. Moreover, since July 1999, EU restrictions on the export of British cow meat not older than 30 months have been canceled.

France resisted the longest. London and Paris even sued over this in Strasbourg. In fact, the problem has not yet been resolved. Scientists believe the disease may be common among older Britons.

HARRIET KIMBELL, professor, member of the government commission on spongiform encephalopathy: “We found it in a 74-year-old man. She may be hiding among other elderly people. We mistake her for Alzheimer’s. Only an autopsy will tell how common the disease is.And in our country it is not very customary to open the bodies of elderly people. “

The Minister of Health called the incident a national tragedy and apologized to his fellow citizens.

How serious are the fears that something similar to what our correspondent in London told about will happen in Russia

Our program is broadcast live – Minister of Health Yuriy Shevchenko (see video)

Mad cow disease appeared in the USA

The first case of mad cow disease has been identified on a Washington state farm on the west coast of the United States.In this regard, several countries, including Russia, have temporarily stopped imports of American beef.

Preliminary laboratory tests indicated that one of the dairy cows slaughtered on 9 December was most likely infected with mad cow disease. However, the diagnosis has not been conclusively confirmed. This disease of cattle is dangerous for humans because it can lead to the development of a disease of the central nervous system – spongiform encephalopathy, a form of Creutzfeldt-Jakob disease.

US Secretary of Agriculture Anne Veneman said that meat, as well as the brain and spinal cord of an animal suspected of being infected with the mad cow virus has been removed from processing plants, and therefore the possibility of infection can not be feared. “We are confident that there is no threat to public health in connection with this incident,” said Ann Veneman.

Speaking on the morning news program, the Secretary of Agriculture revealed that a farm in the town of Mableton, Washington state, has been quarantined and measures have been taken to trace how a cow with suspected disease got there. tags, it will not be difficult to establish where this cow was brought to the farm from.

USDA officials said that three meat processing plants had already received the cow’s meat prior to testing positive for Mad Cow Disease.

Mad cow disease was first discovered in the UK in 1986. As a result of the outbreak of this disease in the late 1980s and until the mid-1990s, more than a hundred people died in the world and significant damage was caused to livestock in the countries of the European Union.

A few hours after the announcement of the suspicion of the disease, Russia, Japan, South Korea, Singapore, Thailand, Taiwan, Hong Kong, Malaysia and Brazil imposed a ban on the import of American beef. In fifteen EU countries, a similar ban was in effect before, due to fears of the use of growth hormones in animal husbandry.

90,000 A Muscovite was diagnosed with a rare fatal disease – Moscow 24, 14.05.2015

A Muscovite was diagnosed with an extremely rare fatal Creutzfeldt-Jacob disease or mad cow disease, according to the Moskva 24 TV channel.

In March, a woman went to the clinic with complaints of malaise. However, she was not diagnosed with a disease and was simply prescribed vitamins. Meanwhile, the undiagnosed disease continued to progress. As a result, her condition deteriorated sharply and hallucinations began. Within two months, the woman went to bed and became completely immobilized. Now a Muscovite is in a rehabilitation center, does not react to relatives and friends, she needs to be turned over and fed through a tube.

In a closed case history, which is not handed out, the diagnosis of Creutzfeldt-Jakob disease is recorded, but doctors cannot officially diagnose it, since it must be confirmed.This can take months or years. All over the world, only 1-2 cases are detected per year, and in Russia there are no official statistics for this disease at all.

The patient’s children are struggling to get a diagnosis, albeit posthumously. According to them, this will allow other patients to get a chance for recovery and not waste time on diagnosis.

Creutzfeldt-Jakob disease is a pathological change in a protein in the brain tissue, which is caused by prions – proteins with an abnormal structure.According to various hypotheses, prions either appear in the brain on their own, or they can be infected from meat products.

Typically, people die about 3-12 months after the onset of illness, often from pneumonia. Approximately 5-10% of patients survive for 2 years or more.

Once in the body, harmful prions begin to destroy healthy brain cells. First of all, the central nervous system suffers, holes appear in the brain, and it turns into a sponge.When the disease has already penetrated into the central nervous system, the changes are irreversible, since the physical destruction of the tissue is taking place.

90,000 A case of “mad cow disease” may have been detected in the USA :: Society :: RBC

In the US, there may be a new case of mad cow disease in cattle, said John Clifford, a spokesman for the American veterinary service.

According to him, during a routine test, the virus was detected in the blood of one cow.Currently, the Ministry of Agriculture is conducting additional tests to confirm or deny data from preliminary analyzes, reports (C) Associated Press.

If the diagnosis is confirmed, many countries that have lifted the ban on the import of beef from the United States will reintroduce it. Let us also remind that within 6 weeks the European Parliament must make a final decision on lifting the ban on imports of beef from Great Britain.

Meanwhile, in Sweden on March 3 of this year. the first case of mad cow disease was confirmed.Bovine spongiform encephalopathy (BSE) virus was tested positive for a 12-year-old cow from central Westmanland County. The analyzes were carried out in an EU-controlled laboratory in Weybridge, UK.

Mad cow disease, or spongiform encephalopathy, affects the nervous system of animals. Scientists believe that human consumption of the meat of infected animals can cause people to have Kreuzfeldt-Jakob disease, which is almost always fatal.

The disease has killed 150 people worldwide, mainly in the UK, which was at the epicenter of the epidemic in the 1990s. In the United States, the first case of mad cow disease in animals was identified in February 2004.

90,000 Ugra goes berserk

The most dangerous infection on the planet was found in the Khanty-Mansi Autonomous Okrug. “This is worse than the bubonic plague and the Ebola fever

The most dangerous infection on the planet has been discovered in the Khanty-Mansi Autonomous Okrug.”It’s worse than the bubonic plague and Ebola”

Be extremely careful. In Ugra, at least one person has already died from an incurable disease that burns a sick person in a matter of days. How many people actually died from the terrible virus is unknown. Doctors are hiding the size of the disaster and are doing everything not to make a frightening diagnosis. But the worst thing is that it is almost impossible to protect yourself. “URA.Ru” can only convey the advice of virologists.

Artem Rudan from Nizhnevartovsk buried his mother.The disease developed at a rapid pace. Literally in a month from simple headaches and fatigue – to the state of a vegetable. The woman stopped recognizing her son, walked by herself and could not eat. Doctors of the Nizhnevartovsk district hospital suggested that it could be Creutzfeldt-Jakob disease (CJD), better known as mad cow disease.

As reported in a conversation with URA.Ru, Doctor of Medical Sciences, Professor, Member of the Presidium of the Russian Academy of Natural Sciences, Member of the New York Academy of Sciences, Vice President of the International Academy of Sciences of Nature and Society, Head of the Department of Microbiology of Latent Infections, Virologist of the Institute.Gamalei Victor Zuev, Creutzfeldt-Jakob disease belongs to the category of “slow infections”. They were discovered over sixty years ago.

CJD is a very rare disease. Its frequency is one in a million per year. The disease is caused not by viruses, bacteria and parasites, the expert says, but by a proteinaceous pathogen. In the 90s in Britain, the CJD assumed the scale of an epidemic. The veterinary services of Russia then did everything to prevent the infection from entering the country.

“This is a really serious and fatal disease, it does not give in to treatment. CJD has a long incubation period, like all slow infections. As a rule, this is a disease of the elderly, ”says Viktor Zuev.

Contact with a young man from Nizhnevartovsk, who survived a terrible tragedy, failed: his phone does not answer. Artyom left the city where he buried his mother, and will return only a few days later. Irina Litvinenko told the URA.Ru correspondent that the CJL is banned in Russia. A girl from Moscow experienced exactly the same nightmare as Artem: her mother died two weeks ago.

“Nobody wanted to take responsibility. They said that senile dementia. We were transferred to the Botkin hospital only after this story began to be covered in the media, – says Irina. – And in the end, the doctors refused to do the autopsy. In Russia, they believe that the risk of infection with such a procedure is too great. We were diagnosed by symptoms, although this disease can only be confirmed by examining the brain tissue after autopsy. ”

According to the girl, she had to literally break through the wall of medical indifference.It turned out that in Moscow there is an order from the local health department, which prescribes hospitalization of all patients with suspected mad cow disease. However, this document is almost secret – doctors do not risk making such a serious diagnosis. The infected are advised to “drink vitamins” and wait for recovery.

Irina says that she is aware of at least three such cases. In addition to Artyom, a resident of Barnaul contacted her: her father had the same symptoms. The Barnaul doctors sent the man home to die.“With Artyom, everything was more human. His mother was immediately hospitalized. They did all the necessary tests – they refused to put us in the hospital, and we ourselves paid at the beginning for the research. In addition, in Nizhnevartovsk, doctors agreed to do an autopsy to confirm the diagnosis, ”says Irina Litvinenko.

Director of the Health Department of the Khanty-Mansi Autonomous Okrug Alexander Filimonov confirmed to URA. Ru that the deceased mother of Artyom Rudan in the hospital in Nizhnevartovsk was indeed diagnosed with Creutzfeldt-Jakob disease.

Doctors came to this conclusion during the patient’s lifetime. Initially, encephalitis was also considered, but in the end it was decided to focus on “mad cow disease”. “She was being treated at the second district hospital. The disease progressed rather quickly. After the diagnosis was confirmed by pathologists. Therefore, this diagnosis was also indicated in the death certificate, ”Filimonov comments.

All three cases of infection of Russians occurred at about the same time.However, the source of the infection is still unknown. It is possible that people caught the disease due to contaminated meat (this is how the disease spread in the 90s in the UK). And it is quite possible that people were infected not even in Russia. In particular, Artem suspects that his mother could catch mad cow disease at the end of November in Thailand.

Yugorsk doctors admit that exotic diseases are often brought to the Khanty-Mansi Autonomous Okrug from abroad, which are difficult even to diagnose, not to mention the correct treatment.“In practice, there are rare diseases, there are incomprehensible cases – for example, from Vietnam. People try exotic things, get infected, and then come to us for treatment. And we are racking our brains what to do with them. In Vietnam, they do not know how to cure our opisthorchiasis, but we are fighting their sores here, ”said Ilya Salmanov, chief physician of the Nizhnevartovsk District Hospital No. 2.

Alexander Filimonov noted that for the final confirmation of the CJD case, the biological material of the deceased was sent for examination to Moscow.Before the incident with the mother of Artem Rudan, the doctors of the Khanty-Mansi Autonomous Okrug had not encountered such a disease. If a positive answer comes from Moscow, the district hospital No. 2 will have to carry out the appropriate treatment (if it has not yet been carried out), and the sanitary doctors of Nizhnevartovsk will have to take the situation with the new fatal infection under control.

According to Viktor Zuev, “mad cow disease” is transmitted through blood. Previously, doctors were infected with the disease, who opened sick people after death. During autopsies, pathologists may have cut the glove.As for the determination of a 100% diagnosis, here, according to Zuev, there is a difficulty: in Russia today there are no test systems that are used to diagnose CJD.

“Yes, these diseases are not registered in our country at all – that’s the point! Also, to make an accurate diagnosis, it is necessary to build up from a number of other clinical pictures. In any case, it is quite obvious that a high qualification of the doctor who observed this woman is required. Was there (in Nizhnevartovsk – ed.) such a doctor – I don’t know, ”says the expert virologist. However, it is quite possible that this terrible disease really took place in Nizhnevartovsk, sums up Viktor Zuev. According to the signs that were publicly available, what happened is very similar to the CJD.

Today in Russia there is no official statistics on how many people died from an incurable disease. Mad cow disease is not even studied in the country. “It’s scary to even imagine how many people did not receive at least some kind of treatment – they could simply be weeded out at the level of district polyclinics.Send home like the father of a girl from Barnaul. Artem was lucky at least that the doctors did not hide the disease and made a diagnosis. But this is an exception – such patients are incurable, hopeless. Therefore, there is no disease – no problem. I think this is how doctors treat it, ”says Irina Litvinenko.

It is impossible even to calculate approximately how many people could have died from Creutzfeldt-Jakob disease – both in Russia and in the Khanty-Mansi Autonomous Okrug. Perhaps the real numbers are comparable to the epidemics of more well-known diseases: CJD is today placed by the World Health Organization on a par with Ebola, plague and anthrax.