What is BSE and how does it affect cows. How can humans contract a version of BSE. What measures are in place to prevent the spread of this disease. How does the FDA ensure food safety in relation to BSE.

Understanding Bovine Spongiform Encephalopathy: The Science Behind Mad Cow Disease

Bovine Spongiform Encephalopathy (BSE), commonly known as mad cow disease, is a progressive neurological disorder that affects cattle. The term “bovine” refers to cows, “spongiform” describes the spongy appearance of affected brain tissue, and “encephalopathy” indicates a brain disease. This complex condition has intrigued scientists and concerned consumers for decades.

What causes BSE? The primary culprit is believed to be a misfolded protein called a prion. These abnormal prions accumulate in the brain, causing damage to the central nervous system. Unlike typical pathogens, prions are not living organisms, making them particularly challenging to detect and combat.

The Prion Puzzle: Unraveling the Cause of BSE

Prions are fascinating yet perplexing entities in the world of molecular biology. In BSE, normal prion proteins undergo a transformation, adopting an abnormal configuration that proves harmful to the animal. The exact mechanism triggering this change remains a subject of ongoing research.

Why are prions so difficult to combat? Unlike viruses or bacteria, prions don’t contain genetic material. This unique characteristic means that the cow’s immune system fails to recognize them as a threat, allowing the disease to progress unimpeded.

Recognizing the Signs: How BSE Manifests in Cattle

Identifying BSE in its early stages presents a significant challenge for farmers and veterinarians. The disease has a lengthy incubation period, typically ranging from four to six years. During this time, infected cows appear completely normal, showing no outward signs of illness.

• Incoordination and difficulty walking
• Trouble standing up
• Nervous or aggressive behavior
• Rapid progression of symptoms

Can BSE be treated or prevented in cows? Unfortunately, there is currently no known treatment or vaccine for BSE. Once symptoms appear, the disease invariably leads to death within two weeks to six months.

The Diagnostic Dilemma: Detecting BSE in Cattle

One of the most significant challenges in managing BSE is the lack of a reliable test for live animals. Definitive diagnosis can only be made post-mortem, by examining brain tissue under a microscope. Scientists look for the characteristic spongy appearance caused by the accumulation of abnormal prions.

Recent advances have led to the development of test kits capable of detecting abnormal prions in brain tissue. These tools have greatly improved our ability to identify BSE cases and monitor its prevalence in cattle populations.

The Origin and Spread of BSE: A Cautionary Tale of Recycled Feed

How does BSE spread among cattle? The primary transmission route is through contaminated feed. In the past, the rendering process that converted animal byproducts into protein-rich feed supplements inadvertently became a vector for prion transmission.

When parts from a BSE-infected cow were processed into feed, the abnormal prions survived the rendering process. Healthy cows consuming this contaminated feed became infected, perpetuating the cycle of disease transmission.

The Critical First Year: When Cows Are Most Vulnerable

Research has shown that cows are most susceptible to BSE infection during their first year of life. This finding has important implications for disease prevention and control strategies in the cattle industry.

Why is early exposure so significant? The long incubation period of BSE means that cows infected as calves may not show symptoms until they are five years old or even older. This delayed onset complicates efforts to trace the origin of infections and implement effective control measures.

From Cows to Humans: The Emergence of Variant Creutzfeldt-Jakob Disease

Can humans contract BSE? While humans cannot develop BSE itself, they can contract a related condition called variant Creutzfeldt-Jakob disease (vCJD). This rare but fatal neurodegenerative disorder is believed to result from consuming beef products contaminated with BSE prions.

As of 2019, 232 cases of vCJD have been reported worldwide, with the majority occurring in the United Kingdom. The low number of cases relative to the potentially exposed population suggests that the species barrier between cows and humans provides some protection against prion transmission.

Debunking Myths: Understanding vCJD Transmission

Is vCJD contagious between humans? Unlike many infectious diseases, vCJD cannot be transmitted through casual contact. It is not spread through coughing, sneezing, or other forms of person-to-person interaction.

Are dairy products a risk for vCJD? Research has conclusively shown that milk and dairy products, even from BSE-infected cows, do not transmit the disease to humans. This finding has been crucial in maintaining public confidence in dairy consumption.

Safeguarding the Food Supply: FDA’s Multi-Pronged Approach to BSE Prevention

How does the FDA protect consumers from BSE-related risks? The U.S. Food and Drug Administration has implemented a comprehensive strategy to prevent BSE in cattle and protect human health. These measures focus on eliminating potential sources of prion contamination in the food chain.

1. Feed restrictions: Since August 1997, the FDA has banned the use of most mammalian proteins in cattle feed.
2. Enhanced safeguards: In April 2009, additional regulations were introduced to prevent cross-contamination in all animal feeds.
3. High-risk tissue ban: Certain cow parts with the highest risk of prion contamination are prohibited from use in any animal feed, including pet food.

Why focus on animal feed? By ensuring that cattle are not exposed to potentially contaminated feed, the FDA aims to break the cycle of BSE transmission and protect both animal and human health.

The 30-Month Rule: A Key Component of BSE Prevention

What is the significance of the 30-month age limit in BSE regulations? The FDA has identified that the risk of BSE increases in older cattle. As a result, stricter controls are placed on tissues from cows aged 30 months or older, particularly the brain and spinal cord.

This age-based approach allows for targeted risk management, balancing the need for effective disease control with the practical considerations of the beef industry.

Global Impact: BSE’s Influence on International Trade and Policy

How has BSE affected the global beef industry? The emergence of BSE in the 1980s and 1990s had far-reaching consequences for international trade in beef and cattle products. Many countries implemented strict import bans on beef from affected regions, leading to significant economic losses and shifts in global meat production patterns.

The BSE crisis also catalyzed major changes in agricultural and food safety policies worldwide. Enhanced surveillance, improved traceability systems, and more stringent feed regulations have become standard practices in many countries.

Lessons Learned: The Legacy of BSE in Modern Agriculture

What lasting impacts has the BSE crisis had on the agricultural sector? The BSE epidemic highlighted the interconnectedness of animal health, human health, and food safety. It underscored the importance of the “One Health” approach, which recognizes that the health of humans, animals, and the environment are inextricably linked.

The crisis also led to increased transparency in food production and heightened consumer awareness about the origins of their food. These shifts have contributed to the growth of movements promoting local, sustainable, and ethically produced food.

Future Directions: Advancing BSE Research and Prevention

What are the current frontiers in BSE research? Scientists continue to investigate the fundamental nature of prions and their role in neurodegenerative diseases. This research not only aids in understanding BSE but also has implications for human conditions like Alzheimer’s and Parkinson’s disease.

• Developing live animal tests for BSE
• Exploring potential treatments for prion diseases
• Improving methods for decontaminating prion-infected materials
• Investigating the potential for prion adaptation and evolution

How might climate change impact BSE risk? As global temperatures rise, there is growing concern about the potential for environmental persistence of prions. Research is underway to understand how changing climate conditions might affect prion stability and transmission in both wild and domestic animal populations.

The Role of Genomics in BSE Prevention

Can genetic research help prevent BSE? Advances in genomic technologies are opening new avenues for BSE prevention and control. Scientists are exploring genetic factors that may influence susceptibility to prion diseases in both cattle and humans.

This research could lead to the development of breeding programs to produce cattle with enhanced resistance to BSE, as well as improved risk assessment tools for human populations.

As our understanding of BSE and related prion diseases continues to evolve, so too do our strategies for prevention and control. The ongoing collaboration between scientists, policymakers, and industry stakeholders remains crucial in safeguarding both animal and human health against this complex and challenging disease.

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?

• Contact the FDA’s Center for Veterinary Medicine at 240-402-7002 or [email protected].
• U.S. Department of Agriculture, BSE Frequently Asked Questions
• Centers for Disease Control and Prevention, Variant Creutzfeldt-Jakob Disease (vCJD)
• Centers for Disease Control and Prevention, BSE Cases Identified in the United States
• University of Edinburgh, The National CJD Research & Surveillance Unit (NCJDRSU)

Symptoms, Causes and Treatments for vCJD

Written by Michael W. Smith, MD

• 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?
• Does Cooking Food Kill the Prion That Causes Mad Cow Disease?
• Does Mad Cow Disease Affect Humans?
• What Are the Symptoms of vCJD?
• Is it Possible to Get vCJD From Eating Food Purchased in the U.S.?
• Can You Get vCJD From Drinking Milk From an Infected Cow?
• What About Other Products Made From Cow By-Products?
• What Is the Current Risk of vCJD to Americans Traveling Abroad?
• How Long Have Health Officials Been Concerned About Mad Cow Disease?
• What Other Countries Have Reported Cases of Mad Cow Disease?
• More

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.

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.

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.

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.

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.

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 help ensure that BSE is not a risk to consumers.

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.

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.

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.

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.

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.

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A terrible disease called “mad cow disease” or encephalopathy, the human form of which is considered incurable at the current level of medical development, is sweeping across Europe by leaps and bounds.

A few days ago, the first yet officially confirmed case of “mad cow disease” was detected in Italy. The discovery of an infected carcass at one of the Cremonini factories was reported by the country’s Ministry of Health. The carcass is isolated for final testing. This Italian meat processing company exports meat to the United States. Her other clients include restaurants and fast food outlets at train stations across Europe.

Meanwhile, the British Food Standards Agency announced its intention to conduct a study to determine the likelihood of transmission of “mad cow disease” through milk. One such study has already been carried out. However, according to scientists, there is still no convincing evidence that the human form of encephalopathy – the so-called Creutzfeldt-Jakob disease, or spastic pseudosclerosis – is transmitted to humans only through contaminated meat products. A new UK study on the subject will be conducted by the government’s Central Veterinary Laboratory. The entire work will take about three years. Unlike the questionable previous study, which was conducted in mice, the current one is supposed to be carried out in calves, which will eliminate such factors as the species barrier.

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Veterinarians warn: who is at risk of mad cow disease?

The prion protein is to blame for the development of pathology (hence the second name – prion disease). It is also found in healthy people. However, when sick, a modified form of this substance accumulates in the body.

Prion disease (spongiform encephalitis) is not fully understood. There is another name for it – mad cow disease due to characteristic changes in the behavior of diseased livestock.

It has been established that the altered protein can enter the human body with meat from infected cows. For this reason, since the early 1990s, more than 85 people have died in England alone.

Animals – carriers of the disease – do not show any anxiety for a long time until a severe degree of the disease occurs. Therefore, veterinary specialists have developed a special leaflet on bovine spongiform encephalopathy.

What is important to know for prevention? Most often, the disease in animals develops for a long time up to 8 years. The result is 100% lethality.

Under natural conditions, adult animals are susceptible to the modified protein, especially at the age of four. To a greater extent – dairy cattle, mainly cows, less often – breeding bulls. The causative agent of the disease is transmitted from a sick animal to a healthy one by eating contaminated food (meat and bone meal).
Eating the brain and spinal cord of slaughtered animals is especially dangerous for humans.

In order to prevent the occurrence and spread of bovine BSE, animal owners, individual entrepreneurs should comply with the following requirements: not to allow the importation of pedigree cattle, meat, canned food, offal and semi-finished products, meat and bone meal and other products, feed of animal origin from ruminants. They may be infected.

Pay special attention to the accompanying documents, which must be available.
It is important to regularly identify all types of farm animals.

For newly brought cows, bulls, arrange a 30-day quarantine.
All movements, delivery for slaughter, sale of livestock products should be carried out only under the control of the State Veterinary Service.