Mad cow disease other names. Mad Cow Disease: Understanding BSE, Its Impact, and Prevention Measures

22.08.202308.07.2023 by alexxlab

What is Bovine Spongiform Encephalopathy. How does BSE affect cattle and humans. What measures are in place to prevent the spread of mad cow disease. How is BSE diagnosed and monitored in the United States. What are the global implications of BSE outbreaks.

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The Origins and Nature of Bovine Spongiform Encephalopathy

Bovine Spongiform Encephalopathy (BSE), commonly known as mad cow disease, is a fatal neurological disorder that affects cattle. It belongs to a group of diseases called transmissible spongiform encephalopathies (TSEs) or prion diseases. These diseases are caused by abnormally folded proteins called prions, which can trigger a chain reaction of protein misfolding in the brain, leading to neurodegeneration.

How does BSE develop in cattle? The disease typically has a long incubation period of 4-6 years before symptoms appear. During this time, the misfolded prions accumulate in the central nervous system, causing progressive damage to brain tissue. As the disease advances, affected cattle may exhibit behavioral changes, abnormal posture, and difficulty in movement.

The First BSE Case in the United States: A Turning Point

On December 23, 2003, the U.S. Department of Agriculture (USDA) announced a presumptive diagnosis of the first known case of BSE in the United States. The case involved an adult Holstein cow from Washington State. This discovery marked a significant moment in U.S. agricultural history and triggered a series of events and policy changes.

How was the first U.S. BSE case identified? The infected cow was non-ambulatory (a “downer cow”) at slaughter, prompting brain tissue samples to be taken as part of USDA’s targeted surveillance for BSE. Initially, the animal’s condition was attributed to complications from calving. However, subsequent testing revealed the presence of BSE.

Tracing the Origin of the Infected Cow

Investigations revealed that the BSE-infected cow was likely imported from Canada in August 2001. This finding highlighted the importance of robust tracing systems and international cooperation in managing animal health risks. The case also underscored the need for stringent import regulations and surveillance programs to prevent the introduction and spread of BSE.

The Human Health Risk: Variant Creutzfeldt-Jakob Disease

The discovery of BSE in cattle raised serious concerns about potential transmission to humans. Strong evidence indicates that BSE has been transmitted to humans, primarily in the United Kingdom, causing a variant form of Creutzfeldt-Jakob disease (vCJD).

What is the relationship between BSE and vCJD? Variant Creutzfeldt-Jakob disease is believed to be caused by consuming beef products contaminated with the BSE agent. However, a substantial species barrier appears to protect humans from widespread illness. As of December 1, 2003, a total of 153 vCJD cases had been reported worldwide, with 143 cases occurring in the United Kingdom.

Assessing the Risk in the United States

Despite the discovery of BSE in U.S. cattle, the risk to human health from BSE in the United States is considered extremely low. This assessment is based on the limited number of cases detected and the stringent control measures implemented following the 2003 case.

Surveillance and Monitoring Systems for BSE and CJD

In response to the BSE threat, comprehensive surveillance and monitoring systems have been established in the United States to detect and track both BSE in cattle and potential cases of vCJD in humans.

CDC monitors trends and current incidence of CJD by analyzing death certificate information from U.S. multiple cause-of-death data.
Follow-up reviews of clinical and neuropathology records are conducted for CJD decedents younger than 55 years of age.
The National Prion Disease Pathology Surveillance Center at Case Western Reserve University provides free diagnostic services and helps monitor for emerging forms of prion diseases.

How effective are these surveillance systems? These multi-faceted approaches allow for early detection of potential BSE-related cases in both animals and humans, enabling rapid response and containment strategies if necessary.

Prevention Measures Against BSE Spread

To prevent BSE from entering and spreading within the United States, several critical measures have been implemented:

Import restrictions on live ruminants and certain ruminant products from countries where BSE is known to exist.
Extension of these restrictions to include importation from all European countries.
Regulations on the use of ruminant tissue in animal feed.

Why are feed regulations crucial in preventing BSE? The use of ruminant tissue in animal feed is believed to be the primary route of BSE transmission. By implementing strict controls on feed ingredients, the risk of prion transmission through the food chain is significantly reduced.

Global Implications of BSE Outbreaks

The occurrence of BSE has had far-reaching consequences beyond the immediate health concerns. It has impacted international trade, agricultural practices, and public trust in food safety systems worldwide.

How have BSE outbreaks affected global beef trade? Many countries imposed bans or restrictions on beef imports from nations with reported BSE cases, leading to significant economic losses for affected countries. These trade disruptions have prompted the development of more robust international standards for animal health and food safety.

Lessons Learned and Ongoing Challenges

The BSE crisis has taught valuable lessons about the interconnectedness of animal and human health, the importance of transparent communication during health crises, and the need for proactive risk management in agricultural practices.

What ongoing challenges does BSE present? While the incidence of BSE has dramatically decreased globally, vigilance is still required. Emerging forms of prion diseases, potential environmental reservoirs of prions, and the long incubation period of these diseases necessitate continued research and surveillance efforts.

Advances in BSE Research and Diagnostic Methods

Since the discovery of BSE, significant advances have been made in understanding prion diseases and developing more effective diagnostic tools.

What new diagnostic methods have been developed for BSE? Rapid post-mortem tests have been introduced, allowing for more efficient screening of cattle at slaughter. Additionally, research is ongoing to develop ante-mortem tests that could detect BSE in live animals, potentially revolutionizing surveillance efforts.

Exploring Potential Treatments

While there is currently no cure for BSE or vCJD, researchers are exploring various approaches to treat prion diseases:

Antibody therapies that target misfolded prions
Small molecule compounds that could prevent or reverse prion misfolding
Gene therapies aimed at reducing the expression of the prion protein

How close are we to an effective treatment for prion diseases? While progress has been made, developing effective treatments for BSE and related diseases remains challenging due to the unique nature of prions and the complexity of targeting proteins in the brain.

The Future of BSE Management and Food Safety

As our understanding of BSE and other prion diseases continues to evolve, so too do the strategies for managing these risks and ensuring food safety.

What future developments can we expect in BSE management? Emerging technologies such as blockchain for improved traceability, advanced genetic testing for identifying BSE-resistant cattle breeds, and more sensitive detection methods are likely to play significant roles in future BSE prevention and control efforts.

One Health Approach to BSE

The BSE crisis has underscored the importance of a One Health approach, which recognizes the interconnectedness of human, animal, and environmental health. This holistic perspective is becoming increasingly crucial in addressing complex health challenges like BSE.

How does the One Health approach benefit BSE management? By fostering collaboration between veterinary, medical, and environmental health professionals, the One Health approach enables more comprehensive risk assessment, surveillance, and response strategies for BSE and other zoonotic diseases.

Public Awareness and Consumer Confidence

Rebuilding and maintaining public trust in the safety of beef products has been a critical challenge in the aftermath of BSE outbreaks.

What measures have been taken to restore consumer confidence? Transparency in reporting BSE cases, clear communication about risk factors and preventive measures, and stringent food safety regulations have all played roles in reassuring consumers. Additionally, education campaigns have helped to inform the public about the nature of BSE and the safeguards in place to protect human health.

The Role of Media in BSE Communication

Media coverage has significantly influenced public perception of BSE risks. Responsible reporting that balances scientific accuracy with public interest is crucial in managing public response to BSE-related news.

How can media contribute positively to BSE awareness? By collaborating with scientific experts, providing context for BSE-related developments, and avoiding sensationalism, media outlets can play a constructive role in educating the public and maintaining proportionate concern about BSE risks.

BSE Cases Identified in the United States BSE (Bovine Spongiform Encephalopathy) | Prion Diseases

On December 23, 2003, the U.S. Department of Agriculture (USDA) announced a presumptive diagnosis of the first known case of BSE in the United States. It was in an adult Holstein cow from Washington State. This diagnosis was confirmed by an international reference laboratory in Weybridge, England, on December 25. Trace-back based on an ear-tag identification number and subsequent genetic testing confirmed that the BSE-infected cow was imported into the United States from Canada in August 2001.

Because the animal was non-ambulatory (a “downer cow”) at slaughter, brain tissue samples were taken by USDA’s Animal and Plant Health Inspection Service as part of its targeted surveillance for BSE. However the animal’s condition was attributed to complications from calving. After the animal was examined by a USDA Food Safety and Inspection Service (FSIS) veterinary medical officer both before and after slaughter, the carcass was released for use as food for human consumption. During slaughter, the tissues considered to be at high risk for the transmission of the BSE agent were removed.

On December 24, 2003, FSIS recalled beef from cattle slaughtered in the same plant on the same day as the BSE positive cow. (see Bovine Spongiform Encephalopathy in a Dairy Cow—Washington State, 2003.)

Preliminary Investigation Suggests BSE-Infected Cow in Washington State Was Likely Imported from Canada

On December 23, 2003, the U.S. Department of Agriculture (USDA) announced a presumptive diagnosis of bovine spongiform encephalopathy (BSE, or “mad cow” disease) in an adult Holstein cow from Washington State. Samples were taken from the cow on December 9 as part of USDA’s BSE surveillance program. The BSE diagnosis was made on December 22 and 23 by histopathology and immunohistochemical testing at the National Veterinary Services Laboratory, Ames, Iowa. The diagnosis was confirmed by an international reference laboratory in Weybridge, England, on December 25. Preliminary trace-back based on an ear-tag identification number suggests that the BSE-infected cow was imported into the United States from Canada in August 2001.

USDA, in close cooperation with Canadian agricultural authorities, has launched an epidemiologic investigation to determine the source of the disease. Beef from the slaughtered cow had been processed for human consumption. On December 23, 2003, the Food Safety and Inspection Service (FSIS), USDA announced the recall of all beef from cattle slaughtered on December 9 at the involved slaughter plant.

Strong evidence indicates that BSE has been transmitted to humans primarily in the United Kingdom, causing a variant form of Creutzfeldt-Jakob disease (vCJD). In the United Kingdom, where over 1 million cattle may have been infected with BSE, a substantial species barrier appears to protect humans from widespread illness. As of December 1, 2003, a total of 153 vCJD cases had been reported worldwide; of these, 143 cases had occurred in the United Kingdom. The risk to human health from BSE in the United States is extremely low.

CDC monitors the trends and current incidence of CJD in the United States by analyzing death certificate information from U.S. multiple cause-of-death data compiled by the National Center for Health Statistics. With the support of the Council of State and Territorial Epidemiologists, CDC conducts follow-up review of clinical and neuropathology records of CJD decedents younger than 55 years of age. In addition, during 1996-1997, in collaboration with the American Association of Neuropathologists (AANP), CDC established the National Prion Disease Pathology Surveillance Centerexternal icon at Case Western Reserve University in Cleveland, Ohio. This pathology center provides free, state-of-the-art diagnostic services to U.S. physicians. It also helps to monitor the possible occurrence of emerging forms of prion diseases, such as vCJD, in the United States. For more information about the center visit its website at:

United States Department of Agriculture’s (USDA): BSE Surveillance Information Centerexternal icon
FDA Bovine Spongiform Encephalopathy external icon
CDC Prion Diseases
NIH NINDS Creutzfeldt-Jakob Disease Information Pageexternal icon

Prevention BSE (Bovine Spongiform Encephalopathy) | Prion Diseases

Prevention Measures against BSE Spread

To prevent BSE from entering the United States, severe restrictions were placed on the importation of live ruminants, such as cattle, sheep, and goats, and certain ruminant products from countries where BSE was known to exist. These restrictions were later extended to include importation of ruminants and certain ruminant products from all European countries.

Because the use of ruminant tissue in ruminant feed was probably a necessary factor responsible for the BSE outbreak in the United Kingdom and because of the current evidence for possible transmission of BSE to humans, the U.S. Food and Drug Administration instituted a ruminant feed ban in June 1997 that became fully effective as of October 1997. As of October 26, 2009, a regulation issued by FDA in April 2009 came into effect establishing an enhanced BSE-related feed ban in the U.S. This enhanced feed ban will further harmonize BSE feed control measures in the U.S. with those in Canada (see below). In addition, FDA continues to enforce its important 1997 mammalian-to-ruminant feed ban through its BSE inspection and BSE feed testing programs.

As of July 12, 2007, an enhanced BSE-related feed ban came into effect in Canada. CFIA established this ban to more effectively prevent and quickly eliminate BSE from Canada. The enhanced ban prohibits most proteins, including potentially BSE infectious tissues known as “specified risk materials” (SRM) from all animal feeds, pet foods, and fertilizers, not just from cattle feed as required by the ban instituted in 1997. The 1997 feed ban in Canada was similar to the feed ban instituted in the United States that same year. As recently reported by CFIA, removing SRM from the entire animal feed system addresses risks associated with the potential contamination of cattle feed during production, distribution, storage, and use. Applying the same measure to pet food and fertilizer materials addresses the possible exposure of cattle and other susceptible animals to these products. With this ban in place, CFIA expects BSE should be eliminated from the Canadian cattle herd by about the year 2017.

In late 2001, the Harvard Center for Risk Assessment study of various scenarios involving BSE in the U.S. concluded that the FDA ruminant feed rule provides a major defense against this disease.

BSE/TSE Action Plan of the Department of Health and Human Services (DHHS)

On August 23, 2001, the Department of Health and Human Services (HHS) issued a department-wide action plan outlining steps to improve scientific understanding of BSE and other transmissible spongiform encephalopathies (TSEs). The action plan has four major components:

Surveillance for human disease is primarily the responsibility of CDC.

Protection is primarily the responsibility of the Food and Drug Administration (FDA).

Research is primarily the responsibility of the National Institutes of Health (NIH).

Oversight is primarily the responsibility of the Office of the Secretary of DHHS.

Read the Department of Health and Human Services press release: USDA and HHS Strengthen Safeguards Against Bovine Spongiform Encephalopathyexternal icon

what you need to know about mad cow disease

The Department of Veterinary Medicine of the Sverdlovsk Region warns owners of cattle about the danger of importing into the country from disadvantaged zones or countries of breeding stock, meat, canned food, offal and semi-finished products, meat and bone meal, sperm, embryos, technical fat, intestinal raw materials and other products and feeds of animal origin from ruminants. In order to prevent mad cow disease infection, it is necessary to inform veterinary clinics in a timely manner.

What is mad cow disease?

Bovine spongiform encephalopathy (BSE), or mad cow disease, is a slowly developing infectious prion transmissible disease of adult cattle, characterized by a long, up to 2.5-8 years, incubation period and manifested by damage to the central nervous system with 100% mortality.

Historical reference.

Spongiform encephalopathy was first reported in 1985-1986 in the UK under the name “mad cow disease”. In the next 10 years, BSE spread to other countries: France, Portugal, Switzerland, Germany, the Netherlands, Italy, Denmark, Slovakia, Finland, etc.

scrapie cattle (scrapie) – a similar agent (causative agent of sheep scrapie), found in meat and bone meal, which was included in the diet of cattle. In Russia, the disease has not been registered.

Economic damage.

BSE caused enormous economic damage to European countries, due to the fact that about 4 million heads of cattle were destroyed. The UK alone suffered an economic loss of £7 billion.

Epizootological data.

Under natural conditions, cattle are susceptible to BSE, especially at 4 years of age. The source of the causative agent of infection are sick and animals in the incubation period. The factors of transmission of the infectious agent are the products of slaughter of sheep with scrapie, and cattle with EH.

Course and symptoms of the disease.

The incubation period is from 2.5 to 8 years, in some cases it can be extended up to 25-30 years. The course of the disease is progressive, without remissions. The disease proceeds without an increase in body temperature of the animal, with continued appetite. Despite a normal appetite, cows have reduced milk production.

The clinical manifestation of the disease is characterized by signs of damage to the central nervous system.

Three types of nervous phenomena are detected during GE.

The first type of nervous phenomena is accompanied by the development in animals of a feeling of fear, nervousness, aggressiveness, gnashing of teeth, restlessness, timidity. The above symptoms occur in 98% of sick animals.
The second type of nervous phenomena is characterized by the presence of movement disorders in sick animals: trotting movements, “raking in the front limbs”, “lowering” of the hind legs, raised tail
In the third type of nervous phenomena, there is a violation of sensitivity, when in sick animals we note hyperesthesia with noise, touch and light. The duration of the disease is from several weeks to 12 months or more. The disease always ends in the death of the animal.

Pathological changes.

At autopsy of dead animals, characteristic pathoanatomical changes are either absent or mild.

Diagnosis.

Send to the laboratory for diagnostic tests:

– bovine brain after testing for rabies and other viral infections after the diagnosis was not confirmed;

– the brain of cattle from meat processing plants (0. 01% of slaughtered animals older than 3 years).

Pathological material (brain) is taken from animals with clinical signs of damage to the central nervous system). In this case, the brain for research must be taken from animals immediately after their slaughter or death.

Specific prophylaxis.

BSE does not produce either cellular or humoral immunity, so no vaccine has been developed in the world to date.

Treatment.

Treatment is ineffective, the prognosis for the disease is unfavorable.

Prevention.

The basis of prevention for prosperous countries is: preventing the importation of pedigree cattle, meat, canned food, by-products and semi-finished products, meat and bone meal, semen, embryos, technical fat, intestinal raw materials and other products and feeds of animal origin from ruminants; careful control over purchases of breeding stock and biological tissues, especially from disadvantaged countries; prohibition of feeding ruminants with meat-and-bone and bone meal from cattle and sheep.

If BSE is suspected, contact a veterinary clinic.

In order to prevent bovine spongiform encephalopathy, owners of susceptible animals must strictly comply with the requirements provided for in paragraph 7 of the Veterinary Rules for the implementation of preventive, diagnostic, restrictive and other measures, the establishment and lifting of quarantine and other restrictions aimed at preventing the spread and eliminating foci of large spongiform encephalopathy approved (hereinafter referred to as the Veterinary Rules), including paragraphs 6 and 7 of clause 7 of the Veterinary Rules regarding the incineration of waste from the slaughter of susceptible animals and the prohibition of feeding animals meat and bone, bone meal, protein briquettes, as well as other feed and feed additives for animals containing ruminant proteins in their composition.

“Mad cow disease” is gaining momentum – RBC

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