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

22.08.202308.07.2023 by alexxlab

What is Mad Cow Disease. How does BSE spread. What measures are in place to prevent BSE. How does BSE affect human health. What are the economic impacts of BSE outbreaks. How is BSE diagnosed and monitored. What is the current global status of BSE.

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The Origins and Nature of Mad Cow Disease

Mad Cow Disease, scientifically known as Bovine Spongiform Encephalopathy (BSE), is a progressive neurological disorder affecting cattle. This fatal brain disease belongs to a family of diseases called transmissible spongiform encephalopathies (TSEs), which are caused by prions – abnormally folded proteins that can trigger a cascade of neurological damage.

What causes BSE? The disease is believed to be caused by cattle consuming feed contaminated with the rendered remains of other cattle or animals infected with prions. These prions can survive standard rendering processes, allowing them to persist in animal feed and subsequently infect healthy cattle.

Key Characteristics of BSE

Long incubation period (2-8 years)
Progressive neurological symptoms
Always fatal
No cure or treatment available

How does BSE affect cattle? Infected animals typically show changes in behavior, such as aggression, nervousness, or lack of coordination. As the disease progresses, affected cattle may lose weight, experience decreased milk production, and eventually become unable to stand.

The First U.S. Case: A Turning Point

On December 23, 2003, the United States faced a significant agricultural crisis when the U.S. Department of Agriculture (USDA) announced the first presumptive diagnosis of BSE in the country. This case, involving an adult Holstein cow from Washington State, sent shockwaves through the American beef industry and prompted immediate action from regulatory authorities.

What were the immediate actions taken after the discovery? The USDA quickly initiated a comprehensive investigation and recall process:

Confirmation of diagnosis by an international reference laboratory in Weybridge, England
Trace-back investigation based on ear-tag identification
Genetic testing to determine the origin of the infected cow
Recall of beef from cattle slaughtered in the same plant on the same day

The investigation revealed that the BSE-infected cow had been imported from Canada in August 2001, highlighting the importance of robust import regulations and surveillance programs.

BSE Surveillance and Diagnostic Procedures

The discovery of the first U.S. case of BSE underscored the critical importance of effective surveillance and diagnostic procedures. How are potentially infected animals identified? The USDA’s Animal and Plant Health Inspection Service conducts targeted surveillance, focusing particularly on non-ambulatory or “downer” cows, which may be at higher risk for BSE.

What is the process for BSE diagnosis?

Initial screening of brain tissue samples
Histopathology examination
Immunohistochemical testing
Confirmation by international reference laboratories

The National Veterinary Services Laboratory in Ames, Iowa, plays a crucial role in the initial diagnosis of BSE cases in the United States. However, international confirmation is sought to ensure accuracy and maintain global confidence in the diagnostic process.

The Human Health Risk: Variant Creutzfeldt-Jakob Disease

The discovery of BSE raised significant concerns about potential transmission to humans. What is the human form of BSE? Strong evidence indicates that BSE can be transmitted to humans, causing a variant form of Creutzfeldt-Jakob disease (vCJD), a rare and fatal neurological disorder.

How significant is the risk to human health? While the potential for transmission exists, the risk appears to be relatively low due to several factors:

Substantial species barrier protecting humans from widespread illness
Removal of high-risk tissues during slaughter
Stringent feed regulations and surveillance programs

As of December 1, 2003, a total of 153 vCJD cases had been reported worldwide, with 143 cases occurring in the United Kingdom. This concentration of cases in the UK is likely due to the extensive BSE outbreak that occurred there in the 1980s and 1990s.

Global Monitoring and Surveillance Efforts

The global nature of the BSE threat has necessitated comprehensive international monitoring and surveillance efforts. In the United States, multiple agencies and organizations collaborate to track and study prion diseases:

Centers for Disease Control and Prevention (CDC)
National Center for Health Statistics
Council of State and Territorial Epidemiologists
American Association of Neuropathologists (AANP)
National Prion Disease Pathology Surveillance Center

How does the CDC monitor CJD trends in the United States? The CDC analyzes death certificate information from U.S. multiple cause-of-death data and conducts follow-up reviews of clinical and neuropathology records for CJD decedents younger than 55 years of age.

What role does the National Prion Disease Pathology Surveillance Center play? Established in 1996-1997, this center provides free, state-of-the-art diagnostic services to U.S. physicians and helps monitor the possible occurrence of emerging forms of prion diseases, such as vCJD, in the United States.

Prevention Strategies and Import Restrictions

Preventing the spread of BSE is crucial for protecting both animal and human health. What measures have been implemented to prevent BSE from entering and spreading within the United States?

Severe restrictions on the importation of live ruminants (cattle, sheep, and goats)
Limitations on importing certain ruminant products from countries with known BSE cases
Extended restrictions to include ruminants and products from all European countries
Regulations on the use of ruminant tissue in animal feed

Why are feed regulations important in BSE prevention? The use of ruminant tissue in animal feed is considered a primary risk factor for BSE transmission. By implementing strict controls on feed ingredients and production processes, authorities aim to break the cycle of infection and prevent new cases from occurring.

Enhanced Feed Regulations

What specific feed regulations have been implemented to combat BSE?

Ban on the use of most mammalian protein in feeds for ruminant animals
Prohibition of high-risk tissue materials in all animal feed
Stringent requirements for feed production facilities to prevent cross-contamination
Regular inspections and testing of feed ingredients and finished products

Economic Impact and Trade Implications

The discovery of BSE cases can have severe economic consequences for affected countries. How does BSE impact the beef industry and international trade?

Immediate bans on beef imports from affected countries
Loss of consumer confidence in beef products
Increased costs for surveillance, testing, and prevention measures
Potential long-term damage to export markets

What was the economic impact of the first U.S. BSE case? The 2003 discovery led to immediate import bans from numerous countries, causing a significant drop in U.S. beef exports. The USDA estimated that the BSE crisis cost the U.S. beef industry billions of dollars in lost export value and decreased domestic consumption.

How have countries worked to regain market access after BSE outbreaks? Affected nations have implemented comprehensive BSE control measures, enhanced surveillance programs, and engaged in extensive negotiations with trading partners to demonstrate the safety of their beef products and regain market access.

Advancements in BSE Research and Diagnostics

Ongoing research into BSE and other prion diseases has led to significant advancements in our understanding and ability to combat these disorders. What are some key areas of BSE research?

Prion biology and pathogenesis
Improved diagnostic techniques
Potential treatments for prion diseases
Genetic factors influencing susceptibility

How have diagnostic techniques for BSE improved? Recent years have seen the development of more sensitive and rapid testing methods, including:

Real-time quaking-induced conversion (RT-QuIC) assays
Protein misfolding cyclic amplification (PMCA)
Advanced neuroimaging techniques

These advancements allow for earlier detection of BSE and other prion diseases, potentially improving surveillance efforts and reducing the risk of transmission.

Emerging Therapeutic Approaches

While there is currently no cure for BSE or vCJD, researchers are exploring various therapeutic approaches. What are some promising avenues for treatment?

Immunotherapies targeting prion proteins
Small molecule compounds to stabilize normal prion protein conformation
RNA interference techniques to reduce prion protein expression
Stem cell therapies for neuronal regeneration

Although these approaches are still in experimental stages, they offer hope for future treatment options for both animal and human prion diseases.

Global BSE Status and Ongoing Challenges

Since the peak of the BSE crisis in the 1990s, global efforts to control the disease have shown significant success. What is the current global status of BSE?

Dramatic reduction in BSE cases worldwide
Many countries now classified as having “negligible BSE risk” by the World Organisation for Animal Health (OIE)
Continued surveillance and prevention measures in place

Despite these successes, challenges remain in the fight against BSE and other prion diseases. What are some ongoing concerns?

Potential for atypical BSE cases in older cattle
Maintaining effective surveillance in low-incidence environments
Balancing regulatory measures with industry needs
Addressing public concerns and maintaining consumer confidence

How are countries adapting their BSE strategies? Many nations are moving towards more targeted, risk-based surveillance approaches, focusing resources on high-risk populations while maintaining overall vigilance against the disease.

International Collaboration and Knowledge Sharing

The global nature of the BSE threat has highlighted the importance of international collaboration in disease control efforts. How do countries work together to combat BSE?

Sharing of surveillance data and research findings
Harmonization of diagnostic standards and procedures
Coordinated response to new BSE cases or emerging prion diseases
Joint research initiatives and funding programs

These collaborative efforts help ensure a unified and effective global approach to BSE prevention and control, ultimately benefiting both animal and human health worldwide.

The Future of BSE Management and Prevention

As our understanding of BSE and prion diseases continues to evolve, so too must our approaches to management and prevention. What future developments can we anticipate in BSE control?

Advanced genetic testing to identify BSE-resistant cattle breeds
Development of prion-free animal feed alternatives
Implementation of blockchain technology for enhanced traceability in the beef supply chain
Continued refinement of risk assessment models and surveillance strategies

How might climate change impact BSE risk? Researchers are beginning to explore potential links between environmental factors and prion disease prevalence. Future BSE prevention strategies may need to consider the effects of changing ecosystems and agricultural practices in the context of a warming planet.

Emerging Technologies in BSE Research

What cutting-edge technologies are being applied to BSE research?

CRISPR gene-editing techniques for studying prion protein function
Artificial intelligence algorithms for predicting protein misfolding
Advanced proteomics for identifying biomarkers of early infection
3D organoid models for studying prion disease progression in vitro

These technologies offer the potential for significant breakthroughs in our understanding of BSE and related disorders, potentially leading to more effective prevention and treatment strategies in the future.

As we continue to grapple with the challenges posed by BSE and other prion diseases, ongoing research, international collaboration, and adaptive management strategies will be crucial in protecting both animal and human health. The lessons learned from the BSE crisis have not only improved our ability to prevent and control this specific disease but have also enhanced our overall approach to emerging zoonotic threats, providing valuable insights for addressing future public health challenges.

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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