Bovine Spongiform Encephalopathy

​​​​​ This page outlines how the Australian Government, in partnership with state and territory governments and industry, has prevented BSE from entering the Australian cattle herd. It also outlines how Australia is prepared to mount an animal health response in the highly unlikely event of a BSE case in Australian cattle. Much of the information on this page is sourced from Australia’s Transmissible Spongiform Encephalopathy Freedom Assurance Program website.
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Background

Transmissible Spongiform Encephalopathies

TSEs in animals are a class of rare brain diseases that are associated with the accumulation of abnormal prion protein in the brain and therefore affect the central nervous system. These diseases are very rare, fatal and are characterised by spongy degeneration of the brain. There are no validated live animal tests, no treatments and no vaccines for these diseases.

There are a number of TSEs which variously affect people and animals. Of most interest to Australia's livestock industries are:

  • bovine spongiform encephalopathy (BSE) which affects cattle and other ruminants and is commonly referred to as "mad cow disease"
  • scrapie, a TSE which affects sheep and goats
  • chronic wasting disease (CWD) which is a TSE that affects cervids such as mule deer, white tail deer and Rocky Mountain elk
  • feline spongiform encephalopathy (FSE) which is found in domestic cats and captive exotic cats
  • transmissible mink encephalopathy (TME) which is a very rare disease of farmed mink
  • Creutzfeldt-Jakob disease (CJD) which is a rare and fatal form of TSE that affects humans worldwide. A new form of CJD, called variant Creutzfeldt-Jakob disease (vCJD) was identified in 1996 as the human form of BSE linked to the consumption of certain tissues of BSE-infected cattle.
Bovine Spongiform Encephalopathy

BSE is a progressive, fatal disease that affects the nervous system of cattle.

Australia is internationally recognised as a ‘negligible risk’ country for BSE.

In common with other TSEs, BSE has a typical incubation period of four to six years.  Most cases are seen in cattle over four years of age. Signs of the disease can appear gradually, however once clinical signs have appeared the affected animals die within two weeks to six months. There is no fever. Nervous signs are always present, but variable, and can fall into three categories:

  • changes in behaviour, such as nervousness, apprehension and frenzy when cattle are confronted by gateways and other obstacles
  • abnormal posture and gait, such as staggering, swaying, high lifting of legs when walking, tremors, falling over and being unable to get up when lying down, and
  • extreme sensitivity to sound and touch.

A number of proven tests for BSE are available, but not in live cattle. A combination of these proven tests may be used on suspect cases. These include examining slices of the brain through a microscope, with or without staining with special antibodies, and a number of rapid tests which rely on detecting the abnormal protein that accumulates in certain parts of the brain.

The World Organisation for Animal Health (OIE) sets international standards for BSE in the Terrestrial Animal Health Code. The OIE has developed a three category country classification system assessing members as either ‘negligible’, ‘controlled’ or ‘undetermined’ risk countries.

As of September 2009, 11 countries have been assessed as ‘negligible risk’: Argentina, Australia, Chile, Finland, Iceland, New Zealand, Norway, Paraguay, Singapore, Sweden and Uruguay.

As of September 2009, 32 countries have received a ‘controlled risk’ assessment: Austria, Belgium, Brazil, Canada, Chinese Taipei, Colombia, Cyprus, Czech Republic, Denmark, Estonia, France, Germany, Greece, Hungary, Ireland, Italy, Japan, Latvia, Lichtenstein, Lithuania, Luxembourg, Malta, Mexico, Netherlands, Poland, Portugal, Slovak Republic, Slovenia, Spain, Switzerland, United Kingdom and United States.

The OIE also recognises that only certain cattle tissues present a risk of transmitting BSE and recommends that these be removed from cattle from BSE affected countries at slaughter if they are above a certain age. These tissues are tonsils, part of the small intestine, brains, eyes, spinal cord, skull and vertebral column. These OIE recommendations on tissue removal do not apply to Australia.

The OIE BSE standard in the Terrestrial Animal Health Code is available from the OIE website. A disease card that provides further technical information on the disease is also available from the OIE website.

Occurrences in Australia

Australia was one of the first countries to implement effective quarantine measures against BSE entering the country (see the section Australia’s approach to BSE). Australia meets international requirements for a BSE negligible risk country. This has been confirmed by national and international risk assessments, for example those conducted by the Food Standards Australia New Zealand, the New Zealand Food Safety Authority and the European Food Safety Authority. An assessment by the OIE was released in May 2007 and concluded that Australia met the requirements for a 'negligible BSE risk' country.

BSE, CWD and TME have never been recorded in Australia. Scrapie has occurred once, in imported sheep on a single property in 1952 and was promptly eradicated. Two cases of FSE have been diagnosed in imported animals in Australian zoos in 1992 (cheetah) and 2002 (Asiatic golden cat). In both cases exposure before importation to feeds derived from BSE affected cattle are thought to have caused the disease. In both instances, effective response measures were taken.

The Global Context - BSE

BSE was first reported in the UK in 1986. It is thought to have originated from the ingestion by cattle of scrapie infected material from sheep, although the original source of BSE infection remains under debate.

Changes in rendering practices that occurred in the UK around 1980 are believed to have allowed increased survival of the BSE agent in meat and bone meal used as an ingredient in cattle feed. In this way the BSE agent was recycled in the UK cattle population. Due to the long BSE incubation period of several years, this recycling continued unabated without obvious clinical signs of disease, eventually leading to the UK epidemic.

Other European countries were also affected, although at a much lower rate than the UK, as a result of importing infected live cattle and stockfeed (meat and bone meal) from the UK. These include Austria, Belgium, Czech Republic, Denmark, Finland, France, Germany, Greece, Ireland, Italy Liechtenstein, Luxembourg, the Netherlands, Poland, Portugal, Slovakia, Slovenia, Switzerland, Spain, and Sweden.

A series of countermeasures were introduced by the UK and other countries to break the cycle of cattle re-infection. These measures included a ruminant protein feed ban as well as bans on importation of live cattle and ruminant protein from infected countries. The feed ban helped to reduce the BSE epidemic in Europe, with the numbers of BSE cases there tailing off after 2000. This evidence suggests that BSE may eventually be eradicated. Outside of Europe, other countries that have diagnosed indigenous cases of BSE include Israel, Japan, Canada and the United States. Again, these cases can be linked to the importation of cattle and/or meat and bone meal from Europe.

Relevant information available from the OIE

International BSE incidences

The OIE Terrestrial Animal Health Code (the Code) aims to assure the sanitary (disease related) safety of international trade in land based animals and their products. This is achieved through the detailing of health measures to be used by the veterinary authorities of importing and exporting countries to avoid the transfer of agents pathogenic for animals or humans, while avoiding unjustified sanitary barriers. The Code Chapter on BSE details international standards for measures used in preventing the trade related transfer of BSE between countries.

Links to BSE information in other countries:

United States
Canada
The European Union
United Kingdom
New Zealand

Australia’s Approach to BSE

Summary

For beef producing countries such as Australia, the issues surrounding BSE are highly significant. Australia currently meets international criteria for a BSE negligible risk country, and this can only be assured by continuing to apply vigorous risk reduction measures, verified by an ongoing surveillance program. Through the government/industry Transmissible Spongiform Encephalopathy Freedom Assurance Program (TSEFAP), these processes are well coordinated, nationally uniform, transparent and auditable in order to demonstrate BSE freedom and maintain access to export markets for cattle, beef and by-products.

The Australian Government recognises that despite the best efforts to exclude serious animal diseases such as BSE, it is possible incursions could occur. Preparedness arrangements for large scale responses to emergency animal diseases are therefore a key element in Australia’s defences. Through the Council of Australian Governments, Australia’s preparedness to mount an effective, whole of government animal health response has been reviewed and upgraded. Furthermore, these arrangements are tested by disease simulation exercises to ensure a high level of preparedness.

How could BSE be introduced to Australia?

Key factors in the epidemiology of BSE are well established. These factors point to three main pathways for the possible introduction of the BSE disease agent to a country’s cattle herd.

Importation of certain animals from TSE-affected countries

Live cattle cannot be imported into Australia from BSE affected countries. The cattle that are still alive from the limited number of previous imports have been permanently identified utilising the National Livestock Identification Scheme (NLIS). They are also in official ‘lifetime quarantine’ so that they will never enter the human food or animal feed chains. Risk assessments have shown that there is a negligible risk that BSE was introduced into Australia by importation of these cattle. Information on the NLIS can be found at the Meat and Livestock Australia website.

Importation of contaminated feedstuff originating from BSE affected countries

The importation of animal-derived meat and bone-meal (except for fishmeal) from all countries except New Zealand was banned in Australia in 1966 as a measure against the importation of anthrax spores. Risk based import controls minimise the chance that other imported stockfeeds or stockfeed ingredients have been cross-contaminated with meat and bone meal. Current import policies are detailed on the Biosecurity Australia website.

Importation of biologicals contaminated with the BSE agent

Quarantine risk assessments have been made of vaccines and other biological materials that involve cattle and sheep products in their manufacture and that are intended for use in animals. Restrictions on the importation of these products have been extended in line with emerging knowledge of the BSE status of countries throughout the world. Current Biosecurity Australia policies are detailed on the Biosecurity Australia website.

Stringent controls are in place against the introduction of BSE through these pathways. In the unlikely event that the BSE agent is introduced through one of these pathways, the legislated bans in Australia on feeding ruminant animals meat and bone meal derived from mammals, birds or fish (i.e. restricted animal material) will prevent BSE being propagated and amplified.

Transmissible Spongiform Encephalopathy Freedom Assurance Program (TSEFAP)

Prior to establishment of the TSEFAP, the many animal TSE risk reduction measures in Australia were managed and funded in different ways. The TSEFAP integrates all TSE measures into one national program with clear and nationally integrated operational components and a transparent funding framework. Information on the TSEFAP can be found at the Animal Health Australia website.

The purpose of TSEFAP is to enhance market confidence that Australian animals and animal products are free from TSEs through the nationally integrated management of animal-related TSE activities. The purpose of the program will be served by meeting the following objectives:

  • to carry out sufficient surveillance to meet international requirements and assure trading partners, markets and consumers that Australian animals and animal products are free of TSEs and to ensure the early detection of a TSE (should it occur)
  • to demonstrate that no restricted animal material is fed to ruminants
  • to manage the risks posed by animals imported from countries that have had cases of TSE
  • to provide a nationally coordinated approach to TSE-related research and development
  • to communicate Australia's favourable status for TSEs consistently and efficiently
  • to ensure Australia is adequately prepared to address any TSE case, should it occur
  • to identify emerging TSE-related issues and provide a framework for their management, if required
  • to provide a forum to involve all stakeholders in addressing animal-related TSE issues, and
  • to increase the efficiency and consistency of management of animal-related TSE activities.

TSEFAP activities fall into six project areas:

  • surveillance (the NTSESP and any other required surveillance)   
  • ruminant feeding restrictions
  • imported animal surveillance (including zoo animals) and 'buy-back' schemes for imported cattle
  • research and development 
  • management and coordination, and   
  • communications.

In developing the TSEFAP Business Plan, major stakeholders including all agricultural authorities from all Australian Governments, industry bodies representing producers, meat processors and the stockfeed animal feeding industry, and Food Standards Australia New Zealand.

Surveillance

Active surveillance for BSE commenced in Australia in 1990 and was modified in 1998 with the development of the National Transmissible Spongiform Encephalopathy Surveillance Program (NTSESP), which is a core activity of TSEFAP. The primary purpose of the program is to support trade by maintaining a surveillance system for BSE that is consistent with the OIE Terrestrial Animal Health Code, verifying that Australia’s BSE risk reduction measures are effective.

The NTSESP is a targeted component of Australia's overall disease surveillance effort. The overall system comprises both general and targeted surveillance. General surveillance comprises a wide range of activities to maintain a continuous watch over the livestock disease profile so that unexpected changes can be recognised. Activities include pre and post slaughter inspection at meatworks, inspection of animals at sale yards and other points of aggregation, farm visits by private and government veterinarians and results from laboratory testing.

Raising local awareness among veterinary practitioners and producers of the need to report nervous diseases in cattle is carried out by government officers, relevant industry organisations and veterinary practitioners. Training of practitioners and government officers includes awareness of BSE and other TSEs of animals, nature of the diseases, selection of eligible animals as well as specimen collection and laboratory submission to ensure the occurrence of TSEs in Australian livestock can be ruled out and alternative diagnoses confirmed whenever possible.

Current policies, programs and links to international sites are detailed at the Animal Health Australia website.

Ruminant Feed Ban

In accordance with a recommendation from the World Health Organisation, a voluntary ban on the feeding of ruminant meat and bone meal to ruminants was adopted in Australia in 1996. This acted as a fail-safe control measure to rule out the possibility that feeding will amplify the BSE agent in the unlikely event that it was introduced to Australia. Laws to enact the ban were introduced in all Australian jurisdictions in 1997. The ban has since been extended to 'restricted animal material', being any meal derived from animal origin including fish and birds. A uniform national approach to official inspection and auditing, coupled with industry-based quality assurance programs and third party audits, verifies compliance with the ban. A number of education initiatives have been undertaken to ensure that all industry segments are aware of their responsibilities. Current policies and programs are detailed at the Animal Health Australia website.

Imported Cattle Quarantine and Surveillance

Australia suspended the importation of cattle from the UK in 1988 and from other European countries in 1991, and from other BSE affected countries from the date the disease was first reported.

Cattle imported from countries that subsequently have reported BSE cases have been traced, and those remaining alive at the time have been placed under official permanent quarantine in accordance with Section 55A, Regulation 36 of the Commonwealth Quarantine Act (1908). This section of the Act prohibits the unauthorised movement of cattle, or their sale for slaughter, and ensures that their carcases will be disposed of in an approved manner upon death or destruction. All of these cattle have been permanently identified in accordance with the NLIS and their details recorded in the NLIS database. These measures allow the normal commercial management of the animals, but prohibit their use for the production of human or animal food.

Some imported cattle were slaughtered before BSE was reported in their country of origin. However, scientific risk assessments have shown that there is a negligible likelihood that BSE became established within the Australian cattle herd as a result of the importation of cattle.

Current policies and programs are detailed at the Animal Health Australia website.

Emergency Preparedness

The Memorandum of Understanding (MOU) – National response to a foot-and-mouth disease outbreak PDF [113kb], is the result of an agreement by the Council of Australian Governments that an outbreak of foot-and-mouth disease and its consequences must be managed on a national basis. While this MOU primarily focuses on foot-and-mouth disease, it could be adapted by agreement of the parties for handling other major animal disease emergencies such as BSE. The MOU sets out a national coordination framework to ensure close integration of responsibilities and actions within and across jurisdictions which builds on existing animal disease and emergency management plans. The MOU is at the Council of Australian Governments (COAG) website.

The Emergency Animal Disease Response Agreement is a landmark agreement that has been ratified by Australia's governments and livestock industries, with the aim of ensuring a rapid and efficient response to exotic animal disease incursions in Australia's livestock sector. The agreement is a world first and includes mechanisms for formal government/industry consultation on resource allocation, funding, training and risk mitigation. Details of the agreement are at the Animal Health Australia website.

The Australian livestock industries and governments have taken a proactive approach to being prepared for a BSE case. The AUSVETPLAN Disease Strategy for BSE provides a technical response plan that describes the animal health response a BSE case in Australia. The document was first produced in 1996 and has been updated since. It sets out guidance on control and eradication of BSE and is based on sound analysis and linking policy, strategies, implementation, coordination and emergency-management plans.

The AUSVETPLAN is available from the Animal Health Australia website.

These plans are regularly tested through simulation exercises and reviewed in light of new scientific information.

Timeline

This timeline outlines how Australia has prevented BSE from entering the Australian cattle herd.
DateInternationalAustralia
1952
Scrapie in Australia - promptly eradicated
1966
Australia bans imports of stockfeed and ingredients of animal origin, except for products from New Zealand and fishmeal.
1984UK suspects first BSE case.
1986UK identifies BSE. Southwood working party formed to provide advice on BSE.
1988BSE notifiable in the United Kingdom. Ban on ruminant protein from sheep and cattle feeds in the UK.Australia suspends imports of live cattle from the UK and Ireland.
1989

Southwood report states BSE is unlikely to be a threat to human health.

Tyrell Committee established to advise on research priorities for BSE.

Spongiform Encephalopathy Advisory Committee (SEAC) established.

EU bans UK cattle born before 18 July 1988 and offspring of affected or suspect cows.

Specified bovine offal banned from use in human food in the UK.


1990

First case of feline spongiform encephalopathy confirmed.

UK Chief Medical Officer states beef is safe to eat.

Australia commences a surveillance program involving the examination of the brains of cattle that will identify BSE.

Imported UK cattle place under quarantine observation.

1991Reports of BSE in France and Switzerland.Australia extends the restrictions on live cattle to include France and Switzerland. Imported French and Swiss cattle place under quarantine observation.
1992SEAC states that existing measures should protect human health.
1993United Kingdom Chief Medical Officer reiterates that beef is safe to eat.
1994BSE shown to be orally transmissible in cattle.
1996

SEAC announced probable link between BSE and vCJD.

EU bans British beef.

UK bans cattle older than 30 months from the food chain.

WHO issues recommendations on banning the feeding of ruminant meat and bone meal to ruminants.

Livestock industry adopts voluntary ban on the feeding of ruminant-derived meat-and-bone-meal (MBM) to ruminants.

Imported cattle from countries that are now BSE affected were traced and those remaining alive placed under lifetime quarantine.

BSE AUSVETPLAN manual released.

1997

Phillips Inquiry established.

OIE BSE Chapter adopted.

Legislation is passed in all States and Territories banning ruminant-derived MBM being fed to ruminants.
1998

National TSE Surveillance Program established.

First national audit of the ruminant feed ban.

1999
Ban on ruminant-derived MBM being fed to ruminants further extended to cover the feeding of specified mammalian material to ruminants in all states and territories.
2000

Phillips Inquiry report published.

First indigenous cases of BSE discovered in Germany, France, Italy and Spain.

Australia assessed by the EC as Geographical BSE Risk Level I:(most favourable).

Second national audit of the ruminant feed ban.

2001

EU suspends the use of meat and bone meal in foodstuffs for farm animals and will test all animals aged over 30 months destined for human consumption.

It also extends the list of specified risk materials to include the entire intestine of bovines.

Japan announces first case of BSE.

Biosecurity Australia completes a risk assessment that finds that there was negligible risk that BSE was introduced to Australia through the importation of European cattle.

Third and fourth national audits of the ruminant feed ban.

Imported Japanese cattle traced and placed in lifetime quarantine.

Ruminant feed ban extended to include feeding all animal materials to ruminants, with the internationally recognised exceptions of gelatin, milk and tallow.

2002

COAG MOU on national response finalised.

2003

Canada announces first case of BSE.

US identifies BSE in an imported Canadian cow.

Ongoing audit program for ruminant feed ban in accordance with national guidelines.

Imported US and Canadian cattle traced and placed in lifetime quarantine.

BSE AUSVETPLAN manual – major update.

2004

Australia established the TSEFAP. This nationally coordinated program will incorporate the NTSESP, as well as established surveillance, compliance, research and development initiatives that are already undertaken by both government and industry participants.

Biosecurity Australia completes a risk assessment that finds that there was a negligible risk that BSE was introduced to Australia through the importation of US or Canadian cattle.

Australia assessed as most favourable BSE status by New Zealand and European Commission.

2005US announces first indigenous (atypical) BSE case.

BSE AUSVETPLAN manual - updated.

TSEFAP website launched

2006

OIE concludes that Australia meets the requirements of a 'BSE free' country.
2007OIE concludes that the following countries meet 'Negligible BSE Risk' requirements - New Zealand , Argentina, Uruguay, Singapore; or 'Controlled BSE Risk' requirements - Brazil, Canada, Chile, Switzerland, Chinese Taipei, United States.OIE concludes that Australia meets the requirements of a 'BSE negligible risk' country (this category supersedes the previous 'BSE free' category)
2008OIE concludes that the following countries meet 'Negligible BSE Risk' requirements - Finland, Iceland, Norway, Paraguay, Sweden; or 'Controlled BSE Risk' requirements - Austria, Belgium, Cyprus, Czech Republic, Denmark, Estonia, France, Germany, Greece, Hungary, Ireland, Italy, Latvia, Lichtenstein, Lithuania, Luxembourg, Malta, Mexico, Netherlands, Poland, Portugal, Slovak Republic, Slovenia, Spain, United Kingdom. 
2009OIE concludes that the following countries meet 'Negligible BSE Risk' requirements - Chile; or 'Controlled BSE Risk' requirements - Colombia, Japan

Information released under the FOI Act

The department received a Freedom of Information Act 1982 request (FOI 2013/14-63) for information regarding the conditions applied to British cattle in 1990 under the terms of Bovine spongiform encephalopathy (BSE) quarantine surveillance.

The department’s search was restricted to four hardcopy files, chosen by the applicant from a list previously provided under FOI 2013/14-55. All four files were opened in 1990.

In response, seven documents were released to the applicant on 24 July 2014:

Related links

These are links to Australian and international online information about bovine spongiform encephalopathy (BSE) in animals and Creutzfeldt-Jakob disease (both variant and classical) in humans.

Australian links

International links