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BSE: Bovine Spongiform    Encephalopathy

• About the disease
• General resources
• Reports and inquiries
• Creutzfeldt-Jakob Disease (CJD)
• Recommended research databases and indexes
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This page is maintained with the assistance of Dr Robert Dixon of the University of Sydney, Faculty of Veterinary Science and Dr Andrew Turner.

By Dr Robert Dixon and Dr Andrew Turner

Also known as "mad cow disease", this is one of a number of transmissible encephalopathies that affect a range of species. Its cause is considered to be due to a 'prion', a mutant protein that is able to infect cells and induce the natural non-mutant protein in these cells to change its conformation into the mutant protein. This mutant protein proliferates and accumulates, inducing cellular disfunction and death.

There is a long incubation in the disease with an insidious onset of signs. Central nervous signs include behavioural changes where animals become apprehensive or more nervous when having to go through gates etc. Animals will also show abnormalities in gait and posture including hindlimb ataxia and hypermetria. Affected cattle may also be hyperaesthetic reacting to sound or touch. All of these signs may be present. Increasing debility and weight loss occurs and progression to death can take up to several months.

The important feature of the mutant prion proteins of TSE diseases are that they are relatively indestructible compared to normal brain cell proteins by being resistant to formalin and most disinfectants and highly resistant to heat. Temperatures of 1340C in steam at 3 bar pressure for 20 min is required to destroy prion activity.

The prion diseases are spread by ingestion, absorption through broken skin or iatrogenically through injection or transplants. Following ingestion, prion proteins can be demonstrated in tonsil and gastrointestinal lymphoid tissue and prion proteins are seen subsequently in the brain in areas associated with innervation of such lymphoid tissues.

Bovine Spongiform Encephalopathy BSE - APHIS
Published by the United States Department of Agriculture (USDA), Animal and Plant Health Inspection Service (APHIS), this site provides a wide range of information on BSE and other Transmissible Spongiform Encephalopathies (TSE's), including Creutzfeldt-Jakob Disease (CJD), Transmissible Mink Encephalopathy (TME), Scrapie and Chronic Wasting Disease of deer and elk. Included is the USDA /APHIS and Food Safety and Inspection Service, Bovine Spongiform Response Plan Summary. This gives information about the disease including clinical signs, causative agent, diagnosis, transmission, similar diseases of humans and other animals, USA and international data. Some documents are in .pdf format and require Adobe Acrobat Reader.

Bovine Spongiform Encephalopathy (BSE) and Other Animal Related Transmissible Spongiform Encephalopathies
Published by the United States Department of Agriculture (USDA), this site is a bibliohgraphy of selected resources on Bovine Spongiform Encephalopathy (BSE) and other related Transmissible Spongiform Encephalopathies. A brief introduction anout the disease is also provided.

Bovine Spongiform Encephalopathy (BSE)
The Food Standards Australia New Zealand website features a collection of reports, media releases and fact sheets discussing the Australian response to overseas outbreaks of BSE.

Manual of Bovine Spongiform Encephalopathy
Written by John W. Willesmith, Veterinary Laboratory Agency, Surrey, UK and published by the FAO of the UN. This detailed manual looks at the epidemiology, clinical signs, and diagnosis of BSE as well as control measures and public health implications.

AUSVETPLAN: Bovine Spongiform Encephalopathy
Version 3.1 published in 2005 by the Primary Industries Ministerial Council. Part of the Australian Veterinary Emergency Plan, this is a strategy for dealing with outbreaks of BSE. It includes information about the disease and transmission methods, and outlines disease control principles.

Prion Diseases
This is a chapter from the electronic version of the text Principles of Molecular Virology, Third Edition by Alan J. Cann of the University of Leicester, UK. It discusses prion diseases (spongiform encephalopathies) in humans and animals, encompassing infection, pathogenesis and treatment.

Information concerning BSE for the scientific world
This site is edited by Stephen Dealler, a medical microbiologist at the Pathology Laboratory at the Bunrnley General Hospital in the UK. It is constantly updated and scientists can input data on-line. There are links to BSE research data and statistics, reports from meetings and committees, publications, a news index and related web sites. There is also a comprehensive address book of researchers in the BSE field.

BSE - Bovine Spongiform Encephalopathy "Mad Cow Disease "
This University of Illinois site gives comprehensive and current information on BSE, TSEs and CJD. The scope is historical (timelines, epidemic maps and plots of outbreaks) as well as current opinion and practice. There is a useful list of definitions of TSE-related terms and abbreviations and information on the clinical signs, transmission, tests and recommended preventative measures. Various theories on the nature of TSE disease transmission are explained. There is also information on human TSEs. This site contains frames.

Bovine spongiform encephalopathy (BSE) - USFDA
Information is provided by the US Food and Drug Administration (FDA) and other US Government sources. Fact sheets, vaccine related publications and guidance documents aimed at both consumers and professionals are included on this web site. As at June 2001 the United States was BSE and nvCJD free.

Bovine Spongiform Encephalopathy (BSE) - WHO
Published by the World Health Organization this website is a collection of resources on BSE and CJD. It includes full text versions of WHO publications as well as articles from other sources. Many of these are in .pdf format.

Bovine spongiform encephalopathy (BSE) - USDA
Published by the Foreign Agricultural Service of the US Department of Agriculture, this website lists a series of links to US and EU government reports guidelines, and directives.

Bovine Spongiform Encephalopathy (BSE) - DEFRA
The information in this site is supplied by the Department for Environment, Food & Rural Affairs in the UK. It covers the symptoms of BSE and gives detailed information on epidemiology, pathogenesis, diagnosis and transmission. There are links to articles on TSE and CJD.

Bovine Spongiform Encephalopathy (BSE) - CFIA
This fact sheet published by the Canadian Food Inspection Agency covers the basics of BSE including symptoms, transmission and diagnosis. There is also a FAQ section and links to Creutzfeldt-Jakob Disease (CJD) resources.

Bovine Spongiform Encephalopathy (BSE) - CBER
Published by the Center for Biologics Evaluation and Research, an agency of the USFDA with the the authority to regulate biological products and ensure their safety for the American public. The site has links to resources dicussing vaccines manufactured with bovine-derived materials, vaccination, and the transmission of CJD.

Bovine Spongiform Encephalopathy (BSE)
Published by the Center for Veterinary Medicine, an agency of the USFDA. Features links to resources focussing on prevention of BSE, and feed regulation, inspection and enforcement.

BSE and CJD Information and Resources
Published by the National Center for Infectious Diseases (US), this website contains a series of articles, fact sheets and updates on various aspects of BSE and CJD.

Fact Sheet: Bovine Spongiform Encephalopathy (BSE) - WHO
This Fact Sheet has been prepared by the World Health Organization in Geneva. It covers the causes of BSE, case studies and preventative measures. There is information on TSEs in animals and on CJD in humans. The final section covers the work done by WHO on TSEs, their advice on research and recommendations for control of the disease.

BSE - Food Standards Agency (UK)
Published by the Food Standards Agency (UK), an independent food safety watchdog set up by an Act of Parliament in 2000 to protect the public's health and consumer interests in relation to food. The website contains resources on BSE including facts and figures, BSE and beef, news updates, an in depth guide to the BSE, and a world wide news feed.

Fact Sheet: Bovine Spongiform Encephalopathy (BSE) - WHO
This Fact Sheet has been prepared by the World Health Organization in Geneva. It covers the causes of BSE, case studies and preventative measures. There is information on TSEs in animals and on CJD in humans. The final section covers the work done by WHO on TSEs, their advice on research and recommendations for control of the disease.

Bovine Spongiform Encephalopathy (BSE) - EU
Published by the European Union as part of its Food Safety: from the farm to the fork website. It includes links to pages on general information, scientific advice, legislation, inspection and control, impact on trade, and statistics. Information is available in several European languages including English.

BSE Monthly Reports
Published by the Consumer's Association (UK), this website is an archive of monthly reports from January 2002 onwards. The reports contain general, scientific and statistical information and are in .pdf format.

Pathology of mad cow disease
Resource prepared by Michelle Fleetwood, DVM, DACVP of the Department of Veterinary Pathology, Armed Forces Institute of Pathology. Outlines the pathogenesis, clinical presentation, and diagnosis of bovine spongiform encephalopathy (BSE) and includes gross and microscopic images.

Bovine Spongiform Encephalopathy(BSE)
This report was written in 1993 by Richard H. Kimberlin of the Scrapie and Related Diseases Advisory Service (SARAD), Edinburgh, UK, and published by the Food and Agriculture Organization (FAO). It provides an introduction to the disease including its aetiology, epidemiology, clinical signs, pathology including the protocol for histopathological diagnosis, diagnosis, strategies for prevention, geographical distribution, economic implications, control and eradication. It also covers related diseases: scrapie; transmissible mink encephalopathy (TME); Kuru and Creutzfeldt-Jakob disease (CJD). A list of references is provided.

Bovine Spongiform Encephalopathy (BSE) Inquiry
The BSE Inquiry was announced in the UK Parliament on 22 December 1997, and set up on 12 January 1998, to establish and review the history of the emergence and identification of BSE and new variant CJD in the UK, and of the action taken in response to it up to 20 March 1996; to reach conclusions on the adequacy of that response, taking into account the state of knowledge at the time. The enquiry reported on these matters to the Minister of Agriculture, Fisheries and Food, the Secretary of State for Health and the Secretaries of State for Scotland, Wales and Northern Ireland. This site contains the full text of the final report, as well as copies of evidence that emerged during the inquiry.

Creutzfeldt-Jakob Disease (CJD)

Australia's Health Response to bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD).
This Web site has been developed by the Department of Health and Aged Care to provide comprehensive, accurate information on measures taken by the Commonwealth Government to protect the health of the Australian public. This page carries specific media releases and fact sheets related to BSE and CJD.

The UK Creutzfeldt-Jakob Disease Surveillance Unit
This is the Web site of the CJD surveillance unit based at the Western General Hospital in Edinburgh, Scotland. The site summarises the research in progress at the CJD unit and provides some background information about CJD and other human spongiform encephalopathies. Links to other resources and contrary points of view available on the Web are also provided.

Variant CJD
This is Volume 8 of The BSE Inquiry: The Report. The BSE Inquiry was set up by the UK Parliament in 1998 to investigate BSE and a new variant CJD in the UK. This volume reviews the work of the CJD Surveillance Unit and details the diagnosis, treatment and care of CJD patients.

New Variant CJD: Fact Sheet
Published by the US Department of Health and Human Services. Provides brief details and statistics of cases of new variant CJD and points out prevention measures.

Variant CJD
Information sheet published by the Alzheimer's Society (UK). Examines the link between variant CJD and BSE as well as discussing its symptoms and diagnosis.

Updated projections of future vCJD deaths in the UK
A research article published in BMC Infectious Diseases 2003 3:4. The death rate from vCJD appears to be decreasing.

Research databases and indexes

Recommended research databases and indexes for this area of study are: BIOSIS Previews, CAB Abstracts, MEDLINE, AGRICOLA and Web of Science.