View source for Bovine spongiform encephalopathy

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{{Infobox_Disease |
  Name           = {{PAGENAME}} |
  Image          = Aphis.usda.gov BSE 3.jpg|
  Caption        = Classic image of a cow with BSE. A notable feature of such disease is the inability (of the infected animal) to stand.<p> <small> Source: APHIS </small>|
  DiseasesDB     = 1559|
  ICD10          = |
  ICD9           = |
  ICDO           = |
  OMIM           = |
  MedlinePlus    = |
  MeshID         = |
}}
{{Bovine spongiform encephalopathy}}

{{CMG}}

{{SK}} Mad cow disease; BSE
==Overview==

'''Bovine spongiform encephalopathy'''  is a fatal, [[neurodegenerative]] disease of cattle, which infects by a mechanism that surprised biologists upon its discovery in the late 20th century. In the UK, the country worst affected, 179,000 cattle were infected and 4.4 million killed as a precaution.<ref name=Brown>Brown, David. [http://www.telegraph.co.uk/news/main.jhtml?xml=/news/2000/10/27/nbse527.xml "The 'recipe for disaster' that killed 80 and left a £5bn bill"], ''The Daily Telegraph'', June 19, 2001.</ref>

It is believed that the disease may be transmitted to human beings who eat infected carcasses.<ref>[http://www.cfsan.fda.gov/~comm/bsefaq.html Commonly Asked Questions About BSE in Products Regulated by FDA's Center for Food Safety and Applied Nutrition (CFSAN)] US Food and Drug Administration. September 14 2005. Accessed August 21 2007.</ref> In humans, it is known as new variant [[Creutzfeldt-Jakob disease]] (vCJD or nvCJD), and by June 2007, it had killed 165 people in Britain, and six elsewhere<ref>[http://www.cjd.ed.ac.uk/vcjdworld.htm "Variant Creutzfeld-Jakob Disease, June 2007"], The National Creutzfeldt-Jakob Disease Surveillance Unit, Edinburgh University. The number of dead in the UK from Creutzfeld-Jakob Disease had reached 1,206 by June 4, 2007.</ref> with the number expected to rise because of the disease's long incubation period. Between 460,000 and 482,000 BSE-infected animals had entered the human food chain before controls on high-risk offal were introduced in 1989.<ref>[http://news.bbc.co.uk/1/hi/health/1671737.stm "CJD deaths 'may have peaked'"], BBC News, November 13, 2001.</ref>

A British inquiry into BSE concluded that the epidemic was caused by feeding cattle, who are normally [[herbivore]]s, the remains of other cattle in the form of meat and bone meal (MBM), which caused the infectious agent to spread.<ref name=DEFRA/BSE>[http://www.defra.gov.uk/animalh/bse/controls-eradication/causes.html "BSE: Disease control & eradication - Causes of BSE"], Department for Environment, Food, and Rural Affairs, March 2007.</ref><ref>[http://www.bseinquiry.gov.uk/ "The BSE Inquiry"], led by Lord Phillips of Worth Matravers, report published October 2000.</ref> The origin of the disease itself remains unknown. The current scientific view is that infectious proteins called [[prion]]s developed through spontaneous mutation, probably in the 1970s, and there is a possibility that the use of [[Organophosphate|organophosphorus pesticides]] increased the susceptibility of cattle to the disease.<ref>[http://www.bseinquiry.gov.uk/report/volume1/execsum4.htm "Volume 1: Findings and Conclusions. Executive Summary of the Report of the Inquiry. 3. The cause of BSE"], Phillips Inquiry, October 2000.</ref> The infectious agent is distinctive for the high temperatures it is able to survive; this contributed to the spread of the disease in Britain, which had reduced the temperatures used during its [[Rendering (food processing)|rendering]] process.<ref name=DEFRA/BSE/> Another contributory factor was the feeding of infected protein supplements to very young calves.<ref name=DEFRA/BSE/><ref name=Harden>Harden, Blaine. [http://archives.seattletimes.nwsource.com/cgi-bin/texis.cgi/web/vortex/display?slug=madcowdairy28&date=20031228 "Supplements used in factory farming can spread disease"], ''The Washington Post'', December 28, 2003.</ref>

== Infectious agent ==
[[Image:Aphis.usda.gov BSE 5.jpg|thumb|200px|left|Microscopic "holes" of tissue sections are examined in the lab. <small>Source: APHIS</small>]]

The infectious agent in BSE is believed to be a specific type of misfolded [[protein]] called [[prion]]. Misfolded prion proteins carry the disease between individuals and cause deterioration of the [[brain]]. BSE is a type of [[transmissible spongiform encephalopathy]] (TSE).<ref name = TSE>{{cite web|url=http://www.aphis.usda.gov/publications/animal_health/content/printable_version/BSEbrochure12-2006.pdf|title=An Overview of Bovine Spongiform Encephalopaphy}}</ref> TSEs can arise in animals that carry an [[allele]] which causes normal prions to contort by themselves from an alpha helical arrangement to a beta pleated sheet, which is the disease-causing [[chemical conformation|shape]] for the particular protein. Transmission can occur when healthy animals come in contact with tainted tissues from others with the disease. In the brain these proteins cause native cellular prion protein to deform into the infectious state, which then goes on to deform further prion protein in an exponential cascade. This results in protein aggregates, which then form dense [[Senile plaques|plaque]] fibers, leading to the microscopic appearance of "holes" in the brain, degeneration of physical and mental abilities, and ultimately death.

Different theories exist for the origin of prion proteins in cattle.  Two leading theories suggest that it may have jumped species from the disease [[scrapie]] in sheep, or that it evolved from a spontaneous form of "mad-cow disease" which has been seen occasionally in cattle for many centuries.<ref>New Scientist, 17 March 2007, p 11</ref> Publius Flavius Vegetius Renatus records cases of a disease with similar characteristics in the 4th and 5th Century AD.<ref>Digesta Artis Mulomedicinae, Publius Flavius Vegetius Renatus</ref> The British Government enquiry took the view the cause was not scrapie as had originally been postulated, and was some event in the 1970s which was not possible to identify.<ref>http://www.bseinquiry.gov.uk/report/volume1/execsum4.htm</ref>

== The BSE epidemic in British cattle ==

Cattle, like most other food animals, are normally [[herbivores]]. In nature, cattle eat grass or grains. In modern industrial cattle-farming, various commercial feeds are used, which may contain ingredients including [[antibiotic]]s, [[hormone]]s, [[pesticide]]s, [[fertilizer]]s, and [[protein]] supplements. 

The use of meat and bone meal, produced from the ground and cooked left-overs of the slaughtering process as well as from the cadavers of sick and injured animals such as cattle, sheep, or chickens, as a protein supplement in cattle feed was widespread in Europe prior to about 1987. Worldwide, soya bean meal is the primary plant-based protein supplement fed to cattle. However, soya beans do not grow well in Europe, so cattle raisers throughout Europe turned to the less expensive animal by-product feeds as an alternative. 

A change to the rendering process in the early 1980s may have resulted in a large increase of the infectious agents in the cattle feed. A contributing factor was suggested to have been a change in British laws that allowed a lower temperature sterilization of the protein meal. While other European countries like Germany required said animal byproducts to undergo a high temperature steam boiling process, this requirement had been eased in Britain as a measure to keep prices competitive.  

Later the British Inquiry dismissed this theory saying "changes in process could not have been solely responsible for the emergence of BSE, and changes in regulation were not a factor at all."<ref>http://www.bseinquiry.gov.uk/report/volume2/chaptea3.htm#820576</ref> 

Following an [[epizootic]] of BSE in Britain, 165 people (up until 2007) acquired and died of a disease with similar neurological symptoms subsequently called [[Creutzfeldt-Jakob disease|vCJD]], or (new) variant Creutzfeldt-Jakob disease. This is a separate disease from 'classical' [[Creutzfeldt-Jakob disease]], which is not related to BSE and has been known about since the early 1900s. 

Three cases of vCJD occurred in people who had lived in or visited Britain &mdash; one each in Ireland, Canada and the United States. There is also some concern about those who work with (and therefore inhale) cattle meat and bone meal, such as [[horticulture|horticulturists]], who use it as fertilizer. Up to date statistics on all types of CJD are published by the [http://www.cjd.ed.ac.uk UK CJD Surveillance Centre] in Edinburgh.

For many of the vCJD patients, direct evidence exists that they had consumed tainted [[beef]], and this is assumed to be the mechanism by which all affected individuals contracted it. Disease incidence also appears to correlate with slaughtering practices that led to the mixture of [[nervous system]] tissue with hamburger and other beef. 

It is estimated that 400,000 cattle infected with BSE entered the human food chain in the 1980s. Although the BSE epizootic was eventually brought under control by culling all suspect cattle populations, people are still being diagnosed with vCJD each year (though the number of new cases currently has dropped to less than 5 per year). This is attributed to the long incubation period for prion diseases, which are typically measured in years or decades. As a result the full extent of the human vCJD outbreak is still not fully known.

The scientific consensus is that infectious BSE prion material is not destroyed through normal cooking procedures, meaning that contaminated beef foodstuffs prepared "well done" may remain infectious.<ref>http://www.mayoclinic.com/health/mad-cow-disease/ID00012</ref><ref>http://www.fsis.usda.gov/Fact_Sheets/Bovine_Spongiform_Encephalopathy_Mad_Cow_Disease/index.asp</ref>

In 2004 researchers reported evidence of a second contorted shape of prions in a rare minority of diseased cattle. In other words, this implies a second strain of BSE prion. Very little is known about the shape of disease-causing prions, because their insolubility and tendency to clump thwarts application of the detailed measurement techniques of [[structural biology]]. But cruder measures yield a "biochemical signature" by which the newly discovered cattle strain appears different from the familiar one, but similar to the clumped prions in humans with traditional CJD Creutzfeldt-Jakob Disease.

The finding of a second strain of BSE prion raises the possibility that transmission of BSE to humans has been underestimated, because some of the individuals diagnosed with spontaneous or "sporadic" CJD may have actually contracted the disease from tainted beef. So far nothing is known about the relative transmissibility of the two disease strains of BSE prion.

In 2005 a controversial paper in ''[[The Lancet]]'' introduced a theory that BSE might have originated in British cattle when they ate imported animal feed that included infected human remains from [[cremation|Hindu funeral ceremonies]] in India. [http://www.guardian.co.uk/bse/article/0,2763,1561292,00.html] This paper is merely a conjecture, however, and the authors suggest only that further investigation should occur. 

===UK epizootic and UK licensed medicines===

During the course of the investigation into the BSE epizootic, an enquiry was also made into the activities of the [[British Department of Health|Department of Health]] and its Medicines Control Agency. On May 7, 1999, in his written statement [http://www.bseinquiry.gov.uk/files/ws/s476.pdf#search='David%20Osborne%20Hagger' number 476] to the BSE Inquiry, David Osborne Hagger reported on behalf of the Medicines Control Agency that in a previous enquiry the Agency had been asked to:

<blockquote>''"... identify relevant manufacturers and obtain information about the bovine material contained in children’s vaccines, the stocks of these vaccines and how long it would take to switch to other products."'' It was further reported that the: ''"... use of bovine insulin in a small group of mainly elderly patients was noted and it was recognised that alternative products for this group were not considered satisfactory."'' A medicines licensing committee report that same year recommended that: 

''"... no licensing action is required at present in regard to products produced from bovine material or using prepared bovine brain in nutrient media and sourced from outside the United Kingdom, the Channel Isles and the Republic of Ireland provided that the country of origin is known to be free of BSE, has competent veterinary advisers and is known to practise good animal husbandry."'' 

In 1990 the British Diabetic Association became concerned regarding the safety of bovine insulin and the government licensing agency assured them that: ''"... there was no insulin sourced from cattle in the UK or Ireland and that the situation in other countries was being monitored."'' In 1991 a European Community Commission: ''"... expressed concerns about the possible transmission of the BSE/scrapie agent to man through use of certain cosmetic treatments."'' Sources in France reported to the British Medicines Control Agency: ''"... that there were some licensed surgical sutures derived from French bovine material."'' Concerns were also raised: ''"... regarding a possible risk of transmission of the BSE agent in gelatin products.''"</blockquote>

==Husbandry practices in the United States relating to BSE==

Soybean meal is cheap and plentiful in the United States. As a result, the use of animal byproduct feeds was never common, as it was in Europe. However, U.S. regulations only partially prohibit the use of animal byproducts in feed. In 1997, regulations prohibited the feeding of mammalian byproducts to [[ruminant]]s such as cows and goats. 

However, the byproducts of ruminants can still be legally fed to pets or other livestock such as pigs and poultry such as chickens. In addition, it is legal for ruminants to be fed byproducts from some of these animals. [http://www.prwatch.org/books/madcow.html] A proposal to end the use of cow blood, restaurant scraps, and poultry litter(fecal matter, feathers)<ref>The term "chicken litter" also includes spilled chicken feed as well as fecal matter and feathers. It is still legal in the United States to use ruminant protein to feed chickens. Thus, ruminant protein can get into the food chain of cattle in this round about way.</ref> in January 2004 has yet to be implemented [http://www.organicconsumers.org/madcow/cow110504.cfm], despite the efforts of some advocates of such a policy, who cite the fact that cows are herbivores, and that blood and fecal matter could potentially carry BSE.

In February 2001, the USGAO reported that the [[United States Food and Drug Administration|FDA]], which is responsible for regulating feed, had not adequately policed the various bans. [http://www.organicconsumers.org/madcow/cnn22602.cfm] Compliance with the regulations was shown to be extremely poor before the discovery of the Washington cow, but industry representatives report that compliance is now 100%. Even so, critics call the partial prohibitions insufficient. 

Indeed, US meat producer Creekstone Farms alleges that the [[United States Department of Agriculture|USDA]] is preventing BSE testing from being conducted [http://www.3buddies.com/creekstone/news-appeal-response.html].

Japan was the top importer of U.S. beef, buying 240,000 tons valued at $1.4 billion in 2003. After the discovery of the first case of BSE in the U.S. on December 23, 2003, Japan stopped U.S. beef imports in December 2003. In December 2005, Japan once again allowed imports of U.S. beef, but reinstated its ban in mid-January 2006 after a technical violation of the U.S.-Japan beef import agreement: a vertebral column, which should have been removed prior to shipment, was included in a shipment of veal.

Tokyo yielded to U.S. pressure to resume imports, ignoring consumer worries about the safety of U.S. beef, said Japanese consumer groups. Michiko Kamiyama from Food Safety Citizen Watch said about this: "The government has put priority on the political schedule between the two countries, not on food safety or human health."

Possibly due to pressure from large agribusiness, the United States has drastically cut back on the number of cows inspected for BSE. <ref name="USA Today"> USA Today, August 3, 2006, archived at [http://www.usatoday.com/news/opinion/editorials/2006-08-03-our-view_x.htm] </ref>

Sixty-five nations have full or partial restrictions on importing U.S. beef products because of concerns that U.S. testing lacks sufficient rigor. As a result, exports of U.S. beef declined from $3.8 billion in 2003, before the first mad cow was detected in the US, to $1.4 billion in 2005. <ref name="USA Today"/>

On December 31, 2006, [http://www.hematech.com/ Hematech], a biotechnology company based in Sioux Falls, South Dakota, announced that it had used genetic engineering and cloning technology to produce cattle that lacked a necessary gene for prion production - thus theoretically making them immune to BSE.<ref>{{cite news |first= Rick|last=Weiss |title=Scientists Announce Mad Cow Breakthrough|url=http://www.washingtonpost.com/wp-dyn/content/article/2006/12/31/AR2006123100672.html|publisher=The Washington Post|date=[[2007-01-01]]|accessdate=2007-01-01}}</ref>

==BSE statistics by country==
{| class="wikitable" align="left" style="font-size:70%"
|-
!Country
!BSE cases
![[Creutzfeldt-Jakob disease#Variant Creutzfeldt-Jakob Disease .28vCJD.29|vCJD]] cases
!Country
!BSE cases
![[Creutzfeldt-Jakob disease#Variant Creutzfeldt-Jakob Disease .28vCJD.29|vCJD]] cases
|-
|Austria
|5
|0
|Belgium
|125
|0
|-
|Canada
|10
|1
|Czech Rep
|9
|0
|-
|Denmark
|15
|0
|Falkland Islands
|1
|0
|-
|Finland
|1
|0
|France
|900+
|11
|-
|Germany
|312
|0
|Greece
|1
|0
|-
|Hong Kong
|2
|0
|Israel
|1
|0
|-
|Italy
|117
|1
|Japan
|26
|1
|-
|Lichtenstein
|2
|0
|Luxembourg
|2
|1
|-
|Netherlands
|75
|1
|Oman
|2
|0
|-
|Poland
|21
|0
|Portugal
|875
|2
|-
|Republic of Ireland
|1353
|4
|Slovakia
|15
|0
|-
|Slovenia
|7
|0
|Spain
|412
|2
|-
|Sweden
|1
|0
|Switzerland
|453
|0
|-
|Thailand
|n/a
|2
|UK
|183823
|160
|-
|US
|3
|3
|'''Total'''
|'''188535'''
|'''170''' (+ 6 results pending)
|}

<br clear="left"/>

[[Image:Global mad cow cases map.PNG|thumb|right|400px|Dark green areas are countries that have confirmed human cases of Creutzfeldt-Jakob disease and light green are countries that have bovine spongiform encephalopathy cases.]]

The table to the right summarizes reported cases of BSE and of vCJD by country. BSE is the disease in ''cattle'', while vCJD is the disease in ''people''.

The tests used for detecting BSE vary considerably as do the regulations in various jurisdictions for when, and which cattle, must be tested. For instance, in the EU the cattle tested are older (30 months+), while many cattle are slaughtered earlier than that. At the opposite end of the scale, Japan tests all cattle at the time of slaughter. Tests are also difficult as the altered prion protein has very small levels in blood or urine, and no other signal has been found. Newer tests are faster, more sensitive, and cheaper, so it is possible that future figures may be more comprehensive. Even so, currently the only reliable test is examination of tissues during an autopsy.

It is notable that there are no cases reported in Australia and New Zealand where cattle are mainly fed outside on grass pasture and, mainly in Australia, non-grass feeding is done only as a final finishing process before the animals are processed for meat.

As for vCJD in humans, autopsy tests are not always done and so those figures too are likely to be too low, but probably by a lesser fraction. In the UK anyone with possible vCJD symptoms must be reported to the UK Creutzfeldt-Jakob Disease Surveillance Unit and so it is unlikely that any cases would be missed. In the U.S., the CDC has refused to impose a national requirement that physicians and hospitals report cases of the disease. Instead, the agency relies on other methods, including death certificates and urging physicians to send suspicious cases to the National Prion Disease Pathology Surveillance Center (NPDPSC) at Case Western Reserve University in Cleveland, which is funded by the [[Centers for Disease Control and Prevention|CDC]].

==References==
{{Reflist|2}}

==External links==
{{wikinews|Category:Bovine spongiform encephalopathy}}
{{wiktionary|Bovine spongiform encephalopathy}}
'''General/News'''
* [http://science.howstuffworks.com/mad-cow-disease.htm HowStuffWorks] Craig C. Freudenrich, Ph.D.
'''Government Pages'''
* [http://www.aphis.usda.gov/newsroom/hot_issues/bse/index.shtml APHIS - BSE Information] - U.S. Department of Agriculture
* [http://www.inspection.gc.ca/english/anima/heasan/disemala/bseesb/bseesbfse.shtml Canadian BSE FAQs] - Canadian Food Inspection Agency
* [http://www.defra.gov.uk/animalh/bse/index.html BSE Homepage] - Department for Environment, Food and Rural Affairs (UK)
* [http://www.bseinquiry.gov.uk UK government inquiry into BSE from discovery to 1996]
* [http://www.cjd.ed.ac.uk/ UK Creutzfeldt-Jakob Disease Surveillance Unit]
* [http://www.bseinquiry.gov.uk/files/ws/s476.pdf#search='David%20Osborne%20Hagger' British Medicines Control Agency statement] PDF - (Excerpts cited above.)
* [http://www.foodsafety.gov/%7Efsg/bse.html BSE Resources] - Food Safety.gov
* [http://www.ers.usda.gov/features/bse/index.htm Impact of BSE on U.S. Economy] - Economic Research Service
* [http://www.state.ma.us/dph/cdc/factsheets/madcow.htm Mad Cow FAQs] - Massachusetts Public Health
* [http://www.hc-sc.gc.ca/fn-an/securit/animal/bse-esb/index_e.html BSE (Mad Cow Disease)] - Health Canada
* [http://a257.g.akamaitech.net/7/257/2422/10apr20061500/edocket.access.gpo.gov/cfr_2006/aprqtr/21cfr589.2000.htm List of prohibited substances in ruminant feed] - U.S. Code of Federal Regulations
* [http://www.didgood.com/health/BSE/northamerica.html Bovine Spongiform Encephalopathy in North America] - enumeration of reported cattle incidents.
'''Consumer/Health Groups'''
* [http://www.organicconsumers.org/madcow.htm Mad Cow Disease News and Information] - Organic Consumers Association
* [http://pediatrics.about.com/cs/inthenews/a/madcow.htm Pediatrician's Evaluation of BSE Risk]- About.com
* [http://www.bna.com/webwatch/madcow.htm Mad Cow Information] - Bureau for National Affairs
<!-- *[http://kidshealth.org/EhcServlet.jsp?id=83CDF2AE-1739-11D4-A2D1-00508B62BE1F&cachedate=2003-12-22%2B16%3A23%3A00.0&dn=ehc&lic=97&cat_id=20028&article_set=33955 Mad Cow Disease and Children's Health] - CJW Medical Center -->
<!--  * [http://www.foodsafetynetwork.ca Mad Cow FAQs] - Food Safety Network    
 Link goes to main page only, need more specific target for BSE
-->
* [http://www.hbsef.org Human BSE Foundation] - Charity Providing Support for Families of CJD Victims
* [http://www.ific.org/publications/qa/bseqa.cfm BSE FAQs] - International Food Information Council
<!-- 404'd - *[http://www.nmagriculture.org/mad%20cow%20frms.htm BSE Info Page] - New Mexico Agriculture -->
* [http://www.i-pets.com/rpet2.html Pets and Mad Cow Disease] - BSE Information for pet owners
* [http://www.prwatch.org/books/madcow.html Mad Cow USA] - Center for Media and Democracy
* [http://www.cspinet.org/foodsafety/saferbeef.html Choosing Safer Beef to Eat] - [[Center for Science in the Public Interest]]

'''Science/Researcher Pages'''
*[http://www.thelancet.com/search/results?search_searchuri=%2Fsearch%2Fadvanced&search_resulturi=%2Fsearch%2Fresults&search_preview=no&search_reqfirst=1&search_reqcount=20&search_submode=citation&update_search=no&search_mode=journal&search_cluster=thelancet&search_discipline=cluster&journal_picker=lancet&restrictterm_lancet=lancet&restrictname_lancet=lancet&restricttype_lancet=journal&restrictdesc_lancet=The+Lancet&restrictterm_laneur=&restrictname_laneur=laneur&restricttype_laneur=journal&restrictdesc_laneur=The+Lancet+Neurology&restrictterm_laninf=&restrictname_laninf=laninf&restricttype_laninf=journal&restrictdesc_laninf=The+Lancet+Infectious+Diseases&restrictterm_lanonc=&restrictname_lanonc=lanonc&restricttype_lanonc=journal&restrictdesc_lanonc=The+Lancet+Oncology&search_volume=368&search_issue=9552&search_startpage=2061&Submit=Search BSE and blood transfusion] (The Lancet - Vol. 368, Issue 9552, 09 December 2006, Pages 2061-2067)
* [http://www.priondata.org/data/A_USBSE.html BSE update] - Prion Data News
* [http://www.cmpharm.ucsf.edu/cohen/media/images/Scientifc_American.pdf Scientific American] - Article from 2004
* [http://www.ext.nodak.edu/extpubs/ansci/beef/as1206w.htm Mad Cow Information] - North Dakota State University 
* [http://www.iowabeefcenter.org/content/bsemain.htm BSE Information Center] - Iowa State University
* [http://www.markpurdey.com/science_the_origins_of_bse.htm The Origins of BSE] - Mark Purdy

'''Beef/Cattle Industry Pages'''
* [http://www.aamp.com/foodsafety/BSEnews.asp BSE News & Information] - American Association of Meat Processors
* [http://www.afia.org Livestock Feed Industry News] - American Feed Industry Association
* [http://www.purdeyenvironment.com/ BSE: environmental causation?]

{{Prion diseases}}

[[Category:Bovine diseases]]
[[Category:Neurology]]
[[Category:Prions]]
[[Category:Transmissible spongiform encephalopathies]]
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[[id:Bovine spongiform encephalopathy]]
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