mad-cow disease

ウシ海綿状脳症 bovine spongiform encephalopathy BSE。狂牛病

WordNet

1. an impairment of health or a condition of abnormal functioning
2. female of domestic cattle: "`moo-cow is a childs term" (同)moo-cow
3. mature female of mammals of which the male is called `bull
4. a large unpleasant woman
5. caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological
6. informal terms for a mother (同)mama, mamma, mom, momma, mommy, mammy, mum, mummy

PrepTutorEJDIC

1. (体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
2. 女性の話術芸人 =diseur
3. 『雌牛』,乳牛 / (鯨・象・サイなどの)雌
4. …'を'おどす
5. 『気が狂った』,気違いの / ばかげた,無謀の,向こう見ずな / 《補語にのみ用いて》《話》(人・物事に)夢中になって,(…を)熱望して《+『about』(『for』)+『名』》 / 《補語にのみ用いて》《話》『怒った』,腹を立てた(angry) / 狂ったように興奮した,熱狂した / (イヌが)狂犬病にかかった
6. 病気にかかった / 病的な,不健全な(morbid)
7. 《M-》《話》おかあちゃん(mamma)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/04/14 19:52:31」(JST)

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Image of a cow with BSE. A feature of such disease is the inability of the infected animal to stand.

Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is a fatal neurodegenerative disease (encephalopathy) in cattle that causes a spongy degeneration in the brain and spinal cord. BSE has a long incubation period, about 30 months to 8 years, usually affecting adult cattle at a peak age onset of four to five years, all breeds being equally susceptible. In the United Kingdom, the country worst affected, more than 180,000 cattle have been infected and 4.4 million slaughtered during the eradication program.

The disease may be most easily transmitted to human beings by eating food contaminated with the brain, spinal cord or digestive tract of infected carcasses. However, it should also be noted that the infectious agent, although most highly concentrated in nervous tissue, can be found in virtually all tissues throughout the body, including blood. In humans, it is known as new variant Creutzfeldt–Jakob disease (vCJD or nvCJD), and by October 2009, it had killed 166 people in the United Kingdom, and 44 elsewhere. 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.

A British and Irish inquiry into BSE concluded the epizootic was caused by cattle, which are normally herbivores, being fed the remains of other cattle in the form of meat and bone meal (MBM), which caused the infectious agent to spread. The cause of BSE may be from the contamination of MBM from sheep with scrapie that were processed in the same slaughterhouse. The epidemic was probably accelerated by the recycling of infected bovine tissues prior to the recognition of BSE. The origin of the disease itself remains unknown. The infectious agent is distinctive for the high temperatures at which it remains viable, over 600 degrees Celsius (about 1100 degrees Fahrenheit). This contributed to the spread of the disease in the United Kingdom, which had reduced the temperatures used during its rendering process. Another contributory factor was the feeding of infected protein supplements to very young calves.

Cause

The infectious agent in BSE is believed to be a specific type of misfolded protein called a prion. Prions are not destroyed even if the beef or material containing them is cooked or heat-treated. Prion proteins carry the disease between individuals and cause deterioration of the brain. BSE is a type of transmissible spongiform encephalopathy (TSE). TSEs can arise in animals that carry an allele which causes previously normal protein molecules to contort by themselves from an alpha helical arrangement to a beta pleated sheet, which is the disease-causing 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 plaque fibers, leading to the microscopic appearance of "holes" in the brain, degeneration of physical and mental abilities, and ultimately death.

Different hypotheses exist for the origin of prion proteins in cattle. Two leading hypotheses suggest it may have jumped species from the disease scrapie in sheep, or that it evolved from a spontaneous form of "mad cow disease" that has been seen occasionally in cattle for many centuries. In the fifth century BCE, Hippocrates described a similar illness in cattle and sheep, which he believed also occurred in man. Publius Flavius Vegetius Renatus recorded cases of a disease with similar characteristics in the fourth and fifth centuries CE. The British Government enquiry took the view the cause was not scrapie, as had originally been postulated, but was some event in the 1970s that was not possible to identify.

Research in 2008 suggested that mad cow disease also is caused by a genetic mutation within a gene called the prion protein gene. The research showed, for the first time, that a 10-year-old cow from Alabama with an atypical form of bovine spongiform encephalopathy had the same type of prion protein gene mutation as found in human patients with the genetic form of Creutzfeldt–Jakob disease (genetic CJD). This form of Creutzfeldt-Jakob disease is called variant CJD.

Epidemic in British cattle

Cattle are naturally herbivores, eating grass. However in modern industrial cattle-farming, various commercial feeds are used, which may contain ingredients including antibiotics, hormones, pesticides, fertilizers, and protein supplements. The use of meat and bone meal, produced from the ground and cooked leftovers of the slaughtering process, as well as from the carcasses of sick and injured animals such as cattle or sheep, as a protein supplement in cattle feed was widespread in Europe prior to about 1987. Worldwide, soybean meal is the primary plant-based protein supplement fed to cattle. However, soybeans do not grow well in Europe, so cattle raisers throughout Europe turned to the cheaper animal byproduct feeds as an alternative. The British Inquiry dismissed suggestions that changes to processing might have increased the infectious agents in cattle feed, 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." (The prion causing BSE is not destroyed by heat treatment.)

The first confirmed animal to fall ill with the disease occurred in 1986 in the United Kingdom, and lab tests the following year indicated the presence of BSE; by November 1987, the British Ministry of Agriculture accepted it had a new disease on its hands. Subsequently, 165 people (until October 2009) contracted and died of a disease with similar neurological symptoms subsequently called (new) variant Creutzfeldt-Jakob disease (vCJD). 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 the UK – one each in the Republic of Ireland, Canada and the United States of America. There is also some concern about those who work with (and therefore inhale) cattle meat and bone meal, such as horticulturists, who use it as fertilizer. Up-to-date statistics on all types of CJD are published by the National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) in Edinburgh, Scotland.

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.^{[citation needed]} Disease incidence also appears to correlate with slaughtering practices that led to the mixture of nervous system tissue with mince and other beef. An estimated 400,000 cattle infected with BSE entered the human food chain in the 1980s.^{[citation needed]} 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 fewer than five 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 known.

The scientific consensus is that infectious BSE prion material is not destroyed through cooking procedures, meaning that even contaminated beef foodstuffs prepared "well done" may remain infectious. In fact the infectious agent remains viable, over 600 degrees Celsius (about 1100 degrees Fahrenheit)

Alan Colchester, a professor of neurology at the University of Kent, and Nancy Colchester, writing in the 3 September 2005 issue of the medical journal The Lancet, proposed a theory that the most likely initial origin of BSE in the United Kingdom was the importation from the Indian Subcontinent of bone meal which contained CJD-infected human remains. The government of India vehemently responded to the research calling it "misleading, highly mischievous; a figment of imagination; absurd," further adding that India maintained constant surveillance and had not had a single case of either BSE or vCJD. The authors responded in the 22 January 2006 issue of The Lancet that their theory is unprovable only in the same sense as all other BSE origin theories are and that the theory warrants further investigation.

Epizootic in the United Kingdom

During the course of the investigation into the BSE epizootic, an enquiry was also made into the activities of the Department of Health and its Medicines Control Agency (MCA). On 7 May 1999, David Osborne Hagger, a retired civil servant who worked in the Medicines Division of the Department of Health between 1984 and 1994, produced a written statement to the BSE Inquiry in which he gave an account of his professional experience of BSE.

In February 1989, the MCA had been asked to "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." In July, "[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." In September, the BSE Working Party of the Committee on the Safety of Medicines (CSM) 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. The CSM 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, the European Commission "[expressed] concerns about the possible transmission of the BSE/scrapie agent to man through use of certain cosmetic treatments."

In 1992, sources in France reported to the MCA "that BSE had now been reported in France and there were some licensed surgical sutures derived from French bovine material." Concerns were also raised at a CSM meeting "regarding a possible risk of transmission of the BSE agent in gelatin products."

The ban on British beef

The BSE crisis led to the European Union banning exports of British beef with effect from March 1996; the ban would last for 10 years before it was finally lifted on 1 May 2006 despite attempts in May 1996 by British prime minister John Major to get the ban lifted. The ban resulted in trade controversies between the UK and other EU states, dubbed "beef war" by media.

Russia was proceeding to lift the ban sometime after November 2012 after 16 years; the announcement was made during a visit by the UK's chief veterinary officer Nigel Gibbens.

It was successfully negotiated that beef from Wales was allowed to be exported to the Dutch market, which had formerly been an important market for Northern Irish beef. Of two approved export establishments in the United Kingdom in 1999, one was in Scotland – an establishment to which live beef was supplied from Northern Ireland. As the incidence of BSE was very low in Northern Ireland – only six cases of BSE by 1999 – partly due to the early adoption of an advanced herd tagging and computerization system in the region, calls were made to remove the EU ban on exports with regard to Northern Irish beef.

Epidemiology

Country	BSE cases	vCJD cases
Austria	5	0
Belgium	133	0
Canada	17	2
Czech Republic	28	0
Denmark	14	0
Falkland Islands	1	0
Finland	1	0
France	900	27
Germany	312	0
Greece	1	0
Hong Kong	2	0
Republic of Ireland	1,353	4
Israel	1	0
Italy	138	2
Japan	26	1
Liechtenstein	2	0
Luxembourg	2	1
Netherlands	85	3
Oman	2	0
Poland	21	0
Portugal	875	2
Saudi Arabia	0	1
Slovakia	15	0
Slovenia	7	0
Spain	412	5
Sweden	1	0
Switzerland	453	0
Thailand		2
United Kingdom	183,841	176
United States	4	3
Total	188,579	280

Dark green areas are countries with confirmed human cases of vCJD; light green shows countries which have reported cases of only BSE.

The table to the right summarizes reported cases of BSE and of vCJD by country. (The vCJD column is by county of residence of patient at time of diagnosis and not suspected origin of beef.) 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 or older), while many cattle are slaughtered younger 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 low levels in blood or urine, and no other signal has been found. Newer tests are faster, more sensitive, and cheaper, so future figures possibly may be more comprehensive. Even so, currently the only reliable test is examination of tissues during a necropsy.

It is notable^{[citation needed]} that there are no cases reported in Argentina, Australia, Brazil, New Zealand, Uruguay, and Vanuatu, where cattle are mainly fed outside on grass pasture and, mostly in Australia, grain feeding is done only as a final finishing process before the animals are slaughtered for meat.^{[citation needed]}

As for vCJD in humans, autopsy tests are not always done, so those figures, too, are likely to be too low, but probably by a lesser fraction. In the United Kingdom, anyone with possible vCJD symptoms must be reported to the Creutzfeldt-Jakob Disease Surveillance Unit. In the United States, 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 CDC.

In order to control potential transmission of vCJD within the United States, the American Red Cross has established strict restrictions on individuals' eligibility to donate blood. Individuals who have spent a cumulative time of 3 months or more in the United Kingdom between 1980 and 1996, or a cumulative time of 5 years or more from 1980 to present in any combination of country(ies) in Europe, are prohibited from donating blood.

North America

The first reported case in North America was in December 1993 from Alberta, Canada. Another Canadian case was reported in May 2003. The first known U.S. occurrence came in December of the same year, though it was later confirmed to be a cow of Canadian origin imported to the U.S. Canada announced two additional cases of BSE from Alberta in early 2005. In June 2005, Dr. John R. Clifford, chief veterinary officer for the United States Department of Agriculture animal health inspection service, confirmed a fully domestic case of BSE in Texas.

Practices in the United States relating to BSE

Soybean meal is cheap and plentiful in the United States. The 1.5 million tons of cottonseed meal produced in the U.S. every year that is not suitable for humans or any other simple-stomach animal is even cheaper than soybean meal. Historically, meat and bone meal, blood meal and meat scraps have almost always commanded a higher price as a feed additive than oilseed meals in the U.S., so there was not much incentive to use animal products to feed ruminants. 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 ruminants such as cattle and goats. However, the byproducts of ruminants can still be legally fed to pets or other livestock, including pigs and poultry, such as chickens. In addition, it is legal for ruminants to be fed byproducts from some of these animals. A proposal to end the use of cattle blood, restaurant scraps, and poultry litter (fecal matter, feathers) in January 2004 has yet to be implemented.

In February 2001, the USGAO reported the FDA, which is responsible for regulating feed, had not adequately enforced the various bans. Compliance with the regulations was shown to be extremely poor before the discovery of a cow in Washington state infected with BSE in 2003, but industry representatives report that compliance is now total. Even so, critics call the partial prohibitions insufficient. Indeed, U.S. meat producer Creekstone Farms was forcibly prevented from conducting BSE testing by the USDA, who under an obscure 1913 law had the authority to restrict sales of BSE testing kits, allegedly to protect other producers from being forced to conduct the same tests to stay competitive.

The USDA has issued recalls of beef supplies that involved introduction of downer cows into the food supply. Hallmark/Westland Meat Packing Company was found to have used electric shocks to prod downer cows into the slaughtering system in 2007. Possibly due to pressure from large agribusiness, the United States has drastically cut back on the number of cows inspected for BSE.

Effect on the US beef industry

Japan was the top importer of US beef, buying 240,000 tons valued at $1.4 billion in 2003.^{[citation needed]} After the discovery of the first case of BSE in the US on 23 December 2003, Japan halted US beef imports. In December 2005, Japan once again allowed imports of US. beef, but reinstated its ban in January 2006 after a violation of the US-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 US pressure to resume imports, ignoring consumer worries about the safety of US beef, said Japanese consumer groups. Michiko Kamiyama from Food Safety Citizen Watch and Yoko Tomiyama from Consumers Union of Japan said about this: "The government has put priority on the political schedule between the two countries, not on food safety or human health."

Sixty-five nations implemented full or partial restrictions on importing U.S. beef products because of concerns that US testing lacked sufficient rigor. As a result, exports of US beef declined from 1,300,000 metric tons in 2003, (before the first mad cow was detected in the US) to 322,000 metric tons in 2004. This has increased since then to 771,000 metric tons in 2007.

On 31 December 2006, Hematech, Inc, a biotechnology company based in Sioux Falls, South Dakota, announced 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.

In April 2012, some South Korean retailers ceased importing beef from the United States after a case of BSE was reported. Indonesia also suspended imports of beef from the US after a dairy cow with mad cow disease was discovered in California.

Brazil

On 8 December 2012, the Japanese government issued a ban on imports of raw beef from Brazil, based on reports that a cow which died in 2010 in southern Brazil carried disease-carrying proteins. The Brazilian Ministry of Agriculture maintains that its beef is free of BSE. The Brazilian Ministry of Agriculture informed that the animal in question didn't manifest the disease and didn't die of this cause.

Japan

With 36 confirmed cases, Japan experienced one of the largest number of cases of BSE outside Europe. It was the only country outside Europe and the Americas to report non-imported cases. Reformation of food safety in the light of the BSE cases resulted in the establishment of a governmental Food Safety Commission in 2003.

Clinical signs in cattle

Cows affected by BSE are usually apart from the herd and will show progressively deteriorating behavioural and neurological signs. One notable sign is an increase in aggression. Cattle will react excessively to noise or touch and will slowly become ataxic.

Systemic signs of disease, such as a drop in milk production, anorexia and lethargy, are present.

Diagnosis

Diagnosis of BSE continues to be a practical problem. It has an incubation period of months to years, during which there are no symptoms, though the pathway of converting the normal brain prion protein (PrP) into the toxic, disease-related PrP^Sc form has started. At present, there is virtually no way to detect PrP^Sc reliably except by examining post mortem brain tissue using neuropathological and immunohistochemical methods. Accumulation of the abnormally folded PrP^Sc form of PrP is a characteristic of the disease, but it is present at very low levels in easily accessible body fluids such as blood or urine. Researchers have tried to develop methods to measure PrP^Sc, but no methods for use in materials such as blood have been accepted fully.

The traditional method of diagnosis relies on histopathological examination of the medulla oblongata of the brain, and other tissues, post mortem. Immunohistochemistry can be used to demonstrate prion protein accumulation.

In 2010, a team from New York described detection of PrP^Sc even when initially present at only one part in a hundred billion (10⁻¹¹) in brain tissue. The method combines amplification with a novel technology called Surround Optical Fiber Immunoassay (SOFIA) and some specific antibodies against PrP^Sc. After amplifying and then concentrating any PrP^Sc, the samples are labelled with a fluorescent dye using an antibody for specificity and then finally loaded into a microcapillary tube. This tube is placed in a specially constructed apparatus so it is totally surrounded by optical fibres to capture all light emitted once the dye is excited using a laser. The technique allowed detection of PrP^Sc after many fewer cycles of conversion than others have achieved, substantially reducing the possibility of artefacts, as well as speeding up the assay. The researchers also tested their method on blood samples from apparently healthy sheep that went on to develop scrapie. The animals’ brains were analysed once any symptoms became apparent. The researchers could therefore compare results from brain tissue and blood taken once the animals exhibited symptoms of the diseases, with blood obtained earlier in the animals’ lives, and from uninfected animals. The results showed very clearly that PrPSc could be detected in the blood of animals long before the symptoms appeared. After further development and testing, this method could be of great value in surveillance as a blood or urine-based screening test for BSE.

Control

A ban on feeding cattle meat and bone meal has resulted in a reduction in cases in countries where the disease was present. In disease-free countries, control relies on import control, feeding regulations and surveillance measures.

At the abattoir in the UK, the brain, spinal cord, trigeminal ganglia, intestines, eyes and tonsils from cattle are classified as specified risk materials, and must be disposed of appropriately.

See also

• Health crisis
• Meat on the bone
• US beef imports in Japan
• US beef imports in Taiwan
• US beef imports in South Korea, 2008 US beef protest in South Korea
• Beef war

References

Further reading

• Altman, Lawrence K. "Four States Watching for Brain Disorder." New York Times, 9 April 1996a.
• Altman, Lawrence K. "U.S. Officials Confident That Mad Cow Disease of Britain Has Not Occurred Here." New York Times, 27 March 1996d: 12A.
• "Apocalypse Cow: U.S. Denials Deepen Mad Cow Danger." PR Watch, 3.1 (1996): 1–8
• Berger, Joseph R., et al. "Creutzfeldt-Jakob Disease: A Ten-Year Experience." Neurology, 44 (1994): A260.
• Bleifuss, Joel. "Killer Beef." In These Times, 31 May 1993: 12–15.
• Boller F., Lopez O. L., Moossy J. (1989). "Diagnosis of Dementia". Neurology 38 (7 Suppl 2): 76–79. doi:10.1212/WNL.39.1.76. PMID 3290719. 
• Boule, Margie. "Despite Anecdotal Evidence, Docs Say No Mad Cow Disease Here." The Oregonian, 16 April 1996: C01.
• "Brain Disease May Be Commoner Than Thought – Expert." Reuters Information Service, 15 May 1996.
• Brayne, C. "How Common are Cognitive Impairment and Dementia?" Dementia and Normal Aging, Cambridge University Press, 1994.
• Brown Paul (1989). "Central Nervous System Amyloidoses". Neurology 39 (8): 1103–1104. doi:10.1212/WNL.39.8.1103. 
• Davanpour, Zoreth, et al. (1993) "Rate of Creutzfeldt-Jakob Disease in USA." Neurology, 43: A316.
• Flannery, Mary. "Twelve – Fifteen 'Mad Cow' Victims a Year in Area." Philadelphia Daily News, 26 March 1996: 03.
• Folstein, M. "The Cognitive Pattern of Familial Alzheimer's Disease." Biological Aspects of Alzheimer's Disease. Ed. R. Katzman. Cold Spring Harbor Laboratory, 1983.
• Gruzen, Tara. "Sheep Parts Fail to Cause Mad Cow Disease in U.S. Test." Seattle Times, 29 March 1996: A11.
• Hager, Mary and Mark Hosenball. "'Mad Cow Disease' in the U.S.?" Newsweek, 8 April 1996:58–59.
• Harrison Paul J., Roberts Gareth W. (1991). "'Life, Jim, But Not as We Know It'? Transmissible Dementias and the Prion Protein". British Journal of Psychiatry 158 (4): 457–70. doi:10.1192/bjp.158.4.457. PMID 2054560. 
• Holman R. C. et al. (1995). "Edidemiology of Creutzfeldt-Jakob Disease in the United States, 1979–1990". Neuroepidemiology 14 (4): 174–181. doi:10.1159/000109793. PMID 7643951. 
• Hoyert, Donna L. "Vital and Health Statistics. Mortality Trends for Alzheimer's Disease, 1979–1991." Washington: U.S. Dept. of Health and Human Services, 1996.
• Little Brian W. et al. (1993). "The Epidemiology of Creutzfeldt-Jakob Disease in Eastern Pennsylvania". Neurology 43: A316. 
• Mahendra, B. Dementia, Lancaster: MTP Press Limited, 1987: 174.
• Manuelidis Elias E (1985). "Presidential Address". Journal of Neuropathology and Experimental Neurology 44 (1): 1–17. doi:10.1097/00005072-198501000-00001. PMID 3880808. 
• Manuelidis Elias E., Manuelidis Laura (1989). "Suggested Links between Different Types of Dementias: Creutzfeldt-Jakob Disease, Alzheimer Disease, and Retroviral CNS Infections". Alzheimer Disease and Associated Disorders 2: 100–109. doi:10.1097/00002093-198903010-00009. 
• McKhann Guy et al. (1984). "Clinical Diagnosis of Alzheimer's Disease". Neurology 34 (7): 939. doi:10.1212/WNL.34.7.939. 
• Prusiner S (1984). "Some Speculations about Prions, Amyloid, and Alzheimer's Disease". New England Journal of Medicine 310 (10): 661–663. doi:10.1056/NEJM198403083101021. PMID 6363926. 
• Perry R.T. et al. (1995). "Human Prion Protein Gene: Two Different 24 BP Deletions in an Atypical Alzheimer's Disease Family". American Journal of Medical Genetics 60 (1): 12–18. doi:10.1002/ajmg.1320600104. PMID 7485229. 
• Scully, R. E., et al. "Case Records of the Massachusetts General Hospital." New England Journal of Medicine, 29 April 1993: 1259–1263.
• Teixeira, F., et al. "Clinico-Pathological Correlation in Dementias." Journal of Psychiatry and Neuroscience, 20 (1995): 276–282.
• United States Department of Commerce. Statistical Abstract of the United States, Washington: Bureau of the Census, 1995.
• Van Duijn, C. M. "Epidemiology of the Dementia: Recent Developments and New Approaches." Neuroepidemiology, 60 (1996): 478–488.
• Wade J. P. H. et al. (1987). "The Clinical Diagnosis of Alzheimer's Disease". Archives of Neurology 44 (1): 24–29. doi:10.1001/archneur.1987.00520130016010. PMID 3800717. 
• Wlazelek, Ann (September 23, 1990). "Fatal Brain Disease Mystifies Experts". The Morning Call. Retrieved 9 November 2013. : B01
• Wlazelek, Ann (September 27, 1990). "Scientists Try To Track Fatal Disease International Expert Visits Area To Study Unusual Incidence Rate". The Morning Call. Retrieved 9 November 2013. : B04
• "World Health Organization Consultation on Public Health Issues Related to Bovine Spongiform Encephalopathy and the Emergence of a New Variant of Creutzfeldt-Jakob Disease.", Morbidity and Mortality Weekly Report, 12 April 1996: 295–303.*/

External links

• Bovine spongiform encephalopathy at DMOZ
• OIE – World Organisation for Animal Health: BSE situation in the world and annual incidence rate

UpToDate Contents

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• 1. 変異型クロイツフェルト・ヤコブ病 variant creutzfeldt jakob disease
• 2. クロイツフェルト・ヤコブ病 creutzfeldt jakob disease
• 3. プリオンによる中枢神経系疾患 diseases of the central nervous system caused by prions
• 4. 供血者の病歴 blood donor medical history
• 5. プリオンの生物学および遺伝学 biology and genetics of prions

English Journal

• Spontaneous Obesity-Linked Type 2 Diabetes in the Absence of Islet Amyloid in a Cynomolgus Monkey Infected With Bovine Spongiform Encephalopathy.

• Strom A, Yutzy B, Kruip C, Völker I, Schloot NC, Roden M, Scott FW, Löwer J, Holznagel E.SourceGerman Diabetes Center, Leibniz Center for Diabetes Research at Heinrich-Heine University Düsseldorf, Düsseldorf, Germany.
• Veterinary pathology.Vet Pathol.2013 Feb 4. [Epub ahead of print]
• A 9-year-old cynomolgus monkey (Macaca fascicularis) infected orally with bovine spongiform encephalopathy (BSE) was presented for necropsy following euthanasia 4 years post infection (p.i.). This macaque R984 was exposed to a BSE dose that causes a simian form of variant Creutzfeldt-Jakob disease (
• PMID 23381923

• Susceptibility of domestic cats to chronic wasting disease.

• Mathiason CK, Nalls AV, Seelig DM, Kraft SL, Carnes K, Anderson KR, Hayes-Klug J, Hoover EA.SourceDepartment of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, USA.
• Journal of virology.J Virol.2013 Feb;87(4):1947-56. doi: 10.1128/JVI.02592-12. Epub 2012 Dec 12.
• Domestic and nondomestic cats have been shown to be susceptible to feline spongiform encephalopathy (FSE), almost certainly caused by consumption of bovine spongiform encephalopathy (BSE)-contaminated meat. Because domestic and free-ranging nondomestic felids scavenge cervid carcasses, including tho
• PMID 23236066

• Communicating food risks in an era of growing public distrust: three case studies.

• Lofstedt R.AbstractThe communication and regulation of risk has changed significantly over the past 30 years in Europe and to a noticeable but lesser extent in the United States. In Europe, this is partly due to a series of regulatory mishaps, ranging from mad cow disease in the United Kingdom to contamination of the blood supply in France. In the United States, general public confidence in the American government has been gradually declining for more than three decades, driven by a mix of cultural and political conflicts like negative political advertising, a corrosive news media, and cuts in regulatory budgets. While the former approach is based on an objective assessment of the risk, the latter is driven more by the perception of the risk, consumer sentiment, political will, and sectoral advocacy. In this article, the author examines three U.S.-based food case studies (acrylamide, bisphenol A, and artificial food colorings) where regulations at the local and state levels are increasingly being based on perceived risk advocacy rather than on the most effective response to the risk, be it to food safety or public health, as defined by regulatory interpretation of existing data. In the final section, the author puts forward a series of recommendations for how U.S.-based regulators can best handle those situations where the perceived risk is markedly different from the fact-based risk, such as strengthening the communication departments of food regulatory agencies, training officials in risk communication, and working more proactively with neutral third-party experts.
• Risk analysis : an official publication of the Society for Risk Analysis.Risk Anal.2013 Feb;33(2):192-202. doi: 10.1111/j.1539-6924.2011.01722.x. Epub 2011 Nov 2.
• The communication and regulation of risk has changed significantly over the past 30 years in Europe and to a noticeable but lesser extent in the United States. In Europe, this is partly due to a series of regulatory mishaps, ranging from mad cow disease in the United Kingdom to contamination of the
• PMID 22050421

Japanese Journal

• BSE阻止の決め手 発病前にプリオンをつかめ (感染症の脅威--パンデミックへの備えは万全か) -- (対策は十分か?)

• Prusiner Stanley B.
• 別冊日経サイエンス (163), 110-119, 2008-11
• NAID 40016355966

• 情報ワイド番組における「ニュース・ストーリー」の構成と理解の実践過程^[○!R] : BSE問題における「リスク」を事例に

• 是永 論,酒井 信一郎
• マス・コミュニケーション研究 (71), 107-128, 2007-07-30
• This paper attempts to understand risk, namely that of BSE in Japan from morning news programs. We analyzed broadcast from January and February 2006 that examined BSE and the ensuing ban on import of …
• NAID 110006421055

• 食育とシンボル再編の社会的力学 : 「スローフード」と政策形成過程

• 中村 麻理
• フォーラム現代社会学 (6), 118-130, 2007-05-26
• 食育基本法が2005年に成立し、食育推進は国民の責務であるとされた。基本法で規定された食育の内容は広範なものであり、健康の増進や食料自給率の向上、食の安心・安全の確保といった課題に加え、感謝の念を育むといった側面が強調されている。このように食育には規範的傾向があるため、存在意義が自明視され、その政策形成過程はあまり問い直されることがなかった。そこで、第1の課題として、食育政策のスタート地点にさかの …
• NAID 110009564787

Related Links

• Official Mad Cow Disease Home Page

The Internet's largest and most authoritative site for prions, mad cow disease (bovine spongiform encephalopathy or BSE), scrapie, Creutzfeldt-Jakob Disease (CJD and nvCJD), kuru,and chronic wasting disease (CWD). Over 6966 ...

• mad cow diseaseの意味 - 英和辞書 - 英語辞書 - goo辞書

mad cow diseaseとは。意味や和訳。[U]牛海綿状脳症，狂牛病. 正式にはBSE (bovine spongiform encephalitis). - goo英和辞書は14万項目以上を収録し、発音、音声、慣用句、例文が分かる英和辞書です。

Related Pictures

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★リンクテーブル★

関連記事	「disease」「cow」「c」

「disease」

　　[★]

• n.

疾患：illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)

特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)

• something that is very wrong with people's attitudes, way of life or with society.

関

ail、ailment、disease entity、disorder、ill、illness、malady、sick、sickness

• 注意

disease ≠ illness ≠ disorder

　 　 　

「cow」

　　[★]

• n.

• 雌ウシ、乳牛

　 　 　 　 　

「c」

　　[★]

• 光速, speed of light