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         Creutzfeldt-jacob Disease:     more detail
  1. Chronic neurological diseases: Subacute Sclerosing Panencephalitis, progressive multifocal Leukoencephalopathy, Kuru, Creutzfeldt-Jakob Disease by Jacob A Brody, 1976

61. Haema/Áßìá
creutzfeldtjacob disease and blood transfusion. A review creutzfeldt-jacob disease (CJD) is a rare and fatal human neurodegenerative condition that
http://www.mednet.gr/eae/haema/h21-1.htm
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E-mail: eae@hol.gr Creutzfeldt-Jacob Disease and blood transfusion. A review Velsa Fakitsa, Kyriaki Sofroniadou National Blood Transfusion Centre and Blood Derivatives Unit, Nikea General Hospital, Piraeus, Greece Haema 1999; 2(1): 12-17 Key words: Creutzfeldt-Jacob • spongiform encephalopathy Creutzfeldt-Jacob disease (CJD) is a rare and fatal human neurodegenerative condition that occurs worldwide. It is classified as a transmissible spongiform encephalopathy (TSE) because of characteristic spongy degeneration of the brain and its ability to be transmitted to laboratory animals. TSE also affects a range of animal species including sheep, goat, cow, mink and deer. Bovine spongiform encephalopathy (BSE), a TSE affecting cattle, was first reported in the United Kingdom in 1986 and over 168.000 cases have been reported since then in that country alone. Relatively small numbers of cases have also been reported in native cattle in France, the Republic of Ireland, the Nertherlands, Portugal and Switzerland. Small numbers of cases have also been reported in Canada, Denmark, the Falkland islands, Germany, Italy and Oman, but solely in animals imported from the United Kingdom The United Kingdom Government made BSE a notifiable disease in June 1988 and shortly afterwards a statutory ban on the feeding of protein derived from ruminants (e.g.cattle, sheep or goat) to any ruminant was introduced. The use in the food chain of those bovine offals considerd to pose a potential risk to humans was banned in the United Kingdom in 1989 and subsequently in other European Union countries where BSE was identified.

62. Go Ask Alice!: BSE (Mad Cow Disease) From Gelatin?
What are the mathematical chances of contracting the disease from gelatin in a food creutzfeldtjacob disease (CJD) is a rare disorder that causes
http://www.goaskalice.columbia.edu/3010.html
Mac users: please note that our site is optimized for the Safari browser Colds, aches, pains, and other ailments BSE (Mad cow disease) from gelatin? Originally Published: February 25, 2005 Dear Alice, I would like to know if I should be concerned with the risk of contracting "bovine spongiform encephalopathy (BSE)" or its human variant, from the use of vitamins, supplements, over-the-counter medication, or pills prescribed by a doctor which contain gelatin? I understand that one of the main components of gelatin comes from the bones and skin of cows and that it is often imported from other countries. I also understand that it is highly unlikely that the standard processes involved in producing gelatin would ever remove or disable the BSE contamination. It is also very alarming that BSE has a long incubation period (years) whereby the infected person has no symptoms. However, when symptoms do occur, the person dies a horrible death, via a deformed and shrinking brain, usually soon thereafter. Since gelatin is used in so many food products, is it realistic to try avoiding all gelatin?

63. EDUCATION PLANET - 16 Web Sites For Mad Cow Disease
encephalopathy (BSE), scrapie, creutzfeldtjacob disease (CJD) prion disease, kuru, fatal familial insomnia (FFI), Gerstmann-Scheindler-Schinker (GSS),
http://www.educationplanet.com/search/Science/Environment/Agriculture/Mad_Cow_Di
Search 100,000+ top educational sites, lessons and more! Home Science Environment Agriculture Found 16 ' Mad Cow Disease ' Web Sites. Also for ' Mad Cow Disease 6 Lesson Plans Web Sites (1 - 10 of 16): Official Mad Cow Disease Home Page - By far the Internet's largest site for mad cow disease, bovine spongiform encephalopathy (BSE), scrapie, Creutzfeldt-Jacob Disease (CJD) prion disease, kuru, fatal familial insomnia (FFI), Gerstmann-Scheindler-Schinker (GSS), chronic wasting disease (CWD...
Grades: Cache Report Link Problem Add Comment Mad Cow Panic - learn the facts behind the beef scare, from the Why Files. POSTED 8 FEB 2001 Years after it was supposedly vanquished, mad cow disease, the brain infection that kills cows and some people who eat them, is on a comeback. The disease has spread widely in Eu...
Grades: Higher Ed Cache Report Link Problem Add Comment MAD COW DISEASE: THE BSE EPIDEMIC IN GREAT - The announcement by British health authorities that bovine spongiform encephalopathy (BSE, pictured in medulla of cow, left), also known as mad cow disease, may have been transmitted to humans has led to a chaotic situation in the UK with ripple effects o...
Grades: Cache Report Link Problem Add Comment - Welcome to the companion Web site to the NOVA program "The Brain Eater," scheduled for re-broadcast on August 17, 1999. This program retraces the scientific sleuthing that linked mad cow disease in cattle to a related brain disease in humans. Fo...

64. Exploring The BSE - NvCJD Link
creutzfeldtjacob disease (CJD), first characterized in 1921 by Alfons Jacob Interest in creutzfeldt-jacob disease recently has reached new levels due
http://www.stanford.edu/~siegelr/ajai.html
Ajai Dandekar
The Vaccine Revolution
22 March 1997
Last modified 29 August 1997
"Mad Cow Disease" and Humans: The link between Bovine Spongiform Encephalopathy and Creutzfeldt-Jacob Disease
Introduction
Creutzfeldt-Jacob Disease (CJD), first characterized in 1921 by Alfons Jacob (Jacob, 1921), remains an enigmatic disease. CJD, along with diseases as kuru, Gerstmann-Straussler-Scheinker syndrome (GSS) and Fatal Familial Insomnia (FFI), are "prional" diseases caused by large aggregations of protein in the brain ( ). These proteins cause macroscopic vacuolation of brain tissue and as a result the diseases are known as "spongiform encephalopathies." Prional diseases uniquely do not appear to rely on nucleic acids for their transmission (Griffith, 1967). Diseases such as CJD apparently are the result of a polymerization reaction in with the disease isoform of a protein (designated PrPsc, for pr ion p rotein sc rapie) causes a conversion of the normal host-encoded protein, PrPc ( c ellular), to the PrPsc isoform through an autocatalytic process (Prusiner, 1991 and Kocisko, 1994). PrPsc protein does not appear to have its amino-acid sequence modified although its glycosylation pattern almost certainly varies from PrPc. Therefore, the conversion is not a covalent altration but rather a conformational change. Since there are phenotypically distinguishable strains of CJD, it is hypothesized that each strain converts the protein in a unique manner. Interest in Creutzfeldt-Jacob Disease recently has reached new levels due largely to an unusual outbreak of CJD in Britain, which has been associated with a epizootic of another prion disease, bovine spongiform encephalopathy (BSE). The epizootic was first discovered in 1986: since then, there have been over 164 000 cases of BSE reported in the United Kingdom (Coursens, 1997), affecting almost one-third of all cattle herds. BSE is unusually species-nonspecific for a prion disease, and has been shown to cause encephalopathies in many species, including cats and ungulates, sheep and goats (Bruce, 1994). The disease has been shown, surprisingly, to be transmitted by ingestion of BSE-infected brain homogenates.

65. Prions As New Disease Agents.
transmission of the disease agent for the creutzfeldtjacob disease(CJD) to a factor in his getting creutzfeldt-jacob disease should be considered.
http://www.unsafescience.com/prion.html
Return to UnsafeScience home page.
Prions: New Epidemics for the Future?
THE FUTURE IS NOW!
Synopsis: In the media coverage of the British “Mad Cow” disease, the scientific community has been uncharacteristically quiet. This is partly because of the accidental transmission of the disease agent for the Creutzfeldt-Jacob Disease(CJD) to children in a major scientific experiment. But perhaps more important is the potential danger to scientist’s incomes from shifting public consciousness from genetic engineering to a new, non-nucleic acid disease agent, the prion. Not widely discussed is the similarity of the symptoms of CJD and Alzheimer’s disease which is not considered transmissible. But the chilling possibility is suggested by the fact that the research program of the prion discoverer has been funded as Alzheimer’s research for many years. Furthermore, drug companies are concerned that the disease may be transmitted in protein used as a base for cosmetics. Search the Web for “prion +Pruisner” and a month’s reading will appear on your screen. But why the “Prusiner?” Dr. Stanley B. Prusiner, M.D., is a professor of neurology and biochemistry at the University of California, San Francisco. He received the 1994 Albert Lasker Basic Medical Research Award for his discovery of the new class of disease-causing agents he named prions (pronounced pree-ons). The Lasker Award is the most prestigious medical research prize awarded in the United States.

66. Creutzfeldt-Jacob Disease I.
First Previous Next Last Index Home Text. Slide 15 of 30.
http://www.biomedicine.dote.hu/cselenyi/phd3/sld015.htm

67. Dictionary Page CO.UK - Agriculture
creutzfeldtjacob disease critical control point critical habitat crop acreage base crop insurance crop reports crop residue
http://agriculture.f.dictonarypage.co.uk/
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Zoonotic diseases

68. Cow Madness: Disease's U.S. Emergence Highlights Role Of Feed Ban: Science News
Also, even though variant creutzfeldtjacob disease (CJD) can be distinguished from sporadic CJD (all other), it s difficult to do so.
http://www.sciencenews.org/20040110/fob2.asp

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Week of Jan. 10, 2004; Vol. 165, No. 2 , p. 19
Cow Madness: Disease's U.S. emergence highlights role of feed ban
Ben Harder The first appearance in the United States of the cattle-killing ailment known as mad cow disease has rocked the beef industry and raised fears of an outbreak of a similar deadly brain disease in people. However, the threat to both people and animals in the United States remains low as long as the government enforces specific feed-processing and slaughter regulations, risk analysts say. The most crucial safeguard, according to the researchers, is a ban put in place in 1997 that prohibits feeding potentially infective animal parts to cattle. That practice, which provided cheap protein for animal feed, is the main route of transmission of the misshapen proteins that cause mad cow disease, which is formally known as bovine spongiform encephalopathy (BSE). The proteins, called prions, may spread when animals consume brain, spinal cord tissue, bone particles, eyes, and small intestines of infected animals. In people, prions occasionally cause the lethal brain condition known as variant Creutzfeldt-Jakob disease, or vCJD. New safety measures that the government has announced in recent weeks might further reduce the odds of a U.S. outbreak of vCJD in people, says George Gray of the Harvard Center for Risk Analysis in Boston. One of these rules would delay the sale of meat from cattle carcasses until they're tested for BSE, and another would ban or restrict the use of processing technologies that may mix bits of nerve tissue with meat.

69. Amyloid - Information & News
Abnormal prion proteins are little understood disease agents involved in causing brainwasting diseases such as creutzfeldt-jacob disease in people,
http://www.news-medical.net/?page=2&cat=&month=&year=&keyword=Amyloid&action=Key

70. Redirect To New JH HEIC Home Page
creutzfeldtjacob disease (CJD), variant CJD (vCJD), Gerstmann-Straussler Scheinker Disease (GSS), kuru, and other diseases caused by prions (small,
http://hopkins-heic.org/prevention/prions.html
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71. Annals Of Surgery - UserLogin
Collinge J. Variant creutzfeldtjacob disease. Lancet 1999; 354 (9175) 317-23. Bagot d Arc M. The otolaryngolist and creutzfeldt-jacob disease.
http://www.annalsofsurgery.com/pt/re/annos/fulltext.00000658-200202000-00026.htm
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72. BSEInfo.org The Source For Bovine Spongiform Encephalopathy Information
creutzfeldtjacob disease (CJD) and variant creutzfeldt-jacob disease (vCJD) are different diseases.Each has its own unique clinical and histopathological
http://www.bseinfo.org/resoTSE.aspx
Tuesday, September 20, 2005
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The Basic Facts Transmissible Spongiform Encephalopathies (TSEs) are a class of brain diseases, some of which affect humans while others affect animals. TSEs are associated with the accumulation of abnormal prion protein (PrP) in the brain.This abnormal protease-resistant protein is referred to as PrPRes(protease resistant protein) or PrPSc (PrP Scrapie). All mammals produce normal PrP in cells of the central nervous system and other tissues. Changes in normal PrP are believed to lead to an altered protein referred to by some scientists as a “prion” (proteinacious infectious particle). Scientists believe that when abnormal PrP comes in contact with normal PrP, it distorts the normal protein structure. Scientists do not know what factors trigger this conversion. Some believe the abnormal PrP itself causes the conversion, while others believe a virus-like entity may be involved. Most scientists agree that the accumulation of abnormal PrP in brain cells results in altered function and eventual death or loss of function of cells. 1, CJD Creutzfeldt-Jacob Disease (CJD) and variant Creutzfeldt-Jacob Disease (vCJD) are different diseases.Each has its own unique clinical and histopathological features. CJD was first characterized in the 1920s, and the Centers for Disease Control and Prevention (CDC) report the rate of CJD cases in the United States remains consistent with the rate in many other countries, which is approximately one case per million people each year. It is important to note that this incidence rate represents an average over time. Because age is a key factor in evaluating CJD distribution, and because the disease tends to strike people over the age of 55, the actual rate is higher for older people.

73. CWD Q&A - Montana Fish, Wildlife & Parks
There has been one cases of new variant creutzfeldtjacob disease diagnosed in the US, however that case was diagnosed in an individual that was born and
http://fwp.state.mt.us/hunting/chronicWD.html
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74. ISG Website:  Image Processing For The Detection Of Creutzfeldt-Jacob Disease:
Image processing for the detection of creutzfeldtjacob disease Creutzfeld-Jakob disease (CJD) is a rare fatal transmissible illness that presents,
http://www.ee.ed.ac.uk/~neural/oldISG/research/cjd.html
Home Research Conferences Publications ... ISG Talks Image processing for the detection of Creutzfeldt-Jacob disease: Home research / image processing for the detection of creutzfeldt-jacob disease /
CJD Diagnosis using MRI
Diagnosis of CJD
Creutzfeld-Jakob disease (CJD) is a rare fatal transmissible illness that presents, in most cases, with rapidly evolving dementia including ataxia, myoclonia and altered higher cortical function. Due to symptomatic similarity with other neurological disorders it can be a difficult condition to diagnose and diagnostic confirmation can only currently be made post-mortem via neuropathological examination.
Early evidence suggested Magnetic Resonance Imaging (MRI) may provide a route to in-vivo diagnosis of CJD and more promising results have since been observed in the MRI data for patients suffering from new-variant CJD (nvCJD) which is the human prion disease suspected to be causally linked to Bovine Spongiform Encephalopathy (BSE) in cattle.
Through the analysis of a large set of post-mortem MR images of the human brain, collected from patients that presented with symptoms of neurological disorder, the aim of this study is to investigate the potential of MRI data for use as an indicator of condition in suspected cases of CJD or nvCJD. Project participants: Mark Harding
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75. Page Title
He died at age 20 of creutzfeldtjacob disease, a relentlessly creutzfeldt-jacob disease (CJD) is a human spongiform encephalopathy70 whose standard
http://www.linkny.com/~civitas/page317.html
6 May 1996 home
The Public Health Implications of Mad Cow Disease
by Michael Greger , MD
references
continued

76. Man Death In Casablanca Not Caused By Mad Cow Disease, Minister
Following the spread of the creutzfeldtjacob disease (CJD), it banned in 2000 the import of beef, livestock and meat products from countries where BSE was
http://www.arabicnews.com/ansub/Daily/Day/050305/2005030527.html

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Man death in Casablanca not caused by mad cow disease, minister
Morocco, Health, 3/5/2005 Moroccan health minister, Mohamed Cheikh Biadillah, categorically denied the mad cow disease was the cause of the death of a man who passed away in a casablanca hospital.
Biadillah told on Friday the national TVM channel the first signs show the death of the man was caused by the Creutzfeldt-Jacob disease, a sporadic type of the illness discovered in 1920. The minister explained there are four types of the Creutzfeldt-Jacob disease: the sporadic type, the family type, the Iatrogene type, while the fourth type is a new variant of the disease discovered in 1994 in the United Kingdom.
Biadillah stressed Morocco took all the needed measures to prevent the mad cow disease since it appeared.
The Moroccan agriculture ministry said Thursday no cases of Bovine Spongiform Encephalopathy (BSE), have been reported so far in the cattle.
Morocco has taken preventive measures since 1990 to protect the country from the disease. Following the spread of the Creutzfeldt-Jacob Disease (CJD), it banned in 2000 the import of beef, livestock and meat products from countries where BSE was declared. It also established a monitoring system to follow up the state of the cattle.

77. DaytonDailyNews: Dayton, Ohio, News And Information
Ency. home Disease C creutzfeldtjacob disease donors that indicate a history of diagnosed or possible creutzfeldt-jacob disease rule out the use
http://www.daytondailynews.com/health/healthfd/shared/health/adam/ency/article/0
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Disease C Creutzfeldt-Jacob disease See images Overview Symptoms Treatment ... Prevention Alternative names: Transmissible Spongiform Encephalopathy; New variant CJD - "the human form of mad cow disease" Prevention Risk of transfer of the organism on equipment or tissue is minimized by the health care provider. Treatment equipment is sterilized to kill organisms that may cause the disease. Medical histories of potential cornea donors that indicate a history of diagnosed or possible Creutzfeldt-Jacob disease rule out the use of those corneas for transplantation. Most countries now have strict guidelines for management of infected cows and strict restrictions regarding what they are fed, to avoid the potential for transmission of CJD to humans. Ency. home

78. Oprah On Trial* (This Column Was First Printed In The January 19
disease was linked to an outbreak of human creutzfeldtjacob disease. disease in this country and creutzfeldt-jacob disease remains very rare.
http://www.acsu.buffalo.edu/~insrisg/nature/nw98/oprah.html
Oprah on Trial* (This column was first printed in the January 19, 1998 Buffalo News Oprah goes on trial tomorrow. The central target of the lawsuit is former cattleman Howard Lyman, who is being sued by a Texas feedlot operator for $2 million in damages plus punitive fines. Ms. Winfrey along with production and distribution companies for the Oprah Winfrey Show are secondary targets, included because her talk show last April provided the forum for Lyman's remarks and coincidentally because of their deep pockets. Isn't it interesting that the subject of that show transmissible spongiform encephalopathies should cause Ms. Winfrey and her guest so much trouble. Surely this is not a topic that could attain the ratings of the more usual lightweight talk show subjects like "Teens Who Want to Marry their Pets" or "The Eat-Everything-in-Sight Weight Loss Program." The court case is based on the application of a 1995 Texas statute designed to protect agricultural products from disparagement. Lyman said: "Bovine spongiform encephalopathy could make AIDS look like the common cold.... A hundred thousand cows per year in the United States are fine at night, dead in the morning. The majority of those cows are rounded up, ground up, fed back to other cows. If only one of them has mad cow disease, it has the potential to affect thousands." And the thousands he spoke of included humans as well as cows.
Consider some background. Transmissible spongiform encephalopathy is medical jargon for a "communicable disease that turns the brain spongy." The acronym TSE commonly represents these diseases which come in many forms. The one Lyman talked about is bovine or cow TSE but there are also types that were until recently considered specific to sheep called scrapie , New Guinea cannibals called kuru and even civilized humans called Creutzfeldt-Jacob Disease. All are 100% fatal.

79. Senator Dick Durbin On The Issues
Increases surveillance of creutzfeldtjacob disease in humans to promote research efforts. US Department of Agriculture BSE fact sheet.
http://durbin.senate.gov/issues/madcow.cfm
Return to Previous Page MAD COW DISEASE Prion diseases are brain wasting diseases of humans and animals. These include BSE ("mad cow disease") in cattle, chronic wasting disease in deer and elk and Creutzfeldt-Jacob Disease in humans. Senator Durbin has introduced this public health, animal health, and food safety bill to strengthen our response to BSE and improve surveillance and research for prion disease in other animals and in humans. Although just one cow within the U.S. has tested positive for mad cow disease, the meat and meat by-products from that animal potentially contaminated 2.8 million pounds of meat, animal feed, cosmetics and other products by the time the recall order was issued. The BSE and Other Prion Disease Prevention and Public Health Protection Act (S. 2007) makes a number of improvements to the current system: Better Testing The Durbin bill modifies USDA procedures to require all cattle over the age of 30 months to be tested for BSE. Currently, USDA tests only animals that are "downers" or showing symptoms of neurological disease – just 40,000 out of the 35 million cattle to be slaughtered in 2004. While animals over 30 months of age constitute less than 10% of total cattle slaughtered annually, animals at this age are the most likely to harbor detectible BSE. Targeting Risk Materials The Durbin bill updates and expands the definition of "Specified Risk Materials (SRM)" – those parts of an animal that can harbor the infective agent of prion diseases. The legislation defines SRMs to include the small intestinal tract, brain, skull, eyes, tonsils, spinal cord, spinal column and other central nervous system tissue.

80. OLPA - Legislative Updates - Animal Disease Risk Assessment, Prevention, And Con
and prevalence of the transmission of foot and mouth disease, BSE, variant creutzfeldtjacob disease, and related diseases in the United States,
http://olpa.od.nih.gov/legislation/107/publiclaws/planimaldisease.asp
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As part of his introductory remarks in the Congressional Record, Senator Ben Nighthorse Campbell (R-CO) noted that the purpose of S. 700 is to establish a Federal task force to prevent the spread of mad cow disease, foot and mouth disease, and related livestock diseases within the United States. On the same day Senator Campbell introduced S. 700, he testified at a hearing of the Senate Commerce Subcommittee on Consumer Affairs, Foreign Commerce and Tourism that addressed specific measures that should be taken in the United States to prevent BSE or mad cow disease. At the hearing, Senator Campbell said that Congress needed to do everything necessary to protect the livestock industry with careful contingency planning by creating a task force to report back to Congress on efforts and plans undertaken at agencies to prevent the spread of animal diseases and make recommendations for the future. On April 4, 2001, S. 700 was introduced by Senator Campbell as the Mad Cow and Related Diseases Prevention Act of 2001. On April 5, S. 700 was placed on the Senate Legislative Calendar for consideration. Senator Orrin Hatch (R-UT) offered an amendment in the nature of a substitute on the Senate floor, which was agreed to. The amendment changed the name of the bill to the Animal Disease Risk Assessment, Prevention, and Control Act of 2001 but did not substantively change the legislation. After passing the Senate under unanimous consent on April 5, 2001, the measure proceeded to the House for consideration. On May 9, 2001, the House passed S. 700 by voice vote and it was signed into law (P.L. 107-9) on May 24, 2001.

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