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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. Does Mad Cow Disease Threaten Our Blood Supply?
encephalopathies (TSEs) by doctors, these diseases include scrapie in sheep, madcow disease in cattle, and kuru and creutzfeldtjacob disease in humans.
http://www.txtwriter.com/Onscience/Articles/prions.html
View articles by date ON SCIENCE Home Page View articles by topic Does Mad Cow Disease Threaten Our Blood Supply? Last month a special committee set up by the Food and Drug Administration (FDA) recommended that the Red Cross and other agencies refuse to accept blood donated by people who have visited England since 1980. This alarming recommendation was made because of fear that such blood might introduce a disorder known as "mad cow disease" into the US blood supply. What is going on here? Just what is the danger that is making the FDA sweat? "Mad cow disease" is one of a peculiar group of fatal brain diseases that have fascinated scientists for decades. These diseases, transmissible from one individual to another, have the unusual property that it is years and often decades before the disease is detected in infected individuals. The brains of infected individuals develop numerous small cavities as nerve cells die, producing a marked spongy appearance. Central nervous system function is progressively degraded, until death eventually occurs. There is no cure. Given the jaw-breaking name "transmissible spongiform encephalopathies" (TSEs) by doctors, these diseases include scrapie in sheep, "mad cow" disease in cattle, and kuru and Creutzfeldt-Jacob disease in humans. During the 1980s physician Stanley Prusiner, despite the scorn of much of the scientific community, amassed a body of experimental evidence that Alper and Griffith were right, and that the infectious agent in TSE preparations was a protein, which he named a prion. In 1997, Prusiner was awarded the Nobel prize for his pioneering work on prions.

62. BSE And CJD Linkage
Possible link between BSE and creutzfeldtjacob disease. Characteristicsof variant creutzfeldt-jacob disease - vCJD. Dr. RG Will (Mar.
http://w3.aces.uiuc.edu/AnSci/BSE/Human_vCJD_Linkage_to_BSE.htm
Possible link between BSE and Creutzfeldt-Jacob Disease Four cases of CJD among dairy farmers in the UK occurred in 1994-5 These cases resembled typical sporadic CJD . They did, however, highlight concern about the possible transmission of BSE to humans. In 1995, Britton and Bateman identified cases (2) of CJD in teenagers that had unusually kuru-type plaques. These are normally seen in only 5% of CJD cases. Patients have behavioral and psychiatric disturbances, early and progressive ataxia (failure of muscular coordination). The new cases had extensive plaque formation and identifiable patterns of prion protein immunostaining. These cases had similarities to iatrogenic CJD associated with peripheral inoculation of prions (cadaveric growth-hormone cases) and kuru which are also characterized by progressive ataxia, behavioral and psychiatric disturbances. These patients had no recognized risk factors for CJD (iatrogenic routes or previously recognized prion gene mutations), However, they did have some familial history of dementia (Collinge Subsequently, a consistent and previously unrecognized disease pattern was identified in 10 (currently 14) other cases of CJD. Six of these were diagnosed in 1994 and four in 1995. Some of the features of the disease are different from those of classic CJD and a consideration of the medical histories, genetic analysis, possibility of increased ascertainment and other possible explanations failed to provide an adequate explanation for the pattern. This cluster is being referred to as Variant CJD (

63. DHSS, Creutzfeld-Jacob Disease
CLINICAL DESCRIPTION. creutzfeldtjacob (CJD) disease is a progressiveneurological disorder caused by an unconventional filterable agent.
http://www.state.nj.us/health/cd/cd_creutzfeld.htm
Disease Index NJ InTouch Surveillance Case Definition Creutzfeld-Jacob Disease
CLINICAL DESCRIPTION
Creutzfeldt-Jacob (CJD) disease is a progressive neurological disorder caused by an "unconventional" filterable agent. It is characterized by confusion, progressive dementia and variable ataxia. Later myoclonic jerks appear, together with a variable spectrum of other neurological signs. CJD must be differentiated from other forms of dementia: Alzheimer disease, other slow infections, toxic and metabolic encephalopathies, and occasionally from tumors or other space occupying lesions. The new form of CJD (vCJD) related in UK to consumption of beef differs from CJD in three ways. The vCJD occurs in a younger age group (20-30 years of age vs. 35 years or older for CJD). The characteristic EEG changes seen in CJD are absent in vCJD. Finally the clinical course of vCJD is much longer than CJD (12-15 months vs. 3-6 months).
CASE CLASSIFICATION
  • CONFIRMED
    A clinically compatible case supported by:
    • neuropathological examination (brain biopsy, post-mortem examination)
  • 64. : Diagnosis Of Bovine Spongiform Encephalopathy, Scrapie Disease And Creutzfeldt
    199708-04 Update Date 1998-07-27 Title Diagnosis of Bovine Spongiform encephalopathy,Scrapie disease and creutzfeldt-jacob Syndrome in Cows, Sheep and
    http://www.inserm.fr/serveur/Prions.nsf/397fe8563d75f39bc12563f60028ec43/ca12c8e

    65. Creutzfeldt-Jakob Disease Foundation, Inc.
    American organization provides historical and theoretical information along with a message board. Contact info included as well.
    http://cjdfoundation.org/
    Information
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    66. UK Creutzfeldt-Jakob Disease Surveillance Unit
    Summarizes the research in progress at the CJD unit and also provides background information about Category Health Conditions and diseases Creutzfeldt Jakob disease......The UK CreutzfeldtJakob disease Surveillance Unit. The incidence of Creutzfeldt availableon the Web. Creutzfeldt-Jakob disease Surveillance.
    http://www.cjd.ed.ac.uk/
    The UK Creutzfeldt-Jakob Disease Surveillance Unit
    The incidence of Creutzfeldt-Jakob disease (CJD) is monitored in the UK by the CJD surveillance unit based at the Western General Hospital in Edinburgh, Scotland. The unit brings together a team of clinical neurologists, neuropathologists and scientists specialising in the investigation of this disease. This document is intended to summarise the research in progress at the CJD unit and also provide some background information about CJD and other human spongiform encephalopathies. We have also provided some links to other resources and contrary points of view available on the Web.
    Creutzfeldt-Jakob Disease Surveillance.
    • Figures for the number of confirmed cases of the new variant of CJD and referrals of suspected cases of CJD to the surveillance unit over the last twelve years. NCJDSU protocol for CJD surveillance across the UK. Text and figures of the latest annual report of the CJD unit (published 9th July 2002). Reporting CJD cases to public health departments - guidance document Potential treatments for Creutzfeldt-Jakob disease.

    67. Lancet 1996; 347: 921 25
    A new variant of CreutzfeldtJakob disease in the UK. We report ten casesof CJD in the UK with clinical onset of disease in 1994 and 1995.
    http://www.cjd.ed.ac.uk/lancet.htm
    Lancet
    A new variant of Creutzfeldt-Jakob disease in the UK
    R G Will, J W Ironside, M Zeidler, S N Cousens, K Estibeiro, A Alperovitch, S Poser, M Pocchiari, A Hofman, P G Smith Summary Background Epidemiological surveillance of Creutzfeldt-Jakob disease (CJD) was reinstituted in the UK in 1990 to identify any changes in the occurrence of this disease after the epidemic of bovine spongiform encephalopathy (BSE) in cattle. Methods Case ascertainment of CJD was mostly by direct referral from neurologists and neuropathologists. Death certificates on which CJD was mentioned were also obtained. Clinical details were obtained for all referred cases, and information on potential risk factors for CJD was obtained by a standard questionnaire administered to patients' relatives. Neuropathological examination was carried out on approximately 70% of suspect cases. Epidemiological studies of CJD using similar methodology to the UK study have been carried out in France, Germany, Italy, and the Netherlands between 1993 and 1995. Findings Ten cases of CJD have been identified in the UK in recent months with a new neuropathological profile. Other consistent features that are unusual include the young age of the cases, clinical findings, and the absence of the electroencephalogram features typical for CJD. Similar cases have not been identified in other countries in the European surveillance system.

    68. Bovine Spongiform Encephalopathy: Main Index, CDC
    Both disorders are invariably fatal brain diseases with unusually long incubation periods measured in years, and are caused by an unconventional transmissible agent. in Europe of a disease in cattle, called bovine spongiform or "mad cow disease"), and a disease in humans, called variant CreutzfeldtJakob disease (vCJD). Both disorders are
    http://www.cdc.gov/ncidod/diseases/cjd/cjd.htm
    Site Contents
    BSE and CJD

    CJD: Infection Control Practices

    vCJD Fact Sheet
    BSE and CJD Home BSE and CJD Information and Resources
    New Information:
    Probable Variant Creutzfeldt-Jakob Disease in a U.S. Resident Florida, 2002 Update 2002: Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease July 2002 Variant CJD: Fact Sheet
    Since 1996, evidence has been increasing for a causal relationship between ongoing outbreaks in Europe of a disease in cattle, called bovine spongiform encephalopathy (BSE, or "mad cow disease"), and a disease in humans, called variant Creutzfeldt-Jakob disease (vCJD). Both disorders are invariably fatal brain diseases with unusually long incubation periods measured in years, and are caused by an unconventional transmissible agent. BSE has not been detected in the United States, despite active surveillance efforts since May 1990. It is extremely unlikely that BSE would be a foodborne hazard in this country. CDC monitors the trends and current incidence of CJD in the United States, while the U.S. Department of Agriculture has conducted active surveillance efforts for BSE since May 1990.

    69. Blood Recall/Withdrawal - Creutzfeldt-Jakob Disease (CJD)
    Indeed, It is the only thing That ever has. Margaret Mead. Welcome tothe Blood Recall/Withdrawal Creutzfeldt-Jakob disease (CJD) website.
    http://members.aol.com/debbieoney/blood.htm
    Never doubt
    That a small group
    Of dedicated people
    Can change the world.
    Indeed,
    It is the only thing
    That ever has.
    Margaret Mead
    Welcome to the Blood Recall/Withdrawal - Creutzfeldt-Jakob Disease (CJD) website. This website was created to fill the needs of people who have received notices that the blood products that they, or their children, received, or that were used in the In Vitro Fertilization (IVF) cultures that produced their children, were recalled/withdrawn from the market because a member of the donor pool was diagnosed with Creutzfeldt-Jakob Disease (CJD) or was at risk for the disease. CJD is an infectious rapidly progressive fatal brain-deteriorating disease for which there is no treatment or cure. It is a member of the class of diseases called Transmissible Spongiform Encephalopathies (TSEs). Another TSE is Bovine Spongiform Encephalopathy (i.e. Mad Cow Disease) which is found in England . The jury is still not in on whether CJD is transmitted through blood. The risk is said to be theoretical. There have been no documented cases of such transmission in humans. However, the CJD infectious agent has been found in blood. People who have received blood recall/withdrawal notifications can still donate blood.

    70. OMIM ENTRY 123400
    From Champion Expanding Encyclopedia Of Mortuary Practices Number 625. pp 251417, 1995 CREUTZFELDT-JAKOB disease AND RELATED DISORDERS By James H. Bedino, Chemist/Dir. Research, The Champion Company INTRODUCTION Creutzfeldt-Jakob disease is a disease of bizarre and realities of the disease itself. There is a profession concerning Creutzfeldt-Jakob disease and unfortunately it
    http://www3.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?123400

    71. REGENT Antiseptics: Knowledge, Special Report: Creutzfeldt Jacob Disease
    REGENT Antiseptics knowledge Creutzfeldt Jacob disease Creutzfeldt Jacob disease,as an emerging disease has been the source of major concern within
    http://www.regentantiseptics.com/regentweb/knowledge.nsf/page/5gpfn7
    You are in: Home Knowledge Special Reports > Special Report
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    Select... Ask the Experts Clinical Bibliographies General Hand Disinfection Job Files Microbiology Special Reports Whole Body Washing
    Special Report: Creutzfeldt Jacob Disease
    By Kate Woodhead RGN DMS
    Creutzfeldt Jacob Disease, as an emerging disease has been the source of major concern within communities in Europe recently, where the incidence is highest.
    The disease is being actively researched in many corners of the world in order to understand how it affects patients and how it may be best managed by health professionals.
    Currently there are more questions than answers, although additional data is coming to light all the time. Ironically, the dynamic nature of the scientific information is also a problem for families of victims, hospitals and health care professionals. CJD is presenting a huge challenge to the scientific, epidemiological and health care professionals to identify and reduce possible transmissions of this new disease whose causative agent is even now, little understood.(1)
    85% of all Creutzfeldt Jacob Disease is caused by sporadic CJD, the incidence worldwide is 1 in a million per year and it was first described in 1921. (2) The form of the disease which has raised the profile and has been the cause of the most publicity, is the variant form which appears to have a link with BSE, an animal disease. Variant CJD was not described until 1996.(3) No-one can forget the press coverage of cows staggering and foaming at the mouth and it seems almost incredulous that humans are affected by a similar form of the disease.

    72. Crutzfeldt Jacob Disease
    The Creutzfeldt Jacob Syndrome in humans. Click here to see an animatedGIF preview. The progression of the pathogenic agent has
    http://www.proinformatix.com/cjd.html
    Our mission Publications Modelling Arts ... Links The Creutzfeldt Jacob Syndrome in humans. Click here to see an animated GIF preview. The progression of the pathogenic agent has been illustrated in a 3 min animation. This animation illustrates a travel through the patients head, the brain, further inside the neurons and end at the cellular membrane in the neuron (see image). At this stage the uptake of copper is clearly illustrated in combination with animated lipid-layers and protein "flipase-like" movement across the membrane. The electrostatic interaction between the pathogenic conformer and the normal prion protein is also clearly illustrated, to show the progression of the malignant-conformers inside the neurons. A voice-over is found in the full version to guide the student/listener to what happens simultaneously with the animation. This movie has won the SWITCH-art award in animation. Academic institutions: Friendly price. Click here to purchase the movie in full screen format on CD-ROM for your classes webmaster@proinformatix.com
    Revised: February 23, 2003

    73. BSE, CJD, Mad Cow Disease, Disease Information, NCID, CDC
    Skip Navigation Links. Centers for disease Control and Prevention. CDC Home,Search, Health Topics AZ, Useful Sites, Infectious disease Information.
    http://www.cdc.gov/ncidod/diseases/submenus/sub_bse.htm
    Infectious Disease
    Information Contents

    Infectious Diseases Information Index

    Useful Sites

    Infectious Disease Information BSE (Bovine Spongiform Encephalopathy, known as Mad Cow Disease, and Creutzfeldt-Jakob Disease, CJD) BSE and CJD Information and Resources
    Information sheets and publications Travelers' Health: Diseases
    Information and updates on risks, precautions Top of Page NCID Home Disease Information NCID Organization ... Health Topics A-Z This page last reviewed May 23, 2001 National Center for Infectious Diseases
    Centers for Disease Control and Prevention
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    74. Human Growth Hormone And Creutzfeldt-Jakob Disease
    The CreutzfeldtJakob disease (CJD) Foundation Inc. Creutzfeldt-Jakob disease (FactSheet of the National Institute of Neurological Disorders and Stroke, NIH).
    http://www.niddk.nih.gov/health/endo/pubs/creutz/creutz.htm

    Nonprofit Organizations Offering Support and Information
    MAGIC (Major Aspects of Growth in Children) Foundation is a national, nonprofit organization that provides support and education about growth disorders in children and growth hormone deficiency in adults. Staff will help connect you with others who have similar interests or concerns. The Human Growth Foundation (HGF) is a nonprofit organization concerned with children's growth disorders and adult growth hormone deficiency. HGF can send you a brochure on adult growth hormone deficiency. The foundation also sponsors an Internet mailing list to support the exchange of information about adult growth hormone deficiency and adult growth hormone replacement therapy. To subscribe, follow the instructions on the HGF web page The Creutzfeldt-Jakob Disease (CJD) Foundation Inc. was created in 1993 by two families who lost relatives to CJD and the neurologist who treated the patients. This nonprofit corporation seeks to promote research, education, and awareness of CJD and to reach out to people who have lost loved ones to this illness.
    General Information
    Update: Follow-Up Study of NHPP Growth Hormone Recipients (Public Health Service, June 1999)

    75. Bovine Spongiform Encephalopathy (BSE); Treatment, Prevention, Cure
    How does the disease cross species to infect humans as Variant CreutzfeldtJacobdisease? What factors affect whether a human will contract the disease?
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    76. CJD Creutzfeldt Jacob Disease
    CJD Creutzfeldt Jacob disease. This article submitted by Anne on 12/19/96.Author's Email I've been searching for clinicians with
    http://neuro-www.mgh.harvard.edu/neurowebforum/GeneralFeedbackArticles/12.19.961
    CJD Creutzfeldt Jacob Disease
    This article submitted by Anne on 12/19/96.
    Author's Email:
    I've been searching for clinicians with updated information and family members who have lost a relative to this disease. My father died almost five years ago and his diagnosis was confirmed by autopsy. Research indicates that the virus is always fatal, can lie dormant for years, and the path of the contagion is still unclear. I would like to hear from other people who have searched for more answers and who would like to collect more specific data on the frequency and geographic locations. In addition, I would like to hear about other families either currently experiencing this loss or who want to share their stories and questions th others. Next Article
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    77. Coastal Post Article - Mad Cows Do It:
    Although there is no evidence whatsoever that eating beef resulted in any of the14 unusual cases of Creutzfeldt Jacob disease, the scare has focused attention
    http://www.coastalpost.com/96/6/2.htm
    The Coastal Post - June, 1996
    Mad Cows Do It:
    Jobs Threatened, Rural Economics In Disarray, European Union Threatened BY JIM SCANLON As the price of meat falls, thousands of jobs are threatened, and thousands of farm families (even organic farmers) in rural economies face disaster. The British Conservative Government also seems likely to fall and the unification of Western Europe, so painstakingly put together since the agony of World War II, is in serious trouble. Although there is no evidence whatsoever that eating beef resulted in any of the 14 unusual cases of Creutzfeldt Jacob disease, the scare has focused attention on what cows are fed, and what their bodies are turned into. It seems that everything but the "moo" is turned into something. Gallstones are sold in the Far East as sexual stimulants and as jewelry. Horns and hooves make gelatin and collagen; bones and blood become fertilizer; hide becomes leather goods; and ovaries, spleen, adrenal and pituitary glands, lungs and pancreas are processed into medicines. Cow lips go to Mexico for taco fillings; cheeks are made into baloney; hearts go to Russia for sausage; stomach, kidneys, testes, liver and brains are all consumed one way or another by use for our pets. All this in edition to the muscle tissue commonly called meat. So the mad cow crisis has focused attention not only on what cows feed on (it's a cow eat cow world!) but what parts of cows we feed on! How do you know exactly what is in your fast food burger, or your homeburger or sausage? How do you know where the material came from in your moisturizing lotion or the casings of your vitamin supplements?

    78. STATS At Work Mad Hype Disease
    Meanwhile, the number of recorded deaths in Britain from the variant of the CreutzfeldtJacobdisease is 19 and the head of the British Government's advisory
    http://www.stats.org/statswork/madcow.htm

    79. Healthinfo4u - Creutzfeldtz Jacob Disease
    (Work of the Prion Clinic in London which provides information and support topatients and families affected by VCJD (Variant Creutzfeldt Jacob disease).
    http://www.healthinfo4u.org/results_pages/Creutzfeldtz_Jacob_Disease-1.html
    Search Help Encyclopaedia About ... Home Topic: Creutzfeldtz Jacob Disease Last Updated: February 2003
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    Title: Overview of spongiform encephalopathies: terminology and treatment options
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    80. CREUTZFELDT-JAKOB DISEASE AND RELATED DISORDERS
    CREUTZFELDTJAKOB disease AND RELATED DISORDERS. Part 1. Nothing is more fearfulthan complete rack of information concerning a potentially deadly disease.
    http://www.biomed.lib.umn.edu/hw/creutzfeld.html
    From: Champion Expanding Encyclopedia Of Mortuary Practices Number 625. pp 2514-17, 1995
    CREUTZFELDT-JAKOB DISEASE AND RELATED DISORDERS
    Part 1
    By James H. Bedino, Chemist/Dir. Research, The Champion Company
    ABSTRACT : An in-depth discussion and explanation of Creutzfeldt-Jakob disease along with several related prion driven disorders are covered for the elucidation and education of the embalmer. Early history of the various diseases is outlined with the interrelationships to kuru, scrapie, BSE and other similar neuro-degenerative diseases. The bizarre nature of the causative agent is discussed in detail along with its remarkable survivability. The dangers and concerns that embalmers have are delineated and placed in perspective. A suggested protocol to minimize risk during embalming is presented. A summation and suggestions for embalmers completes the article.
    INTRODUCTION : Creutzfeldt-Jakob disease is a disease of bizarre nature that almost every embalmer or funeral director has heard of but has virtually no idea concerning the facts and realities of the disease itself. There is a very small amount of information that has been available to the embalming profession concerning Creutzfeldt-Jakob disease and unfortunately it is either incomplete, misleading or outright incorrect. There is more conjecture and rumor involved with this disease than almost any other disease and there is virtually no valid information available to help the embalmer. This has resulted in a definite uncertainty and even panic in most situations involving embalmers when they encounter this disease during the course of their professional career. Nothing is more fearful than complete rack of information concerning a potentially deadly disease.

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