21. Ask NOAH About: Neurological And Neuromuscular Disorders Spongiform Encephalopathy (BSE) World Health Organization Bovine Spongiform Encephalopathy and New Variant creutzfeldt jakob disease - CDC Chronic Wasting http://www.noah-health.org/english/illness/neuro/neuropg.html

Ask NOAH About: Neurological and Neuromuscular Disorders Specific Neurological and Neuromuscular Disorders Alzheimer's Disease and Dementia Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease) Arnold Chiari Malformation Batten Disease ... Information Resources Neurological and Neuromuscular Disorders Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease) The Basics ALS - National Institute of Neurological Disorders and Stroke (Also in Spanish ALS, MS, MD: What's the Difference - Center for Neurological Study ALS Dictionary - ALS Survival Guide Amyotrophic Lateral Sclerosis (ALS) - Methodist Health Care System, Houston TX (also in Spanish Amyotrophic Lateral Sclerosis (ALS) - MEDLINEplus (also in Spanish ) (Interactive Flash Presentation) Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - American Academy of Family Physicians Fact Sheet: ALS - Family Caregiver Alliance Facts About Amyotrophic Lateral Sclerosis (ALS) - Muscular Dystrophy Association Facts about Amyotrophic Lateral Sclerosis (ALS) - MDA ... What You Need to Know About Amyotrophic Lateral Sclerosis - Cleveland Clinic Foundation Diagnosis ALS Diagnosis - North Shore-Long Island Jewish Health System, NY

22. MedlinePlus Creutzfeldt-Jakob Disease N O P Q R S T U V W XYZ List of All Topics. creutzfeldtjakob disease recent research articles on creutzfeldt-jakob disease . creutzfeldt-jakob disease http://www.nlm.nih.gov/medlineplus/creutzfeldtjakobdisease.html

23. CJD Foundation Inc. Welcome to the creutzfeldtjakob disease Foundation Website. We hope you will find all the The Cruetzfeldt-jakob disease Foundation consists of members who are concerned about http://www.cjdfoundation.org/

CJD Conference Info Welcome to the Creutzfeldt-Jakob Disease Foundation Website. We hope you will find all the information you need. Please feel free to call us on the HelpLine or email us at crjakob@aol.com . We will respond promptly and will address your concerns or answer your questions. The volunteers of the CJD Foundation are here to help. MISSION STATEMENT: WHO ARE WE AND WHY WE EXIST The Cruetzfeldt-Jakob Disease Foundation consists of members who are concerned about the complexity of issues surrounding this fatal brain disease. Our mission is to support families and loved ones touched by CJD. We carry out this mission by means of A national toll free HelpLine, (800) 659-1991 Publication of CJD Information pamphlet, September 2003 Political and public policy advocacy A Family Conference Collaboration with the National Prion Disease Pathology Surveillance Center and the Centers for Disease Control and Prevention Monitoring and communicating by newsletter all of the current research and Foundation activities Collaboration with international support groups involved in similar work including: The CJD Support Network, U.K.

24. Creutzfeldt-Jakob Disease Fact Sheet creutzfeldtjakob disease (CJD) fact sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS). What is creutzfeldt-jakob disease? http://www.ninds.nih.gov/health_and_medical/pubs/creutzfeldt-jakob_disease_fact_

National Institute of Neurological Disorders and Stroke Accessible version Science for the Brain The nation's leading supporter of biomedical research on disorders of the brain and nervous system Browse all disorders Browse all health organizations More about a disorder Studies with patients Research literature Press releases Search NINDS... (help) Contact us My privacy NINDS is part of the National Institutes of Health Creutzfeldt-Jakob Disease Fact Sheet Get Web page suited for printing Email this to a friend or colleague Request free mailed brochure Table of Contents What is Creutzfeldt-Jakob Disease? What are the Symptoms of the Disease? How is CJD Diagnosed? How is the Disease Treated? ... Where Can I Get Help? What is Creutzfeldt-Jakob Disease? Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide; in the United States there are about 200 cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of patients die within 1 year. In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur. There are three major categories of CJD: In sporadic CJD, the disease appears even though the person has no known risk factors for the disease. This is by far the most common type of CJD and accounts for at least 85 percent of cases.

25. 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, creutzfeldtjakob disease (CJD and nvCJD), kuru and chronic cow http://www.mad-cow.org/

7,651+ articles on mad cow and new variantCreutzfeldt-Jakob disease, prions, bovine spongiform encephalopathy, scrapie, BSE, CJD, CWD, TME, and TSE. Last Updated: 17 Apr 01 . . a project of the Sperling Biomedical Foundation . Note: news, policy, commentar, medicine have moved to new site The Official Mad Cow Disease Home Page You are what you eat: now serving number Bovine-CJD: might you already have it? ... Visit charity pages Early warnings: insomnia, memory loss, depression, anxiety, withdrawal, fearfulness) News archives Best links Search site 3D prions ... Science archives NEWS 2001 has moved Apr 17.. Webmaster: the next generation Apr 28.. Global TSE News Mar 24.. Slaughter on suspicion Mar 17.. Second epidemic raging Feb 27.. Canada censors scientists Feb 16.. Diagnostic news Feb 12.. Cosmetics ban on 13 countries Feb 11.. Genetically modified foods Feb 09.. Bogus BSE in Brazil? Jan 30.. Texas cattle quarantined Jan 13.. FDA scolds feed mills Jan 08.. Canadian concerns Jan 03.. FDA issues vaccine alert NEWS 2000... Dec 28..

26. Introduction To CJD creutzfeldtjakob disease. Introduction. creutzfeldt-jakob disease (CJD) is a rare and fatal neurodegenerative disease of unknown cause. http://www.cjd.ed.ac.uk/intro.htm

Creutzfeldt-Jakob Disease Introduction Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disease of unknown cause. Patients are usually aged between 50 and 75 and typical clinical features include a rapidly progressive dementia associated, myoclonus and a characteristic electroencephalographic pattern. Neuropathological examination reveals cortical spongiform change, hence the term `spongiform encephalopathy'. H.G. Creutzfeldt is credited with the first description of the disorder in 1920, although by current diagnostic criteria his case would be highly atypical. A year later another German neurologist, A. Jakob, described four cases, at least two of whom had clinical features suggestive of the entity we recognise as CJD. Aetiology The nature of the transmissible agent is the matter of some controversy. Previously considered a `slow virus' no viral agent has ever been convincingly demonstrated and no evidence of an immunological response seen. Additionally the infectious pathogen shows a remarkable resistance to treatments that would normally be expected to inactivate viruses. The viral hypothesis has been elegantly challenged by the prion (` pro teinaceous in Epidemiology The majority of cases are sporadic (85%), between 10-15% are familial and the remainder are iatrogenic.

27. Department Of Health Creutzfeldt-Jakob Disease CJD Bovine Spongiform Encephalopa Information from the Department of Health (England) on CJD and BSE. Includes monthly statistics, press releases, and details of publications and guidance. http://www.doh.gov.uk/cjd/cjd1.htm

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28. CDC - Bovine Spongiform Encephalopathy And Variant Creutzfeldt-Jakob Disease: Ba Bovine Spongiform Encephalopathy and Variant creutzfeldtjakob disease Background, Evolution, and Current Concerns. Variant creutzfeldt-jakob disease (vCJD). http://www.cdc.gov/ncidod/EID/vol7no1/brown.htm

Current Issue Vol. 7, No. 1 Jan–Feb 2001 Download Article PDF Help Feedback Perspective Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease: Background, Evolution, and Current Concerns Paul Brown,* Robert G. Will,† Raymond Bradley,‡ David M. Asher,§ and Linda Detwiler¶ *National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA; †National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, Scotland; ‡Central Veterinary Laboratory, New Haw, Addlestone, UK; §Center for Biologics Evaluation and Research, Food and Drug Administration, Rockville, Maryland, USA; ¶Animal and Plant Health Inspection Service, U.S. Department of Agriculture, Robbinsville, New Jersey, USA The epidemic of bovine spongiform encephalopathy in the United Kingdom, which began in 1986 and has affected nearly 200,000 cattle, is waning to a conclusion, but leaves in its wake an outbreak of human Creutzfeldt-Jakob disease, most probably resulting from the consumption of beef products contaminated by central nervous system tissue. Although averaging only 10-15 cases a year since its first appearance in 1994, its future magnitude and geographic distribution (in countries that have imported infected British cattle or cattle products, or have endogenous BSE) cannot yet be predicted. The possibility that large numbers of apparently healthy persons might be incubating the disease raises concerns about iatrogenic transmissions through instrumentation (surgery and medical diagnostic procedures) and blood and organ donations. Government agencies in many countries continue to implement new measures to minimize this risk.

29. Guardian Unlimited | Special Reports | Special Report: BSE And CJD Ongoing collection of news, commentary, audio, graphics and interactive guides about Bovine Spongiform Encephalopathy (BSE) and variant creutzfeldtjakob disease (CJD). http://www.guardian.co.uk/bse/0,8250,388290,00.html

@import url(/external/styles/global/0,14250,,00.css); Sign in Register Go to: Guardian Unlimited home UK news World news Archive search Arts Books Business EducationGuardian.co.uk Film Football Jobs Life MediaGuardian.co.uk Money The Observer Online Politics Shopping SocietyGuardian.co.uk Sport Talk Travel Audio Email services Special reports The Guardian The weblog The informer The northerner The wrap Advertising guide Crossword Dating Headline service Syndication services Events / offers Help / contacts Information Living our values Newsroom Reader Offers Style guide Travel offers TV listings Weather Web guides Working at GNL Guardian Weekly Money Observer Home UK Business Online ... Quiz Search this site Go to... BSE and CJD article archive Special report: what's wrong with our food? BSE inquiry links Official site DEFRA website EU response Chronology of BSE ... Related sites Queniborough Summary of investigation Presentation to health authority meeting Victims Human BSE foundation Other BSE links British Medical Journal CJD page Health Department BSE page Ministry of Agriculture BSE page The UK CJD Disease Surveillance Unit ... January 2002: report on vCJD in the future - Parliamentary Office of Science and Technology Cartoon Steve Bell on the BSE report Latest British firm raises hopes of vCJD blood test May 26: The rogue protein linked to the human form of BSE and similar diseases has been detected in a patient's blood for the first time, raising hopes that a reliable test for variant CJD can be developed.

30. MedlinePlus Medical Encyclopedia Creutzfeldt-Jakob Disease creutzfeldtjakob disease. Definition Return to top. creutzfeldt-jakob disease is a disorder involving rapid decrease of mental function and movement. http://www.nlm.nih.gov/medlineplus/ency/article/000788.htm

31. CJD Foundation, Inc. On The World Wide Web creutzfeldtjakob disease Foundation, Inc. The Foundation has moved. Our new website address is cjdfoundation.org. Please update your bookmarks! http://members.aol.com/crjakob/

Creutzfeldt-Jakob Disease Foundation, Inc. The Foundation has moved. Our new website address is: cjdfoundation.org Please update your bookmarks!

32. WHO: Variant Creutzfeldt-Jakob Disease Variant creutzfeldtjakob disease. Variant creutzfeldt-jakob disease (vCJD) is a rare and fatal human neurodegenerative condition. http://www.who.int/mediacentre/factsheets/fs180/en/

English Search Home Countries Health topics ... Multimedia Media centre Location: WHO WHO sites Media centre Fact sheets ... printable version Fact sheet N°180 Revised November 2002 Variant Creutzfeldt-Jakob disease Variant Creutzfeldt-Jakob disease (vCJD) is a rare and fatal human neurodegenerative condition. As with Creutzfeldt-Jakob disease, vCJD is classified as a Transmissible Spongiform Encephalopathy (TSE) because of characteristic spongy degeneration of the brain and its ability to be transmitted. vCJD is a new disease that was first described in March 1996. Before the identification of vCJD, CJD was recognized to exist in only three forms. Sporadic cases, which have an unknown cause and occur throughout the world at the rate of about one per million people, account for 85–90% of CJD cases. Familial cases are associated with a gene mutation and make up 5–10% of all CJD cases. Iatrogenic cases result from the accidental transmission of the causative agent via contaminated surgical equipment or as a result of cornea or dura mater transplants or the administration of human-derived pituitary growth hormones. Less than 5% of CJD cases are iatrogenic. In contrast to the traditional forms of CJD, vCJD has affected younger patients (average age 29 years, as opposed to 65 years), has a relatively longer duration of illness (median of 14 months as opposed to 4.5 months) and is strongly linked to exposure, probably through food, to a TSE of cattle called Bovine Spongiform Encephalopathy (BSE).

33. Medicine-Worldwide: Creutzfeldt-Jakob-Disease (CJD) Translate this page creutzfeldt-jakob-disease (CJD). Seite 1 2 vor. Allgemeines. Die creutzfeldt-jakob-Krankeit (=disease) wurde 1920 erstmals beschrieben. http://www.m-ww.de/krankheiten/prionenkrankheiten/cjd.html

Sie befinden sich hier: startseite krankheiten prionenkrankheiten Prionenkrankheiten ... Über uns Creutzfeldt-Jakob-Disease (CJD) Seite: vor Kapitelübersicht: Zusammenfassung Allgemeines Erreger Symptome ... Ratgeber Zusammenfassung Die Creutzfeldt-Jakob-Erkrankung (CJD), die 1920 zum ersten Mal beschrieben wurde und sehr selten auftritt, hat durch Bekanntwerden einer neuen Variante (nvCJD) Allgemeines (sporadisch) auftretende Form von einer dominant vererbbaren Die so genannte neue Variante der Krankheit nvCreutzfeldt-Jakob-Krankheit hier Erreger Es gilt mittlerweile als nahezu sicher, dass Prionen die Erreger dieser Krankheit sind. Sc C ) gebildet werden. Der Zusatz "Sc" steht für Scrapie- spezifisch, der von "C" für cellulär, also für körpereigen. Die Prionen (PrP Sc Sc C Symptome Tremor, also Zittern von Armen, Beinen oder dem Kopf Zerebellare Ataxie, d.h. ein unkoordinierter Bewegungsablauf Inkubationszeit Diagnose Die Diagnose wird anhand des neurologischen Befundes gestellt. Die erste Veränderung, die man an Hirnbiopsien unter dem Mikroskop sehen kann, ist die Astrogliose. Darunter versteht man die Tatsache, dass zunächst die Astrozyten im Bereich ihrer Endplatten, die den Hirngefäßen anliegen, beginnen anzuschwellen und dann später die gesamte Zelle. Als Neuroglia ("Leim") bezeichnet man das Hüll- und Stützgewebe des Gehirns. Es besteht aus Astrozyten (Makroglia), Oligodendrozyten (Oligodendroglia) und Hortegazellen (Mikroglia). Außerdem wird vermehrt ein bestimmtes Eiweiß mit der Bezeichnung glial fibrillary acidic protein (GFAP) gebildet. Dieses Eiweiß ist nach einer besonderen Anfärbung im Mikroskop sichtbar.

34. Medicine-Worldwide: New Variant Creutzfeldt- Jakob-Disease (nvCJD) Translate this page New variant creutzfeldt - jakob-disease (nvCJD). Seite 1 2 vor. http://www.m-ww.de/krankheiten/prionenkrankheiten/nvcjd.html

Sie befinden sich hier: startseite krankheiten prionenkrankheiten Prionenkrankheiten ... Über uns New variant Creutzfeldt - Jakob-Disease (nvCJD) Seite: vor Kapitelübersicht: Zusammenfassung Allgemeines Erreger Symptome ... Ratgeber Zusammenfassung Allgemeines (CJD). Insgesamt sind an dieser Krankheit nachgewiesenermassen bisher 107 Personen in England, 3 in Frankreich und eine in Irland verstorben (Stand: Oktober 2001). Erreger Es gilt mittlerweile als nahezu sicher, dass Prionen die Erreger dieser Krankheit sind. Sc C ) gebildet werden. Das Gen, welches die Information für dieses Prion-Protein trägt, liegt auf dem Chromosom 20. Der Zusatz "Sc" steht für Scrapie- spezifisch, der von "C" für cellulär, also für körpereigen. Die Prionen (PrP Sc Diese Erkrankung war bis vor wenigen Jahren nahezu unbekannt und auch die "normale" Creutzfeldt- Jakob- Erkrankung kam nur extrem selten vor. Dies hat sich in allerletzter Zeit teilweise dramatisch geändert. Im Mutterland von BSE, also in England, sind bis Mai 2001 99 Menschen an der nvCJD verstorben. In Frankreich hat es bisher 3 Todesfälle an nv CJD gegeben, sowie einen in Irland. Der englische Epidemiologe Roy Anderson aus Oxford hält bis zu 500.000 Tote in den nächsten 30 Jahren für nicht unmöglich. Symptome Tremor, also Zittern von Armen, Beinen oder dem Kopf

35. Health Canada - Creutzfeldt-Jakob Disease (Variant CJD) - First Canadian Case Of May 2003. Information. First Canadian Case of Variant creutzfeldtjakob disease (Variant CJD). In April 2002, a suspected case of variant http://www.hc-sc.gc.ca/english/diseases/cjd/

Backgrounders First Canadian Case of Variant CJD Frequently Asked Questions about Variant CJD Health Canada's CJD Surveillance System Variant CJD ... Blood Safety and Variant CJD Other Links CJD Surveillance System Infection Control Guidelines (CJD) Information on Mad Cow Disease (BSE) May 2003 Information First Canadian Case of Variant Creutzfeldt-Jakob Disease (Variant CJD) In April 2002, a suspected case of variant CJD was reported to Health Canada's CJD Surveillance System. Because of the clinical presentation, age of the patient and past multiple stays in the United Kingdom (UK), this was classified as a possible variant CJD case. However, the diagnosis of classical CJD was also considered. As part of the investigation, a full medical history was obtained. The following information on this case can be shared: The patient, a male under the age of 50, had multiple stays in the UK during the outbreak of bovine spongiform encephalopathy (BSE), commonly know as "mad-cow disease." While staying in the UK, the patient ate processed meat products. Processed meat products made from cows infected with BSE carried a high risk of transmission of BSE.

36. Health Canada - Diseases - Mad Cow Disease Inspection Agency. Health Canada Information on Variant creutzfeldtjakob disease (vCJD) - human form of Mad Cow disease. Fact Sheets. First http://www.hc-sc.gc.ca/english/diseases/bse/

CFIA (Canadian Food Inspection Agency) Province of Alberta's Agriculture, Food and Rural Development Mad Cow Disease (BSE) Statement by The Honourable Bob Speller Minister of Agriculture and Agri-Food and Minister Responsible for the Canadian Food Inspection Agency regarding imports of U.S. beef Statement by The Honourable Bob Speller Minister of Agriculture and Agri-Food and Minister Responsible for the Canadian Food Inspection Agency Statement by The Honourable Bob Speller Minister Responsible for the Canadian Food Inspection Agency Health Canada Information on Variant Creutzfeldt-Jakob Disease (vCJD) - human form of Mad Cow disease Fact Sheets First Canadian Case of Variant CJD Frequently Asked Questions about Variant CJD Health Canada's Creutzfeldt-Jakob Disease (CJD) Surveillance System Variant CJD ... Blood Safety and Variant CJD Other Links CJD Surveillance System Infection Control Guidelines: Classic CJD in Canada Government of Canada Actions on Specified Risk Material Federal Regulations Amended to Enhance BSE Controls by Preventing Specified Risk Material (SRM) From Entering Food Supply Statement by the Honourable Anne McLellan, Minister of Health

37. Alzheimer's Society - CJD Support Network UK support network for all forms of creutzfeldtjakob disease. Offers information about the group as well as the disorder and contact details. http://www.alzheimers.org.uk/CJD

breadCrumbs("http://www.alzheimers.org.uk","/","index.htm","crumb","crumb","crumb","0"); Contact us Make a donation Membership Shop ... Fundraising events The CJD Support Network is now an independent charity. The contact details are: CJD Support Network Birchwood, Heath Top Ashley Heath Market Drayton Shropshire CJD Support Network helpline: 01630 673973 Email support@cjdsupport.net Website http://www.cjdsupport.net This page should redirect you to the page you require. If not, please click http://www.cjdsupport.net back to top Privacy policy Feedback Credits Contact us ... Links

38. CREUTZFELDT-JAKOB DISEASE AND RELATED DISORDERS creutzfeldtjakob disease AND RELATED DISORDERS. Part 1. There are at feast 250+ diagnosed cases of creutzfeldt-jakob disease every year in the United States. 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.

39. Alzheimer's Society Information Sheet - What Is CJD? What is creutzfeldtjakob disease (CJD)? Download PDF file. Prions The best-known prion disease is creutzfeldt-jakob disease, or CJD. This http://www.alzheimers.org.uk/Facts_about_dementia/What_is_dementia/info_CJD.htm

breadCrumbs("http://www.alzheimers.org.uk","/","index.htm","crumb","crumb","crumb","0"); Helpline / Ask a question Contact us Make a donation Membership ... Fundraising events What is Creutzfeldt-Jakob disease (CJD)? Prions are infectious agents that attack the central nervous system and then invade the brain, causing dementia. The best-known prion disease is Creutzfeldt-Jakob disease, or CJD. This information sheet provides an overview of the symptoms and different types of CJD. Prion disease Prions are abnormal forms of protein that are extremely hardy and cannot be eradicated by normal sterilisation procedures. Their presence in the brain results in spongiform encephalopathy, so-called because areas of the brain where cells have died take on a sponge-like appearance when viewed under the microscope. There are a number of prion diseases that affect humans. These include Creutzfeldt-Jakob disease, as well as two extremely rare inherited diseases, Gerstmann-Straussler-Scheinker disease (GSS) and fatal familial insomnia (FFI). Creutzfeldt-Jakob disease (CJD) Creutzfeldt-Jakob disease was first reported by two German doctors (Creutzfeldt and Jakob) in 1920.

40. Creutzfeldt-Jakob Disease - Health And Medical Information: Diseases And Conditi creutzfeldtjakob disease (CJD). further information on prion function see this reference. creutzfeldt-jakob disease At A Glance. http://www.medicinenet.com/Creutzfeldt-Jakob_Disease/article.htm

MedicineNet Home > Creutzfeldt-Jakob Disease Advanced Search Printer-Friendly Format Add to Favorites Email to a Friend ... Next page Creutzfeldt-Jakob Disease (CJD) What is CJD? What are the biochemical features of CJD? Creutzfeldt-Jakob Disease At A Glance What is CJD? Creutzfeldt-Jakob disease (CJD) is the dementing form of the human prion diseases (also known as spongiform transmissible encephalopathies or infectious amyloidoses). A prion is an unconventional, transmissible agent (not a virus or a bacterium). Prions are special proteins that can be transmitted from animal to animal to cause a group of degenerative diseases of the nervous system. These diseases can be manifest as sporadic, infectious, or inherited disorders. CJD is characterized by forgetfulness and nervousness; jerky, trembling hand movements; unsteady gait; myoclonus; chronic dementia; severe balance disturbance; and muscular rigidity. Patients can have characteristic brain electrical changes referred to as periodic electroencephalogram (EEG) complexes. Brain biopsy (pathology under the microscope) includes typical tissue changes referred to as status spongiosus, diffuse nerve cell degeneration, and glial proliferation. There is no known treatment or cure for CJD. Bovine spongiform encephalopathy (BSE) represents another, similar disease of cattle. About 15% of cases of CJD have a family history that demonstrates an inherited form of the illness (autosomal dominant transmission). Onset of disease is significantly earlier in familial cases. Temporal and spatial separations between affected relatives suggested that incubation periods range at least from 1 to 4 decades. Affected sibs tend to die at the same age and not at the same time. The mean age of onset is 61.5 years, and 90 % of patients die within a year of onset.

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