81. Pathology Of Degenerative CNS Diseases creutzfeldtjakob disease. creutzfeldt-jakob disease (CJD) is rare, affecting less than one person in a million per year. Review creutzfeldt-jakob disease. http://medlib.med.utah.edu/WebPath/TUTORIAL/CNS/CNSDG.html

CNS Degenerative Diseases Return to the tutorial menu. Alzheimer's Disease Senile dementia of the Alzheimer's type (SDAT), or Alzheimer's disease (AD) is becoming more common in developed nations as the population includes more and more older persons. There is no known cause for the disease. It is not known why some people present as early as 30 or 40 years of age with dementia while others do not present until their late 70's or 80's. Familial cases with a defined inheritance pattern account for only 5 to 10% of Alzheimer's disease. Familial cases tend to have an earlier age at onset. Genetic defects in familial cases have been identified on chromosomes 21, 19, 14, 12 and 1. The so-called "early onset" cases of AD in persons in their 30's, 40's, and 50's may have a genetic basis. Less than 1% of early onset AD cases are linked to a genetic defect on chromosome 21 (which may explain the appearance of Alzheimer's disease in persons with Down syndrome surviving to middle age) which affects amyloid precursor protein (APP), resulting in fibrillar aggregates of beta-amyloid that is toxic to neurons. About half of early onset AD cases are linked to mutations in the presenilin 1 gene on chromosome 14. A presenilin 2 gene has been discovered on chromosome 1, but this defect accounts for less than 1% of cases. The more typical "late onset" cases of AD occurring after age 60 may have underlying genetic defects. A genetic locus on chromosome 19 encodes for a cholesterol transporter called apolipoprotein E (apoE). The E4 variant of apoE, which increases deposition of fibrillar beta-amyloid, can be found in 40% of AD cases. However, the presence of apoE4 is neither necessary nor sufficient for development of AD, so testing for it is not warranted. A genetic locus on chromosome 12 that encodes for alpha-2-macroglobulin may be found in 30% of AD cases. Mutations in the tau gene which codes for

82. What Is Sporadic CJD? Table 1. Clinical characteristics of sporadic creutzfeldtjakob disease. Percentage of patients with symptoms or signs. Symptoms/Signs. At onset. On first exam. http://www.cjdinsight.org/Deana/sporadiccjd.html

'IN THIS TOGETHER' Our Story TSE: the disease group The History of TSE (Prion Diseases) Sporadic CJD ... Email SPORADIC CJD Sporadic CJD occurs in a random distribution all over the world, and has no known genetic or environmental cause. It apparently arises from a one-in-a-million spontaneous conversion of a normal cellular protein (encoded by a gene on chromosome 20) into an abnormal 3-dimensional shape, or configuration. Once converted, the abnormal molecule is thought in some way to compel normal molecules of newly synthesized protein to assume the same abnormal configuration, leading to aggregated deposits in and around cells of the central nervous system, and causing neuronal degeneration and symptomatic disease. Sporadic CJD chiefly affects people between 50 and 75 years of age, but can occur as early as adolescence, and as late as the 9th decade of life (by which time it may be mistaken as 'senility'). The age distribution of a large series of cases referred to the National Institutes of Health is shown in Figure 1 Symptoms We need only look at the brains of deceased patients to understand the great variety of neurological symptoms and signs that can be seen in CJD.

83. Entrez PubMed A new variant of creutzfeldtjakob disease in the UK. Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A, Poser http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8

84. Creutzfeldt-Jakob Disease creutzfeldtjakob disease. What is creutzfeldt-jakob disease? creutzfeldt-jakob disease, commonly known as CJD, is a very rare form of dementia. http://www.irishhealth.com/?level=4&con=306

85. Creutzfeldt-Jakob Disease creutzfeldtjakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. Subscribe now . creutzfeldt-jakob disease. http://healthlink.mcw.edu/article/921396214.html

Search Articles: search tips Please Take the HealthLink Survey Email this article Print this article Find related articles: By topic: Neurology By keywords: Receive Health Link via email! Subscribe now >> 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 worldwide and about 200 people in the United States. 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. In hereditary CJD , the person has a family history of the disease and/or tests positive for a genetic mutation associated with CJD. About 5 to 10 percent of cases of CJD in the United States are hereditary.

86. Creutzfeldt-Jakob Disease - Online Diagnosis. New Treatments, March 2, 2004 Online diagnosis of creutzfeldtjakob disease based on the patient s symptoms. New Treatments, March 2, 2004. creutzfeldt-jakob disease - Online Diagnosis. http://www.medical-library.org/journals/mddx/chronic_behavior_change_and_dementi

Online Medical Diagnosis The National Medical Society Creutzfeldt-Jakob Disease - Online Diagnosis Online diagnosis of Creutzfeldt-Jakob Disease based on the patient's symptoms. New Treatments, March 2, 2004. For how many months have the behavior changes and memory problems been present? How old is the patient? Does the patient have difficulty remembering events in the distant past (more than 6 months ago)? Yes No Does the patient have difficulty remembering events in the recent past (less than 2 hours ago)? Yes No Does the patient have difficulty remembering names? Yes No Does the patient have difficulty recognizing faces? Yes No Does the patient have difficulty getting dressed or grooming? Yes No Judith Harper, MD back to the library Online Diagnosis of Symptoms and Medical Diseases Online Diagnosis of Psychiatric Disorders Online Treatment Advisor and New Treatments ... Diagnosis by Disease This interactive medical program finds a diagnosis for Creutzfeldt-Jakob Disease.

87. Creutzfeldt-Jakob Disease (CJD) And The Blood Supply products do not result in interim product shortages. creutzfeldtjakob disease. In January 1997, Dr. Lawrence Schonberger summarized for http://www.hhs.gov/asl/testify/t970731d.html

This is an archive page. The links are no longer being updated. Testimony on Creutzfeldt-Jakob Disease (CJD) and the Blood Supply by David Satcher, M.D., Ph.D. Director, Centers for Disease Control and Prevention Public Health Service U.S. Department of Health and Human Services Before the House Committee on Government Reform and Oversight, Subcommittee on Human Resources July 31, 1997 Good morning. I am Dr. David Satcher, Director of the Centers for Disease Control and Prevention (CDC). I am accompanied by Dr. Mary Chamberland, Dr. Bruce Evatt and Dr. Lawrence Schonberger with CDC's National Center for Infectious Diseases. We are pleased to be here this morning to discuss with you issues related to blood safety; specifically, the safety implications of plasma pool sizes and our surveillance efforts related to Creutzfeldt-Jakob Disease (CJD) and the blood supply. As the report published nearly a year ago by this subcommittee found, the Nation's blood supply is safer than it has ever been. However, the blood supply continues to face infectious disease challenges from both recognized, as well as unknown threats. Ensuring that the Nation's supply of blood and blood products is free of infectious agents is a public health responsibility shared within the Department of Health and Human Services (HHS) by CDC, the Food and Drug Administration (FDA), and the National Institutes of Health (NIH). While CDC has no regulatory responsibility for blood safety, as the Nation's Prevention Agency, it has the expertise and responsibility for surveillance and detection of public health risks associated with receipt of blood and blood products. In collaboration with FDA, CDC also keeps the public informed concerning such risks.

88. Creutzfeldt-Jakob Disease creutzfeldtjakob disease. DESCRIPTION Rare transmissible virus dementia. ICD-9-CM 046.1 jakob-creutzfeldt disease SEE ALSO Kuru. Web http://www.5mcc.com/Assets/SUMMARY/TP0236.html

Creutzfeldt-Jakob disease DESCRIPTION: Rare, usually fatal, transmissible spongiform encephalopathy, occurring in middle life in which there is partial degeneration of the pyramidal and extrapyramidal systems accompanied by progressive dementia, tremor, muscle wasting, athetosis and spastic dysarthria. Usual course - progressive; 15-20 months incubation; 6 months average duration. Four subtypes described: classic, iatrogenic, familial, and new variant (nvCJD). The latter was first described in the United Kingdom in 1996 and has been linked to bovine spongiform encephalopathy (BSE), also known as "mad cow disease." CAUSES: Scrapie PrP (prion protein) Synonyms: spastic pseudosclerosis corticostriatal-spinal degeneration transmissible virus dementia ICD-9-CM: 046.1 Jakob-Creutzfeldt disease SEE ALSO: Kuru Web references: The Prion Diseases Author(s): Mark R. Dambro, MD

89. Creutzfeldt-Jakob Disease creutzfeldtjakob disease. http://vetgate.ac.uk/browse/cabi/74464a534f3e8d13eae3779b3153c0a9.html

low graphics Creutzfeldt-Jakob disease broader: human diseases prion diseases other: nervous system diseases Bovine spongiform encephalopathy : BSE Written and published on the Web by the United States Department of Agriculture's (USDA's) Animal and Plant Health Inspection Service (APHIS), this site provides information on BSE and other transmissible spongiform encephalopathies (TSE's), including Creutzfeldt-Jakob disease. Information is provided on a variety of BSE and TSE related topics, including BSE in the UK, BSE in the USA, Clinical Signs of BSE, Diagnosis of BSE, Scrapie, and chronic wasting disease (CWD) of deer and elk. Some of these documents are in PDF, which requires Adobe Acrobat Reader. A bovine spongiform encephalopathy (BSE) response plan summary prepared by APHIS and the Food Safety and Inspection Service (FSIS) is available in PDF. scrapie prion diseases cattle diseases bovine spongiform encephalopathy ... Bovine Spongiform Encephalopathy - "mad cow disease" Published on the Web by Agriculture, Fisheries and Forestry - Australia (AFFA), this site provides access to a range of information on bovine spongiform encephalopathy (BSE). Information available includes a BSE fact sheet, BSE FAQ, geographical risk of BSE country ratings, BSE glossary of terms, and details of variant Creutzfeld- Jakob Disease (vCJD). In addition, information is provided on Australia's beef industry, and a collection of links to related Web resources is provided. bovine spongiform encephalopathy Creutzfeldt-Jakob disease Australia BSE, CJD, nvCJD information resource

90. EMS - Creutzfeldt-Jakob Disease, Mad Cow Disease creutzfeldtjakob disease Fast Facts. Expert Contacts. Reseachers Find Mad Deer disease Can Infect Humans. Where to Get More Information? http://www.ems.org/creutzfeldt-jakob_disease/facts.html

Wednesday, 2 June 2004 Website Overview Search for: Printer-friendly version Fast Facts Last update: August 15, 2001 The technical name for mad cow-like diseases are transmissible spongiform encephalopathies or TSEs. TSEs are a family of deadly dementia diseases that include mad cow disease, scrapie in sheep, chronic wasting disease (CWD) in deer and elk, and New Variant Creutzfeldt-Jakob disease (nvCJD) in people worldwide. New Variant Creutzfeldt-Jakob disease is the suspected human form of mad cow disease, a chronic wasting disease that spreads through the food chain. It spreads by way of little-understood proteins called prions. Epidemiologists believe that human beings can be infected when they eat animals that have TSE. Mad cow disease is sometimes referred to by scientists as BSE, or bovine spongiform encephalopathy. Scientists believe that the practice of feeding animals to animals, such as in livestock feed, greatly accelerates the spread of TSEs. TSE is occurring at epidemic levels in deer and elk in the western United States and on game farms, and scientists believe people may already be dying in the U.S. from consuming infected game. A 1999 study funded by the National Institutes of Health demonstrated for the first time that TSE can pass from animals into humans in the form of nvCJD.

91. MC Factoids: New & Recent to have bowed to pressure from Congress and are investigating a potential cluster of cases of a fatal brain disorder called creutzfeldtjakob disease in the http://www.madcowboy.com/01_FactsMC.000.html

ABOUT FACTOIDS ABOUT HOWARD THE BOOK, ETC. ... SITE MAP PAGE UPDATED: 24-may-04 FACTOIDS AND RESOURCE LINKS Mad Cow Disease Vegetarianism The Environment Human Health Animal Rights NEW MAD COW DISEASE FACTOIDS INDEX MAD COW DISEASE New Factoids/Info Definitions/History In Humans/Other Species Feed/Testing/Rendering Politics/Economics ... Resources/Links of Note UPDATES/ARCHIVAL: 05/24/04: New Factoids/Info Politics/Economics In Humans/Other Speciess Feed/Testing/Rendering Definitions/History (Food-borne illness factoids will return in June 2004 "Mad cow disease is important today, not just as a deadly food-borne illness, but also as a powerful symbol of all that is wrong about the industrialization of farm animals." Eric Schlosser, "Fast Food Nation," Afterword: pg. 272)

92. Creutzfeldt-Jakob Disease --  Encyclopædia Britannica creutzfeldtjakob disease Encyclopædia Britannica Article. To cite this page MLA style creutzfeldt-jakob disease. Encyclopædia Britannica. 2004. http://www.britannica.com/eb/article?eu=28323&tocid=0&query=jakob bohme

93. Creutzfeldt-Jakob Disease --  Britannica Student Encyclopedia creutzfeldtjakob disease Britannica Student Encyclopedia. To cite this page MLA style creutzfeldt-jakob disease. Britannica Student Encyclopedia. 2004. http://www.britannica.com/ebi/article?eu=334736&query=jakob wassermann&ct=ebi

94. CJD Support Network - CJD Support Network creutzfeldtjakob disease (CJD) is one of a small group of fatal diseases caused by infectious agents called prions. These attack http://www.cjdsupport.net/

Topics in this section are introduction what is CJD? common questions ... links Related topics CJD fact sheets back to the home page Creutzfeldt-Jakob disease (CJD) is one of a small group of fatal diseases caused by infectious agents called prions. These attack the brain, killing cells and creating gaps in tissue. The brain takes on a characteristic sponge-like appearance. There are four strains of CJD with differing symptoms and causes, Sporadic or Classical CJD, Familial CJD, vCJD and Iatrogenic. Early symptoms include memory problems, mood changes and lack of co-ordination. The disease progresses to shakiness and dementia. Those affected are eventually unable to move or speak. The CJD Support Network provides help and support for people with Creutzfeldt-Jakob disease, their carers and concerned professionals. Founded in 1994 by relatives of people with CJD and is now recognised as the leading charity for all forms of CJD. Charity registration number 1097173

95. Topic Tracks: Creutzfeldt-Jakob Disease creutzfeldtjakob disease and Mad Cow disease . Guides. Bastian, Frank O. (ed.). creutzfeldt-jakob disease and other transmissible spongiform encephalopathies. http://www.library.umc.edu/TopicTracks/tt-variant-creutt.html

Creutzfeldt-Jakob Disease and "Mad Cow Disease" This guide is an introductory list to help locate research materials at the Rowland Medical Library. Variant Creutzfeldt-Jakob disease (vCJD) is the human form of bovine spongiform encephalopathy (BSE), or "mad cow disease", which results from infection by an unconventional transmissible agent. The nature of this agent is still unknown, but generally believed is prion protein, a pathogenic form of the protein that is both less soluble and more resistant to enzyme degradation than the normal form. Consumption of contaminated beef products, whole blood transfusions by pathogen-carrier donors might cause transmission of the disease. Guides Handbooks, encyclopedias, dictionaries Selected textbooks More books ... Selected electronic sources Guides Bastian, Frank O. (ed.). Creutzfeldt-Jakob disease and other transmissible spongiform encephalopathies WL 359 C738 1991 Bair, Frank E. (ed.). Alzheimer's, stroke, and 29 other neurological disorders sourcebook: basic information for the layperson . p.127-133, 146. CONSUMER HEALTH WL 348 A477 1993 Krause, Richard M. (ed.).

96. Creutzfeldt-Jakob Disease creutzfeldtjakob disease. broader prion diseases. BSE Inquiry. The BSE inquiry was set up by the UK government in 1997. The Inquiry http://agrifor.ac.uk/browse/cabi/74464a534f3e8d13eae3779b3153c0a9.html

low graphics Creutzfeldt-Jakob disease broader: prion diseases BSE Inquiry The BSE inquiry was set up by the UK government in 1997. The Inquiry aims "to establish and review the history of the emergence and identification of BSE and the new variant CJD in the United Kingdom, and of the action taken in response to it up to the 20th March 1996." The site provides access to the full-text report, it's findings and conclusions, and all the transcripts, witness statements, recently issued documents and revised factual accounts offered as evidence. Many of the documents are available in html, word and PDF format. public health government bovine spongiform encephalopathy UK ... Bovine spongiform encephalopathy (BSE) Written and published on the Web by the United States Department of Agriculture's (USDA's) Animal and Plant Health Inspection Service (APHIS), this site provides information on BSE and other Transmissible Spongiform Encephalopathies (TSE's). Information is provided on a variety of BSE and TSE related topics, including fact sheets on BSE, TME, scrapie, and chronic wasting disease (CWD). Some of these documents are in PDF, requiring Adobe Acrobat Reader. The site also provides news, BSE surveillance, and details of recent initiatives. scrapie prion diseases cattle diseases bovine spongiform encephalopathy ... Bovine Spongiform Encephalopathy - "mad cow disease" Published on the Web by Agriculture, Fisheries and Forestry - Australia (AFFA), this site provides access to a range of information on bovine spongiform encephalopathy (BSE). Information available includes a BSE factsheet, BSE FAQ, geographical risk of BSE country ratings, BSE glossary of terms, and details of variant Creutzfeld- Jakob Disease (vCJD). In addition, information is provided on Australia's beef industry, and a collection of links to related Web resources is provided.

97. CJD Support Group Pty. Ltd. (CJDSGN) - Creutzfeldt-Jakob Disease Support Website Australian CJD Support Group Network Inc CJDSGNI - creutzfeldt-jakob disease support website. CJD Support Group Pty. Ltd. http://www.cjdsupport.org.au/

CJD Support Group Pty. Ltd. This is the web site of CJD Support Group Pty. Ltd. (CJDSGN). Contents Some Background Two thousand one hundred Australians were treated with human pituitary hormones under the Australian Human Pituitary Hormone Program (AHPHP) which ran in Australia from 1967 until 1985. Unlike similar programs undertaken in a number of overseas countries at around the same time, the AHPHP concentrated on the treatment of infertile women with human pituitary gonadotrophin (hPG) with around six hundred children treated with human growth hormone (hGH) for stunted growth. Five Australians so far have developed and died from iatrogenic Creutzfeldt-Jakob disease (CJD) as a result of receiving pituitary hormone products. The program was suspended in 1985 following hGH related CJD deaths in the United States. All those treated under the program are at risk of developing CJD, because the manufacturing protocols allowed cross-infection of production batches. The AHPHP was run under the auspices of the Commonwealth Department of Health. The hormones were manufactured by the then government owned Commonwealth Serum Laboratory in Melbourne. The AHPHP was conceived and operated by the Human Pituitary Advisory Committee (HPAC) until its activities ceased in 1985. The committee was finally disbanded.

98. Is Mad Cow Back? - Creutzfeldt-Jakob Disease STORIES Is Mad Cow Back? creutzfeldtjakob disease. AJ.Cann. The thin slice creutzfeldt-jakob disease. In 1920, Drs. Hans Gerhard creutzfeldt http://www.mos.org/cst/article/368/1.html

STORIES: Is Mad Cow Back? Creutzfeldt-Jakob Disease A.J.Cann The thin slice of diseased brain tissue shows the microscopic holes of CJD Creutzfeldt-Jakob Disease In 1920, Drs. Hans Gerhard Creutzfeldt and Alfons Jakob observed large numbers of holes in the brains of some their patients who had died. They realized that the holes were caused by the death of many brain cells. The two doctors also noticed spongy webs of fiber in the dead patients' brains. So the doctors described the disease, now named for the pair, as a "spongiform encephalopathy." ( Encephalopathy means a disease of the brain.) Patients start to lose their memories and endure mood changes. Soon after, patents' balance deteriorates and they suffer dementia. Victims usually die within six months. NEWS BYTES STORIES EXHIBITS LIVE EVENTS ... A New Wave of Mad Cow? Related Exhibits MedTech Exhibit: Beyond the Cutting Edge [view] Related Live Events HIV: Lifecycle of a Virus Presentation [view] Related News Bytes Health News [view] Related Stories Foot and Mouth Disease Diabetes: Are You at Risk? The War on HIV

99. UCSF Memory And Aging Center -- Education: Creutfzeldt-Jakob Disease creutzfeldtjakob disease (CJD). creutzfeldt-jakob disease (CJD or jakob-creutzfeldt disease) is a rapidly progressive, fatal neurodegenerative disease. http://memory.ucsf.edu/Education/education_cjd.html

University of California, San Francisco About UCSF A-Z Web Listing UCSF Search Campus Directory diseases -AD -CBD -CJD -DLB -FTD -HD -MCI ... links education Indicates link to glossary definition CREUTZFELDT-JAKOB DISEASE (CJD) Human prion diseases include: Creutzfeldt-Jakob disease (CJD) Fatal Familial Insomnia (FFI) Kuru New Variant CJD (nvCJD or vCJD) prion disease biology The prion protein is a normal protein found throughout the body and brain. In prion disease, this protein has the ability to take on an abnormal three-dimensional shape and acts as a template that facilitates the conversion of surrounding normal prion proteins into this abnormal form. By this mechanism, abnormal prions accumulate within the brain. The end result of this accumulation is neuronal dysfunction followed by neuronal death and gliosis. On neuropathology the brain shows neuronal loss, gliosis and a spongiform appearance with vacuoles ( vacuolation ) marking sites where the prion protein has injured pre- and post-synaptic terminals.

100. Creutzfeldt-Jakob Disease - Encyclopedia Article About Creutzfeldt-Jakob Disease encyclopedia article about creutzfeldtjakob disease. creutzfeldt-jakob disease in Free online English dictionary, thesaurus and encyclopedia. http://encyclopedia.thefreedictionary.com/Creutzfeldt-Jakob Disease

Dictionaries: General Computing Medical Legal Encyclopedia Creutzfeldt-Jakob Disease Word: Word Starts with Ends with Definition Creutzfeldt-Jakob Disease (CJD) is a brain For other articles about other subjects named brain see brain (disambiguation). In the anatomy of animals, the brain , or encephalon , is the supervisory center of the nervous system. Although the brain is usually cited as the supervisory center of vertebrate nervous systems, the same term can also be used for the invertebrate central nervous system. Click the link for more information. disorder characterized by memory loss, jerky movements, gait A gait can refer to: a particular way or manner of moving on foot: walking and running are the two basic human gaits. horse gaits: any of the ways, such as a canter, trot, or walk, by which a horse can move by lifting the feet in different order or rhythm. See also: Gait Analysis Click the link for more information. disorder, rigid posture While not moving, a human can be: standing; requires sufficient headroom, e.g. it is not possible in a regular car; one can stand freely or lean against a wall, a pole, etc.; sitting; requires a chair or some other low, more or less horizontal structure, e.g. a low wall or a table, or can be done on the ground; special ways of sitting are with the legs horizontal, and in an inclined seat;

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