61. Creutzfeldt-Jakob Disease (CJD) creutzfeldtjakob disease is a rapid, progressive neurological disease that can affect people and animals. http://alzheimers.about.com/od/diagnosisissues/a/cjd.htm

var zLb=2; zJs=10 zJs=11 zJs=12 zJs=13 zc(5,'jsc',zJs,9999999,'') zfs=0;zCMt='a26' About Alzheimer's Disease Diagnosis Types of Dementia Creutzfeldt-Jakob Disease (CJD) Alzheimer's Disease Essentials What is Alzheimer's ? Mini Mental State Examination ... Help zau(256,140,140,'el','http://z.about.com/0/ip/417/C.htm','');w(xb+xb+' ');zau(256,140,140,'von','http://z.about.com/0/ip/496/6.htm','');w(xb+xb); FREE Newsletter Sign Up Now for the Alzheimer's Disease newsletter! See Online Courses Search Alzheimer's Disease Stay up to date! Compare Prices Email to a friend ... Print this page Recent Discussions Need your help New here... Oral hygiene Most Popular Stages of Alzheimer's The Alzheimer's Quiz Mini Mental State Examination Blood Pressure Machines ... 6 Types of Elder Abuse What's Hot 6 Types of Elder Abuse Mini Mental State Examination A Social Worker's Perspective Can cells cure Alzheimer's? ... Psychiatric medication adunitCM(150,100,'x55') Creutzfeldt-Jakob Disease From Christine Kennard Your Guide to Alzheimer's Disease FREE Newsletter. Sign Up Now! Creutzfeldt-Jakob disease (CJD) is a very rare dementing disease first identified in the 1920s. Creutzfeldt-Jakob disease is believed to be caused by something called proteinaceous prions that can live in the body for years before any signs of the disease become obvious. CJD affects people between the ages of 50 and 70 years.

62. Creutzfeldt-Jakob Disease Despite the press around the socalled human form of mad cow disease, creutzfeldt-jakob (CJD)disease is rare. In this article I review the main features of http://alzheimers.about.com/b/a/168514.htm

zJs=10 zJs=11 zJs=12 zJs=13 zc(5,'jsc',zJs,9999999,'') zfs=0;zCMt='a26' About Alzheimer's Disease Alzheimer's Disease Essentials ... Help zau(256,140,140,'el','http://z.about.com/0/ip/417/C.htm','');w(xb+xb+' ');zau(256,140,140,'von','http://z.about.com/0/ip/496/6.htm','');w(xb+xb); FREE Newsletter Sign Up Now for the Alzheimer's Disease newsletter! See Online Courses Search Alzheimer's Disease Creutzfeldt-Jakob Disease Alzheimers Disease Blog Main From Christine Kennard Your Guide to Alzheimer's Disease FREE Newsletter. Sign Up Now! May 09, 2005 Creutzfeldt-Jakob Disease Despite the press around the so-called human form of mad cow disease, Creutzfeldt-Jakob (CJD)disease is rare. In this article I review the main features of this neuro-degenerative disease that causes dementia and, for most people, death within a year. Read more about Creutzfeldt-Jakob Disease Email to a Friend Display Latest Headlines Read Archives powered by Movable Type Advertisement Most Popular Stages of Alzheimer's The Alzheimer's Quiz Mini Mental State Examination Blood Pressure Machines ... 6 Types of Elder Abuse What's Hot 6 Types of Elder Abuse Mini Mental State Examination A Social Worker's Perspective Can cells cure Alzheimer's?

63. NEJM -- Sign In Perspective from The New England Journal of Medicine creutzfeldtjakob disease. http://content.nejm.org/cgi/content/full/348/8/681

HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP Access to this article requires sign-in. Subscribers have access to all content. Other registered users have access to research articles six months old and older. If you're a registered user or an activated subscriber SIGN IN User Name Password Forgot your Password? Click here and we'll e-mail it to you. If you do not use cookies, sign in here. Remember my User Name and Password. Log in via Athens. PURCHASE THIS ARTICLE Purchase a single article and get immediate online access for just $10. If you're a subscriber but have not yet activated your full online access If you'd like to purchase a subscription to NEJM If you're not a subscriber and want FREE limited access... ACTIVATE YOUR SUBSCRIPTION Subscribers to NEJM are entitled to full access to all online content and features, including 20 FREE online CME exams. OR Receive full access to ALL current content and online features including Personal Archives, PDF article downloads, PDA access, E-mail alerts and 20 FREE online CME exams. OR Receive FREE online access to NEJM Original and Special Articles 6 months after publication and choose to receive the Table of Contents and notification of early release articles via e-mail.

64. NEJM -- Extraneural Pathologic Prion Protein In Sporadic Creutzfeldt-Jakob Disea Background In patients with sporadic creutzfeldt–jakob disease, creutzfeldtjakob disease Presenting as Refractory Nonconvulsive Status Epilepticus. http://content.nejm.org/cgi/content/abstract/349/19/1812

HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP Please sign in for full text and personal services Previous Volume 349:1812-1820 November 6, 2003 Number 19 Next Markus Glatzel, M.D., Eugenio Abela, Manuela Maissen, M.S., and Adriano Aguzzi, M.D., Ph.D. Full Text PDF PDA Full Text PowerPoint Slide Set ... PubMed Citation ABSTRACT Background disease, pathologic disease-associated prion protein (PrP Sc has been identified only in the central nervous system and olfactory-nerve tissue. Understanding the distribution of PrP Sc disease is important for classification and diagnosis and perhaps even for prevention. Methods involving the concentration of PrP Sc by differential precipitation with sodium phosphotungstic acid, which increased the sensitivity of Western blot analysis by up to three orders of magnitude Sc in extraneural organs of 36 patients 1996 and 2002. Results PrP Sc was present in the brain tissue of all patients. In addition, we found PrP Sc in 10 of 28 spleen specimens and in 8 of 32 skeletal-muscle samples. Three patients had PrP Sc in both spleen and muscle specimens. Patients with extraneural

65. Creutzfeldt-Jakob Disease creutzfeldtjakob disease. From MayoClinic.com Special to CNN.com. Overview. Before the mad cow disease epidemic in the mid-1990s, few people had heard of http://www.cnn.com/HEALTH/library/DS/00531.html

International Edition MEMBER SERVICES The Web CNN.com Home Page World U.S. Weather ... Autos SERVICES Video E-mail Newsletters Your E-mail Alerts RSS ... Contact Us SEARCH Web CNN.com In association with: Degenerative Diseases Alzheimer's disease Creutzfeldt-Jakob disease Mild cognitive impairment INFORMATION CENTERS: Pick a category Health Centers Family Health Men's Health Women's Health Children's Health Seniors' Health Working Life Pain Management Condition Centers Immune System Allergy Alzheimer's Arthritis Respiratory System Cancer Endocrine System Digestive System Heart and Blood Infectious Disease Mental Health Note: All links within content go to MayoClinic.com Diseases and Conditions Creutzfeldt-Jakob disease From MayoClinic.com Special to CNN.com Overview Before the mad cow disease epidemic in the mid-1990s, few people had heard of Creutzfeldt-Jakob disease (CJD). That's because this invariably fatal, degenerative brain disorder has always been considered rare. Worldwide, doctors typically diagnose one case per million people each year, most commonly in older adults. Although "classic" CJD doesn't appear to be connected to mad cow disease, it's similar to vCJD in many respects. Both are thought to occur when misshapen brain proteins (prions) attack brain cells, creating sponge-like holes in brain tissue. Both cause profound mental and physical deterioration, and both appear to have long incubation periods before signs and symptoms appear.

66. Classic Creutzfeldt-Jakob Disease In Canada - CCDR Volume 28s5 November 2002 Several parasitic diseases have emerged recently in Canada, mainly as the result of cases detected in Canadian travelers, immigrants, or refugees. http://www.phac-aspc.gc.ca/publicat/ccdr-rmtc/02vol28/28s5/

Infection Control G uidelines Classic Creutzfeldt-Jakob Disease in Canada Health Canada Centre for Infectious Disease Prevention and Control Bureau of Infectious Diseases* * Now the Health Care Acquired Infections Division, Blood-borne Pathogens Section. Classic Creutzfeldt-Jakob Disease in Canada 110 Pages - 403 KB in PDF Format Table of Contents Executive Summary Table of Contents Summary Health Canada's Infection Control Guidelines Program List of Participants Steering Committee on Infection Control Guidelines Health Canada AD HOC Advisory Committee on Infection Prevention and Control and Creutzfeldt-Jakob Disease Acknowledgements Introduction A. How to Use this Document B. Glossary and Abbreviations (See Appendix II for CJD Surveillance Definitions) C. The Purpose of the Infection Prevention and Control Guidelines for CJD and Other Human TSEs D. Goals of the Guideline Part A. Overview of CJD and Other Human TSEs Background Information A. Etiology

67. Statistics - Creutzfeldt-Jakob Disease - Blood Safety Surveillance And Health Ca creutzfeldtjakob disease - Blood Safety Surveillance and Health Care Acquired Infections Division. http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats_e.html

Creutzfeldt-Jakob Disease CJD Home Page CJD-SS Family Information Physician Information ... United States Creutzfeldt-Jakob Disease (CJD) Creutzfeldt-Jakob Disease Surveillance System (CJD-SS) Toll Free: 1-888-489-2999 Statistics Referrals of Suspected CJD Reported to CJD-SS CJD Cases Reported to CJD-SS CJD Cases Reported by Province Incidence of CJD Cases Reported to CJD-SS in Canada ... How to download PDF documents Referrals of Suspected CJD Reported to CJD-SS As of August 1, 2005 Year of Reporting # of Referrals Total Referrals Notes: CJD-SS began in April 1998 Data before April 1998 is retrospective and partial, data from 1999 to 2003 is complete, and data for 2004/2005 is provisional Adobe Downloadable Document (33 KB) CJD Deaths Reported to CJD-SS As of August 1, 2005 Deaths of Definite and Probable CJD Year Sporadic Iatrogenic Familial GSS vCJD Total Total Notes: CJD-SS began in April 1998 Data before April 1998 is retrospective and partial, data from 1999 to 2003 is complete, and data for 2004/2005 is provisional

68. Creutzfeldt-Jakob Disease creutzfeldtjakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. 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.

69. Creutzfeldt-Jakob Disease (CJD) Fact Sheet What is creutzfeldtjakob disease (CJD)? CJD is a rare incurable disease of humans that affects the nervous system and results in rapidly progressive http://dhfs.wisconsin.gov/communicable/communicable/factsheets/creutzfeldt.htm

Topics A-Z Reference Center Search AIDS-HIV ... Tuberculosis Disease Fact Sheet Series: Creutzfeldt-Jakob Disease (CJD) Printable Version What is Creutzfeldt-Jakob disease (CJD)? CJD is a rare incurable disease of humans that affects the nervous system and results in rapidly progressive dementia, loss of motor control, paralysis, and death. It is one of several related diseases called transmissible spongiform encephalopathies or TSEs for short. The term "encephalopathy" means the brain is affected, and the term "spongiform" refers to the microscopic holes seen in the brain, giving it a sponge-like appearance. Various animal species have distinct types of TSEs. In addition to CJD which affects humans, other TSEs include bovine spongiform encephalopathy (BSE, also known as "mad cow disease"), scrapie in sheep, and chronic wasting disease (CWD) in deer and elk. What causes CJD? It is caused by a recently identified agent called a prion, which is a self-replicating protein. The current theory is that the normal form of the prion, found in all people, is converted into an abnormal form. This abnormal CJD prion produces the brain lesions that result in the disease. What causes this conversion of normal protein into a disease producing prion?

70. LII - Results For "creutzfeldt-jakob Disease" World Health Organization Fact Sheets Variant creutzfeldtjakob disease As with creutzfeldt-jakob disease, vCJD is classified as a Transmissible http://www.lii.org/search?searchtype=subject;query=Creutzfeldt-Jakob disease;sub

71. Creutzfeldt-Jakob Disease CHC Wausau Hospital s Medical Library and Patient Education Center provides research services and healthcare information to physicians, http://www.chclibrary.org/micromed/00044340.html

Main Search Index Definition Description Causes ... Resources Creutzfeldt-Jakob disease Definition Creutzfeldt-Jakob disease (CJD) is a transmissible, rapidly progressing, fatal neurodegenerative disorder related to "mad cow disease." Description Before 1995, Creutzfeldt-Jakob disease was little-known outside of the medical profession; even within it, most doctors did not know much about it, and hardly any had ever seen a case. But with the discovery of a "new variant" form, the possibility that those with it became infected simply by eating beef, and the radical theory that the infectious agent is a rogue protein, CJD has become one of the most talked-about diseases in the world, and has taken on a significance far beyond the small number of deaths it currently causes each year. First described in the 1920s, CJD is a neurodegenerative disease causing a rapidly progressing dementia which ends in death, usually within eight months of the onset of symptoms. It is also a very rare disease, affecting only about one in every million members of the population worldwide. In the United States, CJD is thought to affect about 250 people each year. CJD affects adults of all ages, but is rare in young adults and most common between ages 50 and 75. Spongiform encephalopathies The most obvious pathologic feature of CJD is the formation of numerous fluid-filled spaces in the brain (vacuoles), giving it a sponge-like appearance. CJD is one of several human "spongiform encephalopathies," diseases that produce this characteristic change in brain tissue. Others are kuru; Gerstmann-Straussler- Scheinker disease, predominantly characterized by cerebellar ataxia; and fatal familial insomnia, associated with progressive insomina, autonomic system disfunction, and weakness caused by motor system disfunction.

72. Encyclopaedia Topic : Creutzfeldt-Jakob Disease, Section : Introduction NHS Direct Online Health Encyclopaedia. creutzfeldtjakob disease creutzfeldt-jakob disease (CJD) is a rapidly progressing disease of the nervous system http://www.nhsdirect.nhs.uk/en.asp?TopicID=139

73. InteliHealth: Creutzfeldt-Jakob Disease InteliHealth Featuring Harvard Medical School s consumer health information. For more than 550 diseases and conditions, learn What Is It?, Symptoms, http://www.intelihealth.com/IH/ihtIH/WSIHW000/9339/9768.html

chrome_imgPreload('gifChr_mid_but_home_mo_1','http://img.intelihealth.com/i/C/Chr_mid_but_home-o.gif'); chrome_imgPreload('gifChr_mid_but_comm_mo_2','http://img.intelihealth.com/i/C/Chr_mid_but_comm-o.gif'); chrome_imgPreload('gifChr_mid_but_dental_mo_3','http://img.intelihealth.com/i/C/Chr_mid_but_dental-o.gif'); chrome_imgPreload('gifChr_mid_but_drug_mo_4','http://img.intelihealth.com/i/C/Chr_mid_but_drug-o.gif'); chrome_imgPreload('gifChr_mid_but_askexpert_mo_5','http://img.intelihealth.com/i/C/Chr_mid_but_askexpert-o.gif'); chrome_imgPreload('gifChr_mid_but_medical_mo_6','http://img.intelihealth.com/i/C/Chr_mid_but_medical-o.gif'); chrome_imgPreload('gifChr_mid_but_chats_mo_7','http://img.intelihealth.com/i/C/Chr_mid_but_chats-o.gif'); chrome_imgPreload('gifchr_mid_but_news_mo_8','http://img.intelihealth.com/i/c/chr_mid_but_news-o.gif'); chrome_imgPreload('gifChr_mid_but_privacy_mo_9','http://img.intelihealth.com/i/C/Chr_mid_but_privacy-o.gif'); Advertisement Creutzfeldt-Jakob Disease (CJD) What Is It? Symptoms Diagnosis Expected Duration ... Additional Info What Is It? Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain disorder associated with the presence of a misshapen protein in the brain, known as a prion. Although prion-associated diseases are not well understood, scientists theorize that prions cause damage by causing healthy proteins in the brain to take the prion shape, so these too become damaging particles. This slow chain reaction of damaged proteins occurs over many years and results in brain damage.

74. Mad Cow Disease (Harpers.org) Apr 8, Three deer hunters have died of creutzfeldtjakob disease, it was reported, and two of them were good friends who hunted together. http://www.harpers.org/MadCowDisease.html

Mad Cow Disease Sources Events Related To Mad Cow Disease USDA officials said that there was no need to test all cattle for mad cow disease before they are eaten [Newsday] Week of Aug 1 A British Health Department bulletin revealed that fourteen Britons have died of mad cow disease so far this year; scientists have said that 500,000 people could die of the disease by 2030. A family of Vermont sheep farmers vowed to prevent the government from slaughtering their flock of Belgian dairy sheep; four sheep descended from the flock tested positive for an ovine form of mad cow disease Week of Oct 31 French supermarkets were selling meat thought to be contaminated with mad cow disease Week of Nov 7 People in Galway, Ireland, dug up the carcass of a mad cow and placed it in the owner's farmyard; burying a cow suffering from bovine spongiform encephalapathy could contaminate ground water with the prions that apparently cause the disease Scientists warned again that chronic wasting disease , a type of transmissible spongiform encephalopathy infecting deer and elk in the American West, can probably be transmitted to humans, much like its cousin mad cow disease ; up to half the deer in some areas are infected with the disease.

75. Alzheimer's Society Information Sheet - What Is CJD? The bestknown prion disease is creutzfeldt-jakob disease, or CJD. These include creutzfeldt-jakob disease, as well as two extremely rare inherited 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.

76. Creutzfeldt-Jakob Disease (CJD) Definition - Medical Dictionary Definitions Of P Online Medical Dictionary and glossary with medical definitions. http://www.medterms.com/script/main/art.asp?articlekey=2863

77. Creutzfeldt-Jakob Disease: Types, Symptoms And Treatment Discusses creutzfeldtjakob disease symptoms, diagnosis, and medications, and provides tips for people with the disease. http://www.helpguide.org/elder/creutzfeldt_jakob.htm

Home About Us Contact Site Map Search Helpguide Rotary Club of Santa Monica Creutzfeldt-Jakob Disease: Types, Symptoms and Treatment On this page: What is Creutzfeldt-Jakob Disease? What are the types of Creutzfeldt-Jakob Disease? What are the symptoms of Creutzfeldt-Jakob Disease? What is the treatment for Creutzfeldt-Jakob Disease? ... Related articles Did You Know? Creutzfedlt-Jakob Disease (CJD) is a the most known prion disease which affects humans. Prion diseases are rare, invariably fatal brain disorders, which occur both in humans and certain animals. They first came to public attention in the mid 1980s in the form of the BSE epidemic in the United Kingdom. BSE (bovine spongiform encephalopathy) is also known as mad cow disease and it is a prion disease found in cattle. About one person per million per year is affected by Creutzfeldt-Jakob, which means about 250-300 new cases in the United States per year. (Information provided by Creutzfeldt-Jakob Foundation What is Creutzfeldt-Jakob Disease? Creutzfeldt-Jakob Disease is degenerative brain disorder, known as a prion disease. Prions are types of protein. Normal prion proteins are harmless, but infectious forms of the prion protein are thought to cause Creutzfeldt-Jakob disease and other diseases. Prion diseases affect both humans and certain animals, including cows and sheep. Mad cow disease (bovine spongiform encephalopathy) is a prion disease. Prion diseases are rare and fatal. Creutzfeldt-Jakob Disease affects men and women between the ages of 50-75 years. The incident rate is reportedly one case per million people a year, although this may be a low estimate because the disease is very difficult to diagnose. There is no definitive diagnosis without a brain biopsy or an autopsy, and the procedures are not done very often because they do not help the patient.

78. Australian Creutzfeldt-Jakob Disease Support Group - Contact Us Suzanne Solvyns 13 Araluen Place GLENHAVEN NSW 2156. National Toll Free 1800 052 466 Tel (02) 9894 5168 Fax (02) 9899 8905 email s.solvyns@cjdsupport.org.au http://www.cjdsupport.org.au/supportgroup/contactus.html

CJD SUPPORT GROUP NETWORK Background Support Group Purpose Structure Contact Us Chronology of Events Testimonials Calendar / Events Overview of CJD Articles of Interest Infection Control Guidelines Counselling FAQ Dedication Overseas Support Networks Links email us Directory - CJD Support Groups Postal Address CJD Support Group Pty. Ltd. Reply Paid 70 13 Araluen Place GLENHAVEN NSW 2156 Coordinators NSW, ACT and QLD Suzanne Solvyns 13 Araluen Place GLENHAVEN NSW 2156 National Toll Free 1800 052 466 Tel Fax email s.solvyns@cjdsupport.org.au TAS, VIC, and WA Carol Wilson 2399 Gordon River Road NATIONAL PARK TAS 7140 National Toll Free 1800 818 683 Tel Fax email c.wilson@cjdsupport.org.au SA David Mullan 47 Crown Terrace ROYAL PARK SA 5014 Tel Fax email d.mullan@cjdsupport.org.au Human Growth Recipient (hGH) Representative David Ralston 64 Berallier Drive SOUTH CAMDEN NSW 2570 Tel Fax email d.ralston@cjdsupport.org.au Department of Health and Ageing General information recipients for human pituitary hormone recipients National Toll Free 1800 802 306 CJDSGN acknowledges the funding provided by the Department of Health and Ageing (DoHA) for the provision and maintenance of this website. CJDSGN asserts that apart from providing the funding the DoHA is not involved in anyway in the complication of this website and the views expressed herein.

79. Creutzfeldt-Jakob Disease - Health And Medical Information Produced By Doctors - creutzfeldtjakob disease - Health and Medical Information Doctor-produced health and medical information written for you to make informed decisions about your health concerns. http://www.medicinenet.com/Creutzfeldt-Jakob_Disease/article.htm

document.writeln(''); MedicineNet Home > Creutzfeldt-Jakob Disease search help Printer-Friendly Format FREE Newsletters Email to a Friend ... Next 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.

80. THE MERCK MANUAL--SECOND HOME EDITION, Creutzfeldt-Jakob Disease In Ch. 90, Prio creutzfeldtjakob disease affects 1 of 1 million people each year throughout the world. One form of creutzfeldt-jakob disease results from spontaneous http://www.merck.com/mmhe/sec06/ch090/ch090b.html

var externalLinkWarning = "The link you have selected will take you to a site outside Merck and The Merck Manuals.*n*nThe Merck Manuals do not review or control the content of any non-Merck site. The Merck Manuals do not endorse and are not responsible for the accuracy, content, practices, or standards of any non-Merck sources."; Search The Second Home Edition , Online Version Search Index A B C D ... Z Sections Accidents and Injuries Blood Disorders Bone, Joint, and Muscle Disorders Brain, Spinal Cord, and Nerve Disorders ... Women's Health Issues Resources Anatomical Drawings Multimedia Pronunciations Weights and Measures ... , Online Version Section Brain, Spinal Cord, and Nerve Disorders Chapter Prion Diseases Topics Introduction Creutzfeldt-Jakob Disease Fatal Familial Insomnia Gerstmann-Str¤ussler-Scheinker Disease Kuru Creutzfeldt-Jakob Disease Buy The Book Print This Topic Email This Topic Pronunciations cerebrospinal fluid dementia electroencephalography encephalography ... valproate Creutzfeldt-Jakob disease (subacute spongiform encephalopathy) is a prion disease characterized by progressive deterioration of mental function, muscle twitching, and staggering when walking. Creutzfeldt-Jakob disease affects 1 of 1 million people each year throughout the world. It affects adults, mainly in their late 50s and 60s.

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