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    Creutzfeldt Jakob Disease

    Important
    It is possible that the main title of the report Creutzfeldt Jakob Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

    Synonyms

    • CJD
    • Jakob-Creutzfeldt Disease
    • Subacute Spongiform Encephalopathy
    • Jakob's Disease

    Disorder Subdivisions

    • Variant Creutzfeldt-Jakob Disease (V-CJD)

    General Discussion

    Creutzfeldt-Jakob disease (CJD) is an extremely rare degenerative brain disorder (i.e., spongiform encephalopathy) characterized by sudden development of rapidly progressive neurological and neuromuscular symptoms. With symptom onset, affected individuals may develop confusion, depression, behavioral changes, impaired vision, and/or impaired coordination. As the disease progresses, there may be rapidly progressive deterioration of cognitive processes and memory (dementia), resulting in confusion and disorientation, impairment of memory control, personality disintegration, agitation, restlessness, and other symptoms and findings. Affected individuals also develop neuromuscular abnormalities such as muscle weakness and loss of muscle mass (wasting); irregular, rapid, shock-like muscle spasms (myoclonus); and/or relatively slow, involuntary, continual writhing movements (athetosis), particularly of the arms and legs. Later stages of the disease may include further loss of physical and intellectual functions, a state of unconsciousness (coma), and increased susceptibility to repeated infections of the respiratory tract (e.g., pneumonia). In many affected individuals, life-threatening complications may develop less than a year after the disorder becomes apparent.

    In approximately 90 percent of cases, CJD appears to occur randomly for no apparent reason (sporadically). About 10 percent of affected individuals may have a hereditary predisposition for the disorder. Reports in the medical literature suggest that familial cases of CJD are consistent with an autosomal dominant mode of inheritance. In addition, in some extremely rare cases, CJD may take an infectious form. The disorder is thought to result from changes (mutations) in the gene that regulates the production of the human prion protein or direct contamination (transmission) with abnormal prion protein in infected brain tissue.

    A variant form of CJD (V-CJD) has been reported in the United Kingdom that affects younger people (median age at onset: 28 years) than does classic CJD. In 1996, experts suggested the possibility that this variant might be associated with consumption of beef from cows with a related infectious brain disorder known as Bovine Spongiform Encephalopathy (BSE) or "Mad Cow Disease." BSE was first identified in the UK in 1986 and the number of reported cases grew rapidly, peaking in the winter of 1992-93 when almost 1,000 new cases were reported each week. Later, BSE also began to appear in some other European countries. Scientific research and debate continue concerning the potential link between BSE and V-CJD. In addition, coordinated national and international efforts are in place concerning the prevention, study, and surveillance of BSE and CJD. In early December 2000, European Union agriculture ministers agreed upon new measures to combat the spread of mad cow disease, including incinerating any cow over 30 months of age that had not tested negative for BSE. (BSE is thought to become detectable and infectious when cattle are approximately 30 months old.)

    Resources

    Alzheimer's Association
    225 N. Michigan Avenue
    17th Floor
    Chicago, IL 60601
    USA
    Tel: (312)335-8700
    Fax: (866)699-1246
    Tel: (800)272-3900
    TDD: (312)335-5886
    Email: info@alz.org
    Internet: http://www.alz.org

    Alzheimer's Disease Education and Referral Center
    P.O. Box 8250
    Silver Spring, MD 20907-8250
    Tel: (301)495-3311
    Fax: (301)495-3334
    Tel: (800)438-4380
    Email: adear@nia.nih.gov
    Internet: http://www.nia.nih.gov/alzheimers

    Centers for Disease Control and Prevention
    1600 Clifton Road NE
    Atlanta, GA 30333
    Tel: (404)639-3534
    Tel: (800)232-4636
    TDD: (888)232-6348
    Email: cdcinfo@cdc.gov
    Internet: http://www.cdc.gov/

    National Hospice and Palliative Care Organization
    1731 King Street, Suite 100
    Alexandria, VA 22314
    USA
    Tel: (703)837-1500
    Fax: (703)837-1233
    Tel: (800)658-8898
    Email: nhpco_info@nhpco.org
    Internet: http://www.nhpco.org

    NIH/National Institute of Allergy and Infectious Diseases
    Office of Communications and Government Relations
    6610 Rockledge Drive, MSC 6612
    Bethesda, MD 20892-6612
    Tel: (301)496-5717
    Fax: (301)402-3573
    Tel: (866)284-4107
    TDD: (800)877-8339
    Email: ocpostoffice@niaid.nih.gov
    Internet: http://www.niaid.nih.gov/

    NIH/National Institute of Neurological Disorders and Stroke
    P.O. Box 5801
    Bethesda, MD 20824
    Tel: (301)496-5751
    Fax: (301)402-2186
    Tel: (800)352-9424
    TDD: (301)468-5981
    Internet: http://www.ninds.nih.gov/

    World Health Organization (WHO)
    Avenue Appia 20
    Geneva 27, 1211
    Switzerland
    Tel: 41227912111
    Fax: 41227913111
    Internet: http://www.who.int/en/

    Creutzfeldt-Jakob Disease Foundation, Inc.
    PO Box 5312
    Akron, OH 44334
    USA
    Tel: (330)665-5590
    Fax: (330)668-2474
    Tel: (800)659-1991
    Email: help@cjdfoundation.org
    Internet: http://www.cjdfoundation.org

    CJD Voice
    Internet: http://www.cjdvoice.org

    CJD Aware!
    2527 South Carrollton Avenue
    New Orleans, LA 70118-3013
    USA
    Tel: (504)861-4627
    Email: info@cjdaware.com
    Internet: http://www.cjdaware.com

    National Prion Disease Pathology Surveillance Center
    Institute of Pathology
    Case Western Reserve University
    2085 Adelbert Road, Room 418
    Cleveland, OH 44106-4907
    USA
    Tel: (216)368-0587
    Fax: (216)368-4090
    Email: cjdsurv@cwru.edu
    Internet: http://www.cjdsurveillance.com

    Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg, MD 20898-8126
    Tel: (301)251-4925
    Fax: (301)251-4911
    Tel: (888)205-2311
    TDD: (888)205-3223
    Internet: http://rarediseases.info.nih.gov/GARD/

    UCSF Memory and Aging Center
    350 Parnassus Avenue
    Suite 905
    San Francisco, CA 94117
    Tel: (415)476-6880
    Fax: (415)476-4800
    Email: mgeschwind@memory.ucsf.edu
    Internet: http://www.memory.ucsf.edu

    C-Mac Informational Services, Inc.
    120 Clinton Lane
    Cookeville, TN 38501-8946
    Tel: (931)268-1201
    Email: caregiver_cmi@hotmail.com
    Internet: http://www.caregivernews.org

    For a Complete Report:

    This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

    The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

    It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

    This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

    For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

    Last Updated: 4/7/2009
    Copyright 1984, 1985, 1987, 1988, 1990, 1991, 1992, 1994, 1996, 1997, 1998, 1999, 2000, 2001, 2003, 2007, 2009 National Organization for Rare Disorders, Inc.

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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