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  • Variant Creutzfeldt-Jakob Disease

    (Human Mad Cow Disease; vCJD)

    Definition

    Variant Creutzfeldt-Jakob disease (vCJD) is a type of prion disease. Bovine spongiform encephalopathy is a prion disease that affects cows. There is evidence that this illness can be transmitted to humans, producing vCJD. This illness is often called mad cow disease.

    Causes

    Prion diseases are a unique form of infectious diseases. The disease is not produced by a bacterial or viral infection. Instead, the illness is related to the build up of prions, which are infectious protein particles. The central nervous system is slowly damaged as these prions build up.
    The Nervous System
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    Copyright © Nucleus Medical Media, Inc.

    Risk Factors

    Exposure to prion containing tissue is the primary risk factor. Other risk factors include:
    • Eating beef from infected cows
    • Receiving human growth hormone (HGH) injections prior to the mid-1980s—Changes in the preparation of HGH in the mid-1980s eliminated this risk.
    • Working with brain tissue
    • Receiving a corneal or brain lining transplant
    Five to ten percent of all cases of the nonvariant form of Creutzfeldt-Jakob are inherited.

    Symptoms

    The average age of people who get this disease is 29 years old. Rare cases have been reported in children.
    After you are exposed, it can take up to 20 years until symptoms develop. When symptoms develop, they usually follow these three phases:
    • Early phase (0 to 6 months)— psychiatric symptoms, such as depression , anxiety , withdrawal, memory problems, and difficulty pronouncing words
    • Middle phase—neurologic symptoms predominate, such as abnormal gait, problems with coordination, muscle jerks and stiffness, and impaired speech
    • Late phase—mute, immobility
    The average length of time from first symptoms to death is 13 months, with a range of 6-39 months.

    Diagnosis

    The clinical history and physical exam are the primary diagnostic tools. If your doctor suspects vCJD, additional tests may be needed, such as:
      You may need to have your bodily fluids and tissues tested. This can be done with: You may need to have pictures taken of your bodily structures. This can be done with:
    • You may need to have your brain function evaluated. This can be done with an EEG.
    In many cases, final diagnosis requires autopsy and additional studies.

    Treatment

    Currently, there is no cure for vCJD. Treatment is primarily supportive, maximizing function and minimizing discomfort.

    Prevention

    About 200 worldwide cases of vCJD have occurred to date. Most of these were associated with beef consumption in the United Kingdom. There is a great deal of controversy regarding the safety of US beef. Bovine spongiform encephalopathy have been detected in the US. However, no cases of vCJD have been attributed to eating US beef. US patients with vCJD were deemed to have obtained it outside of the US.
    To minimize risk, it is advised that you avoid beef products, particularly processed meat like sausages and hotdogs, or beef items containing brain, spinal cord, or bone marrow.

    RESOURCES

    Creutzfeldt-Jakob Disease Foundation http://www.cjdfoundation.org

    The National CJD Research & Surveillance Unit http://www.cjd.ed.ac.uk

    CANADIAN RESOURCES

    Health Canada http://www.hc-sc.gc.ca

    Public Health Agency of Canada http://www.phac-aspc.gc.ca

    References

    Brown K, Mastrianni JA. The prion diseases. J Geriatr Psychiatry Neurol. 2010;23:277-98.

    Churg-Strauss syndrome. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed . Updated September 5, 2011. Accessed February 21, 2013.

    Creutzfeldt-Jakob disease. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed . Updated March 5, 2012. Accessed February 21, 2013.

    Creutzfeldt-Jakob disease information page. National Institute of Neurological Disorders and Strokes website. Available at: http://www.ninds.nih.gov/disorders/cjd/cjd.htm . Updated May 16, 2012. Accessed February 21, 2013.

    Dawidowska K. Where’s the (safe) beef? Prevention . 2004;56:34.

    Hill AF, Butterworth RJ, Joiner S, et al. Investigation of variant Creutzfeld-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet . 1999;353:1183-1189.

    Holman RC, Belay ED, Christensen KY, et al. Human prion diseases in the United States. PLoS One . 2010;5(1):e8521.

    Kasper DL, Braunwald E, Fauci AS, et al. Harrison's Principles of Internal Medicine. 16th ed. New York, NY: McGraw-Hill; 2005.

    Patry D, Curry B, Easton D, Mastrianni JA, Hogan DB. Creutzfeld-Jakob disease (CJD) after blood product transfusion from a donor with CJD. Neurology. 1998;50(6):1872-1873.

    Rinne ML, McGinnis SM, Samuels MA, Katz JT, Loscalzo J. Clinical problem-solving. A startling decline. N Engl J Med. 2012;366(9):836-842

    Moo-ve over, beef burgers: EN finds many alternatives. Environmental Nutrition . 2004;27:5.

    Prusiner SB. Detecting mad cow disease. Scientific American . 2004;291:60-67.

    Raloff J. Better protection from mad cow disease. Science News . 2004;165:93.

    Smith-Bathgate B. Creutzfeldt-Jakob disease: diagnosis and nursing care issues. Nursing Times . 2005;101:52.

    Variant Creutzfeldt-Jakob disease. World Health Organization website. Available at: http://www.who.int/mediacentre/factsheets/fs180/en/ . Updated February 2012. Accessed February 21, 2013.

    Zeidler M, Sellar R, Collie DA, et al. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet . 2000;355:1412-1419.

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