• Creutzfeldt-Jakob Disease

    (Subacute Spongiform Encephalopathy; CJD)

    Definition

    Creutzfeldt-Jakob disease (CJD) is a rare, fatal, degenerative brain disorder characterized by rapidly progressive dementia .
    CJD can be categorized into different subtypes:
    • Sporadic CJD—also called classical CJD; most common type; usually affects people aged 50 years and older
    • Familial CJD—an inherited form of the disease
    • Iatrogenic CJD—contracted through medical procedures, such as injections of growth hormone or receipt of corneal transplants or dura mater implants from affected donors
    • New variant CJD (vCJD) —caused by eating contaminated beef products, which may cause bovine spongiform encephalopathy (BSE)
      • BSE is commonly known as mad cow disease. vCJD differs from other forms of CJD because it affects younger people and has a longer average time course.

    Causes

    There has been a lot of scientific research about the cause of CJD. Today, it is generally believed that most noninherited cases are caused by infectious proteins called prions. Prions can transform normal protein molecules into abnormal, disease-causing molecules.

    Risk Factors

    Factors that can increase your chance of developing CJD include:
    • Age: 50-75 years
    • Use of cadaveric growth hormone
    • Cornea transplants
    • Dura mater grafts
    • Family members with CJD—Approximately 10%-15% of cases are inherited
    • Eating beef products produced by countries with an epidemic of BSE
    • Healthcare workers who work with brain tissues
    • Blood transfusion from someone with CJD

    Symptoms

    Initially, there are no symptoms. As CJD progresses, symptoms that may occur include:
    • Memory lapses
    • Difficulty concentrating
    • Impaired judgment
    • Difficulty with speech
    • Loss of coordination
    • Blurred vision
    • Behavior and mood changes
    • Muscle spasms
    • Seizures
    • Loss of mental and physical function
    Depending on the type of CJD, the disease may last from 3-36 months or longer. It is almost always fatal.

    Diagnosis

    Your doctor will ask about your symptoms and medical history. A physical exam will be done.
    CJD is a difficult disease to diagnose. There is no single test to detect it. The following tests may be used to help make a diagnosis:
    MRI Scan of the Brain
    MRI of the Brain
    Copyright © Nucleus Medical Media, Inc.

    Treatment

    There is no cure for CJD. The aim of treatment is to relieve pain and alleviate symptoms.
    Drug therapy may include:
    • Opiates to treat pain
    • Anticonvulsive drugs to help decrease neuromuscular problems

    Prevention

    To avoid the new variant form of this condition, it is recommended that you avoid eating beef produced in areas that may have BSE. There are no known ways to prevent other forms of CJD.

    RESOURCES

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

    National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov

    World Health Organization http://www.who.int/en

    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(4):277-98.

    Churg-Strauss syndrome. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated September 5, 2011. Accessed February 25, 2013.

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

    Mastrianni JA. The genetics of prion disease. Genet Med. 2010;12(4):187-95.

    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):836-42

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