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—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
    Variant CJD (vCJD) is a different prion disease caused by eating contaminated beef products. This variant form differs from other forms of CJD because it affects younger people and has a longer average time course.
  • Causes

    It is generally believed that CJD is caused by infectious proteins called prions. Prions are normal proteins in the body. If these prions fold up in a different way than normal, they may transform into the protein that causes the illness. The build-up of abnormal prions may be linked to the brain damage associated with CJD.
  • Risk Factors

    People over 50 years old have an increased chance of getting sporadic CJD.
    Family members with CJD increase your chance of getting familial CJD. Approximately 10% of cases are inherited.
    Factors that may increase your chance of getting iatrogenic CJD include:
    • Use of cadaveric growth hormone
    • Cornea transplants
    • Dura mater grafts
    • Healthcare workers who work with brain tissues
    • Blood transfusion from someone with CJD
  • Symptoms

    Symptoms may 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
  • 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. Tests may include:
    • Blood tests
    • Electroencephalogram (EEG) to record the electrical activity of the brain
    • Cerebrospinal fluid analysis
    • Brain biopsy
    • Tonsillar biopsy and brain biopsy
    Imaging tests take pictures of your internal body structures. Imaging tests may include:
    In many cases, final diagnosis requires an autopsy after death.
    MRI Scan of the Brain
    MRI of the Brain
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  • Treatment

    There is no cure for CJD. The aim of treatment is to relieve pain and alleviate symptoms.
    Your doctor may recommend:
    • Prescription pain relievers
    • Anticonvulsive medication for neuromuscular symptoms
  • Prevention

    There are no current guidelines to prevent sporadic CJD.
    If you have a family history of CJD, consider talking to a genetic counselor to better understand your risk.
    The World Health Organization and Centers for Disease Control and Prevention have strict infection control guidelines to prevent iatrogenic CJD.

    Creutzfeldt-Jakob Disease Foundation, Inc.

    National Institute of Neurological Disorders and Stroke


    Health Canada

    Public Health Agency of Canada


    About CJD (Creutzfeldt-Jakob disease, classic). Centers for Disease Control and Prevention website. Available at: Updated November 15, 2012. Accessed June 27, 2013.

    Brown K, Mastrianni JA. The prion diseases. J Geriatr Psychiatry Neurol. 2010;23(4):277-298.

    CDC's diagnostic criteria for Creutzfeldt-Jakob Disease (CJD), 2010. Centers for Disease Control and Prevention website. Available at: Updated August 26, 2010. Accessed June 27, 2013.

    Creutzfeldt-Jakob disease. EBSCO DynaMed website. Available at: Updated March 5, 2012. Accessed June 27, 2013.

    Creutzfeldt-Jakob disease fact sheet. National Institute of Neurological Disorders and Stroke website. Available at: Updated June 6, 2013. Accessed June 27, 2013.

    Mastrianni JA. The genetics of prion disease. Genet Med. 2010;12(4):187-195.

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

    Rinne ML, McGinnis SM, et al. Clinical problem-solving. A startling decline. N Engl J Med. 2012;366(9):836-842.

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