Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rare, fatal, degenerative brain disorder characterized by rapidly progressive dementia. CJD can be categorized into different subtypes: 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.

  • 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.

  • 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:

    • MRI scan
    • CT scan
    • SPECT imaging


    In many cases, final diagnosis requires
    an
    autopsy
    after death.

    MRI Scan of the Brain
    MRI of the Brain
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  • 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.

  • 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

  • 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