• Subacute Sclerosing Panencephalitis

    (SSPE; Dawson Disease)

    Definition

    Subacute sclerosing panencephalitis (SSPE) is a condition that affects the brain and spine. It is a gradual break down of nerve cells from constant swelling. SSPE is a rare condition that can occur after getting the measles .
    When left untreated, SSPE almost always leads to death. Contact your doctor immediately if you think you or your child may have this condition.
    Central Nervous System
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    Causes

    SSPE is caused by an altered form of the measles virus. It occurs anywhere from 2-10 years after contracting measles.

    Risk Factors

    The following factors are thought to increase the risk of SSPE:
    • Age: 5-15 years old
    • Gender: male
    • Measle infection in infancy
    • Not being vaccinated against measles
    • Ethnicity:
      • Arabs and Sephardic Jews have an incidence that is six times higher than Ashkenazi Jews.
      • Caucasians have a four-fold higher incidence than African Americans in the United States.

    Symptoms

    Symptoms of SSPE may include:
    • Abnormal behavior
    • Irritability
    • Loss of intellectual abilities
    • Memory loss
    • Involuntary movements
    • Seizures
    • Inability to walk
    • Speech impairment with poor comprehension
    • Difficulty swallowing
    • Blindness
    • Muteness
    • Coma

    Diagnosis

    Your doctor will ask about your child’s symptoms and medical history. A physical exam will be done. Tests that may be done include:
    • Blood tests—to look for the measles antibody (sign that there was a measle infection)
    • Electrocardiogram (ECG, EKG)—a test that records the heart’s activity by measuring electrical currents through the heart muscle
    • CT scan
    • MRI scan

    Treatment

    Talk with your doctor about the best treatment plan. Treatment options include:

    Supportive Therapy

    With advanced disease, tube feedings and nursing care may be necessary.

    Medications

    Anticonvulsant medications can reduce some symptoms of SSPE. In addition, there is some evidence that certain medications may help stabilize the disease and/or delay its progression. These may include:
    • Inosine pranobex
    • Interferon alpha
    • Interferon beta
    • Ribavirin

    Prevention

    The best way to prevent SSPE is to avoid contracting measles. The measles vaccine is generally given at 12-15 months of age and again at 4-6 or 11-12 years. If you have not been vaccinated, avoid contact with people who are infected with measles until all of their symptoms are gone.

    RESOURCES

    Centers for Disease Control and Prevention http://www.cdc.gov/

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

    CANADIAN RESOURCES

    Canadian Neurological Sciences Federation http://www.ccns.org/

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

    References

    Campbell H, Andrews N, Brown KE, Miller E. Review of the effect of measles vaccination on the epidemiology of SSPE. Int. J. Epidemiol . 2007;36:1134-48.

    Complications of measles. Center for Disease Control (CDC) website. Available at: http://www.cdc.gov/measles/about/complications.html . Accessed September 20, 2012.

    Measles. EBSCO Health Library website. Available at: http://www.ebscohost.com/thisMarket.php?marketID=14 . Accessed September 20, 2012.

    Subacute sclerosing panencephalitis (SSPE). EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Accessed September 20, 2012.

    Subacute sclerosing panencephalitis information page. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/subacute%5Fpanencephalitis/subacute%5Fpanencephalitis.htm . Updated February 16, 2011. Accessed September 20, 2012.

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