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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.
    When left untreated, SSPE almost always leads to death.
    Central Nervous System
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  • Causes

    SSPE is caused either by an altered form of the measles virus or an abnormal immune response to measles. It occurs anywhere from 2-10 years after contracting measles.
  • Risk Factors

    SSPE is more common in males, and in those aged 5-15 years old. Other factors that may increase your chance of SSPE include:
    • Measles infection in infancy
    • Not being vaccinated against measles
    • Ethnicity:
      • Arabs and Sephardic Jews have an incidence that is 6 times higher than Ashkenazi Jews.
      • Caucasians have a 4-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 understanding
    • Difficulty swallowing
    • Blindness
    • Muteness
    • Loss of consciousness
  • Diagnosis

    Your doctor will ask about your symptoms and medical history. A physical exam will be done.
    Tests may include blood tests and an electrocardiogram (EKG).
    Imaging tests to evaluate bodily structures may include:
  • 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.
  • Prevention

    The best way to prevent SSPE is to get immunized 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.cnsfederation.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;36:1134-1148.

    Chiu MH, Meatherall B, Nikolic A, et al. Lancet Infect Dis. 2016 Jan [Epub ahead of print].

    Complications of measles. Center for Disease Control (CDC) website. Available at: http://www.cdc.gov/measles/about/complications.html. Updated February 17, 2015. Accessed February 12, 2016.

    Measles. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated February 11, 2016. Accessed February 12, 2016.

    NINDS 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 April 30, 2015. Accessed February 12, 2016.

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