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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. Contact your doctor right away if you think you or your child may have this condition.
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

Factors that may increase the risk of SSPE include:
  • Age: 5-15 years old
  • Gender: male
  • Measles 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 understanding
  • Difficulty swallowing
  • Blindness
  • Muteness
  • Loss of consciousness

Diagnosis

Your doctor will ask about your child’s symptoms and medical history. A physical exam will be done.
Your bodily fluids may be tested. This can be done with blood tests.
Images may need to be taken of your bodily structures. This can be done with:

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 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;36:1134-48.

Complications of measles. Center for Disease Control (CDC) website. Available at: http://www.cdc.gov/measles/about/complications.html . Updated October 23, 2012. Accessed May 20, 2013.

Measles. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated April 4, 2013. Accessed May 20, 2013.

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 May 20, 2013.

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