Subacute Sclerosing Panencephalitis
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Subacute Sclerosing Panencephalitis

(SSPE; Dawson Disease)

En Español (Spanish Version)

Definition

Subacute sclerosing panencephalitis (SSPE) is a central nervous system (brain and spine) condition. It occurs rarely, a few years after getting the measles . It usually results in progressive deterioration from inflammation of the brain and nerve cell death. When left untreated, SSPE almost always leads to death. Contact your healthcare provider immediately if you think your or your child may have this condition.

Central Nervous System

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© 2008 Nucleus Medical Art, Inc.

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
  • 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
  • Intellectual deterioration
  • 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, and perform a physical exam. Other tests may include:

  • Blood tests—to look for the measles antibody
  • 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 (eg, inosine pranobex, interferon alpha, interferon beta, ribavirin) may help stabilize the disease and/or delay its progression.

Prevention

The best way to prevent SSPE is to avoid contracting measles, for example, by getting the measles vaccine (usually 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.

Measles. EBSCO Health Library website. Available at: http://www.ebscohost.com/thisMarket.php?marketID=14 . Accessed May 24, 2007.

Solomon T, Knee R. Subacute sclerosing panencephalitis. In: Gilman S, editor. MedLink Neurology. San Diego, CA: MedLink Corporation. Available at: http://www.medlink.com . Accessed August 10, 2007.

Subacute sclerosing panencephalitis (SSPE). EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Accessed May 24, 2007.

Subacute sclerosing panencephalitis information page. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/subacute_panencephalitis/subacute_panencephalitis.htm . Accessed May 24, 2007.



Last reviewed April 2008 by Rimas Lukas, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

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