Subacute Sclerosing Panencephalitis

(SSPE; Dawson Disease)

Definition

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

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

Risk Factors

SSPE is more common in male, 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

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

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

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

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.

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
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_panencephalitis/subacute_panencephalitis.htm
Updated February 16, 2011. Accessed May 20, 2013.

Last reviewed January 2015 by Rimas Lukas, MD
Last Updated: 5/6/2014

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