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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 breakdown of nerve cells from constant swelling.

When left untreated, SSPE almost always leads to death.

Central Nervous System

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Copyright © Nucleus Medical Media, Inc.

Causes    TOP

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    TOP

SSPE is more common in males, and in those aged 5-15 years. 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 US.

Symptoms    TOP

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    TOP

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    TOP

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    TOP

The best way to prevent SSPE is to get immunized to avoid contracting measles. The measles vaccine is generally given at age 12-15 months and again at age 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
https://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:
...(Click grey area to select URL)
Updated February 17, 2015. Accessed February 12, 2016.
Measles. EBSCO DynaMed Plus website. Available at:
...(Click grey area to select URL)
Updated October 19, 2016. Accessed September 28, 2016.
NINDS subacute sclerosing panencephalitis information page. National Institute of Neurological Disorders and Stroke website. Available at:
...(Click grey area to select URL)
Updated April 30, 2015. Accessed February 12, 2016.
Last reviewed March 2017 by EBSCO Medical Review Board Rimas Lukas, MD
Last Updated: 2/12/2016

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