Carl R. Darnall Army Medical Center - Health Library

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
si1210_97870_1_central_nervous

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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 2-10 years after contracting measles.

Risk Factors  ^

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  ^

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  ^

You will be asked 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 needed.

Medications

Antiseizure 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. Vaccination is the primary means of preventing 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: http://www.cdc.gov/measles/about/complications.html. Updated March 3, 2017. Accessed February 15, 2018.

Measles. EBSCO DynaMed Plus website. Available at:http://www.dynamed.com/topics/dmp~AN~T116399/Measles. Updated August 9, 2017. Accessed February 15, 2018.

NINDS subacute sclerosing panencephalitis information page. National Institute of Neurological Disorders and Stroke website. Available at: https://www.ninds.nih.gov/Disorders/All-Disorders/Subacute-Sclerosing-Panencephalitis-Information-Page. Accessed February 15, 2018.

Rota PA, Moss WJ, Takeda M, et al. Measles. Nat Rev Dis Primers. 2016;2:16049.

Last reviewed February 2018 by EBSCO Medical Review Board Rimas Lukas, MD  Last Updated: 2/12/2016