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Subacute sclerosing panencephalitis


Definition:

Subacute sclerosing panencephalitis (SSPE) is a progressive, debilitating, and deadly brain disorder related to measles (rubeola) infection.

Alternative Names:

SSPE; Subacute sclerosing leukoencephalitis; Dawson´s encephalitis

Causes, incidence, and risk factors:

Ordinarily, the measles virus does not cause brain damage, but an abnormal immune response to measles or, possibly, certain mutant forms of the virus may cause severe illness and death. This leads to brain inflammation (swelling and irritation) that may last for years.

SSPE has been reported in all parts of the world, but in western countries it is considered a rare disease.

Fewer than 10 cases per year are reported in the United States, a dramatic decrease that followed the nationwide measles immunization program. However, in some countries like India, over 20 cases of SSPE per million people are reported each year.

SSPE tends to occur several years after an individual has measles, even though the person seems to have fully recovered from the illness. Males are more often affected than females, and the disease generally occurs in children and adolescents.

Symptoms:
  • Bizarre behavior
  • Dementia (loss of cognitive (thought), emotional, and social abilities)
  • Coma
  • Gradual behavioral changes
  • Myoclonic jerking (quick muscle jerking or spasms)
  • School problems
  • Seizures
  • Unsteady gait
  • Very tense muscles or muscles that lack tone, with weakness in both legs
Signs and tests:

There may be a history of measles in an unvaccinated child. A physical examination may reveal:

  • Damage to the optic nerve, which is responsible for sight
  • Damage to the retina, the part of the eye that receives light
  • Muscle twitching
  • Poor performance on motor (movement) coordination tests

The following tests may be performed:

Treatment:

No cure for SSPE exists. However, certain antiviral drugs may slow the progression of the disease.

Expectations (prognosis):

Persons with this disease frequently die 1 to 2 years after diagnosis, but some may survive for longer periods. The condition is always deadly.

Complications:

Prior to death, there may be:

  • Behavior changes
  • Dementia
  • Stupor and coma
  • Seizures and subsequent injuries
Calling your health care provider:

Call your health care provider if your child has not completed their scheduled vaccines. The measles vaccine is included in the MMR vaccine.

Prevention:

Immunization against measles is the only known prevention for SSPE. The measles vaccine has been highly effective in reducing the numbers of affected children.

Measles immunization should be done according to the recommended American Academy of Pediatrics and Centers for Disease Control schedule. See: MMR immunization

References:

Maldonado YA. Rubeola virus (measles and subacute sclerosing panencephalitis). In: Long SS, ed. Principles and Practice of Pediatric Infectious Diseases. 3rd ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 227.


Review Date: 8/1/2012
Reviewed By: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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