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Alternative names Return to top
SSPE; Subacute sclerosing leukoencephalitis; Dawson´s encephalitisDefinition Return to top
Subacute sclerosing panencephalitis (SSPE) is a progressive, debilitating, and fatal brain disorder caused by infection with a mutant measles (rubeola) virus. A mutant virus is one that has undergone genetic changes (mutations).Causes, incidence, and risk factors Return to top
Ordinarily, the measles virus does not cause brain damage, but certain mutant forms may invade the brain, causing severe illness and death. 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, following 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 (rubeola), 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.
Affected individuals frequently die 1 to 2 years after being diagnosed with this condition, but some may survive for longer periods.
SSPE is due to the direct invasion of brain cells by the mutant measles virus, which provokes brain inflammation (swelling and irritation) that may last for years.
Symptoms Return to top
Signs and tests Return to top
There may be a history of measles in an unvaccinated child. A physical examination may show signs of optic nerve damage, damage to the retina (the part of the eye that perceives light and looks red when a photograph is taken with flash), poor motor cordination tests, and muscle twitching.
The following tests may be performed:
Treatment Return to top
No cure for SSPE exists. However, certain anti-viral drugs can slow the progression of the disease. A combination of oral isoprinosine and interferon alfa injected directly into the brain ventricles appears to be the most effective treatment.
Ribavirin and amantadine are other anti-viral medications that have been used with limited success. Patients responding to treatment need to receive it for the rest of their lives. Effective immunization against measles to prevent development of this condition is the only solution presently available.
Expectations (prognosis) Return to top
SSPE is always fatal.Complications Return to top
Prior to death, there may be:Calling your health care provider Return to top
Call your health care provider if your child has not completed scheduled immunizations. Measles immunization is included in the MMR immunization (vaccine).Prevention Return to top
Immunization against measles is the only known prevention for SSPE. This should be accomplished during the recommended American Academy of Pediatric and Centers for Disease Control immunization schedule. See MMR immunization. Update Date: 8/10/2004 Updated by: Joseph V. Campellone, M.D., Division of Neurology, Cooper University Hospital, Camden, NJ. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |