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Alternative Names Return to topSSPE; Subacute sclerosing leukoencephalitis; Dawson´s encephalitis
Definition Return to top
Subacute sclerosing panencephalitis (SSPE) is a progressive, debilitating, and deadly brain disorder related to measles (rubeola) infection.
Causes Return to top
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 Return to top
Exams and Tests Return to top
There may be a history of measles in an unvaccinated child. A physical examination may reveal:
The following tests may be performed:
Treatment Return to top
No cure for SSPE exists. However, certain antiviral drugs may slow the progression of the disease.
Outlook (Prognosis) Return to top
Persons with this disease frequently die 1 to 2 years after diagnosis, but some may survive for longer periods. The condition is always deadly.
Possible Complications Return to top
Prior to death, there may be:
When to Contact a Medical Professional Return to top
Call your health care provider if your child has not completed their scheduled vaccines. The measles vaccine is included in the MMR vaccine.
Prevention Return to top
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 Return to top
Maldonado YA. Rubeola virus (measles and subacute sclerosing panencephalitis). In: Long SS, Pickering LK, Prober CG, eds. Principles and Practice of Pediatric Infectious Diseases. 2nd ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2003:chap 227.Update Date: 8/24/2008 Updated 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., Inc.