SSPE; Subacute sclerosing leukoencephalitis; Dawson's encephalitis
Subacute sclerosing panencephalitis (SSPE) is a progressive, debilitating, and deadly brain disorder related to
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 response 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. This dramatic decrease 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. The disease generally occurs in children and adolescents.
There may be a history of measles in an unvaccinated child. A physical examination may reveal:
The following tests may be performed:
No cure for SSPE exists. However, certain antiviral drugs may slow the progression of the disease.
People with this disease frequently die 1 to 2 years after diagnosis, but some may survive for longer periods. The condition is always deadly.
Prior to death, there may be:
Call your health care provider if your child has not completed their scheduled vaccines. The measles vaccine is included in the MMR vaccine.
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.
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.
Ropper AH, Samuels MA. Viral infections of the nervous system, chronic meningitis, and prion diseases. In: Ropper AH, Samuels MA, eds. Adams & Victor's Principles of Neurology. 10th ed. New York, NY: McGraw Hill; 2014:chap 33.
Last reviewed on: 9/3/2014
Reviewed by: Joseph V. Campellone, MD, Department of Neurology, Cooper University Hospital, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.