Subacute sclerosing panencephalitis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Subacute sclerosing panencephalitis is a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by defective measles virus (which can be a result of a mutation of the virus itself). SSPE is 'incurable' but the condition can be managed by medication if treatment is started at an early stage. Much of the work on SSPE has been completed by the NINDS.
Causes
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.
Epidemiology and Demographics
SSPE is an incredibly rare condition although there is still relatively high incidence in Asia and the Middle East. However, the number of reported cases is declining since the introduction of the measles vaccine — eradication of the measles virus prevents the SSPE mutation and therefore the progression of the disease or even the initial infection itself.
Risk Factors
SSPE tends to occur several years after an individual has measles, even though the person seems to have fully recovered from the illness. 1 in 100,000 people infected with measles are at risk.
Diagnosis
History and Symptoms
Characterized by a history of primary measles infection before the age of 2 years, followed by several asymptomatic years (6–15 on average), and then gradual, progressive psychoneurological deterioration, consisting of personality change, seizures, myoclonus, ataxia, photosensitivity, ocular abnormalities, spasticity, and coma.
Treatment
Medical Therapy
No cure for SSPE exists. However, certain antiviral drugs may slow the progression of the disease.
Primary 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.