SSPE
Key points
- Subacute sclerosing panencephalitis (SSPE) is a progressive, debilitating, and fatal brain disorder caused by infection with a mutant measles (rubeola) virus.
- Ordinarily, the measles virus does not cause brain damage, but certain mutant forms may invade the brain, causing severe illness and death.
- There may be a history of measles in an unvaccinated child.
- However, certain anti-viral drugs can slow the progression of the disease.
Alternative names
Subacute sclerosing panencephalitis; Subacute sclerosing leukoencephalitis; Dawson's encephalitis
Definition
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
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
- Gradual onset of behavioral changes
- School problems
- Bizarre behavior
- Myoclonic jerking (quick muscle jerking or spasms)
- Seizures
- Dementia (loss of cognitive, emotional, and social abilities)
- Unsteady gait
- Coma
- Rigidity or flaccidity, with weakness in both legs
Signs and tests
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:
- Electroencephalogram (EEG), a test of the electrical activity of the brain, which may show a wave pattern which is typical of SSPE
Written and revised by
Andrey Nailor, M.D.
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