subacute sclerosing panencephalitis (Dawson disease, SSPE)
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Etiology
- may be late complication of measles
- may develop due to reactivation of the measles virus or an inappropriate immune response to the measles virus[2]
Epidemiology
- 85% < 15 years old
- 3-10 times more likely in males
- occurs years after clinical measles
Pathology
- dementia in children & occasionally in adolescents due to persistent replication of the measles virus in the brain many years after initial infection
Clinical manifestations
- good health, then insidious mental deterioration
- incoordination, seizures, myoclonus, ataxia & ultimately death
Laboratory
Diagnostic procedures
- characteristic periodic EEG
Radiology
- CT & MRI show multifocal white matter lesions, atrophy & ventriculomegaly
Management
- no treatment uniformly effective
- isoprinosine controversial
More general terms
References
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 144
- ↑ 2.0 2.1 NORD: National Organization for Rare Disorders Subacute Sclerosing Panencephalitis https://rarediseases.org/rare-diseases/subacute-sclerosing-panencephalitis/
- ↑ National Institute of Neurological Disorders and Stroke (NINDS) Subacute Sclerosing Panencephalitis Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Subacute-Sclerosing-Panencephalitis-Information-Page
Patient information
subacute sclerosing panencephalitis patient information