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subacute sclerosing panencephalitis (Dawson disease, SSPE)
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:
1) 85% < 15 years old
2) 3-10 times more likely in males
3) 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:
1) good health, then insidious mental deterioration
2) incoordination, seizures, myoclonus, ataxia & ultimately death
Laboratory:
1) increased measles antibody in CSF & serum
2) patients lack antibody to measles virus protein M
Special laboratory:
-> characteristic periodic EEG
Radiology:
-> CT & MRI show multifocal white matter lesions, atrophy & ventriculomegaly
Management:
1) no treatment uniformly effective
2) isoprinosine controversial
Interactions
disease interactions
General
viral encephalitis
Properties
ETIOLOGY: measles virus
Database Correlations
OMIM 260470
References
- Harrison's Principles of Internal Medicine, 13th ed.
Isselbacher et al (eds), McGraw-Hill Inc. NY,
1994, pg 144
- 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