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subacute sclerosing panencephalitis

 

Definition: a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by the measles virus. Characterized by a history of primary measles infection before the age of 2 years, followed by several asymptomatic years, and then gradual, progressive psychoneurologic deterioration, consisting of personality change, seizures, myoclonus, ataxia, photosensitivity, ocular abnormalities, spasticity, and coma. Characteristic periodic activity is seen on EEG; pathologically, the white matter of both the hemispheres and brainstem is affected, as well as the cerebral cortex, and eosinophilic inclusion bodies are present in the cytoplasm nuclei of neurons and glial cells. Death usually occurs within 3 years.

Synonym(s): Bosin disease, Dawson encephalitis, inclusion body encephalitis, sclerosing leukoencephalitis, subacute inclusion body encephalitis, subacute sclerosing leukoencephalitis, van Bogaert encephalitis

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.
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