A rare, slowly progressive encephalitis caused by chronic infection with the MEASLES VIRUS. The condition occurs primarily in children and young adults, approximately 2-8 years after the initial infection. A gradual decline in intellectual abilities and behavioral alterations are followed by progressive MYOCLONUS; MUSCLE SPASTICITY; SEIZURES; DEMENTIA; autonomic dysfunction; and ATAXIA. DEATH usually occurs 1-3 years after disease onset. Pathologic features include perivascular cuffing, eosinophilic cytoplasmic inclusions, neurophagia, and fibrous gliosis. It is caused by the SSPE virus, which is a defective variant of MEASLES VIRUS. (From Adams et al., Principles of Neurology, 6th ed, pp767-8)

DUI

D013344 MeSH Browser

CUI

M0020645

Previous indexing

Cerebral Sclerosis, Diffuse (1966-1970)

History note

71; VAN BOGAERT'S LEUKOENCEPHALITIS was see under SUBACUTE SCLEROSING PANENCEPHALITIS 1971-78; was see under CEREBRAL SCLEROSIS, DIFFUSE 1963-70

Online note

use SUBACUTE SCLEROSING PANENCEPHALITIS to search VAN BOGAERT'S LEUKOENCEPHALITIS 1971-78; use CEREBRAL SCLEROSIS, DIFFUSE 1966-70

Public note

71; VAN BOGAERT'S LEUKOENCEPHALITIS was see under SUBACUTE SCLEROSING PANENCEPHALITIS 1971-78; was see under CEREBRAL SCLEROSIS, DIFFUSE 1963-70