Subacute Sclerosing Panencephalitis

Synonyms

Encephaltis, Inclusion Body, Measles

Inclusion Body Encephalitis, Measles

Leukoencephalitides, Subacute Sclerosing

Leukoencephalitis, Subacute Sclerosing

Leukoencephalitis, Van Bogaert

Leukoencephalitis, Van Bogaert's

Leukoencephalitis, Van Bogaerts

Measles Inclusion Body Encephalitis

Panencephalitides, Subacute Sclerosing

Panencephalitis, Subacute Sclerosing

SSPE

Sclerosing Leukoencephalitides, Subacute

Sclerosing Leukoencephalitis, Subacute

Sclerosing Panencephalitides, Subacute

Sclerosing Panencephalitis, Subacute

Subacute Sclerosing Leukoencephalitides

Subacute Sclerosing Leukoencephalitis

Subacute Sclerosing Panencephalitides

Van Bogaert Leukoencephalitis

Van Bogaert's Leukoencephalitis

Van Bogaerts Leukoencephalitis

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)