Definition: 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)
Other names Van Bogaert's Leukoencephalitis; SSPE; Panencephalitis, Subacute Sclerosing; Measles Inclusion Body Encephalitis; Leukoencephalitis, Subacute Sclerosing; Van Bogaerts Leukoencephalitis; Van Bogaert Leukoencephalitis; Subacute Sclerosing Panencephalitides; Subacute Sclerosing Leukoencephalitis; Subacute Sclerosing Leukoencephalitides; Sclerosing Panencephalitides, Subacute; Sclerosing Leukoencephalitides, Subacute; Panencephalitides, Subacute Sclerosing; Leukoencephalitis, Van Bogaerts; Leukoencephalitis, Van Bogaert; Leukoencephalitides, Subacute Sclerosing; Sclerosing Panencephalitis, Subacute; Sclerosing Leukoencephalitis, Subacute; Leukoencephalitis, Van Bogaert's; Inclusion Body Encephalitis, Measles
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Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy
