Subacute Sclerosing Panencephalitis

**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)

**Tree numbers:** - C01.207.245.340.600 - C01.207.399.750.600 - C01.925.182.525.600 - C01.925.782.580.600.500.500.800 - C01.925.839.862 - C10.228.140.430.520.750.600 - C10.228.228.245.340.600 - C10.228.228.399.750.600 - C10.586.250.520.750.600

**Synonyms:** - Sclerosing Panencephalitis, Subacute - Panencephalitis, Subacute Sclerosing - Leukoencephalitis, Van Bogaert's - Van Bogaert's Leukoencephalitis - Sclerosing Leukoencephalitis, Subacute - Leukoencephalitis, Subacute Sclerosing - Measles Inclusion Body Encephalitis - Inclusion Body Encephalitis, Measles - Encephalitis, Inclusion Body, Measles - SSPE