| umls-concept:C0038522 | skos:definition | MSH: 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),CSP: rare chronic progressive encephalitis caused by the measles virus and occurring primarily in children and young adults; death usually occurs within three years; characterized by primary measles infection before the age of two years.,NCI: A rare, slowly progressive post-infectious neurological disorder affecti | lld:umls |
