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subacute sclerosing panencephalitis (DOID:8970)
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subacute sclerosing panencephalitis +   
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)
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Exact Synonyms: SSPE ;   Subacute Sclerosing Leukoencephalitis ;   Van Bogaert leukoencephalitis ;   Van Bogaert's leukoencephalitis ;   Van Bogaert's sclerosing leukoencephalitis ;   Van Bogaerts leukoencephalitis ;   immunosuppressive measles encephalitis ;   inclusion body encephalitis, measles ;   subacute sclerosing leukoencephalitides ;   subacute sclerosing leukoencephalopathy ;   subacute sclerosing panencephalitides
Primary IDs: MESH:D013344
Alternate IDs: OMIM:260470
Xrefs: GARD:7708 ;   ICD10CM:A81.1 ;   ICD9CM:046.2 ;   NCI:C85171
Definition Sources: "DO" "DO", MESH:D013344

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