Fulminant subacute sclerosing panencephalitis: Clinical and neuropathological observations

Abstract

We describe a 3-year-old boy with subacute sclerosing panencephalitis (SSPE) who died 4 months after its onset. His initial symptoms were drowsiness and left hemiplegia. He became comatose in 10 days, and developed a decortical posture after 45 days. He suffered from multiple cerebral hemorrhage and infarction 3 months later. Oligodendrocytes were positively stained by immunocytochemical stain with a complement-fixing measles antibody. Light microscopy revealed glial nodules, perivascular cuffing and reactive gliosis. Small arteries showed intimal thickening with resultant occlusion and occasional recanalization. These findings suggested vascular involvement in SSPE. This case illustrates the difference between the fulminant and chronic forms of SSPE.