Abstract
We report on a 5 year-old girl who suffered from Epstein-Barr virus-associated hemophagocytic syndrome (EB-VAHS), accompanied by non-convulsive status epileptics and transient organic mental disorder as central nervous system complications. Disseminated intravascular coagulopathy was observed in the acute phase. Based on EEG, CT and MRI studies, symptoms of the central nervous system were thought to be caused by intracranial hemorrhagic infarction due to circulatory insufficiency and microthrombosis. Chemotherapy using HLH-94 protocol was introduced and remission has been maintained for the past 1 year. Although the mental disorder was spontaneously resolved in about two and a half months, epilepsy focused on the left occipital lobe and visual dysgnosia have persisted as late sequelae.