Abstracts

Rationale: ASE is a rare entity in childhood, with most cases occurring in children with idiopathic generalized epilepsy or epileptic encephalopathy. New onset presentation of ASE (de novo ASE) has been reported in middle-aged or elderly patients without prior history of seizures. We report two such cases in developmentally normal children with no prior history of seizures. Methods: A search of our EEG database was performed to explore the frequency of this condition. A retrospective chart and serial EEG review of an 8-year-old girl, and a 12-year-old boy presenting with several hours of altered mental status and EEG evidence of ASE was performed. Results: Out of a database of 23,976 EEG’s, 2 cases of de novo ASE were identified in children and 4 in adults. Both children were admitted to the PICU, and had a negative work up with CT, MRI, and LP. EEG upon presentation revealed continuous generalized epileptiform discharges consistent with ASE. After resolution of status epilepticus with intravenous benzodiazepines and fosphenytoin, both children were transitioned to sodium valproate with excellent long-term response. At 2-month and 15-month follow-up both children remained seizure free on valproate. Both children continued to perform normally in school without apparent sequelae. Conclusions: Although rare, ASE may present de novo in children and should be considered in the differential diagnosis of childhood acute mental status change. Urgent EEG will confirm this diagnosis and allow appropriate therapy.