Abstracts

Rationale: Children with ESES often present with language delays, aphasia, and/or behavioral disturbance, even when seizures are fully controlled with AEDs. Previous studies have demonstrated improvement in language and behavior during treatment with pulse dose prednisone (PDP) in patients with Landau-Kleffner Syndrome and in some children with autism and EEG abnormalities. However, the effectiveness of PDP in children who do not have a history of distinct language or behavioral regression is less certain. This study was aimed at comparing the effectiveness of PDP in children presenting with ESES who either evidenced acute language or behavioral disturbance versus those who presented with language or behavioral disturbance of a long-standing and developmental nature.Methods: Thirteen male and 6 female patients ranging in age from 5 to 11 years at baseline assessment underwent neuropsychological testing prior to and during treatment with one or two day per week prednisone (2 mg/kg/day, divided into two doses over 24 hours). All 19 patients had EEG abnormalities characterized by frequent focal, multifocal, or generalized spike and wave activity while awake that increased in sleep. During sleep these discharges occurred in prolonged runs or continuous discharges (ESES). Patients were classified as either aphasic or developmentally language delayed based on a language sample, test results and parent reported history. Patients were also classified as having experienced recent behavioral regression versus long-standing autism or disruptive behavior disorder (other than an inattentive form of ADHD). Patients were seen for follow up neuropsychological testing three to 27 months following initial assessment. Patients' EEGs, test scores, parent-reported history, and behavioral observations during testing were compared.Results: Nine of 19 patients (47%) evidenced a distinct improvement in language or behavior during prednisone treatment. The EEG was normalized by follow up in eight patients. Patients' whose EEG remained abnormal were as likely as those whose EEG was normalized to experience improvement in language or behavior (50% vs. 45%, respectively). Similarly, those who evidenced aphasia were only slightly, but not significantly, more likely than those with developmental language problems to experience improvement (50% vs. 23%, respectively). Those with acute behavioral regression were somewhat more likely than those with developmental behavioral disturbance to experience a distinct improvement in behavior in response to prednisone (83% vs. 17%, p = .08).Conclusions: A large proportion of children with ESES experience distinct improvement in aphasia, developmental language disorders or behavior in response to pulse dose prednisone that is unrelated to effect of treatment on EEG abnormalities. Although those with acquired aphasia or behavioral regression are somewhat more likely to respond to prednisone treatment, some patients with developmental language or behavioral disorders also experienced distinct improvement in response to treatment.