Language and behavioral outcomes of treatment with pulse-dose prednisone for electrical status epilepticus in sleep (ESES)

Few studies have examined treatment response in electrical status epilepticus in sleep (ESES), and fewer still have evaluated the effect of corticosteroid treatment employing a pulse-dose regimen. The aim of this study was to examine the effectiveness of pulse-dose prednisone in treating language and behavioral disturbances that often accompany ESES. The sample included 17 patients age 5 to 10 years at time of baseline electroencephalogram (EEG) and neuropsychological assessments. For all patients, focal, multifocal, or generalized spike and wave activity occupied greater than 50% of the nonrapid eye movement (REM) sleep record. Patients were seen for follow-up EEG recording and neuropsychological testing with an average of 10 months following initiation of pulse-dose prednisone. Improvement in language or behavior was examined in relation to resolution of ESES on EEG, age at seizure onset and treatment, duration of ESES, duration of treatment, lesional versus nonlesional epilepsy, history of language or behavioral regression, seizure control at follow-up, and intelligence quotient (IQ). With the exception of a greater likelihood of patients with low IQ to demonstrate improvement in language or behavior, improvement was seen in most patients, irrespective of ESES or other factors.