Efficacy and Safety of Pulse Intravenous Methylprednisolone in Pediatric Epileptic Encephalopathies: Timing and Networks Consideration

Abstract

Background: Epileptic encephalopathies (EE) are characterized by severe drug-resistant seizures, early onset, and unfavorable developmental outcomes. This article discusses the use of intravenous methylprednisolone (IVMP) pulse therapy in pediatric patients with EE to evaluate its efficacy and tolerability. Methods: This is a retrospective study from 2020 to 2023. Inclusion criteria were ≤18 years at the time of IVMP pulse therapy and at least 6 months of follow-up. Efficacy and outcome, defined as seizure reduction > 50% (responder rate), were evaluated at 6 and 9 months of therapy, and 6 months after therapy suspension; quality of life (QoL) was also assessed. Variables predicting positive post-IVMP outcomes were identified using statistical analysis. Results: The study included 21 patients, with a responder rate of 85.7% at 6 and 9 months of therapy, and 80.9% at 6 months after therapy suspension. Variables significantly predicting favorable outcome were etiology (p = 0.0475) and epilepsy type (p = 0.0475), with the best outcome achieved in patients with genetic epilepsy and those with encephalopathy related to electrical status epilepticus during slow-wave sleep (ESES). All patients evidenced improvements in QoL at the last follow-up, with no relevant adverse events reported. Conclusions: Our study confirmed the efficacy and high tolerability of IVMP pulse therapy in pediatric patients with EE. Genetic epilepsy and ESES were positive predictors of a favorable clinical outcome. QOL, EEG tracing, and postural-motor development showed an improving trend as well. IVMP pulse therapy should be considered earlier in patients with EE.

Keywords: childhood epilepsy; epileptic encephalopathy; methylprednisolone; pulse therapy.

Figure 1

We decided to highlight in different colors the clinical outcome of our patients at different time point: in grey non responders (NR) and partial responders (PR), and in green good responders (GR) and seizure-free (SF) patients. (A) shows that patients who were NR in the fourth month of IVMP therapy remained NR (60%) or became PR (40%); (B) shows that patients who were SF in the fourth month remained SF until the sixth month of follow-up; (C) shows that 100% of patients who were GR at fourth month of IVMP therapy demonstrated an improving trend becoming SF at the end of follow-up, and patient who were PR showed a stable or improving trend except for 1 patient who became NR; (D) showed that the sixth month of therapy was the first time point practically showing comparable data observed at the 6-month follow-up. Legend: NR: non responders; PR: partial responders; GR: good responders; SF: seizure-free; FU: follow-up.