Efficacy and Safety of Pulse Intravenous Methylprednisolone in Pediatric Epileptic Encephalopathies: Timing and
Angelo Russo1, Serena Mazzone1, Laura Landolina1,2
1IRCCS, Istituto delle Scienze Neurologiche di Bologna, UOC Neuropsichiatria Dell'età Pediatrica, 40139 Bologna, Italy.
Insights
Intravenous methylprednisolone (IVMP) pulse therapy effectively reduced seizures in pediatric patients with epileptic encephalopathies (EE), showing high tolerability and improved quality of life. Genetic epilepsy and ESES predicted better outcomes, suggesting earlier IVMP consideration.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Pharmacology
Background:
- Epileptic encephalopathies (EE) present with severe, drug-resistant seizures, early onset, and poor developmental trajectories.
- Intravenous methylprednisolone (IVMP) pulse therapy is explored for its potential in managing pediatric EE.
- Evaluating the efficacy and tolerability of IVMP is crucial for treatment optimization in this population.
Purpose of the Study:
- To assess the efficacy and tolerability of intravenous methylprednisolone (IVMP) pulse therapy in pediatric patients diagnosed with epileptic encephalopathies (EE).
- To identify predictors of favorable outcomes following IVMP treatment.
- To evaluate the impact of IVMP on patients' quality of life (QoL).
Main Methods:
- Retrospective study conducted between 2020 and 2023.
- Inclusion criteria: pediatric patients (≤18 years) receiving IVMP pulse therapy with a minimum 6-month follow-up.
- Efficacy measured by >50% seizure reduction (responder rate) at 6 and 9 months of therapy, and 6 months post-therapy; QoL assessed.
Main Results:
- A responder rate of 85.7% at 6 and 9 months, and 80.9% at 6 months post-therapy suspension was observed in 21 pediatric patients.
- Favorable outcomes were significantly predicted by etiology and epilepsy type, with genetic epilepsy and ESES showing the best results.
- All patients reported improved QoL, with no significant adverse events noted during IVMP treatment.
Conclusions:
- IVMP pulse therapy demonstrates significant efficacy and high tolerability in pediatric patients with EE.
- Genetic epilepsy and electrical status epilepticus during slow-wave sleep (ESES) are positive predictors for favorable clinical outcomes.
- Early consideration of IVMP pulse therapy is recommended for patients with EE, given the observed improvements in QoL, EEG, and motor development.
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.
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