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Role of pulse methylprednisolone in epileptic encephalopathy: A retrospective observational analysis
Aparajita Chatterjee1, Ravindranadh Chowdary Mundlamuri1, Raghavendra Kenchaiah1
1Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, 560011, India.
Insights
Monthly pulses of intravenous methylprednisolone (IVMP) effectively reduced seizures in children with epileptic encephalopathy (EE). Over 50% seizure reduction was observed in most patients, with many achieving long-term seizure freedom, indicating IVMP
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Pharmacology
Background:
- Epileptic encephalopathy (EE) presents significant challenges in pediatric neurological care.
- Intravenous methylprednisolone (IVMP) pulse therapy is explored as an adjunctive treatment for refractory epilepsy syndromes.
- Assessing the efficacy and safety of IVMP in diverse pediatric EE populations is crucial.
Purpose of the Study:
- To evaluate the impact of monthly IVMP pulses on seizure reduction and global outcomes in children diagnosed with EE.
- To identify specific EE subtypes and EEG patterns that predict response or non-response to IVMP therapy.
- To determine the safety profile and optimal duration of IVMP treatment in this pediatric cohort.
Main Methods:
- Retrospective analysis of 97 children with EE treated with IVMP for at least 3 months in a tertiary care center.
- Inclusion criteria required pre- and post-steroid EEG recordings and outcome assessment at 3 months and beyond.
- Data collected included seizure types, etiology, concurrent anti-seizure medications, IVMP duration, adverse events, and seizure burden reduction.
Main Results:
- A significant seizure burden reduction (>50%) was achieved in 66% of patients at 3 months, with 25% achieving seizure freedom.
- Long-term seizure freedom (46%) was observed with continued steroid therapy beyond 3 months.
- Idiopathic EEs, West syndrome, normal neuroimaging, and myoclonic jerks predicted a better response, while burst-suppression and GPFA predicted a poorer response.
Conclusions:
- Adjunct monthly IVMP pulses are safe and effective for reducing seizures and improving outcomes in select pediatric EE populations, including idiopathic EE, West syndrome, and those with myoclonic jerks.
- A subset of patients may experience delayed seizure freedom, suggesting that extended IVMP therapy beyond 3 months may be beneficial.
- EEG findings like burst-suppression and GPFA indicate a need for alternative or additional treatment strategies.
Objectives:
To study the effect of monthly pulses of intravenous methylprednisolone (IVMP) on seizure and global outcomes in children with epileptic encephalopathy (EE).
Methods:
This retrospective study was undertaken in a tertiary care epilepsy center in India. Consecutive patients with EE who had received IVMP as adjunctive therapy for a minimum of 3 months and had at least one pre-and post-steroid EEG each, were identified and a structured questionnaire was used to collect information including outcomes at 3 months post-steroid course completion and beyond, as available.
Results:
Ninety-seven patients (M:F=71:26) fulfilling the inclusion criteria with a mean age at onset of seizures being 20.52 ± 25.69 months were included. Commonest seizure types were myoclonic (66%); Lennaux-Gastaut and West Syndromes accounted for 57 % and 24 % patients respectively. The etiology was unknown in 52 %. All children were on a combination of standard anti-seizure drugs. The duration of IVMP pulse therapy was 7.72 ± 6.25 months. One-fourth (26 %) patients experienced minor adverse events. Greater than 50 % seizure burden reduction was seen in 66 % patients at 3 months with seizure-freedom in 25 %. A total of 45 (46 %) patients became seizure-free in the cohort eventually with continuation of steroids beyond 3 months. Children with idiopathic EEs, normal neuroimaging, myoclonic jerks, and West syndrome showed the best response. The presence of burst-suppression and generalized paroxysmal fast activity (GPFA) predicted inadequate response.
Conclusions:
Adjunct pulse doses of IVMP are safe, well-tolerated, and effective in reducing seizures and improving global outcomes in children with idiopathic EEs, West syndrome, normal neuroimaging, and myoclonic jerks. Seizure freedom might be delayed in a subset of these patients, hence duration of therapy beyond 3 months may be warranted.
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