Aug 2026· Brain & development (Tokyo. 1979)· Vol 48 5, pp.
104587
· 0 citations· 32 references
Medicine
TL;DR
High-dose oral prednisolone and ACTH therapies demonstrated comparable efficacy in reducing electrographic and clinical seizure burden in children with IESS and improvement in epileptiform activity, as reflected by reductions in the BASED score is strongly associated with better clinical outcomes.
Abstract
Objective
This study aimed to compare the efficacy of adrenocorticotropic hormone (ACTH) and oral prednisolone therapies in children with infantile epileptic spasms syndrome (IESS), utilizing the Burden of Amplitudes and Epileptiform Discharges (BASED) score to assess electrographic severity and the Early Childhood Epilepsy Severity Scale (E-Chess) to evaluate clinical outcomes.
Methods
This retrospective cross-sectional study included 40 children aged 1 to 16 months with IESS. Children received either ACTH (n = 23) or high-dose oral prednisolone (n = 17). Electroencephalographic severity was assessed before treatment and on day 28 post-treatment using the BASED score. Clinical epilepsy severity was evaluated at one year post-treatment using the E-Chess score.
Results
Both treatment groups showed significant reductions in BASED scores (overall cohort p < 0.001; ACTH group p < 0.001; prednisolone group p = 0.007). However, there was no statistically significant difference between the ACTH and prednisolone groups regarding post-treatment BASED or E-Chess scores. While clinical factors such as gender, age, and etiology were not associated with favorable outcomes, a lower post-treatment BASED score was significantly associated with a better clinical prognosis (p = 0.007). Notably, a higher number of pretreatment anti-seizure medications (ASMs) was a strong predictor of poor clinical outcome (OR: 5.474, 95% CI: 1.849-16.207; p = 0.002).
Conclusion
High-dose oral prednisolone and ACTH therapies demonstrated comparable efficacy in reducing electrographic and clinical seizure burden in children with IESS. Improvement in epileptiform activity, as reflected by reductions in the BASED score, is strongly associated with better clinical outcomes. Additionally, a high pretreatment medication burden may serve as an early indicator of a refractory clinical course.
It is suggested that ACTH may be associated with sustained electroclinical improvement in selected genetically defined DEEs, and the identified network-level interactions between ion channel-related genes and intracellular signaling pathways provide a potential molecular framework for understanding variability in treat...
Çisem Duman Kayar, Sinan Akbaş, Fulya Kürekçi et al.· Epileptic disorders· 0 citations
BACKGROUND
Convulsive status epilepticus (CSE) is a time-sensitive neurological emergency in which delayed treatment may contribute to pharmacoresistance and poor outcomes. Evidence comparing third-line antiseizure medications in children remains limited. Objective To compare intravenous lacosamide and phenytoin in ped...
Michael Nabil Halim, Rasha Hussein Aly Hussein, O. El-Rashidy et al.· Epilepsy & Behavior· 0 citations
Abstract Objective Cannabidiol (CBD) has demonstrated promising effectiveness and tolerability as adjunctive treatment in patients with severe childhood epilepsies. This study investigated the effectiveness and tolerability of CBD in adults with a history of Dravet syndrome (DS), Lennox–Gastaut syndrome (LGS), or tuber...
Sara Sánchez-Gamino, María Isabel Chamorro Muñoz, P. Serrano-Castro et al.· Epilepsia Open· 0 citations
Abstract Objective To evaluate long‐term developmental outcomes and identify independent predictors of favorable developmental outcomes at 3 years of age in children with infantile epileptic spasms syndrome (IESS) treated with a standardized stepwise vigabatrin and high‐dose prednisolone protocol. Methods This prospect...
Soyoung Jang, Hui-Jin Shin, Se Hee Kim et al.· Epilepsia Open· 0 citations
Abstract Objective Vigabatrin is an effective treatment for infantile epileptic spasms syndrome (IESS), but relapse remains a clinical challenge. The ideal dose and duration of treatment after response are unknown. We set out to identify treatment‐related predictors of IESS relapse after initial vigabatrin response. Me...
Yaretson I Carmenate, Hayoung E Ahn, Haley Peters et al.· Epilepsia Open· 0 citations
OBJECTIVE
This study was undertaken to assess cenobamate (CNB) effectiveness, tolerability, and dosing in pediatric developmental and epileptic encephalopathies (DEEs), testing prespecified hypotheses on response by syndrome, etiology, electroencephalographic pattern, seizure type, CNB dose (mg/kg/day), and concomitant...
Á. Aledo-Serrano, Adrián Valls-Carbó, E. González-Alguacil et al.· Epilepsia· 0 citations
We use cookies to run the site and, with your consent, for analytics and to show ads.
See our Cookie Policy.