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September 14, 2026Epilepsia OpenOpen Access

Long‐term developmental outcome in infantile epileptic spasms syndrome after high‐dose prednisolone and vigabatrin treatment

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Authors

SJSoyoung JangHSHui Jin ShinSKSe Hee Kim

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Overview

Prospective cohort study reveals that etiology and treatment response drive three-year developmental outcomes in infantile epileptic spasms syndrome, highlighting the need for tailored therapy.

Key Points

  • To evaluate three-year developmental outcomes and determine independent predictors of favorable development in children with infantile epileptic spasms syndrome treated with a standardized stepwise protocol of vigabatrin and high-dose prednisolone.
  • Prospective single-center cohort study of 236 children with infantile epileptic spasms syndrome managed with initial vigabatrin and add-on high-dose oral prednisolone for nonresponders between March 2016 and April 2022 at Severance Children's Hospital.
  • Developmental outcome at 3 years was categorized as favorable (mental quotient ≥ 70 and psychomotor quotient ≥ 70) or unfavorable using the Bayley Scales of Infant and Toddler Development, Second Edition, with multivariable logistic regression via Firth's penalized method.
  • Overall treatment response occurred in 175 of 236 patients (74.2%), and 49 patients (20.8%) reached a favorable developmental outcome at 3 years.
  • Multivariable analysis showed structural etiology (aOR 0.085; 95% CI 0.026–0.281; p < 0.001), genetic etiology (aOR 0.023; 95% CI 0.002–0.357; p = 0.007), and treatment unresponsiveness (aOR 0.030; 95% CI 0.002–0.523; p = 0.02) independently decreased the odds of favorable development.
  • Zero of 49 patients with a confirmed genetic etiology and zero of 61 unresponsive patients achieved a favorable developmental outcome.

Cite This Study

Jang et al. (2026) studied this question.

synapsesocial.com/papers/6aa7b3df0926e14a848b332bhttps://doi.org/10.1002/epi4.70319
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Analysis of treatment outcome variations in infantile epileptic spasms syndrome2026
  2. 2Management of Infantile Epileptic Spasms Syndrome: A survey of US pediatric hospitals2026
  3. 3Epileptic spasms relapse is associated with response latency but not conventional attributes of post‐treatment <scp>EEG</scp>2024 · 5 citations
  4. 4No single treatment modality exhibits clear superiority for Infantile Spasms Syndrome: insights from a retrospective cohort study.2024
  5. 5Infantile Epileptic Spasms Syndrome2025 · 1 citations