Treatment Practices for Infantile Epileptic Spasms Syndrome: Consensus and Variation in Major Pediatric Epilepsy Centers.
Publication/Presentation Date
10-30-2025
Abstract
BACKGROUND: Infantile epileptic spasms syndrome (IESS) is a developmental and epileptic encephalopathy that requires prompt, effective treatment to optimize outcomes. While the first therapies for IESS with adrenocorticotrophic hormone, prednisolone, or vigabatrin are widely established as a standard, we hypothesized that the treatment protocols of how these therapies should be implemented varied across medical centers.
METHODS: The Pediatric Epilepsy Research Consortium Infantile Spasms Special Interest Group distributed a REDCap survey to 75 US epilepsy centers. Predefined treatment pathway characteristics were extracted and compared. Standard therapy regimens were defined before data collection.
RESULTS: Thirty-six centers participated (48% completion rate). Most (89%, n = 32) had IESS treatment pathways, with 72% (n = 23) influenced by insurance barriers such as prior authorizations. Of these, 75% (n = 24) contributed pathways for analysis. Most protocols (88%, n = 21) recommended a standard treatment course for new-onset IESS. Of these, 63% (n = 15) endorsed a sequential approach to using hormonal therapy and vigabatrin, while 17% (n = 4) recommended combination therapy with both for all children. Thirteen centers (54%) provided recommendations for treating persistent epileptic spasms. Approaches to side-effect mitigation varied widely, with gastrointestinal prophylaxis and blood pressure control being the most common (79%, n = 19). Half of the pathways mentioned ketogenic diet (58%) or epilepsy surgery (46%).
CONCLUSIONS: While there was broad consensus regarding first and second therapy treatment for IESS, variability existed in using sequential versus combination therapy, third therapies, and adverse event monitoring. These findings will guide next research steps in defining key questions on sequential versus combination therapy, third line therapy, and adverse event monitoring in order to develop a standardized consensus-driven treatment protocol for IESS in the future.
Volume
174
First Page
46
Last Page
53
ISSN
1873-5150
Published In/Presented At
Briscoe, C., Katyayan, A., Harini, C., Hussain, S. A., Bhalla, S., Singh, A., Donatelli, S., Sandoval Karamian, A. G., Samanta, D., Sirsi, D., Catenaccio, E., Planchart Ferretto, M. A., Thio, L. L., Hunter, S. E., Spriha, P., Rao, C. K., Bhatia, S., Erdemir, G., Shrey, D. W., & Numis, A. L. (2025). Treatment Practices for Infantile Epileptic Spasms Syndrome: Consensus and Variation in Major Pediatric Epilepsy Centers. Pediatric neurology, 174, 46–53. Advance online publication. https://doi.org/10.1016/j.pediatrneurol.2025.10.005
Disciplines
Medicine and Health Sciences | Pediatrics
PubMedID
41172580
Department(s)
Department of Pediatrics
Document Type
Article