Comparison of Initial Treatment Regimens for Epileptic Spasms in Children with Trisomy 21
Abstract number :
2.44
Submission category :
4. Clinical Epilepsy / 4C. Clinical Treatments
Year :
2022
Submission ID :
2232939
Source :
www.aesnet.org
Presentation date :
12/4/2022 12:00:00 PM
Published date :
Nov 22, 2022, 05:28 AM
Authors :
Henry Chen, BA – University of California San Francisco; Renée Shellhaas, MD MS – University of Michigan; Sucheta Joshi, MD MS – University of Michigan; Zachary Grinspan, MD MS – Weill Cornell Medical College; Dallas Armstrong, MD – University of Texas Southwestern Medical Center; John Mytinger, MD – The Ohio State University; Debopam Samanta, MD – University of Arkansas for Medical Sciences; Rani Singh, MD – Atrium Health-Levine Children's Hospital; Shaun Hussain, MD – University of California Los Angeles; Danielle Takacs, MD – Baylor College of Medicine; Kelly Knupp, MD – University of Colorado, Anschutz Medical Campus; Adam Numis, MD – University of California San Francisco
This is a Late Breaking abstract
Rationale: Epileptic spasms (ES) are the most frequent seizure type in children with Trisomy 21, with a prevalence of 1.8%. First-line treatments for ES include corticotropin (ACTH), oral corticosteroids, and vigabatrin. In children with Trisomy 21 and ES, treatment with ACTH or oral corticosteroids may yield higher response rates compared to vigabatrin. However, prior studies are largely single-center investigations with potential for selection bias. Using the multi-center National Infantile Spasms Consortium (NISC) database, we present the efficacy of treatments for ES in children with Trisomy 21.
Methods: We used a nested cohort study design within the NISC dataset. NISC was a prospective study designed to evaluate the efficacy of treatments in children with infantile epileptic spasms syndrome from 2012 to 2018. Here, we restricted analyses to children with a genetically confirmed diagnosis of Trisomy 21. We evaluated the efficacy of first-line treatments for ES at 2-weeks and 3-months. Continuous variables were analyzed with a student t-test and one-way ANOVA; Categorical variables were analyzed using Chi-square/Fisher’s exact test and Kruskal-Wallis test. Models were adjusted for lag time from diagnosis of ES to treatment initiation using logistic regression.
Results: Thirty-four children of 644 (5.3%) with ES were diagnosed with Trisomy 21. Given the small sample size, we highlight large effects even if they are not statistically significant. Twenty (59%) were initially treated with ACTH, nine (26%) with oral corticosteroids, and five (15%) with vigabatrin. Baseline demographics did not vary between treatment groups. All children had ES as their presenting seizure type. The overall response/remission rate at 2-weeks was 53% with a trend towards improved efficacy with ACTH (65%) compared to oral corticosteroids (33%) and vigabatrin (40%; p=0.09; Figure 1A). The overall response rate at 3-months ( >90% reduction in ES) was 47% with a trend towards improved efficacy in children initially treated with ACTH (60%) compared to oral corticosteroids (33%) and vigabatrin (20%; p=0.07; Figure 1B). Among 30 of 34 (88%) participants presenting with hypsarrhythmia, the highest rates of EEG improvement at 3 months were in children treated with ACTH (74%) or oral corticosteroids (83%) compared to vigabatrin (20%; p=0.03; Figure 1C). Adjustment for lag time from diagnosis of ES to treatment initiation did not alter results.
Conclusions: In children with Trisomy 21, ES may be the initial manifestation of epilepsy, underscoring the need for early counseling after birth. Initial treatment with ACTH may result in an improved response after 3-months of follow-up and improved resolution of hypsarrhythmia at 3 months compared to vigabatrin, similar to the general population with ES.
Funding: This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Clinical Epilepsy