Response to Oral Corticosteroid in Infantile Epileptic Spasms Syndrome: A Multicenter Retrospective Cohort Study
Abstract number :
1.293
Submission category :
7. Anti-seizure Medications / 7C. Cohort Studies
Year :
2023
Submission ID :
274
Source :
www.aesnet.org
Presentation date :
12/2/2023 12:00:00 AM
Published date :
Authors :
Presenting Author: Minhye Kim, MD – Seoul National University Childrens Hospital
Hye Jin Kim, MD – Seoul National University Children's Hospital; Seoyun Jang, MD – Seoul National University Children's Hospital; Soo Yeon Kim, MD,PhD – Seoul National University Children's Hospital; Byung Chan Lim, MD,PhD – Seoul National University Children's Hospital; Jong Hee Chae, MD,PhD – Seoul National University Children's Hospital; Ki Joong Kim, MD,PhD – Seoul National University Children's Hospital; Hyewon Woo, MD – Chungbuk National University Hospital; Jon Soo Kim, MD,PhD – Chungbuk National University Hospital; Ji Yeon Han, MD – Seoul National University Bundang Hospital; Hunmin Kim, MD,PhD – Seoul National University Bundang Hospital; Min-jee Kim, MD – Asan Medical Center Children's Hospital; Mi-Sun Yum, MD,PhD – Asan Medical Center Children's Hospital; Jiwon Lee, MD,PhD – Sungkyunkwan Uinversity, School of Medicine, Samsung Medical Center; Jeehun Lee, MD,PhD – Sungkyunkwan Uinversity, School of Medicine, Samsung Medical Center; Sun Ah Choi, MD – Ewha Womans University Mokdong Hospital; Woo Joong Kim, MD – Seoul National University Children's Hospital
Rationale: Oral corticosteroid is well established first line treatment for infantile epileptic spasms syndrome (IESS), but response rates vary among patients and there is still a lack of high-quality evidence for managing these patients. Given that oral corticosteroid come with considerable side effect profiles, timely and effective usage is essential. We aimed to elucidate the treatment response of oral corticosteroid in IESS from a single national cohort.
Methods: We conducted a retrospective analysis of 87 patients who were diagnosed with IESS at six tertiary centers in Korea between 2000 and 2020. Our study population consisted of patients who were diagnosed with IESS before 24 months of age and were followed up for at least two years or until their demise. We specifically focused on patients who received oral corticosteroids as part of their treatment. Exclusion criteria were applied to individuals with an age at onset older than two years and those with incomplete information regarding the first six weeks after treatment. The etiology of IESS cases was categorized based on the ILAE classification, encompassing structural, genetic, metabolic, infectious, immunological, and unknown factors. For outcome assessment, we defined responders as patients who achieved seizure-free status during maximum corticosteroid usage, with clinical remission defined as one month of spasm-free status.
Results: Of the 87 patients, 62 received steroids as an add-on treatment to vigabatrin, 20 received concurrent vigabatrin and steroids from the beginning, and 5 received steroids as the initial treatment. Overall, 36 out of 87 patients (41.4%) were classified as responders to steroid. 61.1% (22/36) of responders achieved clinical remission within 14 days, 86.1% (31/36) of responders achieved clinical remission within 30 days, and 100% of responders achieved remission within 60 days. Patients with structural etiology were less likely to respond to steroid treatment. (10/36 vs. 26/51, p=0.046) Concurrent use of vigabatrin and steroids was more effective than adding steroids to existing vigabatrin regimen. (13/20 vs. 22/62, p=0.036) Non-responders more likely to have developmental delay than responders. (27/36 vs. 47/51, p=0.035)
Conclusions: Our findings suggest steroid responders will respond mostly within 30 days and at the latest within 60 days. Patients with structural etiology were less likely to respond to steroid treatment, while concurrent use of vigabatrin and steroids was more effective than adding steroids to an existing vigabatrin regimen. Neither delayed introduction of oral corticosteroid or other seizures before infantile spasm significantly affected the response rate, indicating oral corticosteroid can be tried in any steroid-naïve patients.
Funding: None
Anti-seizure Medications