Treatment Outcome of Vigabatrin as First Line Therapy in Patients with Infantile Spasms: A Multicenter Cohort Study
Abstract number :
1.235
Submission category :
4. Clinical Epilepsy / 4D. Prognosis
Year :
2023
Submission ID :
281
Source :
www.aesnet.org
Presentation date :
12/2/2023 12:00:00 AM
Published date :
Authors :
Presenting Author: Ji Yeon Han, MD – Bundang Seoul national university hospital
Hunmin Kim, MD – Bundang Seoul national university hospital; Hye Jin Kim, MD – Seoul National University Children's Hospital; Jon Soo Kim, MD – Chungbuk National University Hospital; Minhye Kim, MD – Seoul National University Children's Hospital; Min-Jee Kim, MD – Asan Medical Center Children’s Hospital; Woo Joong Kim, MD – Seoul National University Children's Hospital; Jeehun Lee, MD – Sungkyunkwan Uinversity, School of Medicine, Samsung Medical Center; Jiwon Lee, MD – Sungkyunkwan Uinversity, School of Medicine, Samsung Medical Center; Byung Chan Lim, MD – Seoul National University Children's Hospital; Hyewon Woo, MD – Chungbuk National University Hospital; Mi-Sun Yum, MD – Asan Medical Center Children’s Hospital; Sun Ah Choi, MD – Ewha Womans University Mokdong Hospital
Rationale:
Infantile spasms (IS) is a rare but severe form of epileptic encephalopathy. Currently, vigabatrin (VGB) has been widely used as the first-line therapy for IS due to its effectiveness in stopping or reducing the frequency of spasms. The aim of this study is to evaluated the efficacy of VGB as first line treatment for patients with IS and evaluate clinical characteristics of patients who achieved remission by VGB monotherapy.
Methods:
In this retrospective study, we included patients from multiple centers who were diagnosed with infantile spasms between January 2010 and April 2022 and were treated with VGB as their first-line therapy. We analyzed the clinical features, EEG characteristics, treatment response, and developmental and seizure outcomes of patients who achieved remission with VGB monotherapy. We compared clincal features and outcome of VGB monotherapy remission group to VGB monotherapy non-remission group.
Results:
A total of 152 patients diagnosed with IS were included, and 129 of them were treated with VGB as the first-line treatment. Mean follow up duration was 6.3 years. Among these 129 patients, 41 patients (31.8%) achieved remission of spasm with VGB monotherapy. Among the 41 patients who achieved initial remission, 3 patients (7.3%) experienced a relapse and 26 patients (63.4%) developed other types of seizures during the follow-up period. Six patients (14.6%) developed Lennox-Gastaut syndrome.There was no significant difference in relapse, seizure outcome, development of LGS, other type of seizure and developmental outcome between VGB remission group and VGB non-remission group at last follow-up. Among, VGB remission group, idiopathic etiology was statstically significantly higer in patient with normal development outcome compared to patients with abnormal development outcome at last follow-up (p=0.024).
Conclusions:
VGB monotherapy achieved remission of spasms in one-third of IS patients. More than half developed other seizures after remission of spasm. Initial response to VGB seems not to affect the long term developmental and seizure outcome. In the VGB remission group, patients with normal developmental outcomes had a higher proportion of Idiopathic etiology compared to patients with abnormal developmental outcomes. These findings suggest that VGB monotherapy may not be sufficient for improving long-term outcome and additional therapies is necessary. Further studies are required to determine optimal treatment approaches for IS.
Funding: N/A
Clinical Epilepsy