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Abstracts

Risk Factors for Development of Lennox-Gastaut Syndrome Among Patients with Infantile Epileptic Spasms Syndrome

Abstract number : 2.112
Submission category : 4. Clinical Epilepsy / 4A. Classification and Syndromes
Year : 2023
Submission ID : 699
Source : www.aesnet.org
Presentation date : 12/3/2023 12:00:00 AM
Published date :

Authors :
Presenting Author: David Tabibzadeh, – University of California, Los Angeles

Norah Gidanian, BS – University of California, Los Angeles; Aria Terango, BS – University of California, Los Angeles; Rujuta Sathe, BS – University of California, Los Angeles; Emmi Deckard, BS – University of California, Los Angeles; Lauren Waldron, MD – University of California, Los Angeles; Rajsekar Rajaraman, MD – University of California, Los Angeles; Hiroki Nariai, MD PhD – University of California, Los Angeles; Shaun Hussain, MD – University of California, Los Angeles

Rationale: Lennox-Gastaut Syndrome (LGS) is a common epilepsy outcome among children who first present with Infantile Epileptic Spasms Syndrome (IESS), even among patients who respond to initial therapy for IESS. Prior studies have linked LGS to the following: (1) developmental delay prior to IESS, (2) known etiology, and (3) poor response to IESS treatment. Using a large cohort of children with IESS, we first set out to replicate these associations. Second, among responders to IESS treatment, we explored potential links between development of LGS and EEG characteristics at the time of IESS treatment.

Methods: This is a retrospective cohort study. Clinical data were abstracted from the medical record. LGS diagnosis was based on neurology progress notes. EEG characteristics were based on review of EEG reports rather than independent review of digital EEG tracings. Comparisons of medians and percentages were accomplished using the Wilcoxon rank-sum and Fisher exact tests, respectively. Multivariable analysis was carried out with multiple logistic regression.

Results: A total of 435 children (42% female) with IESS were identified, with median (IQR) age of onset of 6.0 (4.0 – 9.0) months. Etiology was known among 293 (67%) and development was normal as IESS onset among 190 (44%). Response to IESS treatment and relapse of epileptic spasms were observed among 250 (57%) and 97 (23%). With median (IQR) follow-up of 54 (17 – 118) months, 98 (23%) of children received a diagnosis of LGS. On a sequential basis, development of LGS was associated with age of onset > 15mo (OR 2.7; P = 0.02), and response to IESS treatment response (OR 0.61; P = 0.03). LGS was not associated with etiology or developmental status at the time of IESS onset. Among patients who responded to IESS treatment, LGS was associated with presence of other seizures at the time of response (OR 2.4; P = 0.02), IESS duration > 3 months prior to response (OR 3.4; P = 0.003), and subsequent relapse (OR 4.8; P < 0.001). Among IESS treatment responders, LGS was not associated with any post-treatment EEG features (presence and distribution of slowing; abundance and distribution of epileptiform discharges).    

Conclusions: This study confirms the impression that LGS is associated with delayed or lack of response to IESS treatment, as well as subsequent epileptic spasms relapse. Given that LGS is relatively common after successful IESS treatment and not associated with any EEG features at the time of response, this study suggests that subsequent post-response EEG surveillance may be useful to detect—and potentially abort—evolution to LGS.

Funding: This study was accomplished with support from the John C. Hench Foundation, the Elsie and Isaac Fogelman Endowment, the Mohammed F. Alibrahim Endowment, the Hughes Family Foundation, and the UCLA Children’s Discovery and Innovation Institute.

Clinical Epilepsy