Authors :
Presenting Author: Sariha Moyen, MD – University of California, Los Angeles
Haley Peters, BS – University of California, Los Angeles
Aria Terango, BS – University of California, Los Angeles
Nathan Tabibzadeh, BS – University of California, Los Angeles
Gurpreet Seehra, MD – University of California, Los Angeles
Hiroki Nariai, MD, PhD, MS – Department of Pediatrics, Division of Pediatric Neurology, David Geffen School of Medicine at the University of California, Los Angeles, California, USA
Rajsekar Rajaraman, MD – David Geffen School of Medicine; UCLA Mattel Children’s Hospital
Shaun A. Hussain, MD, MS – Division of Pediatric Neurology, Department of Pediatrics, UCLA Mattel Children's Hospital, David Geffen School of Medicine
Rationale:
Cenobamate (CBM) is an effective medication for refractory focal seizures in adults. However, there is limited data regarding its efficacy not only outside of this age group, but also in other epilepsy syndromes. In this study, we summarize our center’s experience with off-label cenobamate treatment for children with epileptic spasms.Methods:
We identified patients (< 18 years old) who were prescribed CBM through an automated search of our electronic medical record database. Information on seizure-type(s) immediately before CBM exposure was obtained through a manual review of the electronic medical record. For subjects with epileptic spasms and confirmed CBM exposure, we collected data on demographics, epilepsy syndrome classification, burden of epileptic spasms, CBM exposure (peak dose and duration of treatment), latency from Infantile Epileptic Spasms Syndrome (IESS) diagnosis to CBM initiation, and treatment-related adverse events. Results:
We identified 34 children with history of IESS, ongoing epileptic spasms, and confirmed CBM exposure. The median age at CBM initiation was 6.8 years (IQR 3.1, 14.1), and median latency from initial IESS diagnosis to starting CBM was 5.5 years (IQR 2.8, 11.7). Median peak dosage was 137.5 mg/day (IQR 100.0, 168.8) or 5.6 mg/kg/day (IQR 2.6, 8.9). At the most recent follow-up, 22 (65%) remained on cenobamate. Median duration of CBM exposure was 18.8 months (IQR 9.8, 24.1) in patients who continued CBM treatment at most recent follow-up and 5 months (IQR 3.1, 6.8) in patients who discontinued CBM treatment. Reasons for discontinuation included lack of efficacy (n = 8), poor tolerability (n = 3), and increased seizures (n = 2). Overall, 20 (57%) patients exhibited at least subjective benefit with respect to epileptic spasms burden, from the perspective of either the treating neurologist or caregiver. Epileptic spasms resolved in 7 (20%) patients. Conclusions:
These data suggests that CBM may be an effective treatment for epileptic spasms, especially if implemented in a less refractory cohort, with shorter latency from IESS diagnosis to treatment. Prospective studies with uniform video-EEG quantification of epileptic spasms burden before and after CBM exposure is an essential next step.
Funding: This study was accomplished with support from the John C. Hench Foundation