Authors :
Presenting Author: Nathan Tabibzadeh, BS – University of California, Los Angeles
Haley Peters, BS – University of California, Los Angeles
Ainsley McCabe, BS – University of California, Los Angeles
Nicole Mazaheri, BS – University of California, Los Angeles
Aria Terango, BS – University of California, Los Angeles
Rajsekar Rajaraman, MD – David Geffen School of Medicine; UCLA Mattel Children’s Hospital
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
Aria Fallah, MD, MSc, MBA – Department of Neurosurgery, UCLA Medical Center, David Geffen School of Medicine
Shaun A. Hussain, MD, MS – Division of Pediatric Neurology, Department of Pediatrics, UCLA Mattel Children's Hospital, David Geffen School of Medicine
Rationale:
Infantile Epileptic Spasms Syndrome (IESS) is a form of epileptic encephalopathy that is linked to adverse long-term epilepsy and developmental outcomes. Full length corpus callosotomy (CC) has been touted as an effective surgical approach to either directly eliminate epileptic spasms, or identify patients with subtle occult malformations (e.g. focal cortical dysplasia, FCD) who are favorable candidates for surgical resection. In this retrospective cohort study, we describe the UCLA experience with CC among children with epileptic spasms.Methods:
Children with IESS who underwent CC at UCLA Mattel Children’s Hospital were identified with search of a clinical epilepsy surgery database. Clinical and demographic characteristics of the study cohort were abstracted from the electronic medical record.Results:
Results: We identified 24 children (8 female) with refractory symmetric (clinical and EEG) epileptic spasms who underwent CC at a median age of 1.9 yr (IQR 1.4 – 6.6). Median number of prior anti-seizure medications was 8 (IQR 7 – 10), and all subjects failed to respond to a trial of standard first-line therapy (prednisolone, ACTH, vigabatrin, or a combination thereof). Six (25%) patients were seizure-free at most recent follow-up. All six exhibited new lateralized abnormalities on EEG and/or PET upon CC, suggestive of previously occult subtle FCD. Patient 1 achieved seizure-freedom immediately upon CC and has maintained seizure-freedom ( >3 years). Patient 2 achieved seizure-freedom (now >1 yr) in response to cannabidiol and clobazam, prior to proposed surgical resection. Patients 3 and 4 underwent resection of FCD (ILAE 1a and 1c, respectively) and both remain seizure-free > 1yr. Patients 5 and 6 underwent resection of FCD (both ILAE IIa) and were temporarily seizure-free. After relapse with tonic seizures and epileptic spasms, both patients have since responded to cenobamate and remain seizure-free > 1yr. Two additional patients are in the midst of ongoing re-evaluation for surgical resection following CC. Except for patient 2, who has exhibited excellent developmental gains, developmental trajectory has been generally poor in this cohort. Of note, all subjects exhibited long durations of IESS prior to CC (median 1.5 yr, IQR 1.2 – 5.5).
Conclusions:
This study suggests that corpus callosotomy may aid in treatment of children with IESS, though the response rate in this series is lower than prior reports (Baba et al, 2018). Nevertheless, CC appears to be helpful in a substantial minority of patients, and CC should perhaps be considered much earlier in the course of epilepsy. Further study is clearly warranted to identify best candidates for CC in the setting of IESS.Funding:
This study was supported by the John C. Hench Foundation.