Responsive Neurostimulation: A New Era in Treatment of Pediatric Drug-resistant Epilepsy in Tuberous Sclerosis Complex
Abstract number :
1.174
Submission category :
3. Neurophysiology / 3E. Brain Stimulation
Year :
2022
Submission ID :
2204819
Source :
www.aesnet.org
Presentation date :
12/3/2022 12:00:00 PM
Published date :
Nov 22, 2022, 05:26 AM
Authors :
Cemal Karakas, MD – University of Louisville; Kimberly Houck, MD – Baylor College of Medicine; Maureen Handoko, MD, PhD – Baylor College of Medicine; Cristina Trandafir, MD – Baylor College of Medicine; Rohini Coorg, MD – Baylor College of Medicine; James Riviello, MD – Baylor College of Medicine; Howard Weiner, MD – Baylor College of Medicine; Daniel Curry, MD – Baylor College of Medicine; Irfan Ali, MD – Baylor College of Medicine
Rationale: Tuberous sclerosis complex (TSC) is an autosomal-dominant neurocutaneous syndrome with most patients experiencing seizures starting from infancy. Responsive Neurostimulation (RNS) is a recently developed FDA-approved tool for the treatment of drug-resistant epilepsy (DRE) in patients who are not surgical candidates due to seizure onset from eloquent cortex or multiple brain regions, which is frequently the case in TSC patients. However, limited literature is available describing the safety and efficacy of RNS in pediatric DRE, particularly in patients with TSC. We aim to review the clinical outcomes of a series of pediatric TSC patients with DRE treated with the RNS System.
Methods: We performed a retrospective chart review of all patients implanted with an RNS device between July 2016 and May 2022 and identified six patients who had TSC. Change in clinical seizure frequency was calculated based on the caregivers’ report at the last follow-up visit relative to the pre-implantation baseline seizure frequency.
Results: The average age of the RNS implantation was 13.8 years (range, 5-21) and all patients were female. The average epilepsy duration before the RNS implantation was 12 years (range, 5-20). Three patients had prior surgeries before RNS implantation, including the vagal nerve stimulation in two patients, left parietal resection in one, and corpus callosotomy in one. The average number of anti-seizure medications tried prior to RNS was around eight. The rationale for RNS included eloquent cortex in four patients and multifocal seizures in two. The average follow-up duration was 22 months (range, 17-25), and no patient experienced device-related complications. The maximum current density for each patient ranged between 1- and 3.5 micro-Coulombs per centimeter square. The average daily stimulations were 2250 (range, 400-4200). There was an average of 88.4% (range, 66%-100%) seizure reduction in five patients. One patient reported no change in frequency of seizures at the last follow-up.
Conclusions: There was an average of 88.4% improvement in seizure frequency in 83% of the pediatric patients with DRE secondary to TSC treated with the RNS System. RNS is a safe and effective treatment for DRE in children with TSC.
Funding: None
Neurophysiology