Responsive Neurostimulation for the Treatment of Children With Drug-Resistant Epilepsy in Tuberous Sclerosis Complex

Pediatr Neurol. 2023 Aug:145:97-101. doi: 10.1016/j.pediatrneurol.2023.05.008. Epub 2023 May 19.

Abstract

Background: To review seizure outcomes in children with tuberous sclerosis complex (TSC) and drug-resistant epilepsy (DRE) treated with the responsive neurostimulation (RNS) System.

Methods: We retrospectively reviewed children (<21 years old) with TSC implanted with the RNS System at Texas Children's Hospital between July 2016 and May 2022.

Results: Five patients meeting the search criteria were identified (all female). The median age of the RNS implantation was 13 years (range: 5 to 20 years). The median epilepsy duration before the RNS implantation was 13 years (range: 5 to 20 years). Surgeries before RNS implantation included vagus nerve stimulator placement (n = 2), left parietal resection (n = 1), and corpus callosotomy (n = 1). The median number of antiseizure medications tried before RNS was 8 (range: 5 to 12). The rationale for the RNS System implantation included seizure onset in eloquent cortex (n = 3) and multifocal seizures (n = 2). The maximum current density for each patient ranged between 1.8 and 3.5 μC/cm2, with an average daily stimulation of 2240 (range: 400 to 4200). There was an 86% median seizure reduction (range 0% to 99%) at a median follow-up duration of 25 months (range: 17 to 25 months). No patient experienced implantation or stimulation-related complications.

Conclusions: We observed a favorable improvement in seizure frequency in pediatric patients with DRE secondary to TSC treated with the RNS System. The RNS System may be a safe and effective treatment for DRE in children with TSC.

Keywords: Epilepsy surgery; Neurostimulation; Pediatric; RNS; Tuberous sclerosis complex.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Drug Resistant Epilepsy* / surgery
  • Drug Resistant Epilepsy* / therapy
  • Epilepsy* / surgery
  • Female
  • Humans
  • Retrospective Studies
  • Seizures
  • Treatment Outcome
  • Tuberous Sclerosis* / complications
  • Tuberous Sclerosis* / therapy
  • Young Adult