Real-World Preliminary Experience With Responsive Neurostimulation in Pediatric Epilepsy: A Multicenter Retrospective Observational Study

Abstract

BACKGROUND Despite the well-documented utility of responsive neurostimulation (RNS, NeuroPace) in adult epilepsy patients, literature on the use of RNS in children is limited. OBJECTIVE To determine the real-world efficacy and safety of RNS in pediatric epilepsy patients. METHODS Patients with childhood-onset drug-resistant epilepsy treated with RNS were retrospectively identified at 5 pediatric centers. Reduction of disabling seizures and complications were evaluated for children (<18 yr) and young adults (>18 yr) and compared with prior literature pertaining to adult patients. RESULTS Of 35 patients identified, 17 were <18 yr at the time of RNS implantation, including a 3-yr-old patient. Four patients (11%) had concurrent resection. Three complications, requiring additional surgical interventions, were noted in young adults (2 infections [6%] and 1 lead fracture [3%]). No complications were noted in children. Among the 32 patients with continued therapy, 2 (6%) achieved seizure freedom, 4 (13%) achieved ≥90% seizure reduction, 13 (41%) had ≥50% reduction, 8 (25%) had <50% reduction, and 5 (16%) experienced no improvement. The average follow-up duration was 1.7 yr (median 1.8 yr, range 0.3-4.8 yr). There was no statistically significant difference for seizure reduction and complications between children and young adults in our cohort or between our cohort and the adult literature. CONCLUSION These preliminary data suggest that RNS is well tolerated and an effective off-label surgical treatment of drug-resistant epilepsy in carefully selected pediatric patients as young as 3 yr of age. Data regarding long-term efficacy and safety in children will be critical to optimize patient selection.

Responsive Neurostimulation Targeting the Anterior, Centromedian and Pulvinar Thalamic Nuclei and the Detection of Electrographic Seizures in Pediatric and Young Adult Patients

“…Importantly, RNS System electrographic seizure detection was observed in all 11 of these patients. One of the largest retrospective studies of outcomes in pediatric and young adult patients with drug-resistant epilepsy excluded patients with active bilateral thalamic leads, although patients with a single thalamic lead were included so long as the second lead targeted cortical structures ( Nagahama et al, 2021 ). The focus of that study on FDA-approved indications for RNS therapy, which requires patients to have ≤ 2 seizure foci, likely accounts for their exclusion of patients with bilateral thalamic leads.…”

Seizure freedom after laser amygdalohippocampotomy guided by bilateral responsive neurostimulation in pediatric epilepsy: illustrative case

“…Theoretically, a child’s skull grows to near its adult volume by 7 years of age, with the youngest reported child undergoing RNS implantation to date being 3 years of age. 6 Nevertheless, many families prefer laser ablation over resection for children given its minimally invasive nature, potentially reduced overall risk, and hope for seizure freedom.…”

“…Increasingly, however, case series are emerging to support the feasibility, safety, and comparable efficacy of RNS in children for achieving seizure reduction. [3][4][5][6][8][9][10] Complications related to RNS in children have included infection and lead fracture, with no serious adverse events yet reported (e.g., hemorrhages, strokes, or device malfunctions). Theoretically, a child's skull grows to near its adult volume by 7 years of age, with the youngest reported child undergoing RNS implantation to date being 3 years of age.…”

“…In these patients, including children, responsive neurostimulation (RNS) can be used as a palliative treatment to achieve seizure reduction, although seizure freedom from RNS is not as common. [3][4][5][6][7][8][9][10] Accurate seizure localization is tantamount to selecting an effective treatment. Despite the current arsenal of noninvasive and invasive epilepsy monitoring modalities available, some patients' typical seizures may remain difficult to localize or can prove to be different in the real world than in an atypical, inpatient hospital setting with unusual daily rhythms and under the influence of ASM withdrawal.…”

Responsive neurostimulation in pediatric patients with drug-resistant epilepsy

“…The researchers found that there were no statistically significant differences in the rates of seizure reduction or complications between children and young adults in this cohort or between the cohort as a whole and the adult literature on RNS. 12 The limited evidence in the pediatric population highlights the need for further assessment of RNS as a treatment modality for children with DRE. In this study we sought to assess the potential role of RNS for the treatment of children with DRE in whom other more conventional treatments have failed, and to determine the potential diagnostic criteria for optimal patient selection and system configuration.…”