It is suggested that repeat sEEGs may help guide effective treatment in children with multifocal epilepsy, while multifocal networks may necessitate additional sEEG evaluations and subsequent surgeries, favorable seizure outcomes can be achieved.
Abstract
Abstract Objective Tuberous sclerosis complex (TSC) is a rare genetic disorder associated with early‐onset drug‐resistant epilepsy (DRE) secondary to intracranial tubers. Many patients undergo stereo‐EEG (sEEG) for seizure onset localization, sometimes requiring multiple sEEGs and subsequent epilepsy surgeries. Our objective was to evaluate the effects of single versus multiple sEEGs on seizure outcomes in pediatric patients with TSC‐associated DRE. Methods Retrospective review of 45 pediatric TSC patients who underwent epilepsy surgery at our institution (2/2013–4/2026). Collected data included demographics, seizure history, number of sEEGs, surgical interventions, and outcomes. Primary outcomes were Engel scores after definitive epilepsy surgery. Results Among 34 identified TSC patients who underwent sEEG prior to surgery, 19 had one sEEG, 15 had two or more (mean ± SE: 2.4 ± 0.2, range: 2–5). 68% of single sEEG patients had one presumed seizure focus on phase I vEEG versus only 27% of multiple sEEG patients (p = 0.03). Phase I vEEG and MEG data were concordant in 67% of single sEEG patients and 91% of multiple sEEG patients. Single ictal focus epilepsy on vEEG was significantly associated with use of only single sEEG on logistic regression (p = 0.01). Patients with a single sEEG more often underwent laser ablation (53% vs. 27%), while those with multiple sEEGs more commonly underwent resective surgery (73% vs. 47%, p = 0.171). Four patients (20%) in the single sEEG group had additional treatment surgeries without repeat sEEG. At one‐year and most recent (2.3 ± 0.4 vs. 2.8 ± 0.4 years) follow‐up in patients who had at least 1 year of follow up after their most recent surgery (n = 24, 82%), favorable seizure outcomes (Engel I/II) were observed in 86% and 93% in single sEEG patients, and 64% and 79% in multiple sEEG patients (p > 0.99). Significance TSC‐associated DRE is often multifocal and challenging to treat, requiring sEEG for localization. Presurgical multifocal vEEG or MEG findings may predict need for repeat sEEG. While multifocal networks may necessitate additional sEEG evaluations and subsequent surgeries, favorable seizure outcomes can be achieved. Plain Language Summary Tuberous sclerosis complex (TSC) is a rare genetic condition that often causes difficult‐to‐control epilepsy in children. Some patients need a procedure called stereo‐EEG (sEEG) to locate where seizures begin in the brain before surgery. We reviewed 34 children with TSC who underwent sEEG before epilepsy surgery. Children with a single seizure focus were more likely to need only one sEEG, while those with multiple seizure areas often required repeat sEEGs and additional surgeries. Despite more complex epilepsy, many patients still achieved good seizure control after treatment. These findings suggest that repeat sEEGs may help guide effective treatment in children with multifocal epilepsy.
OBJECTIVE
Epilepsy surgery in tuberous sclerosis complex (TSC) is underutilized but associated with improved seizure control and better neurocognitive outcomes. Here we focus on utilization of repeated surgeries to improve seizure control.
METHODS
We performed a retrospective chart review of 62 pediatric patients with TSC, who underwent a total of 120 epilepsy surgeries at Boston Children's Hospital and Stanford University from 2011 to 2025. We collected patient seizure control over time, following single or multiple surgeries, details of the surgery performed, the location(s) and timing of post-operative seizure recurrence.
RESULTS
Twenty-nine patients (47%) had a single surgery and 33 (53%) underwent repeat epilepsy surgeries. There were no significant differences in surgical approaches (laser vs open) or demographic or clinical factors between patients with single vs repeated surgeries. Patients with one or two surgeries had similar rates of seizure freedom from the targeted epileptic foci: 76% single and 66% two or more surgeries. Overall seizure freedom from all foci was 69% for a single surgery and 55% for two or more surgeries. Seizure freedom rates for the targeted foci decreased following the third through fifth surgeries, but some patients still achieved overall seizure freedom: 33% following the third and 40% the fourth surgery. Seizure recurrences were primarily from prior targeted foci (57%), but 29% were from new foci that became clinically apparent after the first surgery. The presence of interictal epileptiform activity prior to surgery had modest sensitivity 75% (25/33) for prediction of an eventual seizure focus. Timing of surgery had some impact on outcome, with earlier surgery associated with increased seizure freedom.
SIGNIFICANCE
Repeat surgeries whether near a prior surgical site or addressing a novel seizure focus have the potential to render most children with TSC seizure-free. Earlier surgery had some evidence for improving outcomes and timing of surgery should be studied in greater detail.
Alexandria Valdrighi, Kevin Pearsson, Jurriaan M. Peters et al.· Epilepsia· 0 citations
Childhood drug-resistant epilepsy was the strongest predictor of persistent adult seizures and disability and TSC2 mutations were linked to a more severe phenotype but does not predict the course of epilepsy in adulthood, supporting early, optimized seizure management.
Q. Calonge, A. Hanin, Maëlle Dade et al.· Journal of Neurology· 0 citations
OBJECTIVE
Children with generalized or multifocal drug-resistant epilepsy (DRE) without a clearly localizable and safely resectable seizure focus present a significant clinical challenge, as traditional resective interventions are often not viable. Emerging evidence supports the use of neuromodulation-particularly deep brain stimulation (DBS) and responsive neurostimulation (RNS)-for generalized and multifocal epilepsy in adults. However, safety and efficacy data for thalamic neuromodulation in pediatric populations remain limited. This single-institution case series describes, to our knowledge, the largest pediatric cohort treated with thalamic DBS or RNS for generalized or multifocal DRE, providing descriptive data on safety and seizure burden.
METHODS
The authors performed a retrospective chart review of pediatric patients with DRE who underwent thalamic neuromodulation using RNS or DBS at Seattle Children's Hospital between January 2020 and July 2025 with at least 6 months of follow-up. Clinical, surgical, and seizure outcome data were collected, including seizure frequency and complications. Seizure outcomes were stratified into categorical frequency bins, and a trend analysis was performed to evaluate postoperative shifts in seizure burden.
RESULTS
Twenty-six patients (mean age 14.5, range 6-20 years) underwent thalamic neuromodulation with DBS (n = 12) or RNS (n = 14). The centromedian nucleus was the target in 24 cases; 1 patient each underwent targeting of the anterior nucleus and pulvinar nucleus. At the last follow-up (median 30.2 months), 65.4% of patients met the responder criteria (≥ 50% seizure reduction), with a higher response in the DBS group (83.3%) compared with the RNS group (50.0%). The median seizure reduction was 75.7% for DBS and 37.5% for RNS. A significant downward shift in seizure frequency was observed postoperatively across the entire cohort (p = 0.031), including among patients with the highest baseline seizure burden. No intraoperative complications occurred. Two patients (7.7%) required device explantation, 1 due to infection and 1 due to behavioral side effects, and 1 patient discontinued therapy without explantation. No instances of sudden unexpected death in epilepsy or hardware malfunction were observed.
CONCLUSIONS
Thalamic neuromodulation using DBS and RNS was safe and well tolerated in pediatric patients with DRE, including multifocal and generalized seizure onsets. Meaningful seizure reduction was observed across a range of epilepsy phenotypes, with a trend toward greater benefit in the DBS group. These findings support thalamic neuromodulation as a promising treatment option in children with nonlesional DRE and highlight the need for prospective multicenter studies with extended follow-up.
Dominic Nistal, Benjamin D. Edmonds, Adriel Barrios-Anderson et al.· Journal of Neurosurgery: Ped...· 0 citations
This review examines the evolving landscape of pediatric OLE, highlighting the shift from syndromic to etiological classification and management strategies and the need to monitor cognitive comorbidities and syndrome evolution.
Preeti Srivastava, D. Nag, Shikha Swaroop et al.· World Journal of Clinical Pe...· 0 citations
Complete resection of the epileptogenic zone offers the best chance of seizure control in patients with drug-resistant epilepsy. However, surgical management remains challenging when the epileptogenic zone is located within or adjacent to eloquent brain regions. Awake craniotomy (AC), combined with intraoperative functional mapping, has the potential to maximize resection while preserving neurological function. Despite its increasing use, evidence regarding its safety and efficacy in epilepsy surgery remains limited. This study aimed to systematically evaluate the feasibility, reported safety, and reported seizure outcomes of awake craniotomy in patients undergoing surgery for drug-resistant non-oncological epilepsy.
A systematic literature search was conducted in PubMed, Scopus, and Web of Science databases in accordance with PRISMA guidelines.
Seven retrospective studies comprising 231 patients from six countries were included; 120 patients underwent AC. Four studies (57.1%) included mixed adult and pediatric populations and three (42.9%) included adults only; however, none provided age-stratified seizure or complication outcomes, precluding separate pediatric analysis. Reported seizure duration ranged from 2 to 39 years. The frontal lobe was the most frequently involved region (
n
= 52; 43.3%), followed by the temporal lobe (
n
= 27; 22.5%), and cortical dysplasia was the most common underlying pathology (
n
= 30; 25.0%). Language and sensorimotor functions were the most commonly mapped eloquent areas, each reported in five studies (71.4%). Seizure outcomes were predominantly assessed using the Engel classification. Complete seizure freedom (Engel class I) was reported in 69 of 120 AC patients (57.5%); a random-effects single-arm synthesis estimated an Engel I proportion of 59.3% (95% CI: 46.3–71.2%; I2 = 31.9%). Comparisons with surgery under general anesthesia (GA) were available in only two non-randomized studies and should be interpreted cautiously. No study reported conversion from AC to GA. Postoperative neurological deficits were largely transient, with a low reported incidence of permanent morbidity. Owing to substantial differences in patient selection, surgical indications, and study design, these findings should be considered exploratory and should not be interpreted as demonstrating comparable seizure outcomes or treatment effectiveness.
In carefully selected patients with drug-resistant non-tumor epilepsy involving or adjacent to eloquent cortex, AC with intraoperative functional mapping appears feasible and may help preserve neurological function while enabling tailored resection. The current evidence base remains limited by retrospective designs, heterogeneous populations and techniques, inconsistent outcome reporting, and sparse comparative data; therefore, efficacy and equivalence with surgery under general anesthesia should not be inferred.
M. Mofatteh, M. Mashayekhi, Sho Giersztein et al.· BMC Surgery· 0 citations
OBJECTIVE
Use of neuromodulation strategies targeting thalamic nuclei, including deep brain stimulation (DBS) and responsive neurostimulation (RNS), for treatment of pediatric drug-resistant epilepsy (DRE) is increasing, despite limited evidence for efficacy and safety. We present the initial results from the Comparative Multicenter Evaluation of Thalamic Neuromodulation for Treatment-Resistant Epilepsy in Children consortium, which was created to study thalamic modulation in children with DRE.
METHODS
We performed a retrospective cohort study of children who underwent thalamic DBS or RNS for DRE between January 2015 and December 2024 across 12 centers. Primary outcomes were percent seizure reduction, Engel class, and responder rates (>50% seizure reduction) at 3, 6, and 12 months postoperatively. Secondary outcomes included antiseizure medications and surgical complications. Differences were quantified using ordinal logistic regression, generalized estimating equations with center clustering, and mixed-effects models. Stratified analyses were performed by thalamic target (centromedian vs. anterior nucleus) and epilepsy diagnosis (Lennox-Gastaut syndrome, generalized, focal, and multifocal).
RESULTS
Among the 221 included patients (111 DBS, 110 RNS) included, DBS and RNS achieved comparable Engel scores and 12-month responder rates, but different temporal trajectories were observed. There was a significant device × time interaction (odds ratio = 1.10/month, 95% confidence interval = 1.01-1.20, p = .04), with RNS response rates increasing from 26.9% at 3 months to 55.6% at 12 months (paired p < .001) but with DBS response rates reaching 41.7% at 3 months and remaining comparatively stable over time. DBS achieved significantly higher 12-month seizure freedom (9.5% vs. 1.2%, p = .03). Neither thalamic target selection nor diagnosis predicted seizure outcome. Safety profiles were equivalent.
SIGNIFICANCE
This multicenter analysis provides pediatric-specific data to inform surgical indications, device selection, and preoperative counseling. DBS and RNS were effective for treatment of pediatric DRE, with equivalent 12-month seizure outcomes but different temporal trajectories. DBS provided earlier seizure control and higher rates of overall seizure freedom.
S. Tenhoeve, N. Warsi, Allison M Ludwick et al.· Epilepsia· 0 citations
