Ernesto Gonzalez-Giraldo

Objective: Despite being safe and effective, surgery for pediatric epilepsy is underutilized. Social determinants of health (SDoH) are important to consider when examining this treatment gap. This study examined the potential systemic inequities at three different stages in the journey toward epilepsy surgery across the Pediatric Epilepsy Research Consortium (PERC). Methods: Any youth in the PERC prospective surgery database (≤18 years) was eligible and included if sociodemographic factors were also available. We examined if there were differences in disparity factors in: (1) referral to Phase I evaluation, (2) offering of surgery, and (3) completion of surgery. Results: Of 1309 patients, the majority were male (53%, p = .04), White (82.2%, p < .001), not Hispanic or Latino (84.3%, p < .001), and privately insured (56.3%, p < .001). Of the youth who were offered surgical treatment ( n = 1103, 84.3%), there were no significant differences in race/ethnicity or sex. Those with a structural lesion were more likely to be offered surgery, and historically marginalized youth were more likely to have a lesion. Of patients who completed surgery ( n = 927, 84.0%), White patients were 1.88 times (95% confidence interval [CI]: 1.14–3.04) more likely than Black patients to complete surgery ( p = .01). Significance: Youth who began surgery evaluation are not representative of the demographics of those with epilepsy in the United States, with greater representation of White, non‐Hispanic male individuals. In addition, youth who began surgery evaluation disproportionally have private insurance. At the next stage of offering surgery, there were no differences based on SDoH. However, there is evidence that underrepresented youth that were magnetic resonance negative were less likely to be referred for surgical workup and that Black youth were less likely to undergo surgery. Therefore, efforts to provide equitable opportunity for all youth with refractory epilepsy should be concentrated at the referral for surgery evaluation point of care; however, additional efforts are also needed at later stages.

Focal cortical dysplasia (FCD) is the most common cause of surgically treatable drug-resistant epilepsy (DRE) in children. Surgical outcomes are poorly defined in early-onset FCD-DRE. The purpose of this study was to evaluate clinical and presurgical characteristics relating to surgical outcomes in early-life (seizure onset <4 months old) FCD-DRE. A multicenter prospective cohort was analyzed from the Pediatric Epilepsy Research Consortium (PERC) Surgery Database to identify patients with pathologically confirmed FCD-DRE and seizure onset younger than 4 months old. Clinical characteristics, presurgical workup, and surgical intervention and outcomes data were collected and analyzed. Primary outcome was to evaluate whether earlier surgery is associated with seizure freedom; secondary outcomes evaluated clinical/presurgical predictors of seizure freedom and safety. Thirty-one patients with FCD-DRE were identified from 18 PERC centers. Median age at seizure onset was 2.4 months (interquartile range 1.2-3.6 months). Four patients had focal to bilateral tonic-clonic seizures (FBTCS); 35% (n = 11) had epileptic spasms. Median age at phase 1 referral was 2.0 years (0.7-4.0 years). Median age at surgery was 2.6 years (1.1-5.5 years). Pathology was type II, 65% (n = 20); type I, 26% (n = 8); and type III, 6% (n = 2). Seizure freedom was achieved in 65% (n = 20) with median follow-up 2.5 years (1.3-4 years). Age at seizure onset/referral/surgery, surgery type, and experiencing FBTCS were not associated with seizure-free outcome. Type IIB pathology had 89% (n = 8) seizure-free outcome. Epileptic spasms had 45% seizure-free outcome. Transient neurologic deficits occurred in 2 patients, and an expected neurologic deficit in 1 (visual field cut from occipital lobectomy). There were no deaths. This study finds high rates of seizure-free outcome in epilepsy surgery for early-onset FCD-DRE across all pathologies and procedures with minimal complication rates and no deaths. Focal cortical dysplasia type IIB is associated with very high rates of seizure-free outcome. Epileptic spasms were associated with lower seizure-free outcome. The study also fails to confirm a high rate of multilobar unilateral hypoplasia with severe epilepsy in children, a type I FCD variant that has been reported as a common etiology of early-life FCD.

Despite strong evidence supporting timely surgical evaluation, many children with drug-resistant epilepsy undergo multiple antiseizure medication (ASM) trials before surgery. Because guidelines recommend evaluation after failure of 2 appropriate ASMs, evaluation after failure of >2 ASMs serves as a clinically relevant benchmark. The aim of this study was to identify factors associated with initiation of surgical evaluation after failure of >2 ASMs and evaluate its association with seizure freedom. We performed a retrospective analysis using the Pediatric Epilepsy Research Consortium Surgery Database, including 24 US pediatric epilepsy centers. Children aged 18 years and younger who initiated epilepsy surgery evaluation between January 2018 and February 2023 were included. Timing of evaluation was defined by the number of ASM failures before first phase I evaluation (≤2 vs >2). Unadjusted analyses and multivariable logistic regression were used to identify predictors of later evaluation and assess its association with seizure freedom, adjusting for etiology, seizure type, MRI findings, and surgical procedure. Among 1,767 patients, 802 (45.4%) initiated surgical evaluation after failing ≤2 ASMs and 965 (54.6%) after failing >2 ASMs, with a median age at seizure onset of 5.96 and 4.00 years, respectively. Factors independently associated with later initiation of surgical evaluation included genetic etiology (odds ratio [OR] 1.83, 95% CI 1.28-2.60), generalized seizures (OR 2.64, 95% CI 1.58-4.40), daily seizures (OR 1.69, 95% CI 1.33-2.14), multiple seizure types (OR 1.59, 95% CI 1.39-1.82), normal MRI (OR 1.82, 95% CI 1.52-2.18), and abnormal neurologic examination (OR 2.44, 95% CI 2.01-2.96). Surgical intervention rates were similar (∼50%) between groups. Patients who initiated surgical evaluation after failure of ≤2 ASMs had significantly higher seizure freedom rates (60.8% vs 39.3%, p < 0.001). On multivariable analysis, failure of >2 ASMs before surgical evaluation was independently associated with lower odds of seizure freedom (OR 0.66, 95% CI 0.45-0.96, p = 0.028). Initiation of surgical evaluation after failure of more than 2 ASMs is associated with more complex epilepsy phenotypes and lower rates of seizure freedom. However, 80% of these patients still experienced a >50% reduction in seizures, highlighting the therapeutic benefits of timely epilepsy surgery-even when seizure freedom is unlikely-regardless of epilepsy subtype.

Rationale: Longer duration of epilepsy before surgery is a predictor of poor outcome. While referral delays of surgical candidates are well documented, factors causing delay during the presurgical evaluation remain unclear and may vary depending on institutional characteristics. By benchmarking the duration of presurgical evaluation across multiple centers and identifying patient and evaluation characteristics contributing to duration, we can ascertain best practices and address modifiable contributors to reduce delays.Methods: We queried the Pediatric Epilepsy Research Consortium Surgery Database, a prospective, observational multicenter study enrolling children 0–18 years at 27 US pediatric epilepsy centers, for all patients undergoing initial presurgical evaluation for drug‐resistant epilepsy (DRE). We included patients with completed evaluations and data on duration from initiation of presurgical evaluation to final surgical decision. We compared patient characteristics and evaluation components between those with long duration evaluations (> 75% quartile) and those with short evaluations (< 25% quartile). Akaike information criteria selection identified variables associated with longer duration. From these, we developed a logistic prediction model for evaluation duration, using a random 80/20 training/testing split of the entire cohort. The model was tested among institutions with ≥ 10 patients in the cohort to assess its accuracy in predicting long durations. Linear models for each site assessed each variable's impact on duration. Variables with < 10% of the patient population at each site were excluded. Beta values were compared to identify intra‐ and inter‐institution variability and to delineate institutions with the shortest added duration for each variable.Results: Of 2318 patients undergoing surgical evaluation, 1655 (71%) from 23 sites had complete data. Median evaluation duration was 8 weeks (interquartile range 3–22); 453 (27%) were short‐duration evaluations and 414 (25%) were long‐duration evaluations. Multiple patient and evaluation characteristics were associated with duration (Table 1). Table 6 provides the average duration each variable contributes to evaluation by site, highlighting the shortest durations compared with other groups.Conclusions: Duration of presurgical evaluation for DRE can be accurately modeled using multiple patient characteristics and testing strategies commonly employed in epilepsy surgery evaluations. This predictive model can not only estimate evaluation duration but also identify opportunities to improve systemic efficiency. Institution‐level modeling identifies specific program strengths, providing an opportunity to learn from successful processes. Subsequent research will focus on institutional process mapping to better understand systemic practices that lead to improved efficiencies, then sharing these processes across the consortium to shorten evaluation durations.

This report highlights a case of PEA arrest which mimicked a tonic seizure and was discovered using electroencephalogram recordings.

Purpose: The best possible outcomes in infantile epileptic spasms syndrome require electroclinical remission; however, determining electrographic remission is not straightforward. Although the determination of hypsarrhythmia has inadequate interrater reliability (IRR), the Burden of AmplitudeS and Epileptiform Discharges (BASED) score has shown promise for the reliable interictal assessment of infantile epileptic spasms syndrome. Our aim was to develop a BASED training program and assess the IRR among learners. We hypothesized moderate or better IRR for the final BASED score and the presence or absence of epileptic encephalopathy (+/−EE). Methods: Using a web-based application, 31 learners assessed 12 unmarked EEGs (length 1–6 hours) from children with infantile epileptic spasms syndrome. Results: For all readers, the IRR was good for the final BASED score (intraclass correlation coefficient 0.86) and +/−EE (Marginal Multirater Kappa 0.63). For all readers, the IRR was fair to good for all individual BASED score elements. Conclusions: These findings support the use of our training program to quickly learn the BASED scoring method. The BASED score may be a valuable clinical and research tool. Given that the IRR for the determination of epileptic encephalopathy is not perfect, clinical acumen remains paramount. Additional experience with the BASED scoring technique among learners and advances in collaborative EEG evaluation platforms may improve IRR.