A multi-center comparison of surgical techniques for corpus Callosotomy in pediatric drug-resistant epilepsy.

OBJECTIVES: Corpus callosotomy (CC) is used to reduce seizures, primarily in patients with generalized drug-resistant epilepsy (DRE). The invasive nature of the procedure contributes to underutilization despite its potential superiority to other palliative procedures. The goal of this study was to use a multi-institutional epilepsy surgery database to characterize the use of CC across participating centers. METHODS: Data were acquired from the Pediatric Epilepsy Research Consortium (PERC) Surgery Database, a prospective observational study collecting data on children 0-18 years referred for surgical evaluation of DRE across 22 U.S. pediatric epilepsy centers. Patient, epilepsy, and surgical characteristics were collected across multiple CC modalities. Outcomes and complications were recorded and analyzed statistically. RESULTS: Eighty-three patients undergoing 85 CC procedures at 14 participating epilepsy centers met inclusion criteria. Mean age at seizure onset was 2.3 years (0-9.4); mean age for Phase I evaluation and surgical intervention were 9.45 (.1-20) and 10.46 (.2-20.6) years, respectively. Generalized seizure types were the most common (59%). Complete CC was performed in 88%. The majority of CC procedures (57%) were via open craniotomy, followed by laser interstitial thermal therapy (LiTT) (20%) and mini-craniotomy/endoscopic (mc/e) (22%). Mean operative times were significantly longer for LiTT, whereas mean estimated blood loss was greater in open cases. Complications occurred in 11 cases (13%) and differed significantly between surgical techniques (p < .001). There was no statistically significant difference in length of postoperative stay across approaches. Mean follow-up was 12.8 months (range 1-39). Favorable Engel outcomes were experienced by 37 (78.7%) of the patients who underwent craniotomy, 10 (58.8%) with LiTT, and 12 (63.2%) with mc/e; these differences were not statistically significant. SIGNIFICANCE: CC is an effective surgical modality for children with DRE. Regardless of surgical modality, complication rates are acceptable and seizure outcomes generally favorable. Newer, less-invasive, surgical approaches may lead to increased adoption of this efficacious therapeutic option for pediatric DRE.

Super-selective Wada test for pre-surgical leg motor function assessment: A case report.

INTRODUCTION: Wada test is well-known to assess lateralization of memory and language functions; however, super-selective Wada (ss-Wada) to evaluate motor leg function is rare. We present a ss-Wada test within the anterior cerebral artery (ACA) to assess the motor function of the leg. METHODS: Retrospective chart review. RESULTS: Comprehensive phase-I/II surgical evaluation revealed an ictal focus around the left post-central gyrus with immediate involvement around the left para-central regions. To avoid potential right leg motor dysfunction with the surgery, the patient underwent a ss-Wada procedure. Angiography revealed bilateral ACAs were supplied by the left A1 segment. Super-selective microcatheter injection of amobarbital into the left ACA was performed to avoid cross-filling the contralateral ACA. The ss-Wada test confirmed no right leg motor impairment. Afterward, a craniotomy with direct cortical stimulation confirmed that the left-sided ictal/peri-ictal zone had no clear leg motor function. The patient underwent disconnection of that region and remained seizure-free at 10-month post-op follow-up without any motor or sensory deficits in the right limbs. CONCLUSION: This case demonstrates the proof of concept for ss-Wada in assessing lower extremity motor function. The ss-Wada procedure accurately predicted no motor deficits in the right leg, consistent with preserved motor function post-surgery.

Externalization of an infected vagus nerve stimulator generator without a break in anti-seizure therapy: a technical note.

INTRODUCTION: Vagus nerve stimulation (VNS) is a widely used treatment for drug-resistant epilepsy. An infection of the VNS generator pocket occurs in 3-8% of cases. Current standard of care requires the removal of the device, antibiotic therapy, and then replacement of the device. The consequential break in VNS therapy leaves patients highly susceptible to seizures. METHODS: Retrospective case report. RESULTS: The externalized generator continued to provide electroceutical coverage of the patient's seizures while the pocket was sterilized with IV antibiotics as well as betadine and local antibiotics. The externalized generator was kept safe against the patient's chest with ioban and an entriely new system was implanted on post-externalization day 5. The patient is now 7 months post-operative and without any evidence of infection. CONCLUSIONS: We report successful management of an infected VNS generator with externalization and short interval replacement of the entire system without any break in anti-seizure therapy.

Predictive value of video alone in diagnosis of epileptic vs paroxysmal nonepileptic events in children.

OBJECTIVE: Previous studies examined the use of video-based diagnosis and the predictive value of videos for differentiation of epileptic seizures (ES) from paroxysmal nonepileptic events (PNEE) in the adult population. However, there are no such published studies strictly on the pediatric population. Using video-EEG diagnosis as a gold standard, we aimed to determine the diagnostic predictive value of videos of habitual events with or without additional clinical data in differentiating the PNEE from ES in children. METHODS: Consecutive admissions to our epilepsy monitoring unit between June 2020 and December 2020 were analyzed for events of interest. Four child neurologists blinded to the patient's diagnosis formulated a diagnostic impression based upon the review of the video alone and again after having access to basic clinical information, in addition to the video. Features of the video which helped to make a diagnosis were identified by the reviewers as a part of a survey. RESULTS: A total of 54 patients were included (ES n = 24, PNEE n = 30). Diagnostic accuracy was calculated for each reviewer and combined across all the ratings. Diagnostic accuracy by video alone was 74.5% (sensitivity 80.8%, specificity 66.7%). Providing reviewers with basic clinical information in addition to the videos significantly improved diagnostic accuracy compared to viewing the videos alone. Inter-rater reliability between four reviewers based on the video alone showed moderate agreement (κ = 0.51) and unchanged when additional clinical data were presented (κ = 0.51). The ES group was significantly more likely to demonstrate changes in facial expression, generalized stiffening, repetitive eye blinks, and eye deviation when compared with the PNEE group, which was more likely to display bilateral myoclonic jerking. CONCLUSIONS: Video review of habitual events by Child Neurologists may be helpful in reliably distinguishing ES from PNEE in children, even without included clinical information.

Social determinants do not affect access to specialized epilepsy care in veterans.

INTRODUCTION: It is well established that sociodemographic and neighborhood determinants impact access to healthcare. Veterans with epilepsy (VWE) face unique challenges that may limit access to specialized epilepsy care, though institutional initiatives have aimed to minimize disparities. We assessed the extent to which surrogate markers of access to quality care in VWE were impacted by sociodemographic and neighborhood determinants. METHODS: The sample included 180 VWE. Surrogate markers included time between initial diagnosis and admission to epilepsy monitoring unit (EMU) (time to referral, TTR), and the number of CT, MRI, and EEGs conducted prior to initial EMU evaluation. Sociodemographic and neighborhood determinants included age, sex, race, education, neighborhood advantage, rural status, distance from home to the nearest VAMC, and number of service connection (SC) conditions. Significant correlations across variables of interest were entered into a linear regression. Group differences between social factors were assessed for early and late TTR groups (based on 1st and 4th quartiles). RESULTS: The mean TTR was 12 years (SD ±â€¯13.18). Longer TTR was correlated to older age (p < 0.001) and fewer SC conditions (p = 0.03). None of the other factors were significantly correlated to TTR. Older age significantly predicted longer TTR on regression. The earlier TTR group was younger, had more SC conditions, lived closer to a VAMC, and was more likely to be female. Greater geographic distance was correlated with fewer CT scans (p = 0.01). A greater number of MRIs was correlated with older age (p = 0.04). Younger age (p < 0.01) and greater education (p = 0.01) were correlated with more SC. CONCLUSION: Access to epilepsy care among VWE was largely unimpacted by social determinants, with the exception of older age leading to longer TTR. The TTR in VWE was considerably shorter than has been reported in the literature for civilian patients. The Veterans Health Administration model of care may harbor certain advantages in epilepsy treatment.

Home video prediction of epileptic vs. nonepileptic seizures in US veterans.

OBJECTIVE: Using video-EEG (v-EEG) diagnosis as a gold standard, we assessed the predictive diagnostic value of home videos of spells with or without additional limited demographic data in US veterans referred for evaluation of epilepsy. Veterans, in particular, stand to benefit from improved diagnostic tools given higher rates of PNES and limited accessibility to care. METHODS: This was a prospective, blinded diagnostic accuracy study in adults conducted at the Houston VA Medical Center from 12/2015-06/2019. Patients with a definitive diagnosis of epileptic seizures (ES), psychogenic nonepileptic seizures (PNES), or physiologic nonepileptic events (PhysNEE) from v-EEG monitoring were asked to submit home videos. Four board-certified epileptologists blinded to the original diagnosis formulated a diagnostic impression based upon the home video review alone and video plus limited demographic data. RESULTS: Fifty patients (30 males; mean age 47.7 years) submitted home videos. Of these, 14 had ES, 33 had PNES, and three had PhysNEE diagnosed by v-EEG. The diagnostic accuracy by video alone was 88.0%, with a sensitivity of 83.9% and specificity of 89.6%. Providing raters with basic patient demographic information in addition to the home videos did not significantly improve diagnostic accuracy when comparing to reviewing the videos alone. Inter-rater agreement between four raters based on video was moderate with both videos alone (kappa = 0.59) and video plus limited demographic data (kappa = 0.60). SIGNIFICANCE: This study demonstrated that home videos of paroxysmal events could be an important tool in reliably diagnosing ES vs. PNES in veterans referred for evaluation of epilepsy when interpreted by experts. A moderate inter-rater reliability was observed in this study.

Vagus nerve stimulation and heart rate variability: A scoping review of a somatic oscillatory signal.

The goal of this review is to synthesize the literature on vagus nerve stimulator (VNS)-related changes in heart rate variability (HRV) in patients with drug-resistant epilepsy (DRE) and assess the role of these changes in seizure relief. A scoping literature review was performed with the following inclusion criteria: primary articles written in English, involved implantable VNS in humans, and had HRV as a primary outcome. Twenty-nine studies were retrieved, however with considerable heterogeneity in study methods. The overall depression in HRV seen in DRE patients compared to healthy controls persisted even after VNS implant, indicating that achieving "healthy" HRV is not necessary for VNS therapeutic success. Within DRE patients, changes in frequency domain parameters six months after VNS implant returned to baseline after a year. The mechanism of how VNS reduces seizure burden does not appear to be significantly related to alterations in baseline HRV. However, the subtlety of sympathetic/parasympathetic signaling likely requires a more structured approach to experimental and analytic techniques than currently found in the literature.

A Comprehensive Examination of Clinical Characteristics and Determinants of Long-Term Outcomes in Pediatric Cerebral Sinus Venous Thrombosis.

BACKGROUND: To analyze the clinical and neuroimaging features, risk factors, treatment choices, and long-term clinical outcomes in children with cerebral sinus venous thrombosis (CSVT). METHODS: This is a retrospective cohort study of children diagnosed with CSVT between 2002 and 2018 at Texas Children's Hospital. RESULTS: A total of 183 children (male: 62.3%) with CSVT were included. The average presenting age was 7.7 years (S.D.: 5.6). The mean follow-up duration was 33.7 months (S.D.: 38.6). The most common presenting clinical feature was headache (36.6%). Head and neck infections other than meningitis (36.6%) were the most common risk factors. Prevalent neurological examination findings included motor deficit (21.3%) and altered mental status (AMS, 20.2%). Neuroimaging features included hemorrhagic infarction (19.6%), ischemic infarction (8.2%), and intracranial hemorrhage without infarction (5.5%). The most common site of thrombosis was the superior sagittal sinus (37.2%), with 78.2% of patients demonstrating involvement of multiple sinuses. Treatment of choice was low-molecular-weight heparin in 69.4% of patients. Factors associated with worse clinical outcomes included head and neck infections, malignancy (other than hematologic), cardiac disease, and recent surgery; seizure and dehydration on initial presentation; motor abnormalities and AMS on initial examination; ischemic infarct only; and involvement of vein of Trolard on neuroimaging. Thrombus condition on repeat imaging, receiving any anticoagulant/antithrombotic treatment, treatment duration, or follow-up duration was not associated with severity of long-term outcome. CONCLUSIONS: CSVT may lead to unfavorable long-term outcomes in a remarkable portion of pediatric patients. Thus, a high index of suspicion and early and appropriate management of pediatric CSVT is imperative.

Efficacy of vagus nerve stimulation in managing drug-resistant absence epilepsy syndromes.

PURPOSE: Around 11% of patients with absence epilepsy develop drug-resistant absence epilepsy (DRAE), and are at increased risk for developing psychiatric and neurologic comorbidities. Current therapeutic options for DRAE are limited. The purpose of this study was to assess the efficacy of vagus nerve stimulation (VNS) in treating DRAE. METHODS: Our institution maintains a database of patients who received VNS between 2010 and 2022. We identified DRAE patients who were <18 years of age at seizure onset, were electro-clinically diagnosed with an absence epilepsy syndrome (childhood absence, juvenile absence, or Jeavons Syndrome) by an epileptologist, and had normal brain imaging. The primary outcome measure was post-VNS absence seizure frequency. RESULTS: Twenty-six patients (M/F:14/12) were identified. Median age at seizure onset was 7 years (IQR 4-10) and patients experienced seizures for 6 years (IQR 4.3-7.6) before VNS. After VNS, the median absence seizure frequency reduced to 1.5 days (IQR 0.1-3.5) per week from 7 days (IQR 7-7), a 66% reduction seizure frequency. VNS responder rate was 80%, and seven patients achieved seizure freedom. There was no significant effect on VNS efficacy between the time from DRAE diagnosis to VNS placement (p = 0.067) nor the time from first seizure onset to VNS implant (p = 0.80). The median follow-up duration was 4.1 years (IQR 2.4-6.7), without any significant association between follow-up duration and VNS efficacy (r2=0.023) CONCLUSIONS: VNS is effective in managing DRAE. The responder rate was 80%; seizure improvement was independent of age at both seizure onset and latency to VNS after meeting DRAE criteria.

Responsive neurostimulation of thalamic nuclei for regional and multifocal drug-resistant epilepsy in children and young adults.

OBJECTIVE: Responsive neurostimulation (RNS) is a US FDA-approved form of neuromodulation to treat patients with focal-onset drug-resistant epilepsy (DRE) who are ineligible for or whose condition is refractory to resection. However, the FDA approval only extends to use in patients with one or two epileptogenic foci. Recent literature has shown possible efficacy of thalamic RNS in patients with Lennox-Gastaut syndrome and multifocal epilepsy. The authors hypothesized that RNS of thalamic nuclei may be effective in seizure reduction for patients with multifocal or regionalized-onset DRE. METHODS: The authors performed a retrospective chart review of all patients who had an RNS device managed at Texas Children's Hospital between July 2016 and September 2023, with at least one active electrode in the thalamic nuclei and ≥ 12 months of postimplantation follow-up. Information conveyed by the patient or their caregiver provided data on the change in the clinical seizure frequency, quality of life (QOL), and seizure severity between the preimplantation baseline visit and the last office visit (LOV). RESULTS: Thirteen patients (ages 8-24 years) were identified with active RNS leads in thalamic nuclei (11 centromedian and 2 anterior nucleus). At LOV, 46% of patients reported 50%-100% clinical seizure reduction (classified as responders), 15% reported 25%-49% reduction, and 38% reported < 25% reduction or no change. Additionally, 42% of patients reported subjective improvement in QOL and 58% reported improved seizure severity. Patients with focal cortical dysplasia (FCD) responded strongly: 3 of 5 (60%) reported ≥ 80% reduction in seizure burden and improvement in seizure severity and QOL. Patients with multifocal epilepsy and bilateral thalamocortical leads also did well, with all 3 reporting ≥ 50% reduction in seizures. CONCLUSIONS: RNS of thalamic nuclei shows promising results in reducing seizure burden for patients with multifocal or regional-onset DRE, particularly in a bilateral thalamocortical configuration or when addressing an underlying FCD.

Advancing thalamic neuromodulation in epilepsy: Bridging adult data to pediatric care.

Thalamic neuromodulation has emerged as a treatment option for drug-resistant epilepsy (DRE) with widespread and/or undefined epileptogenic networks. While deep brain stimulation (DBS) and responsive neurostimulation (RNS) depth electrodes offer means for electrical stimulation of the thalamus in adult patients with DRE, the application of thalamic neuromodulation in pediatric epilepsy remains limited. To address this gap, the Neuromodulation Expert Collaborative was established within the Pediatric Epilepsy Research Consortium (PERC) Epilepsy Surgery Special Interest Group. In this expert review, existing evidence and recommendations for thalamic neuromodulation modalities using DBS and RNS are summarized, with a focus on the anterior (ANT), centromedian(CMN), and pulvinar nuclei of the thalamus. To-date, only DBS of the ANT is FDA approved for treatment of DRE in adult patients based on the results of the pivotal SANTE (Stimulation of the Anterior Nucleus of Thalamus for Epilepsy) study. Evidence for other thalamic neurmodulation indications and targets is less abundant. Despite the lack of evidence, positive responses to thalamic stimulation in adults with DRE have led to its off-label use in pediatric patients. Although caution is warranted due to differences between pediatric and adult epilepsy, the efficacy and safety of pediatric neuromodulation appear comparable to that in adults. Indeed, CMN stimulation is increasingly accepted for generalized and diffuse onset epilepsies, with recent completion of one randomized trial. There is also growing interest in using pulvinar stimulation for temporal plus and posterior quadrant epilepsies with one ongoing clinical trial in Europe. The future of thalamic neuromodulation holds promise for revolutionizing the treatment landscape of childhood epilepsy. Ongoing research, technological advancements, and collaborative efforts are poised to refine and improve thalamic neuromodulation strategies, ultimately enhancing the quality of life for children with DRE.

Determinants of successful ictal SPECT injection in phase 1 epilepsy presurgical evaluation: Findings from the pediatric epilepsy research consortium surgery database project.

OBJECTIVES: The main goal of presurgical evaluation in drug-resistant focal epilepsy is to identify a seizure onset zone (SOZ). Of the noninvasive, yet resource-intensive tests available, ictal single-photon emission computed tomography (SPECT) aids SOZ localization by measuring focal increases in blood flow within the SOZ via intravenous peri-ictal radionuclide administration. Recent studies indicate that geographic and center-specific factors impact utilization of these diagnostic procedures. Our study analyzed successful ictal SPECT acquisition (defined as peri-ictal injection during inpatient admission) using surgery-related data from the Pediatric Epilepsy Research Consortium (PERC) surgery database. We hypothesized that a high seizure burden, longer duration of video EEG monitoring (VEEG), and more center-specific hours of SPECT availability would increase the likelihood of successful ictal SPECT. METHODS: We identified study participants (≤18 years of age) who underwent SPECT as part of their phase 1 VEEG from January 2018 to June 2022. We assessed association between ictal SPECT outcomes (success vs. failure) and variables including patient demographics, epilepsy history, and center-specific SPECT practices. RESULTS: Phase 1 VEEG monitoring with ictal SPECT injection was planned in 297 participants and successful in 255 participants (85.86%). On multivariable analysis, the likelihood of a successful SPECT injection was higher in patients of non-Hispanic ethnicity (p = 0.040), shorter duration VEEG (p = 0.004), and higher hours of available SPECT services (p < 0.001). Higher seizure frequency (p = 0.033) was significant only in bivariate analysis. Patients treated at centers with more operational hours were more likely to experience pre-admission protocols prior to VEEG (p = 0.002). SIGNIFICANCE: There is inter-center variability in protocols and SPECT acquisition capabilities. Shorter duration of EEG monitoring, non-Hispanic ethnicity (when on private insurance), extended operational hours of nuclear medicine as noted on multivariate analysis and higher seizure frequency in bivariate analysis are strongly associated with successful ictal SPECT injection. PLAIN LANGUAGE SUMMARY: In pediatric patients with drug-resistant epilepsy, single-photon emission computed tomography (SPECT) scans can be helpful in localizing seizure onset zone. However, due to many logistical challenges described below, which include not only the half-life of the technetium isotope used to inject intravenously during a seizure (called the ictal SPECT scan) but also available nuclear scanner time in addition to the unpredictability of seizures, obtaining an ictal SPECT during a planned elective inpatient hospital stay is not guaranteed. Thus, as healthcare costs increase, planning a prolonged hospital stay during which an ictal SPECT scan is not feasible is not optimal. We leveraged our prospective surgery database to look at center-specific factors and patient-specific factors associated with an ictal SPECT injection in the first, pediatric-focussed, large-scale, multicenter, prospective, SPECT feasibility study. We found that longer availability of the scanner is the most important center-specific factor in assuring ictal SPECT injection. Although seizure frequency is an important patient-specific factor on bivariate analysis, this factor lost statistical significance when other factors like patient insurance status and video EEG duration were also considered in our multivariable logistical model.

Pediatric neuromodulation for drug-resistant epilepsy: Survey of current practices, techniques, and outcomes across US epilepsy centers.

Neuromodulation via Responsive Neurostimulation (RNS) or Deep Brain Stimulation (DBS) is an emerging treatment strategy for pediatric drug-resistant epilepsy (DRE). Knowledge gaps exist in patient selection, surgical technique, and perioperative care. Here, we use an expert survey to clarify practices. Thirty-two members of the Pediatric Epilepsy Research Consortium were surveyed using REDCap. Respondents were from 17 pediatric epilepsy centers (missing data in one): Four centers implant RNS only while 13 implant both RNS and DBS. Thirteen RNS programs commenced in or before 2020, and 10 of 12 DBS programs began thereafter. The busiest six centers implant 6-10 new RNS devices per year; all DBS programs implant <5 annually. The youngest RNS patient was 3 years old. Most centers (11/12) utilize MP2RAGE and/or FGATIR sequences for planning. Centromedian thalamic nuclei were the unanimous target for Lennox-Gastaut syndrome. Surgeon exposure to neuromodulation occurred mostly in clinical practice (14/17). Clinically significant hemorrhage (n = 2) or infection (n = 3) were rare. Meaningful seizure reduction (>50%) was reported by 81% (13/16) of centers. RNS and DBS are rapidly evolving treatment modalities for safe and effective treatment of pediatric DRE. There is increasing interest in multicenter collaboration to gain knowledge and facilitate dialogue. PLAIN LANGUAGE SUMMARY: We surveyed 32 pediatric epilepsy centers in USA to highlight current practices of intracranial neuromodulation. Of the 17 that replied, we found that most centers are implanting thalamic targets in pediatric drug-resistant epilepsy using the RNS device. DBS device is starting to be used in pediatric epilepsy, especially after 2020. Different strategies for target identification are enumerated. This study serves as a starting point for future collaborative research.

Pediatric Palliative Epilepsy Surgery: A Report From the Pediatric Epilepsy Research Consortium (PERC) Surgery Database.

BACKGROUND: Epilepsy surgery is an underutilized resource for children with drug-resistant epilepsy. Palliative and definitive surgical options can reduce seizure burden and improve quality of life. Palliative epilepsy surgery is often seen as a "last resort" compared to definitive surgical options. We compare patient characteristics between palliative and definitive epilepsy surgical patients and present palliative surgical outcomes from the Pediatric Epilepsy Research Consortium surgical database. METHODS: The Pediatric Epilepsy Research Consortium Epilepsy Surgery database is a prospective registry of patients aged 0-18 years undergoing evaluation for epilepsy surgery at 20 pediatric epilepsy centers. We included all children with completed surgical therapy characterized as definitive or palliative. Demographics, epilepsy type, age of onset, age at referral, etiology of epilepsy, treatment history, time-to-referral/evaluation, number of failed anti-seizure medications (ASMs), imaging results, type of surgery, and postoperative outcome were acquired. RESULTS: Six hundred forty patients undergoing epilepsy surgery were identified. Patients undergoing palliative procedures were younger at seizure onset (median: 2.1 vs 4 years, P= 0.0008), failed more ASM trials before referral for presurgical evaluation (P=<0.0001), and had longer duration of epilepsy before referral for surgery (P=<0.0001). During presurgical evaluation, patients undergoing palliative surgery had shorter median duration of video-EEG data collected (P=0.007) but number of cases where ictal data were acquired was similar between groups. The most commonly performed palliative procedure was corpus callosotmy (31%), followed by lobectomy (21%) and neuromodulation (82% responsive neurostimulation vs 18% deep brain stimulation). Palliative patients were further categorized into traditionally palliative procedures vs traditionally definitive procedures. The majority of palliative patients had 50% reduction or better in seizure burden. Seizure free outcomes were significantly higher among those with traditional definitive surgeries, 41% (95% confidence interval: 26% to 57%) compared with traditional palliative surgeries and 9% (95% confidence interval: 2% to 17%). Rate of seizure freedom was 46% at 24 months or greater of follow-up in the traditional definitive group. CONCLUSIONS: Patients receiving palliative epilepsy surgery trialed more ASMs, were referred later after becoming drug resistant, and had longer gaps between drug resistance and epilepsy surgery compared with patients undergoing definitive epilepsy surgery. The extent of surgical evaluation is impacted if surgery is thought to be palliative. A majority of palliative surgery patients achieved >50% seizure reduction at follow-up, both in groups that received traditionally palliative and traditionally definitive surgical procedures. Palliative surgical patients can achieve greater seizure control and should be referred to an epilepsy surgery center promptly after failing two appropriate anti-seizure medications.

Leveraging ChatGPT in the Pediatric Neurology Clinic: Practical Considerations for Use to Improve Efficiency and Outcomes.

BACKGROUND: Artificial intelligence (AI) is progressively influencing healthcare sectors, including pediatric neurology. This paper aims to investigate the potential and limitations of using ChatGPT, a large language model (LLM) developed by OpenAI, in an outpatient pediatric neurology clinic. The analysis focuses on the tool's capabilities in enhancing clinical efficiency, productivity, and patient education. METHOD: This is an opinion-based exploration supplemented with practical examples. We assessed ChatGPT's utility in administrative and educational tasks such as drafting medical necessity letters and creating patient educational materials. RESULTS: ChatGPT showed efficacy in streamlining administrative work, particularly in drafting administrative letters and formulating personalized patient education materials. However, the model has limitations in performing higher-order tasks like formulating nuanced differential diagnoses. Additionally, ethical and legal concerns, including data privacy and the potential dissemination of misinformation, warrant cautious implementation. CONCLUSIONS: The integration of AI tools like ChatGPT in pediatric neurology clinics has demonstrated promising results in boosting efficiency and patient education, despite present limitations and ethical concerns. As technology advances, we anticipate future applications may extend to more complex clinical tasks like precise differential diagnoses and treatment strategy guidance. Careful, patient-centered implementation is essential for leveraging the potential benefits of AI in pediatric neurology effectively.

Use of video alone for differentiation of epileptic seizures from non-epileptic spells: A systematic review and meta-analysis.

INTRODUCTION: Review of videos (without electroencephalography) to differentiate epileptic seizures (ES) from non-epileptic spells (NES) may be helpful where epilepsy monitoring is not feasible. Previous studies of video-based diagnosis have suffered from variable accuracy, sensitivity, and specificity. METHODS: We systematically reviewed relevant literature in PubMed, Embase, and Web of Science from inception to September 2022, identifying articles that reported on the video-based diagnosis of ES and NES. In primary analysis, for each study, the most expert group was chosen when different groups of reviewers classified the videos (e.g., epilepsy specialists and general neurologists). In secondary analysis, we compared the diagnostic accuracy of different expertise levels (e.g., epileptologists, general neurologists, residents, medical students). Meta-analysis was performed to obtain pooled estimates of reliability measures. RESULTS: From 5245 articles identified, 13 met the inclusion criteria, with cumulative data from 683 patients (696 videos) reviewed by 95 independent reviewers in primary analysis. Video alone had a strong ability to differentiate ES from NES as evidenced by the following metrics- area under the curve- 0.9 (considered "outstanding"), sensitivity- 82.2% (95% Confidence Interval [C.I], 80.2%-84.0%), specificity- 84.7% (C.I., 82.8%-86.5%), and diagnostic odds ratio- 24.7 (C.I., 11.5-52.9). The secondary analysis showed reviewer-dependent accuracy with epileptologists showing the highest accuracy (DOR 81.2, C.I., 90.0%-94.6%). CONCLUSIONS: Video alone has reliable diagnostic performance for differentiating ES from NES. Meta-analysis limitations include inter-study heterogeneity including variable video quality and reviewer expertise. Combined video-EEG remains the gold standard for the diagnosis of epilepsy and NES.

Seizure control during spontaneous battery failure with responsive neurostimulation.

OBJECTIVE: We examined seizure control during spontaneous responsive neurostimulation (RNS) battery failure to investigate the persistent benefits of RNS in the absence of neurostimulation. We hypothesized that the loss of direct RNS does not immediately result in seizure worsening. METHODS: Patients with RNS battery failure between August 2019 and December 2020 at a single institution were studied. During periods of battery depletion, the RNS device does not store any electrocorticography records, so we used a telephone-based survey and chart review to assess seizure control during battery depletion. RESULTS: Eight of 10 eligible participants responded to the survey, with a mean duration of battery failure of 36 days (range: 10-219 days). During battery depletion, one patient reported decreased seizures and five reported no changes in seizure frequency. Only two patients reported increased seizure frequency. SIGNIFICANCE: The benefits of RNS treatment may outlast the immediate effects of direct neurostimulation, lending support to the notion of persistent neuroplasticity. Larger studies should confirm our preliminary findings.

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

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.

Responsive Neurostimulation in Drug-Resistant Pediatric Epilepsy: Findings From the Epilepsy Surgery Subgroup of the Pediatric Epilepsy Research Consortium.

BACKGROUND: Responsive neurostimulation (RNS), a closed-loop intracranial electrical stimulation system, is a palliative surgical option for patients with drug-resistant epilepsy (DRE). RNS is approved by the US Food and Drug Administration for patients aged ≥18 years with pharmacoresistant partial seizures. The published experience of RNS in children is limited. METHODS: This is a combined prospective and retrospective study of patients aged ≤18 years undergoing RNS placement. Patients were identified from the multicenter Pediatric Epilepsy Research Consortium Surgery Registry from January 2018 to December 2021, and additional data relevant to this study were retrospectively collected and analyzed. RESULTS: Fifty-six patients received RNS during the study period. The mean age at implantation was 14.9 years; the mean duration of epilepsy, 8.1 years; and the mean number of previously trialed antiseizure medications, 4.2. Five patients (9%) previously trialed dietary therapy, and 19 patients (34%) underwent prior surgery. Most patients (70%) underwent invasive electroencephalography evaluation before RNS implantation. Complications occurred in three patients (5.3%) including malpositioned leads or transient weakness. Follow-up (mean 11.7 months) was available for 55 patients (one lost), and four were seizure-free with RNS off. Outcome analysis of stimulation efficacy was available for 51 patients: 33 patients (65%) were responders (≥50% reduction in seizure frequency), including five patients (10%) who were seizure free at follow-up. CONCLUSIONS: For young patients with focal DRE who are not candidates for surgical resection, neuromodulation should be considered. Although RNS is off-label for patients aged <18 years, this multicenter study suggests that it is a safe and effective palliative option for children with focal DRE.