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Edgar Durand,1 Alexander G Weil,2 Marc Lévêque1,3 1Espace éthique de l’Assistance publique-Hôpitaux de Paris et Département de recherche en éthique, South Paris University, Paris, France; 2Pediatric Neurosurgical Department, Sainte-Justine Hospital, Montreal, QC, Canada; 3Service de Neurochirurgie, Hôpital de la Pitié-Salpêtrière, Paris, France We read with interest the article entitled “Anterior capsulotomy improves persistent developmental stuttering with a psychiatric disorder: a case report and literature review” published in Neuropsychiatric Disease and Treatment.1 The authors report on a 28-year-old man with persistent developmental stuttering who was treated by bilateral anterior capsulotomy in the People’s Republic of China. To our knowledge, this is a new and previously unreported application of this technique for this indication. Accordingly, as the authors highlight, “the evidence for surgical treatment of persistent developmental stuttering and associated psychiatric disorders is limited”, placing their approach within the clinical research forum. In this experimental context, this case report brings forward several important reflections on patient evaluation, technique utilized, and postoperative follow-up. Read the original article

Purpose: Tropomyosin receptor kinase (TRK) fusions are detected in less than 2% of central nervous system tumors. There are limited data on the clinical course of affected patients., Experimental Design: We conducted an international retrospective cohort study of patients with TRK fusion-driven central nervous system tumors., Results: A total of 119 patients were identified. The median age at the time of diagnosis was 4.5 years. The majority were reported to have a histology consistent with a diagnosis of high-grade glioma (HGG; 57.1%) followed by low-grade glioma (LGG; 27.7%). Pediatric patients had a better prognosis, with a median overall survival of 185.5 months compared with 24.8 months in adults (P < 0.0001). Patients with LGG also had a better outcome when compared with HGG (P = 0.0012). The objective response was 68.8% with larotrectinib compared with 38.1% for nontargeted treatment., Conclusions: Children with LGG had a favorable outcome compared with adult glioma and HGG. TRK inhibitors seem to improve tumor control., (©2024 The Authors; Published by the American Association for Cancer Research.)

Significance: Maximal safe resection of brain tumors can be performed by neurosurgeons through the use of accurate and practical guidance tools that provide real-time information during surgery. Current established adjuvant intraoperative technologies include neuronavigation guidance, intraoperative imaging (MRI and ultrasound), and 5-ALA for fluorescence-guided surgery., Aim: We have developed intraoperative Raman spectroscopy as a real-time decision support system for neurosurgical guidance in brain tumors. Using a machine learning model, trained on data from a multicenter clinical study involving 67 patients, the device achieved diagnostic accuracies of 91% for glioblastoma, 97% for brain metastases, and 96% for meningiomas. Here, the aim is to assess the generalizability of a predictive model trained with data from this study to other types of brain tumors., Approach: A method was developed to assess the generalizability of the model, quantifying performance for tumors including astrocytoma, oligodendroglioma and ependymoma, pediatric glioblastoma, and classification of glioblastoma data acquired in the presence of 5-ALA induced fluorescence. Statistical analyses were conducted to assess the impact of vibrational bands beyond contributors identified in our previous research., Results: A machine learning brain tumor detection model showed a positive predictive value (PPV) of 70% for astrocytoma, 74% for oligodendroglioma, and 100% for ependymoma. Furthermore, the PPV was 100% in classifying spectra from a pediatric glioblastoma and 90% for detecting adult glioblastoma labeled with 5-ALA-induced fluorescence. Univariate statistical analyses applied to individual vibrational bands demonstrated that the inclusion of Raman biomarkers unexploited to date had the potential to improve detectability, setting the stage for future advances., Conclusions: Developing predictive models relying on the inelastic scattering contrast from a wider pool of Raman bands may improve detection accuracy for astrocytoma and oligodendroglioma. To do so, larger tumor datasets and a higher Raman photon signal-to-noise ratio may be required., (© 2025 The Authors.)

Epilepsy is a neurological disorder characterized by recurring seizures that can cause a wide range of symptoms. Stereo-electroencephalography (SEEG) is a diagnostic procedure where multiple electrodes are stereotactically implanted within predefined brain regions to identify the seizure onset zone, which needs to be surgically removed or disconnected to achieve remission of focal epilepsy. This procedure is complex and challenging due to two main reasons. First, as electrode placement requires good accuracy in desired brain regions, excellent knowledge and understanding of the 3D brain anatomy is required. Second, as typically multiple SEEG electrodes need to be implanted, the positioning of intracerebral electrodes must avoid critical structures (e.g., blood vessels) to ensure patient safety. Traditional SEEG surgical planning relies on 2D display of multi-contrast volumetric medical imaging data, and places a high cognitive demand for surgeons' spatial understanding, resulting in potentially sub-optimal surgical plans and extensive planning time (~ 15 min per electrode). In contrast, virtual reality (VR) presents an intuitive and immersive approach that can offer more intuitive visualization of 3D data as well as potentially enhanced efficiency for neurosurgical planning. Unfortunately, existing VR systems for SEEG surgery only focus on the visualization of post-surgical scans to confirm electrode placement. To address the need, we introduce the first VR system for SEEG planning that integrates user-friendly and efficient visualization and interaction strategies while providing real-time feedback metrics, including distances to nearest blood vessels, angles of insertion, and the overall surgical quality scores. The system reduces the surgical planning time by 91%., Competing Interests: Conflict of interestThe authors declare no conflict of interest., (© The Author(s) 2024.)

Epileptic spasms (ES) are a unique seizure type typically presenting in the form of infantile epileptic spasms syndrome (IESS) with characteristic hypsarrhythmia on scalp EEG and a preponderance with developmental delay or regression. While pharmacotherapy is the mainstay of treatment, surgical options, including disconnective or resective procedures, are increasingly recognized as viable therapeutic options for recurrent or persistent ES. However, limited data on safety, effectiveness, and prognostic factors hinder informed decision-making regarding surgery indications, timing, and intervention type. We performed a systematic review and an individual patient data meta-analysis (IPDMA) in accordance with PRISMA guidelines, focusing on surgical interventions for ES and reporting seizure outcomes using the Engel or ILAE scales. Twenty-six studies encompassing 358 ES patients undergoing resection/callosotomy were included. Participants undergoing other approaches (e.g., multiple subpial transections) or multimodality approaches were excluded from analysis. The median age at spasm onset was 6 months (IQR = 3.0-15.6), with a median age at surgery of 37 months (IQR = 17.2-76.8). Most patients (74.1%) exhibited additional seizure types. A total of 136 patients (35.8%) underwent corpus callosotomy (CC), of whom 125 (91.9%) had a complete callosotomy, while 11 (8.1%) had a partial callosotomy. Resective surgery was performed on 222 patients (58.4%). Among those who underwent resection, 109 (49.1%) had both lesional MRI findings and lateralized EEG abnormalities. Overall, 201 patients (56.1%) remained spasm-free at a median postoperative follow-up of 36 months (interquartile range, IQR = 21-60), including 52 (38.2%) from the callosotomy group and 149 (67.1%) from the resective surgery group. In the resective surgery cohort, patients with MRI-confirmed lesions ( p = 0.026; HR = 0.53, 95% CI = 0.31-0.93) and those who underwent hemispherectomy ( p = 0.026, HR = 0.46, 95% CI = 0.23-0.91) had better seizure outcomes. Only a minority (24.4%) underwent invasive EEG monitoring prior to ES surgery. Surgical treatment of ES proves effective, with two thirds of patients undergoing resective surgery and a third undergoing CC becoming spasm free. Post-operative developmental improvement was observed in 44 participants (65.7% of those with available data). The presence of lesional MRI and more extensive resection/disconnection (e.g., hemispherectomy) emerged as significant prognostic factors for spasm freedom and can inform clinical decision-making., Competing Interests: AW has served as a paid consultant for Monteris Medical inc and on an advisory board for Synergia SA. GI receives investigator-initiated grant funding, speak-ing fees, and advisory fees from LivaNova, and advisory fees from Medtronic. AF is a consultant for Esplorstis Medical. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Cottier, Niazi, Goël, Korman, Porte, Ducruet, Cossu, Briscoe, Singh, Harini, Ibrahim, Fallah, Weil and Hadjinicolaou.)

Background and Objectives: There is a paucity of data directly comparing the outcome of surgical techniques available for the treatment of hypothalamic hamartomas (HHs). This study aims to evaluate the safety and efficacy of commonly used surgical approaches in the treatment of HH-related epilepsy., Methods: A systematic review and individual participant data (IPD) meta-analysis was conducted. The PubMed, Embase, and Scopus online databases were searched without any date restrictions for original studies with more than 1 participant reporting on patients with HH-related epilepsy who underwent surgical treatment. Random-effects modeling was used to calculate the pooled proportions of seizure freedom (Engel I) at the last follow-up. IPD were used to perform mixed-effects logistic regression to identify predictors of seizure freedom and major postoperative complications., Results: Sixty-four studies were included, and IPD on 517 patients were obtained. The overall quality of evidence was low. After the index procedure, the pooled proportion of overall seizure freedom was 50.0% (95% CI 42.7%-57.4%), which increased to 64.5% (95% CI 57.2%-71.5%) after multiple treatments. Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) and radiofrequency thermocoagulation (RFTC) demonstrated the highest efficacy at the last follow-up, with seizure freedom rates of 74.5% (95% CI 66.8%-81.7%) and 78.5% (95% CI 71.6%-84.8%), respectively. Factors independently associated with a lower likelihood of seizure freedom included multiple seizure types (odds ratio [OR] 0.296, 95% CI 0.140-0.624, p = 0.001) and previous surgery (OR 0.418, 95% CI 0.198-0.884, p = 0.023). Stereotactic radiosurgery (SRS) was the safest approach, with a pooled proportion of major complications of 0.0% (95% CI 0.0%-1.4%). Only surgical technique emerged as an independent predictor of major complications, with SRS (OR 0.024, 95% CI 0.002-0.292, p = 0.004), RFTC (OR 0.133, 95% CI 0.026-0.692, p = 0.017), and MRgLITT (OR 0.234, 95% CI 0.056-0.968, p = 0.045) being associated with a lower likelihood of major complications., Discussion: MRgLITT and RFTC offer superior efficacy and safety compared with open microsurgery and should be considered as first-line options. Despite its lower efficacy, SRS is associated with few reported long-term complications, making it a viable alternative for select cases, such as small HHs with good baseline functioning. Direct comparisons between techniques are limited by short follow-up durations in RFTC and MRgLITT cohorts. Further large-scale, multicenter studies directly comparing these modalities are warranted.

Background: Microsurgical resection of drug-resistant epilepsy-associated perirolandic lesions can lead to postoperative motor impairment. Magnetic resonance imaging (MRI)-guided laser interstitial thermal therapy (MRgLITT) has emerged as a less invasive alternative, offering reduced surgical risks and improved neurological outcomes. Electrophysiological tools routinely used for motor mapping in resective microsurgery are incompatible with intraoperative MRI. The utilization of advanced neuroimaging adjuncts for eloquent brain mapping during MRgLITT is imperative. The authors present the case of a 17-year-old athlete who underwent MRgLITT for a perirolandic long-term epilepsy-associated tumor (LEAT). They performed probabilistic multi-tissue constrained spherical deconvolution (MT-CSD) tractography to delineate the corticospinal tract (CST) for presurgical planning and intraoperative image guidance. The CST tractography was integrated into neuronavigation and MRgLITT workstation software to guide the ablation while monitoring the CST throughout the procedure., Observations: The integration of CST tractography into neuronavigation workstation planning and laser ablation workstation thermoablation is feasible and practical, facilitating complete ablation of a deep-seated perirolandic LEAT while preserving motor function., Lessons: Probabilistic MT-CSD tractography enhanced MRgLITT planning as well as intraprocedural CST visualization and preservation, leading to a favorable functional outcome. The limitations of tractography and the predictability of thermal output distribution compared to the gold standard of microsurgical resection merit further discussion. https://thejns.org/doi/10.3171/CASE24139.

Background and Objectives: Complete resection of epileptogenic zone is the single most important determinant of favorable seizure outcomes in resective surgery. However, identifying and resecting this zone is challenging in patients harboring diffuse; MRI-occult malformations of cortical development, such as focal cortical dysplasia; or acquired pathology, such as Rasmussen encephalitis. Intraoperative adjuncts that can aid in identifying the lesion and/or epileptogenic zone can optimize the extent of resection and seizure outcome. We sought to study a novel intraoperative tool, brain tonometer, to measure brain stiffness and correlate with histopathological and radiological findings., Methods: Brain stiffness was measured at various presumed normal and abnormal areas of the cortex during surgery in 2 patients with drug-resistant epilepsy. These results were correlated with preoperative and intraoperative neuroimaging and histopathology., Results: We found brain stiffness correlated well with the degree of inflammation and cortical disorganization., Conclusion: Brain tonometry may help to intraoperatively identify inflammatory brain tissue along with structural and histopathological abnormalities. In select cases, this could potentially allow more tailored resections of the underlying lesion, to ensure complete removal of the epileptogenic lesion and improve the probability of achieving seizure freedom, while sparing normal brain leading to better functional outcomes., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Introduction: Cannabis products have been used in the management of headaches in adults and may play a role in pediatric chronic pain. Canadian pediatricians report increasing use of cannabis for the management of chronic headaches, despite no well-controlled studies to inform its dosing, safety, and effectiveness. The aim of our clinical trial is to determine the dosing and safety of a Cannabidiol (CBD)-enriched Cannabis Herbal Extract (CHE) for the treatment of chronic headaches in adolescents., Methods and Analysis: Youth, parents, and an expert steering committee co-designed this tolerability study. Twenty adolescents (aged 14 to 17 years), with a chronic migraine diagnosis for more than 6 months that has not responded to other therapies will be enrolled into an open label, dose escalation study across three Canadian sites. Study participants will receive escalating doses of a CBD-enriched CHE (MPL-001 with a THC:CBD of 1:25), starting at 0.2-0.4 mg/kg of CBD per day and escalating monthly up to 0.8-1.0 mg/kg of CBD per day. The primary objective of this study is to determine the safety and tolerability of CBD-enriched CHE in adolescents with chronic migraine. Secondary objectives of this study will inform the development of subsequent randomized controlled trials and include investigating the relationship between the dose escalation and change in the frequency of headache, impact and intensity of pain, changes in sleep, mood, function, and quality of life. Exploratory outcomes include investigating steady-state trough plasma levels of bioactive cannabinoids and investigating how pharmacogenetic profiles affect cannabinoid metabolism among adolescents receiving CBD-enriched CHE., Discussion: This protocol was co-designed with youth and describes a tolerability clinical trial of CBD-enriched CHE in adolescents with chronic headaches that have not responded to conventional therapies. This study is the first clinical trial on cannabis products in adolescents with chronic headaches and will inform the development of future comparative effectiveness clinical trials., Trial Registration: CAN-CHA trial is registered with ClinicalTrials.gov with a number of register NCT05337033., Competing Interests: Lauren E Kelly is the Scientific Director for The Canadian Collaborative for Childhood Cannabinoid Therapeutics (C4T) academic research team. She holds funding from the Canadian Institutes of Health Research, Canadian Cancer Society, Research Manitoba, the Sick Kids Foundation, the Children’s Hospital Research Institute of Manitoba, the University of Manitoba and a Mitacs Accelerate award in partnership with Canopy Growth. Dr. Kelly was a member of the Scientific Advisory Board for Health Products Containing Cannabis at Health Canada and is president elect for the Board of Directors of the Canadian Consortium for the Investigation of Cannabinoids (CCIC). Richard J. Huntsmanis a clinical lead for both the Cannabinoid Research Initiative of Saskatchewan and C4T. He was the co-chair of the Scientific Advisory Committee for Health Products Containing Cannabis at Health Canada. Katelynn E Boerner’s time is supported by a fellowship from the Canadian Child Health Clinician Scientist Program, and she currently holds unrelated funding from the Society of Pediatric Psychology, BC Children’s Hospital Research Institute, Canadian Institutes of Health Research, ZonMw: The Netherlands Organization for Health Research and Development, and the CHILD-BRIGHT Network. Evan C. Lewis has received speaking honoraria from Spectrum Therapeutics, Biome Grow, MedReleaf and Miravo Healthcare. He holds a non-salaried position as the Chief Medical Advisor for the JMCC Group. He was the Vice President of Neurology Services for Numinus, a psychedelic medicine treatment and research company. He is a member of the Expert Committee of the Medical Cannabis Clinicians Society (MCCS) in the United Kingdom and sits on the Advisory Councils for Cannabis Patient Advocacy & Support Services (CPASS) and MedCan. In the last 12 months, Samantha Lee Irwin has received honoraria for authoring a chapter for the Canadian Pharmacy Association (CPhA) and for doing an online lecture for NeuroDiem. She also receives compensation for scientific consulting (Impel NeuroPharma Inc, Biohaven Pharmaceuticals and Lundbeck A/S) and has had research support from the Duke Clinical Research Institute. Manik Chhabra has been granted the 2022 Research Manitoba-George & Fay Yee Centre for Healthcare Innovation in Health Research PhD Studentship Award. There are no patents, products in development or marketed products associated with this research to declare. This does not alter our adherence to PLOS ONE policies on sharing data and materials., (Copyright: © 2024 Chhabra et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Histone H3-mutant gliomas are deadly brain tumors characterized by a dysregulated epigenome and stalled differentiation. In contrast to the extensive datasets available on tumor cells, limited information exists on their tumor microenvironment (TME), particularly the immune infiltrate. Here, we characterize the immune TME of H3.3K27M and G34R/V-mutant gliomas, and multiple H3.3K27M mouse models, using transcriptomic, proteomic and spatial single-cell approaches. Resolution of immune lineages indicates high infiltration of H3-mutant gliomas with diverse myeloid populations, high-level expression of immune checkpoint markers, and scarce lymphoid cells, findings uniformly reproduced in all H3.3K27M mouse models tested. We show these myeloid populations communicate with H3-mutant cells, mediating immunosuppression and sustaining tumor formation and maintenance. Dual inhibition of myeloid cells and immune checkpoint pathways show significant therapeutic benefits in pre-clinical syngeneic mouse models. Our findings provide a valuable characterization of the TME of oncohistone-mutant gliomas, and insight into the means for modulating the myeloid infiltrate for the benefit of patients., (© 2024. The Author(s).)

Introduction: Operculo-insular epilepsy (OIE) is a rare condition amenable to surgery in well-selected cases. Despite the high rate of neurological complications associated with OIE surgery, most postoperative deficits recover fully and rapidly. We provide insights into this peculiar pattern of functional recovery by investigating the longitudinal reorganization of structural networks after surgery for OIE in 10 patients., Methods: Structural T1 and diffusion-weighted MRIs were performed before surgery (t 0 ) and at 6 months (t 1 ) and 12 months (t 2 ) postoperatively. These images were processed with an original, comprehensive structural connectivity pipeline. Using our method, we performed comparisons between the t 0 and t 1 timepoints and between the t 1 and t 2 timepoints to characterize the progressive structural remodeling., Results: We found a widespread pattern of postoperative changes primarily in the surgical hemisphere, most of which consisted of reductions in connectivity strength (CS) and regional graph theoretic measures (rGTM) that reflect local connectivity. We also observed increases in CS and rGTMs predominantly in regions located near the resection cavity and in the contralateral healthy hemisphere. Finally, most structural changes arose in the first six months following surgery (i.e., between t 0 and t 1 )., Discussion: To our knowledge, this study provides the first description of postoperative structural connectivity changes following surgery for OIE. The ipsilateral reductions in connectivity unveiled by our analysis may result from the reversal of seizure-related structural alterations following postoperative seizure control. Moreover, the strengthening of connections in peri-resection areas and in the contralateral hemisphere may be compatible with compensatory structural plasticity, a process that could contribute to the recovery of functions seen following operculo-insular resections for focal epilepsy., Competing Interests: MD is co-owner and chief scientific officer at Imeka Solutions Inc. (www.imeka.ca). The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (Copyright © 2024 Obaid, Guberman, St-Onge, Campbell, Edde, Lamsam, Bouthillier, Weil, Daducci, Rheault, Nguyen and Descoteaux.)

Surgical management of drug-resistant epilepsy (DRE) in patients with multiple periventricular nodular heterotopias (PVNHs) is challenging. Identifying the location of seizure onset within these complex epileptic networks is difficult, and open resection carries risks of injury to surrounding functional white matter tracts such as optic radiations (ORs). The authors demonstrate tractography-assisted laser ablation of a single nodule in a patient with DRE and multiple PVNHs. Following surgery, visual fields were intact, highlighting the benefits of OR tractographic reconstruction. At 12 months postoperatively, the patient remained seizure free, suggesting the potential efficacy of targeting a single heterotopia within complex networks in well-selected cases. The video can be found here: https://stream.cadmore.media/r10.3171/2024.4.FOCVID2417., Competing Interests: Disclosures Dr. Weil reported personal fees from Monteris Medical outside the submitted work, and advisory board member for Synergia SA.Dr. Weil reported personal fees from Monteris Medical outside the submitted work, and advisory board member for Synergia SA., (© 2024, The Authors.)

Study Design: Systematic review of the literature., Objectives: In recent years, brain-computer interface (BCI) has emerged as a potential treatment for patients with spinal cord injury (SCI). This is the first systematic review of the literature on invasive closed-loop BCI technologies for the treatment of SCI in humans., Materials and Methods: A comprehensive search of PubMed MEDLINE, Web of Science, and Ovid EMBASE was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines., Results: Of 8316 articles collected, 19 studies met all the inclusion criteria. Data from 21 patients were extracted from these studies. All patients sustained a cervical SCI and were treated using either a BCI with intracortical microelectrode arrays (n = 18, 85.7%) or electrocorticography (n = 3, 14.3%). To decode these neural signals, machine learning and statistical models were used: support vector machine in eight patients (38.1%), linear estimator in seven patients (33.3%), Hidden Markov Model in three patients (14.3%), and other in three patients (14.3%). As the outputs, ten patients (47.6%) underwent noninvasive functional electrical stimulation (FES) with a cuff; one (4.8%) had an invasive FES with percutaneous stimulation, and ten (47.6%) used an external device (neuroprosthesis or virtual avatar). Motor function was restored in all patients for each assigned task. Clinical outcome measures were heterogeneous across all studies., Conclusions: Invasive techniques of BCI show promise for the treatment of SCI, but there is currently no technology that can restore complete functional autonomy in patients with SCI. The current techniques and outcomes of BCI vary greatly. Because invasive BCIs are still in the early stages of development, further clinical studies should be conducted to optimize the prognosis for patients with SCI., Competing Interests: Conflict of Interest Alexander G. Weil is a paid consultant for Monteris Medical, Inc and a Clinical Advisory Board member for Synergia. Lior M. Elkaim is an employee with Neuralink. The remaining authors reported no conflict of interest., (Copyright © 2023 International Neuromodulation Society. Published by Elsevier Inc. All rights reserved.)

Background: Refractory (RSE) and super-refractory status epilepticus (SRSE) are serious neurological conditions requiring aggressive management. Beyond anesthetic agents, there is a lack of evidence guiding management in these patients. This systematic review and individual participant data meta-analysis (IPDMA) seeks to evaluate and compare the currently available surgical techniques for the acute treatment of RSE and SRSE., Methods: A systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses for Individual Participant Data (PRISMA-IPD). Only patients who underwent surgery while in RSE and SRSE were included. Descriptive statistics were used to compare various subgroups. Multivariable logistic regression models were constructed to identify predictors of status epilepticus (SE) cessation, long-term overall seizure freedom, and favorable functional outcome (i.e., modified Rankin score of 0-2) at last follow-up., Results: A total of 87 studies including 161 participants were included. Resective surgery tended to achieve better SE cessation rate (93.9%) compared to non-resective techniques (83.9%), but this did not reach significance ( p  = 0.071). Resective techniques were also more likely to achieve seizure freedom (69.1% vs. 34.4%, p  = <0.0001). Older age at SE (OR = 1.384[1.046-1.832], p  = 0.023) was associated with increased likelihood of SE cessation, while longer duration of SE (OR = 0.603[0.362-1.003], p  = 0.051) and new-onset seizures (OR = 0.244[0.069-0.860], p  = 0.028) were associated with lower likelihood of SE cessation, but this did not reach significance for SE duration. Only shorter duration of SE prior to surgery (OR = 1.675[1.168-2.404], p   = 0.0060) and immediate termination of SE (OR = 3.736 [1.323-10.548], p  = 0.014) were independently associated with long-term seizure status. Rates of favorable functional outcomes (mRS of 0-2) were comparable between resective (44.4%) and non-resective (44.1%) techniques, and no independent predictors of outcome were identified., Conclusion: Our findings suggest that emergency neurosurgery may be a safe and effective alternative in patients with RSE/SRSE and may be considered earlier during the disease course. However, the current literature is limited exclusively to small case series and case reports with high risk of publication bias. Larger clinical trials assessing long-term seizure and functional outcomes are warranted to establish robust management guidelines., Competing Interests: AF serves on the board for Pediatric Epilepsy Surgery Alliance. GI serves on the advisory board of Synergia Medical. AW is a consultant for Monteris and serves on the advisory board of Synergia Medical. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The handling editor SS declared a shared affiliation with the author NS at the time of review., (Copyright © 2024 Niazi, Han, Stamm, Shlobin, Korman, Hoang, Kielian, Du Pont-Thibodeau, Ducharme Crevier, Major, Nguyen, Bouthillier, Ibrahim, Fallah, Hadjinicolaou and Weil.)

Background and Objectives: Hemimegalencephaly (HME) is a rare diffuse malformation of cortical development characterized by unihemispheric hypertrophy, drug-resistant epilepsy (DRE), hemiparesis, and developmental delay. Definitive treatment for HME-related DRE is hemispheric surgery through either anatomic (AH) or functional hemispherectomy (FH). This individual patient data meta-analysis assessed seizure outcomes of AH and FH for HME with pharmacoresistant epilepsy, predictors of Engel I, and efficacy of different FH approaches., Methods: PubMed, Web of Science, and Cumulative Index to Nursing and Allied Health Literature were searched from inception to Jan 13th, 2023, for primary literature reporting seizure outcomes in >3 patients with HME receiving AH or FH. Demographics, neurophysiology findings, and Engel outcome at the last follow-up were extracted. Postsurgical seizure outcomes were compared through 2-tailed t -test and Fisher exact test. Univariate and multivariate Cox regression analyses were performed to identify independent predictors of Engel I outcome., Results: Data from 145 patients were extracted from 26 studies, of which 89 underwent FH (22 vertical, 33 lateral), 47 underwent AH, and 9 received an unspecified hemispherectomy with a median last follow-up of 44.0 months (FH cohort) and 45.0 months (AH cohort). Cohorts were similar in preoperative characteristics and at the last follow-up; 77% (n = 66) of the FH cohort and 81% (n = 38) and of the AH cohort were Engel I. On multivariate analysis, only the presence of bilateral ictal electroencephalography abnormalities (hazard ratio = 11.5; P = .002) was significantly associated with faster time-to-seizure recurrence. A number-needed-to-treat analysis to prevent 1 additional case of posthemispherectomy hydrocephalus reveals that FH, compared with AH, was 3. There was no statistical significance for any differences in time-to-seizure recurrence between lateral and vertical FH approaches (hazard ratio = 2.59; P = .101)., Conclusion: We show that hemispheric surgery is a highly effective treatment for HME-related DRE. Unilateral ictal electroencephalography changes and using the FH approach as initial surgical management may result in better outcomes due to significantly lower posthemispherectomy hydrocephalus probability. However, larger HME registries are needed to further delineate the predictors of seizure outcomes., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Introduction: Hypothalamic hamartomas (HHs) are deep-seated congenital lesions that typically lead to pharmacoresistant epilepsy and a catastrophic encephalopathic syndrome characterised by severe neuropsychological impairment and decline in quality of life. A variety of surgical approaches and technologies are available for the treatment of HH-related pharmacoresistant epilepsy. There remains, however, a paucity of literature directly comparing their relative efficacy and safety. This protocol aims to facilitate a systematic review and meta-analysis that will characterise and compare the probability of seizure freedom and relevant postoperative complications across different surgical techniques performed for the treatment of HH-related pharmacoresistant epilepsy., Methods and Analysis: This protocol was developed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses for Individual Participant Data guidelines. Three major databases, PubMed, Embase and Scopus, will be systematically searched from database inception and without language restrictions for relevant articles using our predefined search strategy. Title-abstract and full text screening using inclusion and exclusion criteria created a priori will be performed by two independent reviewers to identify eligible articles. Conflicts will be resolved via discussion with a third team member. Following data extraction of both study-level and individual patient data (IPD), a study-level and IPD meta-analysis will be performed. Study-level analysis will focus on assessing the degree of heterogeneity in the data and quantifying overall seizure outcomes for each surgical technique. The IPD analysis will use multivariable regression to determine perioperative predictors of seizure freedom and complications that can guide patient and technique selection., Ethics and Dissemination: This work will not require ethics approval as it will be solely based on previously published and available data. The results of this review will be shared via conference presentation and submission to peer-reviewed neurosurgical journals., Prospero Registration: CRD42022378876., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background and Objectives: Gelastic seizures due to hypothalamic hamartomas (HH) are challenging to treat, in part due to an incomplete understanding of seizure propagation pathways. Although magnetic resonance imaging-guided laser interstitial thermal therapy (MRgLITT) is a promising intervention to disconnect HH from ictal propagation networks, the optimal site of ablation to achieve seizure freedom is not known. In this study, we investigated intraoperative post-ablation changes in resting-state functional connectivity to identify large-scale networks associated with successful disconnection of HH., Methods: Children who underwent MRgLITT for HH at two institutions were consecutively recruited and followed for a minimum of one year. Seizure freedom was defined as Engel score of 1A at the last available follow-up. Immediate pre- and post- ablation resting-state functional MRI scans were acquired while maintaining a constant depth of general anesthetic. Multivariable generalized linear models were used to identify intraoperative changes in large-scale connectivity associated with seizure outcomes., Results: Twelve patients underwent MRgLITT for HH, five of whom were seizure-free at their last follow-up. Intraprocedural changes in thalamocortical circuitry involving the anterior cingulate cortex were associated with seizure-freedom. Children who were seizure-free demonstrated an increase and decrease in connectivity to the pregenual and dorsal anterior cingulate cortices, respectively. In addition, children who became seizure-free demonstrated increased thalamic connectivity to the periaqueductal gray immediately following MRgLITT., Discussion: Successful disconnection of HH is associated with intraoperative, large-scale changes in thalamocortical connectivity. These changes provide novel insights into the large-scale basis of gelastic seizures and may represent intraoperative biomarkers of treatment success., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Objectives: Although hemispheric surgeries are among the most effective procedures for drug-resistant epilepsy (DRE) in the pediatric population, there is a large variability in seizure outcomes at the group level. A recently developed HOPS score provides individualized estimation of likelihood of seizure freedom to complement clinical judgement. The objective of this study was to develop a freely accessible online calculator that accurately predicts the probability of seizure freedom for any patient at 1-, 2-, and 5-years post-hemispherectomy., Methods: Retrospective data of all pediatric patients with DRE and seizure outcome data from the original Hemispherectomy Outcome Prediction Scale (HOPS) study were included. The primary outcome of interest was time-to-seizure recurrence. A multivariate Cox proportional-hazards regression model was developed to predict the likelihood of post-hemispheric surgery seizure freedom at three time points (1-, 2- and 5- years) based on a combination of variables identified by clinical judgment and inferential statistics predictive of the primary outcome. The final model from this study was encoded in a publicly accessible online calculator on the International Network for Epilepsy Surgery and Treatment (iNEST) website (https://hops-calculator.com/)., Results: The selected variables for inclusion in the final model included the five original HOPS variables (age at seizure onset, etiologic substrate, seizure semiology, prior non-hemispheric resective surgery, and contralateral fluorodeoxyglucose-positron emission tomography [FDG-PET] hypometabolism) and three additional variables (age at surgery, history of infantile spasms, and magnetic resonance imaging [MRI] lesion). Predictors of shorter time-to-seizure recurrence included younger age at seizure onset, prior resective surgery, generalized seizure semiology, FDG-PET hypometabolism contralateral to the side of surgery, contralateral MRI lesion, non-lesional MRI, non-stroke etiologies, and a history of infantile spasms. The area under the curve (AUC) of the final model was 73.0%., Significance: Online calculators are useful, cost-free tools that can assist physicians in risk estimation and inform joint decision-making processes with patients and families, potentially leading to greater satisfaction. Although the HOPS data was validated in the original analysis, the authors encourage external validation of this new calculator., (© 2023 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

Background: There is little data on patient and caregiver perceptions of spine surgery in children and youth. This study aims to characterize the personal experiences of patients, caregivers, and family members surrounding pediatric spine surgery through a qualitative and quantitative social media analysis., Methods: The Twitter application programming interface was searched for keywords related to pediatric spine surgery from inception to March 2022. Relevant tweets and accounts were extracted and subsequently classified using thematic labels. Tweet metadata was collected to measure user engagement via multivariable regression. Sentiment analysis using Natural Language Processing was performed on all tweets with a focus on tweets discussing the personal experiences of patients and caregivers., Results: 2424 tweets from 1847 individual accounts were retrieved for analysis. Patients and caregivers represented 1459 (79.0%) of all accounts. Posts discussed the personal experiences of patients and caregivers in 83.5% of tweets. Pediatric spine surgery research was discussed in few posts (n=90, 3.7%). Within the personal experience category, 975 (48.17%) tweets were positive, 516 (25.49%) were negative, and 533 (26.34%) were neutral. Presence of a tag (beta: -6.1, 95% CI -9.7 to -2.5) and baseline follower count (beta<0.001, 95% CI <0.001 to <0.001) significantly affected tweet engagement negatively and positively, respectively., Conclusions: Patients and caregivers actively discuss topics related to pediatric spine surgery on Twitter. Posts discussing personal experience are most prevalent, while posts on research are scarce, unlike previous social media studies. Pediatric spine surgeons can leverage this dialogue to better understand the worries and needs of patients and their families., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Lennox-Gastaut syndrome (LGS) is a severe form of epileptic encephalopathy, presenting during the first years of life, and is very resistant to treatment. Once medical therapy has failed, palliative surgeries such as vagus nerve stimulation (VNS) or corpus callosotomy (CC) are considered. Although CC is more effective than VNS as the primary neurosurgical treatment for LGS-associated drop attacks, there are limited data regarding the added value of CC following VNS. This study aimed to assess the effectiveness of CC preceded by VNS., Methods: This multinational, multicenter retrospective study focuses on LGS children who underwent CC before the age of 18 years, following prior VNS, which failed to achieve satisfactory seizure control. Collected data included epilepsy characteristics, surgical details, epilepsy outcomes, and complications. The primary outcome of this study was a 50% reduction in drop attacks., Results: A total of 127 cases were reviewed (80 males). The median age at epilepsy onset was 6 months (interquartile range [IQR] = 3.12-22.75). The median age at VNS surgery was 7 years (IQR = 4-10), and CC was performed at a median age of 11 years (IQR = 8.76-15). The dominant seizure type was drop attacks (tonic or atonic) in 102 patients. Eighty-six patients underwent a single-stage complete CC, and 41 an anterior callosotomy. Ten patients who did not initially have a complete CC underwent a second surgery for completion of CC due to seizure persistence. Overall, there was at least a 50% reduction in drop attacks and other seizures in 83% and 60%, respectively. Permanent morbidity occurred in 1.5%, with no mortality., Significance: CC is vital in seizure control in children with LGS in whom VNS has failed. Surgical risks are low. A complete CC has a tendency toward better effectiveness than anterior CC for some seizure types., (© 2023 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

Objective: Microvascular decompression (MVD) as a treatment for trigeminal neuralgia (TGN) has high success rate but is associated with risks of complication. This study analyzes Twitter to provide insights into discussions surrounding MVD for patients with TGN., Methods: A Twitter search performed in April 2022 yielded 491 tweets from 426 accounts. Tweets and accounts were classified thematically, and descriptive statistics were used for various social media metrics. Using a natural language processing machine learning algorithm, sentiment analysis (SA) was performed to evaluate patient perspectives before and after surgery, and a multivariate regression model was used to identify predictors of higher engagement metrics (likes, retweets, quote tweets, replies)., Results: Most accounts were patients, caregivers, and other members of the public (70%). The most encountered themes were research (47%) and personal experiences (33.4%). SA of tweets about patient experiences showed that 40.2% of tweets were positive, 31.1% were neutral and 28.7% were negative. Negative tweets decreased significantly in postoperative tweets and mostly discussed complications or failure of surgery (63%). On multivariate analysis, only inclusion of media (photo or video) in a Tweet was associated with higher engagement metrics., Conclusions: This study provides a comprehensive review of Twitter use discussing MVD in TGN and is the first to assess patient satisfaction after treatment using SA. The data presented on patient perspectives on social media could help physicians establish direct lines of communication with patients, fostering a more patient-focused care., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Polycomb Repressive Complex 2 (PRC2)-mediated histone H3K27 tri-methylation (H3K27me3) recruits canonical PRC1 (cPRC1) to maintain heterochromatin. In early development, polycomb-regulated genes are connected through long-range 3D interactions which resolve upon differentiation. Here, we report that polycomb looping is controlled by H3K27me3 spreading and regulates target gene silencing and cell fate specification. Using glioma-derived H3 Lys-27-Met (H3K27M) mutations as tools to restrict H3K27me3 deposition, we show that H3K27me3 confinement concentrates the chromatin pool of cPRC1, resulting in heightened 3D interactions mirroring chromatin architecture of pluripotency, and stringent gene repression that maintains cells in progenitor states to facilitate tumor development. Conversely, H3K27me3 spread in pluripotent stem cells, following neural differentiation or loss of the H3K36 methyltransferase NSD1, dilutes cPRC1 concentration and dissolves polycomb loops. These results identify the regulatory principles and disease implications of polycomb looping and nominate histone modification-guided distribution of reader complexes as an important mechanism for nuclear compartment organization., Highlights: The confinement of H3K27me3 at PRC2 nucleation sites without its spreading correlates with increased 3D chromatin interactions.The H3K27M oncohistone concentrates canonical PRC1 that anchors chromatin loop interactions in gliomas, silencing developmental programs.Stem and progenitor cells require factors promoting H3K27me3 confinement, including H3K36me2, to maintain cPRC1 loop architecture.The cPRC1-H3K27me3 interaction is a targetable driver of aberrant self-renewal in tumor cells.

Objective: MR-guided laser interstitial thermal therapy (MRgLITT) is associated with lower seizure-free outcome but better safety profile compared to open surgery. However, the predictors of seizure freedom following MRgLITT remain uncertain. This study aimed to use machine learning to predict seizure-free outcome following MRgLITT and to identify important predictors of seizure freedom in children with drug-resistant epilepsy., Methods: This multicenter study included children treated with MRgLITT for drug-resistant epilepsy at 13 epilepsy centers. The authors used clinical data, diagnostic investigations, and ablation features to predict seizure-free outcome at 1 year post-MRgLITT. Patients from 12 centers formed the training cohort, and patients in the remaining center formed the testing cohort. Five machine learning algorithms were developed on the training data by using 10-fold cross-validation, and model performance was measured on the testing cohort. The models were developed and tested on the complete feature set. Subsequently, 3 feature selection methods were used to identify important predictors. The authors then assessed performance of the parsimonious models based on these important variables., Results: This study included 268 patients who underwent MRgLITT, of whom 44.4% had achieved seizure freedom at 1 year post-MRgLITT. A gradient-boosting machine algorithm using the complete feature set yielded the highest area under the curve (AUC) on the testing set (AUC 0.67 [95% CI 0.50-0.82], sensitivity 0.71 [95% CI 0.47-0.88], and specificity 0.66 [95% CI 0.50-0.81]). Logistic regression, random forest, support vector machine, and neural network yielded lower AUCs (0.58-0.63) compared to the gradient-boosting machine but the findings were not statistically significant (all p > 0.05). The 3 feature selection methods identified video-EEG concordance, lesion size, preoperative seizure frequency, and number of antiseizure medications as good prognostic features for predicting seizure freedom. The parsimonious models based on important features identified by univariate feature selection slightly improved model performance compared to the complete feature set., Conclusions: Understanding the predictors of seizure freedom after MRgLITT will assist with prognostication.

Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has emerged as a popular minimally invasive alternative to open resective surgery for drug-resistant epilepsy (DRE). We sought to perform a systematic review and individual participant data meta-analysis to identify independent predictors of seizure outcome and complications following MRgLITT for DRE. Eleven databases were searched from January 1, 2010 to February 6, 2021 using the terms "MR-guided ablation therapy" and "epilepsy". Multivariable mixed-effects Cox and logistic regression identified predictors of time to seizure recurrence, seizure freedom, operative complications, and postoperative neurological deficits. From 8705 citations, 46 studies reporting on 450 MRgLITT DRE patients (mean age = 29.5 ± 18.1 years, 49.6% female) were included. Median postoperative seizure freedom and follow-up duration were 15.5 and 19.0 months, respectively. Overall, 240 (57.8%) of 415 patients (excluding palliative corpus callosotomy) were seizure-free at last follow-up. Generalized seizure semiology (hazard ratio [HR] = 1.78, p = .020) and nonlesional magnetic resonance imaging (MRI) findings (HR = 1.50, p = .032) independently predicted shorter time to seizure recurrence. Cerebral cavernous malformation (CCM; odds ratio [OR] = 7.97, p < .001) and mesial temporal sclerosis/atrophy (MTS/A; OR = 2.21, p = .011) were independently associated with greater odds of seizure freedom at last follow-up. Operative complications occurred in 28 (8.5%) of 330 patients and were independently associated with extratemporal ablations (OR = 5.40, p = .012) and nonlesional MRI studies (OR = 3.25, p = .017). Postoperative neurological deficits were observed in 53 (15.1%) of 352 patients and were independently predicted by hypothalamic hamartoma etiology (OR = 5.93, p = .006) and invasive electroencephalographic monitoring (OR = 4.83, p = .003). Overall, MRgLITT is particularly effective in treating patients with well-circumscribed lesional DRE, such as CCM and MTS/A, but less effective in nonlesional cases or lesional cases with a more diffuse epileptogenic network associated with generalized seizures. This study identifies independent predictors of seizure freedom and complications following MRgLITT that may help further guide patient selection., (© 2023 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

Background and Objectives: Hemispheric surgery effectively treats unihemispheric pediatric drug-resistant epilepsy (DRE) by resecting and/or disconnecting the epileptic hemisphere. Modifications to the original anatomic hemispherectomy have generated multiple functionally equivalent, disconnective techniques for performing hemispheric surgery, termed functional hemispherotomy. While a myriad of hemispherotomy variants exist, all of them can be categorized according to the anatomic plane they are performed in, which includes vertical approaches at or near the interhemispheric fissure and lateral approaches at or near the Sylvian fissure. This meta-analysis of individual patient data (IPD) aimed to compare seizure outcomes and complications between the hemispherotomy approaches to better characterize their relative efficacy and safety in the modern neurosurgical treatment of pediatric DRE, given emerging evidence that outcomes may differ between them., Methods: CINAHL, Embase, PubMed, and Web of Science were searched from inception to September 9, 2020, for studies reporting IPD from pediatric patients with DRE who underwent hemispheric surgery. Outcomes of interest were seizure freedom at last follow-up, time-to-seizure recurrence, and complications including hydrocephalus, infection, and mortality. The χ 2 test compared the frequency of seizure freedom and complications. Multivariable mixed-effects Cox regression controlling for predictors of seizure outcome was performed on propensity score-matched patients to compare time-to-seizure recurrence between approaches. Kaplan-Meier curves were made to visualize differences in time-to-seizure recurrence., Results: Fifty-five studies reporting on 686 unique pediatric patients treated with hemispheric surgery were included for meta-analysis. Among the hemispherotomy subgroup, vertical approaches resulted in a greater proportion of seizure free patients (81.2% vs 70.7%, p = 0.014) than lateral approaches. While there were no differences in complications, lateral hemispherotomy had higher rates of revision hemispheric surgery due to incomplete disconnection and/or recurrent seizures than vertical hemispherotomy (16.3% vs 1.2%, p < 0.001). After propensity score matching, vertical hemispherotomy approaches independently conferred longer time-to-seizure recurrence than lateral hemispherotomy approaches (hazard ratio 0.44, 95% CI 0.19-0.98)., Discussion: Among functional hemispherotomy techniques, vertical hemispherotomy approaches confer more durable seizure freedom than lateral approaches without compromising safety. Future prospective studies are required to definitively determine whether vertical approaches are indeed superior and how it should influence clinical guidelines for performing hemispheric surgery., (© 2023 American Academy of Neurology.)

Objective: Delayed cerebral ischemia (DCI) associated with vasospasm is well described in the setting of aneurysmal subarachnoid hemorrhage (SAH). In addition, DCI is very infrequently encountered in patients who have undergone resection of a brain tumor with unclear pathophysiology. The occurrence of DCI in the pediatric population is exceedingly rare, and outcomes in this population have, to the authors' knowledge, never been systematically reviewed. Therefore, the authors present what is to their knowledge the largest series of pediatric patients with this complication and systematically reviewed the literature for individual participant data., Methods: The authors conducted a retrospective review of 172 sellar and suprasellar tumors in pediatric patients who underwent surgery at the Montreal Children's Hospital between 1999 and 2017 to identify cases of vasospasm occurring after tumor resection. Descriptive statistics, including patient characteristics, intraoperative and postoperative findings, and outcome status, were collected. A systematic review was also conducted using three databases (PubMed, Web of Science, Embase) to identify reported cases available in the literature of vasospasm after tumor resection in children and collect individual participant data on these patients for further analysis., Results: Six patients treated at Montreal Children's Hospital were identified, with an average age of 9.5 years (range 6-15 years). The prevalence of vasospasm after tumor resection was 3.5% (6/172). Vasospasm in all 6 patients occurred after craniotomy was performed to treat a suprasellar tumor. The average interval from surgery to symptoms was 3.25 days (range 12 hours-10 days). The most common tumor etiology was craniopharyngioma, seen in 4 cases. Extensive tumor encasement of blood vessels requiring significant operative manipulation was described in all 6 patients. A rapid decrease in serum sodium (exceeding 12 mEq/L/24 hrs or below 135 mEq/L) was seen in 4 patients. On final follow-up, 3 patients were left with significant disability, and all patients had persistent deficits. A systematic review of the literature revealed a total of 10 other patients whose characteristics and treatment were compared with those of the 6 patients treated at Montreal Children's Hospital., Conclusions: Vasospasm after tumor resection in children and youth is likely a rare entity, with a prevalence of 3.5% in this case series. Suprasellar tumor location (particularly craniopharyngioma tumor etiology), significant encasement of blood vessels by the tumor, and postoperative hyponatremia may be predictive factors. Outcome is poor, with most patients having significant persistent neurological deficits.

Objective: To perform a systematic review and meta-analysis to identify whether tuberectomy and tuberectomy plus are associated with different postoperative seizure outcomes in patients with tuberous sclerosis complex (TSC) -related epilepsy., Methods: Electronic databases (PubMed, Embase, Cochrane, Proquest, Web of Science, Scopus, Biosis Previews) were searched without date restriction. Retrospective cohort studies of participants with TSC-associated epilepsy undergoing resective surgery that reported demographics, presurgical evaluation, extent of resection and postoperative seizure outcomes were included. Title, abstract and the full text were checked independently and in duplicate by two reviewers. Disagreements were resolved through discussion. One author extracted data which was verified by a second author using identified common standard in advance, including using a risk of bias tool we agreed on to evaluate study quality., Results: Five studies, with a total of 327 participants, were included. One hundred and sixty patients received tuberectomy, and 93 of them (58.1%) achieved postoperative seizure freedom, while the other 167 patients underwent tuberectomy plus, and 128 of them (76.6%) achieved seizure freedom after adequate follow-ups (RR=0.72, 95% CI [0.60, 0.87], P<0.05). Subgroup analysis found that 40 of 63 (63.5%) patients after tuberectomy and 66 of 78 (84.6%) patients after tuberectomy plus of a single tuber achieved seizure freedom (RR = 0.71, 95% CI [0.56,0.91], P<0.05). In the multituber subrgroup, 16 of 42 (38.1%) and 21 of 31 (67.7%) patients achieved seizure freedom, after tuberectomy and tuberectomy plus, respectively (RR = 0.57, 95% CI [0.32,1.03], P = 0.06)., Conclusions: Tuberectomy plus is a more effective treatment than tuberectomy for patients with TSC-related intractable epilepsy., Competing Interests: Declaration of Competing Interest The authors declare that they have no competing interests., (Copyright © 2023. Published by Elsevier Ltd.)

Background: A subpopulation of patients with Sturge-Weber syndrome (SWS) develop medically intractable epilepsy. There is a paucity of literature on preoperative factors that predict postoperative seizure outcomes in these patients. An individual participant data meta-analysis (IPDMA) was performed to discern preoperative variables associated with favorable seizure outcomes in pediatric SWS patients undergoing epilepsy surgery., Methods: PubMed, Cochrane, Web of Science, and Scopus were independently queried following PRISMA guidelines. Studies that reported seizure outcomes in individual pediatric SWS patients were selected. Preoperative demographic variables and disease characteristics were recorded and evaluated in a time-to-event fashion via Cox regression and Kaplan-Meier analysis with log-rank test., Results: A total of 18 studies with 108 patients were included for meta-analysis. Median age at seizure onset was 4.5 months, and 85 patients (78.7%) were seizure-free at last follow-up (median: 72 months). On multivariable Cox regression, no variables were independent predictors of post-operative seizure freedom duration, including the extent of hemispheric resection. There were also no differences in time-to-seizure recurrence on Kaplan-Meier analysis when comparing those treated with hemispheric surgery and those with less than hemispheric surgery (p = 0.52)., Conclusion: This IPDMA showed that both resective and hemispheric epilepsy surgery achieve favorable and comparable seizure outcomes in pediatric SWS patients. The best available evidence using IPD suggests that resective surgery may be an appropriate alternative to hemispheric epilepsy surgery in well-selected patients. Prospective multi-institutional studies with greater follow-up are warranted to further investigate predictors of seizure outcome in pediatric SWS patients., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Insular epilepsy (IE) is an increasingly recognized cause of drug-resistant epilepsy amenable to surgery. However, concerns of suboptimal seizure control and permanent neurological morbidity hamper widespread adoption of surgery for IE. We performed a systematic review and individual participant data meta-analysis to determine the efficacy and safety profile of surgery for IE and identify predictors of outcomes. Of 2483 unique citations, 24 retrospective studies reporting on 312 participants were eligible for inclusion. The median follow-up duration was 2.58 years (range, 0-17 years), and 206 (66.7%) patients were seizure-free at last follow-up. Younger age at surgery (≤18 years; HR = 1.70, 95% CI = 1.09-2.66, P = .022) and invasive EEG monitoring (HR = 1.97, 95% CI = 1.04-3.74, P = .039) were significantly associated with shorter time to seizure recurrence. Performing MR-guided laser ablation or radiofrequency ablation instead of open resection (OR = 2.05, 95% CI = 1.08-3.89, P = .028) was independently associated with suboptimal or poor seizure outcome (Engel II-IV) at last follow-up. Postoperative neurological complications occurred in 42.5% of patients, most commonly motor deficits (29.9%). Permanent neurological complications occurred in 7.8% of surgeries, including 5% and 1.4% rate of permanent motor deficits and dysphasia, respectively. Resection of the frontal operculum was independently associated with greater odds of motor deficits (OR = 2.75, 95% CI = 1.46-5.15, P = .002). Dominant-hemisphere resections were independently associated with dysphasia (OR = 13.09, 95% CI = 2.22-77.14, P = .005) albeit none of the observed language deficits were permanent. Surgery for IE is associated with a good efficacy/safety profile. Most patients experience seizure freedom, and neurological deficits are predominantly transient. Pediatric patients and those requiring invasive monitoring or undergoing stereotactic ablation procedures experience lower rates of seizure freedom. Transgression of the frontal operculum should be avoided if it is not deemed part of the epileptogenic zone. Well-selected candidates undergoing dominant-hemisphere resection are more likely to exhibit transient language deficits; however, the risk of permanent deficit is very low., (© 2022 The Authors. Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

Objective: Cognitive measures are an important primary outcome of pediatric, adolescents, and childhood epilepsy surgery. The purpose of this systematic review and meta-analysis is to assess whether there are long-term alterations (≥ 5 years) in the Full-Scale Intelligence Quotient (FSIQ) of pediatric patients undergoing epilepsy surgery., Methods: Electronic databases (EMBASE, MEDLINE, and Scopus) were searched for English articles from inception to October 2022 that examined intelligence outcomes in pediatric epilepsy surgery patients. Inclusion criteria were defined as the patient sample size of ≥ 5, average follow- up of ≥5 years, and surgeries performed on individuals ≤ 18 years old at the time of surgery. Exclusion criteria consisted of palliative surgery, animal studies, and studies not reporting surgical or FSIQ outcomes. Publication bias was assessed using a funnel plot and the Quality in Prognosis Studies (QUIPS) toolset was used for quality appraisal of the selected articles. A random-effects network meta-analysis was performed to compare FSIQ between surgical patients at baseline and follow-up and Mean Difference (MD) was used to calculate the effect size of each study. Point estimates for effects and 95% confidence intervals for moderation analysis were performed on variables putatively associated with the effect size., Results: 21,408 studies were screened for abstract and title. Of these, 797 fit our inclusion and exclusion criteria and proceeded to full-text screening. Overall, seven studies met our requirements and were selected. Quantitative analysis was performed on these studies (N = 330). The mean long-term difference between pre- and post- operative FSIQ scores across all studies was noted at 3.36 [95% CI: (0.14, 6.57), p = 0.04, I2 = 0%] and heterogeneity was low., Conclusion: To our knowledge, this is the first meta-analysis to measure the long-term impacts of FSIQ in pediatric and adolescent epilepsy patients. Our overall results in this meta-analysis indicate that while most studies do not show long-term FSIQ deterioration in pediatric patients who underwent epilepsy surgery, there was an increase of 3.36 FSIQ points, however, the observed changes were not clinically significant. Moreover, at the individual patient level analysis, while most children did not show long-term FSIQ deterioration, few had significant decline. These findings indicate the importance of surgery as a viable option for pediatric patients with medically refractory epilepsy., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to disclose., (Copyright © 2023. Published by Elsevier Ltd.)

Invasive brain-computer interfaces hold promise to alleviate disabilities in individuals with neurologic injury, with fully implantable brain-computer interface systems expected to reach the clinic in the upcoming decade. Children with severe neurologic disabilities, like quadriplegic cerebral palsy or cervical spine trauma, could benefit from this technology. However, they have been excluded from clinical trials of intracortical brain-computer interface to date. In this manuscript, we discuss the ethical considerations related to the use of invasive brain-computer interface in children with severe neurologic disabilities. We first review the technical hardware and software considerations for the application of intracortical brain-computer interface in children. We then discuss ethical issues related to motor brain-computer interface use in pediatric neurosurgery. Finally, based on the input of a multidisciplinary panel of experts in fields related to brain-computer interface (functional and restorative neurosurgery, pediatric neurosurgery, mathematics and artificial intelligence research, neuroengineering, pediatric ethics, and pragmatic ethics), we then formulate initial recommendations regarding the clinical use of invasive brain-computer interfaces in children.

Background: Cerebral hemispherectomy can effectively treat unihemispheric epilepsy. However, posthemispherectomy hydrocephalus (PHH), a serious life-long complication, remains prevalent, requiring careful considerations in technique selection and postoperative management. In 2016, we began incorporating open choroid plexus cauterization (CPC) into our institution's hemispherectomy procedure in an attempt to prevent PHH., Objective: To determine whether routine CPC prevented PHH without exacerbating hemispherectomy efficacy or safety., Methods: A retrospective review of consecutive patients who underwent hemispherectomy for intractable epilepsy between 2011 and 2021 was performed. Multivariate logistic regression was used to identify factors independently associated with PHH requiring cerebrospinal fluid (CSF) shunting., Results: Sixty-eight patients were included in this study, of whom 26 (38.2%) underwent CPC. Fewer patients required CSF shunting in the CPC group (7.7% vs 28.7%, P = .033) and no patients who underwent de novo hemispherectomy with CPC developed PHH. Both cohorts experienced seizure freedom (65.4% vs 59.5%, P = .634) and postoperative complications, including infection (3.8% vs 2.4%, P = .728), hemorrhage (0.0% vs 2.4%, P = .428), and revision hemispherectomy (19.2% vs 14.3%, P = .591) at similar rates. Patients without CPC had greater odds of developing PHH requiring CSF shunting (odds ratio = 8.36, P = .026). The number needed to treat with CPC to prevent an additional case of PHH was 4.8, suggesting high effectiveness., Conclusion: Preventing PHH is critical. Our early experience demonstrated that routinely incorporating CPC into hemispherectomy effectively prevents PHH without causing additional complications, especially in first-time hemispherectomies. A multicenter randomized controlled trial with long-term follow-up is required to corroborate the findings of our single-institutional case series and determine whether greater adoption of this technique is justified., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)

Despite the widespread use of intraoperative electrocorticography (iECoG) during resective epilepsy surgery, there are conflicting data on its overall efficacy and inability to predict benefit per pathology. Given the heterogeneity of iECoG use in resective epilepsy surgery, it is important to assess the utility of interictal-based iECoG. This individual patient data (IPD) meta-analysis seeks to identify the benefit of iECoG during resective epilepsy surgery in achieving seizure freedom for various pathologies. Embase, Scopus, and PubMed were searched from inception to January 31, 2021 using the following terms: "ecog", "electrocorticography", and "epilepsy". Articles were included if they reported seizure freedom at ≥12-month follow-up in cohorts with and without iECoG for epilepsy surgery. Non-English articles, noncomparative iECoG cohorts, and studies with <10% iECoG use were excluded. This meta-analysis followed the PRISMA 2020 guidelines. The primary outcome was seizure freedom at last follow-up and time to seizure recurrence, if applicable. Forest plots with random effects modeling assessed the relationship between iECoG use and seizure freedom. Cox regression of IPD was performed to identify predictors of longer duration of seizure freedom. Kaplan-Meier curves with log-rank test were created to visualize differences in time to seizure recurrence. Of 7504 articles identified, 18 were included for study-level analysis. iECoG was not associated with higher seizure freedom at the study level (relative risk = 1.09, 95% confidence interval [CI] = 0.96-1.23, p = .19, I 2  = 64%), but on IPD (n = 7 studies, 231 patients) iECoG use was independently associated with more favorable seizure outcomes (hazard ratio = 0.47, 95% CI = .23-.95, p = .037). In Kaplan-Meier analysis of specific pathologies, iECoG use was significantly associated with longer seizure freedom only for focal cortical dysplasia (FCD; p < .001) etiology. Number needed to treat for iECoG was 8.8, and for iECoG in FCD it was 4.7. We show iECoG seizure freedom is not achieved uniformly across centers. iECoG is particularly beneficial for FCD etiology in improving seizure freedom., (© 2022 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

Objective: Minimally invasive magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has been proposed as an alternative to open epilepsy surgery, to address concerns regarding the risk of open surgery. Our primary hypothesis was that seizure freedom at 1 year after MRgLITT is noninferior to open surgery in children with drug-resistant epilepsy (DRE). The secondary hypothesis was that MRgLITT has fewer complications and shorter hospitalization than surgery. The primary objective was to compare seizure outcome of MRgLITT to open surgery in children with DRE. The secondary objective was to compare complications and length of hospitalization of the two treatments., Methods: This retrospective multicenter cohort study included children with DRE treated with MRgLITT or open surgery with 1-year follow-up. Exclusion criteria were corpus callosotomy, neurostimulation, multilobar or hemispheric surgery, and lesion with maximal dimension > 60 mm. MRgLITT patients were propensity matched to open surgery patients. The primary outcome was seizure freedom at 1 year posttreatment. The difference in seizure freedom was compared using noninferiority test, with noninferiority margin of -10%. The secondary outcomes were complications and length of hospitalization., Results: One hundred eighty-five MRgLITT patients were matched to 185 open surgery patients. Seizure freedom at 1 year follow-up was observed in 89 of 185 (48.1%) MRgLITT and 114 of 185 (61.6%) open surgery patients (difference = -13.5%, one-sided 97.5% confidence interval = -23.8% to ∞, p Noninferiority  = .79). The lower confidence interval boundary of -23.8% was below the prespecified noninferiority margin of -10%. Overall complications were lower in MRgLITT compared to open surgery (10.8% vs. 29.2%, respectively, p < .001). Hospitalization was shorter for MRgLITT than open surgery (3.1 ± 2.9 vs. 7.2 ± 6.1 days, p < .001)., Significance: Seizure outcome of MRgLITT at 1 year posttreatment was inferior to open surgery. However, MRgLITT has the advantage of better safety profile and shorter hospitalization. The findings will help counsel children and parents on the benefits and risks of MRgLITT and contribute to informed decision-making on treatment options., (© 2022 International League Against Epilepsy.)

Background: Juvenile Pilocytic Astrocytomas (JPAs) are one of the most common pediatric brain tumors, and they are driven by aberrant activation of the mitogen-activated protein kinase (MAPK) signaling pathway. RAF-fusions are the most common genetic alterations identified in JPAs, with the prototypical KIAA1549-BRAF fusion leading to loss of BRAF's auto-inhibitory domain and subsequent constitutive kinase activation. JPAs are highly vascular and show pervasive immune infiltration, which can lead to low tumor cell purity in clinical samples. This can result in gene fusions that are difficult to detect with conventional omics approaches including RNA-Seq., Methods: To this effect, we applied RNA-Seq as well as linked-read whole-genome sequencing and in situ Hi-C as new approaches to detect and characterize low-frequency gene fusions at the genomic, transcriptomic and spatial level., Results: Integration of these datasets allowed the identification and detailed characterization of two novel BRAF fusion partners, PTPRZ1 and TOP2B, in addition to the canonical fusion with partner KIAA1549. Additionally, our Hi-C datasets enabled investigations of 3D genome architecture in JPAs which showed a high level of correlation in 3D compartment annotations between JPAs compared to other pediatric tumors, and high similarity to normal adult astrocytes. We detected interactions between BRAF and its fusion partners exclusively in tumor samples containing BRAF fusions., Conclusions: We demonstrate the power of integrating multi-omic datasets to identify low frequency fusions and characterize the JPA genome at high resolution. We suggest that linked-reads and Hi-C could be used in clinic for the detection and characterization of JPAs., (© 2022. The Author(s).)

Canonical (H3.1/H3.2) and noncanonical (H3.3) histone 3 K27M-mutant gliomas have unique spatiotemporal distributions, partner alterations and molecular profiles. The contribution of the cell of origin to these differences has been challenging to uncouple from the oncogenic reprogramming induced by the mutation. Here, we perform an integrated analysis of 116 tumors, including single-cell transcriptome and chromatin accessibility, 3D chromatin architecture and epigenomic profiles, and show that K27M-mutant gliomas faithfully maintain chromatin configuration at developmental genes consistent with anatomically distinct oligodendrocyte precursor cells (OPCs). H3.3K27M thalamic gliomas map to prosomere 2-derived lineages. In turn, H3.1K27M ACVR1-mutant pontine gliomas uniformly mirror early ventral NKX6-1 + /SHH-dependent brainstem OPCs, whereas H3.3K27M gliomas frequently resemble dorsal PAX3 + /BMP-dependent progenitors. Our data suggest a context-specific vulnerability in H3.1K27M-mutant SHH-dependent ventral OPCs, which rely on acquisition of ACVR1 mutations to drive aberrant BMP signaling required for oncogenesis. The unifying action of K27M mutations is to restrict H3K27me3 at PRC2 landing sites, whereas other epigenetic changes are mainly contingent on the cell of origin chromatin state and cycling rate., (© 2022. The Author(s), under exclusive licence to Springer Nature America, Inc.)

Background: High-grade gliomas (HGG) with BRAF V600E mutation represent a unique subset of central nervous system tumors. Targeted therapies including BRAF and MEK inhibitors are now being explored as possible new treatment options., Methods: We report an 18-year-old female with a grade 3 pleomorphic xanthoastrocytoma treated upfront with dabrafenib and trametinib. We also conducted a systematic literature review of patients with HGG and BRAF V600E mutations treated with BRAF inhibitors., Results: Despite local recurrences resected surgically, the patient has been on dabrafenib and trametinib for more than 54 months. Thirty-two patients with HGG and BRAF V600E mutations treated with BRAF inhibitors were retrieved through our systematic review of the literature. Only 1 young patient with an anaplastic ganglioglioma was treated upfront with a BRAF inhibitor with a curative intent. Best response reported with radiation therapy and systemic therapy was a stable disease (SD) for 18 patients (56.3%) and progressive disease (PD) for 9 patients (28.1%). Responses to treatment regimens that included BRAF inhibitors were reported in 31 patients and included 4 complete responses (12.9%), 23 partial responses (74.2%), 2 SDs (6.5%), and 2 PDs (6.5%)., Conclusions: Our patient had durable disease control with dabrafenib and trametinib. Given favorable responses reported in patients with HGG treated with BRAF inhibitors, we believe that upfront targeted therapy is a possible treatment approach that should be studied in the context of a clinical trial., (© The Author(s) 2022. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)

Rationale: Up to 40% of pediatric epilepsy cases are drug-resistant and associated with neurocognitive, psychosocial, developmental comorbidities, and risk of early mortality. Epilepsy surgery (ES) may be considered after the failure of two anti-seizure medications (ASM) to provide patients with the opportunity to attain seizure freedom. However, only a small proportion of eligible patients receive surgical treatment. This scoping review aims to elucidate barriers to pediatric ES to understand the reasons for its underutilization., Methods: Embase, PubMed, and Scopus were searched from inception through August 2022 for the following PICO terms: "pediatric", "parents", "epilepsy", "surgery", and "decision-making". Studies exploring barriers to ES were included and qualitatively synthesized. We adopted an inductive thematical approach, and barriers hindering ES were assigned to four thematic categories. PRISMA Sc-R guidelines were followed., Results: Of 3400 retrieved studies, 17 were included. Barriers to ES were classified into 4 categories. Parental barriers originating from misperception, lack of knowledge regarding surgical outcomes, and emotional vulnerability were highlighted in 76% of included studies. Physician-based barriers, including lack of clinical expertise, trust, and communication, leading to inadequate informed consent and referral to surgical evaluation, were described in 65% of articles. Patient-based barriers were reported in 47% of studies and included clinical characteristics modulating acceptance of ES. Only 18% of studies described healthcare system-based barriers, including intricate insurance policies not adapted to sociodemographic disparities., Conclusion: This study highlights the complexity of barriers to pediatric ES. Our findings emphasize the need for multileveled strategies to increase the utilization of ES among eligible pediatric patients., Competing Interests: Declaration of Competing Interest All authors have no relevant financial relationships or conflicts of interest to disclose., (Copyright © 2022. Published by Elsevier Ltd.)

Objective: This study aims to glean patient and caregiver perspectives surrounding deep brain stimulation (DBS) in children and youth through an analysis of patterns of social media usage., Methods: The authors performed a comprehensive search of the Twitter Application Programming Interface (API) database for all tweets about DBS use in children and youth, with no date restriction. Data pertaining to each tweet were extracted for analysis. Results were analyzed using qualitative and quantitative methodologies. These included thematic analysis of tweets, accounts, and descriptive statistics. Sentiment analysis of extracted tweets was also performed. A multivariable regression model was used to identify predictors of higher engagement metrics (likes, retweets, and quotes)., Results: A comprehensive search of the Twitter database yielded 877 tweets from 816 unique accounts meeting study inclusion criteria. Most tweets were from patients or caregivers, researchers, or news media outlets. The most common themes among analyzed tweets were research discussing novel findings (45.2%) or personal experiences of patients or caregivers (27.4%). Sentiment analysis showed that 54.5% of tweets were positive, 35.1% were neutral, and 10.4% were negative. The presence of pictures or videos increased the tweet engagement count by an average of 10.5 (95% CI 7.3-13.6). Tweets about personal patient experiences (β = 6, 95% CI 0.95-12) and tweets tagging other accounts (β = 3.2, 95% CI 0.63-5.8) were also significantly associated with higher engagement metrics., Conclusions: The current study is the first to assess patient and caregiver perspectives surrounding pediatric DBS through a comprehensive analysis of social media usage. Given the nascent field, social media presents an opportunity to share experiences and promote patient and healthcare professional education surrounding pediatric DBS.

Objective: Long-term seizure outcomes of pediatric epilepsy surgery are understudied. A systematic review and independent patient data meta-analysis was performed to study seizure outcomes ≥ 10 years following pediatric resective epilepsy surgery., Methods: Electronic literature searches of PubMed, Web of Science, and CINAHL were conducted for relevant articles from inception to April 2020. The following search terms were used in various combinations: "pediatric", "child", "adolescent", "epilepsy", "resective", "surgery", "long-term", "longitudinal", "10 year". Two reviewers (W.B.H., T.B.C.) performed title, abstract, and full-text screening. All relevant perioperative factors reported that may be associated with long-term seizure outcomes were recorded at a study or individual participant level. The primary outcome was long-term (≥ 10 year) seizure freedom measured by the Engel Classification scale, and available data on functional outcomes were also reviewed., Results: Twenty-five articles met criteria for inclusion in the study, which were analyzed for proportions of 10-year seizure freedom ranging from 57.6% at the study level to 64.8% at the individual patient level. At the study level, the proportion of patients remaining seizure free at least 10 years postoperatively (61.2%; 95% CI 52.5-69.3) was significantly less than at 1 year (74.2%; 95% CI 69.3-78.6; p = 0.008) but not at 2 years (67.9%; 95% CI 58.6-76.0) or 5 years (63.7%; 95% CI 55.4-71.2). No differences in long-term seizure freedom were detected by etiology or surgery type. At the individual patient level, univariate logistic regression analyses of all variables putatively associated with seizure freedom demonstrated that lobectomy (OR 0.280, 95% CI 0.117-0.651, p = 0.003) was associated with decreased long-term seizure freedom (41.9%) compared to lesionectomy (75.7%) and hemispherectomy (69.4%), which achieved similar results., Conclusion: Resective surgery is a durable and potentially curative treatment option for select pediatric patients with refractory epilepsy. On a group level, two-thirds of children have long-term seizure freedom ≥ 10 years after resective epilepsy surgery. Given the greatest rate of change occurs in the first 2 years, this may serve as the best short-term follow-up period to predict long-term outcome. Although lobectomy appears to be a strong predictor for lower likelihood of long-term seizure freedom, long-term prognostication on an individual patient level is still not possible. Uniform data reporting and prospective, multicenter studies collecting high quality, stratified (e.g., by etiology, surgery type) data over an extended postoperative interval are recommended to further examine the durability of resective surgery as a treatment for pediatric epilepsy., Competing Interests: Declaration of Competing Interest None of the authors has any conflict of interest to disclose., (Copyright © 2022. Published by Elsevier Ltd.)

Objective: The prevalence of long-term postoperative sensorimotor deficits in children undergoing perirolandic resective epilepsy surgery is unclear. The risk of developing these deficits must be weighed against the potential reduction in seizure frequency after surgery. In this study, the authors investigated the prevalence of sensorimotor deficits after resective surgery at ≥ 1 year postoperatively., Methods: A systematic review and individual patient data meta-analysis was conducted using PubMed, Embase, and Scopus databases. Subgroups of patients were identified and categorized according to their outcomes as follows: group A patients were denoted as seizure free with no postoperative sensorimotor deficits; group B patients experienced seizure recurrence with no deficit; group C patients were seizure free with deficits; and group D patients were not seizure free and with deficits. Rates of sensory deficits were examined in patients undergoing postcentral gyrus resection, and rates of motor deficits were aggregated in patients undergoing precentral gyrus resection., Results: Of 797 articles resulting from the database searches, 6 articles including 164 pediatric patients at a mean age of 7.7 ± 5.2 years with resection for drug-resistant perirolandic epilepsy were included in the study. Seizure freedom was observed in 118 (72.9%) patients at a mean follow-up of 3.4 ± 1.8 years. In total, 109 (66.5%) patients did not develop sensorimotor deficits at last follow-up, while 55 (33.5%) had permanent deficits. Ten (14.3%) of 70 patients with postcentral gyrus resection had permanent sensory deficits. Of the postcentral gyrus resection patients, 41 (58.6%) patients were included in group A, 19 (27.1%) in group B, 7 (10.0%) in group C, and 3 (4.3%) in group D. Forty (37.7%) of 106 patients with precentral resections had permanent motor deficits. Of the precentral gyrus resection patients, 50 (47.2%) patients were in group A, 16 (15.1%) in group B, 24 (22.6%) in group C, and 16 (15.1%) in group D. Patients without focal cortical dysplasia were more likely to have permanent motor deficits relative to those with focal cortical dysplasia in the precentral surgery cohort (p = 0.02)., Conclusions: In total, 58.6% of patients were seizure free without deficit, 27.1% were not seizure free and without deficit, 10.0% were seizure free but with deficit, and 4.3% were not seizure free and with deficit. Future studies with functional and quality-of-life data, particularly for patients who experience seizure recurrence with no deficits (as in group B in the present study) and those who are seizure free with deficits (as in group C) after treatment, are necessary to guide surgical decision-making.

Importance: Pediatric patients with high-grade gliomas have a poor prognosis. The association among the extent of resection, tumor location, and survival in these patients remains unclear., Objective: To ascertain whether gross total resection (GTR) in hemispheric, midline, or infratentorial pediatric high-grade gliomas (pHGGs) is independently associated with survival differences compared with subtotal resection (STR) and biopsy at 1 year and 2 years after tumor resection., Data Sources: PubMed, EBMR, Embase, and MEDLINE were systematically reviewed from inception to June 3, 2022, using the keywords high-grade glioma, pediatric, and surgery. No period or language restrictions were applied., Study Selection: Randomized clinical trials and cohort studies of pHGGs that stratified patients by extent of resection and reported postoperative survival were included for study-level and individual patient data meta-analyses., Data Extraction and Synthesis: Study characteristics and mortality rates were extracted from each article. Relative risk ratios (RRs) were pooled using random-effects models. Individual patient data were evaluated using multivariate mixed-effects Cox proportional hazards regression modeling. The PRISMA reporting guideline was followed, and the study was registered a priori., Main Outcomes and Measures: Hazard ratios (HRs) and RRs were extracted to indicate associations among extent of resection, 1-year and 2-year postoperative mortality, and overall survival., Results: A total of 37 studies with 1387 unique patients with pHGGs were included. In study-level meta-analysis, GTR had a lower mortality risk than STR at 1 year (RR, 0.69; 95% CI, 0.56-0.83; P < .001) and 2 years (RR, 0.74; 95% CI, 0.67-0.83; P < .001) after tumor resection. Subtotal resection was not associated with differential survival compared with biopsy at 1 year (RR, 0.82; 95% CI, 0.66-1.01; P = .07) but had decreased mortality risk at 2 years (RR, 0.89; 95% CI, 0.82-0.97; P = .01). The individual patient data meta-analysis of 27 articles included 427 patients (mean [SD] age at diagnosis, 9.3 [5.9] years), most of whom were boys (169 of 317 [53.3%]), had grade IV tumors (246 of 427 [57.7%]), and/or had tumors that were localized to either the cerebral hemispheres (133 of 349 [38.1%]) or midline structures (132 of 349 [37.8%]). In the multivariate Cox proportional hazards regression model, STR (HR, 1.91; 95% CI, 1.34-2.74; P < .001) and biopsy (HR, 2.10; 95% CI, 1.43-3.07; P < .001) had shortened overall survival compared with GTR but no survival differences between them (HR, 0.91; 95% CI, 0.67-1.24; P = .56). Gross total resection was associated with prolonged survival compared with STR for hemispheric (HR, 0.29; 95% CI, 0.15-0.54; P < .001) and infratentorial (HR, 0.44; 95% CI, 0.24-0.83; P = .01) tumors but not midline tumors (HR, 0.63; 95% CI, 0.34-1.19; P = .16)., Conclusions and Relevance: Results of this study show that, among patients with pHGG, GTR is independently associated with better overall survival compared with STR and biopsy, especially among patients with hemispheric and infratentorial tumors, and support the pursuit of maximal safe resection in the treatment of pHGGs.

This review covers the broad topic of brain surgery in the treatment of pediatric intractable epilepsy. The authors review the latest advancements in the presurgical workup as well as the mandatory tests needed to explore the epilepsy workup in these children. They describe the different types of epilepsy from a surgical standpoint (temporal, extratemporal, multifocal, and hemispheric epilepsies) and various surgical procedures that can be proposed depending on the clinical scenario: lesionectomies, lobectomies, hemispherectomies, neuromodulation, and palliative surgeries. They also describe the key differences of the pediatric patient as compared with the adult patient in such pathologic conditions., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)