OBJECTIVE:Surgery is increasingly recognized as an effective treatment for drug-resistant epilepsy in children but remains under-utilized. Evidence on its safety and benefits in infants 6 months or younger is very limited, leaving early surgical decision-making insufficiently supported. METHODS:A matched cohort study included 23 infants with age-dependent epileptic encephalopathy (ADEE) and structural brain abnormalities who underwent epilepsy surgery within 6 months and 115 matched non-surgical controls. Neurodevelopment was assessed using developmental quotient (DQ). Multivariable linear regression and propensity score matching (PSM) were used to examine the association between surgery and neurodevelopmental outcomes, whereas surgical safety and long-term seizure control were also evaluated. RESULTS:Among the 23 surgical infants, the median age at seizure onset was .27 months, and all had congenital brain malformations. Hemimegalencephaly was the most common etiology (n = 13). The mean age at surgery was 3.5 months. Hemispherotomy was the most commonly performed surgical procedure (n = 14). No perioperative death or permanent severe complications were observed. Four (17.4%) infants developed transient unilateral limb weakness and 13.6% had postoperative hydrocephalus. The rate of Engel class Ia was 82.6% at 1 year postoperatively and 78.2% at a mean follow-up of 44.4 months. At last follow-up, 69.6% of the infants had discontinued anti-seizure medications (ASMs). The DQ scores of the control group showed a continuous decline over time, whereas the surgical group showed a more favorable trajectory. Multivariable linear regression analysis revealed that surgery was significantly associated with a higher DQ (β = 30.2, 95% confidence interval [CI]: 19.5-40.9; p < .001). After 1:1 PSM to control confounding variables, the surgical group had significantly higher DQ scores than the control group in all five neurodevelopmental domains (p < .05). SIGNIFICANCE:Epilepsy surgery performed by an experienced team is safe and feasible for very young infants due to congenital brain malformations, without severe perioperative complications. It provides good long-term seizure control, supports ASM withdrawal, and may protect brain development, potentially stabilizing or even improving neurodevelopment in some infants.

SUMMARY:Direct electrical stimulation (DES) has long been used in patients undergoing epilepsy surgery for two key purposes: functional brain mapping and seizure triggering. In this review, the findings from DES applications to the opercula are synthesized, with the aim of mapping opercular functions and investigating seizure induction. Clinical responses to opercular DES are frequent and diverse, showing a partial segregation with spatial overlap, and exhibiting topographical differences: Emotion is evoked in the most anterior part of frontal operculum, expressive aphasia is the mid-posterior part, followed by oropharyngeal, dysarthria, and gustatory in the central opercula, somatosensory and vestibular in the posterior opercula, and auditory (or rarely visual) in the temporal opercula. No studies have specifically looked at DES-induced seizures from the opercula. Only a small number of studies about insulo-opercular epilepsy have mentioned that DES of the opercula induced seizures. Overall, opercular DES studies converge on the view that the opercula comprise a complex multimodal cortex integrating especially sensorial perception. Each subdivision had a different dominant response to DES, whose organization helps understand the different subgroups of opercular epilepsy.

OBJECTIVE:To summarize the clinical features of collagen type IV alpha 1/2 chain (COL4A)1/2-related epilepsy and the seizure outcomes of patients undergoing epilepsy surgery. METHODS:We retrospectively analyzed the clinical, electroencephalography, and neuroimaging data; genetic characteristics; surgical details; and prognosis of 8 patients (4 boys) treated for COL4A1/2-related epilepsy at Tsinghua University Yuquan Hospital. RESULTS:Two of the probands had COL4A1 variants and six had COL4A2 variants. Four of the variants were de novo. Prenatal abnormalities consisted of intrauterine growth retardation and ventriculomegaly. Three patients had a low birth weight, and one had perinatal retinal hemorrhage. The median age of seizure onset was 8 months, with 75% (6/8) experiencing epilepsy before age 1. Status epilepticus occurred in 38% (3/8) of patients. All patients experienced focal seizures, and 50% (4/8) had focal epileptic spasms. Hemiparesis was observed in 88% (7/8) of patients, and all 8 had developmental delays. The median number of anti-seizure drugs was 5, and all patients had drug-resistant epilepsy. Seven patients had seizures localized to one of the posterior quadrants, consistent with the magnetic resonance imaging features of blurring of the gray-white matter junction and positron emission tomography features of metabolic abnormalities. Other neuroimaging features included bilateral mild white matter abnormalities; unilateral porencephaly near the basal ganglia; ventriculomegaly; focal cerebral calcification; contralateral schizencephaly; and contralateral cortical thickening and cerebellar abnormalities. Six patients underwent unilateral posterior quadrant disconnection, five (83%) of whom had no recurrence for at least 11 months and experienced developmental improvement. No surgical complications were reported. Pathological examination revealed malformations of cortical development in all six surgical cases (five with focal cortical dysplasia [FCD] type Ia and one with FCD type II). SIGNIFICANCE:The results of this case series suggest that early surgical intervention in patients with COL4A1/2-related epilepsy with well-defined epileptogenic zones may improve seizure control and developmental outcomes. PLAIN LANGUAGE SUMMARY:In this case series of eight patients, epilepsy related to variants in the collagen type IV alpha 1/2 chain genes was characterized by drug-resistant localized seizures with an early onset, one-sided muscle weakness, and developmental delay. Neuroimaging revealed various brain abnormalities. Structural abnormalities outside the seizure-onset zone did not appear to affect surgical prognosis. Early surgical intervention in patients with well-defined seizure-onset zones improved seizure control and developmental outcomes.

Both the imbalance of neuronal excitation and inhibition, and the network disorganization may lead to hyperactivity in epilepsy. However, the insufficiency of seizure data poses the challenge of elucidating the network mechanisms behind the frequent and recurrent abnormal discharges. Our study of two extensive intracranial EEG datasets revealed that the seizure onset zone exhibits recurrent synchronous activation of interictal events. These synchronized discharges formed repetitive sequential patterns, indicative of a stable and intricate network structure within the seizure onset zone (SOZ). We hypothesized that the frequent replay of interictal sequential activity shapes the structure of the epileptic network, which in turn supports the occurrence of these discharges. The Hopfield-Kuramoto oscillator network model was employed to characterize the formation and evolution of the epileptic network, encoding the interictal sequential patterns into the network structure using the Hebbian rule. This model successfully replicated patient-specific interictal sequential activity. Dynamic change of the network connections was further introduced to build an adaptive Kuramoto model to simulate the interictal to ictal transition. The Kuramoto oscillator network with adaptive connections (KONWAC) model we proposed essentially combines two scales of Hebbian plasticity, shaping both the stereotyped propagation and the ictal transition in epileptic networks through the interplay of regularity and uncertainty in interictal discharges. ### Competing Interest Statement The authors have declared no competing interest. ### Funding Statement This study was funded by the National Key R&D Program of China(2017YFA0205904). ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: Ethics committee/IRB of Yuquan Hospital of Tsinghua University gave ethical approval for this work. All participants, or their legal guardians, provided informed consent to participate in this study. I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable. Yes All data produced in the present study are available upon reasonable request to the authors.

Focal Cortical Dysplasia (FCD) is a frequent cause of drug-resistant focal epilepsy in children and young adults. The international FCD classifications of 2011 and 2022 have identified several clinico-pathological subtypes, either occurring isolated, i.e., FCD ILAE Type 1 or 2, or in association with a principal cortical lesion, i.e., FCD Type 3. Here, we addressed the DNA methylation signature of a previously described new subtype of FCD 3D occurring in the occipital lobe of very young children and microscopically defined by neuronal cell loss in cortical layer 4. We studied the DNA methylation profile using 850 K BeadChip arrays in a retrospective cohort of 104 patients with FCD 1 A, 2 A, 2B, 3D, TLE without FCD, and 16 postmortem specimens without neurological disorders as controls, operated in China or Germany. DNA was extracted from formalin-fixed paraffin-embedded tissue blocks with microscopically confirmed lesions, and DNA methylation profiles were bioinformatically analyzed with a recently developed deep learning algorithm. Our results revealed a distinct position of FCD 3D in the DNA methylation map of common FCD subtypes, also different from non-FCD epilepsy surgery controls or non-epileptic postmortem controls. Within the FCD 3D cohort, the DNA methylation signature separated three histopathology subtypes, i.e., glial scarring around porencephalic cysts, loss of layer 4, and Rasmussen encephalitis. Differential methylation in FCD 3D with loss of layer 4 mapped explicitly to biological pathways related to neurodegeneration, biogenesis of the extracellular matrix (ECM) components, axon guidance, and regulation of the actin cytoskeleton. Our data suggest that DNA methylation signatures in cortical malformations are not only of diagnostic value but also phenotypically relevant, providing the molecular underpinnings of structural and histopathological features associated with epilepsy. Further studies will be necessary to confirm these results and clarify their functional relevance and epileptogenic potential in these difficult-to-treat children.

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.

Objective:To analyze the characteristics of stereoelectroencephalography (SEEG) in children with drug-resistant epileptic spasms (ES), and to explore the surgical strategy of children with spastic seizure under the guidance of SEEG.Methods:The clinical data of 156 children with ES who were preoperatively evaluated in the Department of Neurosurgery Ward 3, Tsinghua University Yuquan Hospital from January 2014 to December 2021 were retrospectively reviewed.All children were evaluated in the second stage of stereotactic electrode placement after a non-invasive preoperative evaluation.The characteristics of intracranial EEG, surgical strategy and prognosis were analyzed.Results:A total of 19 eligible children were included, involving 13 boys and 6 girls.The age of first onset and surgical age of them ranged 1 month to 4 years, and 2 years to 13 years, respectively.The SEEG was divided into 3 types in children with ES at the onset.Five children were SEEG type A, presenting with the focal seizure discharges at the beginning and a gradual propagation to widespread fast-wave bursts.Ten children were SEEG type B, presenting a focal leading spike followed by diffused fast-wave bursts.Four children were SEEG type C, presenting a diffuse fast wave rhythm onset.Although some electrode discharges appeared slightly " leading", they covered more than one brain region.After focal resection or thermocoagulation, 13/19 patients did not have the onset of seizures, and 5/19 and 8/19 were graded as SEEG type A, and B, respectively.During the intermittent period of SEEG attacks in children with SEEG type A and B, a significant phenomenon of focal epileptic discharge consistent with the onset of the attack was observed, and surgical removal of these areas effectively controlled spastic seizures.Conclusions:Epileptic spasms may be triggered by a focal neocortical discharge.Intracranial EEG showed that the focal seizure onset evolves into spasm or a focal " leading spike" is a good indicator of surgical prognosis.

Researchers have widely acknowledged the therapeutic value of epilepsy surgery for drug-resistant epilepsy. Nonetheless, there is a substantial gap in the surgical treatment for appropriate candidates owing to several factors, particularly in the population of young children. To standardize the protocols of preoperative evaluation and surgery of young children for epilepsy surgery, the China Association Against Epilepsy has appointed an expert task force to standardize the protocols of preoperative evaluation and surgery in pediatric epilepsy patients. It adopted the modified Delphi method and performed two rounds of surveys through an anonymous inquiry among 75 experts from four subgroups including pediatric neurologists, epileptologists, pediatric epilepsy surgeons, and functional neurosurgeons. The survey contents contained: (1) the participants, comprising children aged ≤ 6 years; (2) adopted DRE definition proposed by the International League Against Epilepsy in 2010; and (3) investigated epilepsy surgery, principally referring to curative epilepsy surgeries. The neuromodulation therapies were excluded because of the differences in treatment mechanisms from the above-mentioned surgeries. According to the Delphi process, a consensus was achieved for most aspects by incorporating two rounds of surveys including preoperative assessment, surgical strategies and techniques, and perioperative and long-term postoperative management, despite controversial opinions on certain items. We hope the results of this consensus will improve the level of surgical treatment and management of intractable epilepsy in young children.

Objective:To record stereoelectroencephalography (SEEG) data and to induce cortical electrical stimulation in children with tuberous sclerosis complex (TSC), thus exploring the epileptogenicity of different types of cortical tubers.Methods:The SEEG recording and cortical electrical stimulation data of 50 children with TSC who underwent preoperative evaluation for drug-resistant epilepsy at Epilepsy Center, Tsinghua University Yuquan Hospital from November 2016 to September 2022 were retrospectively analyzed, involving 27 boys and 23 girls with the age of (5.5±3.4) years.According to the results of 3.0T magnetic resonance imaging (3T-MRI) and computed tomography(CT), cortical tubers were classified.The incidences of electroclinical seizures, electrical seizures and seizures induced by cortical electrical stimulation in different types of tubers recorded by SEEG were analyzed, and the differences in the proportion of the above seizures among different types of tubers were compared using the Fisher′ s exact test. Results:A total of 303 cortical tubers were explored using SEEG in 50 patients.The tubers were divided into 6 types, including Type A, B, C, D and E, and focal cortical dysplasia like (FCD-like) type, among which Type E was for the first time proposed in the world.Among these explored tubers, 7 tubers had electrical seizures, and 57 tubers had electroclinical seizures.A total of 64 tubers (21.1%) were epileptogenic.The incidence of epileptogenic tubers in Type A-E and FCD-like type were 3.6%, 1.4%, 19.0%, 77.8%, 77.5%, and 90.0%, respectively. Fisher′ s exact test and Bonferroni correction were performed for pairwise comparisons( P<0.003). There was no significant difference in the incidence of epileptogenic tubes among Type A, B and C. There was significant difference in the incidence of epileptogenic tubes between Type A-C with Type D, Type E and FCD-like type, respectively.There was no significant difference in the incidence of epileptogenic tubes between Type D, Type E and FCD-like like.Electrical stimulation-induced seizures occurred in 36 cortical tubers (11.9%). The positive rate of electrical stimulation seizures in Type A-E and FCD-like type were 0.7%, 1.4%, 4.8%, 44.4%, 45.0%, and 70.0%, respectively.There was significant difference in the positive rate of electrical stimulation seizures between Type A-B and Type D, Type E and FCD-like type, respectively, so as that between Type C versus Type E and FCD-like type.No significant difference in the positive rate of electrical stimulation seizures was found between other pairwise comparisons. Conclusions:This study proposed a new classification of cortical tubers in TSC patients, and Type E is proposed for the first time in the world.SEEG records confirmed great differences in epileptogenicity indifferent types of cortical tubers.Type D, Type E and FCD-like type have higher epileptogenicity, which is of great value for the preoperative evaluation of TSC epilepsy surgery and the placement strategy of SEEG electrodes.

目的 总结起始于运动前区皮层(PMC)癫痫的电临床特征.方法 回顾性分析2014年5月一2021年1月在清华大学玉泉医院行术前评估并经颅内电极脑电图证实起始于运动前区皮层的6例难治性癫痫患者的临床资料,包括症状学、头皮及颅内脑电图、影像学、手术及随访,并将6例患者分成腹侧组、背侧组、内侧面组3组进行比较.结果 腹侧组有3例患者,背侧组有1例患者,内侧面组有2例患者.腹侧组症状较多,包括过度运动、面部强直(撇嘴)、肢体强直、偏转;内侧面组主要为单侧或双侧肢体非对称强直.头皮脑电图主要为前头部(额中央区)放电.3例MRI阴性,3例MRI提示局灶性皮质发育不良.6例患者均行PET-CT检查,2例有代谢减低区.5例患者行致痫区切除,术后病理均为局灶性皮质发育不良(FCD),1例为FCD Ⅰ型,其余均为FCD Ⅱ型.5例手术切除患者术后随访无发作,未手术切除的1例患者先后行热凝和激光治疗,治疗当天发作即消失.结论 运动前区癫痫发作主要为运动症状,越靠近腹侧,症状越复杂,如过度运动、偏转、面肌强直(撇嘴);越近内侧面,症状越简单,以单侧或双侧肢体强直多见.借助于颅内电极,准确定位,局限性皮层切除可以取得较好的预后,另外,对于运动前区沟底FCD,激光等微创技术也有较好的治疗效果.

To develop and validate a model to predict seizure freedom in children undergoing cerebral hemispheric surgery for the treatment of drug‐resistant epilepsy.

背景 癫痫性痉挛(ES)是儿童最常见的发作类型之一.关于ES患儿手术疗效的数据却很少,ES既往被认为术后疗效不佳,此外,关于ES的电临床特征及其对术后疗效的影响尚不清楚.由于痉挛发作症状表现的对称性和癫痫发作起始放电的广泛性,致痫区定位困难,癫痫手术难度大.但最新研究表明,若选择适合手术条件的痉挛发作类型,可以达到和其他局灶性癫痫相似的手术疗效.因此,探讨影响儿童ES手术预后的影响因素,有助于帮助更多的药物难治性痉挛发作患儿达到手术治愈的目的.

背景 眶额皮质(Orbitofrontal cortex,OFC)是大脑皮层中被研究最少的区域之一,而起始于眶额的癫痫发作,特别是起始于眶额不同部位癫痫的电临床特点还不被人们熟悉.其主要原因可能是由于眶额解剖分区的复杂性、病例罕见、头皮电极很难记录到该部位放电而需要进一步颅内电极的植入来明确发作起始.

Objective:To investigate the application value of surface-based morphometry (SBM) in preoperative evaluation of refractory epilepsy.Methods:Clinical data of 51 patients with refractory epilepsy admitted to Epilepsy Center, Yuquan Hospital, Tsinghua University from November 2013 to July 2018 were retrospectively analyzed. Fifty-one patients underwent resection or radiofrequency thermo-coagulation of epileptogenic zone with the outcome of Engel Ⅰ assessed at over 6 months post operation. The three-dimensional high-resolution MRI T1-weighed imaging data were analyzed to calculate the proportion of positive results by using three characteristic values of cortical surfaces, i. e. gyrification index (GI), sqrt-transformed sulcus depth (SQ) and thickness (TH), which were obtained by SBM post-procession.Results:According to imaging characteristics and the scope of resection or radiofrequency thermocoagulation of epileptogenic zone, 51 cases were divided into four groups as follows: 13 cases as MRI negative (MN) group, 27 as MRI positive region coinciding with surgical site (PCS) group, 4 as MRI positive region including surgical site (PIS) group, and 7 as MRI positive region noncompatible with surgical site (PNS) group. PCS group was further divided into three types: 13 cases as mesial temporal lobe epilepsy (PCS-MTLE), 5 as focal cortical dysplasia (PCS-FCD), and 9 as encephalomalacia or malformations of cortical development (PCS-EMCD). With all three characteristic values, the lesion positive proportion were 3/13, 21/27, 2/4 and 1/7 for MN group, PCS group, PIS group and PNS group respectively and 8/13, 4/5 and 9/9 for the three types of PCS patients respectively. Lesion positive proportion by using GI was 4/4 in PIS group and 22/27 in PCS group, which were higher than those obtained by SQ(2/4 and 18/27)and TH(1/4 and 21/27). Lesion positive proportion obtained by SQ in MN was 5/13 and that obtained by TH in PNS group was 3/7, while those by GI in MN group and in PNS group were 3/13 and 2/7 respectively.Conclusions:The preliminary study suggests that for patients with refractory epilepsy who have distinctive lesions, GI could be used to locate the lesions with a relatively high positive proportion and good display on the margins of lesions. For MRI-negative patients, SQ may be a relatively sensitive target for lesion detection. For patients with incongruent indications from MRI and other clinical measures, TH may be the optimal target for lesion detection.

目的 利用比格犬动物实验,探讨采用国产激光消融治疗系统(LS1)和激光消融微创治疗套件(LS—T1)行磁共振引导下激光间质内热疗的安全性和有效性.方法 健康比格犬15只,行3T磁共振引导下激光间质内热疗,功率8W时间50 s,消融过程中磁共振实时扫描监测消融区域温度,系统软件计算最大消融横截面面积.消融后行增强T1磁共振扫描,测量增强毁损灶最大横截面面积.实验结束2周后安乐死比格犬,测量切片最大消融横截面积.对比分析系统计算、术后3D增强T1及切片测量的最大消融横截面积.结果 15只比格犬实验后均正常存活,系统计算最大消融截面面积为115.8±9.9 mm2,增强T1显示最大消融截面面积为115.7±1.0mm2,激光消融2周后组织切片最大消融截面面积为112.7±9.5 mm2.系统计算最大横截面积与增强T1显示最大横截面积差异无统计学意义(P=0.91),系统计算最大横截面积与切片测量最大横截面积差异有统计学意义(P＜0.01),增强T1显示最大横截面积与切片测量最大横截面积之间差异有统计学意义(P＜0.01).结论 应用国产激光消融治疗系统行磁共振引导下激光热疗是安全有效的,系统计算消融面积与消融后增强T1显示消融面积无明显差异.

OBJECTIVE:This study was undertaken to determine whether the vertical parasagittal approach or the lateral peri-insular/peri-Sylvian approach to hemispheric surgery is the superior technique in achieving long-term seizure freedom. METHODS:We conducted a post hoc subgroup analysis of the HOPS (Hemispheric Surgery Outcome Prediction Scale) study, an international, multicenter, retrospective cohort study that identified predictors of seizure freedom through logistic regression modeling. Only patients undergoing vertical parasagittal, lateral peri-insular/peri-Sylvian, or lateral trans-Sylvian hemispherotomy were included in this post hoc analysis. Differences in seizure freedom rates were assessed using a time-to-event method and calculated using the Kaplan-Meier survival method. RESULTS:Data for 672 participants across 23 centers were collected on the specific hemispherotomy approach. Of these, 72 (10.7%) underwent vertical parasagittal hemispherotomy and 600 (89.3%) underwent lateral peri-insular/peri-Sylvian or trans-Sylvian hemispherotomy. Seizure freedom was obtained in 62.4% (95% confidence interval [CI] = 53.5%-70.2%) of the entire cohort at 10-year follow-up. Seizure freedom was 88.8% (95% CI = 78.9%-94.3%) at 1-year follow-up and persisted at 85.5% (95% CI = 74.7%-92.0%) across 5- and 10-year follow-up in the vertical subgroup. In contrast, seizure freedom decreased from 89.2% (95% CI = 86.3%-91.5%) at 1-year to 72.1% (95% CI = 66.9%-76.7%) at 5-year to 57.2% (95% CI = 46.6%-66.4%) at 10-year follow-up for the lateral subgroup. Log-rank test found that vertical hemispherotomy was associated with durable seizure-free progression compared to the lateral approach (p = .01). Patients undergoing the lateral hemispherotomy technique had a shorter time-to-seizure recurrence (hazard ratio = 2.56, 95% CI = 1.08-6.04, p = .03) and increased seizure recurrence odds (odds ratio = 3.67, 95% CI = 1.05-12.86, p = .04) compared to those undergoing the vertical hemispherotomy technique. SIGNIFICANCE:This pilot study demonstrated more durable seizure freedom of the vertical technique compared to lateral hemispherotomy techniques. Further studies, such as prospective expertise-based observational studies or a randomized clinical trial, are required to determine whether a vertical approach to hemispheric surgery provides superior long-term seizure outcomes.

To investigate surgical prognostic factors in order to establish a surgical plan for children with drug-resistant epileptic spasms. We retrospectively analysed 64 children with drug-resistant spasms who were operated on in Beijing; the electroclinical features, surgical procedures, and surgical outcomes of these children were discussed in detail. We divided the seizure-free patients into several groups according to imaging, aetiology, and application of stereo-electroencephalography in order to investigate the extent of the various influencing factors. Fifty-three (82.8%) patients had favourable outcome, and 11 (17.2%) had unfavourable outcome. Based on the univariate analysis, the factors associated with favourable seizure outcome were interictal high γ frequency (χ 2 = 4.161; p = 0.041), concordance between MRI and interictal epileptic discharges (IEDs) (χ 2 = 6.148; p =0.013), and concordance between PET and IEDs (χ 2 = 4.281; p = 0.039). Concordance between MRI and IEDs (OR = 0.083, 95% CI = 0.014-0.483; p = 0.006) and continuous discharges on electrocorticography (OR = 0.109, 95% CI = 0.019-0.639; p = 0.014) were important factors associated with a favourable surgical outcome. Resective surgery is an effective treatment for drug-resistant ES in children. A deeper understanding of the predictors of seizure outcome is beneficial for establishing a standard, one-stage resection procedure for spasms in order to benefit more patients who have not previously considered surgery. We propose a workflow for presurgical evaluation in children with epileptic spasms.

Objective:To summarize the surgical treatment of tuberous sclerosis with refractory epilepsy in 42 children.Methods:Forty-two children who were clinically and pathologically diagnosed with tuberous sclerosis with refractory epilepsy in Yuquan Hospital from April 2008 to March 2019 were selected. All children underwent magnetic resonance imaging, 41 underwent long time video electroencephalogram (EEG) monitoring, and 30 underwent genetic examination. All children underwent detailed preoperative evaluation, and either direct resection or intracranial electrode thermocoagulation was then selected.Results:Among the 42 children, 31 (73.8%) had Engel grade Ⅰ, 6 (14.3%) had grade Ⅱ, 3 (7.1%) had grade Ⅲ, and 2 (4.8%) had grade Ⅳ. Among the 31 children without seizures, 19 had EEG abnormality, among whom 16 cases had discharge, 3 cases had EEG abnormality in 1 year, but returned to normal after 1 year. The EEG of the other 12 children was normal after operation. One case of generalized seizure was free of seizure in 6 years after surgery.Conclusion:The epileptogenic foci of tuberous sclerosis with refractory epilepsy in children are usually single or have major localized areas. Detailed preoperative evaluation can accurately identify the epileptogenic nodules and help obtain a good surgical effect.