婴幼儿难治性癫痫外科治疗探讨

目的总结婴幼儿难治性癫痫的外科治疗效果。方法以2014年10月至2016年10月在广州市妇女儿童医疗中心神经外科住院并接受外科手术治疗的28例婴幼儿难治性癫痫患儿为研究对象,并对其病程、发作类型、影像学及脑电图结果、手术方式、术后癫痫控制情况、并发症进行回顾性分析。结果 28例婴幼儿难治性癫痫患儿均进行严格的术前评估,根据患儿具体情况采用不同的外科手术方式。其中接受局灶性病变切除术者23例,接受功能性大脑半球切除术者4例,接受迷走神经刺激器植入术者1例。术后随访6个月至2年,按Engel预后分级判断疗效,其中Ⅰ级23例(82. 15%),Ⅱ级2例(7. 14%),Ⅲ级1例(3. 57%),死亡2例(7. 14%)。结论外科手术是婴幼儿难治性癫痫的有效治疗方法之一,详尽的术前评估、选择合适的手术方式及娴熟的显微外科技术是治疗成功的关键。     

小儿难治性癫痫

大多数小儿癫痫经过抗癫痫药物（ＡＥＤ）治疗预后好，但仍有一部分适合手术治疗。外科手术已成为小儿难治性癫痫的一种较好的治疗方法。儿童及成人多采用颞叶切除术，婴幼儿多进行胼胝体切除及半球切除术。小儿手术效果比成人好，术后脑功能恢复的可能性大。小儿难治性癫痫应及早手术治疗。     

小儿难治性癫痫的外科治疗

目的 探讨外科手术用于小儿难治性癫痫的适应证、手术方式及随访结果。方法 1999年8月－2001年5月对69例14岁以下难治性癫痫患儿采用手术治疗，并探讨其适应证。结果 随访6个月以上者53例，疗效满意33例，显著改善11例，良好6例，无效3例。总有效率为83.0%(44/53)，显效率62.2%(33/53)。原有智力缺陷改善8例，占24.2%(8/33)，行为障碍恢复13例，占33.3%(13/40)，学习困难提高15例，占40.5%(15/37)。结论 早期外科手术对小儿难治性癫痫是一种有效治疗方式，行为和心理障碍得到改善，脑最佳康复成为可能。     

小儿难治性癫痫

大多数小儿癫痫经过抗癫痫约物治疗预后好，但仍有一部分适合手术治疗。外科手术已成为小儿难治性癫痫的一种较好的治疗方法。儿童及成人多采用颞叶切除术，婴幼儿多进行胼胝体切除及半球切除术。小儿手术效果比成人好，术后脑功能恢复的可能性大。小儿难 性癫痫应及早手术治疗。     

儿童难治性癫痫的外科治疗

目的探讨儿童难治性癫痫的外科治疗方法。方法采用128导视频脑电图进行头皮及皮层电极描记,结合临床表现、颅脑CT、MRI等影象学检查精确定位,并根据不同部位采取不同的术式。结果手术4例,术后无中枢性感染、脑脊液漏等并发症出现,随访6月～10月,1例发作减少75%以上,余3例未再发作;智力均有不同程度的好转。结论多导联的皮层电极视频脑电监测是儿童难治性癫痫术前精确定位的较好的方法,而手术方法的正确选择是手术成败的关键。智力低下不应成为癫痫手术的禁忌症。     

多学科协作模式诊断和治疗儿童药物难治性癫痫研究

目的 探讨儿童难治性癫痫的多学科协作诊断及治疗模式。方法 回顾分析2012年3月至2014年2月上海儿童医学中心儿童癫痫诊治中心收治的43例难治性癫痫患儿的诊断、治疗及随访情况。对难治性癫痫多学科协作诊断治疗模式、术前评估内容及手术指征选择进行分析。结果 43例难治性癫痫患儿均进行多学科的协作诊治,其中男性25例,女性18例;年龄8个月至12岁,平均5岁。29例病例找到病因,包括产前和围生期因素9例,外伤性颅内出血8例,自发性颅内出血3例,皮质发育障碍2例,脑血管畸形2例,结节性硬化2例,Sturge-Weber综合征1例,感染性因素1例,脑室肿瘤术后1例。经多学科讨论建议其中29例继续药物治疗,12例建议采取手术方案,2例建议进行生酮饮食。最终5例病例同意并施行手术,术后随访3~18月,4例（4/5）预后分级为Engel癫痫疗效分级Ⅰ级,1例（20%）预后分级为Ⅲ级。结论 多学科协作模式在儿童难治性癫痫的诊断和治疗中可以发挥积极的作用,为难治性癫痫患儿选择合适的治疗方案提供帮助,经过综合评估后采取的手术治疗能够有效地提高难治性癫痫的治疗效果,生酮饮食治疗目前患者的接受度还不够,有待进一步宣传推广。     

大脑半球离断术治疗儿童难治性癫痫的疗效分析

目的:总结大脑半球离断术治疗儿童半球性病变所致难治性癫痫的临床经验。方法:收集广州市妇女儿童医疗中心神经外科2016年1月至2019年12月采用大脑半球离断术治疗儿童半球性病变所致难治性癫痫29例,其中,男19例,女10例,年龄3个月至6岁,平均年龄20. 52个月,收集相关临床资料如性别、年龄、发作类型、影像学、脑电...     

吡仑帕奈在2-12岁儿童药物难治性癫痫添加治疗中的疗效观察北大核心CSCD

目的观察吡仑帕奈在儿童药物难治性癫痫添加治疗中的临床疗效。方法收集2020年1月至6月在福建医科大学附属协和医院儿科门诊就诊并使用吡仑帕奈添加治疗的药物难治性癫痫患儿临床资料并进行回顾性分析,评估吡仑帕奈在儿童药物难治性癫痫添加治疗中的疗效。吡仑帕奈添加治疗前、后自身对照统计有效率。结果共计纳入2-12岁药物难治性癫痫患儿20例,其中男14例,女6例,年龄(5.82±2.39)岁,发病年龄(3.41±1.96)岁,病程(2.40±1.48)年。20例患儿中癫痫部分性发作1例,复杂部分性发作7例,全面性发作1例,未能明确发作6例,癫痫综合征5例。吡仑帕奈添加治疗3个月后,癫痫无发作者4例,发作频率减少>50%者8例,发作持续时间减少者2例,发作抽搐严重程度减少者1例,发作未见明显改善者4例,发作频率增加者1例。以癫痫发作频率减少≥50%为标准,吡仑帕奈添加治疗有效率为60%。结论第三代抗癫痫发作药物吡仑帕奈在2-12岁儿童药物难治性癫痫添加治疗中效果明显,并且特别适用于年长患儿复杂部分性发作及特定癫痫综合征。     

儿童胚胎发育不良性神经上皮肿瘤

目的 探讨儿童胚胎发育不良性神经上皮肿瘤(dysembryo plastic neroepithelial tumor,DNT)的临床特点、诊断及手术治疗.方法 回顾性分析了2003年1月至2008年5月手术治疗的13例儿童胚胎发育不良性神经上皮肿瘤的临床和病理资料.结果 患儿以难治性癫痫为主要临床表现.本组患儿均接受了显微外科手术治疗,其中全切10例,近全切3例.磁共振成像示皮质或皮质下低T1、高T2信号,病灶边界清晰,无水肿及占位效应.肿瘤病理学检查可见特异性胶质神经元成分.随访13例患儿中2例于术后1年内出现癫痫发作,近全切的1例表现为强直性发作,全切的1例为失神性发作,均与术前发作形式相同,但发作频率明显减少(频率减少≥75%),术后随访癫痫发作控制满意.结论 DNT为良性肿瘤,手术治疗效果好.准确诊断对本病的治疗有重要意义.     

儿童重型β地中海贫血异基因造血干细胞移植术后中枢神经系统并发症的初步分析

目的 探讨重型β地中海贫血患儿异基因造血干细胞移植(allo-HSCT)术后1年内发生中枢神经系统(CNS)并发症的临床特点,提高CNS并发症的诊断和治疗水平.方法 对2012年1月1日至2016年12月31日在深圳市儿童医院血液肿瘤科给予HSCT治疗的58例重型β地中海贫血病例进行回顾性分析.结果 58例患儿中有9例(15.52%)在allo-HSCT术后1年内发生了CNS并发症,其中大脑后部可逆性脑病综合征4例,病毒性脑炎2例,难治性癫痫、高热惊厥和低镁抽搐各1例.9例CNS并发症病例中,男性供者2例,女性供者7例;4例为HLA全相合,5例为HLA不全相合;使用糖皮质激素5例,未用4例.本研究无死亡病例,所有患儿均脱离地中海贫血输血状态;对上述危险因素进行单因素分析,P均<0.05.1例出现神经系统后遗症(难治性癫痫),其余痊愈.结论 女性供者、HLA不全相合和糖皮质激素的使用可能为重型β地中海贫血患儿allo-HSCT术后发生CNS并发症的高危因素.早期诊断并积极治疗CNS并发症,可减少后遗症的发生并降低病死率,有效改善患儿的生存质量.     

儿童后象限脑皮质发育障碍致难治性癫癎的手术治疗

目的探讨儿童后象限脑皮质发育障碍致难治性癫癎的临床特点、手术策略及疗效。方法分析14例后象限皮质发育不良(PQD)致难治性癫癎患儿的症状学特征及术前评估结果,总结视频脑电图检查、术中监测的定位价值及颞顶枕叶离断术的适应症和优缺点。结果 14例PQD患儿的癫癎发作有多种发作形式,但以痉挛发作最常见。2例皮质发育不良涉及中央区。14例患儿行颞顶枕叶离断手术,术后13例无发作,1例发作减少50%。结论颞顶枕叶离断术是儿童后象限皮质发育障碍所致难治性癫癎外科手术治疗较安全的一种术式,并获得了满意的癫癎控制效果。     

Seizure-free and neuropsychological outcomes after temporal lobectomy with amygdalohippocampectomy in pediatric patients with hippocampal sclerosis

Object Temporal lobe epilepsy is an uncommon clinical syndrome in the pediatric population. The most common underlying pathologies include low-grade gliomas, cortical dysplasia, and, less commonly, hippocampal sclerosis (HS). There is a paucity of data on neuropsychological and seizure-free outcomes in these patients after temporal lobectomy. In this study, the authors reviewed their seizure-free and neuropsychological outcomes after temporal lobectomy for pediatric HS. Methods The authors retrospectively reviewed the medical records of pediatric patients with HS who underwent anterior temporal lobectomy and amygdalohippocampectomy between 1998 and 2011 at the Cleveland Clinic. Results of neuropsychological assessment before and after surgery and seizure-free outcome at last follow-up were obtained. Results Forty-five patients met the inclusion criteria. Thirty-four (76%) patients had pathology of HS alone and 10 (22%) had HS and cortical dysplasia. The mean duration of follow-up was 60.2 months. Eighty-four percent of patients had postoperative Engel Class I or II outcomes. Neuropsychological outcomes remained unchanged or minimally improved postoperatively. Conclusions Seizure-free outcomes in pediatric HS are similar to historical rates in adult HS. Neuropsychological assessments remain stable after temporal lobectomy. Standard temporal lobectomy should be considered in pediatric patients with medically intractable epilepsy secondary to HS.     

药物难治性颞叶癫患儿手术效果及其影响因素:附76例分析

目的总结药物难治性颞叶癫(MITLE)患儿的手术效果,探讨影响其手术预后的因素。方法以2005年6月—2009年3月进行手术,并至少随访1年的76例MITLE患儿为研究对象,应用多因素Logistic回归分析判断影响MITLE患儿手术效果的独立预测因素。结果 MITLE患儿手术后,根据Engel分级法,46例(60.5%)达到Ⅰ级,22例(28.9%)Ⅱ～Ⅲ级。多因素Logistic回归分析提示,首发年龄(P=0.034,OR=4.734,95%CI:1.121～17.067)、发作类型(P=0.033,OR=3.610,95%CI:1.111～11.732)是影响MITLE患儿手术效果的独立预测因素;既往病因、病程、MRI检查结果、发作频率、手术侧别、发作间期放电范围、IQ、服用抗癫药物对手术效果无独立预测作用。结论手术治疗儿童MITLE有效。首发年龄小、继发全面性发作的MITLE患儿手术治疗效果较差。     

Pathology and neuroimaging in pediatric temporal lobectomy for intractable epilepsy.

OBJECTIVES: Firstly, to study the pathology at surgery in children undergoing temporal lobectomy for intractable partial epilepsy. Secondly, to compare neuroimaging techniques (CT, MRI) in the preoperative detection of pathology. Lastly, to examine the surgical outcome in children. METHODS: Forty-two pediatric patients undergoing temporal lobectomy for intractable epilepsy at the Comprehensive Epilepsy Program at the University of Alberta Hospital between the years 1988-1998 were studied. Patients had extensive preoperative investigations including CT and MRI. The pathology at surgery was reviewed and compared to preoperative neuroimaging. Charts were reviewed to determine surgical outcome. RESULTS: Brain tumors were the most common pathology, found in 13/42 patients. Mesial temporal sclerosis (MTS) was found in 8 patients and dual pathology in an additional 5. Focal cortical dysplasia (FCD) was seen in 4 patients, 1 patient had a porencephalic cyst and 4 patients had tubers of tuberous sclerosis. Seven patients had no specific pathology detected. MRI was clearly more sensitive than CT in the detection of pathology. MRI was abnormal in 27/42 cases (64%), while CT scan was found to be abnormal in only 12/39 (31%). Surgical outcome was excellent, with 34/42 patients (80%) having an Engel class I outcome. One patient had significant improvement with an Engel class II outcome, 3 (7%) had little improvement (Engel class III) and 4 (10%) were unchanged (Engel class IV). Three patients (7%) had surgical complications. CONCLUSIONS: A wide variety of developmental pathology is seen following temporal lobectomy for intractable epilepsy of childhood. Brain tumors, FCD and MTS are common. MRI is superior to CT in the detection of pathology, which may be subtle in children. Surgical outcome is excellent, with most children being seizure free and few complications being seen.     

Neurosurgical management of frontal lobe epilepsy in children

Object Pediatric frontal lobe epilepsy (FLE) remains a challenging condition for neurosurgeons and epileptologists to manage. Postoperative seizure outcomes remain far inferior to those observed in temporal lobe epilepsies, possibly due to inherent difficulties in delineating and subsequently completely resecting responsible epileptogenic regions. In this study, the authors review their institutional experience with the surgical management of FLE and attempt to find predictors that may help to improve seizure outcome in this population. Methods All surgically treated cases of intractable FLE from 1990 to 2008 were reviewed. Demographic information, preoperative and intraoperative imaging and electrophysiological investigations, and follow-up seizure outcome were assessed. Inferential statistics were performed to look for potential predictors of seizure outcome. Results Forty patients (20 male, 20 female) underwent surgical management of FLE during the study period. Patients were an average of 5.6 years old at the time of FLE onset and 11.7 years at the time of surgery; patients were followed for a mean of 40.25 months. Most patients displayed typical FLE semiology. Twenty-eight patients had discrete lesions identified on MRI. Eight patients underwent 2 operations. Cortical dysplasia was the most common pathological diagnosis. Engel Class I outcome was obtained in 25 patients (62.5%), while Engel Class II outcome was observed in 5 patients (12.5%). No statistically significant predictors of outcome were found. Conclusions Control of FLE remains a challenging problem. Favorable seizure outcome, obtained in 62% of patients in this series, is still not as easily obtained in FLE as it is in temporal lobe epilepsy. While no statistically significant predictors of seizure outcome were revealed in this study, patients with FLE continue to require extensive workup and investigation to arrive at a logical and comprehensive neurosurgical treatment plan. Future studies with improved neuroimaging and advanced invasive monitoring strategies may well help define factors for success in this form of epilepsy that is difficult to control.     

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??Abstracts?? Objective To study the diagnosis and treatment by the model of multidisciplinary team treatment for intractable epilepsy in children. Methods We retrospectively reviewed the clinical data of 43 pediatric cases of intractable epilepsy in our institution from 2012 to 2014??aged from 8 months to 12 years?? with an average age of 5 years. Results Totally 29 cases could be found the related factors of epilepsy in the past history?? including prenatal and perinatal factors in 9 cases?? 8 cases of traumatic intracranial hemorrhage?? 3 cases of spontaneous intracranial hemorrhage?? 2 cases of disorders of cortical development?? 2 cases of vascular malformation?? 2 cases of tuberous sclerosis??1 cases of Sturge-Weber syndrome?? 1 cases of infection?? and intraventricular tumor after operation in 1 case. Five cases underwent surgery and the follow-up was from 3 to 18 months ??mean 10 months???? 4 of 5 ??80%?? achieved seizure freedom ??Engel ???? and 1 ??20%?? demonstrated worthwhile improvement ??Engel ??. Conclusions Multidisciplinary team treatment is an effective model in diagnosis and treatment of childhood intractable epilepsy??Surgical intervention in children with intractable seizures can yield favorable seizure outcome.     

Clinical and electroencephalographic features of infantile spasms associated with malformations of cortical development

The aim of this study was to reveal the clinical and encephalographic (EEG) features of infantile spasms (IS) with malformations of cortical development (MCDs). The clinical features, EEG findings, neuroimaging studies and outcomes of various therapeutic modalities for 27 patients with IS and MCDs were reviewed. Background activities of EEG on the MCDs, i.e. asymmetric hypsarrhythmia, localized persistent polymorphic slowing, asymmetric slowing and diffuse fast activities, were shown in 22, 15, 9 and 2 patients, respectively. Partial epileptiform discharges such as localized paroxysmal fast activities, spindle-shaped fast activities and subclinical seizures were shown in 15, 8 and 10 patients, respectively, and the lateralized prominence of generalized paroxysmal fast activities and generalized sharp and wave discharges in 4 and 5 patients, respectively. MCDs were suspected in 5 patients, as revealed by EEG and/or functional neuroimagings without distinct magnetic resonance imaging lesions, and confirmed by pathologic findings. Of the 11 patients treated with surgical resection, 8 became seizure free. EEG features can be very useful in the identification of underlying cortical dysgenesis in patients with IS. However, in one patient who underwent epilepsy surgery in early infancy, we observed easy bleeding and difficult hemostasis from friable vascular and parenchymal tissues. Various EEG features can be very useful in screening underlying MCDs. In addition, epilepsy surgery can be an effective therapeutic modality in many patients with otherwise medically intractable IS with MCDs. However, surgical intervention in extremely young infants should be performed with caution.     

Seizure and cognitive outcomes of epilepsy surgery in infancy and early childhood

AimsTo investigate seizure and developmental outcomes following epilepsy surgery in very young children and determine their predictive factors.MethodsWe retrospectively reviewed the clinical data, surgical variables, and outcomes of 30 children under 3 years of age that underwent resection for refractory focal epilepsy in our institution in 2001–2011.ResultsSeizure onset was in the first year of life in 27 (90%) cases and mean age at surgery was 20 months (range 5–33.6). Pathology consisted of cortical malformations in 24 (80%) cases, glioneuronal tumour and infarction with or without cortical dysplasia in three (10%) cases each. Morbidity was comparable with older paediatric cohorts. At 1–11.6 year follow-up (mean 4.1) 21 of 30 (70%) children achieved seizure freedom (Engel I), six (20%) demonstrated worthwhile improvement (Engel II/III) and three (10%) did not benefit from surgery (Engel IV). Intralobar lesionectomy more often resulted in seizure freedom than multilobar or hemispheric surgery. The abundance of non-regional interictal and ictal EEG findings did not preclude seizure freedom. Presurgical developmental impairment was established in 25 of 28 (89%) children; its severity correlated with longer epilepsy duration and determined postoperative developmental outcome. Developmental progress was established in 26 out of 28 (93%) children following surgery, showing stabilized trajectories rather than catch-up.ConclusionsResective surgery in very young children is safe and effective in terms of seizure control and developmental progress. Our findings underline the importance of early intervention in order to timely stop seizures and their deleterious effects on the developing brain.     

Role of surgery in pediatric epilepsy

Twenty five percent of patients with intractable epilepsy have surgically remediable epilepsy syndromes. This article reviews the treatment paradigm for pediatric epilepsy and also the indications, methods, and surgical options for the subgroup of patients with surgically remediable epileptic disorders based on our experience in the management of these children. The article also discusses the rationale for offering surgery and the timing of surgery in these patients. The study of surgically remediable epilepsy can best be divided into focal, sub hemispheric, hemispheric and multifocal epileptic syndromes. These syndromes have both acquired and congenital etiologies and can be treated by resective or disconnective surgery. The surgical management of these conditions (with the exception of multifocal epilepsy) provides Engel's Class 1 outcome(complete seizure freedom) in approximately 80% of children. The consequences of seizure freedom leads to a marked improvement in the quality of life of these children.The benefits to society, of allowing a child to grow to adulthood with normal cognition to earn a livelihood and contribute actively to society, cannot be understated.