胚胎发育不良性神经上皮肿瘤致癫痫的手术治疗

目的 探讨胚胎发育不良性神经上皮肿瘤(DNT)致癫痫患者的手术策略.方法 回顾性分析自2008年1月至2013年8月首都医科大学宣武医院神经外科手术治疗14例DNT患者的经验,术前常规通过MRI及视频脑电图(VEEG)等检查对肿瘤及致痫灶进行评估,术中行皮层脑电监测(EcoG)对致痫灶定位,手术方式:肿瘤病灶加致痫灶切除.结果 14例患者肿瘤均得到全切,手术后病理证实为DNT,未行放疗和化疗,无肿瘤复发及恶性转化.10例患者术后癫痫发作得到完全控制(Engel Ⅰ级),4例患者术后稀少发作(EngelⅡ级).结论 DNT是低级别神经元～胶质细胞肿瘤,常见于青少年,多伴药物难治性癫痫,除切除肿瘤外,积极处理肿瘤外致痫灶可以很好地控制癫痫.     

胚胎发育不良性神经上皮肿瘤的手术治疗

目的探讨胚胎发育不良性神经上皮肿瘤（DNT）的手术方法及其疗效。方法 2007年6月至2013年6月显微手术治疗DNT患者17例,手术方式采用在皮层脑电图指引下行肿瘤加致痫灶扩大切除术。术后至少随访1年,根据Engel预后分级评估手术效果。结果所有患者肿瘤均得到全切,病理学均证实为DNT,其中10例合并局灶性脑皮质发育不良（FCD）,术后未出现永久性功能障碍。患者术后随访1~4.5年,无肿瘤复发,17例中无癫痫发作（EngelⅠ级）的13例（76.5%）,仍有少量癫痫发作（EngelⅡ级）4例（23.5%）。结论 DNT是一种少见的以癫痫为主要临床表现的良性肿瘤,常伴有FCD,多发展为药物难治性癫痫,在皮层脑电图监测下行肿瘤加致痫灶扩大切除可以获得良好的癫痫控制率。     

影响局灶性皮质发育不良所致难治性癫痫手术疗效的相关因素分析

目的分析影响局灶性皮质发育不良(FCD)所致药物难治性癫痫手术疗效的相关因素。方法收集2015年9月至2018年7月在中国科学技术大学附属第一医院神经外科接受手术治疗并经病理确诊为FCD的50例药物难治性癫痫患者的临床资料,包括病史、发作频率、病灶位置、视频脑电结果、头颅MRI表现、术前服药种类、手术切除范围、术后早期癫痫发作情况,采用Engel分级评估患者手术疗效,并将其分为预后良好组和预后不良组。运用统计学方法分析FCD所致难治性癫痫的手术疗效相关影响因素。结果共有50例患者接受手术,其中致痫灶位于颞叶31例,额叶14例,顶叶2例,枕叶2例,颞枕叶1例,手术完整切除致痫灶44例,未完整切除6例,术后病理分型为FCDⅠ、Ⅱ、Ⅲ型分别有19例、17例、14例,术后随访时间为12～46个月,平均(20.7±7.9)个月,术后EngelⅠ级34例、EngelⅡ级3例、EngelⅢ级5例、EngelⅣ级8例,预后良好组34例,预后不良组16例。单因素分析显示病程≥10年、术后出现早期癫痫发作、致痫灶未完全切除者手术疗效较差(均P0.05)。二分类Logistic回归分析结果显示,致痫灶是否完全切除和术后是否出现早期癫痫发作是FCD所致难治性癫痫手术疗效的独立影响因素(均P0.05)。结论致痫灶是否完整切除和术后是否出现早期癫痫发作与FCD致难治性癫痫的手术疗效密切相关。患者的病程也是FCD致难治性癫痫手术疗效的重要预测因素。     

手术治疗结节性硬化的疗效观察

目的 探讨手术治疗结节性硬化引起癫痫的疗效.方法 回顾性分析6例患者的临床资料并系统随访18-30个月,综合评估3例导航下行致痫结节切除+皮层低功率热灼术;2例皮层电极植入后,经过监测,行致痫结节切除+皮层热灼术;1例行胼胝体前段切开术的疗效.结果 癫痫发作完全消失2例(Engel Ⅰ级),偶有发作1例(Engel Ⅱ级),发作减轻3例(Engel Ⅲ级).结论 手术可以有效地控制或者减轻结节性硬化引起的癫痫发作.     

立体定向脑电图在MRI阴性难治性癫痫中的应用价值

目的 探讨立体定向脑电图(SEEG)在治疗MRI阴性难治性癫痫患者术前评估的应用价值。方法 回顾性分析解放军第九八八中心医院神经外科2018年1月—9月收治的7例MRI阴性，在SEEG指导下手术切除致痫灶患者的临床资料;采用Engel分级评估术后癫痫控制效果。结果 7例患者共植入63根SEEG电极，平均每例患者植入9根电极，触点数共548个;植入顺利，未出现电极折断或移位、感染、脑脊液漏等并发症;所有患者均接受可疑致痫灶射频热凝术及随后的致痫灶切除术。术后随访12～18个月，患者的发作频率平均减少92. 8%(P=0. 00)，其中5例患者(71. 4%)发作控制达EngelⅠ级，2例(28. 6%)患者发作控制达EngelⅡ级。结论 SEEG监测范围广，可以准确定位MRI阴性癫痫的发作起始;在MRI阴性癫痫患者术前评估中安全可行，可以有效指引手术切除致痫区，控制癫痫发作。     

胚胎发育不良性神经上皮肿瘤继发癫痫的手术疗效及预后影响因素分析

目的探讨外科手术治疗胚胎发育不良性神经上皮肿瘤(DNT)继发癫痫的临床疗效及影响预后的相关因素。方法回顾性分析北京丰台医院癫痫中心2009年11月—2017年11月收治的37例病理证实为DNT的癫痫患者的临床资料。根据Engel分级评估患者的预后;并分析患者的性别、发病年龄、病程、癫痫发作类型、病变部位、影像学特点、切除方式等因素与预后的关系。结果本组患者术后平均随访(55. 4±28. 0)个月,其中33例患者(89. 2%)术后癫痫发作完全消失(EngelⅠ级)。分析结果显示,病程越短、病灶切除完全的患者预后越好(均P 0. 05)。结论 DNT所致癫痫的手术治疗效果较好,早期、完全切除病变是影响手术预后的重要因素。     

唤醒麻醉和术中功能定位切除临近语言区致痫灶(附3例报告)

目的 探讨切除功能区致痫灶的手术策略及术后疗效.方法 在唤醒麻醉下应用术中皮层电刺激确定语言功能区,根据功能区边界选择处理致痫灶.评价患者的功能结果及癫痫控制程度.结果 3例患者术后随访,均未出现语言障碍,癫痫发作完全控制,符合Engel分级Ⅰ级.致痫灶全切2例,近全切+致痫皮层热灼1例.结论 借助唤醒麻醉进行术中皮质电刺激确定语言功能区准确、安全、可靠.唤醒麻醉下进行术中皮质电刺激结合影像学资料、借助颅内皮层电极的皮质电刺激进行功能区定位,能够最大可能地切除致痫灶而最小化功能区的损害.     

小儿难治性癫痫的手术治疗及病理结果分析

目的 探讨小儿药物难治性癫痫的外科治疗时机及病理结果与疗效的关系.方法 对71例小儿难治性癫痫进行手术治疗,术后至少随访1年,根据Engel预后分级评估手术效果.结果 术后随访的难治性癫痫患儿中,无癫痫发作(Engel Ⅰ级)的45例(63％),其中全切除32例,次全切除13例,仍有癫痫发作(EngelⅡ～Ⅳ级)的26例(37％),效果与致痫灶切除程度密切相关(P＜0.05).病理结果:局灶性脑皮质发育不良31例,低级别肿瘤18例,瘢痕脑回13例,小脑回畸形6例,结节性硬化3例,其中双重病理9例.无手术死亡和永久性并发症.结论 早期外科手术对小儿难治性癫痫是一种安全有效的治疗方式,其病理类型与手术结果存在一定相关性.     

幕上与癫痫有关的海绵状血管瘤的显微外科治疗

目的 探讨幕上表现于癫痫的颅内海绵状血管瘤外科治疗方法和效果.方法 25例幕上表现于癫痫的颅内海绵状血管瘤患者,根据术前影像学、脑电图表现与运动区的关系分为A、B二组.手术在皮层脑电图(ECoG)监测下进行,包括单纯切除血管瘤、切除血管瘤和切除含铁血黄素层,以及辅助部分皮层痫灶切除、皮层热灼或软膜下横切等.结果 本组海绵状血管瘤均令切除,2例术后有一过性轻度肢体功能障碍,无手术死亡.随访10个月-4.5年,A组9例,Engel Ⅰ级6例;Engel Ⅱ B级2例;Engel Ⅲ级1例.B组16例,均无发作.结论 幕上与癫痫有关的海绵状血管瘤在ECoG监测下做病灶和痫灶切除,是控制术后癫痫发作的有效手段;运动区和附近海绵状血管瘤由于手术没有完全切除致痫灶可能是术后癫痫控制率差的原因.     

局灶性皮质发育不良所致癫痫手术预后的影响因素分析

目的探讨影响局灶性皮质发育不良(FCD)致癫痫患者手术预后的相关因素。方法回顾性分析42例FCD致癫痫手术治疗患者的临床资料,FCD均经术后病理证实。术后随访12~36个月,进行Engel分级评定患者的预后;应用Logistic回归分析探讨影响手术预后的相关因素。结果术后EngelⅠ级者27例(64.3%),Ⅱ级者6例(14.3%),Ⅲ级者6例(14.3%),Ⅳ级者3例(7.1%)。Logistic回归分析显示,MEG定位致痫灶与手术范围不一致[OR值6.063,95%可信区间(CI)1.168~31.482)]和致痫灶未完全切除(OR值7.739,95%CI 1.447~41.398)是影响FCD致癫痫患者手术预后的独立危险因素(均P0.05)。而性别、年龄、病程、有无高热惊厥史、致痫灶位置、头颅MRI是否阳性、病理分型对手术预后的影响均无统计学意义。结论发作间期MEG定位致痫灶与手术范围的一致性,以及致痫灶完全切除与FCD致癫痫患者的手术预后良好有关。     

儿童和青少年颞叶癫癎的术后长期疗效观察

目的总结儿童和青少年颞叶癫癎手术后的长期疗效和生活质量。方法回顾性分析31例儿童和青少年颞叶癫癎病人的临床资料,行标准前颞叶切除术26例,扩大前颞叶切除术2例,前颞叶切除术＋软化灶切除术2例,颞后顶下致癎灶切除术1例。评估术后癫癎发作改善情况及认知、生活质量等。结果术后随访5年以上,其中癫癎发作消失、达到EngelⅠ级26例,EngelⅡ级1例,EngelⅢ级2例,EngelⅣ级2例。术后病理示：海马组织硬化13例,皮质发育异常伴胶质增生9例,皮质发育异常伴海马组织硬化6例,神经元胶质肿瘤2例,继发性瘢痕脑回形成1例。术后并发症多数可恢复。结论儿童和青少年颞叶癫癎术后长期疗效良好,生活质量提高。     

Postoperative routine EEG correlates with long-term seizure outcome after epilepsy surgery.

We investigated the correlation of interictal epileptiform discharges (IED) in routine EEG 6 and 24 months after epilepsy surgery with regard to long-term seizure outcome. In 148 patients (74% temporal lobe epilepsy (TLE), 26% extratemporal epilepsy) EEG results (IED present or absent) were correlated with the postoperative outcome using the Engel classification 6 and 24 months after resection (PO6m and PO2y, respectively). Self-evaluation was conducted 3 and 5 years after resection (PO3y and PO5y, respectively). Ninety-one patients (62%) were seizure-free 5 years after resection; 88% of them showed no IED in PO6m. Twenty-eight patients (19%) displayed IED in routine EEG 6 months after resection; 61% of them had recurrent seizures at PO5y, whereas of 120 patients without IED only 33% had recurrent seizures at PO5y; p=0.01. Absence of IED in PO6m and PO2y correlated with good outcome: 71% without IED remained seizure-free, whereas only 25% with IED at PO6m and PO2y remained seizure-free; p=0.001. Seizure-free patients (Engel 1) and patients with less favourable outcome (Engel 3-4) at PO6m and PO2y rarely changed categories of outcome during the following years (p<0.001). Half of the patients with favourable seizure reduction (Engel 2) changed to seizure-free (Engel 1) or to a worse outcome category (Engel 3-4). Postoperative routine EEG is a good prognostic instrument for the prediction of long-term seizure outcome, especially for TLE. It predicts the running up and down of fits in patients with rare seizures (Engel 2).     

小儿难治性癫痫综合征的外科治疗

目的总结外科手术治疗27例小儿难治性癫痫综合征病人的经验。方法术前评估和术中脑电检查显示为局灶性改变者行致痫灶切除、脑叶切除或多软膜下横纤维切断(MST)。检查提示一侧半球为主多灶性改变者,术中行多脑叶切除联合MST或(和)胼胝体部分切开。结果本组随访1-8年,平均4.5年。27例病人中有14例获得Ⅰ级(Engel分级),8例获得Ⅱ级,3例为Ⅲ级。平均智商(IQ)从术前的61.4分提高到75.0分,癫痫病程和术前药物难治的时间越短,智商改善越明显。本组4例病人出现暂时性的并发症,无手术死亡。结论对小儿难治性癫痫综合征进行早期外科干预,可以有效地控制癫痫发作、改善智力损害和避免生活残疾。     

小儿顽固性癫痫的外科治疗

目的回顾性分析2002年6月至2007年6月间,在我院接受手术治疗的142例儿童顽固性癫痫患者的手术经验。方法术前评估和术中脑电检查显示为局灶性改变者行致痫灶切除或脑叶切除和（或）多软膜下横纤维切断（MST）。检查提示一侧半球为主多灶性改变者,术中行多脑叶切除联合MST和/或胼胝体部分切开。结果本组随访1～5年,平均3年。142例中有65例获得I级（Engel分级）,34例获得Ⅱ级,25例为Ⅲ级预后。有效率87.3%,效果优良率为69.7%。平均总智商（FIQ）从术前的65.4分提高到80.9分,癫痫病程和术前药物难治的时间越短,智商改善越明显。本组有16例出现暂时性的并发症,无手术死亡。结论对小儿顽固性癫痫患者,只要病例选择适当,手术方式合理,其手术效果是令人满意的,对这类患儿的早期外科干预,不仅可以有效地控制癫痫发作,而且可改善已有的智力损害和减少生活残疾。     

Supratentorielle Kavernome und epileptische Anfälle

For cavernous haemangiomas, it is the aim of surgical treatment to control epilepsy and eliminate potential sources of intracerebral haematomas. In the following investigation, it was attempted to find indicators for seizure freedom after surgery. Success of therapy was assessed according to three patterns of classification. Thirty patients underwent tailored resection based on findings from preoperative investigations and intraoperative electrocorticography. Follow-up averaged 4 years. Lesionectomy, extended lesionectomy, and modified lobe resection were carried out in 13, 11, and six patients, respectively. For all procedures, including microsurgical lesionectomy, the firm gliotic layer unequivocally differed in colour and consistency from normal brain and was removed. Further tissue resection was carried out only if the electrocortical course suggested persistent spike activity around the resection cavity or if presurgical MRI evaluation (e.g. hippocampal atrophy) or electrophysiology also pointed to pathology distant from the lesion. Of the patients, 53.3% became completely seizure-free (Engel I), and one additional patient had only occasional isolated auras. Dramatic reductions in seizure frequency and severity were exhibited by 26.7%. Outcome in respect to seizure control was not associated with resection procedure, comparing pure lesionectomy with lesionectomy plus cortectomy. In the group of patients with epilepsy surgery, those with hippocampectomy had significantly better outcome than those without. Important prognostic factors were early operation after seizure manifestation (91.7% operated upon within 2 years of seizure onset became seizure-free). Another prognostic factor was unifocal seizure onset (bilateral or multifocal seizure onset was found in care of the ten patients with unfavourable outcome). None of the four patients harbouring multiple cavernomas became seizure-free after resection of one lesion, which was believed to be mostly attributable to the epileptic focus that was removed.     

结节性硬化症致难治性癫痫的手术处理

目的探讨手术治疗结节性硬化症所致难治性癫痫的适应证、方法和预后。方法回顾性分析了我院从2002年1月至2006年6月间,采用外科手术治疗10例结节性硬化症伴难治性癫痫的经验。术前评估和术中脑电检查显示为局灶性改变者行致痫灶切除、脑叶切除或多软膜下横纤维切断（MST）。检查提示一侧半球为主多灶性或弥漫性改变者,术中行多脑叶切除为主的联合手术方式。结果本组随访1～4年,平均2.5年。10例患者中有6例获得I级（Engel分级）,3例获得Ⅱ级,1例为III级。平均智商（IQ）从术前的59.6分提高到74.2分。本组3例患者出现暂时性的并发症,无手术死亡。结论外科手术是治疗结节性硬化症所致难治性癫痫的良好手段。表现为单致痫灶的患者预后较佳,虽有多结节,但临床资料、脑电图、影像学资料吻合的多致痫灶亦具有较好的手术效果。     

儿童难治性癫痫的外科治疗

目的 分析儿童难治性癫痫的病因、外科手术的适应证和预后.方法 回顾性分析采用外科手术治疗342例儿童癫痫的经验.结果 皮质发育障碍是本组儿童难治性癫痫的最重要病因,占18.4%.药物难治性癫痫综合征占13.5%.术后疗效Engel Ⅰ级158例,Ⅱ级76例,Ⅲ级61例,Ⅳ级47例.平均智商(1Q)从术前的69.2分提高到79.8分.术前显示智力低下者术后亦有明显改善.结论 早期外科干预能有效地控制癫痫发作、改善智力损害,智力低下不应是外科治疗的禁忌证.同时,正确认识儿童难治性癫痫的病凶和病理埘儿童癫痫的外科治疗具有重要作用.     

视频脑电图与发作期SPECT对痫灶定位的对比研究

目的 探讨视频脑电图(VEEG)、发作期SPECT在术前痫灶定位中的作用.方法 回顾性对比分析48例难治性癫痫患者术前所行长程VEEG与发作期SPECT定位的情况,比较两者定位的一致性.结果 发作期SPECT定位阳性率为94%,VEEG为96%.两者完全一致占46%,部分一致占29%,完全不一致占25%.阳性率及定侧率差异无统计学意义(P＞0.05),局灶定位差异有统计学意义(P＜0.01).随访结果显示48例患者中Engel Ⅰ级27例,Ⅱ级10例,Ⅲ级8例,Ⅳ级3例;其中EngelⅠ～Ⅱ级的37例中33例来自两者定位一致的患者.结论 发作期SPECT与VEEG检查有较高的一致性,两者联合应用优势互补,可提高定位准确性,获得良好的手术疗效.     

Seizure outcomes of lesionectomy in pediatric lesional epilepsy with brain tumor – Single institute experience

Purpose

To determine the clinical characteristics, surgical strategy, and outcome in pediatric lesional epilepsy patients younger than 5 years of age undergoing surgery in a single institute.

Method

Retrospective data were collected and analyzed on patients younger than 5 years of age who underwent lesionectomy for lesional epilepsy at single institute from January 2001 to August 2010. Fourteen pediatric lesional epilepsy patients were enrolled in this study. Engel classification was used to classify seizure outcome.

Results

Median preoperative seizure period was 1 month (range, 1–21). Median post-operative follow up period was 35 months (range 13–84). Ten patients who underwent gross total resection of tumor showed Engel class Ia seizure outcome without any antiepileptic drug (AED). Subtotal resection was performed in four patients to avoid eloquent area injury. Two of these four patients with subtotal removal became seizure-free (Engel class Ia) without AED, while two were in Engel class Ib with AED medication. There was no significant surgical morbidity or mortality.

Conclusion

Lesionectomy in children younger than 5 years of age is relatively safe and effective in controlling seizures. Short preoperative seizure periods and total removal of tumor might be associated with good outcome. Therefore, early and complete lesionectomy alone may help allow for seizure freedom and optimal brain development in pediatric patients.     

Surgery for epilepsy in children with dysembryoplastic neuroepithelial tumor: clinical spectrum,seizure outcome,neuroradiology, and pathology

Introduction  Dysembryoplastic neuroepithelial tumors (DNTs) were first described by Daumas-Duport et al. in 1988 as a typically cortical tumor affecting young patients with long-standing, drug-resistant epilepsy. Methods  We reviewed the medical records of 29 patients with DNT between 1994 and 2007 at Hacettepe University Children’s Hospital retrospectively; age at the time of surgery, age at seizure onset, electroencephalography (EEG), MRI, medical treatment, surgical procedure, seizure outcome, and pathological findings were documented. Results  Male to female ratio was 15/14. Age at the time of evaluation ranged 4–24 years. Twenty-seven patients (93.1%) had complex partial seizures, one (3.44%) had simple partial seizures, and one patient had generalized seizures. Preop interictal EEG showed epileptiform discharges in 24 patients, while in five patients interictal EEG before surgery showed no epileptiform discharges. Pathologically, 24 of our patients were classified as complex type and five as simple type. MRI showed temporal lesion in 20 (68.9%) patients and nine patients had extratemporal DNT. We choose the type of surgery according to lesion and the epileptojenic zone. Finally, 27 patients had Engel Class IA and two patients had Engel Class IB outcome. Conclusion  Complete resection of the lesion with epileptojenic zone is important for seizure-free outcome. Timing of surgery, extent of surgery, and stopping antiepileptic drugs are still important factors.