27例学龄前难治性颞叶癫痫患儿临床报道

目的探讨学龄前难治性颞叶癫痫患儿影像学、电生理特点及手术方法和疗效。方法回顾性分析解放军联勤保障部队第九八八医院神经外科中心自2014年6月至2019年1月行手术治疗的27例学龄前难治性颞叶癫痫患儿资料,术前评估结合临床发作表现,MRI、磁共振波谱分析(MRS)、正电子发射断层扫描(PET-CT)等影像资料,以及发作间期和发作期视频脑电图(VEEG)资料;术中应用皮层脑电图(ECoG)与深部电极监测定位异常放电区域,指导手术切除致痫灶范围。术后采用Engel分级评估疗效。结果27例患儿均有典型颞叶癫痫临床表现,MRI发现一侧颞叶及海马异常信号影,发作间期及发作期VEEG提示异常放电起始于一侧额颞部。术中ECoG及深部电极监测均发现颞叶明显持续或阵发性尖波、棘波、棘慢复合波等癫痫样放电。27例患儿均采用标准前颞叶+病灶切除+周边异常放电颞叶皮质扩大切除术,其中2例患儿切除部分岛叶长回及额盖皮质热灼处理。随访6个月,EngelⅠ级患儿22例,EngelⅡ级患儿3例,EngelⅢ级患儿2例。结论早期手术、术中ECoG与深部电极联合监测下适度扩大切除范围是改善学龄前难治性颞叶癫痫患儿手术疗效的关键因素。     

难治性颞叶癫痫的手术治疗方式及其疗效研究

目的 探讨难治性颞叶癫痫的临床特点、术前评估方法、手术治疗方式及其疗效。方法 105例难治性颞叶癫痫患者术前行头部CT及MRI检查,同时加行双侧海马磁共振波谱分析(MRS);并均行长程视频脑电图检查。根据患者的术前临床发作特点、影像学和长程视频脑电图检查结果,在术中皮层脑电图监测下行手术治疗。术后随访患者1年以上,观察手术的疗效。结果 本组患者中,48例海马硬化患者行前颞叶切除术(包括大部分海马及杏仁核);另外57例有其他病灶的患者先行病灶切除术,复查皮层脑电图其中23例患者仍有异常放电,对这23例患者加行前颞叶切除术(包括大部分海马及杏仁核),前颞叶切除后有5例患者仍有异常放电,予以皮层热灼术。手术疗效:术后80例患者的癫痫发作完全消失,13例患者较术前明显缓解,12例患者无明显变化;总有效率为88. 6%,优良率为76. 2%。结论 颞叶癫痫的发作形式有一定的临床定位价值,如果患者的神经影像学和视频脑电图检查结果一致,则强烈建议手术治疗。手术一定要在皮层脑电图监测下进行;同时术中在切除颞叶病变后,应根据皮层脑电图监测结果加行前颞叶切除术,必要时再加行皮层热灼术,以解决双重病理现象。     

局灶性皮质发育不良合并难治性癫痫患者头部MRI融合PET-CT进行术前评估的价值

目的探讨颅脑PET-MRI融合图像在表现为局灶性皮质发育不良(focal cortical dysplasia,FCD)的应用价值,并总结患者治疗后的预后,为广大癫痫患者寻求一个更合理的诊治方案。方法回顾性分析2017年1月～2018年8月我院神经内外科、儿科收治的23例表现为难治性癫痫的FCD患者临床表现、影像学特点、视频脑电图特点以及手术疗效。结果表现为难治性癫痫的FCD患者的发作类型多种多样,但以局灶性发作继发全面性发作为主;病灶多见于颞叶15例、额叶6例、顶叶2例; 20例患者癫痫序列MRI及视频脑电发现病灶,3例患者行癫痫序列磁共振及视频脑电未能确定明确的致痫灶,完善PET-MRI融合成像发现致痫灶,病变部位主要在额叶,因此对于头部MRI阴性的患者,则主要通过PET-MRI发现病灶;视频脑电图(EEG)痫样放电部位与PET-MRI融合成像发现的病灶部位有较高一致性,且放电及发作形式对于FCD分型没有明显的相关性; 23例患者术后癫痫发作次数明显减少,有的未再发作。结论表现为难治性癫痫的FCD患者可通过PET-MRI融合成像结合视频脑电脑电进行术前评估,找准致痫灶,手术切除癫痫灶的治疗可取得良好的预后。     

MRI显示双侧病变的难治性癫痫患者的外科治疗

目的探讨MRI显示为双侧病变的难治性癫痫患者的外科手术治疗及疗效。方法回顾性分析29例MRI显示为双侧病变的难治性癫痫患者的临床症状、电生理、影像、手术及术后随访资料。结果通过综合的术前定位评估,均能明确致痫的责任病灶,并行手术切除或合并部分胼胝体切开,术后随访7~40个月,疗效按Engel分级,Ⅰ级17例,Ⅱ级5例,Ⅲ4级例,Ⅳ级2例,失随访1例,无永久性神经功能缺陷发生。结论 MRI显示的双侧病变并非都是引起癫痫发作的原因,通过综合的术前定位评估能明确致痫责任病灶的患者,行外科切除手术治疗能获得很好的控制疗效。     

长程颅内电极监测定位技术在癫痫外科中的应用(附19例临床分析)

目的分析长程颅内电极对难治性癫痫患者的癫痫发作起源区定位作用,评估采用这一技术后的癫痫发作控制以及手术并发症的发生情况。方法19例难治性癫痫患者,采用常规脑电图及其它检查不能明确癫痫发作起源区位置,或癫痫发作起源区与重要功能区关系密切,对怀疑脑区进行颅内电极埋置术,术后进行长程颅内脑电监测,根据脑电情况,确定发作起源区,明确与功能区的部位后,进行切除术或多处软膜下横切术。结果19例患者中,核磁共振有双侧病变者5例,单侧病变9例,核磁共振阴性的患者5例。非侵袭性的术前评估方法结论不一致的有11例。手术后15例患者发作消失,3例患者发作频率减少90%以上,1例癫痫发作控制无效。1例患者发生永久性局限视野缺损,缺损为左上视野区,1例患者电极埋置术后出现一过性失语,切除术后未发生失语。结论对于采用常规脑电图及其它检查不能明确癫痫发作起源区位置,或癫痫发作起源区与重要功能区关系密切的患者,长程颅内脑电图监测能够准确定位发作起源区位置,回避功能皮质区,有效降低并发症的发生率。     

大脑半球切除治疗难治性癫痫的术前评估及手术改良(附58例报告)

目的 研究大脑半球切除治疗由一侧大脑半球广泛病变引起的难治性癫痫的术前评估及手术改良.方法 回顾性分析清华大学玉泉医院癫痫中心2006年11月至2011年7月58例行大脑半球切除治疗癫痫病例.根据患者的临床发作、影像学及脑电图特点进行癫痫起源一侧半球的定侧;根据功能磁共振、磁共振弥散成像、PET -CT等进行术前神经功能分析及术后神经功能预测;选择制定合适的半球切除手术计划.结果 58例均行一侧大脑半球切除,32例左侧半球,26例右侧半球,其中5例行保留运动区的大脑半球切除.无手术死亡,4例少量术腔出血,5例切口缺血愈合不良并脑脊液漏.随访1年至5年8个月,48例术后癫痫无发作;10例术后有程度不等的发作.按照Engle评分Ⅰ级48例(83％);Ⅱ级7例(12％);Ⅲ级3例(5％).结论 改良大脑半球切除对于一侧半球病变引起的难治性癫痫控制效果及脑功能代偿良好,无严重并发症,术后停药复发率低.     

改良大脑半球切除术治疗难治性癫痫1例

目的探讨对一侧大脑半球结构性病变致难治性癫痫的治疗方法。方法难治性癫痫患者1例,经MRI证实其右侧大脑半球广泛软化。术前应用功能MRI和Wada试验等方法评估其大脑功能是否转移,并应用视频脑电图测定其癫痫放电的范围,应用大脑半球切除术进行治疗。结果术后患者无癫痫发作,语言及运动功能与术前无变化。结论大脑半球切除术是治疗由一侧半球结构病变引起的难治性癫痫的有效方法,术后不仅癫痫发作消失,而且神经功能不受影响。     

儿童和青少年癫相关局灶性皮质发育不良22例临床分析

研究背景局灶性皮质发育不良是一组包括皮质分层异常、细胞结构异常和细微白质异常的疾病,是难治性癫的主要病因。本研究总结儿童和青少年局灶性皮质发育不良的临床表现、脑电图和头部MRI特点,以期提高诊断与治疗水平。方法回顾分析22例儿童和青少年局灶性皮质发育不良患者的临床表现、脑电图和头部MRI特点。结果 22例患者中13例(59.09%)单纯表现为局灶性发作,6例(27.27%)单纯表现为全面性发作,3例(13.64%)表现为局灶性发作继发全面性发作;发作频率较频繁,6例(27.27%)每日均有发作,13例(59.09%)≥1次/月,3例(13.64%)1次/月;21例(95.45%)脑电图监测到异常慢波和样放电。12例MRI显示局灶性皮质发育不良位于额叶,头皮脑电图显示7例(7/12)局灶性额区异常慢波和样放电,2例(2/12)广泛性慢波和棘慢复合波,2例(2/12)颞区尖波,1例(1/12)中央中线少量尖波;单纯局灶性发作7例(7/12),全面性发作4例(4/12),局灶性发作继发全面性发作1例(1/12)。6例MRI显示局灶性皮质发育不良位于顶叶,头皮脑电图显示3例(3/6)局灶性顶区异常慢波和样放电,2例(2/6)颞枕区样放电,1例(1/6)无异常;单纯局灶性发作5例(5/6),局灶性发作继发全面性发作1例(1/6)。2例MRI显示局灶性皮质发育不良位于颞叶,头皮脑电图显示1例(1/2)局灶性颞区异常慢波和样放电,1例(1/2)额区样放电;均为全面性发作。2例MRI显示局灶性皮质发育不良位于岛叶,头皮脑电图显示1例(1/2)双侧颞区样放电,1例(1/2)全导联尖波和尖慢复合波;单纯局灶性发作1例(1/2),局灶性发作继发全面性发作1例(1/2)。结论局灶性皮质发育不良患者通常于学龄前期和学龄期发病,主要呈现局灶性或全面性发作,亦可见其他发作类型,发作频率较频繁;头部MRI是诊断局灶性皮质发育不良的重要方法;治疗方法主要是抗癫药物,发作控制欠佳的患者考虑癫外科手术。     

以癫痫为首发症状的岛叶胶质瘤的手术治疗

目的探讨以癫痫为首发症状的岛叶胶质瘤的手术治疗方法及疗效。方法岛叶胶质瘤患者15例,男10例,女5例;年龄9～56岁;病程1月～3年,临床表现均以癫痫发作为首发症状,术前除常规行头颅CT及MRI检查,还加行了常规脑电图检查,另有10例行了长程同步视频脑电图检查。胶质瘤均在术中皮层脑电图(ECoG)监测下行手术切除。结果肿瘤全切12例,次全切除3例。所有患者在切除肿瘤前ECoG监测均有大量的异常痫样放电,且越靠近病变放电越明显,切除肿瘤后复查ECoG发现在术腔周围仍然有痫样放电,但放电范围均较切除前明显缩小。其中13例加行了皮层热灼术,2例加行了多处软脑膜下横纤维切断术。术后随访1年以上发现10例生存良好,3例复发,2例死亡。且术后13例患者无癫痫发作,只有2例仍时有癫痫发作。结论在术中ECoG监测下切除以癫痫为首发症状的岛叶胶质瘤,不但能指导肿瘤切除,同时还能一并将致痫灶切除,值得积极稳妥地推广。     

表现为难治性癫痫的低级别脑胶质瘤的手术治疗(附28例临床分析)

目的探讨表现为难治性癫痫的低级别脑胶质瘤的临床特点,手术治疗策略。方法回顾性分析28例表现为难治性癫痫经病理检查证实为低级别胶质瘤(LGG)患者的临床表现、影像学(CT/MRI/PET)资料、脑电图(EEG/VEEG/ECoG)资料和手术方法。所有病例术前均行动态脑电图(EEG)检查明确致痫灶完成定位诊断,术中行皮质脑电(ECoG)监测,再次验证致痫灶,同时快速冰冻确诊肿瘤性质,完成定性诊断。全部患者在显微镜下先行致痫灶(肿瘤)切除术,并根据致痫灶部位的不同,加行皮质热灼,功能区行软膜下横切、颞叶病灶行患侧海马切除术。结果随访6~40个月,平均24个月癫痫症状消失(EagleI级)26例,癫痫发作次数明显减少(EagleII级)2例,肿瘤均无复发。结论表现为难治性癫痫的脑肿瘤多为低级别脑胶质瘤;术前动态EEG明确致痫灶,术中ECoG监测下切除脑肿瘤及致痫灶是控制癫痫发作的有效方法。     

Epilepsy surgery in a patient with Lennox-Gastaut syndrome and cortical dysplasia

Lennox-Gastaut syndrome (LGS) is classified as a generalized epilepsy, and is often intractable to antiepileptic drugs. Although corpus callosotomy may sometimes control drop attacks, curative epilepsy surgery is rarely possible in LGS. We report, here, a patient with LGS and focal cortical dysplasia, who became seizure-free after epilepsy surgery. The patient was a 24-month-old boy without perinatal insult in whom seizures began 7 days after birth and who experienced development delay. Brain magnetic resonance imaging (MRI) showed focal cortical lesion with calcification in the right frontal area. At age 13 months, his seizure type changed from tonic seizures to head drops (atonic types) and atypical absence seizures. His interictal electroencephalogram (EEG) showed generalized slow spike and wave discharges, and he was diagnosed with LGS. His seizures were intractable to medical treatment, and a ketogenic diet was not effective. He was evaluated prior to surgery by long-term video-EEG monitoring, which detected many seizures consisted of a sudden onset of falling attacks (atonic type) intermittently followed by atypical absence seizures with diffuse slow wave bursts followed by slow spike and wave discharges in ictal EEG, brain positron emission tomography (PET) and MRI. A right frontal lesionectomy with intra-operative electrocorticography (EcoG) was performed. From the time of lesionectomy to the present, the patient has been seizure free and has been developing normally. Our results suggest that focal resective surgery should be considered in patients with LGS and focal epileptic lesions.     

Serial EEG in intractable epilepsy

We compared serial EEGs performed at admission, discharge, and follow-up (mean, 25 months) to clinical outcome in 70 patients with intractable epilepsy. The diagnosis in each case was confirmed by intensive monitoring. EEG features evaluated were background slowing, focal slowing, and focal, bilateral, or generalized epileptiform discharges. Clinical measures were seizure frequency and medication toxicity. No statistically significant correlations were found between improvement in any EEG feature and any clinical measure. EEG did not predict which patients would benefit from intensive monitoring. Serial EEGs may be of little value in assessing the results of treatment in patients with severe epilepsy.     

Surgery for intractable epilepsy in children: pre- and perioperative evaluations with special reference to dipole tracing method estimated from interictal spike

Methods of preoperative and perioperative evaluation methods for surgical treatment of intractable epilepsy in children are described. Among non-invasive diagnostic methods, EEG-video monitoring is the most fundamental. Amygdalohippocampal volume measurement by MR was useful for the differential diagnosis of mesial temporal lobe epilepsy (TLE) from lateral TLE and generalized epilepsy. The dipole tracing method with a realistic head model was useful for identification of epileptic foci from the interictal spikes of scalp EEG, when an abnormal electric source was estimated as an equivalent current dipole (ECD) in the brain of patients with organic lesion and TLE. ECD concentration ratio ranged from 70 to 90% within 20 mm around the lesion. After lesionectomy seizures disappeared in every patient. The mean distance between the centers of the ECD and epileptic focus (identified by subdural electrode recording) was 14 mm (range: 8 to 18 mm). ECDs of mesial TLE were located in the temporal base rather than mesial temporal lobe, whereas those of lateral TLE in the lateral cortex precisely. In unilateral, intermediate and bilateral TLE, 76%, 52% and 36% of ECDs were localized in the ictal onset zone respectively (p = 0.007). Electrical cortical stimulation with chronically placed intracranial electrodes was used to accurately identify eloquent areas to avoid postsurgical complications. Immediately after operation, 10 to 20% of patients showed better or deteriorated results in neuropsychological examinations, which recovered in all patients after one year. Postoperative seizures were absent in three fourths of patients. Further efforts are needed to obtain better seizure control in future.     

立体定向毁损杏仁海马复合体治疗癫痫长期随访

目的 探讨立体定向射频毁损杏仁海马复合体，能否阻止癫痫放电的传播以治疗顽固性癫痫。方法 17例顽固性癫痫病人应用ZD立体定向系统，CT扫描确定杏仁核和海马的靶点，分别毁损杏仁核和海马。术后常规MR复查了解靶点毁损的部位和大小，并排除并发症。结果 本组17例，无手术并发症。随访2～6年，平均3年9个月，结果满意者7例(癫痫发作消失)，显著改善者7例(癫痫发作减少75％)，无效者1例，1例自行停药2月后，癫痫发作又复发，再次服药后仍有癫痫发作。所有患者均需服药，药量无明显减少。结论 立体定向射频毁损杏仁海马复合体治疗癫痫，创伤小，安全，控制癫痫发作基本满意，长期有效。     

Effect of Anterior Callosotomy on Bilaterally Synchronous Spike and Wave and Other EEG Discharges

Summary: We analyzed pre- and postoperative interictal and ictal EEG patterns in relation to seizure outcome in 29 patients with intractable epilepsy who had undergone anterior callosotomy. Twenty-two patients had generalized bilaterally synchronous sharp or spike and slow wave discharges (GSW) and 7 did not: Postoperatively, patients with preoperative GSW showed disruption of synchrony, increased amplitude asymmetry, and a decreased amount of GSW. No significant decrease was noted in the amount of total epileptic discharge after operation. There was a significant relation between seizure outcome and decrease in amount of GSW. The degree of disruption of synchrony, total amount of epileptic discharge, and pre- and postoperative EEG patterns were not good indicators of seizure outcome. Postoperative changes in lateralization of epileptic foci consisted either of increased lateralization, less lateralization but increased independent discharges in the previously nonpredominant hemisphere, or appearance of some lateralization. There appears to be a spectrum of GSW: Both sec-ondary bilateral synchrony and secondarily generalized corticoreticular epileptic discharges are distributed along this spectrum.     

A pediatric case of intractable complex partial seizures associated with mesial temporal lobe astrocytoma: usefulness of interictal epileptiform discharges in the present case]

The patient was a 10-year-old male with normal developmental milestones. He had medically intractable complex partial seizures since the age of 7 years. At the age of 10 years, he had focal motor seizures of the right face, and a head CT scan showed a calcified lesion in the left mesial temporal region. The tumor exhibited low intensity on T 1-weighted and high intensity on T 2-weighted MR images, and was not enhanced by gadolinium-diethylenetriamine pentaacetic acid. Interictal SPECT showed hypoperfusion in the left temporal region. One-day video/EEG monitoring revealed very frequent epileptiform discharges which occurred only during sleep period exclusively in the left anterior-to-middle temporal region. The patient underwent lesionectomy with the guidance of electrocorticography. The histological study of the resected tissue showed astrocytoma. After surgery he has had no seizures for 10 months. It was concluded that very frequent interictal epileptiform discharges strictly localized to the temporal lobe at which MRI-identified tumor was present could be predictive of epileptogenic zone in the present patient in whom clinical symptoms and the results of other studies were also concordant.     

300例癫痫及可疑癫痫患者AEEG分析

目的：对３００例癫痫及可疑癫痫患者的动态脑电图的应用价值进行了初步探讨。方法;使用北京明思公司ＳＷ－ＪＨ系列智能化脑电监护仪描记,并与常规脑电图比较。结果：３００例中ＥＥＧ异常５４例,ＡＥＥＧ异常１７８例ＡＥＥＧ痫样放电检出率明显高天ＥＥＧ。１４４例继发性癫痫患者中,ＥＥＧ异常８例,ＡＥＥＧ异常１０例,二者无显著性差异。     

Electro- and magneto-encephalographic spike source localization of small focal cortical dysplasia in the dorsal peri-rolandic region

ObjectiveSmall focal cortical dysplasia (FCD) may be ambiguous or overlooked on magnetic resonance (MR) imaging. Source localization of EEG and magnetoencephalography (MEG) spikes was evaluated to confirm the diagnosis of small FCD.MethodsThis study included 6 epilepsy patients with a single small lesion on MR imaging suggesting FCD within a single gyrus among 181 consecutive epilepsy patients admitted to our epilepsy monitoring unit over 27 months. Stereotypical interictal spikes were detected on simultaneous EEG and MEG recordings and the onset-related source of averaged spikes was estimated.ResultsAll 6 patients had unique clinical characteristics as follows: leg sensori-motor seizures in 5 patients and eye version in 1 patient; a small MR imaging lesion suggesting FCD in the dorsal peri-rolandic region, which had been overlooked until our evaluation; and both EEG and MEG dipoles were estimated adjacent to the MR imaging lesion.ConclusionsSource localization of EEG and MEG spikes can confirm the diagnosis of FCD based on a single small MR imaging lesion, which was overlooked by previous examination of MR images.SignificanceExamination of MR images should be based on spike source localization as well as seizure semiology to identify subtle MR imaging abnormalities.     

Early onset and focal spike discharges as indicators of poor prognosis for myoclonic-astatic epilepsy

Background: Myoclonic-astatic epilepsy (MAE) is an epileptic syndrome characterized by unique myoclonus, myoclonic-astatic, or astatic seizures in childhood. MAE prognosis vary from spontaneous remission to intractable seizures with profound mental retardation. Aim: Identifying early risk factors may optimize the treatment of children with MAE. Our hypothesis is early onset age and focal spike discharges on EEG indicate a poor MAE prognosis. Methods: Using the medical records of 9 children with MAE, we analyzed their clinical histories, EEG findings, and seizure symptoms. All patients were given follow-up observations/treatments by our department for at least 2 years after MAE onset. Results: Five of the patients were given favorable prognoses because their seizures disappeared within 2 years of onset; the other 4 received poor prognoses because their seizures continued more than 2 years. MAE onset in patient with refractory seizures was earlier than that in those with a favorable prognosis (7–24 months vs. 23–38 months). All the patients with refractory seizures showed moderate or severe mental retardation. Among the 5 patients with good prognosis, EEGs showed two with focal spike discharges and three with only generalized spike discharges. In contrast, all cases with a poor prognosis had focal spike discharges. Conclusions: MAE onset in patients with refractory seizures occurs earlier than in those with favorable prognosis. Prognosis was excellent when EEG findings show no focal spike discharges. Both early seizure onset and the focal spike discharges associated with MAE are indicators of poor prognosis.     

EEG characteristics predict subsequent epilepsy in children with febrile seizure

The role of electroencephalography (EEG) in the work-up of febrile seizure (FS) remains controversial. We investigated the importance of EEG characteristics, especially the localizations of paroxysmal discharges, as predictors for subsequent epilepsy. Patients were referred from the outpatient department for EEG within 7-20 days after the seizure. EEGs were classified as paroxysmally abnormal based on the presence of spikes, sharp waves, or spike-wave complexes, whether focal or generalized, that were considered abnormal for age and state. Of 119 patients with FS, 26 (21.8%) revealed paroxysmal abnormality on EEG and 9 (7.6%) developed epilepsy. Of nine patients with later epilepsy, 6 (66.7%) revealed paroxysmal EEG abnormality. Of 26 patients with paroxysmal abnormality, 6 (23.1%) developed epilepsy. Of 10 patients with generalized paroxysmal spike and wave activity, one (10%) developed epilepsy. Of seven patients with rolandic discharge (RD), two (28.5%) developed epilepsy. Of four patients with paroxysms in the frontal region, three (75%) developed epilepsy. Of five patients with paroxysms in the occipital region, none developed epilepsy. Compared with generalized EEG foci, the relative risk (RR) for patients with frontal EEG foci was 27.0. Patients with frontal EEG paroxysms had a significantly higher risk of developing epilepsy than those with paroxysms in other regions of EEG foci (p=0.035). These findings suggest that patients with FS presenting with frontal paroxysmal EEG abnormalities may be at risk for epilepsy. In patients with frontal paroxysmal EEG abnormalities, serial EEG should be performed, even though it does not contribute to treatment.