结节性硬化症合并多个致痫灶的手术治疗探讨

目的探讨结节性硬化症(TS)病人多个致痫灶的外科处理方法及预后。方法 2002年1月至2008年6月采用外科手术治疗的TS患者21例,经术前评估和术中脑电检查显示一侧半球内有多个致痫灶15例,双侧半球有多个致痫灶,但以一侧半球为主6例。术中行皮层结节切除,并联合脑叶切除和多软膜下横纤维切断术,对6例术前伴有对侧痫样放电的病人行胼胝体前部切开。结果随访1～7年,平均3.2年。疗效按Engel分级,Ⅰ级13例,Ⅱ级4例,Ⅲ级3例,Ⅳ级1例;癫痫完全控制率为61.9%(13/21),有效率95.2%(20/21)。平均智商从术前的(51.7±14.6)分提高到(59.6±11.8)分。本组5例病人出现暂时性的并发症,1例出现脑积水,无手术死亡病例。结论外科手术是治疗一侧半球内或一侧半球放电为主的多灶性TS的良好手段。     

涉及中央区难治性癫痫的外科治疗

目的探讨涉及中央区难治性癫痫的手术治疗。方法3例患者术前除常规致痫灶评估外,还应用fMRI作皮质功能区定位。术中通过皮层EEG（ECoG）对致痫灶定位,通过皮层诱发电位（SEP）及皮质电刺激定位脑功能区,对位于功能区以外的致痫灶行切除性手术,功能区内的致痫灶行软脑膜下横行纤维切断术。结果术后无神经功能障碍,术后3月,2例病人无癫痫发作,1例偶有部分性发作;术后8月,1例无发作,1例偶有部分性发作,1例减少75%发作。结论术前功能区评估、术中电生理监测有助于保护皮质重要功能和提高手术癫痫控制率。     

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。     

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。     

中央沟区病变继发癫痫的显微手术治疗

目的 探讨中央沟区病变继发癫痫的显微手术治疗.方法 总结35例首发症状为癫痫的中央沟区病变,应用长程脑电图、偶极子、皮层电极定位致病灶;MRI结合术中皮质体感诱发电位、皮质刺激术定位脑功能区,显微镜下脑沟人路,保护脑功能区,病变切除后功能区残余癫痫波应用脑皮层热灼.结果 术后随访1-3年,疗效达到Engel I级31例、Ⅱ级4例、没有Ⅲ、Ⅳ级患者.术后3个月肢体功能障碍较术前改善23例,不变7例,加重5例,无手术死亡.结论 准确定位致痫灶和功能区,脑沟入路皮质下切除病变,配合功能区残余癫痫波皮层热灼,能保护肢体功能、控制癫痫的发作.     

兔运动区致痫灶周围皮质软膜下环切对痫性放电及功能影响的实验研究

针对重要功能区皮质致痫灶设计了一种新术式——致痫灶周围皮质软膜下环切术。手术切断致痫灶周围皮质表层的水平纤维连结,而保留皮质的垂直柱状纤维。其目的在于阻断痫性放电,从而控制发作,减轻脑功能损害。本组用家兔25只,分三组进行,用青霉素G钠10.00U在运动区皮质产生局灶性癫痫模型。通过对照组,致痫灶切除组,软膜下环切组,说明软膜下环切术在阻断痫性放电的产生和传播方面能达到致痫灶切除术的同样效果。     

多软膜下横切术治疗功能区顽固性癫痫

目的对于致痫灶位于脑的主要功能区的癫痫病人,采用多软膜下横切术(MST),达到既可控制癫痫的发作,又能保护皮质的正常功能.方法选择20例继发性癫痫病人,术前均做神经影像学、脑电图和神经心理学检查;术中硬脑膜切开后,先用皮质电极核实致痫灶的方位和范围,然后切除远离功能区的病灶,再用横切刀切断功能区痫性放电的神经元树突,最后用皮质电极复查,直至棘波消失.结果除1例无改善外,其他19例都得到了不同程度的控制.满意6例,显著改善6例,良好5例,效差2例,无改善1例,总有效率为95%.永久性重要功能缺失和残疾,无手术死亡率.结论MST它能使脑主要功能区癫痫,在不引起任何机能障碍的情况下,使痫性发作得到有效的控制.     

全麻唤醒和术中电刺激在脑功能区病灶性癫痫手术中的应用

目的 探讨全麻唤醒和术中电刺激在脑功能区病灶性癫痫手术中的应用及意义.方法 对11例涉及脑功能区病灶性癫痫患者术前行MRI和头皮视频脑电图检查,其中6例行fMRI检查.全麻下手术,其中7例行食道咽腔导管插管全麻唤醒.术中皮层脑电图定位痫样放电皮层,皮层电刺激定位皮质功能区,根据病灶、致痫皮层和皮层功能区关系选择相应手术方式,在切除病灶时作皮层电刺激以保护皮质下功能传导束.结果 10例病灶全切除,1例次全切除.术后2例暂时性偏瘫.随访6～24月,患者无神经功能障碍,Kamofsky评分平均100分,使用1种抗癫痫药物,癫痫控制满意.结论 食道咽腔导管插管全麻唤醒屉一种安全、简便的麻醉唤醒方法;术中电刺激监测能够最大限度切除病灶,妥善处理致痫皮层,有效保护脑功能区,改善患者的生活质量.     

功能区肿瘤继发癫痫的手术治疗

目的总结我科近年来治疗功能区肿瘤继发癫痫的治疗经验,探讨功能区肿瘤继发癫痫的外科治疗原则和方法。方法对2011年5月-2013年5月在我院接受手术治疗的50例功能区肿瘤继发癫痫的患者进行回顾性分析。结果全组病例术前均行系统癫痫外科评估。其中12例行颅内电极皮层电刺激功能区定位,根据肿瘤大小和脑电图所监测到的致痫区以及皮层电刺激所确定的功能范围制定手术切除范围,术后1例出现对侧手指轻瘫,1例出现一过性对侧肢体偏瘫,1例出现一过性失语,其余术后随访无明显神经功能障碍。未行颅内埋藏电极患者38例,综合运用MRI、术中皮层脑电监测和术中B超等多种方法来确认手术切除范围,术后神经功能缺损术后加重5例,7例术后出现对侧肢体轻瘫,2例出现对侧偏麻,4例出现一过性失语。全组术后癫痫无发作达到EngelⅠ级42例（84％）。结论功能区肿瘤继发癫痫的外科治疗要兼顾肿瘤的全切、致痫灶的切除以及功能区的保护。术者的经验和熟练的显微操作是基本前提,术前综合运用多种评估方法,结合术中皮层脑电及术中B超等方法对病灶,致痫区以及功能区进行定位,对保证手术效果起重要作用。颅内埋藏电极长程脑电监测定位致痫灶,并经皮层电刺激定位功能区,可进一步明确病变与致痫灶、功能区重叠程度及毗邻关系,从而为手术切除范围的界定提供了关键信息。     

结节性硬化症癫痫的术前评估及手术方式的选择

目的 总结结节性硬化症所致癫痫患者的术前评估及手术方式,观察术后疗效.方法 回顾性分析北京三博脑科医院2004年6月至2011年6月手术治疗的20例结节性硬化症患者临床资料并系统随访1～5年.结果 经过综合评估,单纯致痫结节切除术8例;电极植入后致痫结节切除术1例;脑叶切除术4例;致痫结节切除+致痫皮层热灼术2例;脑叶切除+离断术1例;迷走神经刺激术2例;胼胝体全段切开术2例.术后随访Engel Ⅰ级11例,Ⅱ级2例,Ⅲ级5例,Ⅳ级2例,有效率达90％ (18/20).结论 经过精确术前评估,选择合适的手术方式,可以有效地控制或减轻结节性硬化引起的癫痫发作.     

Magnetoencephalography in patients with tuberous sclerosis and localization-related epilepsy

PURPOSE: To clarify the usefulness of magnetoencephalography (MEG) for diagnosis of the spatial relations between spike foci and suspicious epileptogenic tubers on MRI in patients with tuberous sclerosis (TS) and to compare MEG spike foci with single-photon emission computed tomography (SPECT) findings. METHODS: We analyzed magnetic fields of epileptic spike discharges in 15 patients with TS and localization-related epilepsy (LRE) by using MEG (a whole-head 204-channel magnetometer system). We investigated the spatial relation between the equivalent current dipoles (ECDs) of interictal spike discharges and visible cortical tubers on MRI. We also compared results of MEG and MRI with SPECT findings. RESULTS: MEG detected a cluster of ECDs around one cortical tuber in six of 15 patients and clusters of ECDs around two cortical tubers in five patients. Interictal SPECT was disappointing in detection of epileptic foci in TS. However, MEG spike foci showed spatial consistency with ictal hyperperfusion areas in two patients. Three patients with single ECD clusters underwent surgical treatment: two have been seizure free, and one has obtained seizure reduction of >90%. CONCLUSIONS: ECDs were located around visible tuber nodules. MEG enabled precise localization of the epileptic foci and provided crucial information for surgical treatment in patients with TS and partial epilepsy. TS patients showing a single ECD cluster on MEG may be appropriate candidates for surgical treatment.     

Topographic Comparative Study of Magnetic Resonance Imaging and Electroencephalography in 34 Children with Tuberous Sclerosis

A series of 34 children with confirmed tuberous sclerosis (TS) were studied prospectively by both EEG and magnetic resonance imaging (MRI) at ages ranging from 5 months to 18 years. Size and topography of the cortical tubers were analyzed on axial and coronal views, in T2 sequences, and the large tubers greater than 10 mm were studied. In addition, EEG follow-up data were reviewed retrospectively. Twenty-six patients (76%) had both MRI large cortical tubers and EEG foci, 3 had normal EEG, 2 had normal MRI, and the remaining 3 had only small tubers. The number of large tubers was significantly related to EEG foci. A topographic MRI/EEG correspondence was observed for at least one tuber in 25 of the 26 patients, and correspondence was complete in 10 patients. Large cortical tubers without corresponding EEG foci were observed in 11 patients; these tubers mainly involved the frontal regions and were found before the patients were 2 years old. EEG foci without corresponding tubers were observed in 4 patients. In addition, secondary bilateral synchrony was preferentially observed in patients with frontal lesions and after the age of 2 years. These data confirm that the cortical tubers are epileptogenic and that their expression may be influenced by regional cortical maturation.     

Regional cerebral blood flow in relation to MRI and EEG findings in tuberous sclerosis.

To identify the focus of paroxysmal neuronal activity causing epilepsy in tuberous sclerosis (TS), the regional cerebral blood flow (r-CBF) in 19 patients with TS was assessed using single-photon emission computed tomography (SPECT) with I-123 iodoamphetamine (IMP), in correlation with serial interictal EEGs and organic changes observed on magnetic resonance imaging (MRI). There was a general irregularity of cortical IMP uptake and retention in TS, and two-thirds of the cerebral regions exhibiting high intensity in T2-weighted MRI images (cortical tubers) showed a decrease in r-CBF. In addition to in tubers, decreased r-CBF was observed in regions in which MRI was considered to indicate destruction of the normal cortex, atrophy or vascular abnormalities, although these areas did not consistently show epileptic changes in serial EEGs. Among the cortical regions which consistently showed epileptic foci in serial EEGs, none showed abnormal r-CBF without lesions on MRI. We conclude that IMP-SPECT is useful for visualizing the epileptogenic laterality in cases with bilateral MRI lesions and EEG epileptic changes, and to differentiate epileptogenic foci from electrophysiological propagated areas. However, regarding the severity of epilepsy, the MRI findings showed a better correlation than the IMP-SPECT findings did.     

Evaluation of epileptogenic networks in children with tuberous sclerosis complex using EEG-fMRI

PURPOSE: Ninety percent of patients with tuberous sclerosis complex (TSC) have epilepsy. Identification of epileptogenic areas can be difficult and studies are needed to characterize the epileptogenic network in more detail. METHODS: Five children with TSC and focal epilepsy were studied using simultaneous EEG and functional MRI recordings. Tubers were marked by a neuroradiologist on the anatomical MRI. Spike-associated BOLD (blood oxygenation level-dependent) responses were superimposed with lesions. RESULTS: Thirteen different types of interictal epileptiform discharges (IED) were analyzed with 12 showing a BOLD response, all involving more than one tuber. Five studies had tubers with activations exclusively within the lesion, three studies had lesional activations extending to perilesional areas, and two studies had activations involving exclusively perilesional areas of at least one tuber. Deactivations exclusively within a tuber were found in six studies, lesional deactivations extending to perilesional areas were found in four studies, and tubers with exclusively perilesional deactivations were found in five studies. A BOLD response was found in at least one tuber in the lobe of IED generation and presumed seizure onset (according to telemetry) in all patients. In four patients, the same tubers were involved following different IED localizations. The observed changes were always multifocal, sometimes involving tubers distant from the IED field. DISCUSSION: These findings suggest extended epileptogenic networks in patients with TSC, which exceed networks described in PET and SPECT studies. It was possible to identify specific interictally active tubers. EEG-fMRI provides a noninvasive method to select tubers and areas at their borders for further presurgical investigations.     

结节性硬化合并婴儿痉挛症的外科治疗

目的 探讨结节性硬化所致婴儿痉挛症的手术适应证、方法和预后.方法 回顾性分析采用外科手术治疗17例结节性硬化症伴婴儿痉挛症的经验.结果 术后疗效按Engel分级,Ⅰ级11例,Ⅱ级4例,Ⅲ级2例.术前评估为单致痫灶的2例患者均为Ⅰ级.局限在一侧大脑半球的8例多灶性癫痫患者中,6例Ⅰ级,2例Ⅱ级.双侧大脑半球均有致痫灶但以一侧为主的7例患者中,3例Ⅰ级,2例Ⅱ级,2例Ⅲ级.平均智商(IQ)从术前的52.6分提高到61.8分.结论 结节性硬化所致婴儿痉挛症具有良好的外科预后,对部分药物难治性患者在评估确定责任结节后可以考虑进行外科干预.

Abstract：

Objective To investigate the surgical indications, methods and outcomes of infantile spasms with tuberous sclerosis(TS). Method Surgical treatment of 17 infantile spasms patients with TS was reviewed. Single epileptogenic tuber or lobe was resected when a focal epileptic discharge was indicated according to preoperative evaluation and EcoG, and multiple lobes or tubers resection were chosen to deal with hemisphere limited multiple epileptic foci. Anterior corpus callosotomy was added when contralateral hemisphere showed epileptic discharges. Results Acording to follow - up of 3 years in average after surgery, 11 patients had an Engel Class Ⅰ outcome, 4 patients had rare seizure ( Engel Class Ⅱ ), and 2 patients had a reduction in seizure frequency (Engel Class Ⅲ ). The mean IQ of patients was improved from 52.6 to 61. 8. Conclusions Epilepsy surgery in tuberous sclerosis with infantile spasms showes a favourable outcome,and surgical intervention should be considered in some intractable infantile spasms after corresponding tuber is derermined.     

语言功能区定位与近语言功能区癫(癎)灶切除(附11例分析)

目的 探讨以皮质电刺激为基础的语言功能区皮质的定位方法.方法 总结11例癫(癎)灶邻近语言区病人的治疗经验.将格栅状皮质电极覆盖于脑皮质表面,其中Broca区6例,Wernicke区5例;应用皮质电刺激定位出语言功能区8例,阴性3例;功能磁共振显示语言功能区移位2例.在癫(癎)灶切除过程中,采用局麻2例,术中拔除喉罩2例,常规气管插管全麻7例;术中注意保留具有语言功能的皮质.结果 术后出现-过性语言不流利2例,余9例无明显语言功能障碍.术后随访1年以上,Engel Ⅰ级8例,Ⅲ级2例,Ⅳ级1例.结论 采用埋植式颅内电极进行语言皮质功能绘图方法,可较精确地定位癫(癎)病人的语言功能区.     

局灶性皮质发育不良所致难治性癫痫的微创神经外科治疗

目的应用神经导航结合术中皮质电极描记,微创治疗局灶性皮质发育不良(focal cortical dysplasia,FCD)所致的难治性癫痫。方法 26例局灶性皮质发育不良所致的难治性癫痫患者,术前常规使用CT、磁共振成像(magnetic resonanceimaging,MRI)、长程视频脑电图(digital video signal and electroencephalogram,VEEG)、磁共振波谱分析(MR Spectroscopy,MRS)等检查,如病灶位于功能区则行功能性磁共振成像(functional magnetic resonance imaging,fMRI)。术中通过神经导航确定的病灶与ECoG确定的致痫灶位置及范围进行对比,了解两者的吻合程度及差异,综合分析后精确并标记出癫痫波的起源位置和范围,将局灶性皮质发育不良病灶和周边的致痫皮质切除;如致痫灶位于功能区或附近,在保留功能区皮质的基础上,给予低功率皮质热灼。结果术后病理结果:26例患者病理标本符合FCD。术后患者无明显并发症出现。根据Engel术后效果分级进行评估,Ⅰ级23例,Ⅱ级2例,Ⅲ级1例。结论神经导航结合术中皮质电极描记在局灶性皮质发育不良所致的难治性癫痫手术中,具有定位准确、损伤少的优点,在切除致痫灶的同时能最大程度保护脑功能。     

致灶切除治疗无病灶中央区癫

目的总结致灶切除术治疗无病灶中央区癫的诊治经验。方法回顾性分析11例无病灶中央区癫病人的临床资料,所有病人均放置颅内电极,采用皮质脑电图监测定位癫发作起源区,皮质电刺激定位功能区,采用术中唤醒行致灶切除术。结果随访11例,时间17~32个月,按照Engel分级:Ⅰ级3例,Ⅱ级3例,Ⅲ级2例,Ⅳ级3例。术后出现肢体功能障碍3例,其中2例分别于术后2周及3个月恢复;残留手指活动不灵1例。结论在颅内电极精确定位功能区及致灶的前提下,采用致灶切除术治疗无病灶中央区癫,可获得满意疗效。     

Tuberous Sclerosis and Multiple Tubers: Localizing the Epileptogenic Zone

Summary:  Tuberous sclerosis complex (TSC) is associated with medically refractory seizures and developmental delay in children. These epilepsies are often resistant to antiepileptic drugs (AEDs), may be quite severe, and usually have a negative impact on the child's neurological and cognitive development. It is believed that functional outcome is improved if seizures can be controlled at an early age. The surgical treatment of intractable epilepsy in children and adults with TSC has gained significant interest in recent years. Previously published studies have shown a potential benefit from resection of single tubers, with most of the results noted in relatively older children. All of these reports support the idea that if a single primary epileptogenic tuber/region can be identified, then a surgical approach is appropriate. However, most children with TSC have multiple potentially epileptogenic tubers, rendering localization challenging, and they are therefore rejected as possible surgical candidates. We have utilized a novel surgical approach using invasive intracranial monitoring, which is typically multistaged and bilateral. Here we present an illustrative case of a young boy with TSC and medically refractory epilepsy who underwent a staged surgical approach. This multistage surgical approach has been useful in identifying both primary and secondary epileptogenic zones in TSC patients with multiple tubers. Multiple or bilateral seizure foci are not necessarily a contraindication to surgery in selected TSC patients. Long-term follow-up will determine whether this approach has durable effects. We await better methods for identifying the epileptogenic zone, both noninvasive and invasive.