难治性癫痫的放射外科治疗

难治性癫痫 ,又称顽固性癫痫 ,指癫痫发作频繁、应用适当的第一线抗癫痫药物正规治疗且药物血浓度在有效范围内两年以上、仍不能控制发作且影响日常生活。约有 2 0 %的癫痫病人随着病情发展或治疗不当 ,可能转变为难治性癫痫。容易导致难治性癫痫的因素有 :(1)复杂部分性发作、婴儿痉挛及 L ennox- Gastaut综合征等 ;(2 )发作频繁 ,每天数次 ;(3)出现过癫痫持续状态 ;(4 )对发作频率判断错误 ;(5 )起病后延误治疗 ;(6 )不适当的多种药物联合应用 (包括中药 ) ;(7)同一期间内在几个医疗单位应用不同的治疗计划 ;(8)对合并的精神心理障碍认识…     

难治性癫痫状态的外科治疗

难治性癫痫状态(refractory status epilepticus,RSE)是一种对足够疗程足够大剂量抑制性治疗(high-dose Suppressive therapy,HDST)治疗效果不佳的癫痫状态。目前大多数情况下药物难治性癫痫状态的治疗,主要凭借医生个人经验,HDST是RSE治疗的主要方法,而外科手术切除致痫病灶亦是很有效的治疗手段之一。目前外科治疗RSE相关文献仅限于病例报告及小样本分析。本文就目前外科治疗RSE的现状、手术适应证、手术时机、手术方式、手术并发症和预后进行综述。     

小儿难治性癫痫早期外科治疗探讨

长期反复发作的难治性癫痫可严重影响患者的生活质量,尤其是处于发育阶段的小儿患者,可导致患者智力低下和精神衰退,影响正常的生长发育。随着临床诊疗技术的进步,癫痫患者的早期外科治疗已得到广泛的认同。成人患者已有完善的手术标准作为其治疗指导,而目前对于小儿难治性癫痫的认识及早期手术治疗尚处于探索阶段。本文主要就小儿难治性癫痫早期外科治疗的必要性及术后疗效进行探讨。     

难治性癫痫的外科治疗及术前评估

癫痫是危害较大的神经系统常见病,其中约30%~40%属于难治性癫痫,近年来随着术前评估及外科手术技巧不断改进,特别是显微外科技术的应用,外科治疗尤其是对颞叶内侧癫痫的治疗疗效满意。癫痫的术前评估至关重要,手术效果与术前致痫灶定位准确与否密切相关。癫痫术前评估包括非侵袭性评估(I期)和侵袭性评估(II期),随着新的诊断技术的飞速发展,术前评估通过脑电图、脑磁图、CT、MRI、磁共振波谱、功能MRI成像、单光子发射计算机断层扫描和正电子发射计算机断层扫描等以确定病人致痫灶。癫痫手术方式主要有颞叶切除术、选择性杏仁核海马切除术、胼胝体切开术、大脑半球切除术等。本文就癫痫手术适应证、术前评估、手术方式等予以综述。     

难治性癫痫手术治疗进展

磁共振成像技术与电生理技术为难治性癫痫的诊断提供了有力的技术支持。神经导航技术、手术中磁共振成像技术等提高了操作的准确性。其他新技术例如软膜下多重横切术、迷走神经直接或间接刺激术也有效应用于临床。通过研究经典神经外科切除手术与采用新技术在治疗上的差异,分析了目前难治性癫痫手术的治疗结果,证实手术治疗对于药物难治性癫痫患者是安全有效的。     

药物难治性癫痫MRI表现与脑电图及病理对照研究

目的 探讨药物难治性癫痫发作间期MRI与脑电图(EEG)在致痫灶定位中的相关性以及MRI表现的病理学基础.方法 对40例药物难治性癫痫患者术前行传统MRI及视频EEG检查,并对其中25例颞叶癫痫患者行弥散加权像扫描.手术方式包括标准颞前叶切除术、选择性海马杏仁核切除术及致痫灶切除术等,对切除的脑组织进行病理学检查.结果 33例患者传统MRI检查结果显示的异常包括:海马硬化(16例)、脑发育不良(5例)、颞极蛛网膜囊肿(3例)、颅内肿瘤(3例)、脑软化灶(2例)、脉络膜裂囊肿(2例)、脑软化合并颞极蛛网膜囊肿(1例)和小脑萎缩(1例).发作间期颞叶癫痫患者发作侧海马表观弥散系数(apparent diffusion coefficient,ADC)值[(102.1±4.7)×10-5 cm2/s]较对侧[(84.6±5.9)×10-5 cm2/s]明显升高(t=12.7,P<0.01).所有癫痫患者发作间期EEG均呈异常改变.24例患者传统MRI检查所示病变区与发作间期EEG定位的致痫灶部位一致,4例患者传统MRI检查显示正常而其发作侧海马ADC值却较对侧增高.结论 传统MRI与ADC同时用于评价致痫灶优于任一单独应用者,如结合EEG将更有助于对癫痫的术前评价.     

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。     

难治性癫痫持续状态的药物治疗进展

难治性癫痫持续状态(RSE)是指使用常规抗癫痫治疗不能控制的持续癫痫发作超过1h的状态,有效控制癫痫发作是挽救患者生命、改善其预后的关键。此文回顾分析了2003-2005年国内外治疗RSE的有关文献,将其所用药物用法、用量及作用机制做一综述。     

难治性癫痫:治疗与展望

癫痫是由于大脑神经元过度兴奋,突然异常反复放电,导致大脑功能障碍所引发的临床表现复杂多样的慢性神经系统疾病。经药物或手术治疗后仍无法得到有效控制可发展为难治性癫痫,其机制复杂。不受控制的癫痫发作是危险的,不仅可导致认知、行为退化,还会增加死亡的风险。近年来关于难治性癫痫治疗的研究已取得一定成果。本文将对近年来有关难治性癫痫的相关药物和非药物治疗方法进行综述,以期为临床诊治提供新思路。     

脑磁图定位顽固性癫痫手术治疗51例临床分析

目的　探讨脑磁图在顽固性癫痫术前评估的价值。方法　术前对 5 1例顽固性癫痫患者行脑磁图、磁共振及视频脑电检查 ,结合术中皮层脑电图分别进行选择性海马杏仁核切除术、标准前颞叶切除术及致痫灶切除术等手术 ;并用脑磁图定位神经导航下手术切除致痫灶 2例 ,神经导航下海马杏仁核切除 2例。结果　术后发作完全停止的占 76 5 % ,发作减少 >75 %的占 11 8% ,发作减少>5 0 %的占 5 8% ,发作减少不足 5 0 %的占 5 8% ;脑磁图与视频脑电及皮层脑电图的定位符合率分别为 82 %和 96 % ;脑磁图对内侧颞叶癫痫的定位敏感性较差 ;脑磁图定位导航手术可在切除致痫灶的同时保护脑的重要结构。结论　脑磁图是一项灵敏的无创性癫痫灶定位方法 ,是癫痫外科术前评估中的一项技术突破     

High-resolution MRI enhances identification of lesions amenable to surgical therapy in children with intractable epilepsy

PURPOSE: Many children with refractory epilepsy can achieve better seizure control with surgical therapy. An abnormality on magnetic resonance imaging (MRI), along with corroborating localization by other modalities, markedly increases chances of successful surgical outcome. We studied the impact of high-resolution MRI on the surgical outcome of intractable epilepsy. METHODS: High-resolution MRI using four-coil phased surface array was obtained as part of the comprehensive presurgical protocol for children with focal onset intractable seizures evaluated by our epilepsy center during the first half of 2002. RESULTS: Thirteen consecutive children, ages 5 to 18 years, entered this prospective study. For four patients with a lesion on a recent MRI examination with a standard head coil, management did not change with high-resolution MRI. Standard MRI in the other nine patients did not identify a lesion. However, high-resolution MRI with the phased-array surface coil found previously undiagnosed focal abnormalities in five of nine patients. These abnormalities included hippocampal dysplasia, hippocampal atrophy, and dual pathology with frontal cortical dysplasia. In four of nine patients, no identifiable lesion was identified on the high-resolution MRI. All patients underwent invasive monitoring. In three of five patients, newly diagnosed lesions correlated with EEG abnormalities, and resection was performed. CONCLUSIONS: In our center, high-resolution MRI identified lesions not detected by standard MRI in more than half the children (56%). Technical advances such as four-coil phased surface array MRI can help identify and better delineate lesions, improving the diagnosis of patients who are candidates for surgical treatment of refractory epilepsy.     

多种手术结合治疗顽固性癫痫

目的：对部分复杂的顽固性癫痫病人，将几种手术结合使用，并对其临床效果进行评价。方法：北京天坛医院手术治疗顽固性癫痫230例，其中对15例顽固性癫痫病人采用几种术式结合的方法。病人年龄9-43岁，平均18岁。病程4-40年，平均13年。发作形式为强直阵挛性发作、复杂部分性发作及失神，其中有8例存在两种发作形式。术前均服过抗癫痫药，病程后期同时服用2-3种抗癫痫药。这15例病人术前均行头皮脑电视频连续监测及头MRI检查，其中5例行头PET检查。手术在皮层脑电及深部电极监测下进行。13例行额颞开颅，胼胝体前部切开 颞前叶及海马切除 皮层热灼术；1例行额颞顶枕开颅，枕叶萎缩皮层切除 胼胝体前部切开 选择性海马切除 皮层热灼术；1全先行左额颞开颅，脚间池错构瘤及颞前叶及海马切除 皮层热灼术，术后癫痫减轻但仍发作且术后头皮脑电显示右额棘波明显，故又右额颞开颅，胼胝体前部切开 皮层热灼术。结果：术后无死亡及并发症。术手抗癫痫药物减少，术后12例服用一种抗癫痫药，3例服用两种抗癫痫药。术后随访3-12个月，14例无癫痫发作，1例癫痫发作两次（术前每天发作5-8次）。结论：多种手术结合治疗顽固性癫痫病人安全有效。     

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。     

Surgical treatment for epilepsy

Nearly one-third of patients with newly diagnosed epilepsy will develop medically refractory seizure disorders. The initial response to antiepileptic drug therapy is highly predictive of long-term outcome. Patients with intractable epilepsy may have a progressive disorder that is medically, physically, and socially disabling. Surgical resection of the epileptogenic zone or lesional pathology, or both, may significantly reduce seizure tendency in selected patients. The present review supports the position that early and effective epilepsy surgery may not only render the patient with intractable partial epilepsy seizure-free, but also allow the individual to become a participating and productive member of society. Patients with surgically remediable epileptic syndromes should be identified early in the evaluation and treatment of their seizure disorders. Favorable candidates for focal cortical resection include individuals with medial temporal lobe epilepsy and partial seizures related to selected lesional pathology, e.g. primary brain tumor or vascular anomalies. In conclusion, surgical treatment of intractable partial epilepsy has been shown to compare favorably to antiepileptic drug therapy. Individuals rendered seizure-free may experience a significant improvement in quality of life. Patients who fail to respond to initial antiepileptic drug therapy should be “triaged” to a presurgical evaluation. Ictal semiology combined with structural magnetic resonance imaging and the electroclinical correlation may permit identification of candidates for early and effective surgical treatment.     

Pediatric intractable epilepsy syndromes: reason for early surgical intervention

Drug-resistance in several childhood epilepsy syndromes is common, and these patients may tolerate epilepsy surgery. In this study, the surgical outcomes of 24 pediatric patients with various intractable epilepsy syndromes and three patients with tuberous sclerosis were examined at Xinqiao hospital between 1997 and 2004. The study included nine cases of Lennox-Gastaut syndrome, two cases of Rasmussen's syndrome, one case of Sturge-Weber syndrome, three cases of West syndrome, three cases of tuberous sclerosis and nine cases of mesial temporal lobe epilepsy syndrome. In each case, different surgical procedures were performed according to preoperative evaluation and ECoG. At an average of 4.5 years after surgery, 14 out of 27 patients (51.9%) had an Engel Class I outcome after surgery, and an additional eight patients (29.6%) had rare seizure (Engel ClassII). Three patients showed a significant decrease in seizure frequency (Engel Class III). The mean IQ increased from 61.4+/-12.2 to 75.0+/-11.0, and greater IQ increase was seen in patients with shorter seizure history and drug-resistance. Temporary complications were observed in four patients and there were no deaths. In conclusion, early surgical intervention in pediatric intractable epilepsy syndromes may results in a favorable outcome in a high percentage of cases and may provides an important opportunity to prevent irreversible decline in intelligence and disability.     

选择性海马切除在枕叶癫痫手术中的应用

目的 总结分析枕叶癫痫患者手术中联合选择性海马切除的手术治疗效果和经验.方法 回顾性分析2003年1月至2006年12月在我科接受手术治疗的20例向颢叶内侧传导的枕叶癫痫病例,统计患者的发病、检查及手术治疗情况,并介绍相关手术体会.17例(85%)为部分性发作继发强直.痉挛性发作为主,9例有颢叶先兆或口咽自动症,PET检查中9例有颞叶的代谢异常,MRI及MRS共发现有海马异常13例;脑电图确定癫痫灶位于枕叶外侧皮层8例.内侧皮层6例,枕颞交界区6例,发作期脑电图均显示明显向颢叶传导.手术均行枕叶致痫灶切除和经颞底海马结构切除术.结果 癫痫发作Engle分级示:I级16例,Ⅱ级2例,Ⅲ级2例.出现视野损害3例;推理智商无明显变化15例,下降4例,改善1例.结论 向颞叶传导为主的枕叶癫痫常可以有颢叶的先兆或自动症,并有影像学的异常,手术联合枕叶致痫灶切除和经颞底海马结构切除术,手术效果好.     

Outcome of surgical treatment in non-lesional intractable childhood epilepsy

PurposeThe aim of this study is to investigate seizure outcomes following epilepsy surgery in non-lesional neocortical pediatric epilepsy.MethodsWe performed a retrospective study on 27 patients with intractable childhood epilepsy who received epilepsy surgery between 1999 and 2006 at Sang-gye Paik Hospital and Severance Children's Hospital. None of the patients had any detectable lesions on MRI. Surgical outcome was assessed at least 2 years postoperatively; clinical characteristics, surgical outcome, pathologic findings, types of surgery, localizing features on SPECT, FDG-PET, and long-term video-EEG were reviewed.ResultsEighteen patients (67%) demonstrated an Engel class I outcome postoperatively. The mean follow-up duration was 4.3 years (range, 2.2–9 years). Eighteen out of 27 cases (67%) showed focal localizing features on ictal SPECT, and 21 of 27 cases (78%) showed abnormal findings on PET. Single lobectomy was the most common procedure, and was performed in 20 patients (74%). Multilobar resection was performed in seven patients (26%). Cortical dysplasia was the most common finding during pathological examination, and was observed in 15 (56%) cases. In addition, gliosis was found in two patients (7%) and non-specific pathological findings were described in 10 patients (37%).ConclusionsIn children with intractable epilepsy and a MRI demonstrating no abnormal lesions, epileptic surgery should be strongly considered when cortical pathology can be identified from other studies.     

Cortical resection with electrocorticography for intractable porencephaly-related partial epilepsy

PURPOSE: We evaluated the results of cortical resection of epileptogenic tissue for treatment of intractable porencephaly-related epilepsy. METHODS: We examined clinical features, electrophysiological data, surgical findings, and seizure outcomes after cortical resection in eight patients with intractable epilepsy related to porencephalic cysts. RESULTS: All eight patients had hemiparesis. Five retained motor function in the hemiparetic extremities; six retained visual fields. All had partial seizures, six with secondary generalization. Seven patients had simple and three had complex partial seizures (CPSs); two also had drop attacks. Four patients had multiple seizure types. Long-term scalp video-EEG (LVEEG) localized interictal epileptic abnormalities that anatomically corresponded to the cyst location in three patients. LVEEG recorded ictal-onset zones in five; these anatomically corresponded to the cyst location in three of the five. EEG recorded generalized seizures in two patients, hemispheric in one, and multifocal in two. Intraoperative electrocorticography (ECoG) revealed interictal epileptic areas extending beyond the margins of the cyst in seven patients. We resected ECoG-localized interictal epileptic areas completely in five patients and partially in two. Cortical resection was based on seizure semiology and LVEEG in one patient whose ECoG showed no epileptiform discharges. After a minimum follow-up of 1 year, six patients had excellent seizure outcome (Engel class I), and two had a >90% seizure reduction (Engel class III) without complications. CONCLUSIONS: Cortical resection guided by ECoG allows preservation of motor function and visual field and provides an effective surgical procedure for treatment of intractable epilepsy secondary to porencephaly.     

Pediatric intractable epilepsy: the role of presurgical evaluation and seizure outcome

Surgical experience with long-term follow-up is limited in childhood intractable epilepsy, compared with adult epilepsy. To assess the role of each presurgical evaluation modality and to identify prognostic factors for favorable seizure control after epilepsy surgery, 38 children with intractable epilepsy who underwent surgery were retrospectively reviewed. Among the available preoperative evaluation modalities, PET and neuropsychological testing showed the highest rates of positive results, whereas MRI was the most concordant with EEG findings. During a follow-up period of at least 12 months, 26 of the 38 patients showed favorable seizure control (Engel classifications I and II). The best seizure control was achieved in patients with a temporal resection and discrete lesion on magnetic resonance imaging. In spite of the invasive study, the less satisfactory results followed an extratemporal resection. We conclude that epilepsy surgery benefits children with intractable epilepsy and that the role of invasive study should be re-established according to the area of resection and presence of discrete lesion on MRI. Received: 23 June 1999 Revised: 7 August 1999