难治性癫过度运动发作的临床特征与致灶定位

目的总结以过度运动发作为主要表现的药物难治性癫的诊疗经验。方法回顾性分析25例药物难治性癫病人的临床资料,均以过度运动发作为主要表现。根据症状特点、MRI、头皮视频脑电图及颅内脑电图监测,综合定位致灶,并行手术切除。结果切除额叶致灶23例,切除前颞叶致灶2例。神经病理学检查显示:皮质发育不良(FCD)Ⅰ型8例,FCDⅡ型12例,节细胞胶质瘤1例,皮质微发育不良1例,灰质异位1例,胶质瘢痕1例,海马硬化1例。术后随访12~60个月,按Engel分级:Ⅰ级19例,Ⅱ级2例,Ⅲ级2例,Ⅳ级2例。结论以过度运动发作为特点的难治性癫病人,其致灶主要位于额叶,但也可位于额叶外的脑区,准确切除致灶后疗效满意。     

脑裂畸形继发难治性癫的微创外科治疗

目的探讨脑裂畸形继发难治性癫的致灶定位以及微创外科治疗方法。方法回顾性分析11例脑裂畸形继发难治性癫病人的临床资料,术前通过多模态神经影像和长程视频脑电图进行解剖与功能定位。在神经导航引导下使用皮质电极描记了解脑裂畸形病灶与癫波的关系,显微镜下将脑裂畸形的致灶切除。其中位于功能区的脑裂畸形,可使用功能MRI(fMRI)导航并辅以小功率皮质热灼。结果随访11例,时间12个月。术后癫发作完全消失9例,好转2例。结论多模态神经影像和长程视频脑电图可以对脑裂畸形继发的难治性癫进行致灶的解剖与功能定位,在保护脑功能的基础上将脑裂畸形的致灶切除是手术关键。     

皮层脑电监测下颞叶病变合并癫的手术治疗

目的探讨皮层脑电监测下合并颞叶病变的癫手术治疗效果。方法 21例伴有癫症状的颞叶病变患者,术中通过皮层脑电图确定癫灶,切除病变后,切除或热灼可疑癫疒间灶。术后随访患者的癫发作情况。结果 21例患者切除颞叶病变前均可通过皮层脑电图探及疒间波,病变及疒间灶完全切除后,癫波消失者19例,2例功能区患者虽多次皮层热灼,仍可见偶发棘波。术后20例未再有癫疒间发作,1例有部分性发作,用抗癫疒间药可控制。结论术中皮层脑电监测切除或热灼癫灶是一种有效控制颞叶病变切除术后癫发作的方法。     

颅内电极置入与脑功能区定位技术在功能区难治性癫疒间手术的应用

目的探讨颅内电极埋藏与脑功能区定位技术在功能区起始的难治性癫疒间手术的评估价值。方法回顾性分析7例难治性癫疒间的临床资料,头皮视频脑电图长程监测均考虑致疒间灶可能累及功能区,故行颅内电极置入术,再行皮质电极视频脑电图长程监测,记录发作间期和发作期脑电图,以判断致疒间皮质,并采用皮质电刺激行脑功能区定位,在指导术中尽可能切除致疒间皮质的同时,最大限度保护脑功能。结果癫疒间发作起始区切除2例,癫疒间起始区部分切除加周围皮质热灼5例。随访6个月~1年,术后运动及语言功能均保护良好6例,对侧肢体出现短暂运动障碍后恢复1例。结论颅内电极埋藏与脑功能区定位技术是功能区难治性癫疒间手术必要评估手段,有助于术前明确脑功能区和皮质放电区域,以及两者之间的关系,指导设计手术方式,最大限度提高病人术后生活质量。     

难治性额叶癫(癎)的外科治疗

目的:探讨额叶癫的外科治疗方法。方法:采用EEG、MRI、PET等方法对66例额叶癫灶定侧、定位,并据此进行手术治疗,术中采用皮质刺激以确定运动皮质。结果:所有患者获得定位,MRI和EEG分别对42例及46例作出定位诊断。66例均接受手术治疗:其中病灶切除+癫灶切除42例;癫灶切除11例;癫灶切除+软脑膜下横切术7例;癫灶切除+胼胝体切开术6例。术后癫控制:满意39例,显著改善15例,良好5例,效差7例。无偏瘫、失语等功能障碍。结论:手术治疗额叶癫可取得较满意疗效,EEG和MRI是主要的定位手段,术中采用皮质电刺激可避免运动功能损伤。     

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。     

脑功能区病变继发性癫的外科治疗(附41例分析)

目的探讨功能区病变继发性癫手术治疗的原则及方法。方法回顾性分析41例手术治疗的功能区病变继发性癫病例,总结其诊断原则、功能皮质定位方法、手术方式选择以及预后等。行常规开颅病灶切除14例,立体定向或神经导航下病灶切除7例,颅内电极置入皮质功能定位后病灶切除15例,术中唤醒皮质功能定位后病灶切除5例;其中病灶切除后仍有灶放电者加行皮质双极电凝热灼11例。结果术后无语言及运动功能障碍加重。随访1年以上,EngelⅠ级34例(82.9%),EngelⅡ级7例(17.1%)。结论在功能区病变继发性癫手术治疗中,对功能皮质定位的重要性以及复杂程度往往超过手术本身。通过综合应用各种功能性检查对脑功能区做出正确的定位,是手术成功的关键。     

双极电凝热灼联合病灶切除治疗涉及功能区癫癎的疗效观察

目的观察双极电凝热灼功能区致灶联合非功能区致灶切除治疗涉及功能区癫癎病人的疗效。方法回顾性分析40例致灶涉及功能区的癫癎病人的临床资料,行功能区致癎皮质电凝热灼及非功能区致癎灶切除。结果术后随访18-48个月,平均25个月。5例病人术后对侧有轻度的偏瘫,2例病人术后有轻度的感觉性失语,所有并发症均在1年内恢复。术后观察疗效：EngelⅠ级18例（45%）,EngelⅡ级8例（20%）,EngelⅢ级8例（20%）,EngelⅣ级6例（15%）。结论脑皮质电凝热灼术是治疗功能区癫癎有效且安全的方法,通过病灶切除联合脑皮质电凝热灼术治疗涉及功能区的癫癎,可取得良好的疗效。     

脑电偶极子定位在癫外科的应用

脑电偶极子定位(dipole localization)是利用头皮脑电推算颅内异常放电位置的技术。结合病人的薄层MRI扫描及计算机的影像融合技术,偶极子能精确定位性病灶的解剖位置及与功能区的关系。癫外科据此可进行术前评估、手术入路设计、切除范围测算等。并结合神经导航技术或立体定向技术,设计最佳手术入路,达到以最小的创伤切除癫起源灶,并提高手术疗效的目的。     

神经导航结合术中皮质电刺激在局灶性皮质发育不良癫手术中的应用

目的探讨神经导航结合术中皮质电刺激(CES),在局灶性皮质发育不良(FCD)癫手术中的应用。方法对18例FCD癫病人行MRI薄层扫描及血氧水平依赖性功能磁共振成像(BOLD-fMRI)扫描,结合神经导航引导手术入路,CES定位运动区,皮质脑电图定位致灶,行致灶加病灶切除。结果术后病人肢体运动功能均与术前相同,病理结果均为FCD。术后随访1年,Engel分级:Ⅰ级14例,Ⅱ级2例,Ⅲ级1例,Ⅳ级1例。复查脑电图较术前明显改善,棘波、尖波基本消失。结论神经导航结合BOLD-fMRI、CES及皮质脑电图,在FCD癫手术中定位准确,减少运动功能损伤,达到癫灶切除及最大程度保护运动功能的目的。     

What is needed for resective epilepsy surgery from a neurosurgical point of view?

Invasive versus non-invasive epileptogenic zone definition was analyzed in a series of 89 patients operated on for drug-resistant epilepsy. In the group of 69 cortical resections, 26% needed invasive recordings, 13.5% when foreign-tissue lesions had been detected by MRI and 32% when were absent. In this last group temporal resections had invasive EEG recordings in 23.5% versus 54.5% when the epileptogenic zone was extratemporal. In a group of 43 temporal resections with more than one year follow-up MRI has detected some abnormality in 84%. Excluding focal lesions, MRI detected hippocampal/temporal lobe atrophy in 66% of the cases in agreement with other noninvasive tests and in 4% contralateral to the epileptogenic zone located by subdural strips. The outcome analysis showed that 85% of the patients with MRI-EEG agreement were seizure free in contrast to only 43% when MRI was non-lateralizing. Future studies has to be oriented to better understand the epileptic process of patients without MRI abnormalities.     

Lesion‐negative anterior cingulate epilepsy

MRI‐negative anterior cingulate epilepsy is a rare entity. Herein, we describe a case of MRI and functional imaging‐negative intractable frontal lobe epilepsy in which, initially, secondary bilateral synchrony of surface and intracranial EEG and non‐lateralizing semiology rendered identification of the epileptogenic zone difficult. A staged bilateral stereotactic EEG exploration revealed a very focal, putative ictal onset zone in the right anterior cingulate gyrus, as evidenced by interictal and ictal high‐frequency oscillations (at 250 Hz) and induction of seizures from the same electrode contacts by 50‐Hz low‐intensity cortical stimulation. This was subsequently confirmed by ILAE class 1 outcome following resection of the ictal onset and irritative zones. Histopathological examination revealed focal cortical dysplasia type 1b (ILAE Commission, 2011) as the cause of epilepsy. The importance of anatomo‐electro‐clinical correlation is illustrated in this case in which semiological and electrophysiological features pointed to the anatomical localization of a challenging, MRI‐negative epilepsy. [Published with video sequence]     

Insular and insulo-opercular epilepsy in childhood: An SEEG study

PurposeIn recent years, there have been series analysing the electro-clinical correlations of insular epilepsy in adult populations. In contrast, the ictal semiology in children with insular epilepsy is poorly described. Considering that early and successful surgery may greatly improve the cognitive outcome and quality of life, it is worthwhile to deepen our knowledge of insular epilepsy in children.MethodsWe retrospectively evaluated ten children with drug-resistant focal insular epilepsy who had been consecutively explored with stereoelectroencephalography (SEEG), followed by individually tailored resective surgery that included part of the insula in all cases. A detailed anatomo-electro-clinical analysis of non-invasive EEG and SEEG data was performed. At least one of the electrodes explored the insular cortex. SEEG analysis confirmed that the insular cortex was included in the ictal onset zone.ResultsEpilepsy onset was mostly during the first year of life, characterized by subtle seizures as well as spasms and myoclonic seizures. Later on, neurovegetative signs and asymmetric tonic and hypermotor seizures (HMS) dominated the ictal semiology. The epileptogenic zone was frequently wider than insular with frontal and central predominance. In eight patients, the tailored resection included a lesion. In seven patients, an Engel class 1 outcome as well as neuropsychological and behavioural improvement was obtained.ConclusionsSEEG is feasible and useful in children with drug-resistant insular epilepsy which is often characterized by autonomic symptoms as the initial symptoms and should be suspected in cases with HMS, asymmetric tonic seizures and even asymmetric spasms. Early propagation is mostly frontal and central. Analysis of a larger population is required to refine these findings.     

Minimally resective epilepsy surgery in MRI‐negative children

Aim. Performing epilepsy surgery on children with non‐lesional brain MRI often results in large lobar or multilobar resections. The aim of this study was to determine if smaller resections result in a comparable rate of seizure freedom. Methods. We reviewed 25 children who had undergone focal corticectomies restricted to one aspect of a single lobe or the insula at our institution within a 5.5‐year period. Data collected in the comprehensive non‐invasive pre‐surgical evaluation (including scalp video‐EEG, volumetric MRI, functional MRI, EEG source localization, and SPECT and PET), as well as from invasive recordings performed in each patient, was reviewed. Data from each functional modality was identified as convergent or divergent with the epileptogenic zone using image coregistration. Specific biomarkers (from extra‐operative and invasive testing) previously indicated to be indicative of focal epileptogenicity were used to further tailor each resection to an epileptogenic epicentre. Tissue pathology and postoperative outcomes were obtained from all 25 patients. Results. Two years postoperatively, 15/25 (60%) children were seizure‐free, three (12%) experienced >90% reduction in seizure frequency, two (8%) had a 50–90% reduction in seizure frequency, and the remaining five (20%) had no change in seizure burden. There was no significant difference in outcome based on numerous pre‐ and postoperative factors including location of resection, the number of preoperative functional tests providing convergent data, and tissue pathology. Conclusion. In MRI‐negative children with focal epilepsy, an epileptogenic epicentre within a larger epileptogenic zone can be identified when specific biomarkers are recognized on non‐invasive and invasive testing. When such children undergo resection of a small, well‐defined epileptogenic epicentre, favourable outcomes can be achieved.     

Identification of Frontal Lobe Epileptic Foci in Children Using Positron Emission Tomography

Summary: Purpose: Presurgical evaluation for intractable frontal lobe epilepsy (FLE) is difficult and invasive, partly because anatomic neuroimaging studies with computed tomography (CT) and magnetic resonance imaging (MRI) typically do not show a discrete lesion. In adult patients with FLE, functional neuroimaging of glucose metabolism with positron emission tomography (PET) is less sensitive in detecting focal metabolic abnormalities than in temporal lobe epilepsy (TLE). Comparable data on children with FLE are not available. Methods: We used high-resolution PET scanning of glucose metabolism to evaluate 13 children (age 17 months to 17 years; mean age 9.5 years) with intractable FLE being considered for surgical treatment. Only children with normal CT and MRI scans were included. Results: Hypometabolism including the frontal lobe was evident in 12 of the 13 children, was unilateral in 11 of 13, and was restricted to the frontal lobe in 8 of 13. One child showed bilateral frontal cortex hypometabolism and another had anictal PET scan demonstrating unilateral frontal cortex hyper-metabolism surrounded by hypometabolism. Additional hypo–metabolic areas outside the frontal cortex were observed in 5 children in parietal and/or temporal cortex. Localization of seizure onset on scalp EEG was available in 10 children and corresponded to the location of frontal lobe PET abnormality in 8. However, in 4 of the 10 children, the extent of hypometabolism exceeded the epileptogenic region indicated by ictal EEG. In 2 of the 13 children, the abnormality evident on EEG was more extensive than that evident on PET. In the remaining 3 children for whom only interictal EEG data were available, the PET foci did not correspond in location to the interictal EEG abnormalities. In 11 of the 13 children, the presumed region of seizure onset in the frontal lobe, as based on analysis of seizure semiology, corresponded to the locations of frontal lobe glucose metabolism abnormalities. Conclusions: Although high-resolution PET appears to be very sensitive in localizing frontal lobe glucose metabolic abnormalities in children with intractable FLE and normal CT/ MRI scans, the significance of extrafrontal metabolic disturbances requires further study; these may represent additional epileptogenic areas, effects of diaschisis, seizure propagation sites, or secondary epileptogenic foci.     

Revisiting the role of the insula in refractory partial epilepsy

Purpose:   Recent evidence suggesting that some epilepsy surgery failures could be related to unrecognized insular epilepsy have led us to lower our threshold to sample the insula with intracerebral electrodes. In this study, we report our experience resulting from this change in strategy.
Methods:   During the period extending from October 2004 to June 2007, 18 patients had an intracranial study including 10 with insular coverage. The decision to sample the insula with intracerebral electrodes was made in the context of (1) nonlesional parietal lobe-like epilepsy; (2) nonlesional frontal lobe-like epilepsy; (3) nonlesional temporal lobe-like epilepsy; and (4) atypical temporal lobe-like epilepsy.
Results:   Intracerebral recordings confirmed the presence of insular lobe seizures in four patients. Cortical stimulation performed in 9 of 10 patients with insular electrodes elicited, in decreasing order of frequency, somatosensory, viscerosensory, motor, auditory, vestibular, and speech symptoms.
Discussion:   Our results suggest that insular cortex epilepsy may mimic temporal, frontal, and parietal lobe epilepsies and that a nonnegligeable proportion of surgical candidates with drug-resistant epilepsy have an epileptogenic zone that involves the insula.     

顽固性癫癎病人的术前综合评估(附40例报告)

目的探讨常规脑电图(REEG)、视频脑电图(VEEG)、MRI、SPECT检查对癫病人术前综合评估致灶定位的意义。方法对40例顽固性癫病人进行REEG、VEEG、MRI及SPECT检查,其中32例行手术治疗,术中行皮质电极(ECoG)和深部电极监测,同时对REEG、VEEG、MRI、SPECT定位致灶的情况进行对比研究。结果REEG异常40例,局灶棘波17例(42.5%);VEEG异常40例,局灶棘波35例(87.5%),两组局灶棘波检出率有显著性差异(P <0.01)。SPECT异常32例(80%),MRI异常31例(77.5%)。在32例手术病人中,VEEG与ECoG病灶一致者30例(93.7%),MRI与ECoG一致者27例(84.3%)。结论VEEG、MRI、EEG、SPECT检查结合临床表现,对顽固性癫病人术前致灶的定位和指导手术治疗有较大的应用价值。     

Paediatric epilepsy surgery in the posterior cortex: a study of 62 cases

Past surgical series have emphasized the diagnostic complexity of posterior cortex epilepsy. Available data are sparse, especially in children, and most published series report a high number of surgical failures and post‐operative neurological deficits. In this article, we present a paediatric cohort of 62 children who underwent surgery for drug resistant posterior cortex epilepsy before the age of 16 years with a mean post‐operative follow‐up of 6.94 years (range: 2–16). Mean age at epilepsy onset was 3.2 years and 28 children (45%) had onset before 1 year of age. The mean age at surgery was 7.9 years (range: 1–16). Daily seizures were present in 63% of children. MRI was positive in 58 cases (93.5%) and invasive stereo‐EEG was judged mandatory in 24/62 (39%) of patients. Surgery was confined to the parietal lobe in 11 children, the occipital lobe in 8, the occipito‐parietal region in four, the occipito‐temporal region in 18, and involved both the temporal and parietal lobes in the remaining 21. Following surgery, 53 subjects (85.5%) remained seizure‐free and among those who underwent a SEEG procedure, 75% achieved seizure freedom. Focal cortical dysplasia was the most frequent histopathological diagnosis (50%), followed by tumoural (24%) and gliotic lesions (14.5%). An older age at epilepsy onset, the presence of a rather restricted epileptogenic area, and a complete resection of the epileptogenic zone were predictive of a favourable surgical outcome. These results demonstrate that a good surgical outcome is possible in children with drug resistant posterior cortex epilepsy. Accurate analysis of the chronology of ictal semiology and electrophysiological features, viewed in the context of the complete electroclinical pattern, provides a topographical orientation for posterior cortex epilepsy and, together with the presence of a lesion detectable on imaging, may improve the rate of surgical success of posterior cortex epilepsy at paediatric age.     

Pre-surgical evaluation and surgical treatment in children with extratemporal epilepsy

Introduction This review summarizes some patterns of pre-surgical evaluation and surgical treatment of extratemporal epilepsy in pediatric patients with medically refractory seizures, whose ictal behavior is variable. The most effective treatment for intractable partial epilepsy is a focal cortical resection with excision of the epileptogenic zone (the area of ictal onset and initial seizure propagation). This might be risky, though, in the case of a widespread lesion, sometimes encroaching one or more lobes, given the risk to the functional cerebral cortex. An anterior temporal lobectomy might prove more effective then in preventing seizures with fewer potential complications. If partial extratemporal epilepsy is associated with pharmaco-resistant seizures, the preoperative evaluation and operative strategy are determined according to the epileptogenic zone and to the relationship between a substrate-directed disorder and eloquent areas. The pediatric treatment of extratemporal epilepsy is aimed at controlling the seizures, avoiding morbidity, and improving the patient’s quality of life through psychosocial integration. Since the immature brain is more plastic than when mature, the recovery of functions after surgery is greater in children than in adults.Recommendation Early surgery is recommended for children with intractable epilepsy, and is now accepted as an important therapeutic modality also for children with chronic epilepsy.Conclusion Technological advances in the last two decades, mainly in neuroimaging, have led many medical centers to consider surgical treatment of epilepsy, accuracy being granted by MRI-based neuronavigation systems—an interface between the lesion seen in the preoperative magnetic resonance imaging (MRI) and the operative field, often invisible to the surgeon.     

Parahippocampal epilepsy with subtle dysplasia: A cause of “imaging negative” partial epilepsy

Purpose: Lesion‐negative refractory partial epilepsy is a major challenge in the assessment of patients for potential surgery. Finding a potential epileptogenic lesion simplifies assessment and is associated with good outcome. Here we describe imaging features of subtle parahippocampal dysplasia in five cases that were initially assessed as having imaging‐negative frontal or temporal lobe epilepsy. Methods: We analyzed the clinical and imaging features of five patients with seizures from the parahippocampal region. Results: Five patients had subtle but distinctive magnetic resonance imaging (MRI) abnormalities in the parahippocampal gyrus. This was a unilateral signal abnormality in the parahippocampal white matter extending into gray matter on heavily T₁‐ and T₂‐weighted images with relative preservation of the gray–white matter boundary on T₁‐weighted volume sequences. Only one of these patients had typical electroclinical unilateral temporal lobe epilepsy (TLE); one mimicked frontal lobe epilepsy, two showed bitemporal seizures, and one had unlocalized partial seizures. All have had surgery; four are seizure‐free (one has occasional auras only, follow‐up 6 months to 10 years), and one has a >50% seizure reduction. Histopathologic evaluation suggested dysplastic features in the surgical specimens in all. Discussion: In patients with lesion‐negative partial epilepsy with frontal or temporal semiology, or in cases with apparent bitemporal seizures, subtle parahippocampal abnormalities should be carefully excluded. Recognizing the MRI findings of an abnormal parahippocampal gyrus can lead to successful surgery without invasive monitoring, despite apparently incongruent electroclinical features.