儿童颅内海绵状血管瘤并发癫痫的手术治疗

目的明确儿童颅内海绵状血管瘤并发癫痫的手术治疗效果。方法回顾我院从2010年2月至2014年5月经手术治疗27例儿童颅内海绵状血管瘤并发癫痫患儿的病例资料。分析儿童海绵状血管瘤的临床特点,随访患者手术后癫痫缓解情况。手术后癫痫治疗情况用Engle’s分级进行评估,并对手术效果及预后进行分析。结果所有患者均行手术切除。术后1例基底节区病变切除后出现对侧肢体偏瘫,经治疗后到随访时神经功能已恢复至术前状态。1例术后出现颅内感染,经治疗后好转出院。手术后癫痫缓解良好,Engle’s分级1级25例,2级1例,3级1例。患者中无死亡及严重并发症患者。结论儿童颅内海绵状血管瘤并发癫痫手术治疗可有效治疗癫痫,并可避免海绵状血管瘤再次出血而改善患者预后。对多发病变及长期癫痫患者术前需充分评估。     

外科治疗对癫痫患儿社会能力及行为问题的影响

目的通过对癫痫患儿手术前后行为学测评,探讨外科手术对癫痫患儿行为学的影响。方法用儿童行为量表(CBCL)对2006至2010年手术治疗的92例癫痫患儿进行术前及术后1年、3年、5年以上的行为测评,分别用组平均值及个体水平进行分析。结果无论组间均值还是个体水平,大部分患儿的社会能力及行为问题在术后得到明显改善。结论外科手术对大部分癫痫患儿的社会能力和行为问题有明显的改善作用,对癫痫患儿的术前评估有一定的指导作用。     

迷走神经刺激术对学龄期难治性癫痫患儿认知效率的影响

目的探讨迷走神经刺激术对学龄期难治性癫痫患儿认知效率的影响。方法选取2016年1月至2018年1月安徽省立医院神经外科收治的35例学龄期难治性癫痫患儿,通过术前多模态神经影像评估和神经电生理监测方法明确诊断后行迷走神经刺激术,分别于术前7天、术后12个月采用韦氏儿童智力量表第四版中文版(WISC-Ⅳ)对患儿认知效率进行评估,评价手术前后不同时期患儿认知效率改变,同时采用McHugh疗效分级对术后疗效进行评估。选取30例匹配的健康学龄期儿童作对照,同期用WISC-Ⅳ测试其认知效率,评价前后改变情况并与癫痫组作比较。结果 35例学龄期难治性癫痫患儿VNS术后随访12个月,McHugh疗效分级评估中,Ⅰ-Ⅱ级25例,认知效率分值提高(9.56±2.42)分,Ⅲ级7例,分值提高(4.23±1.34)分,Ⅳ-Ⅴ级3例,分值提高(1.87±0.56)分,各分级认知效率分值提高差异有统计学意义(P0.05)。VNS术后患儿认知效率增加分值(8.67±1.78)较对照组(1.01±0.43)高,差异有统计学意义(P0.05)。结论迷走神经刺激术治疗学龄期儿童难治性癫痫安全有效,可减少患儿癫痫发作频率,同时改善患儿认知效率,且患儿癫痫发作控制效果越好,认知效率改善越明显。     

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。     

有关癫痫的手术治疗问题

介绍了有关癫痫手术的进展、术前的选择标准、术前评估、手术方式及治疗效果。     

儿童难治性癫痫手术前后对认知功能的影响

目的探讨难治性癫痫患儿外科手术治疗前后认知功能的变化。方法 27例儿童难治性癫痫患儿通过结构功能影像学、头皮长程视频脑电图(VEEG),需要时行侵入性脑电图(iEEG)等全面评估后个体化选择癫痫外科手术,于术前7天、术后3个月、6个月及12个月分别采用韦氏儿童智力量表第四版中文版(WISC-Ⅳ)测试患儿认知功能,评价手术前后认知功能,术后采用Engel分级评价手术治疗效果。结果 27例难治性癫痫儿童,术后随访12～36个月(平均随访22个月),17例患儿术后完全无癫痫发作,5例患儿术后发作极少,1年中仅1次,发作频率减少超过90%有3例,2例癫痫发作控制改善不明显,发作频率及症状未见明显改善。27例患儿配合完成了韦氏儿童智力量表第四版中文版(WISC-Ⅳ)测验,癫痫外科手术治疗后12个月患儿智商相比术前增加2.00～12.00(6.70±2.45)分(P0.001),认知功能改善有统计学意义,且开颅手术与迷走神经刺激术对患者认知功能影响的差异之间无统计学意义(P0.05)。结论癫痫外科手术对于难治性癫痫儿童安全且有效,可控制或减少术后发作频率,难治性癫痫儿童术后认知功能有不同程度提高。     

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.     

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。     

晚期外伤性癫痫的手术治疗

目的 总结45例晚期外伤性癫痫患者手术治疗的经验。方法 全部病例术前进行详细评估，术中采用皮质脑电监测，手术方式包括致痫灶切除术、前颞叶切除术、前颞叶及海马切除术和皮层热灼术和胼胝体切开术。结果 术后随访结果表明患者的癫痫发作改善明显，按照Engel分级，I级23例，Ⅱ级15例，Ⅲ级7例，无Ⅳ级的患者。结论晚期外伤性癫痫的患者如果经过系统的内科治疗无效，可以考虑外科治疗。准确的术前评估、恰当的手术方式及术中皮层脑电监测是提高手术疗效的关键。     

大脑半球切开术治疗儿童顽固性癫痫

目的探讨大脑半球切开手术治疗儿童顽固性癫痫的临床效果。方法回顾性分析7例大脑半球性病变致癫痫的患儿的临床资料。患儿综合评估后行大脑半球切开术,手术经外侧裂环岛沟入路,癫痫控制效果以Engel标准评判,疗效以最后1次随访时间为准。结果 7例患儿经6个月~2.5年的随访,癫痫控制均为EngelⅠ级,均无明显并发症;术后对侧肢体感觉、运动功能无减退,认知及生活能力较术前有不同程度的改善。结论大脑半球切开术治疗儿童半球性病变致癫痫的疗效确切,安全、有效。     

Epilepsy surgery in infancy

Surgery for intractable epilepsy is being offered at progressively younger ages, including infancy. The most common causes of catastrophic epilepsy in very young surgical candidates are focal malformations of cortical development and low-grade tumors. Additional causes include Sturge-Weber syndrome, epidermal nevus syndrome, hemimegalencephaly, and prenatal or perinatal infarction. Many infants manifest with focal seizures, whereas some patients have infantile spasms in the setting of a focal epileptogenic lesion. Video electroencepholography, magnetic resonance imaging, and positron emission tomography are critical investigations to explore surgical options. In small series, the percentage of infants free of seizures after surgery was in the range of 60%. This is similar to that seen after epilepsy surgery in older children, adolescents, and adults. However, larger series with long-term follow up will be important. Furthermore, the extensive procedures required in infants for removal of the epileptogenic developmental lesions entail some risk, and should not be offered in the absence of severe epilepsy. Most infant candidates for epilepsy surgery have significant developmental delay. Few data are available, but anecdotal experience suggests that surgical relief of catastrophic epilepsy may result in resumption of developmental progression. For each infant, the timing of surgery must be carefully considered based on full assessment of the relative risks and benefits, derived from a detailed presurgical evaluation.     

Thursday July 6, 2006Thursday July 6, 20067:30–9:00Hall 5ATeaching SessionEpilepsy surgery in infancy and childhood

¹ I. Tuxhorn (   ¹ Epilepsy Center Bethel, Germany )
Epilepsy surgery is no longer a treatment of last resort for children with drug resistant focal epilepsy. It should be considered early to reduce the negative impact of epilepsy on developmental and social outcome.
The spectrum of surgically remediable epilepsy syndromes of childhood has expanded in recent years to include 1) patients with tuberous sclerosis who have a leading resectable focus, 2) infantile spasms or Lennox-Gastaut syndrome (or other age specific epileptic encephalopathies) secondary to a resectable epileptogenic zone and 3) formerly "cryptogenic" cases with very subtle cortical dysplasias.
The goal of the presurgical evaluation is to identify patients who have surgically treatable epilepsy by assessing the risk benefit scenario of surgical intervention.
The accepted standards of evaluation include seizure analysis with video monitoring and scalp recording of the interictal and ictal EEG, high resolution MRI, neuropsychology, WADA test for assessing laterality of mnestic and language functions. Functional imaging including PET, ictal SPECT, postprocessing MRI techniques and fMRI are important techniques to evaluate difficult extratemporal cases.
The role of invasive neurophysiology with subdural, epidural and intracerebral recordings is changing since the advent of functional imaging of the epileptogenic region e.g., EEG triggered MRI and methods outlined above.
Patients with a focal electroclinical syndrome and a congruent MRI lesion are excellent candidates for selection and the seizure outcome prognosis may be considered high if the epileptogenic zone is completely resectable.
In pediatric epilepsy early patient referral for presurgical evaluation should be considered in specific syndromes—catastrophic focal epilepsy, hemispheric syndromes, Sturge Weber syndrome, and well localized symptomatic epilepsies.     

Surgical treatment of two patients with infantile spasms in early infancy

Early surgery for catastrophic epilepsy may prevent developmental deterioration and minimize functional deficit resulting from the surgical procedure. We report two patients who had infantile spasms and underwent surgery for epilepsy in early infancy (younger than 6 months old). Magnetic resonance imaging showed cortical dysplasia in both patients. One patient underwent lesionectomy in the right perirolandic area at 49 weeks conceptional age (CA) and the other infant underwent left temporo-occipital disconnection at 45 weeks CA. Both showed Engel classification I and catch-up developmental progress. However, with the second patient, we were concerned about the high volume transfusion relative to her body weight during the extensive disconnection because of the patient's small blood volume. We discuss the benefits and potential risks of surgical treatment for intractable epilepsy in early infancy.     

Surgical Management of Pediatric Epilepsy: Decision-Making and Outcomes

First-line treatment for epilepsy is antiepileptic drug and requires an interdisciplinary approach and enduring commitment and adherence from the patient and family for successful outcome. Despite adherence to antiepileptic drugs, refractory epilepsy occurs in approximately 30% of children with epilepsy, and surgical treatment is an important intervention to consider. Surgical management of pediatric epilepsy is highly effective in selected patients with refractory epilepsy; however, an evidence-based protocol, including best methods of presurgical imaging assessments, and neurodevelopmental and/or behavioral health assessments, is not currently available for clinicians. Surgical treatment of epilepsy can be critical to avoid negative outcomes in functional, cognitive, and behavioral health status. Furthermore, it is often the only method to achieve seizure freedom in refractory epilepsy. Although a large literature base can be found for adults with refractory epilepsy undergoing surgical treatment, less is known about how surgical management affects outcomes in children with epilepsy. The purpose of the review was fourfold: (1) to evaluate the available literature regarding presurgical assessment and postsurgical outcomes in children with medically refractory epilepsy, (2) to identify gaps in our knowledge of surgical treatment and its outcomes in children with epilepsy, (3) to pose questions for further research, and (4) to advocate for a more unified presurgical evaluation protocol including earlier referral for surgical candidacy of pediatric patients with refractory epilepsy. Despite its effectiveness, epilepsy surgery remains an underutilized but evidence-based approach that could lead to positive short- and long-term outcomes for children with refractory epilepsy.     

Nonpharmacologic Treatment of the Catastrophic Epilepsies of Childhood

Summary:  The catastrophic epilepsy syndromes of childhood are initially treated with a pharmacologic intervention in most cases. However, due to the poor response patients often have to pharmacologic interventions, nonpharmacologic treatment options are an important part of a comprehensive treatment plan for this group of children. Additionally, nonpharmacologic therapy may offer a method to minimize associated morbidity and mortality. This article discusses the use of epilepsy surgery, the ketogenic diet, and vagus nerve stimulation in the treatment of patients with infantile spasms, Lennox-Gastaut syndrome, and progressive myoclonic epilepsy. Efficacy of the nonpharmacologic treatment options, as measured by reduction in seizure frequency, as well as by developmental progress or behavioral improvement, varies according to the specific catastrophic epilepsy disorder and the treatment option.     

Surgical treatment of epilepsy in pediatric patients

Surgery has become an accepted treatment modality for carefully selected adults with intractable focal epilepsy. More recently, increasing numbers of pediatric patients with intractable epilepsy are also being referred for surgical consideration. Key elements of surgical candidacy include medically intractable focal epilepsy, a localized epileptogenic zone, and a low risk for new postoperative neurologic deficits. The most common etiologies of the epilepsies in pediatric surgical candidates are malformation of cortical development and low grade tumor but some patients with childhood onset temporal lobe epilepsy due to hippocampal sclerosis also present for early surgery. Based on results from several recent pediatric surgical series, the chance for favorable seizure outcome after surgery is not adversely affected by young age, with seizure-free postoperative outcome reported for 60% to 65% of infants, 59% to 67% of children, and 69% of adolescents, compared to 64% reported in a large, predominantly adult series. Some subgroups of patients have higher percentages of seizure-free outcome, including those with hippocampal sclerosis or low grade tumor. In addition to seizures, developmental issues are also a major concern in children with intractable epilepsy. Few quantitative data are available, but some anecdotal experience suggests that surgical relief of catastrophic epilepsy may result in resumption of developmental progression after surgery, although the rate of development often remains abnormal. In one series, best developmental outcomes were seen in patients with earliest surgery and highest level of preoperative development. For each patient, the timing of surgery must be carefully considered, based on a full assessment of the relative risks and benefits, derived from a detailed presurgical evaluation.     

Epilepsy surgery in tuberous sclerosis complex: early predictive elements and outcome

Aim  The aim of the study was to evaluate the surgical treatment of epilepsy and detection of possible early surgery predictive elements in patients with tuberous sclerosis complex (TSC). Materials and methods  Forty-two TSC patients with epilepsy were selected and divided into two main groups: definite and fruste forms. Definite forms were divided into different groups: patients with pharmacologically controlled epilepsy, patients with pharmacoresistant epilepsy excluded from surgery after an extensive presurgical assessment, and patients with a pharmacoresistant epilepsy who underwent surgery. We compared the definite TSC groups to identify elements that predict surgical candidacy. Second, we compared all operated patients to assess surgical outcome. Conclusion  We found several factors that could predict a surgical intervention even if identification of patients with refractory epilepsy who can benefit from surgery is an evolving process. Also, several positive factors for good surgical outcome were identified. Patients with the fruste form had excellent surgical outcome.     

儿童灾难性癫痫手术治疗的探讨(附10例报告)

目的探讨儿童灾难性癫痫的手术治疗。方法回顾性分析10例手术治疗的儿童灾难性癫痫患者资料，比较手术前后癫痫发作情况及智能情况。结果除1例脱失外，余随访12月以上，所有患者术后无并发神经功能损害和智能障碍的加重。疗效评定为癫痫控制满意4例，显著改善2例，良好2例，较差1例。行为异常、语言障碍术后均有改善和提高；与术前比较。术后智商提高不明显。结论积极的手术治疗不仅能有效地控制儿童灾难性癫痫的发作，还能改善部分智能障碍。     

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。     

Application of a Multidisciplinary Model to a Case Example of Presurgical Epilepsy Planning

This article presents a case example which illustrates the multidisciplinary model for presurgical assessment for epilepsy patients. Nearly three million people in the United States are diagnosed with epilepsy and more than one third of this population is refractory to pharmacological treatment. Poor seizure control is associated with additional impairment in quality of life and cognitive and social functioning, and even with premature death. In accordance with these concerns, surgical intervention is increasingly recognized as a viable treatment option, which should be considered soon after drug resistance becomes apparent. Despite the widespread evidence of effectiveness surgery is often delayed, in part because of the necessity, and difficulties, of correctly applying a multidisciplinary approach to presurgical assessment. And yet, a multidisciplinary team is crucial in the evaluation of risks and benefits of possible surgical intervention and in guiding the surgical procedure to maximize seizure control and minimize risk to eloquent cortex. In the model and complex case presented, the neuropsychologist has a critical role in the presurgical evaluation, as well as in the postsurgical evaluation of outcome.