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。     

Outcome and long term follow-up after corpus callosotomy in childhood onset intractable epilepsy

Introduction Epilepsy surgery is a standard of care in the treatment of medically intractable epilepsy. Twenty five percent of patients with intractable epilepsy in childhood can be candidates for epilepsy surgery. Corpus callosotomy is a surgical treatment option for patients with potentially injurious drop attacks and disabling generalized seizures. Postoperative improvement of cognition and speech are important gains after epilepsy surgery particularly during childhood. The aim of this study is to evaluate the outcome of corpus callosotomy for the treatment of childhood onset medically intractable epilepsy in a developing pediatric epilepsy surgery center.Method We report 16 patients who underwent two thirds anterior corpus callosotomy for treatment of refractory seizures in childhood.Results All patients had drop attacks or multiple types of seizures, yet some showed focal onset with secondary generalization on electroencephalogram (EEG). One patient was seizure free (class 1 outcome), five had class 2A outcome, five had class 2B outcome, and five had class 3 outcome. Overall 11/16 (69%) of our patients improved significantly after anterior callosotomy.Conclusion Corpus callosotomy remains to be a fairly good choice of surgical treatment for childhood onset medically intractable epilepsy in selected patients.     

颅内电极埋藏在枕叶癫痫中的作用

目的探讨运用颅内电极埋藏进行视频脑电图监测在定位困难的枕叶癫痫中的作用。方法通过对9例枕叶癫痫但定侧定位困难的患者,向颅内可疑部位植入硬膜下条状电极,进行视频脑电图监测,记录发作间期及发作期脑电图变化,确定癫痫病灶起始区。通过手术切除致痫灶。结果本组9例埋藏时间为3～9d,平均5d,均记录到间歇期痫样放电及发作期脑电图情况。行枕叶局部皮层切除6例及枕叶切除3例。术后按照Engel评分,I级7例,II级2例。所有病例均未出现埋藏电极引起的并发症。结论在致痫灶定位困难的顽固性枕叶癫痫中,采用颅内电极埋藏进行脑电图监测,可以精确定位致痫灶,从而提高癫痫的治愈率。     

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。     

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。     

Outcome following corpus callosotomy

Although corpus callosotomy has been performed since 1940 to treat severe medically intractable seizures, there remains controversy as to when, or even if, the surgery should be done. Unlike most surgical therapies for epilepsy where the epileptic focus is removed, corpus callosotomy interrupts the propagation of epileptic discharges. The procedure is primarily used in patients with secondarily generalized seizures with or without drop attacks in whom focal resection is not possible. The goal of this surgery is to improve functioning and well-being. In this study, we retrospectively reviewed records from 28 patients undergoing corpus callosotomy to assess improvement following surgery. Parents and patients were also sent a questionnaire to assess quality of life. While surgery reduced the frequency of seizures, definite improvements in quality of life were limited only to patients with frequent daily drop attacks preoperatively. We believe corpus callosotomy offers a significant improvement of both seizure control and quality of life in children with frequent drop attacks of tonic, myoclonic, or atonic origin.     

Intractable Epilepsy in Children

Summary: Although most children with epilepsy have a good prognosis, a small but significant minority have seizures that either do not respond to conventional antiepileptic drugs (AEDs) or have significant adverse reactions to AEDs. Many children may benefit from epilepsy surgery. Surgical treatment of epilepsy is becoming a well-established therapy for infants and young children with severe, medically intractable seizures. As in older children and adults, the presurgical evaluations of possible surgical candidates typically consist of a detailed history, neurologic and neuropsychologic examination, and anatomic and functional neuroimaging. The "gold standard" test, however, is the recording of ictal events by using simultaneous EEG and videomonitoring. Although temporal lobe resection is the most commonly performed surgery in older children and adults, nontemporal lobe resection, corpus callosotomy, and hemispherectomy are commonly performed in younger children. Efficacy of surgery in children compares favorably with results from adult patients. In addition, because the immature brain is more plastic than the mature brain, recovery of function is often greater after surgery in children than in adults. Early surgery in children with intractable epilepsy is recommended.     

Corpus callosotomy

Corpus callosotomy is a palliative surgical procedure that is suitable for some patients with intractable seizures who are not candidates for focal resective surgery. The rationale for this procedure is based on the hypothesis that the corpus callosum is a critical pathway for interhemispheric spread of epileptic activity. Efficacy and relatively low permanent morbidity in corpus callosotomy for medically intractable epilepsy have been demonstrated by more than six decades of experience. Callosotomy best ameliorates drop attacks (tonic and atonic seizures), though tonic-clonic, absence, and frontal lobe complex partial seizures often respond as well. In addition to seizure reduction, behavior and quality of life may improve. Hence, callosotomy is justified as a therapy for appropriate patients with intractable epilepsy.     

顽固性癫癎的外科联合手术治疗

目的探讨联合手术治疗顽固性癫癎的方法和临床疗效。方法对45例顽固性癫癎患者行联合手术治疗。手术方式:多处软脑膜下横切术(multiplesubpialtransection,MST)+致癎病灶切除+胼胝体前部切开术3例;MST+胼胝体前部切开术11例;MST+致癎病灶切除19例;MST+立体定向杏仁核海马毁损术12例。术中进行皮层电极及深部电极监测。结果术中进行皮层电极检测定位更精确。患者术后随访3-28个月,发作完全控制17例(37.78%),显著改善24例(53.33%),良好2例(4.44%),效差2例(4.44%),术后神经功能均保存完好。结论联合手术治疗顽固性癫癎是一种安全有效的方法。     

Bilateral intracranial electroencephalographic monitoring immediately following corpus callosotomy

Although many patients with medically refractory focal epilepsy are candidates for resective surgery, patients with multifocal epilepsy and symptomatic generalized epilepsy remain difficult to treat medically and surgically. Corpus callosotomy has been utilized since 1940 for the treatment of seizures, with reports of efficacy in multiple seizure types. Previous studies have demonstrated subsequent lateralization of bilateral/bisynchronous epileptiform activity following callosotomy. To investigate the efficacy of bilateral intracranial electroencephalographic studies immediately following corpus callosotomy, we retrospectively identified 26 patients who underwent corpus callosotomy at our center, 18 of whom had intracranial monitoring following corpus callosotomy. Five of the 18 had focal resections following intracranial electroencephalography (EEG). No patients were seizure free following callosotomy or resection. No differences in postoperative outcomes were seen between patients with intracranial EEG versus those without.     

Outcomes of epilepsy surgery in childhood-onset epileptic encephalopathy

Purpose: to evaluate the outcomes and role of epilepsy surgery in children with intractable epileptic encephalopathy (EE). Methods: ninety-five children (64 boys, 31 girls) with intractable EE were treated by epilepsy surgery at Severance Children’s Hospital from 2003 to 2008. Surgical treatments included lobar resection, hemispherotomy and corpus callosotomy (CC). Seventy-six children were Lennox–Gastaut syndrome (LGS), and 19 had West syndrome. Results: of the 76 patients with LGS, CC was performed in 37 patients (48.7%), lobar resection in 29 (38.2%) and hemispherotomy in 10 (13.2%). Of the 19 patients with West syndrome, respective surgery was performed in 15 patients (78.9%) and CC in 4 (21.1%). Of the patients receiving respective surgery, Engel’s class I outcomes were achieved for 24 of 39 (61.5%) of LGS patients, and for 9 of 15 (60.0%) of West syndrome. Malformations of cortical development were commonly observed, appearing in 73.5% (36/49). In neuropsychiatric tests, 19 of 27 with LGS demonstrated improvement in postoperative cognitive function. More significant intellectual improvement correlated well with shorter epilepsy duration, good seizure outcomes, and decreased number of antiepileptic drugs. Conclusions: epilepsy surgery should be considered in treating childhood intractable EE with expectation of improvement of both seizure and cognitive outcomes, even in cases of LGS.     

顽固性额叶癫痫的手术治疗

目的 总结与分析顽固性额叶癫痫患者手术治疗的效果和经验。方法 回顾性分析2001年9月到2003年3月在我科接受手术治疗的66例顽固性额叶癫痫病例，统计患者的症状、检查及手术治疗情况，并总结手术体会。结果 手术行额叶癫痫病灶切除 多处软膜下横切(MST)12例。额极切除1例。额叶MST 选择性胼胝体切开 选择性海马杏仁核切除14例，额叶MST 选择性胼胝体切开30例，额叶MST 选择性海马杏仁核切除3例，额叶MST 选择性胼胝体切开 双侧直回内侧切除术2例，单纯MST4例。术后疗效满意49例(74．2％)，显著改善ll例(16．7％)，良好4例(6．1％)，无效2例(3．0％)。结论 顽固性额叶癫痫患者的特点是病情严重，手术效果好。     

儿童难治性癫痫的外科治疗

目的 分析儿童难治性癫痫的病因、外科手术的适应证和预后.方法 回顾性分析采用外科手术治疗342例儿童癫痫的经验.结果 皮质发育障碍是本组儿童难治性癫痫的最重要病因,占18.4%.药物难治性癫痫综合征占13.5%.术后疗效Engel Ⅰ级158例,Ⅱ级76例,Ⅲ级61例,Ⅳ级47例.平均智商(1Q)从术前的69.2分提高到79.8分.术前显示智力低下者术后亦有明显改善.结论 早期外科干预能有效地控制癫痫发作、改善智力损害,智力低下不应是外科治疗的禁忌证.同时,正确认识儿童难治性癫痫的病凶和病理埘儿童癫痫的外科治疗具有重要作用.     

Factors underlying scalp-EEG interictal epileptiform discharges in intractable frontal lobe epilepsy.

AIMS: Scalp-EEG interictal epileptiform discharges (IEDs) may be less predictive of the outcome of frontal lobe epilepsy surgery than of temporal lobe epilepsy surgery. We identified factors associated with the location of scalp-EEG IEDs in intractable frontal lobe epilepsy. METHODS: Ten factors were assessed in a retrospective review of 53 patients with either concordant (frontal lobe seizure focus) or discordant (generalized or outside frontal seizure focus) IED or both, who had excellent surgical outcomes. The Fisher exact test and the Wilcoxon rank sum test determined statistically significant associations. RESULTS: Thirty-six patients (68%) had concordant IED, 24 (45%) discordant IED, and 17 (32%) both. Younger age at onset was significantly associated with discordant IED (mean, 7.5 years versus 17 years for patients without discordant IED; P < 0.01), whereas duration of epilepsy was not. Seizure foci at the frontal convexity were associated with concordant IED. About 72% of patients with a convexity seizure focus had concordant IED, compared with only 33% of patients with mesial frontal foci having concordant IED (P = 0.06). CONCLUSIONS: Early seizure onset in intractable frontal lobe epilepsy is associated with IEDs discordant with seizure focus. Frontal convexity seizure foci are more likely than mesial frontal seizure foci to be associated with concordant discharges.     

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。     

Our Experience with Pediatric Epilepsy Surgery Focusing on Corpus Callosotomy and Hemispherotomy

Summary:  Purpose: In pediatric patients with intractable epilepsy, surgical intervention should be considered in terms of seizure control and prevention of neuronal deterioration. We present our experience with pediatric epilepsy surgery, focusing on corpus callosotomy and hemispherotomy.
Methods: A total of 257 pediatric patients underwent epilepsy surgeries during the last decade in our clinic. The two most common surgical procedures were corpus callosotomy and hemispherotomy. The surgical outcomes of the patients undergoing these operations were analyzed with regard to seizure outcomes and surgical complications.
Results: Corpus callosotomy proved to be extremely effective in controlling drop attacks, especially when the callosum was totally sectioned. Postoperative improvement of psychomotor function also was noticeable in >70% of the cases. In patients who underwent hemispherotomy, the etiology of unilateral hemispheric lesions was closely related to surgical results, with cases of hemimegalencephaly having the worst seizure outcome and most frequent surgical complications. Incomplete section of the callosum, one of the most important causes of residual seizures, was found in three cases.
Conclusions: In pediatric epilepsy surgery, corpus callosotomy is very effective in abolishing drop attacks and improving postoperative psychomotor function. For hemispherotomy, cases of hemimegalencephaly had the worst seizure control and surgical complications. Incomplete section of the corpus callosum should be carefully evaluated as a cause of surgical failure.     

Surgical treatment for intractable epilepsy]

Epilepsy surgery can be divided into two categories, resective and disconnective procedures. The former includes lesionectomy, corticectomy, and lobectomy. The latter comprises MST (multiple subpial transection), corpus callosotomy, and hemispherotomy. In this presentation, the preoperative diagnosis and surgical outcomes of temporal lobectomy and MST will be illustrated. Temporal lobe epilepsy is one of the most common seizures in adult intractable epilepsy. Noninvasive preoperative evaluation, including analysis of seizure semiology, repetitive scalp EEG, and MR imaging, can definitely localize the seizure focus, without depending on invasive monitoring, in 70% of the cases. Seizure outcome after temporal lobectomy is generally satisfactory, with 70% seizure-free and > 90% significantly improved. However, verbal amnesia is an unavoidable sequela when the focus is on the speech-dominant side and preoperative MRI reveals little or no hippocampal atrophy. MST is an epoch-making surgical technique by which surgical treatment of eloquent cortex has become possible. In cases with neocortical epilepsy treated by MST alone or combined with corticectomy, 80% showed significant improvement, that is compatible with reported outcomes of corticectomy. MST can be also applied to treatments of extensive epileptic foci, Rasmussen's encephalitis, or Landau-Kleffner syndrome.     

胼胝体切开术治疗儿童药物难治性癫(癎)

目的 探讨胼胝体切开术治疗无法行局灶性切除的儿童药物难治性癫(癎)的安全性及有效性.方法 回顾性分析113例儿童药物难治性癫(癎)病人的临床资料,均行胼胝体切开术.结果 术后发作完全消失77例(68.1％),发作减少90％以上21例(18.6％),其中失张力性发作病人术后发作完全消失和发作减少90％以上者分别占94.1％和5.9％,而全身强直性发作病人为8.3％和75.0％.术后出现短暂的主动性语言减少3例,并在术后6-8d内恢复.随访113例,时间12个月～5年,病人精神发育状态均有不同程度的改善,无发作加重病例.结论 胼胝体切开术是治疗儿童药物难治性癫(癎)的一种安全有效的方法,特别是对失张力性发作和全身强直性发作效果更佳.     

颅内电极监测的额叶癫痫外科治疗

目的探讨颅内电极监测对额叶癫痫手术治疗的指导作用,总结额叶癫痫手术治疗效果。方法从2007年3月至2012年3月对我科40例额叶癫痫患者,采用颅内电极监测定位致痫灶和功能区,二次手术进行致痫灶处理。术后对患者进行1年到6年随访,对手术疗效进行改良ENGEL分级。结果本组男性25例,女性15例;患者年龄最小为6岁,最大为50岁,平均24．1岁;所有患者均成功行颅内电极植入,其中单侧28例,双侧12例。所有患者均通过颅内电极监测到致痫灶,其中16例致痫灶涉及功能区。对40例患者均行额叶致痫灶切除,并对5例行部分颞叶处理,9例行胼胝体切开,16例致痫灶涉及功能区者行皮层电热灼。电极植入术后,1例患者发生硬膜下血肿,约50ml,进行血肿清除后成功监测并进行致痫灶手术处理,1例致痫灶处理术后发生头皮延迟愈合,经清创术后1月愈合。对术后患者疗效行1年到6年时间随访,按照改良ENGEL分级显示I级23例,占57．5％;Ⅱ级7例,占17．5％;Ⅲ级8例,占20％;Ⅳ级2例,占5％;Ⅰ级和Ⅱ级患者占总体75％,说明手术疗效良好者占比例高。结论颅内电极对额叶癫痫致痫灶和功能区准确定位是额叶癫痫手术治疗取得成功的关键。     

Epilepsy surgery in children with pervasive developmental disorder

Pervasive developmental disorder (PDD) is occasionally associated with medically intractable complex partial seizures. The outcome of PDD was explored in three males and two females who underwent epilepsy surgery at 32 months to 8 years of age (mean = 4 years) after onset of epilepsy at 1 week to 21 months of age (mean = 11 months). Four children had temporal lobe resections (three right, one left; two for focal cortical dysplasia, and two for tumors), and one had a right temporoparieto-occipital resection (for focal cortical dysplasia). Each child underwent repeated evaluations by a pediatric neuropsychologist and psychiatrist. Fourteen to 47 months (mean = 23 months) after operation, one child with persistent seizures had moderate developmental and behavioral improvement, three children (two seizure free, one with rare staring spells) had mild developmental and behavioral improvement, and the remaining child (seizure free) experienced a worsening of her PDD. The four children with mild-to-moderate improvement in postoperative cognitive and behavioral development still demonstrated persistent delay. Cognitive gains were confirmed by neuropsychologic testing in the oldest patient but were not reflected in test results from the three younger children, who had more modest improvement. The child with worsening of her PDD had cognitive and emotional deterioration to babbling, echolalia, aggressiveness, decreased social interaction, and increased mouthing of objects beginning several months postoperatively. These results suggest that families should be counseled that PDD symptoms in children with focal epileptogenic lesions may or may not improve after epilepsy surgery, even if the surgery is successful with respect to seizure control.