大脑半球切除治疗难治性癫痫的术前评估及手术改良(附58例报告)

目的 研究大脑半球切除治疗由一侧大脑半球广泛病变引起的难治性癫痫的术前评估及手术改良.方法 回顾性分析清华大学玉泉医院癫痫中心2006年11月至2011年7月58例行大脑半球切除治疗癫痫病例.根据患者的临床发作、影像学及脑电图特点进行癫痫起源一侧半球的定侧;根据功能磁共振、磁共振弥散成像、PET -CT等进行术前神经功能分析及术后神经功能预测;选择制定合适的半球切除手术计划.结果 58例均行一侧大脑半球切除,32例左侧半球,26例右侧半球,其中5例行保留运动区的大脑半球切除.无手术死亡,4例少量术腔出血,5例切口缺血愈合不良并脑脊液漏.随访1年至5年8个月,48例术后癫痫无发作;10例术后有程度不等的发作.按照Engle评分Ⅰ级48例(83％);Ⅱ级7例(12％);Ⅲ级3例(5％).结论 改良大脑半球切除对于一侧半球病变引起的难治性癫痫控制效果及脑功能代偿良好,无严重并发症,术后停药复发率低.     

大脑半球离断术治疗儿童难治性癫痫

目的 探讨大脑半球离断术治疗儿童难治性癫痫的手术方法及疗效.方法 2007年8月至2011年10月北京三博脑科医院进行12例大脑半球离断手术.经侧裂半球离断术1例,经纵裂半球离断并颞叶切除4例,中央区造瘘半球离断并颞叶切除5例,颞叶、岛盖切除岛周半球离断术2例.手术年龄平均7.6岁(2.1 ～11.9岁).结果 术后随访0.5 -4.5年,Engel Ⅰ级10例,EngelⅡ级1例,Engel Ⅲ级1例.术后因离断不完全再次行离断手术1例.术后患者的认知及生活能力较术前提高,无脑积水等严重神经功能损伤及死亡病例.结论 大脑半球离断术治疗儿童半球性难治性癫痫完全缓解率83％ (10/12),手术疗效确定,是治疗儿童半球病变性癫痫的安全、有效的方法.     

患侧大脑半球多脑叶离断术治疗难治性癫痫

目的应用患侧大脑半球多脑叶离断术治疗该侧半球性病变导致的难治性癫痫,目的是对保留病变半球基本功能的癫痫患者提出一个有效而实用的术式。方法①大脑半球多脑叶离断术的创新:对患侧大脑半球我们分步骤离断额叶,颞叶、和顶枕叶与丘脑、基底节的联系;通过侧脑室额角和枕角离断胼胝体前后部,只保留中央前后回皮层及其与丘脑、基底节和内囊的联系;通过外侧裂切除岛叶皮层;通过颞角切除海马杏仁核。②利用这一术式我们治疗了5例大脑半球病变引发的难治性癫痫患者。结果该术式在5例患者成功实施。结果证实,该手术创伤小,并发症少,术后病人没有任何加重对侧肢体功能障碍的并发症,癫痫得到有效控制。随访13～20个月,Engel I级3例;II级2例。结论大脑半球多脑叶离断术,是对那些保留运动、感觉和语言功能的半球性病变所致的难治性癫痫患者有效、可靠的治疗选择。     

功能MRI对大脑半球弥漫性病变致癫痫患者的术前功能评估

目的通过功能MRI成像（fMRI）技术对大脑半球弥漫性病变致癫痫患者的患侧大脑半球的功能残存情况和健侧大脑半球的功能代偿情况进行综合评估,从而选择合理的手术方案。方法受试者为15例一侧大脑半球弥漫性病变致癫痫的患者,任务包括右手抓握运动、左手抓握运动、右脚抓握运动、左脚抓握运动、舌运动、默读语言等6项,采集fMRI数据并进行统计学分析,获取相应的皮质激活区图像。4例行解剖性大脑半球切除术,11例行多脑叶切除术。结果患侧手运动时的信号激活,6例在双侧半球相应区域;2例信号较弱,无明显激活区域;2例在健侧半球;5例在患侧半球。患侧脚运动时,8例信号激活在双侧半球相应区域;1例信号较弱,无明显激活区域;4例在健侧半球;2例在患侧半球。术后13例偏瘫无明显加重。术后随访20～52个月,12例癫痫控制为EngelⅠA级,2例为EngelⅡA级,1例为EngeⅢA级。结论fMRI能够客观地评估顽固性癫痫患者运动功能的保留和代偿情况,是一种有效且无创的评估手段。     

半球切除治疗小儿半球性病变伴顽固性癫痫

一侧大脑半球病变的小儿伴顽固性癫痫仅仅进行病灶及癫痫灶切除或者脑叶切除,很难彻底控制患儿的癫痫发作.癫痫发作得不到控制,患儿的智力、脑功能的发育将进一步受影响.这个时期如果不采取半球切除进行治疗,将丧失最好的手术时机.因为小儿的脑功能代偿能力很强,此时进行半球切除后,很大部分切除掉的半球功能将被很好的代偿,并且随着癫痫发作的控制,患儿的智力行为及脑功能均可以得到提高和发展.     

功能性大脑半球切除术治疗顽固性癫痫

目的 探讨功能性大脑半球切除术治疗顽固性癫痫伴有偏瘫患者的效果.方法 自2002年4月至2007年12月,笔者协助6家兄弟医院采用功能性大脑球切除术治疗8例癫痫伴有一侧半球萎缩患者.8例均经头皮脑电图和视频脑电图检查,7例病侧半球有痈波,另1例对侧(正常侧)半球有痫波.脑MRI检查均显示一侧半球广泛性萎缩改变,脑室扩大显著.在吸取Schramm等改良的功能性大脑半球切除技术基础上,采用Rasmussen的方法进行手术.结果 随访期8个月至6.4年,平均2.8年.术后癫痫控制结果采用国内术后结果评估方案评估:满意(癫痫发作完全消失)7例,但其中1例术后癫痫完全消失2年,后因自行停药,癫痫再发,经改用2种抗痈药后至今未发作;显著改善(癫痫发作减少70%)1例.术后抗痫药剂量、种类与术前相比有减少者6例,术后停药者2例.本组无死亡及严重的并发症,仅1例在同手术期发生急性癫痫持续状态,经抢救治愈.术后脑电图病侧半球6例无痫波,对侧半球(非手术侧)有痫波者2例.偏瘫情况:7例偏瘫不加重,其中6例瘫痪肢体功能有恢复,但拇指功能大多无改善;1例术后暂时性肌力下降,术后逐渐恢复并有好转.一般生活状态表现为术后性格变得温顺合作,已能上学4例,家务劳动2例,休息2例.结论 功能性大脑半球切除术治疗顽同性癫痫伴一侧半球病损的患者效果满意,且该手术并发症发生率低,值得推广应用.     

功能性大脑半球切除术的长期效果

目的：回顾性分析采用功能性大脑半球切除术治疗的8例顽固性癫痫病人的经验。方法：采用Rasmussen's的方法，个别应用改良的功能脑半球切除术行手术治疗，即切除感觉运动皮质和颞叶，并将残留的额叶及其顶枕叶的部分脑组织失去连接。结果：本组随访3－11年，平均6．7年，8例病人的癫痫得到了满意的控制，病人的生存质量都得到提高，已上学或参加工作，没有发生晚期的脑表浅含铁血黄素沉积症。结论：功能性大脑半球切除术可使频繁的癫痫发作终止或减少，可使病人独立生活。     

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。     

大脑半球离断术后无癫痫发作的脑电图特征及其对停药选择的影响

目的 分析大脑半球离断术后无癫痫发作患者的脑电图(EEG)特征及其对停药的影响。方法 回顾性分析2017年1月—2022年6月首都医科大学三博脑科医院收治的54例行半球离断术后均无癫痫发作的患者。收集术前术后临床资料及抗发作药物服药情况。结果 术前EEG显示36例(66.7%)患者仅在手术侧半球记录到癫痫样放电,10例(18.5%)患者有双侧半球独立放电伴泛化,3例(5.6%)患者有双侧半球同步癫痫样放电;术后6～12个月EEG显示所有患者手术半球背景活动减弱,42例(77.8%)患者仅在手术半球有癫痫样放电,6例(11.1%)患者双侧半球均有癫痫样放电,3例(5.6%)患者放电位于手术半球及中线区,3例(5.6%)患者在两侧均无癫痫样放电,3例(5.6%)患者手术半球出现EEG发作但无临床症状。术后随访平均随访3年,14例患者(25.9%)停用抗发作药物后均无发作。结论 大脑半球离断术对双侧半球癫痫样放电的患者仍可达到术后无发作。术后无临床发作但EEG显示存在癫痫样放电的患者可逐渐减停抗发作药物。     

多脑叶切除联合其他术式治疗半球顽固性癫癎(附18例报告)

目的探讨运用多脑叶切除联合多软膜下横纤维切断术(MST)或／和胼胝体部分切开术治疗脑电图 提示为单侧半球为主的多脑叶或半球弥漫性癎灶患者的手术疗效。方法 回顾性总结、分析采用多脑叶切除联合 MST或／和胼胝体部分切开术所治疗的具有半球(为主)多脑叶或半球弥漫性癎灶的18例重型顽固性癫癎患者。结 果本组术后随访1～5年,平均2年。疗效按Engel的标准评定,I级(术后即无癫癎发作)11例;Ⅱ级(每年仅1 ～2次发作)3例;Ⅲ级(发作频率减少75％以上)2例;Ⅳ级2例,总有效率16／18;效果优良14／18,无于术死亡。结 论采用多脑叶切除联合其他术式治疗具有半球(为主)多脑叶或弥漫性癎灶的重型顽固性癫癎具有疗效好、并发 症相对较少等优点,比大脑半球切除术具有更广泛的适应证。     

Diagnostic Difficulties and Treatment Implications

Summary: Differentiation between types of epileptic seizures has been aided in recent years by the introduction of intensive neurodiagnostic techniques and the development of increasingly detailed classification systems. Paradoxically, these developments have not simplified the task of matching the appropriate antiepileptic drug to a particular seizure type. It is reasonable to assume that anticonvulsant drugs will have different effects on different types of seizures, but faulty, circular reasoning can enter the picture if one also assumes that responses of seizures to different drugs signify different seizure types. There are several examples of differential diagnoses that can fall prey to this problem, including the diagnosis between partial seizures with secondary generalization and generalized tonic-clonic seizures, and the diagnosis between complex partial seizures and absence seizures with automatisms, among others. Considerations of etiology in future classification systems can further complicate the problem: should one then choose an anticonvulsant drug on the basis of individual seizure type or on the basis of the type of epilepsy? Ramifications of this issue extend even to the drug approval process. Official sanction is not given for use of a drug for a seizure type not included in the original efficacy studies, even if later scientific evidence shows that seizure type to be related to a type that is included. New trials must be undertaken. These problems arise from how we choose to classify seizures.     

Cognitive Dysfunction Associated with Antiepileptic Drug Therapy

Summary: Epilepsy is frequently associated with cognitive dysfunction. However, the reasons for this correlation are unclear. Possible influential factors include patient age; duration, frequency, etiology, and type of seizures; hereditary factors; psychosocial issues; and antiepileptic drug (AED) therapy. Whereas many of these factors are beyond the physician's control, AED therapy is one element that can be addressed in treatment decisions by recognizing the potential cognitive effects of particular AEDs. For example, phenobarbital impairs memory and concentration; phenytoin affects attention, problem solving ability, and performance of visuomotor tasks. In contrast, carbamazepine may affect concentration, while valproate would appear to have minimal effects on cognition. Moreover, cognitive effects of AEDs are amplified with coadministration of multiple anticonvulsants (polytherapy). A review of studies on the cognitive effects of monotherapy with AEDs, as opposed to those of polytherapy, provides evidence that drug-related cognitive dysfunction can be reversed if patients are switched to a simpler therapeutic regimen. Future research should be directed toward developing reliable measures for assessing and monitoring cognition, and understanding the particular cognitive side effects of each AED. Physicians also need to revise their opinions about which side effects are "tolerable" for epileptic patients.     

Hepatic Considerations in the Use of Antiepileptic Drugs

Summary: Virtually all of the major antiepileptic drugs (AEDs) can cause hepatotoxicity, although fatal hepatic reactions are rare. The mechanisms, incidences, and risk profiles for such reactions differ from drug to drug. With carbamazepine and phenytoin, hepatotoxicity may be due to drug hypersensitivity. Although the profiles of patients at risk have not been well-defined for these two antiepileptic drugs, it would appear from reports in the literature that older adolescents and adults are at higher risk than children of developing serious or fatal hepatotoxicity. Once hepatotoxicity develops, mortality rates are 10–38% with phenytoin and 25% for carbamazepine. The risk profile for valproate fatal hepatotoxicity has been more clearly defined. Those at primary risk of fatal hepatic dysfunction are children under the age of 2 years who are receiving multiple anticonvulsants and also have significant medical problems in addition to severe epilepsy. The risk is considerably lower for patients over the age of 2 years on valproate monotherapy. In contrast to the risk profile with other AEDs, adults receiving valproate as monotherapy have the lowest risk of hepatotoxicity. Fatal hepatic dysfunction coincident with valproate may be the result of aberrant drug metabolism. Concomitant use of AEDs that induce microsomal P450 enzymes (e.g., phenytoin and phenobarbital) may enhance the production of a toxic metabolite, and hence the greater risk of hepatotoxicity with polypharmacy.     

Vascular Malformations and Epilepsy: Clinical Considerations and Basic Mechanisms

Summary: Vascular malformations (VMs) are associated with epilepsy. The natural history of the various VMs, clinical presentation, and tendency to provoke epilepsy determine treatment strategies. Investigations have probed the mechanisms of epileptogenesis associated with these lesions. Electrophysiologic changes are associated with epileptogenic cortex adjacent to VMs. Putative pathophysiologic mechanisms of epileptogenesis include neuronal cell loss, glial proliferation and abnormal glial physiology, altered neurotransmitter levels, free radical formation, and aberrant second messenger physiology.     

Carbamazepine Efficacy in Adults With Partial and Generalized Tonic-Clonic Seizures

Summary: Carbamazepine and phenytoin are drugs of choice in initial monotherapy for adult partial and secondarily generalized tonic-clonic seizures. These designations reflect the results of the Veterans Administration Epilepsy Cooperative Study Group of 1985. An earlier comparative study of carbamazepine and phenytoin by Ramsay and associates found both drugs equally effective in controlling new-onset seizures. Among the advantages of carbamazepine is that it causes relatively few cognitive and dysmorphic side effects. Its disadvantages are its unavailability in parenteral formulation and its metabolic autoinduction. The latter must be compensated for by planned dosage increases to maintain therapeutic plasma steady-state levels during the first 2 or 3 months of treatment. Carbamazepine is judged a drug of choice in the treatment of these secondarily generalized tonic-clonic seizures, and the drug of choice in children, adolescents, and women susceptible to the dysmorphic side effects associated with other anticonvulsant agents.     

Etiologic and Preventive Aspects of Epilepsy in the Child–Bridging the Gap Between Laboratory and Clinic

Summary: Four broad categories of basic phenomena are pertinent to developing ways to prevent epilepsy. These include mechanisms of epileptogenesis, ictal initiation and temporary entrainment by the seizure discharge of normally functioning brain, seizure propagation, and control mechanisms that function both to restrain the cascade of epileptic events culminating in a seizure and to arrest the epileptic event and restore the interictal state. In newborns and children, hypoxia-ischemia is a major factor leading to epileptogenesis, and several schemes are proposed to classify, quantify, and prevent hypoxic-ischemic encephalopathy. Control mechanisms must be better understood in order to develop prophylactic recommendations for epilepsy, and an experimental model of "kindling antagonism" may increase our understanding of these. Programs of prevention of seizures in children will evolve only if basic researchers and clinicians work productively together to develop an adequate understanding of factors important in epileptogenesis and antiepileptogenic control mechanisms.     

Epilepsy and anomalies of neuronal migration: MRI and clinical aspects

Neuronal migration disorders are the result of disturbed brain development. In such disorders, neurons are abnormally located. In diagnosing these conditions, magnetic resonance imaging is superior to any other imaging technique. This enables us to improve our knowledge of the clinical correlates of neuronal migration. With reference to migrational disorder, a retrospective study of all 303 patients with epileptic seizures referred for magnetic resonance imaging during a 3-year period was performed, 13 patients (aged 12-41, mean age 27) were identified. They represent 4.3% of the entire study group. Of the patients with known epilepsy, 6.7% and of the mentally retarded, 13.7% had migrational disorders. Four patients had schizencephaly as the dominant finding, one was classified as hemimegalencephaly, 2 had isolated heterotopias, and 6 had localized pachy- and/or poly-microgyria. The clinical pictures are complex. Ectopias of grey matter are recognised foci of epilepsy, but from an epileptological and a clinical viewpoint little attention has been given to these disorders. The present study shows that malmigration is not rare in epilepsy patients, especially not in the mentally retarded.     

Carbamazepine Efficacy and Utilization in Children

Summary: Carbamazepine is effective for preventing partial and generalized tonic-clonic seizures in children. Although absence epilepsies are more common in children than adults, an estimated 80% of children with epilepsy have seizure types or epilepsies that are potentially responsive to carbamazepine. The differential diagnosis of ictal staring is an especially important issue in children because absence and atypical absence seizures are more prevalent in children than adults. Age-related pharmacokinetic differences and drug interactions are major considerations in children. On average, children have higher clearance rates of carbamazepine, shorter half-lives, and higher ratios of carbamazepine-10, 11-epoxide to carbamazepine than adults. In addition, children with severe epilepsy are more likely to require multiple-drug therapy, which can lead to complex drug interactions. When carbamazepine is administered along with valproate, drug protein binding interactions can cause intermittent side effects.     

Predisposing and Causative Factors in Childhood Epilepsy

Summary: We review information from large studies of defined populations, examining the role of known factors and especially of prenatal and perinatal factors in contributing to nonfebrile seizure disorders of early childhood. We depend especially, but not exclusively, on the recently completed analyses from the Collaborative Perinatal Project of the National Institute of Neurological and Communicative Disorders and Stroke, the NCPP. About 4% of children in the NCPP who had at least one non-febrile nonsymptomatic seizure by the age of 7 years had a previous seizure during acute neurologic illness, such as meningitis or during the acute illness after trauma. Many such seizures should potentially be preventable. Of children with seizures, 10% had had a neonatal seizure and 13% had had a febrile seizure. Among the hundreds of prenatal and perinatal factors explored as predictors of childhood seizure disorders, the principal predictors identified were congenital malformations of the fetus, cerebral and noncerebral; family history of certain neurologic disorders; and neonatal seizures. In agreement with the British National Child Development Study, labor and delivery factors in the NCPP appeared to contribute very little to childhood seizure disorders. Maldevelopment, rather than damage at birth to an initially intact nervous system, appeared to be the more common mechanism. Most seizure disorders of early childhood remained unexplained by the large set of prenatal and perinatal characteristics examined.     

Classification of methods in transcranial Electrical Stimulation (tES) and evolving strategy from historical approaches to contemporary innovations

Transcranial Electrical Stimulation (tES) encompasses all methods of non-invasive current application to the brain used in research and clinical practice. We present the first comprehensive and technical review, explaining the evolution of tES in both terminology and dosage over the past 100 years of research to present day. Current transcranial Pulsed Current Stimulation (tPCS) approaches such as Cranial Electrotherapy Stimulation (CES) descended from Electrosleep (ES) through Cranial Electro-stimulation Therapy (CET), Transcerebral Electrotherapy (TCET), and NeuroElectric Therapy (NET) while others like Transcutaneous Cranial Electrical Stimulation (TCES) descended from Electroanesthesia (EA) through Limoge, and Interferential Stimulation. Prior to a contemporary resurgence in interest, variations of transcranial Direct Current Stimulation were explored intermittently, including Polarizing current, Galvanic Vestibular Stimulation (GVS), and Transcranial Micropolarization. The development of these approaches alongside Electroconvulsive Therapy (ECT) and pharmacological developments are considered. Both the roots and unique features of contemporary approaches such as transcranial Alternating Current Stimulation (tACS) and transcranial Random Noise Stimulation (tRNS) are discussed. Trends and incremental developments in electrode montage and waveform spanning decades are presented leading to the present day. Commercial devices, seminal conferences, and regulatory decisions are noted. We conclude with six rules on how increasing medical and technological sophistication may now be leveraged for broader success and adoption of tES.