胚胎发育不良性神经上皮肿瘤的致痫灶处理

目的 探讨胚胎发育不良性神经上皮肿瘤(DNT)伴药物难治性癫痫患者的外科手术治疗方法.方法 回顾性分析了第三军医大学新桥医院神经外科采用外科手术治疗14例此类患者的经验.术前和术中均进行致痫灶定位,术中在切除肿瘤的同时一并处理肿瘤外致痫灶.结果 14例患者肿瘤均得到全切,未行放疗和化疗,无肿瘤复发及恶性转化.11例患者癫痫发作得到完全控制(Engel Ⅰ级),2例患者稀少发作(Engel Ⅱ级),1例患者术后仍有频繁癫痫发作,再次手术切除肿瘤周围致痫灶后癫痫得到完全控制.结论 DNT应当按照皮质发育障碍来处理,除切除肿瘤外,积极处理肿瘤外致痫灶可获得较佳的癫痫控制结果.     

胚胎发育不良性神经上皮肿瘤的手术治疗

目的探讨胚胎发育不良性神经上皮肿瘤（DNT）的手术方法及其疗效。方法 2007年6月至2013年6月显微手术治疗DNT患者17例,手术方式采用在皮层脑电图指引下行肿瘤加致痫灶扩大切除术。术后至少随访1年,根据Engel预后分级评估手术效果。结果所有患者肿瘤均得到全切,病理学均证实为DNT,其中10例合并局灶性脑皮质发育不良（FCD）,术后未出现永久性功能障碍。患者术后随访1~4.5年,无肿瘤复发,17例中无癫痫发作（EngelⅠ级）的13例（76.5%）,仍有少量癫痫发作（EngelⅡ级）4例（23.5%）。结论 DNT是一种少见的以癫痫为主要临床表现的良性肿瘤,常伴有FCD,多发展为药物难治性癫痫,在皮层脑电图监测下行肿瘤加致痫灶扩大切除可以获得良好的癫痫控制率。     

影响局灶性皮质发育不良所致难治性癫痫手术疗效的相关因素分析

目的分析影响局灶性皮质发育不良(FCD)所致药物难治性癫痫手术疗效的相关因素。方法收集2015年9月至2018年7月在中国科学技术大学附属第一医院神经外科接受手术治疗并经病理确诊为FCD的50例药物难治性癫痫患者的临床资料,包括病史、发作频率、病灶位置、视频脑电结果、头颅MRI表现、术前服药种类、手术切除范围、术后早期癫痫发作情况,采用Engel分级评估患者手术疗效,并将其分为预后良好组和预后不良组。运用统计学方法分析FCD所致难治性癫痫的手术疗效相关影响因素。结果共有50例患者接受手术,其中致痫灶位于颞叶31例,额叶14例,顶叶2例,枕叶2例,颞枕叶1例,手术完整切除致痫灶44例,未完整切除6例,术后病理分型为FCDⅠ、Ⅱ、Ⅲ型分别有19例、17例、14例,术后随访时间为12～46个月,平均(20.7±7.9)个月,术后EngelⅠ级34例、EngelⅡ级3例、EngelⅢ级5例、EngelⅣ级8例,预后良好组34例,预后不良组16例。单因素分析显示病程≥10年、术后出现早期癫痫发作、致痫灶未完全切除者手术疗效较差(均P0.05)。二分类Logistic回归分析结果显示,致痫灶是否完全切除和术后是否出现早期癫痫发作是FCD所致难治性癫痫手术疗效的独立影响因素(均P0.05)。结论致痫灶是否完整切除和术后是否出现早期癫痫发作与FCD致难治性癫痫的手术疗效密切相关。患者的病程也是FCD致难治性癫痫手术疗效的重要预测因素。     

立体定向脑电图在MRI阴性难治性癫痫中的应用价值

目的 探讨立体定向脑电图(SEEG)在治疗MRI阴性难治性癫痫患者术前评估的应用价值。方法 回顾性分析解放军第九八八中心医院神经外科2018年1月—9月收治的7例MRI阴性，在SEEG指导下手术切除致痫灶患者的临床资料;采用Engel分级评估术后癫痫控制效果。结果 7例患者共植入63根SEEG电极，平均每例患者植入9根电极，触点数共548个;植入顺利，未出现电极折断或移位、感染、脑脊液漏等并发症;所有患者均接受可疑致痫灶射频热凝术及随后的致痫灶切除术。术后随访12～18个月，患者的发作频率平均减少92. 8%(P=0. 00)，其中5例患者(71. 4%)发作控制达EngelⅠ级，2例(28. 6%)患者发作控制达EngelⅡ级。结论 SEEG监测范围广，可以准确定位MRI阴性癫痫的发作起始;在MRI阴性癫痫患者术前评估中安全可行，可以有效指引手术切除致痫区，控制癫痫发作。     

颞叶外节细胞胶质瘤继发癫痫的手术治疗研究

目的研究颞叶外节细胞胶质瘤(GG)继发癫痫的临床特点和手术治疗方式及疗效。方法回顾性分析手术治疗并经病理证实的20例颞叶外节细胞胶质瘤继发癫痫患者的临床资料。术后癫痫控制效果按改良Engel分级标准评定;分析患者的发作表现、病变部位、影像学特点、病理改变、手术方式及病变切除程度与癫痫控制的关系。结果节细胞胶质瘤位于额叶者2例、顶叶7例、枕叶5例、颞枕交界处6例。MRI检查显示肿瘤为囊性者5例、囊实性6例、实性9例;增强扫描病变有强化12例,病变周围水肿5例。CT检查示病变有钙化4例。癫痫发作表现为简单部分性发作(伴意识保留的局灶性发作)者4例,复杂部分性发作(伴意识障碍的局灶性发作)5例,继发性全面性强直阵挛(局灶进展到双侧的强直阵挛发作)11例。肿瘤次全切除者2例,全切除15例,扩大切除3例。术后病理检查示合并脑皮质发育不良者2例。术后随访1~3年,癫痫控制效果为EngelⅠ级17例(85.0%),Ⅱ级2例(10.0%),Ⅲ级1例(5.0%)。预后相关临床因素分析显示,肿瘤切除的方式和程度与预后有关。结论颞叶外节细胞胶质瘤继发癫痫多为药物难治性,手术切除可取得良好效果。术前须综合评估病变部位和临床、影像学特点等资料制定手术策略,术中尽可能行肿瘤加致痫皮层全切除,以提高疗效。必要时可考虑先行SEEG电极植入后,再行致痫灶精准切除术。     

隐源性癫痫致痫灶最常见好发部位及相关病理特征

目的 回顾性分析隐源性癫痫致痫灶最常见的好发部位及手术切除标本的病理改变,探讨隐源性癫痫致痫灶的发病机制.方法 通过向26例头部CT、MRI等影像学检查无特异性表现的患者颅内可疑脑区植入皮层电极及深部电极,行长程视频脑电监测,记录发作间期及发作期脑电图变化,确定癫痫病灶起始区,手术切除致痫灶并送病理,术后定期随访.结果 26例均可以明确致痫灶,其中癫痫发作单独起源于颞叶新皮层及颞叶内侧的13例,占本组病例的50％;送检标本病理结果显示胶质细胞增生、皮层分层紊乱25例,占本组病例的96.15％,其中伴有海马硬化14例.术后随访1年以上,Engel Ⅰ级15例,Engel Ⅱ级8例,Engel Ⅲ级2例,Engel Ⅳ级1例.无严重并发症及手术死亡病例.结论 颞叶新皮层及颞叶内侧是隐源性癫痫致痫灶最常见的好发部位,皮层发育不良是隐源性癫痫手术切除标本中常见的病理改变,其中海马硬化是颞叶内侧常见的病理改变.颞叶新皮层及颞叶内侧病理改变不仅经常相伴出现,而且病理改变轻微.通过外科干预,效果较满意.按Engel分级,位于颞叶新皮层及颞叶内侧的病例手术疗效较其他好,Engel's分级均在Ⅱ级以上.     

幕上与癫痫有关的海绵状血管瘤的显微外科治疗

目的 探讨幕上表现于癫痫的颅内海绵状血管瘤外科治疗方法和效果.方法 25例幕上表现于癫痫的颅内海绵状血管瘤患者,根据术前影像学、脑电图表现与运动区的关系分为A、B二组.手术在皮层脑电图(ECoG)监测下进行,包括单纯切除血管瘤、切除血管瘤和切除含铁血黄素层,以及辅助部分皮层痫灶切除、皮层热灼或软膜下横切等.结果 本组海绵状血管瘤均令切除,2例术后有一过性轻度肢体功能障碍,无手术死亡.随访10个月-4.5年,A组9例,Engel Ⅰ级6例;Engel Ⅱ B级2例;Engel Ⅲ级1例.B组16例,均无发作.结论 幕上与癫痫有关的海绵状血管瘤在ECoG监测下做病灶和痫灶切除,是控制术后癫痫发作的有效手段;运动区和附近海绵状血管瘤由于手术没有完全切除致痫灶可能是术后癫痫控制率差的原因.     

皮层脑电图监测在致痫灶切除术中的应用(附96例报告)

目的 探讨皮层脑电图(ECoG)监测在癫痫手术中指导切除致痫灶的价值.,方法 2005年12月至2008年6月期间,96例难治性癫痫患者在经过详细的术前评估后,进行了手术治疗.术中先切除癫痫病灶,再行描记EcoG,监测异常放电是否消失;若癫痫波仍大量存在,则进一步切除病灶周围的致痫灶,直至EcoG监测异常放电消失或明显减少.结果 术后随访12～44个月.根据Engel疗效分级标准进行疗效评价.Engel Ⅰ级45例,Ⅱ级22例,Ⅲ级17例,Ⅳ级12例.手术有效率达到87.50%.结论 术中应用EcoG监测能大大提高致痫灶空间定位的准确性,有效地指导癫痫的手术.     

MRI正常的难治性癫痫的手术疗效及相关因素分析

目的 探讨颅脑MRI检查正常的难治性癫痫患者的手术疗效及提示手术预后的相关因素.方法 1997年8月至2010年10月间,对解放军第四○一医院手术治疗的难治性癫痫病例(出院≥1年),且经颅脑MRI检查未见异常的患者跟踪随访1-6年,并对获完整资料的62例患者进行分析.Engel分级Ⅰ、Ⅱ级视为预后良好,Ⅲ、Ⅳ级为预后不良,分析相关因素与手术疗效的关系.结果 Engel分级Ⅰ、Ⅱ级共47例,占76％.颞叶切除较额叶及顶枕叶皮质致痫灶切除的预后良好率高(P＜0.01);而额叶及顶枕叶皮质致痫灶切除术后疗效差异无统计学意义;术前轻度发作的较重度发作组术后预后良好(P＜0.05);手术后晚期出现发作的较早期出现发作预后不良(P＜0.05);病理检查异常较病理正常的术后预后良好(P＜0.05);手术时患者的年龄与预后无关.结论 颅脑MRI正常的难治性癫痫,颞叶切除较颞叶外致痫灶切除预后良好,术前发作轻的、术后病理检查异常的也提示预后良好,手术后晚期出现发作者则预后不良.     

VEEG、ECoG、结合MRS定位致痫灶评价(附23例临床分析)

目的探讨在颞叶病变继发颞叶癫痫(Temporal lobe epilepsy,TLE)患者中应用长程视频脑电图(VEEG)、颅脑磁共振波谱分析(MRS)联合皮层脑电图(ECoG综合定位致痫灶指导手术切除致痫灶范围,评价TLE术后临床效果。方法病历资料选自2016年1月至2017年12月期间在蚌埠市第三人民医院癫痫研究所就医的既往有癫痫发作临床症状及不同医院脑电图结果提示有颞叶脑电异常初步依据纳入、排除标准选取病历资料完整的23例颞叶病变继发TLE的患者行手术治疗,术前完善VEEG及MRS检查,术中联合ECoG再检测致痫灶指导手术切除范围,术后采用Engel标准评价手术效果。结果应用VEEG与MRS两种检查分别阳性对不同病因组患者致痫灶定位中,术中在ECoG再检测指导下与MRS、VEEG定位致痫灶比较,MRS与ECoG一致率为95.65%,高于VEEG与ECoG的一致率65.22%,差异有统计学意义(χ~2=6.769,P0.05);在ECoG再检测指导下手术治疗的23例继发性TLE患者,术后随访12～27个月,依据Engel评价标准,结果示:EngelⅠ级18例,EngelⅡ级3例,EngelⅢ级1例,EngelⅣ级1例,术后均无严重并发症及死亡病例,术后临床效果达到显著有效共22例,显著有效率为95.65%。结论术中联合ECoG再检测致痫灶并指导手术切除范围,可显著提高手术治疗效果,TLE患者术后显著有效达95.65%;对一般经济条件需要手术治疗的患者,术前应用MRS与VEEG综合定位致痫灶与术中联合ECoG再检测致痫灶是颞叶病变继发TLE患者一种很好的选择,可避免昂贵的检查费用,获得满意疗效。     

Treatment Considerations in Anticonvulsant Monotherapy

Summary: The long-standing practice of polypharmacy in treating epilepsy is giving way to use of monotherapy. Monotherapy can improve seizure control as well as reduce the risk of serious idiosyncratic reactions, dose-related side effects, and complex drug interactions. Monotherapy also offers improved compliance and cost-effectiveness. The basis of monotherapy is accurate diagnosis and assessment of the patient's seizure type(s), followed by selection of a single appropriate anticonvulsant drug. Many patients currently treated with multiple anticonvulsants can be successfully converted to monotherapy with a carefully monitored program in which troublesome and redundant drugs are gradually withdrawn from the therapeutic regimen.     

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.     

Dextromethorphan: Cellular Effects Reducing Neuronal Hyperactivity

Summary: Dextromethorphan is a dextrorotary morphinan without affinity for opioid receptors, commonly used as an antitussive medication. During the past 5 years, interest in the compound and its demethylated derivative, dextrorphan, has been revived because additional neuroprotective and an-tiepileptic properties were found in in vitro studies, animal experiments, and a few clinical cases. Both morphinans are able to inhibit N -methyl-D-aspartate (NMDA) receptor channels and voltage-operated calcium and sodium channels with different potencies. The inhibition of the NMDA receptor is believed to be the predominant mechanism of action responsible for the anticonvulsant and neuroprotective properties of the compounds.     

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.     

Pediatric Epilepsy Surgery

Summary: The use of implantable arrays of epidural electrodes has made it possible to carry out extraoperative electrocorticography (ECoG) and functional localization in the awake child. This has permitted cortical excisions that are determined by criteria similar to those obtained during surgical procedures performed under local anesthesia in adults. In addition, the method also permits simultaneous ECoG and video monitoring during the child's symptomatic seizures, providing additional important localizing information that is impractical to obtain in operations under local anesthesia. We report our experience with 75 children, ages 5 months to 15 years, whom we have managed with epidural electrode arrays. The method of extraoperative ECoG is described and illustrative cases are presented to demonstrate its feasibility and utility in children. In addition, we call attention to gliomas as a common cause of chronic focal seizures in children. Of 49 children undergoing resection and followed for from 1 to 14 years (mean of 5.8 years), 32 (65%) are either seizure free or have had a significant reduction in seizure frequency that has unambiguously improved their quality of life. The results are analyzed further by relating the surgical outcome to each of the pathologic entities that caused the seizures. This analysis reveals the variety of neurological conditions that commonly cause intractable focal seizure disorder in children and distinguishes those pathologic entities in which the seizure disorder is apt to respond to surgical intervention from those that will not.     

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.     

Anticonvulsant Drugs and Cognitive Function: A Review of the Literature

Summary: Alterations of cognitive function are separate from disturbances of behavior seen in association with epilepsy. The nature of the cognitive disability may to a certain extent depend on the seizure type. Partial seizures, mainly derived from a temporal lobe focus, impair memory tasks, while generalized seizures seem to have more effect on attentional abilities. A number of studies, reviewed in this paper, suggest that anticonvulsant drugs further impair cognitive function. Maximal impairments are seen in patients receiving polytherapy: rationalization of polytherapy improves cognitive abilities. Studies in children and adults have allowed differentiation of the effects of various commonly used antiepileptic agents. Maximal cognitive deficits are seen with. phenytoin, while phenobarbital and sodium valproate induce moderate disturbances, and carbamazepine seems relatively free from such toxicity. Further research is needed on the interrelationship between types of seizure disorders, types of anticonvulsant medications, and cognitive function.