胚胎发育不良性神经上皮肿瘤致癫痫的手术治疗

目的 探讨胚胎发育不良性神经上皮肿瘤(DNT)致癫痫患者的手术策略.方法 回顾性分析自2008年1月至2013年8月首都医科大学宣武医院神经外科手术治疗14例DNT患者的经验,术前常规通过MRI及视频脑电图(VEEG)等检查对肿瘤及致痫灶进行评估,术中行皮层脑电监测(EcoG)对致痫灶定位,手术方式:肿瘤病灶加致痫灶切除.结果 14例患者肿瘤均得到全切,手术后病理证实为DNT,未行放疗和化疗,无肿瘤复发及恶性转化.10例患者术后癫痫发作得到完全控制(Engel Ⅰ级),4例患者术后稀少发作(EngelⅡ级).结论 DNT是低级别神经元～胶质细胞肿瘤,常见于青少年,多伴药物难治性癫痫,除切除肿瘤外,积极处理肿瘤外致痫灶可以很好地控制癫痫.     

颞叶低级别胶质瘤继发顽固性癫痫的外科治疗

目的探讨颞叶低级别胶质瘤继发顽固性癫痫患者的临床特点、术前评估及外科治疗效果。方法对31例颞叶低级别胶质瘤继发顽固性癫痫患者术前进行MRI、视频脑电图（VEEG）、发作间期正电子发射断层扫描（PET）检查,综合分析检查结果,制定相应手术方案。术中行皮层脑电描记（ECoG）,术后对切除组织进行病理检查,并对患者进行术后随访。结果29例（93．5％）患者术后癫痫发作完全或部分缓解,2例（6．5％）患者无明显缓解。结论对于颞叶低级别胶质瘤继发顽固性癫痫的患者．术前进行综合评估,同时切除病灶及致痫灶是控制癫痫发作、改善预后的有效手段。     

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.     

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。     

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.     

以癫痫发作为首发症状的边缘系统病变的手术治疗

目的探讨边缘系统病变致顽固性癫痫手术治疗方法及效果。方法回顾性分析了我院自20081月份至2011年11月手术治疗的以癫痫发作为首发症状的边缘系统病变患者40例,经完善的术前评估、术中监测后,均行标准前颞叶切除＋病灶切除。结果术中病变全切30例,部分切除10例。35例术后随访6～36个月（平均22个月）,EngelⅠ级25例,1／级10例;5例失访。结论边缘系统病变可引起顽固性癫痫,手术切除病灶＋癫痫灶安全,对癫痫控制效果好。     

神经导航引导海马病灶切除联合皮质热灼治疗顽固性颞叶内侧癫痫

目的探讨神经导航引导、皮质电极监测下对海马病灶进行致痫灶切除,辅助以皮质痫灶横纤维热灼术治疗顽固性颞叶内侧癫痫的临床价值。方法通过对16例海马病灶的顽固性癫痫的病人,术前进行24小时脑电图描记定位致痫灶,手术前进行磁共振扫描,数据输人神经导航系统,手术当天进行导航注册配准．术中进行颞叶皮质电极描记,并在导航棒引导下找寻海马病灶,完整切除并辅以皮质热灼治疗致痫灶。结果术后0．5～3年内随访,按Engel癫痫疗效分级：发作完全消失11例(68．8％),明显改善4例(25％),改善1例(6．2％)。结论神经导航有助于海马病灶的准确找寻与切除,在皮质电极监测下,辅助皮质热灼是治疗顽固性颞叶内侧癫痫的一种有效、安全的方法。     

应用脑深部电极准确定位颞叶癫痫致痫灶

目的 探讨脑深部电极准确定位颞叶癫痫致痫灶的临床价值。方法 28例疑似颞叶癫痫病人,均实施脑深部电极植入术,均以颞叶内侧为靶点,对重点怀疑致痫灶侧,分别经额叶、颞叶外侧及顶枕叶最多植入3枚电极,对侧依情况植入1～2枚,疑似双颞叶癫痫者最多植入6枚。结果 27例病人明确了致痫灶的具体部位并实施相应手术治疗,其中颞叶内侧型癫痫19例,颞叶外侧型癫痫5例,颞叶外器质性病变导致癫痫发作3例。CT/MRI显示器质性病变9例,其中1例为非致痫性病变。术后癫痫发作总消失率为62.96%(17/27),其中立体定向海马杏仁复合体毁损术5例,海马横切术3例,前颞叶切除术5例,单纯器质性病变切除术4例。结论 准确应用脑深部电极,可达到颞叶癫痫致痫灶的准确定位效果并减少手术创伤,保护脑组织功能,对于颞叶外器质性病变导致的癫痫发作也有定位价值。     

外科治疗中央区顽固性癫痫

目的探讨累及中央区顽固性癫痫的外科治疗方法,并分析影响预后结果的相关因素。方法回顾性分析2008～2011年手术治疗的25例累及中央区的顽固性癫痫患者的临床资料。所有病例依情况分别结合颅内电极、皮层电刺激功能区描记、神经导航、术中唤醒和术中电生理监测等手段进行病灶、致痫区和功能区定位。结果3例术后出现短暂的对侧肢体肌力下降,2例术后对侧肢体肌力有回升,活动更灵活。术后随访12—24个月：EngelⅠ级16例,Ⅱ级5例,Ⅲ级1例,Ⅳ级3例。结论对累及中央区的顽固性癫痫患者,应在保障安全的前提下尽可能切除致痫灶和病变。颅内电极记录和术中唤醒等技术可用于颅内致痫灶的定位,在术中电生理监测下充分切除病灶及癫痫样放电区对控制癫痫发作效果良好。     

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。     

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.     

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.     

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.     

Neonatal Seizures: Problems in Diagnosis and Classification

Summary: The clinical identification of neonatal seizures is critical for the recognition of brain dysfunction; however, diagnosis is often difficult because of the poorly organized and varied nature of these behaviors. Current classification systems are limited in their ability to communicate motor, autonomic, and electroencephalo-graphic features of seizures precisely and to provide a basis for uniform effective diagnosis, therapy, and determination of prognosis. Recent investigations of neonates, utilizing bedside electroencephalographic/polygraphic/ video monitoring techniques, have provided the basis for improved diagnosis and classification of seizures in the newborn. These studies have demonstrated that not all clinical phenomena currently considered to be seizures require electrocortical epileptiform activity for their initiation or elaboration. In addition, the specific clinical character of the phenomena considered to be seizures, the clinical state of the infant, and the character of the EEG indicate the probable pathophysiological mechanisms involved and suggest probable etiologies, prognosis, and therapy. Similarities between animal models that demonstrate reflex physiology and neonates with motor automatisms and tonic posturing suggest that these clinical behaviors may not be epileptic in origin but, rather, primitive movements of progression and posture mediated by brainstem mechanisms. Although not all clinical behaviors currently considered to be neonatal seizures may have similar pathophysiological mechanisms, they are clinically significant because they all indicate brain dysfunction.     

Valproate Monotherapy in the Management of Generalized and Partial Seizures

Summary: For decades, therapeutic tradition has promoted the concept of polypharmacy in the management of epilepsy. In recent years, however, studies have shown that, for most patients, monotherapy can provide comparable or better seizure control than administration of multiple anticonvulsants, while diminishing the potential for adverse reactions, drug interactions, and poor compliance. Valproate is an important monotherapeutic agent that is highly effective in the control of idiopathic primary and secondarily generalized epilepsies, and partial seizures that do not generalize. Comparative studies have found that valproate is at least as effective as phenytoin and carbamazepine in the treatment of generalized and partial seizures. Given the similar efficacy, other factors such as pharmacokinetics and side effects may therefore determine anticonvulsant selection for monotherapy.     

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.     

Un nouveau cadre conceptuel de travail pour la psychiatrie

In an attempt to place psychiatric thinking and the training of future psychiatrists more centrally into the context of modern biology, the author outlines the beginnings of a new intellectual framework for psychiatry that derives from current biological thinking about the relationship of mind to brain. The purpose of this framework is twofold. First, it is designed to emphasize that the professional requirements for future psychiatrists will demand a greater knowledge of the structure and functioning of the brain than is currently available in most training programs. Second, it is designed to illustrate that the unique domain which psychiatry occupies within academic medicine, the analysis of the interaction between social and biological determinants of behavior, can best be studied by also having a full understanding of the biological components of behavior.     

Special Pharmacokinetic Considerations in Children

Summary: Pediatric patients have greater degrees of pharmacokinetic variability and unpredictability than adults. This variability results from the effects of pharmacogenetics, age and growth, prior and current comedication, and disease. Newborns with seizures have the least predictable dosage requirements, and their needs change as drug-eliminating mechanisms mature in the neonatal period. Infants have the highest relative capacities to eliminate antiepileptics of any age group and require the largest relative doses. In addition to age-related trends, children demonstrate the same drug-specific, pharmacokinetic phenomena that adults do, including nonlinear phenytoin elimination, nonlinear valproate binding, and autoinduction of carbamazepine. Intercurrent illness and drug interactions further modify the age-related pharmacokinetic patterns in children and make dosage requirements even more unpredictable. Recent studies have shown that febrile illness can affect drug elimination, sometimes decreasing drug levels by 50% or more. Intermittent treatment with benzodiazepines administered either orally or rectally can be an important adjunct and help minimize this type of problem for children with marginally controlled epilepsy. Intermittent benzodiazepines are also helpful for children who have febrile seizures and who need only occasional antiepileptic protection.