三维动脉自旋标记灌注成像对致痫性局灶性皮层发育不良的术前评价

目的探讨三维准连续式动脉自旋标记灌注成像(3Dp CASL)对致痫性局灶性脑皮层发育不良(FCD)的术前定位作用。方法回顾性分析20例经手术病理证实的FCD致癫痫患者的ASL资料。获取病灶区CBF,并与对侧进行比较,并结合常规MRI、视频脑电图(VEEG)及PET/CT检查进行分析。结果所有患者均行常规MRI、3Dp CASL扫描、VEEG和PET/CT检查。病变多位于皮层下灰白质交界区,以额颞叶为多(其中额叶8例,颞叶7例)。MRI表现为T1WI呈稍低信号,T2FLAIR呈放射带状、片状高信号,周边境界欠清晰。4例患者的FCD于T2FLAIR呈小片状稍高信号,3Dp CASL未见异常灌注信号,后经PET/CT证实3例患者代谢减低,1例可疑。ASL证实CBF降低14例,与对侧比较差异有统计学意义(P0.05);CBF增高2例。10例患者经术中Eco G确认致痫灶。结论致痫性FCD的影像学具有特征性;ASL检查有助于术前病灶的定位,与VEEG、PET/CT及Eco G检查互补可提高MRI诊断的准确性。     

儿童局灶性皮质发育不良的磁共振高分辨成像特征

目的探讨儿童局灶性皮质发育不良(Focal cortical dysplasia,FCD)的3D高分辨核磁共振(MRI特征。方法回顾性分析2015年4月-2018年6月山东大学齐鲁儿童医院收治的42例经病理证实为FCD的患儿MRI资料,观察下述征象:局灶性灰白质分界模糊、皮质结构异常(增厚或变薄)、T2WI/FLAIR白质信号增高,伴或不伴transmantle征(皮层下白质内向脑室方向延伸的异常信号),T2WI/FLAIR灰质信号增高,异常脑沟或脑回形态及节段性和/或脑叶发育不全/萎缩。结果 42例患儿中,37例(88.1%)可见MRI阳性征象,FCDⅠ型13例(35.1%),主要MRI特征为局灶性灰白质分界模糊、相应部位皮质结构异常及T2WI/FLAIR白质信号增高;FCDⅡ型17例(45.9%),表现为局灶性灰白质分界模糊及皮质结构异常、T2WI/FLAIR白质信号增高及transmantle征;FCDⅢ型共7例(18.9%),其中海马萎缩2例(28.6%)、胚胎发育不良性神经上皮瘤(Dysembryoplastic neuroepithelial tumor,DNET) 2例(28.6%)、节细胞瘤1例(14.3%)、软化灶并胶质增生2例(28.6%)。结论 FCD患儿的3D高分辨MRI特征具有特异性,可提高FCD病灶检出率。     

18氟-氟脱氧葡萄糖-正电子发射断层显像在评估局灶性皮质发育不良所致难治性癫痫致痫区中的应用

我国目前有癫痫患者逾千万,其中30％的患者经过规范的药物治疗后不能满意地控制发作,发展为难治性癫痫.针对其病因学的研究发现,造成难治性癫痫的脑组织病变主要包括皮质发育不良和肿瘤两大类[1].局灶性皮质发育不良(focal cortical dysplasia,FCD)是局限的大脑皮质发育畸形,越来越多的新皮质病灶被高分辨MRI发现,其术后病理均提示FCD的存在[2].然而不少FCD患者高分辨MRI仅表现为局灶性皮质增厚、灰质-白质交界不清,或轻微的白质高信号,这些微小改变很容易漏诊,而且约30％ FCD(特别是Ⅰ型FCD)患者的MRI表现是阴性的[3].     

不同病理分型局部脑皮质发育不良的MRI研究

目的 探讨不同病理分型局部脑皮质发育不良(FCD)在MRI表现上的特点.方法 回顾性分析广州医学院第二附属医院自2006年5月至2010年6月收治的23例FCD患者资料.所有患者均经手术病理证实,按Palmini方法FCDⅠA、FCDⅠB、FCDⅡA、FCDⅡB4型,总结各类型患者MRI表现的特点.结果 23例患者中FCD Ⅰ A 2例,FeD Ⅰ B 6例,FCDⅡA 8例,FCDⅡB7例.FCDⅡ型中,有11例MRI表现为病灶Flair高信号,Ⅰ型中仅有2例,差异有统计学意义(P=0.039).而病灶T2高信号、白质内向脑室延伸带等表现虽然在Ⅰ、Ⅱ型间相差较大,但差异没有统计学意义(P=0.074、0.058).ⅡB型的MRI表现在灰白质分界不清、病灶T2高信号、脑沟加深、灰质增厚、白质内向脑室延伸带等方面均明显高于其他3型,差异有统计学意义(P<0.05).结论 不同病理分型FeD的MRI表现各有特点,特别是ⅡB型,其在MRI上的表现与其他3型明显不同,了解这些特点有助于FCD的早期诊断和术前评估.     

局灶性皮质发育不良合并难治性癫痫患者头部MRI融合PET-CT进行术前评估的价值

目的探讨颅脑PET-MRI融合图像在表现为局灶性皮质发育不良(focal cortical dysplasia,FCD)的应用价值,并总结患者治疗后的预后,为广大癫痫患者寻求一个更合理的诊治方案。方法回顾性分析2017年1月～2018年8月我院神经内外科、儿科收治的23例表现为难治性癫痫的FCD患者临床表现、影像学特点、视频脑电图特点以及手术疗效。结果表现为难治性癫痫的FCD患者的发作类型多种多样,但以局灶性发作继发全面性发作为主;病灶多见于颞叶15例、额叶6例、顶叶2例; 20例患者癫痫序列MRI及视频脑电发现病灶,3例患者行癫痫序列磁共振及视频脑电未能确定明确的致痫灶,完善PET-MRI融合成像发现致痫灶,病变部位主要在额叶,因此对于头部MRI阴性的患者,则主要通过PET-MRI发现病灶;视频脑电图(EEG)痫样放电部位与PET-MRI融合成像发现的病灶部位有较高一致性,且放电及发作形式对于FCD分型没有明显的相关性; 23例患者术后癫痫发作次数明显减少,有的未再发作。结论表现为难治性癫痫的FCD患者可通过PET-MRI融合成像结合视频脑电脑电进行术前评估,找准致痫灶,手术切除癫痫灶的治疗可取得良好的预后。     

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.     

颅脑多发性硬化的临床及MRI诊断

目的 探讨多发性硬化(MS)的临床及MRJ特征,提高对多发性硬化的认识及诊断水平.方法 对20例颅脑MS患者临床资料、病灶部位、形态、MR信号及强化特点、胼胝体改变进行回顾性分析评价.结果 MS以青、中年女性稍多见,急性、亚急性起病,多以视觉障碍或肢体感觉、运动障碍为首发症状.视觉诱发电位大多数异常.MRJ检查18例发现病灶,敏感性90%(18/20).病灶以双侧侧脑室旁、额叶皮层及皮层下、半卵圆中心多发.病灶大、小不等,多数为圆形、卵圆形."直角脱髓鞘征"及"白质变脏征"是两个较为典型的征象.T1WI上表现为等、低信号,T2WI及Flair序列上表现为高信号,Flair序列显示病灶更清晰.增强扫描病灶可呈结节状强化、环状强化、弧形强化或无强化.结论 MS的临床及MRI表现具有一定特征.MRI有助于脑部MS的诊断及鉴别诊断,是诊断MS最敏感的成像方法.     

高分辨MRI在Ⅰ型局灶性皮质发育不良患儿术前评估中的应用

目的 探讨高分辨MRI成像在儿童Ⅰ型局灶性皮质发育不良(FCD)术前评估中的价值.方法 回顾性分析52例经病理学证实的FCD Ⅰ型患儿的MRI及相关临床资料,比较高分辨成像与MRI常规序列对Ⅰ型FCD各主要MRI征象(局灶性灰白质分界模糊、局灶性皮质结构异常、白质异常信号灶及局限性脑叶萎缩/发育不全)的检出率,以及对病...     

磁共振阴性的局灶性脑皮质发育不良的手术治疗

目的探讨磁共振难以确定病灶的致痫性局灶性脑皮质发育不良的诊断和定位方法,提高手术治疗效果。方法回顾性分析联合应用视频脑电图(VEEG)、脑磁图(MEG)及术中皮层电极脑电图监测(ECo G)检查,诊断、定位并经手术后病理证实为局灶性皮质发育不良(FCD)的24例磁共振检查阴性的难治性癫痫患者的临床资料。结果 24例癫痫患者行手术治疗,病理FCDⅠa型5例,FCDⅠb型3例,FCDⅠc型5例,FCDⅡa型6例,FCDⅡb型5例。术后随访1~5年,EngelⅠ级9例,EngelⅡ级5例,EngelⅢ级8例,EngelⅣ级2例。结论联合应用VEEG、MEG和(或)ECo G技术有助于准确诊断和定位磁共振阴性的FCD,提高FCD致难治性癫痫的手术疗效。     

开颅手术治疗顽固性癫(癎)138例分析

目的评价顽固性癫痫的致痫灶定位方法和皮层电极监测下致痫灶切除,加行多处软脑膜下横纤维切断术（MST）治疗癫痫的疗效。方法对138例顽固性癫痫病人的致病灶,采用CT＋MRI＋EEC＋单光子发射计算机体层摄影（SPECT）＋皮层脑电图（ECoG）联合检测定位。对检出的阳性病灶在皮层电极监测显微镜下行致痢灶切除,切除后检测仍有癫痫波者加行MST;致痫灶位于重要功能区者单行MST。结果致痫灶阳性检出率86％。皮层电极监测显微镜下致痂灶切除加MST,术后91％的病人癫痫发作停止,一年后约15％的病人复发,但症状较术前减轻,持续时间较术前短。结论CT＋MRI＋EEG＋SPECT＋ECoG联合检测,对手术定位具有较高价值。皮层电极监测下致痫灶切除术及MST创伤轻微、效果比较可靠、治愈率高、并发症少、复发率低。病灶及致痫灶的不完全切除和形成皮层软化及疤痕,可能是导致癫痫复发的重要原因。     

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.     

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.     

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.     

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.     

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.     

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.