儿童脑干海绵状血管畸形的临床研究及文献复习

目的 探讨儿童脑干海绵状血管畸形的临床特征及预后.方法 回顾27例手术治疗的儿童脑干海绵状血管畸形的临床资料.采用KPS评分对手术前后及随访状况进行评估,比较手术前后与随访KPS评分的差异.结果 全切除26例,部分切除1例,手术前后平均KPS评分分别为(71.85±15.20)、(68.15 ±7.86);随访27例,时间为12-138个月,随访平均KPS评分为(87.78±9.34),均无再次出血.手术前后KPS评分差异无统计学意义(P=0.106),随访KPS评分较术后明显增高(P＜0.01).结论 手术治疗对儿童脑干海绵状血管畸形患者有积极作用,长期预后良好.     

岩斜区脑膜瘤手术治疗及预后分析

目的 分析岩斜区脑膜瘤手术治疗及长期预后.方法 回顾性分析426例岩斜区脑膜瘤临床资料并进行随访,采用KPS评分对术前后状况进行评估,并对预后及远期生存相关因素进行分析.结果 有效随访314例,男85例,女229例,术前KPS评分72.9±10.5.主要手术入路为经岩骨乙状窦前入路(53.5％),肿瘤大小为(4.1±1.0)cm,全切53.8％(Simpson Ⅰ或Ⅱ级),手术死亡率2.1％,术后KPS评分为65.5 ±16.1.平均随访71.2个月,复发28例,死亡23例.随访KPS评分为74.2±28.2.术前KPS评分、肿瘤与脑干粘连和与血管神经关系等均为预后KPS(≥80分)和远期生存的独立因素.5年、10年和15年的总生存率分别为93.0％、90.6％和70.0％.结论 手术为首选治疗策略.以保留患者神经功能和改善预后生活质量为前提,行个体化治疗,未全切者应规律复查,以决定是否辅助放疗.复发病例则应积极治疗.     

脑干海绵状血管畸形的显微外科治疗

目的 探讨脑干海绵状血管畸形的手术入路、手术技巧及预后。方法 回顾性分析2004年1月至2017年1月手术治疗的24例脑干海绵状血管畸形的临床资料。结果 畸形全切除22例,部分切除2例。术后随访6个月~10年,2例发生肺炎,3例发生脑膜炎,经抗生素治疗后痊愈;5例发生永久性神经功能障碍,1例遗留轻微面瘫和吞咽困难,1例昏迷后自动出院。术后KPS评分[（75.83±21.04）分]明显高于术前[（64.58±10.62）分;P<0.05]。结论 对于脑干海绵状血管畸形,应严格掌握手术指征和时机,术中根据位置选择不同的手术入路,利用神经导航及电生理监测,采取两点定位法,对神经功能的保护至关重要。     

延髓海绵状血管畸形临床特征与预后相关因素分析

目的探讨延髓海绵状血管畸形临床特征和预后相关因素。方法回顾性分析16例延髓海绵状血管畸形病人的临床资料,采用枕下后正中入路手术切除病灶,KPS评分对术前、术后及随访状况进行评估,统计分析影响呼吸、吞咽功能和预后KPS评分的相关因素。结果手术全切除16例,术后平均KPS评分(57.5±15.3)。随访16例,时间3～169.3个月,平均45.2个月,随访平均KPS评分(68.8±30.7),均无再次出血。高位延髓病灶较低位延髓病灶者呼吸功能差(P=0.034),而年龄≥40岁者较年龄<40岁者呼吸功能差(P=0.008);病灶偏中线一侧较病灶位于或接近中线者吞咽功能差(P=0.034);年龄≥40岁者较年龄<40岁者随访KPS低(P=0.036)。结论延髓海绵状血管畸形手术预后较好。对于年龄较大、高位延髓或位置偏中线一侧的病灶应加强围手术期治疗。     

显微手术治疗脑干海绵状血管瘤的临床分析

目的 探讨脑干海绵状血管瘤的显微手术治疗方法及其疗效。方法 回顾性分析2011年10月至2015年9月显微手术治疗的19例脑干海绵状血管瘤的临床资料,其中桥脑12例,中脑4例,延髓2例,桥脑延髓交界区1例。经坐位枕下后正中小脑延髓裂入路12例,坐位乙状窦后入路4例,坐位后正中幕下小脑上入路2例,翼点入路1例。结果 病灶全切除17例,次全切除2例。术后症状改善16例,无明显变化3例,无手术死亡病例。术后随访14~58个月,次全切除2例均复发并行二次手术;术前KPS评分为（75±6）分,术后KPS评分为（84±8）分,随访1年KPS评分为（90±5）分。结论 应根据术前影像学检查以及术者经验及习惯选择手术方式,坐位可以作为大部分脑干海绵状血管瘤的常规手术体位;显微手术治疗脑干海绵状血管瘤应在保证安全的前提下做到全切除,使患者获得良好预后。     

延髓海绵状血管畸形出血致呼吸障碍的手术治疗

目的 探讨延髓海绵状血管畸形出血导致严重自主呼吸障碍患者的手术适应证及显微外科治疗.方法 回顾性分析4例延髓海绵状血管畸形出血导致严重自主呼吸障碍的临床资料,术前KPS评分平均17.5分,均行枕下后正中人路病灶切除.结果 4例延髓海绵状血管畸形均手术全切,自主呼吸均改善,肢体麻木和行走困难无显著改善,术后KPS评分平均60分,生活质量改善2例,稳定2例.平均随访15.0个月,无症状加重,随访KPS评分平均70分.结论 手术治疗延髓海绵状血管畸形出血伴自主呼吸障碍可取得良好预后.

Abstract：

Objective To discuss the surgical management of cavernous malformation (CM) in medulla oblongata with dyspnea after hemorrhage.Methods The clinical data of four patients with CM in medulla oblongata with dyspnea after hemorrhage were analyzed retrospectively.The mean preoperative Karnofsky Performance Scale (KPS) was 17.5.All of the patients underwent operation through posterior midline suboccipital approach.Results Complete resection was achieved in four patients.The main clinical symptoms of dyspnea, facial paralysis and pain were totally improved.But there was no change of motor deficits or sensory disturbance.The mean postoperative KPS was 60.Two patients were improved and others were stabilized.The mean follow - up duration was 15.0 months without recurrent hemorrhage.The recent mean KPS was 70.Conclusions Patients with CM in medulla oblongata suffering from dyspnea could obtain favorable prognosis through surgical treatment.     

弥散张量成像对丘脑胶质瘤手术的临床意义

目的探讨弥散张量成像(DTI)对丘脑胶质瘤手术切除的指导意义。方法回顾性分析42例丘脑胶质瘤病例资料。DTI组(术前行MRI+DTI检查)22例,内侧前方入路8例,内侧后方入路3例,前外侧入路2例,后外侧入路8例,后上方入路1例。对照组20例(术前仅行MRI检查),内侧入路11例,前外侧入路1例,后外侧入路8例。比较两组肿瘤全切率及术后4周KPS评分差异。结果对照组肿瘤全切除13例,次全切除6例,部分切除1例,全切率65.0%。DTI组肿瘤全切除13例,次全切除8例,部分切除1例,全切率59.1%。两组全切率比较,无统计学差异(P0.05)。术后4周KPS评分对照组为77.67±19.09分,DTI组为87.29±14.84分,两组KPS评分比较,差异具有统计学意义(P0.05,F=0.269)。结论术前DTI对丘脑胶质瘤制定手术方案有重要临床意义。     

延髓海绵状血管瘤18例分析

目的总结延髓海绵状血管瘤的治疗经验。方法回顾性分析18例延髓海绵状血管瘤的临床资料,术前KPS评分79.4±9.9。未行手术、仅常规对症处理4例;手术治疗14例,其中采用枕下后正中经小脑延髓裂入路13例,远外侧经髁入路1例。结果保守治疗4例;血管瘤全切除12例,近全切除2例。随访12例,时间1～91个月,包括手术治疗10例,术后KPS评分84.1±27.5,生活质量较术前明显改善7例,无变化2例,死亡1例;保守治疗2例,随访时间已达22个月,未见症状加重。结论对延髓海绵状血管瘤,应选择个体化治疗方案;手术治疗可取得较好预后。     

DTI对脑深部胶质瘤手术入路设计的临床意义

目的探讨术前DTI检查及脑白质纤维束重建对设计脑深部胶质瘤手术入路的临床意义。方法随机选取脑深部胶质瘤病人60例,30例术前仅行MRI检查作为对照组,根据易达及避开功能区的原则选择手术入路;30例术前行MRI+DTI检查作为DTI组,行脑白质纤维三维重建,明确脑白质纤维束与肿瘤的三维空间结构关系,以避开重要纤维束及功能区的原则设计手术入路;比较两组病人术后1个月KPS评分的差异。结果对照组镜下全切19例,次全切7例,部分切除4例,全切率63.3%;DTI组镜下全切18例,次全切7例,部分切除5例,全切率60.0%。两组镜下全切率比较,差异无统计学意义(P0.05)。对照组KPS评分为(81.67±20.69)分,DTI组KPS评分为(91.67±16.42)分;两组KPS评分比较,差异有统计学意义(P0.05)。结论术前DTI检查及脑白质纤维束重建对设计脑深部胶质瘤手术入路有重要指导意义,起到保护重要白质纤维束和降低病残率的作用。     

脊髓内海绵状血管瘤的诊断和显微外科治疗

目的 提高脊髓海绵状血管瘤(CA)的诊断水平与显微外科治疗效果.方法 回顾性分析11例资料完整的脊髓CA病例,所有患者均经MRI确诊并行显微外科手术治疗.结果 病灶全切10例,次全切1例.术后脊髓功能改善9例,无变化1例,加重1例,无手术死亡.结论 MRI是脊髓海绵状血管瘤术前最可靠的诊断方法 .全脊髓血管造影可以排除其他脊髓血管畸形.显微手术切除是脊髓海绵状血管瘤的首选治疗方法 ,术前脊髓功能受损程度是影响预后的主要因素.     

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.     

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.