脑动静脉畸形破裂大出血急性期的手术治疗特点

脑动静脉畸形(arteriovenousmalformation,AVM)是一种胚胎时期脑血管发育异常所形成的先天性血管畸形,约占脑血管畸形的90%以上,脑AVM是出血性脑血管病的常见病因,以脑实质内出血为发病特点[1～3]。AVM常常因病变部位脑动静脉直接相通的盗血而造成脑血液动力学紊乱,儿童大AVM甚至可引起心力衰竭,小的皮质内AVM可引起颅内出血、癫发作及进行性神经功能障碍等一系列临床症状,严重影响患者生命质量和生存质量,完全切除病灶并保留神经功能是最理想的治疗结果。脑AVM出血量不多可采取择期手术,大量出血因抢救生命需急诊手术者仅占16%[4],…     

脑动静脉畸形术后癫(癎)发生的危险因素分析

目的 探讨脑动静脉畸形(AVM)术后癫(癎)发生的危险因素.方法 回顾性分析接受手术并有完整临床资料的脑动静脉畸形病例138例,以性别、年龄、主要症状、阳性体征、术前癫(癎)史、病变部位、畸形团大小、引流静脉、手术入路、术后水肿、病灶残留、手术次数、癫(癎)灶切除等13项可能的影响因素为自变量,设定术后发生癫(癎)为因变量,使用Logistic逐步回归分析研究相关的影响因素.结果 术前癫(癎)史、病变部位、畸形团大小、引流静脉、术后水肿等5个因素是术后癫(癎)发生的危险因素.结论 对脑动静脉畸形手术病人,应针对癫(癎)发生危险因素进行防治,以减少脑动静脉畸形术后癫(癎)的发生.     

成人下丘脑错构瘤

目的 总结成人下丘脑错构瘤之临床特点.方法 回顾分析27例成人下丘脑错构瘤患者的临床资料.结果 共19例(70.37%)患者有临床症状与体征,其中仅5例(26.32%)于成年后发病,均因癫(癎)发作而就诊,但发作不频繁且无痴笑样癫(癎)表现.首发症状主要表现为性早熟(3例,15.79%)、痴笑样癫(癎)(9例,47.37%)、失神发作(2例,10.52%)和癫(癎)大发作(5例,26.32%);2例伴有智力障碍;多数患者可于痴笑样癫(癎)后不同时期出现癫(癎)大发作.CT及MRI检查显示脚间池或第三脑室内占位性病变,结合病史,明确诊断为下丘脑错构瘤.6例经翼点入路或胼胝体-透明隔-穹窿间入路手术切除下丘脑错构瘤,治愈3例(Engel分级Ⅰ级);癫(癎)发作基本消失2例(Engel分级Ⅱ级);无效l例(Engel分级V级).γ刀治疗2例,1例无效(Engel分级V级);1例癫(癎)发作频率减少66.67%(Engel分级Ⅳ级).抗癫(癎)药物治疗9例,但对痴笑样癫(癎)无效.结论 成人下丘脑错构瘤患者临床症状相对较轻,较少发生智力障碍,干预措施应慎重.     

脑动静脉畸形大量出血的急诊手术治疗

目的 探讨脑动静脉畸形(AVM)大量出血急诊手术治疗的有效性和安全性.方法 回顾性分析36例AVM大量出血急诊手术治疗的临床资料.结果 本组采用单纯脑内血肿清除者20例,脑内血肿清除同时切除AVM者16例,术后病理证实为AVM.所有病例术后病情稳定后均做全脑血管造影(DSA)检查,结果显示术中已切除AVM者未再发现AVM,未切除者均证实AVM存在.在未切除AVM的病例中,行二期手术切除者8例,血管内栓塞者7例,γ-刀治疗者3例,术后再出血死亡者2例.3个月后随访,恢复良好26例,中残4例,重残3例,植物生存1例.结论 脑动静脉畸形大量出血采用急诊手术清除脑内血肿是救治成功的关键,为患者生存和后续治疗提供条件,但手术风险较大,术中止血困难和术后再出血是死亡的主要原因.     

非酮症糖尿病性癫18例临床分析

目的 探讨非酮症糖尿病性癫(癎)的临床特点.方法 对18例非酮症糖尿病性癫(癎)患者的临床资料进行回顾性分析.结果 本组10例患者在血糖控制不理想状态下发生癫(癎),8例无明确糖尿病史以癫(癎)为首发症状;18例患者血糖均明显升高(16.8～32.1 mmol/L).14例表现为部分运动性发作(77.8%).应用胰岛素积极控制血糖后癫(癎)发作均得到控制.结论 非酮症糖尿病性癫(癎)可没有明确糖尿病史,以癫(癎)为首发,发作类型多为部分性发作,降低血糖是控制癫(癎)的有效方法.     

非酮症糖尿病性癫(癎)18例临床分析

目的 探讨非酮症糖尿病性癫(癎)的临床特点.方法 对18例非酮症糖尿病性癫(癎)患者的临床资料进行回顾性分析.结果 本组10例患者在血糖控制不理想状态下发生癫(癎),8例无明确糖尿病史以癫(癎)为首发症状;18例患者血糖均明显升高(16.8～32.1 mmol/L).14例表现为部分运动性发作(77.8%).应用胰岛素积极控制血糖后癫(癎)发作均得到控制.结论 非酮症糖尿病性癫(癎)可没有明确糖尿病史,以癫(癎)为首发,发作类型多为部分性发作,降低血糖是控制癫(癎)的有效方法.     

脑动静脉畸形血栓形成

本文报告7例脑血管造影未能显示的脑动静脉畸形(AVM)。根据造影所见,其中3例术前被误诊为低度恶性胶质瘤,4例造影正常。最后经手术诊断为AVM血栓形成。文献中有类似表现的脑动静脉畸形只有32例,临床表现以癫癎多见,占72％(本文7例全部表现为顽固性癲癎发作)。其他症状体征的频率分别为,头痛16％,轻偏瘫6％,共济失调3％。病变部位以颞叶多见(占47％),其次有额叶(占28％)和     

脑动静脉畸形血管构筑学因素与癫疒间的关系及其栓塞治疗效果的研究

目的探讨脑动静脉畸形(AVM)相关血管构筑学因素(AVM的大小、位置、供血动脉)与癫发作的关系以及血管内栓塞治疗的效果。方法采用数字减影血管造影(DSA)对95例脑AVM患者进行全脑血管造影,比较不同AVM大小、位置及供血动脉支数患者的癫疒间发生率。观察血管内注射NBCA或ONYX胶进行AVM栓塞的临床疗效。结果脑AVM3～6cm、>6cm患者的癫疒间发生率(52.5%、57.1%)明显高于<3cm的患者(0)(均P<0.01);AVM位于大脑皮质患者的癫疒间发生率显著高于位于基底节、后颅窝的患者(均P<0.01);不同供血动脉支数患者的癫发生率差异无显著性(均P>0.05);本组43例有癫疒间发作患者,AVM栓塞术后20例症状消失,11例明显改善,总有效率72.1%。结论脑AVM的大小及位置与癫疒间有密切关系,供血动脉的支数与癫疒间发作无关;血管内栓塞治疗AVM的效果较好。     

脑胶质瘤卒中误诊为脑动静脉畸形并出血1例

正脑动静脉畸形可引起颅内出血,30%~65%的脑动静脉畸形首发症状是出血,高发年龄15~20岁,可表现为蛛网膜下腔出血、脑(室)内出血或硬脑膜下出血。本文报道1例脑胶质瘤卒中误诊为脑动静脉畸形并出血。1病例资料男性,20岁,因突发右侧肢体活动障碍伴失语1 d入院。入院时体格检查:生命体征平稳,神志清楚,自动睁眼,运动     

脑血管病继发癫癎123例临床特点分析

目的 探讨急性脑血管病与癫癎发作的关系.方法 对9840例经颅脑CT/MRI证实的且无癫癎病史的急性脑血管病并发癫癎患者的发生率、发作类型、发作时间及临床疗效回顾分析.结果 急性脑血管病继发癎疴发生率为1.25%(123/9840).癫癎发作与病变部位有关,累及皮层或邻近皮层者易患.结论 急性脑血管病是症状性癫癎发作的重要原因之一,且大多数发生于卒中后半年内,部分病人以癫癎为首发症状.     

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.