格林-巴利综合征患者肌电图检测及其意义

目的探讨格林-巴利综合征(Guillain-Barre syndrome,GBS)患者肌电图的特点及其意义。方法采用肌电图检测技术,对86例临床诊断为GBS的患者进行肌电图检测,并比较分析与脑脊液检测结果的相关性及其差异性,即其中29例脑脊液生化值(细胞-蛋白分离)异常和16例CFS值正常的肌电图结果分析。结果临床诊断GBS 86例病例中,运动神经传导速度(MCV)异常率最高;其次是胫神经H反射。而腰穿检查结果敏感度低,异常率也低。结论 GBS患者临肌电图特征:以运动神经传导速度(MCV)异常表现为主,MCV传导速度异常高于SCV感觉传导速度;H反射异常率高于F波异常率;脑脊液常规检查蛋白细胞变异明显影响患者周围神经(MCV/SCV/H/F)传导速度。     

磁共振神经成像对吉兰-巴雷综合征的诊断价值

目的探讨磁共振神经成像(MRN)对吉兰-巴雷综合征(GBS)的诊断价值。方法对临床诊断为GBS(28例)患者行腰丛神经MRN检查。测量右侧L4神经干及股神经肌肉信号比,检测患者的脑脊液蛋白-细胞值,分析脑脊液蛋白值与NMSR的相关性。结果 GBS患者脑脊液蛋白及神经干、股神经的NMSR相关检验均呈正相关(rs=0.767、0.667,P<0.001)。结论 GBS脑脊液蛋白的改变会引起神经干、股神经NMSR的改变,MRN可作为GBS的诊断的重要手段。     

Guillain-Barre综合征合并中枢神经系统脱髓鞘病变的临床研究

目的 探讨Guillain-Barre综合征（GBS）合并中枢神经系统（CNS）脱髓鞘病变及两者之间的发病关系和可能的发病机制。方法 对3例合并CNS脱髓鞘病变的GBS患者的临床及实验室资料进行分析。结果 例1男，28岁，主要表现为全身软瘫和昏迷，血气分析结果正常，脑脊液有蛋白细胞分离，寡克隆带阳性，肌电图提示神经源性损害；MRI示双侧脑白质及颈髓广泛多发性脱髓鞘改变；应用血浆和免疫球蛋白治疗后好转，例2女，5岁，因进行性四肢无力，呼吸困难入院，应用呼吸机和血浆治疗过程中出现脑干反射障碍，后放弃治疗死亡，死后行腓肠神经病检示周围神经脱髓鞘病变，例3男，12岁，因进行性四肢无力伴大小便困难入院；颅脑MRI未见异常，脊髓MRI见T5-T4节段广泛脱髓鞘病变；脑脊液有蛋白细胞分离。寡克隆带阳性；肌电图示周围神经传导速度减慢。结论 合并意识障碍的GBS大多病情危重，其发病机制不详，可能与P1碱蛋白引起的自身免疫反应造成周围和GNS的共同脱髓鞘有关。     

变异型Guillain-Barre综合征的临床特点

目的探讨变异型Guillain-Barre综合征(GBS)的临床特点。方法回顾性分析14例变异型GBS患者的临床资料。结果本组10例为女性,4例为男性;均为急性或亚急性起病,病前11例有相关诱因;临床表现为多脑神经型、急性运动轴索型、Miller-Fish综合征及全自主神经功能障碍型。12例行腰椎穿刺术,11例脑脊液蛋白增高;11例行肌电图检查,9例提示为周围神经源性损害;经个体化治疗所有患者的症状均有改善。结论变异型GBS类型及临床表现多样,以女性患者多见;脑脊液蛋白-细胞分离及肌电图检查为周围神经源性损害有助于诊断。     

HIV阳性的颅神经损伤的吉兰-巴雷综合征1例报道及文献复习

<正>吉兰-巴雷综合征(Guillain-Barré syndrome,GBS)是一种自身免疫介导的周围神经病,主要损害多数脊神经根和周围神经,也常累及脑神经;临床特点为急性起病,症状多在2周左右达到高峰,表现为多发神经根及周围神经损害,常有脑脊液蛋白-细胞分离现象,多呈单时相自限性病程,静脉注射免疫球蛋白和血浆置换治疗有效。既往曾有HIV感染合并GBS的报道,但累及颅神经的报道较少,本研究现将本院收治的1例HIV阳性的伴有颅神经损伤的吉兰-     

咽-颈-臂变异型吉兰-巴雷综合征1例报道及文献复习

目的探讨咽-颈-臂(pharyngeal-cervical-brachial,PCB)变异型吉兰-巴雷综合征(GBS)的临床特点和诊断。方法分析我院收入1例PCB变异型GBS患者的临床表现、辅助检查结果和临床治疗效果。结果 PCB变异型GBS临床表现口咽肌、颈部、肩部、双上肢无力,双下肢不受累或轻度受累。本例患者腰穿提示明显蛋白-细胞分离,给予静滴免疫球蛋白和甲强龙冲击治疗后症状明显好转。结论 PCB是GBS少见的变异型,极易出现误诊或漏诊,临床表现为咽-颈-臂肌无力,而下肢不受累或轻度受累的患者,应考虑PCB的可能,可结合脑脊液、电生理检查及早作出诊断和治疗。     

吉兰-巴雷综合征的治疗进展

吉兰-巴雷综合征( GBS)是一种周围神经炎症性脱髓鞘性疾病。主要表现为急性进展性对称性弛缓性瘫痪、腱反射减弱或消失及感觉障碍,脑脊液检查出现蛋白-细胞分离现象,常累及颅神经及自主神经,出现相应神经功能障碍。目前GBS没有治愈方法,治疗目的主要是抑制免疫反应、清除致病因子、阻止疾病发展并促进疾病的恢复。治疗方法主要包括免疫治疗及支持治疗。     

小儿脊髓空洞症8例临床分析

目的 总结小儿脊髓空洞症(SM)的临床表现、脑脊液、神经电生理及影像学特点,提高对该病的认识水平.方法 8例SM患儿,男6例,女2例,年龄8个月～14岁,对本组病例进行临床表现、脑脊液、神经电生理及影像学分析.结果 8例均表现为肢体运动障碍,因小儿感觉的主诉不明显,体检无法配合,故感觉障碍症状不突出.脑脊液检查压力及成分大多正常,空洞大时也可致椎管梗阻,脑脊液蛋白含量增高.SM电生理检查中肌电图因空洞侵蚀的范围不同而异,呈非特异性.MRI可在纵、横段面上清楚显示空洞的位置及大小.结论 MRI是目前诊断本病最准确的方法.     

Arnold ChiariⅠ畸形合并脊髓空洞的手术分析

目的 探讨Arnold Chiari Ⅰ畸形（Arnold Chiari Malformation type Ⅰ,ACM）合并脊髓空洞（Syringomyelia,SM）的外科治疗方法.方法 对2000～2006年36例ACM合并SM的患者采用后颅窝减压、成形术,并根据不同的临床特点予以空洞切开引流、下疝小脑扁桃体切除、颈枕交界区粘连分离以及脊髓中央管口松解术.结果 术后随访1年以上,症状明显缓解28例,稳定无变化7例,1例疼痛加重并出现对侧上肢麻木;MRI检查示2例脊髓空洞消失,26例空洞明显缩小,8例空洞无明显变化.结论 缓解颅颈交界区的压迫和疏通脑脊液循环是手术成功的根本.     

50例吉兰-巴雷综合征临床分析

目的探讨儿童吉兰-巴雷综合征(GBS)的临床特点和治疗方法。方法对符合GBS诊断的50例进行临床分析并对治疗方法及疗效进行对比观察。结果50例患儿中男39例,女11例,主要表现为上行性、对称性、弛缓性麻痹。脑脊液有蛋白细胞分离现象。大剂量静脉用丙种球蛋白在控制病情进展,减少继发感染,缩短病程优于其他方法(P<0.05)。结论静脉用丙种球蛋白治疗儿童GBS是较安全有效的方法。     

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.     

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.     

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.     

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.