静脉滴注大剂量丙种球蛋白治疗Guillain—Barre综合征和多发性硬化的疗效观察

目的 观察静脉滴注大剂量丙种球蛋白（IVIG）治疗Guillain－Barre综合征和多发性硬化（MS）的疗效。方法 应用IVIG,首日0．4g/kg,继而以0．2g/kg/d连用4天,治疗17例GBS和12例MS,与同期应用激素冲击治疗的25例GBS和41例MS的疗效进行比较。结果 IVIG治疗GBS和MS组在按功能评定的临床分级及临床见效时间方面优于激素治疗组,均＜0．05;而临床有效率两组比较无统计学差异。结论 IVIG治疗GBS和MS的近期疗效优于激素冲击治疗组,其远期疗效仍待临床继续观察。     

静脉滴注免疫球蛋白与激素对比治疗格林-巴利综合征的meta分析

目的比较静脉注射大剂量免疫球蛋白（IVIG）与激素治疗格林-巴利综合征（GBS）患者疗效及安全性。方法对国内有关包括IVIG与激素治疗GBS随机对照试验进行meta分析,评价纳入文献的方法学质量后,采用RevMan4．2．10软件对提取的数据进行分析。结果共纳入10个随机对照试验（RCTs）研究,IVIG组共计384例,激素组441例：（1）IVIG与激素治疗GBS的有效率合并检验分析,结果为Z=5．41,P〈0．00001,0R=4．48,95％可信区间（CI）为（2．60,7．72）;（2）IVIG与激素治疗GBS的治愈率合并检验分析结果Z=8．40,P〈0．00001,OR=4．56,95％CI（3．20,6．50）;（3）IVIG与激素治疗GBS的起效时间比较,评价研究发现存在异质性,采用随机效应模型进行综合检验,结果为：Z=17．47,P〈0．00001,WMD=-4．0,95％CI（-4．45,-3．55）。结论IVIG治疗GBS总有效率、治愈率明显优于激素,起效快,在条件允许的情况下,应尽早应用IVIG进行治疗。但IVIG价格昂贵,面对那些不能负担昂贵治疗费用或不能耐受IVIG的患者,激素仍是一个有价值的治疗选择;目前国内IVIG与激素治疗GBS这一领域仍缺乏设计严谨、方法科学的临床研究。     

血浆置换联合激素治疗重症神经系统脱髓鞘疾病

目的　评价血浆置换联合激素治疗重症吉兰 -巴雷综合征 ( Guillain-Barre syndrome,GBS)和多发性硬化( multiple sclerosis,MS)的临床疗效。方法　回顾性分析血浆置换联合激素治疗的 1 3例重症 GBS和 7例重症MS的临床转归及治疗后血中免疫球蛋白滴度变化。结果　患者呼吸肌麻痹于治疗后 5～ 1 4d缓解 ,2个月内临床表现缓解率分别为 GBS76.9% ,MS5 7.1 %。血中免疫球蛋白滴度明显减少 ( P <0 .0 1 )。结论　血浆置换联合激素疗法疗效肯定 ,可作为治疗急性重症神经脱髓鞘疾病首选方法。     

大剂量丙种球蛋白治疗吉兰-巴雷综合征疗效观察

目的 观察大剂量丙种球蛋白(IVIG)治疗吉兰-巴雷综合征(Guillain-Barre Syndrome,GBS)的临床疗效.方法 应用IVIG治疗20例(观察组)GBS患者,与20例(对照组)应用激素治疗GBS患者进行比较.结果 IVIG观察组按功能评定的临床分级及治愈率明显高于对照组,差异有统计学意义(P＜0.05);肌力改善、感觉恢复等时间比对照组明显缩短,但2组临床有效率比较差异无统计学意义.结论 观察组疗程短及痊愈时间明显优于对照组,并发症发生率低,治疗效果较好,为治疗GBS最有效的方法之一.     

甲钴胺治疗急性吉兰-巴雷综合征随机对照研究

目的评价甲钴胺治疗急性吉兰-巴雷综合征（GBS）的疗效。方法43例急性吉兰-巴雷综合征随机分为甲钻胺组22例和对照组21例，分别应用甲钴胺注射液500μg及维生素B12注射液500μg肌内注射，2次／d，连续治疗4周，2组均使用免疫球蛋白、激素及综合治疗，同时观察记录治疗后的症状、体征改善程度和改善时间，并计算有效率。结果治疗组的有效率显著高于对照组（P〈0．05）。结论甲钴胺治疗急性GBS疗效较好。     

轻症吉兰-巴雷综合征患者免疫球蛋白与甲泼尼龙治疗效果比较

目的 比较甲泼尼龙与免疫球蛋白静脉注射(IVIG)治疗轻症吉兰-巴雷综合征(GBS)的临床效果.方法 回顾性分析我院1996年1月～2011年12月92例轻症GBS患者.根据治疗方法不同分为两组,52例采用IVIG治疗的患者为IVIG组,40例采用甲泼尼龙治疗的患者为甲泼尼龙组,观察两组疗效.结果 甲泼尼龙组与IVIG治疗轻症GBS效果显示,甲泼尼龙组治愈18例,显著进步10例,进步12例,总有效率100％；IVIG组治愈24例,显著进步16例,进步12例,总有效率100％；两组治疗效果比较差异无统计学意义(P＞0.05).结论 甲泼尼龙与丙种球蛋白静脉注射均是治疗轻症GBS的有效方法.     

伴脑神经受损的吉兰-巴雷综合征42例临床分析

目的探讨伴有脑神经受累的吉兰-巴雷综合征(Guillain-Barre syndrome,GBS)的临床、神经电生理特点及治疗转归情况。方法搜集2005-1-2015-5我科收治的伴有脑神经受累的GBS患者42例,回顾性分析其临床特点及治疗方案疗效比较。根据Hughes评分的变化评估对患者的疗效,并用Fisher确切概率法对单纯静脉注射免疫球蛋白(intravenous immunogloblin,IVIg)组和IVIg联合糖皮质激素(简称为"激素")组的有效率进行比较。结果 42例患者中单纯脑神经受累7例,伴有脊神经受累35例;脑神经受累的主要临床症状依次为吞咽困难及呛咳21例,眼外肌麻痹17例,鼻唇沟变浅16例。实验室检查:腰穿查脑脊液的患者32例,其中脑脊液蛋白-细胞分离24例。行神经传导速度、F波及H反射检查35例,其中异常31例,包括F波/H反射未引出或出现率减低20例,运动神经潜伏期延长15例,波幅减低15例,运动神经传导速度减慢12例,感觉神经传导速度减慢9例。采用单纯IVIg治疗20例,好转18例(90%),IVIg联合激素治疗17例,好转12例(70.6%),两者疗效差异无统计学意义(P=0.140);其中IVIg联合激素治疗无效的5例患者中,3例患者治疗后病情仍加重,行血浆置换后病情好转;单纯激素治疗3例,好转2例;未治疗自动出院2例。结论脑神经受累的GBS患者中舌咽、迷走、动眼神经及面神经最易受累,也可同时累及脊神经。神经传导速度、F波检查及脑脊液蛋白细胞分离对诊断伴有脑神经受累的GBS均有重要意义。单独使用IVIg或联合激素治疗GBS疗效较好。     

颅脑外科手术后并发吉兰-巴雷综合征2例

目的总结颅脑外科手术后的吉兰-巴雷综合征(GBS)的诊治经验。方法回顾性分析2例颅脑外科手术后出现GBS病例资料,并复习相关文献。结果病人在确诊GBS后,均立即运用丙种球蛋白治疗和甲强龙冲击治疗,病人症状均得到明显改善。结论颅脑外科术GBS早期临床症状特异性低,应提高对本病的警惕性,当出现无法解释的肌无力症状时,应尽早进行脑脊液和肌电图的检查,从而明确诊断,尽早使用丙种球蛋白和激素冲击治疗,对改善病人预后至关重要。     

吉兰-巴雷综合征患者肝功能异常的临床探讨

目的探讨吉兰-巴雷综合征(GBS)患者的肝功能异常(LFD)状况及其意义。方法回顾性分析作者医院1997-06-2005-06收治的252例GBS患者临床资料,分析LFD在GBS患者中的发生率,探讨发病年龄、性别、前驱感染史、病情轻重、激素治疗等因素对其影响,并进一步分析其可能意义。结果GBS组52.7%存在LFD,显著高于对照组(P<0.01)。GBS各亚组LFD发生率具有以下特点:无性别差异(P>0.05),儿童组异常率高(P<0.01),有前驱感染史组比无前驱感染史组高(P<0.05),病情较重组较较轻组高(P<0.01),激素治疗组肝功能恢复正常率较非激素组高(P<0.05),非激素组治疗后LFD率(33.3%)明显低于入院时GBS组(52.7%)(P<0.05)。结论LFD是GBS患者的常见表现且具有一定自限性,可作为感染和免疫损害的证据,提示GBS病情严重。     

免疫球蛋白治疗格林-巴利综合征的疗效观察

目的对照观察免疫球蛋白、糖皮质激素及输血浆对症治疗格林-巴利综合征(GBS)的疗效。方法收集自1998年1月-2004年12月在我院住院治疗的GBS131例,其中36例(A组)每日用免疫球蛋白0．4g／kg,静脉滴注,连续5天。输血浆对症治疗组(B组)为36例,激素治疗组(C组)为59例。观察3种疗法的肌力恢复、并发感染、平均住院天数、辅助呼吸时间、气管切开的例数及死亡率。结果 A组的肌力恢复与并发感染指标、平均住院天数及辅助呼吸时间与B组及C组相比较,有显著性差异(P<0．01,P<0．05),而B组与C组间,则无显著性差异(P> 0．05)。气管切开例数,3组间无显著性差异。结论免疫球蛋白治疗GBS对于肌力恢复、减少并发感染、缩短平均住院天数及辅助呼吸时间等均优于输血浆对症治疗和激素疗法。     

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.     

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.