重症肌无力的治疗

现今认为重症肌无力(MG)是随意肌的突触后膜乙酰胆碱受体(AchR)的自身免疫性肌病。即由于血中的抗AchR抗体对受体的占领、封闭,使它不能与乙酰胆碱(Ach)有效地结合;抗体与受体的复合物在补体C3的参与下可溶解受体;杀伤T细胞及淋巴因子可破坏受体;辅助T细胞可促进B细胞合成抗AchR抗体,这种抗体的大部分在胸腺内合成,T淋巴细胞在胸腺内致敏,可引起MG的自身免疫反应的始动抗原(即肌样细胞)也存在于胸腺。因此,现今治疗的目标基本是针对抗体、淋巴细胞和胸腺组织。1　抗胆碱酯酶剂这是一组能与AchR抗体竞争受体的药物。该药通过抑制胆碱…     

重症肌无力患者的护理体会

重症肌无力（MG）是一种累及神经肌肉接头突触后膜上乙酰胆碱受体（AchR）的自身免疫性疾病.临床症状：主要表现为部分或全身骨骼肌无力,易疲劳,活动后症状加重,经休息和胆碱酯酶抑制剂（ChEI）治疗后症状减轻[1].发病率为8～20/10万,患病率为50/10万,我国南方发病率较高.其中MG患者中65%～80%有胸腺增生,10%～20%伴发胸腺瘤.重症肌无力患者的治疗：包括药物治疗、放射治疗和手术治疗.其中药物治疗,口服肾上腺皮质激素和胆碱酯酶抑制剂是现今国际公认有效的常规保守疗法.手术治疗,胸腺切除加前纵隔脂肪清扫术是目前认为治疗MG最根本的方法.     

研究重症肌无力危象发生的危险因素及其防治措施

目的研究重症肌无力(MG)危象发生的危险因素及防治措施。方法对我院2010-03—2015-10收治的100例重症肌无力患者临床资料进行整理、归纳分析,包括患者年龄、激素治疗、胸腺切除与否等,行单因素及多因素Logistic回归分析,总结重症肌无力危象危险因素并提出相应防治措施。结果 MG危象组平均年龄(33.4±10.5)岁,显著低于MG非危象组的(38.8±11.0)岁,差异有统计学意义(P0.05)。MG危象组激素治疗、合并感染、胸腺切除史比例分别为85.7%、57.1%、54.8%,显著高于MG非危象组的56.9%、12.1%、27.6%,差异有统计学意义(P0.05)。多因素Logistic回归分析年龄、激素治疗、合并感染及胸腺切除史是重症肌无力危象发生的独立危险因素。结论激素治疗、合并感染、胸腺切除术史是重症肌无力危象发生危险因素,需规范激素治疗,积极预防感染及严格筛选胸腺切除手术指征。     

胸腺切除术在重症肌无力治疗中的地位

目的 评估胸腺切除术治疗重症肌无力(MG)的疗效.探讨个体化中西医治疗方案.方法 21例患者均在手术前后口服强的松、溴化吡啶斯的明,中医中药控制无力症状,均行扩大胸腺切除术,术后继续行中西医的内科治疗,1年以后评定疗效.结果 总有效率83%,无效17%,术后早期发生肌无力危象3例,无手术死亡者.结论 胸腺切除术结合中西医内科治疗重症肌无力疗效满意.     

重症肌无力最新治疗进展

重症肌无力(myasthenia gravis, MG)的治疗目的是根据临床表现和亚型进行个体化治疗,以尽快恢复患者功能,并尽量减少不良反应.MG的治疗,包括改善症状的胆碱酯酶抑制剂,糖皮质激素(以下简称"激素")、硫唑嘌呤、霉酚酸酯(mycophenolate mofetil, MMF)、环孢素、他克莫司(FK506)等免疫抑制剂,用于难治性MG的环磷酰胺(CTX)、利妥昔单抗,以及用于肌无力危象抢救的血浆置换(plasma exchange,PE)和静脉大剂量免疫球蛋白(intravenous immunoglobulin,IVIg)治疗等药物治疗及胸腺切除均取得了新进展.     

重症肌无力合并胸腺瘤的外科治疗

采用切除胸腺瘤治疗各型重症肌无力(MG)46例,病理证实胸腺瘤15例,恶性胸腺瘤31例,伴异位胸腺增生1例。术后52.2％的病例发生了MG危象,其危象见于Ⅱ_B和Ⅲ型病例。10.9％的病例在院死亡。术后讨39例病人进行了6个月～12年的随访,症状缓解33.3％,改善20.5％,无变化2.6％,与本病有关的死亡者为38.5％,MG合并胸腺瘤病例术后MG危象发生率高,围手术期和离院后死亡率亦较高。近年来对病情危重病例胸腺瘤切除早期行气管切开,酌情辅助呼吸,是预防和治疗MG危象的重要方法,有助于降低围手术期死亡率。     

22例重症肌无力合并胸腺瘤的手术治疗体会

目的 探讨重症肌无力(myasthenia gravis,MG)合并胸腺瘤患者的围手术期处理方法及手术治疗效果.方法 对本院2006-03～2008-03接受手术治疗的22例重症肌无力合并胸腺瘤患者的临床资料进行回顾性分析.采用改良Osserman标准分为:Ⅰ型9例,Ⅱa型5例,Ⅱb型7例,Ⅲ型1例,手术切口采用胸骨正中切口.手术均行胸腺瘤、胸腺脂肪组织切除及纵隔脂肪组织清扫.结果 22例无手术死亡,3例术后早期发生MG危象,经气管切开、辅助呼吸等抢救治疗治愈.结论 完善围手术期处理措施,减少MG危象的发生,手术治疗重症肌无力合并胸腺瘤可获得良好的疗效.     

重症肌无力169例临床分析

目的研究重症肌无力(MG)临床特点、误诊原因与MG危象临床表现。方法分析本院收治的169例MG患者的临床资料。结果本文患者均行新斯的明试验,阳性率100%。85例行重复神经电刺激(RNS)检查,64例低频波幅递减,8例同时高频波幅递减。157例行胸腺影像学检查,发现胸腺异常80例。发生肌无力危象31例次。121例行甲状腺功能(FT3、FT4、TSH)检测,发现甲状腺功能异常17例。结论MG临床表现多样,时有误诊,易于合并胸腺瘤或胸腺增生及甲状腺功能异常。肌无力危象发生率高。     

重症肌无力分子发病机制的研究进展

重症肌无力(MG)是一种多基因控制的自身免疫病,患者的AchR及其调节因子的基因表达异 常;病情严重程度与AchRAb的滴度无关,而与骨骼肌组织AchRα mRNA的表达量有关。胸腺中存在多 种表达AchR的细胞,并有AchR反应性T、B细胞,AchRAb及其它自身抗体的产生与胸腺内多种细胞因 子的表达有关。HLA表型、AchR基因、免疫球蛋白重链基因和T细胞受体基因等决定MG的易感性。环 境因素如病毒感染、分子模拟和AchR结合新抗原则可能是其促发因素。     

重症肌无力伴胸腺瘤患者术后危象的原因分析

我们对9例伴有胸腺瘤的重症肌无力(MG)术后发生危象的患者,分析其原因并探讨其救治方法.     

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.