引起顽固性呃逆、呕吐的视神经脊髓炎的临床表现和影像学研究

目的 探讨引起顽固性呃逆、呕吐(IHN)的视神经脊髓炎(NMO)患者的临床表现和脑干、脊髓MRI特点.方法 收集中山大学附属第三医院神经科17例NMO患者的临床资料,对其中8例合并IHN的NMO患者临床表现及MRI特点进行分析.结果 IHN在NMO患者中常见,本组17例NMO中有8例合并IHN,临床上表现为IHN、复视、眼球震颤,其中6例表现有线样延髓征(LML)或线样延髓脊髓征(LMSL).脊髓纵向MRI显示病灶常常大于3个椎体节段,且以脊髓中央管为中心;轴位脊髓MRI表现为部分性或横贯性,以脊髓的后角或侧角为主,前角受累较少. 结论 引起IHN的NMO临床上多伴有复视和眼球震颤,延髓脊髓MRI常常可见LML或LMSL征,而且病灶以脊髓中央管为中心,后角或侧角受累为主,这些可与多发性硬化相鉴别.     

视神经脊髓炎脑部病灶的影像学特征及临床表现

目的 探讨视神经脊髓炎(NMO)脑部病灶的影像学特征及主要临床表现.方法 回顾性分析南京医科大学附属脑科医院神经内科自2002年1月至2008年5月收治的19例NMO患者脑部MRI检查结果及相关病史资料.结果 本组19例患者中15例行头颅MRI检查,7例发现脑部病灶;病变累及大脑半球3例,丘脑2例,下丘脑1例,第三脑室和中脑导水管周边1例,中脑1例,桥脑1例,延髓2例;MRI上病灶为点状、斑片状或线样,呈T1低信号、T2和FLAIR高信号改变.临床表现恶心、呕吐、呃逆、复视、眼震、面部麻木、认知功能障碍及嗜睡等.结论 NMO可出现不同部位脑部病灶,以脑室管膜周边和下丘脑处病灶具有疾病特征性.部分NMO患者出现脑部受累表现,少数患者以脑部症状为首发临床事件.     

视神经脊髓炎脊髓磁共振成像特征

目的 比较视神经脊髓炎(NMO)与多发性硬化(MS)的脊髓MRI特点,从MBI的角度重新认识NMO.方法 对20例MS患者和23例NMO患者的脊髓MRI进行同顾性分析.结果 NMO患者脊髓MRI多表现为线样延髓征、线样延髓脊髓征、线样脊髓征、脊髓横贯性或次横贯性损伤,且常超过3个节段(23例),而MS患者脊髓MRI病变节段短(≥3个节段者8例,χ2=19.142,P<0.01),常呈偏心性分布(17例,与NMO组比较,χ2=25.256,P<0.01).结论 NMO不同于MS,在MRI方面,病灶的分布有其自身特征,而MS的脊髓病灶与髓鞘走向一致.因此,我们从影像学角度进一步证实NMO是有异于MS的一种独立的疾病.     

视神经脊髓炎的头部MRI异常表现及对应临床症状

目的研究视神经脊髓炎头部MRI的特异表现及对应临床症状。方法回顾性分析中国医科大学附属第一医院神经内科自2008年1月²012年7月收治的22例NMO脑部MRI检查结果及相关临床表现。结果本组22例患者中12例头部MRI正常,10例发现脑部病灶,病变累及侧脑室旁白质3例,丘脑1例,第三脑室和中脑导水管周边5例,桥脑5例,延髓5例,皮质0例;头部MRI病灶为点状或线样损害,左右对称。临床表现为顽固性呃逆、恶心呕吐、复视、眼震、过度睡眠、血糖增高、中枢性高热等。结论 NMO患者脑室管膜周边和下丘脑处病灶具有特异性,所有病例并未见皮质改变;并能引起顽固性呃逆、恶心呕吐(IHN)、过度睡眠、内分泌改变、复视、眼震等特征性临床表现。     

视神经脊髓炎脊髓损害的磁共振研究

目的分析视神经脊髓炎(NMO)和多发性硬化(MS)患者脊髓MRI特点,以及血清抗水通道蛋白4(AQP4)IgG抗体阳性与阴性NMO患者脊髓MRI特点。方法回顾分析贵州省中枢神经系统脱髓鞘疾病数据库中42例NMO和32例有脊髓损害的MS患者的脊髓MRI资料。结果与MS组比较,NMO患者脊髓病灶累及更长的椎体节段(P0.05),在脊髓MRI矢状位上表现为线样征和纵向延展的脊髓损害(LESCL)(P0.05)。轴位T2WI上亮斑状损害(BSLs)以及中心性、横贯性脊髓损害更常见(P0.05);在病灶部位及强化病灶上,NMO和MS组间比较差异无统计学意义。与血清抗AQP4-IgG抗体阴性NMO患者比较,阳性患者线样征、BSLs、中心性损害更常见(P0.05),在脊髓病灶部位、受累椎体节段数、LESCL、横贯性损害及强化病灶方面,抗AQP4-IgG抗体阳性组和阴性组间比较差异无统计学意义。结论除LESCL、线样征、横贯性损害和中心性损害特点外,BSLs可能是另一个有助于鉴别NMO与MS的脊髓病灶MRI特征。BSLs、线样征、中心性损害特点可能与NMO患者抗AQP4-IgG抗体的血清学状态有关。     

视神经脊髓炎患者头颅磁共振成像特点

目的 分析视神经脊髓炎(neuromyelitis optica,NMO)患者头颅MRI特点.方法 对27例NMO患者的头颅MRI进行回顾性分析.结果 22例(81.5%)NMO患者存在不同类型的头部病灶,并可分为非特异性(7例)、非典型性(1例)、多发性硬化样(MS-like,3例)及脑室-导水管-中央管周围型(11例),但以脑室-导水管-中央管周围型最为常见(40.7%),且NMO患者颅内的病灶数与最后一次MRI检查的时间呈正相关(r=0.475,P=0.025).结论 NMO患者脑内病灶类型多样,但以脑室-导水管-中央管周围型最为常见,可能存在不同机制.对疑似病例早期进行头颅MRI检查相当必要.     

视神经脊髓炎患者33例脑部磁共振分析

目的 探讨视神经脊髓炎(neuromyelitis optica,NMO)患者脑部MRI影像学表现.方法 收集满足最新NMO诊断标准且脑部MRI表现不符合多发性硬化诊断标准的患者33例,均行脑部和脊髓MRI检查,分析其MRI影像学特点.结果 33例NMO中,脑部正常表现者5例(15.2%),异常表现28例(84.8%),其中脑内实质有明确病灶22例(66.7%),另6例(18.2%)脑内虽未见明确病灶,但深部脑白质显示了肉眼可视的对称性弥漫性脱髓鞘高信号影.22例明确病灶中,15例病灶数≥2个,7例为单个病灶.幕上近皮质、皮质下和深部脑白质区的点状非特异性病灶最多,少数为非典型的斑片状融合病灶.幕下脑干是易受累的部位(14/33,42.4%),特别是延髓(7/33,21.2%).结论 NMO患者出现脑内异常较为常见,有脑部的异常不能排除NMO的诊断.认识NMO脑内病灶对完善NMO诊断标准有帮助.     

顽固性呃逆、呕吐的症状对于鉴别急性炎性脱髓鞘疾病和预示复发的临床意义

目的 探讨顽固性呃逆、呕吐(intractable hiccup and nausea,IHN)症状对于鉴别急性炎性脱髓鞘疾病和预示复发的意义.方法 回顾分析1995年～2011年北京协和医院病房收治的临床诊断的24例存在IHN的急性炎性脱髓鞘疾病患者的临床和影像学资料,其中,分析IHN在不同疾病组中的分布,IHN与其他复发症状的关系、IHN相对应的延髓责任病灶的影像学特点、以及血清水通道蛋白4抗体检测结果.结果 24例急性炎性脱随鞘疾病患者包括视神经脊髓炎12例,多发性硬化5例,复发性长节段脊髓炎6例,急性播散性脑脊膜炎1例,共发生IHN35次,其中40％发生在急性脱髓鞘事件之前.视神经脊髓炎患者组、复发性长节段脊髓炎患者组、多发性硬化患者组各组间相互比较,发现复发性长节段脊髓炎组延髓病灶与高颈段脊髓相连的比例是5/9,较多发性硬化组1/9比例明显增高(P =0.039 ＜0.05);视神经脊髓炎组患者血清水通道蛋白4阳性的比例是3/6,较多发性硬化组患者比例0/5明显增高(P =0.032 ＜0.05).各组患者在IHN与伴随症状的时间关系、伴随症状、出现延髓病灶的比例以及延髓病灶强化情况方面相比,无明显差异.结论 IHN可以作为鉴别急性炎性脱髓鞘疾病的参考,往往发生在其他症状之前,预示疾病复发.     

视神经脊髓炎与多发性硬化的临床症状、脊髓MRI表现的对比分析

目的 结合视神经脊髓炎(NMO)与多发性硬化(MS)患者的临床症状和脊髓MRI特点探讨两者之间差异发生的机制.方法 回顾性分析中山大学附属第三医院自2004年1月至2007年1月收治的23例NMO患者及21例MS患者的临床资料,比较其临床症状及脊髓MRI上受损部位MRI上的差异.结果 NMO患者多为女性,且首次发病年龄、扩展病残状况评分(EDSS)评分均高于MS患者;双侧深感觉障碍、束带感、直肠或膀胱括约肌功能障碍3种临床症状在NMO、MS患者中的发生率不同,差异均有统计学意义(P<0.05);上述各临床症状基本能在脊髓MRI找到相应受损病灶.结论 NMO是不同于MS的脱髓鞘疾病,其特殊的发病机制导致其临床症状与脊髓MRI均有自己的特点.     

Chiari畸形的MRI诊断与临床（附40例分析）

40例经 MRI诊断为 Chiari畸形患者进行 MRI图像测量并和临床症状进行对比。结果提示 :( 1)在 Chiari畸形中 ,延髓中央管消失与脊髓空洞症的形成有关 ;( 2 )脊髓空洞症是 Chiari畸形的主要并发症 ,其临床症状主要由脊髓空洞症产生 ;( 3)颅底陷入症是产生延髓 -颈髓成角的原因之一。     

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.     

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.     

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.