干扰素-β治疗视神经脊髓炎谱系疾病两例

正1病例报告患者1,男性,64岁,因"进行性双下肢活动障碍3年"于2013-09-11入作者医院。患者于入院3年前无诱因出现双下肢膝盖远端麻木、乏力,伴双下肢发作性不自主抖动,伴尿频、尿急,可基本正常行走,未予特殊治疗;2个月后乏力感明显加重,且逐渐向肢体近端发展,伴疼痛;6个月后进展至仅能缓慢行走,予营养神经等治疗后症状仍进行性加重,无力感延至腰部,遂于外院行肌电图     

中年女性头晕、行走不稳进行性加重2年余——Gerstmann-Sträussler-Scheinker综合征

1 资料 患者,女,45 岁,家庭主妇,因"头晕伴行走不稳进行性加重2 年余"于2016 年08 月03 日入中山大学附属第一医院神经科诊治. 患者于2013 年12 月开始无明显诱因出现头晕、行走不稳.头晕多为昏沉感,不伴视物旋转,无耳鸣或听力下降.头晕与体位改变有关,曾有跌倒(具体不详).2014年2 月症状加重,...     

1例Hartnup病临床特点及诊疗分析

1病例介绍 患者,男性,19岁,因“发作性跌倒5年,间断双下肢无力麻木伴皮疹4年”入院。患者5年前跑步时突然跌倒,约2min后自行站起,无意识障碍、肢体抽搐。4年半前再次突然跌倒,症状同前。后逐渐出现发作性行走不稳,每次约持续10min。     

马尾副神经节细胞瘤1例

报道 患者男,２７岁,因发作性双下肢疼痛５年,加重伴麻木无力１个月入院。患者于１９９４年无明显诱因出现双下肢疼痛,以右侧为重,腰椎Ｘ片未见明显异常,两月后疼痛消失,但自觉双下肢乏力不能进行跑跳运动。约１个月前,患者再次出现左下肢疼痛,继而出现麻木无力,并进行性加重,行走时多次跪倒,左膝不能过度屈曲,行走时跛行步态,同时出现排尿、排便困难。行腰椎Ｘ线平片及ＣＴ、ＭＲＩ检查,示腰椎椎管扩大,脊髓圆锥下方硬膜内占位病变。患者病程中无发热及食欲减退。入院时检查：腰椎生理曲度,Ｌ２～３棘突压疼,无放射痛,…     

进行性四肢远端麻木2年 行走不稳2个月

正病历摘要患者女性,50岁,主因进行性四肢远端麻木2年,行走不稳2个月,于2016年1月29日入院。患者2年前无明显诱因出现左足趾麻木,无活动异常,未予重视。1年前出现双手指尖麻木,逐渐向上进展至腕部,伴前臂发凉感,同时左足趾麻木逐渐向上进展,出现左足和左小腿麻木。当地医院考虑"颈椎病"或"腰椎病",予针灸、按摩、牵引等物理治疗,症状未见明显好转。2个月前逐渐出现右足、右小腿麻木,伴行走不稳,自觉行走时踩棉花感、需注视地面,夜间行走不     

行走不稳 四肢麻木疼痛

病历摘要患者男性,52岁。主因行走不稳约15 d,2012年8月30日入院。患者入院前约15 d无明显诱因出现行走不稳、易跌倒,但尚可感觉地面平实,曾出现穿鞋上床,伴双侧掌心刺痛及小腿中下段麻木感,无头痛、头晕、复视、饮水呛咳、吞咽困难等症状。病情呈进行性加重,逐渐进展为不能独立行走、站立,吃饭时不能准确地将勺子送入口中,言语含糊,反应稍迟钝,短时记忆减退。自发病以来精神、食欲欠佳,大小便如常,近2年来体质量下降约10 kg。     

髓周动静脉瘘1例并文献复习

1病例报告患者男,68岁因"双下肢乏力进行性加重1 a半伴大小便障碍2月"于2009-01-10入院。患者于2007-07始,时常出现双下肢发作性乏力,严重时须他人扶行并伴双下肢麻木,常休息数小时(1~5 h)可完全好转。无神经根痛,无束带感。于2008-11,患者时出现小便失禁,大便困难时出现排     

四肢麻木进行性加重伴手抖的临床病例分析

正病例资料病史患者住院号:91090×××,女性,66岁,右利手。主诉:四肢麻木3年余,进行性加重伴手抖2年余,于2017年12月19日收入复旦大学附属华山医院神经内科。现病史2014年初患者无明显诱因出现四肢远端麻木,呈手套袜套样感觉异常,伴行走不稳,行走时感足底疼痛,易摔倒。2015年4月出现左上肢活动时不自主抖动,休息后可缓解。门诊予口服激素,症状无改善,     

行走不稳4个月双下肢麻木无力发作性抖动3个月

正患者男性,69岁,主因行走不稳4个月,双下肢麻木无力、发作性抖动3个月,于2015年11月10日入我院神经科。患者4个月前(2015年7月)无明显诱因出现行走不稳,不自主向左侧歪斜;约1个月后(2015年8月)出现双下肢麻木、无力,行走时前屈、前倾,伴静止时双下肢发作性抖动,动作幅度较大,持续数分钟,2~3次/d,夜间加重、活动或自行按摩后缓解,无头晕、头痛,无意识障碍等。外院头部MRI(2015年9月5日)显示,左侧侧脑室后角和周围占位性病     

慢性外伤性桥脑小脑角区血肿1例

患者 女性,35岁。因外伤后阵发性左面部麻木3年,行走不稳半年,于1986年6月10日入院。患者于3年前不慎跌倒,枕部着地,当时昏迷5分钟,醒后仅觉轻度头晕,后渐消失。伤后2个月始出现左面部麻木感,呈阵发性,每次发作时间约10分钟,伴间断性头痛及视物模糊。于半年前出现行走不稳,向左侧倾斜,伴左耳耳鸣及饮水发呛。既往体健,伤时正     

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.