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     

表现为癫癎、失语的多发性硬化1例

患者男 ,4 1岁 ,因抽搐、视力下降 11个月 ,语言、行为异常 2 5天入院。 11月前患者无诱因出现阵发性抽搐 1次 ,家人描述 :双眼向上凝视 ,面色紫绀 ,双上肢屈曲 ,双下肢伸直 ,呼之不应 ,持续几分钟后自行缓解 ,无口吐白沫 ,二便失禁。半月后左眼视物模糊 ,继之右眼视力下降 ,无复视、眼睑下垂 ,诊断为多发性硬化 (MS) ,强的松 4 0mg/d治疗 2个月后症状完全消失 ,减量停药。 4个月前患者受凉后复发 ,左眼视力下降 ,无肢体麻木、疼痛、无力 ,当地医院强的松 30mg/d治疗症状缓解 ,逐渐减量至 5mg/d维持。 2 5天前病员再次受凉 ,出现言语增多 ,…     

发作性抽搐、言语不能伴不自主张口

病历摘要 患者女性,24岁.主诉发作性抽搐、言语不能伴不自主张口2个月,于2008年6月20日入我院治疗.患者于2个月前自诉"受凉"后开始咯血,为鲜血,200 ml/d,无呼吸困难和窒息,当地医院诊断为"双侧下肺肺炎",予以左氧氟沙星(具体剂量不详)抗炎和垂体后叶素(具体剂量不详)止血治疗,2 d后症状缓解,继续应用上述药物.2周后患者出现反应迟钝,言语不清,突发意识障碍,双眼上翻,四肢强直抽搐,伴小便失禁,持续10 min后自行缓解.     

精神行为异常 肢体抽搐5个月

正病例摘要患者女性,17岁,因精神行为异常、反复肢体抽搐5个月,于2016年5月13日入院。患者于入院前5个月(2015年12月5日)出现鼻塞、流涕;3 d后(12月8日)出现发热,体温最高达38℃,随后出现幻听、失眠;2 d后(12月10日)出现言语不清,对答欠合理,伴双手不自主抖动,无下肢不自主运动,无乏力、肌肉疼痛,无头痛、恶心、呕吐,无意识障碍;无大小便失禁,于2015年12月11日入当地医院住院治疗。腰椎     

感觉性共济失调型慢性炎症性脱髓鞘性多发性神经病1例报告

感觉性共济失调型慢性炎症性脱髓鞘性多发性神经病(CIDP)临床较少见,现报告1例如下. 1 病例 男,45岁.因"四肢无力、麻木2个月,走路不稳5d"于2012年11月16日入院.患者2个月前无诱因下出现两下肢麻木、刺痛、无力,持续加重,渐累及两上肢.于1个月前住我科,诊断为"Guillain-Barre综合征".给予地塞米松10mg/d、免疫球蛋白27.5 g/d静脉滴注,4d后改为甲泼尼龙500 mg/d静脉滴注,连用3d后减为80 mg/d.15 d后患者四肢无力、疼痛缓解,但仍有四肢麻木,出院;继用泼尼松(60mg/d)治疗.     

发作性左侧肢体麻木抽搐

病历摘要患者男性,75岁。主因发作性左上肢麻木3个月、左上肢抽动2 d,于2011年2月5日入北京协和医院神经科接受治疗。患者于3个月前无明显诱因出现发作性左上肢麻木,以左手更为显著,每次发作持续8～10 min,可自行缓解,发作1～2次/d,未曾诊治。发病前2 d出现发作性左侧上肢抽搐,不伴意识障碍、双眼上翻、口角流涎、大小便失禁及肢体无力等症状与体征,持续2～3 min后自行缓解,每日发作20余次;1 d前出现左上肢持续抽搐,不伴意识障碍,但症状     

椎管内结核瘤1例报告

女性,22岁,因腰腹部疼痛8个月,不能行走10d入院。患者8月前因不明原因出现腰部隐痛,继而发生右下腹部疼痛,无恶心呕吐,腹胀腹泻,在当地医院行“阑尾切除术”后腹痛缓解,但腰痛逐渐加重,服中、西药无明显好转。入院10d前出现双下肢不能行走,以右脚为甚;早上较重,稍活动后缓解;无麻木抽搐,无结核病史。检查:腹股沟以下浅感觉减退,双下肢肌力下降,腱反射亢进,双侧病理征阳性。全身检查无     

第2例--头痛、行为异常、反应迟钝半个月余

患者，男，43岁，主因“头痛、行为异常、反应迟钝半个月”于2002年3月6口入宣武医院神经科治疗。患者半个月前无明显诱凼出现头痛，以左颞部为主，旱搏动性，能忍受，无呕吐及发热．1d后自行缓解。发病后第3天曾于上班途中出现辨不清方向，不认识熟人 很快恢复正常，未予注意。10d前出现反应迟钝，行为异常．表现为不认识熟人．听不懂别人说话，答非所问．有时胡言乱语，伴寒战、发热．体温     

多系统萎缩与“十字征”一例

患者男性,64岁。主因排尿困难5年,尿潴留2 d,于2010年10日30日入院。患者于6年前(2004年)因右侧上肢震颤行头部MRI检查未发现异常影像(图1),临床诊断为帕金森病,并开始口服美多巴125 mg/d,6个月后因症状无好转。在非专科医师的指导下逐渐加量(但服药期间时有减量,时有中断),至此次入院前药物剂量已达750 mg/d,但震颤无任何缓解,于发病后约1年逐渐出现吞咽困难、饮水呛咳、构音障碍、尿频尿急、排尿不尽、严重便秘、性功能障碍等     

发作性左侧肢体麻木抽搐

患者男性，75岁。主因发作性左上肢麻木3个月、左上肢抽动2d，于2011年2月5日入北京协和医院神经科接受治疗。患者于3个月前无明显诱因出现发作性左上肢麻木，以左手更为显著，每次发作持续8～10min，可自行缓解，发作1～2次，d，未曾诊治。     

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.     

Diagnostic Difficulties and Treatment Implications

Summary: Differentiation between types of epileptic seizures has been aided in recent years by the introduction of intensive neurodiagnostic techniques and the development of increasingly detailed classification systems. Paradoxically, these developments have not simplified the task of matching the appropriate antiepileptic drug to a particular seizure type. It is reasonable to assume that anticonvulsant drugs will have different effects on different types of seizures, but faulty, circular reasoning can enter the picture if one also assumes that responses of seizures to different drugs signify different seizure types. There are several examples of differential diagnoses that can fall prey to this problem, including the diagnosis between partial seizures with secondary generalization and generalized tonic-clonic seizures, and the diagnosis between complex partial seizures and absence seizures with automatisms, among others. Considerations of etiology in future classification systems can further complicate the problem: should one then choose an anticonvulsant drug on the basis of individual seizure type or on the basis of the type of epilepsy? Ramifications of this issue extend even to the drug approval process. Official sanction is not given for use of a drug for a seizure type not included in the original efficacy studies, even if later scientific evidence shows that seizure type to be related to a type that is included. New trials must be undertaken. These problems arise from how we choose to classify seizures.     

Cognitive Dysfunction Associated with Antiepileptic Drug Therapy

Summary: Epilepsy is frequently associated with cognitive dysfunction. However, the reasons for this correlation are unclear. Possible influential factors include patient age; duration, frequency, etiology, and type of seizures; hereditary factors; psychosocial issues; and antiepileptic drug (AED) therapy. Whereas many of these factors are beyond the physician's control, AED therapy is one element that can be addressed in treatment decisions by recognizing the potential cognitive effects of particular AEDs. For example, phenobarbital impairs memory and concentration; phenytoin affects attention, problem solving ability, and performance of visuomotor tasks. In contrast, carbamazepine may affect concentration, while valproate would appear to have minimal effects on cognition. Moreover, cognitive effects of AEDs are amplified with coadministration of multiple anticonvulsants (polytherapy). A review of studies on the cognitive effects of monotherapy with AEDs, as opposed to those of polytherapy, provides evidence that drug-related cognitive dysfunction can be reversed if patients are switched to a simpler therapeutic regimen. Future research should be directed toward developing reliable measures for assessing and monitoring cognition, and understanding the particular cognitive side effects of each AED. Physicians also need to revise their opinions about which side effects are "tolerable" for epileptic patients.     

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.     

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.     

Carbamazepine Efficacy in Adults With Partial and Generalized Tonic-Clonic Seizures

Summary: Carbamazepine and phenytoin are drugs of choice in initial monotherapy for adult partial and secondarily generalized tonic-clonic seizures. These designations reflect the results of the Veterans Administration Epilepsy Cooperative Study Group of 1985. An earlier comparative study of carbamazepine and phenytoin by Ramsay and associates found both drugs equally effective in controlling new-onset seizures. Among the advantages of carbamazepine is that it causes relatively few cognitive and dysmorphic side effects. Its disadvantages are its unavailability in parenteral formulation and its metabolic autoinduction. The latter must be compensated for by planned dosage increases to maintain therapeutic plasma steady-state levels during the first 2 or 3 months of treatment. Carbamazepine is judged a drug of choice in the treatment of these secondarily generalized tonic-clonic seizures, and the drug of choice in children, adolescents, and women susceptible to the dysmorphic side effects associated with other anticonvulsant agents.     

Etiologic and Preventive Aspects of Epilepsy in the Child–Bridging the Gap Between Laboratory and Clinic

Summary: Four broad categories of basic phenomena are pertinent to developing ways to prevent epilepsy. These include mechanisms of epileptogenesis, ictal initiation and temporary entrainment by the seizure discharge of normally functioning brain, seizure propagation, and control mechanisms that function both to restrain the cascade of epileptic events culminating in a seizure and to arrest the epileptic event and restore the interictal state. In newborns and children, hypoxia-ischemia is a major factor leading to epileptogenesis, and several schemes are proposed to classify, quantify, and prevent hypoxic-ischemic encephalopathy. Control mechanisms must be better understood in order to develop prophylactic recommendations for epilepsy, and an experimental model of "kindling antagonism" may increase our understanding of these. Programs of prevention of seizures in children will evolve only if basic researchers and clinicians work productively together to develop an adequate understanding of factors important in epileptogenesis and antiepileptogenic control mechanisms.     

Predisposing and Causative Factors in Childhood Epilepsy

Summary: We review information from large studies of defined populations, examining the role of known factors and especially of prenatal and perinatal factors in contributing to nonfebrile seizure disorders of early childhood. We depend especially, but not exclusively, on the recently completed analyses from the Collaborative Perinatal Project of the National Institute of Neurological and Communicative Disorders and Stroke, the NCPP. About 4% of children in the NCPP who had at least one non-febrile nonsymptomatic seizure by the age of 7 years had a previous seizure during acute neurologic illness, such as meningitis or during the acute illness after trauma. Many such seizures should potentially be preventable. Of children with seizures, 10% had had a neonatal seizure and 13% had had a febrile seizure. Among the hundreds of prenatal and perinatal factors explored as predictors of childhood seizure disorders, the principal predictors identified were congenital malformations of the fetus, cerebral and noncerebral; family history of certain neurologic disorders; and neonatal seizures. In agreement with the British National Child Development Study, labor and delivery factors in the NCPP appeared to contribute very little to childhood seizure disorders. Maldevelopment, rather than damage at birth to an initially intact nervous system, appeared to be the more common mechanism. Most seizure disorders of early childhood remained unexplained by the large set of prenatal and perinatal characteristics examined.     

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.