以癫痫发作为首发症状的自身免疫性脑炎47例临床分析

目的 分析以癫痫发作为首发症状的自身免疫性脑炎(AE)的临床特点.方法 分析以癫痫发作为首发症状的AE的患者47例的自然信息、临床特点、影像学检查、实验室检测结果、免疫治疗药物等.结果 47例患者中,男性29例,女性18例;发病年龄18～79岁,年龄中位值52岁;抗富亮氨酸胶质瘤失活基因-1(leucinie-rich...     

脑囊虫病临床分析及丙硫咪唑治疗观察：（附30例报告）

我院自1988年1月至1991年9月住院治疗脑囊虫病30例,均经CT及囊虫免疫试验证实,用丙硫咪唑治疗,收到较满意效果,现报告如下: 临床资料男19例(66.33％),女11例,农村17例,城市13例。发病年龄为20～65岁,以20～50岁多见(80％)。病程数月至数年,长者可达15年。两年内占83.33％。癫痫发作21例(70％)。大多数为癫痫大发作,部分患者为局限性发作,小发作,其它类型的癫痫发作比较少见。颅内压增高19例(63.33％),主要表现头痛、恶心、呕吐、视力下降甚至失明,视乳头水肿、视网膜出血。脑损害症状14例(46.67％)。表现意识障碍、精神症状、智力减退、颅神经损害和肢体运动障     

儿童头痛型癫痫

头痛型癫痫是内源型癫痫的一种类型，发病者多为儿童，国内文献共有６１３例头痛型癫痫，其中儿童占８０％。本组３０例中７～１０岁的儿童２１例占７０％。儿童以发作性头痛为主要表现。头痛突发无前驱症状。发作时间短暂，一般为几分钟至几十分钟。发作时多有意识障碍，...     

Kleine-Levin综合征

目的报道1例Kleine-Levine综合征,探讨其临床和影像学特点和可能的发病机制。方法对1例周期性发作性嗜睡的患者进行临床、影像学检查。结果患者临床表现为反复发作性嗜睡、精神行为异常,MRI见左侧丘脑类圆形长T2信号影。结论 Kleine-Levin综合征的诊断主要依赖典型的临床症状,主要表现为青少年周期性发作的嗜睡和精神行为异常,丘脑功能失调是Kleine-Levin综合征一个可能的发病机制,MRI见丘脑上有病灶时对诊断本病具有提示意义。     

30例抗γ氨基丁酸B受体脑炎临床特点分析

目的 探讨抗GABABR抗体脑炎的临床表现、脑脊液、头部MRI及预后特点。方法 收集我院2015年12月-2019年6月收治的抗GABABR抗体脑炎患者30例,总结其临床表现、脑脊液、头部MRI特点,并进行随访,评估预后。结果 30例患者平均发病年龄为58岁(15～84岁),男性18例,女性12例。癫痫发作为最常见的首发症状,并以癫痫发作(26例,86. 67%)、精神行为异常(26例,86. 67%)、认知功能损害(24例,80. 00%)为主要临床表现。脑脊液以淋巴细胞比例及白细胞计数升高为主要特点。头部MRI可见内侧颞叶、海马异常信号(10例,35. 71%)。易合并肺癌(特别是小细胞肺癌),在平均随访11 m后近半数患者死亡(13例,48. 15%),死亡患者大多合并肺癌(9例,69. 23%)。结论 抗GABABR抗体脑炎主要表现为癫痫发作,影像学可见颞叶内侧、海马异常信号,同肺癌(小细胞肺癌)密切相关,近半数患者预后不良。     

以癫痫为首发症状的脑型血吸虫病47例分析(摘要)

９８８年６月至１９９７年１０月，收治了以癫痫为首发症状，临床检查诊断为脑型血吸虫病共４７例，其中手术２９例。本组男３８例，女９例。年龄１３～５０岁。临床症状，均以癫痫为首发症状就诊，首次发作为癫痫大发作者１２例，局限性癫痫发展成全身大发作者１８例，局...     

抗γ-氨基丁酸B型受体脑炎的临床特征

目的 分析抗GABA B型受体(GABAB R)脑炎的临床特征,血清和CSF抗体敏感度、滴度变化以及癫痫的治疗.方法 回顾性分析13例抗GABAB R脑炎患者的临床资料.结果 本组男性8例(61.5％),女性5例(38.5％);中位年龄52岁(41～61岁).10例(76.9％)首发症状为癫痫.所有患者均出现痫性发作,...     

症状性癫痫158例临床分析

目的 探讨症状性癫痫的临床特点。方法 回顾性分析158例症状性癫痫的发病年龄、发作类型病因、脑脊液、脑电图、治疗及转归。结果 20－39岁发病者113例（占71．57％）,单纯部分性发作72例（占45．57％）,感染者70例（占44．30％）,脑血管疾病48例（占30．38％）,15例弥漫性慢波患者12例确诊病因为病毒性脑炎（占80％）,本组158例中,25例后遗症期,长期抗癫药物维持,11例死亡。结论 （1）症状性癫痫患者的发病年龄仍以中青年为主;（2）发作类型以单纯部分性发作为主;（3）主要病因为感染和脑血管疾病;（4）脑寄生虫病的诊断：脑脊液检查特异性高;（5）病毒性炎导致全脑弥漫性损害较多见,脑电图显示弥漫性慢波;（6）积极治疗原发病,同时给予抗癫痫药物治疗,多数预后良好,原发病控制不佳者,预后较差,病死率较高。     

中国人婴儿惊厥伴发作性手足舞蹈徐动征临床特征分析

目的探讨我国婴儿惊厥伴发作性手足舞蹈徐动征(ICCA)患者的临床特点。方法分析诊断为ICCA的5例患者的临床表现、脑电图(EEG)、影像学和治疗转归的临床资料。ICCA的入选标准为：3～20个月出现良性婴儿惊厥(BFIC)和在儿童后期或青春期出现发作性运动源性运动障碍(PKC)。结果5例ICCA患者来自4个家系,男性4例,女性1例。BFIC发病年龄在8～12个月,PKC发病年龄在5岁以后,影像学未见异常,部分患者EEG有局限性放电和慢波增多。卡马西平、苯妥英、拉莫三嗪治疗有效。其中2例合并继发性癫痫。ICCA家系中其他发作性疾病患者共9例。结论ICCA可能为常染色体显性遗传疾病,我国该病患者同时具有BFIC和PKC的特征性表现和治疗转归特点,家系中可能有PKC和其他类型的癫痫患者,说明PKC与癫痫之间可能存在某些相似的发病机理,此还有待于进一步研究。     

20例睡眠期电持续状态患者临床特征及分析

目的回顾性分析临床癫痫电持续状态患者临床资料,提高临床医师对睡眠期癫痫性电持续状态(Electrical status epilepticus during sleep,ESES)的认识和诊疗水平。方法收集2018年—2019年我院神经内科收治经视频脑电图(VEEG)和临床确诊的20例ESES患者病例,分析并总结临床症状、脑电图(EEG)特征、发作类型及癫痫综合征分类、影像学表现。结果 20例患者中男12例、女8例,平均年龄(10.96±2.68)岁,首发年龄(8.90±1.93)岁,癫痫发作为首发症状者最为多见,EEG均提示有广泛性或局限性的持续放电,全面强直-阵挛发作是多数患者的主要发作形式,相应的癫痫综合征中以癫痫伴慢波睡眠期持续棘慢波(Epilepsy with continuous spike-and-waves during slow-wave sleep,ECSWS)最为突出。对于微小的病灶通过磁共振成像可以早期发现。结论 ESES多数患者会有部分或全面性癫痫发作形式,留有广泛的认知损害,智力下降等问题。通过长程脑电图监测可以早期发现和诊断,对改善患者的认知功能、行为、神经心理有着重要的意义。     

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.     

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.