非惊厥性癫(癎)持续状态

非惊厥性癫(癎)持续状态(NCSE)系临床常见但易忽视的癫(癎)持续状态(SE)发作类型,据估计,占所有癫(癎)持续状态的20%～50%.2004年,英国癫(癎)研究基金会(ERF)将非惊厥性癫(癎)持续状态定义为:由于持续性癫(癎)样脑电活动导致的一系列非惊厥临床征象[1].     

非惊厥性癫(癎)持续状态的现代观点

2001年,国际抗癫(癎)联盟(ILAE)取消了惊厥性癫(癎)持续状态(CSE)和非惊厥性癫(癎)持续状态(NCSE)的分类,但近年实践发现这种传统分类方法具有其合理性,因此仍被临床广泛应用.     

欧洲神经病学协会联盟的成人癫(癎)持续状态管理指南

本文目的旨在评估文献及探讨针对成人癫(癎)持续状态(SE)各种治疗策略的证据等级.我们对1966年- 2005年1月间MEDLINE和EMBASE上发表的相关文献及2005年1月～2009年1月间最新版本的所有相关出版物进行检索.此外,还对Cochrane临床对照试验注册中心(CENTRAL)进行检索.推荐就是基于这些文献以及我们对主题词相关参考文献的判断并通过建立共识的方式获得.若缺乏证据但共识明确,我们提出良好实践指导.全面性惊厥性癫痫持续状态( GCSE)的推荐治疗路径为直接予以4～8 mg劳拉西泮或10 mg地西泮,继以18 mg/kg苯妥英静脉注射.如首次给药后癫(癎)发作仍持续10 min以上,建议再次予以4 mg劳拉西泮或10 mg地西泮静脉注射.难治性GCSE需要麻醉刺量的巴比妥、咪唑安定或异丙酚治疗,药物剂量需以脑电图发放被抑制来滴定至少24 h.非惊厥性SE的初始治疗取决于其发作类型和病因.复杂部分性SE的初始治疗同GCSE,若难治则予以左乙拉西坦、苯巴比妥或丙戊酸等非麻醉类药物.对于不明显的SE(subtle SE),大多需要静脉注射麻醉药.     

儿童睡眠中癫(癎)性电持续状态36例临床分析

目的:总结儿童睡眠中癫(癎)性电持续状态(ESES)的临床、脑电图(EEG)、神经心理障碍的特点及对治疗的反应.方法:对36例ESES患儿进行V-EEG监测,随访观察临床、EEG、神经心理障碍的情况以及治疗效果.结果:36例中27例(75%)经治疗临床发作完全控制或明显减少,EEG上ESES消失.22例使用肾上腺皮质激素甲基泼尼松龙冲击治疗中的17例(77%)在临床发作改善和抑制(癎)样放电方面都有良好的疗效.32例神经心理障碍患儿26例(81%)经治疗后有明显改善.结论:ESES是一种特殊的EEG现象,非快速眼动(NREM)睡眠期持续放电是神经心理障碍的主要原因.抗癫(癎)治疗除控制其临床癫(癎)发作外,还必须及早消除EEG(癎)样放电持续状态.     

癫(癎)持续状态与脑电图

癫(癎)持续状态(SE)是神经科常见急症之一,若不能尽早终止发作,可导致神经元永久性损害和患者死亡[1-4].除基础病因、年龄等因素外,癫(癎)持续状态持续时间是影响预后的重要因素.根据癫(癎)持续状态的实验研究及临床观察发现,癫(癎)发作持续时间>30 min,即会演变得难以控制[5].     

非惊厥性癫癎持续状态

非惊厥性癫癎持续状态（NCSE）系临床常见但易忽视的癫癎持续状态（SE）发作类型,据估计,占所有癫癎持续状态的20％～50％。2004年,英国癫癎研究基金会（ERF）将非惊厥性癫癎持续状态定义为：由于持续性癫癎样脑电活动导致的一系列非惊厥临床征象。     

癫(癎)性痉挛发作的头皮及颅内脑电图特点

目的:研究难治性癫(癎)性痉挛发作患者的头皮及颅内脑电图(EEG)特点,探讨与痉挛发作相关的EEG变化及其与发作间期放电、神经影像学之间的关系.方法:回顾性分析经外科手术治疗的11例患者的临床资料,分析头皮同步视频脑电图(V-EEG).此11例患者均行术中皮层EEG监测30-60min,其中4例术前行颅内电极长程EEG...     

小儿癫(癎)持续状态的治疗体会

癫(癎)持续状态(SE)又称癫(癎)状态,是指癫(癎)持续频繁的发作,持续 30 min以上或连续多次发作,发作期间意识或神经功能不能恢复至正常水平[1].癫(癎)持续状态下脑缺氧、代谢中间产物蓄积,造成脑水肿、神经元死亡,是小儿神经内科急危重症.我科于2008-08～2010-02,收治32例SE患者,现报道如下.     

部分性癫(癎)持续状态

关于癫(癎)持续状态(SE),以往关注较多的是全面性强直-阵挛性癫(癎)持续状态(即大发作持续状态).实际上,同样作为特殊发作类型,部分性癫(癎)持续状态(PSE)并不比大发作持续状态发生率低,只是关注度不够而已.笔者拟从临床角度对部分性癫(癎)持续状态的相关问题进行概述.     

昏迷患者非惊厥性癫痫持续状态的临床特点

目的 探讨昏迷患者非惊厥性癫痫持续状态(NCSE)的临床特点.方法 回顾性分析6例昏迷NCSE患者的临床资料.结果 6例患者中1例病毒性脑炎,1例大面积脑梗死,2例脑外伤,1例心肺复苏术后缺血缺氧性脑病,1例肾移植后代谢性脑病.其中,2例出现躁动,1例摇头、嘴唇咂动,2例四肢微小抽动,1例颜面抽动.所有患者的持续EEG(CEEG)监测均出现广泛或者局灶并发广泛的异常放电.结论 昏迷患者发生NCSE时多表现为躁动或面部、四肢微小抽动,CEEG监测能发现异常放电,有助于指导临床诊治.     

表现为非惊厥性癫痫持续状态的边缘叶脑炎的临床和脑电图特征

目的探讨为非惊厥性癫痫持续状态(NCSE)的边缘叶脑炎(LE)的临床及EEG特征。方法回顾性分析9例表现有NCSE的LE患者的临床资料。结果 4例患者为急性起病,5例为亚急性起病。首发症状为复杂部分性癫痫持续状态(CPSE)7例,轻微发作癫痫持续状态(SSE)1例,简单部分性癫痫持续状态(SPSE)1例。9例患者均有精神症状、记忆障碍及自主神经功能紊乱,肺癌1例。头颅MRI显示脑实质急性炎症,主要集中于边缘系统,呈双侧对称或不对称信号异常改变,T_1WI为略低信号,T_2WI及Flair呈高信号。EEG表现为θ波背景6例,均可见δ波,其中棘慢波或尖慢波4例;α波背景2例,均可见δ波,表现为δ波背景1例。视频脑电图(VEEG)示1例SSE患者呈持续的痫性放电,在病侧蝶骨电极更显著,但无运动性癫痫发作。1例SPSE患者在皮质和颞近中央区有不同频率的局灶性棘波或棘慢综合波持续发放。7例CPSE患者呈颞区为主的各种形式癫痫性电活动广泛持续发放或反复阵发性出现,如节律性的棘波、尖波、δ或/和θ节律,可向邻近区域或对侧半球扩散,或左右交替;在无凝视反应或刻板自动症时呈现扩散至双侧半球的高波幅棘慢综合波或δ节律爆发。结论表现有NCSE的LE的临床和EEG有特征性改变,EEG和VEEG是LE是否存在NCSE的主要诊断依据。左右半球边缘叶病变出现的精神症状并不相同。各型LE对治疗反应不一,非副肿瘤性LE疗效较满意。     

Efficacy and safety of intravenous lacosamide in refractory nonconvulsive status epilepticus

Koubeissi MZ, Mayor CL, Estephan B, Rashid S, Azar NJ. Efficacy and safety of intravenous lacosamide in refractory nonconvulsive status epilepticus.
Acta Neurol Scand: 2011: 123: 142–146.
© 2010 John Wiley & Sons A/S. Background – Lacosamide (LCM) is a novel antiepileptic drug (AED) recently approved as an adjunctive therapy in the treatment of partial seizures in adults. LCM is available in oral and intravenous formulations, has linear pharmacokinetics and a unique mechanism of action. The aim of this study – To evaluate the safety and efficacy of intravenous LCM in the treatment of nonconvulsive status epilepticus (NCSE) after failure of conventional therapy. Methods – We retrospectively reviewed all patients with NCSE treated with LCM. We reviewed the clinical and electrographic changes before and after LCM administration. We also noted any reported side effects including electrocardiographic changes. Results – We report four cases of NCSE that were refractory to conventional treatment, but readily responsive to LCM. No side effects attributable to LCM were identified. Conclusions – Intravenous LCM may be safe and efficacious as an add‐on AED for the treatment of NCSE when standard therapy fails.     

2例非惊厥癫痫持续状态患者的临床特征及脑电图分析

非惊厥性癫痫持续状态（Nonconvulsive Status Epilepticus,NCSE）年发病率为5.6～18.3/10万[1],约占癫痫持续状态的一半,并非罕见,以往由于对其认识不足和诊断标准不一致[2],常被误诊或漏诊,得不到及时有效的治疗.不同类型的NCSE,临床表现和脑电图特点各不相同[3].现将首都医科大学宣武医院神经内科2012年7～10月收治的2例NCSE患者的临床特征及脑电图结果报道如下.     

The ketogenic diet in treatment of two adults with prolonged nonconvulsive status epilepticus

Prolonged status epilepticus (SE) can be refractory to conventional interventions, with high rates of subsequent morbidity and mortality. A high fat, low protein, low carbohydrate ketogenic diet (KD) has been used successfully to treat intractable epilepsy. However, its possible role in prolonged SE has not been well described. We report successful use of the KD in two adult patients with prolonged nonconvulsive SE (NCSE) refractory to multiple other interventions. Our observations suggest induction of ketosis may be a novel strategy to safely and effectively treat status in adults even after weeks to months of refractory seizures. Although there are few data regarding the use of the ketogenic diet in the treatment of adult epilepsy syndromes, it may be an option for the treatment of adults with refractory, prolonged SE.     

Orofacial automatisms induced by acute withdrawal from high-dose midazolam mimicking nonconvulsive status epilepticus in a child.

Nonconvulsive Status Epilepticus (NCSE) is not uncommon in children, and can be challenging to diagnose and treat. Etiologies vary widely and include infection, trauma and acute withdrawal from medications such as anticonvulsants. We report a child who experienced orofacial dyskinesias concerning for NCSE after withdrawal from high dose benzodiazepines andopiates. Automonic signs typically associated with sedative withdrawal were absent and treatment with benzodiazepines did not improve his symptoms. Diagnostic testing was negative, including electroencephalogram, and resolution was complete within five days. Our case demonstrates the orofacial dyskinesias that may occur during sedative medication withdrawal, and highlights potential confusion with non-convulsive status epilepticus.     

成人顽固性癫痫持续状态的临床分析

目的 探讨成人顽固性癫痫持续状态（RSE）的危险因素、临床特点、治疗及预后。方法 54例癫痫持续状态（SE）．58次发作事件，分为RSE组和非顽固性癫痫持续状态（NRSE）组．对病因、诱因、临床表现、辅助检查、预后等进行对比分析。结果 RSE占SE的43．1％，病毒性脑炎是RSE最主要的病因（P=0．001），相反，既往癫痫发作在NRSE中更常见（P=0．000），相应地药物治疗的改变引起的SE多为NRSE（P=-0．003）；RSE组GCS评分及预后较NRSE组均差（均P=0．000）。结论 SE经一、二线抗癫痫药治疗后仍有很大一部分难以控制，病毒性脑炎是导致RSE的一个重要病因，其预后较差．目前对RSE的治疗还缺乏十分合理的方案。     

Status epilepticus in the elderly

Children and the elderly (≥60 years of age) have the highest incidence of status epilepticus (SE). Because of their general health, elderly individuals are much more likely than younger (<60 years of age) persons to have more severe consequences from seizures. The incidence of SE is 15.5/100 000 in the 60‐69 age group, 21.5/100 000 in the 70‐79 age group and 25.9/100 000 in persons 80 and older. The most common cause in the elderly is acute symptomatic, with stroke and hypoxia the most frequent. The overall mortality of SE is quite high and occurs early, often within the first few days, and is related to the cause, with mortality of more than 80% in persons with anoxia. Although the cause of SE is an important factor in mortality, the aging body and brain may contribute to an unfavorable outcome. Treatment in the elderly is essentially the same as in younger adults with benzodiazepines (lorazepam, diazepam, clonazepam) and longer acting antiseizure drugs (phenytoin, fosphenytoin, valproate, levetiracetam, and lacosamide. At this time there are no evidence‐based studies regarding Axis 2 (etiology) and Axis 4 (age). All current interventions for SE involve antiseizure drugs that were developed for treatment of chronic epilepsy. Treatments should be developed that are more specific for the various etiologies and involve drugs that work on the underlying cause of the SE.     

Oral ketamine controlled refractory nonconvulsive status epilepticus in an elderly patient

Nonconvulsive status epilepticus (NCSE) is a heterogeneous disorder with different seizure types and diverse etiologies, and is mainly characterized by altered consciousness. The recognition of NCSE is more challenging than generalized convulsive SE, and diagnosis and treatment are often delayed. Therefore, some cases can evolve into refractory SE and become pharmacoresistant even with GABAergic anesthetics. Herein we report the successful clinical experience of pharmacoresistant complex partial SE treated with ketamine. An elderly woman was profoundly stuporous and had relentless clonic movements of the right hand and forearm. Electroencephalography revealed repetitive periodic lateralized epileptiform discharges (PLEDs). There was a poor clinical response to standard anticonvulsants and GABAergic anesthetics. Both the clinical and electroencephalographic SE were controlled after intravenous ketamine therapy. Rebound refractory NCSE occurred about six days after discontinuing the intravenous ketamine, which was successfully terminated by oral ketamine treatment. There were no adverse effects observed.     

Clinical risk factors for non-convulsive status epilepticus during emergent electroencephalogram

PurposeEmergent electroencephalograms (EmEEG) are performed to exclude non-convulsive status epilepticus (NCSE) but are resource-intensive. Prior studies have identified a seizure or seizures in the acute setting preceding the EmEEG request as a risk factor of NCSE but few other consistent clinical risk factors have been identified. We aimed to identify clinical risk factors for NCSE in EmEEGsMethodsWe conducted a retrospective analysis of consecutive patients who underwent EmEEG to exclude NCSE over a 20-month period. One blinded investigator extracted clinical information from patient case records using a standardized form. Patients were grouped using EmEEG results into those with and without NCSE. We analyzed differences between these two groups.ResultsA total of 2333 EEGs were performed over the study period, 215 (9.3%) were EmEEGs ordered to exclude NCSE. 21 patients (9.8%) of the 215 patients were found to have NCSE. Three independent clinical risk factors for NCSE were identified – seizure(s) in the acute setting, ocular movements (nystagmus and/or gaze deviation) and ongoing CNS infection. The presence of seizure(s) in the acute setting showed the highest adjusted odds ratio (OR = 8.8, 95% CI 2.0–39.4, p = 0.005). In addition, prevalence of NCSE increased as more clinical risk factors were present.ConclusionSeizures in the acute setting, ocular movements and ongoing CNS infection are associated with NCSE. By using these risk factors at the bedside, clinicians can prioritize patients for EmEEG, recognizing that risk of NCSE increases as more clinical risk factors are present.