结节性硬化的MRI表现与临床表现的关系探讨

结节性硬化是一种较常见的神经皮肤综合征,为常染色体显性遗传.主要临床表现为面部皮脂腺瘤、癫痫发作和智能减退.颅内病变以皮层结节、白质异常、室管膜下结节、室管膜周围巨细胞星形细胞瘤为主.皮层结节可能与癫痫发作和智能减退相关,本文介绍19例结节性硬化患儿的MRI情况,并探讨与临床表现间的关系.     

脑卒中后癫痫109例临床分析

目的探讨脑卒中后癫痫的临床特点及发病机制。方法对1262例脑卒中病例中109例继发性癫痫患者,就其癫痫的发生率、发生时间、发作类型以及发生机制等问题进行了探讨。结果脑卒中后癫痫的发生率为8.6%;急性期癫痫发作81例,占74.3%,恢复期癫痫发作28例,占25.7%;发作类型以强直-阵挛发作(包括强直-阵挛持续状态)最多见,其次为部分继发全面性发作。结论脑卒中后癫痫发作与病程和病变部位有关:出血性脑卒中在急性期易继发癫痫,病灶在脑叶者癫痫发生率高;发作类型以强直-阵挛发作最多见。     

癫痫宁片联合奥卡西平治疗癫痫的临床研究

目的 探讨癫痫宁片联合奥卡西平片治疗癫痫的临床疗效。方法 选取2021年1月-2022年1月在海南省老年病医院就诊的94例癫痫患者，按照计算机随机排列分为对照组和治疗组，各47例。对照组患者口服奥卡西平片，起始剂量1片/次，2次/d，每隔1周增加药物剂量，每次增加2片，分为2次口服，维持剂量2~8片/d。治疗组在对照组基础上口服癫痫宁片，4片/次，3次/d。两组连续治疗3个月。观察两组的临床疗效，比较两组的强直发作频率、阵挛发作频率、痫样放电数、累及导联数、棘波指数以及血清神经元特异性烯醇化酶（NSE）、白细胞介素-1β（IL-1β）、肿瘤坏死因子-α（TNF-α）水平。结果 治疗后，治疗组的总有效率为91.49%，对照组的总有效率为74.47%，组间比较差异有统计学意义（P<0.05）。治疗后，两组强直发作频率、阵挛发作频率明显低于治疗前（P<0.05）；治疗后治疗组强直发作频率、阵挛发作频率明显低于对照组（P<0.05）。治疗后，两组的痫样放电数、累及导联数、棘波指数显著降低（P<0.05）；治疗后治疗组的痫样放电数、累及导联数、棘波指数低于对照组（P<0.05）。治疗后，两组的血清NSE、IL-1β、TNF-α水平低于治疗前（P<0.05），治疗组血清NSE、IL-1β、TNF-α水平较对照组更低（P<0.05）。结论 癫痫宁片联合奥卡西平片能显著提高癫痫的临床疗效，减轻癫痫症状、脑部异常放电和神经细胞的炎症损伤。     

亚胺培南/西司他丁钠致癫痫的临床分析及治疗

目的：探讨亚胺培南/西司他丁钠致癫痫发作的防治措施。方法：从1例肺癌合并肺部感染患者使用亚胺培南/西司他丁钠临床病例出发,结合文献分析癫痫的发生机制及治疗方案。结果：该例患者用药结束6小时癫痫发作,给予地西泮10 mg肌注及停药处理,癫痫缓解。结论：癫痫为亚胺培南/西司他丁钠神经系统严重不良反应之一,应加强重视。     

脑性瘫痪伴癫痫患儿32例影像学及脑电图特征分析

目的探讨脑性瘫痪合并癫痫患儿的临床特点和影像学、脑电图特征。方法总结在该院小儿科住院的脑性瘫痪合并癫痫患儿的癫痫发生率、临床类型、头颅计算机体层成像(CT)、脑电图等方面的特点以及抗癫痫治疗效果。结果 139例脑性瘫痪患儿中有32例合并癫痫,占23.0%;常见的癫痫类型为强直—阵挛发作12例(37.5%)和部分性发作6例(18.8%);癫痫多发生于痉挛性脑性瘫痪(68.8%);25例(78.1%)癫痫首发于1岁前;合并癫痫患儿的头颅影像异常及脑电图异常分别为30例(93.8%)和27例(84.4%),而未合并癫痫患者分别为75例(81.5%)和20例(35.7%),脑电图异常率比较差异有统计学意义(P<0.05)。32例合并癫痫患儿常见的头颅异常为脑发育不良11例(36.7%)、脑积水6例(20.0%),余为脑萎缩、缺氧缺血性脑病样改变等13例(43.3%);脑电图异常中局灶性和弥漫性异常所占比例为48.2%和51.9%;81.2%的癫痫患儿需要2种及2种以上的抗癫痫药物治疗。结论脑性瘫痪患儿中癫痫的发生率较高,其头颅影像学和脑电图分别以脑发育不良和弥漫性异常为主;脑性瘫痪儿童的癫痫大多为难治性,需要联合用药治疗。     

脑性瘫痪伴癫痫患儿32例影像学及脑电图特征分析

目的 探讨脑性瘫痪合并癫痫患儿的临床特点和影像学、脑电图特征.方法 总结在该院小儿科住院的脑性瘫痪合并癫痫患儿的癫痫发生率、临床类型、头颅计算机体层成像(CT)、脑电图等方面的特点以及抗癫痫治疗效果.结果 139例脑性瘫痪患儿中有32例合并癫痫,占23.0%;常见的癫痫类型为强直-阵挛发作12例(37.5%)和部分性发作6例(18.8%);癫痫多发生于痉挛性脑性瘫痪(68.8%);25例(78.1%)癫痫首发于1岁前;合并癫痫患儿的头颅影像异常及脑电图异常分别为30例(93.8%)和27例(84.4%),而未合并癫痫患者分别为75例(81.5%)和20例(35.7%),脑电图异常率比较差异有统计学意义(P＜0.05).32例合并癫痫患儿常见的头颅异常为脑发育不良11例(36.7%)、脑积水6例(20.0%),余为脑萎缩、缺氧缺血性脑病样改变等13例(43.3%);脑电图异常中局灶性和弥漫性异常所占比例为48.2%和51.9%;81.2%的癫痫患儿需要2种及2种以上的抗癫痫药物治疗.结论 脑性瘫痪患儿中癫痫的发生率较高,其头颅影像学和脑电图分别以脑发育不良和弥漫性异常为主;脑性瘫痪儿童的癫痫大多为难治性,需要联合用药治疗.     

妥泰治疗儿童癫痫临床疗效观察

目的观察妥泰治疗儿童癫痫的临床疗效。方法癫痫患儿26例,其中部分性发作9例,全身强直-阵挛发作15例,失神2例,给予妥泰治疗。结果 26例癫痫患儿治疗12周后取得了良好的疗效,无发作的患儿超过1/3,无患儿因不良反应退出治疗。结论妥泰对部分性发作、全身强直-阵挛及失神癫痫患儿的治疗安全有效,副作用轻。     

新疆维吾尔族、汉族癫痫患儿拉考沙胺血药浓度、临床疗效和安全性的差异性研究

目的 探讨新疆维吾尔族、汉族癫痫患儿拉考沙胺(lacosamide，LCM)血药浓度与临床疗效的相关性和差异性。方法 采用超高效液相色谱法测定100例维吾尔族癫痫患儿和194例汉族癫痫患儿的血药浓度，观察其疗效和不良反应，运用Logistic回归和受试者操作特征曲线分析患儿血药浓度和疗效的相关性。结果 维吾尔族患儿的LCM癫痫治疗有效率显著低于汉族患儿(65.00%和78.68%，P<0.05)。维吾尔族患儿的LCM血药浓度显著高于汉族患儿[(7.13±2.88)μg·mL^–1和(6.27±2.53)μg·mL^–1，P<0.05]。维吾尔族、汉族有效组患儿接受LCM单一治疗的比例显著高于无效组，接受LCM合并细胞色素P450酶诱导剂治疗的比例显著低于无效组(P<0.05)。维吾尔族、汉族癫痫患儿的不良反应发生率分别为29.00%和36.60%，发生不良反应的维吾尔族患儿的LCM血药浓度显著高于未发生不良反应组患儿[(8.20±3.60)μg·mL^–1和(6.70±2.43)μg·mL^–1，P<0.05]。结论 临床使用LCM治疗不同民族癫痫患儿的过程中，有必要监测LCM稳态谷浓度，以提高疗效和降低不良反应发生率。     

动态脑电图联合NSE检测对复杂性与单纯性热性惊厥患儿的鉴别诊断价值

目的 探讨动态脑电图联合神经元特异性烯醇化酶（NSE）在复杂性与单纯性热性惊厥患儿中的鉴别诊断价值。方法 以2014年9月至2016年3月郑州儿童医院收治的80例热性惊厥患儿为研究对象,其中,单纯性32例（单纯组）,复杂性48例（复杂组）,同期选择郑州儿童医院80例体检健康儿童作为对照组。单纯组与复杂组入院后行7 d的动态脑电图监测,并在惊厥发作后12、48 小时对其NSE水平进行测定,且与对照组进行比较。单纯组与复杂组患儿均随访1年,记录癫痫的发生情况。结果 单纯组患儿脑电波异常率（18.75％）低于复杂组（37.50％）,差异有统计学意义（P<0.05）;单纯组与复杂组患儿脑电图异常部位额颞叶（50.00% 比 55.56%）、弥漫性（33.33% 比 16.67%）、顶枕叶（33.33% 比 27.78%）与时相Ⅰ相（66.67% 比 61.11%）、Ⅱ相（33.33% 比 38.89%）分布比较,差异均无统计学意义（P>0.05）。惊厥发作后12、48小时,NSE水平复杂组[（15.98±2.96）ng/mL、（13.25±2.96）ng/mL]>单纯组[（13.89±2.86）ng/mL、（11.05±2.05）ng/mL]>对照组[（9.20±2.12）ng/mL、（9.21±2.14）ng/mL],3组间比较差异均有统计学意义（P<0.05）。惊厥发作后48小时,单纯组与复杂组NSE水平均低于惊厥发作后12小时的水平（P<0.05）。单纯组1年癫痫发生率9.36%,低于复杂组的27.08%（P<0.05）。动态脑电图监测与NSE联合检测的ROC曲线下面积为0.96（P<0.05）。结论 复杂性热性惊厥患儿脑电波异常率、NSE水平、癫痫发生率均高于单纯性热性惊厥患儿,动态脑电图监测与NSE联合检测的灵敏度与特异度较高。     

奥卡西平单药和添加治疗儿童癫痫的疗效与安全性

目的 观察奥卡西平治疗儿童部分性发作（PS）和全面性强直-阵挛发作（ GTCS）癫痫患者的疗效、耐受性和副作用. 方法 73例癫痫患儿, 其中56例新诊断者进入单治组, 17例应用过其他抗癫痫药物治疗者进入加治组. 奥卡西平起始剂量5～10 mg&#8226;kg^-1&#8226;d^-1, 最大剂量30～40 mg&#8226;kg^-1&#8226;d^-1, 维持剂量中位值20 mg&#8226;kg^-1&#8226;d^-1, bid. 加治组其他抗癫痫药物的使用不变. 通过自身对比开放性观察, 分析单治组与加治组52周的疗效、不良反应、耐受性和安全性. 结果 全部患者总有效率84.9%、控制率54.8%、累积退出率11.0%, 其中3例（4.1%）失访, 不良反应或其他原因退出者5例（6.8%）, 其中因皮疹退出2例（2.7% ）. 常见不良反应：乏力8例、烦躁7例, 困倦和皮疹各2例. 单治组临床控制率（62.5%）显著高于加治组（29.4%）. 结论 奥卡西平是治疗儿童部分性发作和全面性强直-阵挛发作癫痫相对理想的药物选择.     

126例老年癫癎的临床分析

1991～1996年我院住院的老年癫癎患者126例,占我科同期老年人住院2520例的5％。男90例,女36例,年龄60～78岁,平均(69.34±3.28)岁。类型:强直-阵挛发作75例(59.5％),部分性发作30例(23.8％),其中21例由部分性发作转为全身性发作。精神运动性发作3例(2.4％);癫癎持续状态18例(14.3％),其中死亡8例(44.4％)。CT异常率88.1％,脑电图异常率92.9％,其中以各导联持续性慢波伴散在尖波或尖慢综合波为多。癫癎病因以脑血管病占多数(78例,61.9％)。病灶部位与癫癎有明显关系,脑血管病发作类型以部分性发作多见,病灶位于额顶区或累及额顶区者易发病,蛛网膜下腔出血、脑栓塞更易出现癫癎。其余依次为脑肿瘤(转移瘤多于原发瘤)、颅内炎症、糖尿病、心血管病。另有3例糖尿病患者发生癫癎,表明糖尿病出现癫癎应重视血糖的控制。2例风湿性心脏病也出现癫癎发作,推测与心脏射血指数下降而致大脑缺血有关。鉴于老年患者机体应激能力差、脑细胞活性低、细胞膜稳定性差、兴奋性高、癎阈低,所以对老年人癫癎病因的早期诊断及治疗至关重要。     

Single centre 20 year survey of antiepileptic drug-induced hypersensitivity reactions

BackgroundEpilepsy is a chronic neurological disease which affects about 1% of the human population. There are 50 million patients in the world suffering from this disease and 2 million new cases per year are observed. The necessary treatment with antiepileptic drugs (AEDs) increases the risk of adverse reactions. In case of 15% of people receiving AEDs, cutaneous reactions, like maculopapular or erythematous pruritic rash, may appear within four weeks of initiating therapy with AEDs.MethodsThis study involved 300 epileptic patients in the period between September 1989 and September 2009. A cutaneous adverse reaction was defined as a diffuse rash, which had no other obvious reason than a drug effect, and resulted in contacting a physician.ResultsAmong 300 epileptic patients of Neurological Practice in Kielce (132 males and 168 females), a skin reaction to at least one AED was found in 30 patients. As much as 95% of the reactions occurred during therapies with carbamazepine, phenytoin, lamotrigine or oxcarbazepine. One of the patients developed Stevens-Johnson syndrome.ConclusionSome hypersensitivity problems of epileptic patients were obviously related to antiepileptic treatment. Among AEDs, gabapentin, topiramate, levetiracetam, vigabatrin, and phenobarbital were not associated with skin lesions, although the number o patients in the case of the latter was small.     

急性期癫痫发作的病毒性脑炎临床及脑电图分析

目的探讨急性期癫痫发作的病毒性脑炎临床及脑电图特点。方法选取我院收治的急性病毒性脑炎患者229例,根据患者是否有癫痫发作分为癫痫发作组85例和无癫痫发作组144例。分析患者癫痫发作情况,对比观察癫痫发作患者与无癫痫发作患者的脑电图、视频脑电图（VEEG）、磁共振成像（MRI）及预后情况。结果229例患者在起病0—14d共有85例（37．1％）患者继发癫痫发作。癫痫发作组与无癫痫发作组EEG及VEEG异常程度差异有统计学意义（分别为Hc=22．789,P=0．000和χ^2=5．847,P〈0．05）;2组头颅MRI异常率分别为70．4％和39．0％,其差异有统计学意义（χ^2=18．458,P〈0．005）;2组皮层病灶发生率分别为53．5％和22．0％,差异有统计学意义（χ^2=4．314,P〈0．05）;2组病死率分别为24．7％（21／85）和8．3％（12／144）,自动出院及院内死亡例数差异有统计学意义（χ^2=11．02,P〈0．05）。结论急性期癫痫发作的病毒性脑炎以强直阵挛性发作最常见,EEG及VEEG改变明显,MRI早期病灶及皮层病灶易于继发癫痫发作,癫痫发作患者预后差。     

小儿非癫痫性发作临床分析

目的　对小儿时期的各种非癫痫性发作进行分析。方法　对 3 2例疑似非癫痫性发作的患儿进行临床分析 ,同时进行录像脑电图或 2 4h动态脑电图监测。结果　 3 2例患儿发作期及发作间期脑电图均无异常放电 ,证实为非癫痫性发作 ,以生理性发作最多 (16例 ,占 5 0 % ) ,其平均年龄最小 (3 .5岁 )。结论　儿童时期存在多种形式的非癫痫性发作 ,以生理性发作最多 ,且非癫痫性发作易被误诊为癫痫及其他疾病 ,采用动态脑电图监测对诊断及鉴别诊断均有指导意义     

Safety issues around misuse of antiepileptics

Introduction: Drug misuse is a deliberate or accidental (by omission) nonadherence to medical recommendations, which may range from inappropriate use (missed, increased, or lowered doses or even complete discontinuation of therapy) to compulsive overdosing. Currently, this phenomenon affects as many as 20 – 80% of epileptic patients.

Areas covered: Long-standing research has enabled the identification and understanding of factors behind the phenomenon of nonadherence to medical recommendations. An inappropriate use of antiepileptic drugs usually has serious health implications for both children and adults. These involve increased frequency of seizures in patients who lower their doses or discontinue therapy, which may often lead to pathologies. On the other hand, patients who increase or take extra doses expose themselves to toxic effects of antiepileptic drugs. In both cases, there is an increased need for hospitalization, which further implies extra healthcare costs. The most misused antiepileptic drug is gabapentin (53%), whereas the least misused are lamotrigine, levetiracetam, and phenytoin (all drugs at 32%).

Expert opinion: The prevalence of misuse of antiepileptic drugs among epileptic patients is comparable to that observed in other chronically ill individuals. Preventive strategies have to be based on the reasons leading to nonadherence.     

An evaluation of clobazam tablets and film (AQST-120) for the treatment of Lennox-Gastaut syndrome

ABSTRACT

Introduction: Lennox-Gastaut syndrome (LGS) is a chronic, epileptic encephalopathy, characterized by multiple seizure types, distinctive slow spike-wave patterns in the electroencephalogram (EEG), and severe cognitive and behavioral comorbidities. Seizures are typically refractory and long-term prognosis is poor. No antiseizure drug (ASD) is fully effective as a monotherapy. Clobazam (CLB) was licensed in the United States in 2011 as an adjunctive therapy for seizures in LGS. In 2018, a new formulation, CLB oral soluble film (COSF) (AQST-120), was approved by the Federal Drug Administration (FDA) for the same indication.

Areas covered: The authors summarize current pharmacological options and guidelines for the management of seizures in LGS and efficacy and safety findings from phase II and III randomized controlled trials of adjunctive CLB in patients with LGS. An open-label extension trial is also considered. A pharmacokinetic comparison of COSF and CLB tablets is also undertaken.

Expert opinion: CLB is partly effective as an add-on therapy in treating seizures in LGS. Adverse effects, pharmacokinetic interactions and the potential for tolerance with long-term treatment should be weighed against the clinical benefit when considering the introduction of CLB in this population. COSF has a similar pharmacokinetic profile to CLB tablets and may help to improve adherence to treatment.     

"Epileptic encephalopathy" of infancy and childhood: electro-clinical pictures and recent understandings

There is growing interest in the diagnosis of cognitive impairment among children with epilepsy. It is well known that status of seizures control has to be carefully investigated because it can be sufficient "per se" to cause progressive mental deterioration conditions. Subclinical electroencephalographic discharges may have subtle effects on cognition, learning and sleep patterns, even in the absence of clinical or sub-clinical seizures. In this respect, electroencephalographic monitoring (long-term and nocturnal recording) and in particular an all night video-polysomnography (V-NPSG) record can be crucial to detect the presence of unrecognized seizures and/or an inter-ictal nocturnal EEG discharge increasing. Epileptic encephalopathies (EE) are a group of conditions in which the higher cognitive functions are deteriorate as a consequence of epileptic activity, which, in fact, consists of frequent seizures and/or florid and prolonged interictal paroxysmal discharges, focal or generalized. AEDs represent the first line in opposing the burden of both, the poor seizures control and the poor interictal discharges control, in the cognitive deterioration of EE affected children. Thus, to improve the long-term cognitive/behavioural prognosis in these refractory epileptic children, it should be taken into account both a good seizures control and a strict sleep control, choosing carefully antiepileptic drugs which are able to control not only seizures clinically recognizable but even the EEG discharges onset and its increasing and spreading during sleep. Here, we review the efficacy and safety of the newer AEDs that, to date, are used in the treatment of EE in infancy and childhood.     

Effects of cypermethrin on the electroencephalographic activity of the rat: a model of chemically induced seizures.

Cypermethrin is a potent representative member of the type II pyrethroid insecticides. This pyrethroid is used worldwide and has become a part of our environment. Until the present study, little information about its toxic effects in the central nervous system (CNS) was available. The aim of this study was, then, to determine the effects of repeated exposure to cypermethrin by means of assessing the electroencephalographic (EEG) activity in the rat. Cypermethrin was administered daily in a 300 mg/kg i.p. dose, below the LD50 value. After daily administration, the EEG activity was recorded and evaluated for 30 min. Paroxysmal epileptic activity appeared after the first and second days of cypermethrin administration. Frequency and numbers of bursts of epileptic activity also increased throughout the days of exposure to cypermethrin. Some of the paroxysmal events were present with behavioral anomalies, such as generalized tonic-clonic seizures. These effects are important because they could be related to the incidence of epileptic activity in humans chronically exposed to cypermethrin.     

多发性硬化合并癫痫发作9例临床分析

目的探讨多发性硬化（MS）合并癫痫发作患者的临床特点。方法回顾分析130例临床确诊和实验室确诊为MS患者中9例合并癫痫发作的临床资料。结果MS患者中癫痫发病率为6.9%,明显高于普通人群患病率0.5%～1%。MS患者癫痫发作与皮质-皮质下斑块病灶有关。结论MS患者易发生癫痫。抗癫痫药治疗可以有效控制发作,并减少复发。脑电图检查有助于早期诊断。     

Intravenous levetiracetam in the rat pilocarpine-induced status epilepticus model: Behavioral,physiological and histological studies

PurposeStatus epilepticus is a neurological emergency associated with neuronal injury, lasting behavioral disturbance, and a high rate of mortality. Intravenous levetiracetam (LEV), an anti-epileptic drug approved to treat partial seizures, has recently been introduced. We sought to determine the effect of LEV administered intravenously in a chemoconvulsant model of status epilepticus.MethodsWe examined the effect of intravenous LEV in the rat lithium-pilocarpine model of status epilepticus. Ten or 30 min after the onset of behavioral status epilepticus, animals were treated with LEV (200–1200 mg/kg i.v.) administered in a single bolus. Behavioral responses were recorded. Selected animals had continuous EEG recording before, during and after the administration of LEV. Some animals were sacrificed 24 h after the experiment and processed for histochemical assessment of neuronal injury.ResultsWhen administered 30 min after the onset of behavioral epileptic seizures, transient attenuation of ictal behavior was observed in animals treated with 800 mg/kg or more of LEV. The duration of behavioral attenuation increased sharply as the dose rose to 1000 mg/kg or higher, from a mean of 4–23.6 min. When administered 10 min after seizure onset, 400 mg/kg of LEV resulted in transient ictal behavioral attenuation, and higher doses caused relatively longer periods of attenuation. Pretreatment with LEV prior to pilocarpine also delayed the onset of seizures. EEG recordings, however, showed no significant attenuation of ictal discharge. By contrast, TUNEL staining demonstrated less neuronal injury in hippocampii and other limbic structures in animals that responded behaviorally to LEV.ConclusionsIntravenous administration of LEV in a chemoconvulsant model of status epilepticus results in attenuation of behavioral manifestations of seizure discharge and in reduction of neuronal injury but does not significantly alter ictal discharge recorded by EEG.