部分性发作儿童癫痫的治程探讨

为探讨部分性发作癫痫患儿的合理治程,对1984年～1993年诊治随访的部分性发作癫癎患儿分两组观察。A、组118例,规则治疗至发作控制后3年停药;B组40例,于发作控制后6个月至1年即停药。两组均于停药后继续随访3年。A组复发率为26.3％,B组为85.0％,有非常显著差异(P<0.001);两组复发时间大多均在停药后2年内。EEG癫癎波消失多滞后于临床发作控制。结果提示儿童部分性发作癫癎的治程宜在发作控制后继续维持治疗3年以上。对EEG多次出现局灶性或弥漫性棘—慢综合波的患儿停药需谨慎,停药后继续随访十分重要。     

69例儿童难治性癫痫的临床特点分析

目的：探讨儿童难治性癫痫( refractory epilepsy,RE)的临床特点。方法选取2011年6月至2015年1月在唐山市妇幼保健院小儿神经内科门诊及病房登记的380例癫痫患儿,对其中69例RE患儿进行回顾性分析及前瞻性的随访观察。采用国际抗癫痫联盟指南标准对癫痫及癫痫综合征进行分类。结果平均随访(2.58±1.12)年,69例难治性癫痫患儿中,症状性癫痫52例(75.4％),1岁或1岁之前发病33例(47.8％),频繁发作(发作至少每周1次)50例(72.5％),36例(52.2％)出现多种癫痫发作形式,并发智力障碍者59例(85.5％);影像学异常改变47例(68.1％),脑电图首次异常改变者55例(79.7％);应用3种抗癫痫药物的31例(44.9％),应用4种及以上抗癫痫药物的6例(8.7％)。结论 RE患儿发病年龄早、病初发作频繁、症状性癫痫多、伴发或继发精神运动发育迟缓,影像学及脑电图异常改变多,应用多种抗癫痫药物疗效差。     

7～14岁癫痫患儿睡眠习惯调查及分析

目的探讨7～14岁癫痫儿童的睡眠习惯及相关影响因素。方法 2008年1月至2009年10月华西第二医院小儿神经科确诊的癫痫患儿162例作为癫痫组,通过对儿童父母和监护人的询问,运用专门针对儿童使用的儿童睡眠习惯调查表对癫痫组和162名正常儿童(对照组)的睡眠习惯进行调查分析。结果癫痫组睡眠习惯评分为(46.1±7.5)分,而正常对照组为(32.4±3.6)分,两者间差异有统计学意义(P0.05)。癫痫组运动和认知发育正常者评分为(43.5±8.1)分,落后者评分为(49.9±7.3)分,两者间差异有统计学意义(P0.05)。单药治疗患儿71例,评分为(42.9±6.3)分;2种或2种以上药物治疗患儿22例,评分为(54.3±5.7)分,两者间差异有统计学意义(P0.01)。另外,夜间睡眠发作的癫痫患儿(36例)相对白天清醒时发作患儿(50例)、清醒及睡眠中均有发作患儿(76例)睡眠习惯较差,评分分别为(52.3±5.7)、(44.1±7.6)、(44.5±6.8)分,差异有统计学意义(P0.05);部分性发作的癫痫患儿(46例)相对全身性发作(57例)、部分性泛化全身(59例)对睡眠习惯的影响较小,评分分别为(41.6±4.9)、(47.4±8.7)、(48.4±7.5)分,差异有统计学意义(P0.05)。结论癫痫易出现睡眠障碍,多种因素包括运动和认知发育落后、发作控制及治疗情况、发作类型等可影响患儿睡眠习惯。     

4例以癫痫性负性肌阵挛为首发或唯一发作类型的儿童癫痫临床特征

目的探讨以癫痫性负性肌阵挛(ENM)为首发或唯一发作类型的儿童癫痫的电生理和临床特征。方法回顾分析4例以ENM为首发或唯一发作类型的癫痫患儿的脑电图和临床资料。结果 4例患儿中,男3例、女1例;起病年龄(1.85±0.61)岁(1岁5个月～2岁9个月);病初智力发育正常(发育商均90);头颅影像学无异常,血、尿遗传病筛查无异常。4例患儿均以ENM为首次发作类型,其中3例以ENM为唯一发作类型,1例ENM之后出现局灶性发作。首次视频脑电图(VEEG)背景节律无异常,发作期与发作间期均为中央-颞区(Rolandic区)棘慢波。2例患儿丙戊酸治疗3个月内发作控制(随访至今无发作)。2例治疗曲折但目前暂无发作,其中1例病程中卡马西平(CBZ)治疗加重ENM,停CBZ加氯硝西泮有效;1例治疗5个月后出现睡眠期癫痫电持续状态,甲基泼尼松龙冲击治疗有效。3例患儿末次VEEG仍存在数量不等的放电,存在不同程度的学习困难、行为障碍或多动;1例失访。结论以ENM为首发或唯一发作类型的儿童癫痫具有特定的脑电模式和相似的临床特征。     

以健康测量量表评价癫及哮喘患儿生活质量的对照研究

摘要 目的 以健康测量量表（SF 36）评估癫痫及哮喘患儿的生活质量。方法 采用SF-36量表对2007年6至12月在复旦大学附属儿科医院神经专科、呼吸专科及儿童保健门诊就诊的癫痫患儿、哮喘患儿和健康儿童进行生活质量评定。结果 研究期间85例癫痫患儿（癫痫组）、81例哮喘患儿（哮喘组）和87名健康儿童（正常对照组）进入分析。3组儿童的年龄、性别和受教育程度相匹配。①疾病及治疗情况：癫痫组和哮喘组服用1种药物者分别为70/85例和59/81例,癫痫组63/85例和哮喘组56/81例患病后规则服药,两组在服药种类和依从性上差异无统计学意义（P均＞0.05）;癫痫组36/85例至本次调查前至少有1年癫痫未发作;哮喘组29/81例最大呼气峰流速监测持续保持个人最佳值的80％以上。②生活质量评价：癫痫组的生活质量总分和8个分项的终得分与正常对照组差异均有统计学意义（P<0.01）;哮喘组的生活质量总分及生理功能、身体疼痛、总体健康和活力4个分项的终得分与正常对照组差异均有统计学意义（P<0.01）;无论疾病控制与否,癫痫组生活质量均较哮喘组差;癫痫组情感功能及精神健康状况明显差于哮喘组,其心理障碍不随疾病控制而明显改善。结论 ①癫痫和哮喘患儿生活质量较健康儿童明显下降;②癫痫患儿较哮喘患儿生活质量损害更为明显;③SF-36量表可作为评价疾病控制程度的手段,辅助评价疾病治疗的疗效;④癫痫 患儿的心理障碍应引起临床高度关注。     

体外反搏治疗儿童难治性癫痫的疗效观察

癫痫是一种慢性发作性疾病，儿童患病率为4‰-6％0，通过科学合理地应用抗癫痫药物，70％-80％患儿的发作可得到控制。但迄今仍有20％的患儿尽管正规使用多种抗癫痫药物2-3年仍不能控制发作而成为难治性癫痫。治疗困难性癫痫长期频繁的发作将严重影响患儿的智力发育和认知功能．[第一段]     

30例癫癎儿童个性及认知功能观察

癫痫病是儿童时期最常见的慢性神经系统疾患，发病率高达5％，需长期甚至终生服用抗癫痫药。临床医师往往注重临床发作是否得到控制，而对癫痫儿童的心理行为状况如何很少关注。我们对30例癫痫儿童进行评估，观察癫痫儿童的个性及认知功能的变化，并寻找干预的措施，现将结果报告如下。     

麻醉剂在难治性癫痫持续状态的应用

难治性癫痫持续状态是指尽管用了足量的一线及二线抗癫痫药物仍然不能控制发作的情况,是儿童神经系统急重症,有较高的病死率。多数意见推荐静脉注射麻醉药物治疗难治性癫痫持续状态,以达到全面控制发作,治疗性昏迷及脑电爆发抑制。然而,对于最佳麻醉药物及麻醉深度并无一致意见,持续静脉麻醉诱导昏迷所带来的风险收益比也不十分清楚,麻醉剂治疗难治性癫痫持续状态仍需谨慎。本文介绍了治疗儿童难治性癫痫持续状态常用的静脉麻醉药物,也探讨了临床应用麻醉剂的合理性及争议。     

神经系统及精神疾病

921306 34例儿童复杂性高热惊厥与癫痫的高危因素探讨/刘超∥四川医学.-1991,12(6).-362～363 随访34例复杂性高热惊厥2～9年,分析其发生癫痫的危险因素与性别,惊厥发作前后表现,发作类型,伴随疾病,家族史,围产期因素,既往史均无明显关系。本组9例经临床和脑电图证实转为癫痫(癫痫组);25例     

盐酸哌甲酯治疗儿童良性癫痫伴中央颞区棘波合并注意缺陷多动障碍的安全性研究

目的探讨盐酸哌甲酯治疗儿童良性癫痫伴中央颞区棘波(BECTS)合并注意缺陷多动障碍(ADHD)的安全性及对癫痫发作的影响。方法选取2007年4月至2008年10月广州市儿童医院神经内科确诊的40例BECTS合并ADHD患儿,经抗癫痫药物治疗临床无发作达6个月以上,加用盐酸哌甲酯,每6个月复查1次脑电图(EEG),评价患儿注意力和行为改善情况,记录癫痫发作次数、类型以及其他副反应,将患儿用药前后的癫痫发作情况进行自身对照研究。结果 40例中37例用药时间超过6个月,注意力、多动及对立违拗行为明显改善。4例发生副反应;2例用药1周内发作增多;1例用药1年后出现1次发作;1例用药2d内出现入睡困难,停药后发作及入睡困难停止。患儿服用盐酸哌甲酯前后癫痫发作及其他副反应出现情况差异无统计学意义(P0.05)。结论抗癫痫药控制临床发作的同时,在规范剂量范围内使用盐酸哌甲酯治疗BECTS合并ADHD具有安全性。     

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??Objective To investigate the expression of multidrug resistant-associated protein 1 in the peripheral blood of children with refracotory epilepsy. Methods The expression of MRP1 was detected by RT-PCR and Western blot in children with intractable epilepsy??epilepsy controlled by anti-epileptic drugs ??AEDs?? and healthy children.Results The two methods showed that MRP1 expression in intractable epilepsy was higher than that in epilepsy controlled by anti-epileptic drugs ??AEDs?? and healthy children??P??0.05???? and there was no difference between epilepsy controlled by anti-epileptic drugs ??AEDs?? and healthy children.Conclusion MRP1 overexpresses in peripheral blood of children with refractory epilepsy.     

Factors influencing the academic performance of school children with epilepsy

The academic performance and intelligence quotient (IQ) of 50 children with epilepsy aged between 5 and 14 years, attending normal primary schools in Enugu, were compared with those of their non-epileptic classmates. The academic performance was assessed using the overall scores achieved in terminal examinations in the 2001-2002 academic year. IQ was assessed using the Draw-A-person Test. The influences of school absence rate, Rutter behavioural scores, socio-economic status and seizure-related variables on academic performance were then determined. Twenty-six percent of the children with epilepsy had a low overall score, and therefore poor academic performance, compared with 16% of the controls (p = 0.35). The mean IQ of the children with epilepsy was significantly lower than that of the controls (p = 0.02). The mean school absence rate for the children with epilepsy was significantly higher than that of the controls (p = 0.001). The mean Rutter score of the children with epilepsy was significantly higher than that for the controls (p < 0.001). On multiple linear regression analysis, only IQ (p = 0.01) and seizure type (p = 0.03) had significant predictive effects as risk factors for low overall scores and poor academic performance. It is concluded that the academic performance of epileptic children is influenced by their IQ and type of seizures rather than by other seizure variables or socio-demographic characteristics.     

Clinical experiences with topiramate in children with intractable epilepsy.

At a tertial referral epilepsy centre 39 children were consecutively enrolled in an open add-on study with topiramate (TPM). All children had intractable epilepsy; the mean seizure frequency was 36 per month, and 31 children were treated with polypharmacy. All but five children were mentally retarded. The initial dose of TPM was 0.5-1 mg/kg daily, slowly titrated with 1-3 mg/kg daily every second week with an estimated target dose of 10 mg/kg daily. At latest follow-up 19 children continued on TPM, three (8%) were seizure-free, eight (21%) had a seizure reduction of more than 50% and eight (21%) improved their general condition. Mean follow-up was 13 months (range 9-36 months). Seizure reduction was seen in focal as well as generalized epilepsies. Adverse effects were reported in 21 cases (54%), weight loss and sedation being most frequent. The mean steady state dose in the children continuing on TPM was at latest follow-up: 14 mg/kg daily (< 5 years), 10 mg/kg daily (5-7 years), 5.8 mg/kg daily (8-17 years). The corresponding plasma level varied from 3 to 45 mumol/litre, and a significant correlation between the daily dose in mg/kg and the plasma level was found. Two patients with progressive myoclonus epilepsy are described separately; one had a dramatic general improvement. It is concluded that TPM seems to be a promising new broad-spectrum anti-epileptic drug, which is efficacious even in epilepsy syndromes, intractable to other new anti-epileptic drugs such as vigabatrin and lamotrigine.     

癫(癎)患儿预后与相关因素的远期随访研究

目的 分析影响癫(癎)患儿预后的相关因素,为癫(癎)的诊治和预后评估提供依据.方法 回顾性分析2003年1-12月在北京大学第一医院儿科门诊就诊的290例癫(癎)患儿的临床资料,随访患儿发作、用药、智力及精神运动发育等情况,以至少1年无发作为疗效控制指标.结果 ①经正规抗癫(癎)药物治疗,57.9%患儿发作控制满意,多数可以正常学习或生活;(②各型癫(癎)均有控制发作的可能,不同发作类型癫(癎)的控制率不同;③起病年龄越早,特别是1岁内起病者发作控制较差;④原发性癫(癎)控制率明显高于症状性或隐源性癫(癎),症状性癫(癎)预后最差;⑤大部份病例经单药治疗可以控制发作,2种药物治疗未控制者,再添加药物进行治疗,控制率无明显提高.结论 儿童癫痫的预后大多良好,有下列情况者预后差:①起病年龄小,尤其是1岁者;②同时有多种发作形式;③症状性癫(癎).     

Epilepsy in children with cerebral palsy

This article deals with the clinical profile of children with cerebral palsy and epilepsy, and to study the clinical predictors of response to anti-epileptic drugs. It is a prospective hospital based follow-up study. All the children who presented with cerebral palsy and history of seizure (other than neonatal seizures) over a period of one year were included. Seizures were classified according to ILAE classification. An EEG was obtained in all cases. Neuroimaging was done in all patients. Eighty-five patients were studied and followed for minimum of 12 months. Perinatal factors accounted for 62 (72.3%) cases. The motor deficits seen were quadriparesis (n = 64), hemiplegia (n = 12) and diplegia (n = 9). Associated mental retardation was seen in 80.9% patients with quadriparesis. A predominance of generalised epilepsy was seen with generalised tonic clonic seizures (32.9%) followed by mycolonic seizures (30.6%) and localisation related epilepsy (24.7%). The patients with quadriparesis were more likely to have generalised epilepsy and 52.4% of them required two or more anti-epileptic drugs for control of seizures. Patients with hemiplegia had localisation related epilepsy in 83.3% of cases. On multivariate analysis presence of quadriparesis, microcephaly, mental retardation and myoclonic epilepsy were found to predict the poor response to AED. Epilepsy in patients with cerebral palsy is of severe nature and difficult to control. Presence of quadriparesis, mental retardation and myoclonic seizures was predictive of poor response to anti- epileptic drugs.     

Increased urinary excretion of tubular enzymes and proteins in children with epilepsy

Cross-sectional analysis of children undergoing treatment with anti-epileptic drugs has shown an increased urinary excretion of tubular enzymes and proteins. This has usually been interpreted as a consequence of subclinical renal-tubular damage or enzyme induction. We measured excretion of tubular enzymes and proteins in 29 children who suffered from epileptic seizures and in 27 control children. Investigations were undertaken at diagnosis before the start of treatment and 3-4 months later. At diagnosis we found a slightly, but statistically significant increased excretion of N-acetyl-beta-glucosaminidase and alpha1-microglobulin. There was no significant difference between patients with an idiopathic and symptomatic aetiology of seizures or between patients with different seizure types. At the second investigation, in children treated with carbamazepine or valproate, no further increase occurred. We conclude that the increased excretion of tubular enzymes and proteins in children with epilepsy is most probably not due to a side-effect of the anti-epileptic drugs, but to a physiological alteration associated with the epilepsy itself. While the cause is unknown, the influence of serotonin metabolism is discussed.     

肌阵挛失张力癫(癎)的临床和脑电图特点

目的 总结肌阵挛失张力癫(癎)(MAE)的临床和脑电图特点.方法 分析MAE患儿的临床和视频脑电图及同步肌电图资料,并对治疗效果进行随访.结果 收集MAE患儿共47例,其中25例有热性惊厥史,20例有热性惊厥和(或)癫(癎)家族史,发病前智力运动发育均正常.起病年龄1.4～5.8岁,首次发作为无热的全面强直-阵挛发作(GTCS)41例(87.2%),肌阵挛发作4例,失张力发作2例.病程中均有多种发作类型,包括GTCS46例(97.9%)、肌阵挛失张力发作34例(72.3%)、肌阵挛发作47例(100%)、失张力发作32例(68.1%)、不典型失神36例(76.6%)和强直发作3例(6.4%).出现多种类型的发作时脑电图背景均为弥漫性慢波或顶区为主的θ节律,发作间期呈全导1～4 Hz(以2～3 Hz为主)棘慢波、多棘慢波发放.所有患儿均首选抗癫(癎)药物(AEDs)治疗,41例(87.2%)应用AEDs发作控制,其中37例单用或合用丙戊酸,26例联合应用拉莫三嗪.10例(21.3%)发病后出现智力落后.结论 MAE的临床特点包括:发病前发育正常,多数以GTCS起病,病程中具有多种全面性癫(癎)发作类型,肌阵挛失张力发作是其特征性发作类型;脑电图呈全导棘慢波、多棘慢波发放.早期明确诊断并合理选择AEDs是取得良好预后的关键.

Abstract：

Objective To summarize the electroclinical characteristics of myoclonic atonic epilepsy (MAE) in children. Method The clinical data, video electroencephalogram (EEG) and simultaneous electromyography (EMG) of MAE patients were analyzed. The treatment and its effects were followed up.Result In 47 MAE patients, 25 had a history of febrile seizures ( FS), 20 had a family history of FS or epilepsy. All patients had a normal development before the illness. The age of afebrile seizure onset was between 1.4 years to 5.8 years. The first seizure was generalized tonic-clonic seizure (GTCS) in 41 patients (87.2%). All patients had multiple seizure types, including 47 GTCS (97.9%), 34 myoclonic atonic seizures (72. 3% ), 47 myoclonic seizures ( 100% ), 32 atonic seizures (68. 1% ), 36 atypical absences (76. 6% ) and 3 tonic seizures (6. 4% ). EEG backgrounds were slow or parietal θ rhythm, interictal EEG showed 1-4 Hz (predominant 2-3 Hz) generalized spike and wave or poly spike and wave discharges in all cases. Seizures were controlled by antiepileptic drugs (AEDs) in 41 patients (87.2%). Valproate was used in 37. Lamotrigine was used in 26. Mild mental retardation was observed in 10 children after the onset of the illness. Conclusion The clinical features of MAE included the following: the development was normal before the onset of the illness; the onset of seizure type was often GTCS. All patients had multiple generalized seizure types. Myoclonic atonic seizure was its characteristic seizure type. EEG showed generalized discharges. Early diagnosis and rational choice of AEDs are important for getting a better prognosis.     

卡马西平和丙戊酸钠对癫癎患儿骨代谢的影响

目的探讨长期服用卡马西平（CBZ）或丙戊酸钠（VPA）对癫痫患儿骨代谢的影响。方法对92名单独服用CBZ或VPA2年以上的癫痫患儿及35名年龄匹配的健康儿童的骨密度进行测量,测定骨代谢特异性生化指标,血清骨钙素（OC,骨形成指标）和尿脱氧吡啶酚（DPD,骨吸收指标）,并调查每日钙摄入量。结果癫痫患儿骨密度明显低于对照组（P〈0．05）,服用抗癫痫药物（AEDs）的时间与骨密度呈负相关（rs=-0．21～-0．31,P〈0．05）。癫痫患儿中低骨密度患儿32人,占35％,明显高于对照组14％（P〈0．05）。低骨密度的癫痫患儿多体重指数较高（P〈0．05）,每日钙摄入量较少（P〈0．01）,服用AEDs的时间相对较长（P〈0．01）。癫痫患儿血清OC浓度明显低于对照组（P〈0．01）;而尿DPD浓度与对照组基本一致（P〉0．05）。结论长期服用CBZ或VPA会导致骨代谢异常,骨密度下降、骨转换降低（以骨形成减少为主）;而钙摄入量不足及体重指数偏高是加剧骨骼异常的诱发因素。     

Treatment of newly diagnosed pediatric epilepsy: a community-based study

OBJECTIVE: To determine the patterns and frequency of treatment and use of specific drugs for newly diagnosed pediatric epilepsy. DESIGN AND SETTING: Prospective, community-based study. Children were recruited from physicians in Connecticut from 1993 to 1997. PATIENTS: Children aged 1 month through 15 years at the time of their first seizure, who had 2 or more unprovoked seizures, and who were newly diangosed during the recruitment period were eligible. MAIN OUTCOME MEASURE: Initiation of treatment at diagnosis and within 1 year after diagnosis of epilepsy. RESULTS: Of 613 children, 482 (78.6%) were treated at the time of initial diagnosis. By 6 months another 10.3% were treated, and by 12 months 90% of the cohort had been treated. The most commonly prescribed antiepileptic drug (AED) was carbamazepine (38.8%) followed by sodium valproate (18.4%). Only 1 child received an investigational drug and none received any of the most recently approved drugs as a first AED. Children with idiopathic and secondarily generalized forms of epilepsy were most likely to be treated (90%-100%), whereas children with idiopathic localization-related epilepsy were least likely to be treated (50.8%). Approximately 80% of those with other forms of epilepsy were treated at the time of diagnosis. Use of specific medications reflected current guidelines and recommendations for treatment of specific seizure types and syndromes. CONCLUSIONS: In Connecticut, approximately 20% of children with epilepsy are not treated at the time of initial diagnosis, and around 10% continue to be untreated after 1 year. This most likely reflects the increased understanding of the nature of pediatric epilepsy and concerns regarding the adverse effects of AEDs. The most commonly used first drugs are carbamazepine and valproate. Follow-up of this cohort may help provide information to guide the use of recently approved AEDs.     

Neurocysticercosis in children: clinical characteristics and outcome

A study was undertaken on 176 children with neurocysticercosis to determine the clinical behaviour and long-term outcome of cases. The children were followed up prospectively in the paediatric neurology clinic for 6 years. Diagnosis was based primarily on CT scans. All the CT lesions were parenchymal and mostly single (87%) with ring enhancement and peri-lesional oedema. Partial seizure was the predominant presenting seizure type. About 65% of cases had recurrences at varying intervals during treatment. Albendazole therapy given to some cases did not appear to have any beneficial effect on seizure control. Repeat CTs done at varying intervals after the first seizures showed disappearance of the lesions in about 50% of cases. The majority of lesions disappeared without the use of albendazole. Recurrence of seizures after withdrawal of anti-epileptic medication was observed in 19% of the cases. Parenchymal neurocysticercosis in children commonly presents with partial seizures and ring lesions on CT. The disease can be managed well by anti-epileptic medication alone and the prognosis is good.