儿童失神癫240例治疗及预后随访报告:应用改良的1989年儿童失神癫诊断标准

目的总结儿童失神癫(CAE)的治疗和预后,为CAE的合理用药和评价远期预后提供依据。方法对1999年10月至2005年12月北京市3家医院为研究CAE易感基因收集的CAE患儿的治疗用药、疗效及预后进行随访。CAE诊断标准参考1989年国际抗癫联盟(ILAE)提出的癫及癫综合征分类诊断标准,并制定了统一的CAE纳入标准和排除标准。根据CAE的选药原则进行治疗,评估CAE患儿的远期预后。结果3家医院共收集符合ILAECAE诊断标准的患儿339例,其中296例符合本研究制定的CAE纳入标准。296例患儿中有56例患儿因失访未得到远期预后随访结果,有随访结果者240例(81.1%),其中男94例(39.2%),女146例(60.8%)。失神发作起病年龄为3岁3个月至12岁,其中4～8岁174例(72.5%)。39例(16.2%)有热性惊厥史,18例(7.5%)有热性惊厥家族史和(或)癫家族史,5例(2.1%)有失神癫家族史。失神癫发作频率为每日5～50次,其中每日发作10～30次占80%。出现失神持续状态1例。伴全面强直-阵挛发作12例(5.0%)。所有患儿发作期EEG均表现为双侧对称同步的3Hz棘慢波爆发,头颅影像学检查均未见异常。治疗首选丙戊酸234例,其中217例(92.7%)于服药后3d至6个月发作完全控制,15例加用另一种药物(其中氯硝西泮7例、硝西泮4例及拉莫三嗪4例)后发作控制,余2例单用丙戊酸2年后仍有发作,但发作次数明显减少;首选拉莫三嗪4例,其中3例发作控制,1例服药1年发作未控制,改用丙戊酸1周后发作控制。2例首选托吡酯治疗,其中1例发作控制,1例服药5个月效果不明显,改用丙戊酸2个月后发作控制。240例患儿随访时间为2～7年,158例(65.8%)已停用抗癫药物,其中停药1年以上者96例,停药后均无复发;82例尚未停用抗癫药物患儿中,80例发作完全控制2年以上,仅有2例仍有失神发作。随访的240例患儿在校学习成绩为中等及以上者有171例(71.3%)。结论丙戊酸是治疗CAE的首选药物,对绝大多数患儿疗效好。少数用丙戊酸发作未控制者可选用拉莫三嗪或苯二氮类药物。典型CAE患儿远期预后良好。     

儿童失神癫240例治疗及预后随访报告：应用改良的1989年儿童失神癫诊断标准

目的总结儿童失神癫（CAE）的治疗和预后，为CAE的合理用药和评价远期预后提供依据。方法对1999年10月至2005年12月北京市3家医院为研究CAE易感基因收集的CAE患儿的治疗用药、疗效及预后进行随访。CAE诊断标准参考1989年国际抗癫联盟（ILAE）提出的癫及癫综合征分类诊断标准，并制定了统一的CAE纳入标准和排除标准。根据CAE的选药原则进行治疗，评估CAE患儿的远期预后。结果3家医院共收集符合ILAE CAE诊断标准的患儿339例，其中296例符合本研究制定的CAE纳入标准。296例患儿中有56例患儿因失访未得到远期预后随访结果，有随访结果者240例（81.1%），其中男94例（39.2%），女146例（60.8%）。失神发作起病年龄为3岁3个月至12岁，其中4~8岁174例（72.5%）。39例（16.2%）有热性惊厥史，18例（7.5%）有热性惊厥家族史和（或）癫家族史，5例（2.1％）有失神癫家族史。失神癫发作频率为每日5~50次，其中每日发作10~30次占80%。出现失神持续状态1例。伴全面强直12例（5.0%）。所有患儿发作期EEG均表现为双侧对称同步的3 Hz棘慢波爆发，头颅影像学检查均未见异常。治疗首选丙戊酸234例，其中217例（92.7%）于服药后3 d至6 个月发作完全控制，15例加用另一种药物（其中氯硝西泮7例、硝西泮4例及拉莫三嗪4例）后发作控制，余2例单用丙戊酸2年后仍有发作，但发作次数明显减少；首选拉莫三嗪4例，其中3例发作控制，1例服药1年发作未控制，改用丙戊酸1周后发作控制。2例首选托吡酯治疗，其中1例发作控制，1例服药5个月效果不明显，改用丙戊酸2个月后发作控制。240例患儿随访时间为2～7年，158例（65.8%）已停用抗癫药物，其中停药1年以上者96例，停药后均无复发；82例尚未停用抗癫药物患儿中，80例发作完全控制2年以上，仅有2例仍有失神发作。随访的240例患儿在校学习成绩为中等及以上者有171例（71.3％）。结论丙戊酸是治疗CAE的首选药物，对绝大多数患儿疗效好。少数用丙戊酸发作未控制者可选用拉莫三嗪或苯二氮类药物。典型CAE患儿远期预后良好。     

小儿失神癫痫临床特征和药物治疗观察

目的探讨小儿失神癫痫(CAE)的临床特征及评价药物的疗效。方法对34例CAE的临床及实验室资料进行回顾性分析。结果本组起病年龄是4～11岁,4～8岁患病为多(65％);简单失神14例(41％),复杂失神20例(59％)。对25例患儿取立位进行过度换气诱发实验,目测结果15例复杂失神可伴有不同临床表现,7例为简单失神。34例异常脑电图(EEG)为突然自发或过度换气后呈现双侧对称同步的3Hz棘慢综合波。32例中28例(88％)智商(IQ)正常,4例为边缘状态。本组患儿脑CT、MRI均正常。34例均首选单药治疗26例用丙戊酸钠,5例用德巴金缓释片,用药至发作停止时间是4天至6个月,其中＜1个月者18例(58％);另3例单药治疗期间因故改用多药治疗。随访6个月至4年,单药治疗31例中25例(80％)完全控制发作平均2年。结论CAE发作分型的诊断,是依据临床表现及EEG特征。丙戊酸钠是治疗CAE反应良好的一线药物。     

不典型儿童良性部分性癫(癎)患儿癫(癎)性负性肌阵挛的临床和神经电生理研究

目的 探讨不典型儿童良性部分性癫(癎)(ABPE)患儿癫(癎)性负性肌阵挛(ENM)的临床和神经电生理特点及对治疗的反应.方法 对1998年1月至2007年9月在北京大学第一医院儿科就诊的17例伴ENM的ABPE患儿在视频脑电图(VEEC)监测下行直立伸臂试验,9例同时监测双侧三角肌表面肌电图(EMG).对ENM的临床、神经电生理特征及对抗癫(癎)药物(AED)的治疗反应进行分析.结果 本组起病早期均符合儿童良性癫(癎)伴中央颞区棘波(BECT)的诊断标准,并已接受AED治疗.病程中出现手抖、掉物、点头、站立不稳等症状提示可能存在ENM.所有患儿均在VEEG监测时行直立伸臂试验并观察到ENM发作,与EEG对侧Rolandic区高波幅棘慢波发放同步出现.9例同时进行EMG记录,证实EEG的棘波与EMG的短暂肌电静息具有锁时关系.在出现ENM期间,原有的部分运动性发作频率及发作间期EEG放电明显增多,6例亦有不典型失神发作.部分患儿ENM的出现可能与加用卡马西平、奥卡西平、苯巴比妥等药物或突然减停丙戊酸有关.丙戊酸、苯二氮革类药物及肾上腺皮质激素以不同方式联合治疗可消除多数患儿的ENM发作.结论 ABPE病程中可出现ENM.VEEG监测时行直立伸臂试验及同步EMG记录有助于确诊ENM.ENM的出现常伴有原有惊厥发作频率的增多及发作间期EEG的恶化.卡马西平等AED可能诱发ENM.丙戊酸、苯二氮革类药物及肾上腺皮质激素对消除ENM发作有效. NM期间,原有的部分运动性发作频率及发作间期EEG放电明显增多,6例亦有不典型失神发作.部分患儿ENM的出现可能与加用卡马西平、奥卡西平、苯巴比妥等药物或突然减停丙戊酸有关.丙戊酸、苯二氮革类药物及肾上腺皮质激素以不同方式联合治疗可消除多数患儿的ENM发作.结论 ABPE病程中可 现ENM.VEEG监测时行直立伸臂试验及同步EMG记录有助于确诊ENM.ENM的出现常伴有原有惊厥发作频率的增多及发作间期EEG的恶化.卡马西平等AED可能诱发ENM.丙戊酸、苯二氮革类药物及肾上腺皮质激素对消除ENM发作有效. NM期间,原有的部分运动性发作频率及发作间期EEG放电明显增多,6例亦有不典型失神发作.部分患儿ENM的出现可能与加用卡马西平、奥卡西平、苯巴比妥等药物或突然减停丙戊酸有关.丙戊酸、苯二氮革类药物及肾上腺皮质激素以不同方式联合治疗可消除多数患儿的ENM发作.结论 ABPE病程中可 现ENM.VEEG监测时行直立伸臂试验及同步EMG记录有助于确诊ENM.ENM的出现常伴有原有惊厥发作频率的增多及发作间期     

儿童失神癫GABRG2基因突变筛查

目的对有热性惊厥(FS)和(或)癫家族史的儿童失神癫(CAE)患儿进行γ氨基丁酸A型(GABAA)受体γ2亚单位基因(GABRG2)进行突变筛查,探讨GABRG2基因是否是CAE的易感基因。方法收集30例有FS和(或)癫家族史的CAE患儿(男17例,女13例;发病年龄3岁～10岁6个月)的家系资料,采集患儿及患儿父母的外周血并抽提DNA,采用PCR和DNA直接测序法对其GABRG2基因进行突变筛查。结果30例有FS和(或)癫家族史的患儿家系中,共有71例受累者。其中仅表现为FS16例,CAE30例(其中6例有FS病史,12例有FS家族史,15例有癫家族史,3例同时有FS和癫家族史),失神发作7例(其中3例失神发作前有FS病史),分类不明的癫18例。30例CAE患儿均未发现GABRG2基因突变,但发现4个单核苷酸多态性(SNP),其中315T>C、208T>C和588C>T为已知的SNP,IVS+8C>A为新发现的SNP。结论有FS和(或)癫家族史的CAE患儿,GABRG2基因不是其主要易感基因。     

儿童失神癫癎的单体型相对风险和传递不平衡分析

目的　确认中国儿童失神癫 (childhoodabsenceepilepsy ,CAE)是否与染色体 8q2 4连锁。方法　选取染色体 8q2 4上 5个微卫星DNA标记 (D8S554、D8S53 4、D8S110 0、D8S1783、D8S1753 ) ,对3 0个CAE核心家系的患儿及其父母的单体型进行分析 ,另有 10个正常家系作为对照。从外周血白细胞中常规抽提基因组DNA ,应用PCR方法扩增各个微卫星片断 ,PCR产物用基因测序确定各个等位基因的长度。统计学方法选用基于单体型的单体型相对风险 (HHRR)和传递不平衡检验 (TDT)分析。结果　经HHRR分析显示 ,D8S5544χ2 =5 93 9(P <0 0 5)、D8S110 0 3 χ2 =5 0 81(P <0 0 5)、D8S1783 6χ2 =4 3 0 8,(P <0 0 5) ,差异有显著性 ;TDT显示D8S5544χ2 =4 46(P <0 0 5)、D8S1783 6 χ2 =4(P <0 0 5) ,差异有显著性。为了排除HHRR和TDT分析可能存在的假关联 ,我们对所有患儿家系作了详细的分析 ,结果发现只有在位点D8S1783提示与CAE致病基因存在传递不平衡 ,而另二个位点均为假关联。结论　①中国儿童失神癫可能与染色体 8q2 4连锁 ,与位点D8S1783存在传递不平衡 ;结合国外的结论 ,CAE致病基因可能存在于染色体 8q2 4上的ECA1区域内。②CAE基因在不同地区、不同种族的人群中可能存在遗传异质性。③对于核心家系 ,HHRR和     

Memory function in childhood epilepsy syndromes

OBJECTIVE: Children with epilepsy are at risk of specific cognitive deficits. We aimed to compare and characterize the memory function of children with childhood absence epilepsy (CAE), frontal lobe epilepsy (FLE) and temporal lobe epilepsy (TLE). METHODS: Epilepsy syndrome was identified by clinical data, seizure semiology, interictal and ictal electroencephalogram (EEG). Seventy children aged 6-18 years with CAE, FLE or TLE had neuropsychological assessment including memory function. After adjusting for epilepsy variables, neuropsychological results of the syndrome groups and normative data were compared. RESULTS: Children from all three syndrome groups were at risk of memory difficulties. The duration of epilepsy correlated negatively with memory function. Children with TLE had the worst memory function, significantly lower in verbal memory tasks than children with CAE (P = 0.02) and children with FLE (P = 0.01). The performance of children with TLE was significantly below the normed mean across all verbal and most visual tasks. Compared to the normed means, children with FLE had results that were statistically lower in some verbal and visual tasks, and children with CAE were lower in two visual tasks only. CONCLUSIONS: This study demonstrates memory dysfunction in three common childhood epilepsy syndromes. Children with TLE had the greatest impairment, children with FLE had memory difficulties not previously reported, and children with CAE had subtle memory deficits. Qualitative differences were also evident. Longer duration of intractable epilepsy was associated with reduced memory ability. Memory function and its potential impact on academic achievement are vital considerations when managing children with epilepsy.     

Paroxysmal tonic upgaze of childhood: effect of age-of-onset on prognosis

Paroxysmal tonic upgaze (PTU) is a syndrome of childhood manifesting as sudden ocular movements with sustained upward deviation of the eyes. We describe the outcome of 6 patients, after a follow-up of 10 years, with onset of the disease in childhood. The aims of this study were to clarify some clinical features of this syndrome and to evaluate the long-term prognosis of these children. In all the patients, tonic upgaze episodes disappeared with time to remission, varying from 1 to 4 y, without any therapy and without any change in psychomotor development, EEG and neuroimaging. Only one child had pathologic interictal EEG with temporo-occipital spikes, which persisted after the offset of the disease. CONCLUSION: From a long-term follow-up, we can confirm the good prognosis of PTU and suggest it is possible to define a distinct syndrome of childhood without any neurological abnormalities and with spontaneous resolution.     

The health-related quality of life of childhood epilepsy syndromes

Objective:    There is increasing awareness of the importance of assessing physical, psychological, social and behavioural well-being in chronic disease. The aim of this study was to examine the health-related quality of life (HRQoL) of children with common epilepsy syndromes and to explore if there are HRQoL differences between those syndromes.
Methods:    Each child had their epilepsy syndrome defined according to the International League Against Epilepsy classification. Epilepsy syndromes included symptomatic frontal, temporal, parietal/occipital lobe and partial unlocalized epilepsy, and two idiopathic epilepsies, childhood absence epilepsy (CAE) and benign rolandic epilepsy (BRE). Seizure semiology and ictal/interictal electroencephalogram (EEG) were determined for symptomatic partial epilepsy syndromes by video-EEG monitoring. HRQoL was evaluated with an epilepsy-specific instrument, the Quality of Life in Childhood Epilepsy Questionnaire, and two generic instruments, the Child Health Questionnaire and Child Behavior Checklist.
Results:    Children with symptomatic partial epilepsy syndromes were affected by epilepsy in a similar way and did not have unique HRQoL profiles. However, these children had significantly lower HRQoL scores compared to those with CAE or BRE. All children with epilepsy regardless of syndrome had a higher frequency of behavioural problems compared to normative data.
Conclusion:    These results indicate that children with epilepsy regardless of syndrome require evaluation of the psychosocial implications. There is a greater impact on HRQoL in symptomatic epilepsy compared to idiopathic epilepsy. Specific symptomatic partial syndromes did not differ in the degree they affect HRQoL. These findings have important implications for clinicians caring for children with epilepsy.     

Childhood absence epilepsy and benign epilepsy with centro-temporal spikes: a narrative review analysis

Background:Recent studies have shown a possible coexistence of absence seizures with other forms of epilepsy.The purpose of this study was to ascertain the possible contemporary or subsequent presence of childhood absence epilepsy (CAE) and benign epilepsy with centro-temporal spikes (BECTS) in pediatric epileptic patients.Data sources:A PubMed systematic search indexed for MEDLINE,PubMed and EMBASE was undertaken to identify studies in children including articles written between 1996 and 2015.Retrospective studies,meta-analysis and case reports were included.The list of references of all the relevant articles was also studied.The date of our last search was December 2015.Results:Review of the literature revealed 19 cases,8 females and 11 males,reporting a consecutive or contemporary coexistence of CAE and BECTS within the same patients.Patient's age ranged between 4 and 12 years.Three out of 19 patients presented concomitant features of both syndromes,whereas 16 patients experienced the two syndromes at different times.Conclusions:BECTS and CAE may be pathophysiologically related,and the two epileptic phenotypes may indicate a neurobiological continuum.Further studies are needed to elucidate a probable genetic or functional link between partial and primarily generalized electro-clinical patterns in idiopathic childhood epilepsies.     

Epileptic seizures in childhood: classification, diagnosis, therapy

This is a review about epilepsies of childhood dealing with the elementary principles in classification, diagnosis and treatment. In each case of a patient suffering with epilepsy 3 diagnoses have to be made: the diagnosis of the seizure-type, the diagnosis of the epilepsy-type, the diagnosis of the etiology. The seizure-type has to be diagnosed as generalized (e.g. grand mal, absence seizure) or partial (simple or complex) which can be obtained from the clinical picture and EEG. The classification of seizures is different from the classification of epilepsies: recurrent seizures, only a single type or more types in the same patient, make the diagnosis of the epilepsy-type. The etiologic diagnosis is often not possible to make and requires careful judgment in the use of special procedures; however, the diagnosis of the seizure-type includes their etiology in as much as, for example, awake-grand mal and absence seizures can be classified as idiopathic resp. sleep-grand mal and partial seizures as symptomatic. The most essential principle for the treatment of epilepsies is: the seizures diagnosis determines the appropriate therapy. The drugs of choice for partial seizures and most cases with grand mal are phenytoin and/or carbamazepine; absence seizures and benign myoclonic seizures highly respond to valproate; atonic seizures are most resistant to therapy. To make an effective treatment you have to know the dose, the therapeutic plasma drug level, the drug's half life and the side-effects. The aim of epilepsy-treatment has to be: avoidance of seizures without side-effects.     

Absence epilepsy of early childhood —genetic aspects

Clinical and EEG family data of 140 cases with early childhood epilepsy with absences are presented. The aim of the study was to evaluate, whether the occurrence of generalized tonic clonic seizures (GTCS) as a presenting symptom might correlate with family data, i.e. whether there are indications of heterogeneity. One hundred and forty cases were selected from the epilepsy family data base of the Neuropaediatric Department. The selection parameter was epilepsy with absences manifesting between the 1 st and 5th year of age. The incidence of seizures was evaluated in siblings, parents and parents' siblings. EEG records were available from 103 parents and 106 siblings. The analysis supports the assumption of heterogeneity within early childhood absence epilepsy. Parents and their sibs of cases manifesting with GTCS had seizures twice as often than parents and their sibs in the non-GTCS group. In the affected relatives of the GTCS group early onset GTCS prevailed, whereas in the relatives of the non-GTCS group absences were found more frequently. The EEG of relatives showed elevated incidences of spikes and waves and photosensitivity in both groups, indicating common genetic factors. In parents of the non-GTCS group, however, EEG pathology was significantly more frequent than in parents of the GTCS group. Comparing EEG pathology in parents with seizure risk in siblings, evidence for maternal preponderance in transmission of the seizure liability was found. Mothers' EEG seems to be the best predictor of the seizure risk in probands' siblings. Early childhood epilepsy with absences can be regarded as an intermediate type, showing overlap with early onset GTCS and myoclonic astatic epilepsy on the one side and with childhood absence epilepsy on the other.     

丙戊酸和拉莫三嗪治疗儿童失神癫癎的临床研究

目的：评价丙戊酸和拉莫三嗪单药治疗儿童失神癫癎的临床疗效。方法：通过典型临床表现和视频脑电图（过度换气诱发实验阳性）确诊儿童失神癫癎,将患者进行随机开放对照分组研究。分别给予丙戊酸和拉莫三嗪单药治疗,随访1年,观察患者的发作控制情况,脑电图的改善以及不良反应。结果：共有48例入组,45例患者完成观察,其中丙戊酸治疗组23例,拉莫三嗪治疗组22例。丙戊酸组在服药12月时有17例实现无发作,其中15例脑电图无癎样放电。拉莫三嗪组在服药12个月时12例无发作（P＞0.05）,其中6例脑电图无癎样放电（P＜0.05）。所有患者均未见严重不良反应。结论：丙戊酸和拉莫三嗪均为治疗儿童癫癎的安全有效药物;丙戊酸控制癎样放电可能优于拉莫三嗪。［中国当代儿科杂志,2009,11（8）：653-655］     

Nonalcoholic fatty liver disease during valproate therapy

Valproic acid (VPA) is effective for the treatment of many types of epilepsy, but its use can be associated with an increase in body weight. We report a case of nonalcoholic fatty liver disease (NAFLD) arising in a child who developed obesity during VPA treatment. Laboratory data revealed hyperinsulinemia with insulin resistance. After the withdrawal of VPA therapy, our patient showed a significant weight loss, a decrease of body mass index, and normalization of metabolic and endocrine parameters; moreover, ultrasound measurements showed a complete normalization. The present case suggests that obesity, hyperinsulinemia, insulin resistance, and long-term treatment with VPA may be all associated with the development of NAFLD; this side effect is reversible after VPA withdrawal.     

Benign epilepsy of childhood: a speculation and its ramifications

Most children with generalized tonic-clonic seizures have a benign developmental disorder of seizure threshold that will be outgrown with or without treatment. Such children may have one or more infrequent seizures before adequate threshold is achieved. A small percentage of children will have frequent generalized seizures (epilepsy) due to brain damage or abnormality. Such patients require vigorous anticonvulsant therapy. However, for the children with "benign childhood epilepsy" treatment may be worse than the disease.     

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目的 探讨Gastaut型儿童枕叶癫痫(COE-G)的临床及脑电图特点.方法 随诊分析2004年6月至2008年6月遵义医学院附属医院儿科7例确诊为COE-G患儿的临床、脑电图特点及对治疗的反应.结果 9岁左右起病,临床特征为较频繁的日间视觉症状,常有头眼偏斜及偏头痛症状.3例伴偏侧阵挛,仅1例泛化为全身发作:患儿均有日间发作,2例夜间也有发作,脑电图示枕区为主的后头部高幅痫性放电,常为闭眼诱发,睁眼抑制:1例对多种抗癫痫药物反应不佳,2例伴认知问题.结论 COE-G起病较晚,较具确切特征表现,良性预后尚值得怀疑.     

Parental view of epilepsy in Angelman syndrome: a questionnaire study.

PURPOSE: To explore parents' opinions and concerns about seizures, anticonvulsants, and the effect of treatment in children with Angelman syndrome. DESIGN: A postal questionnaire was sent to members of one of the UK lay groups for Angelman syndrome (ASSERT) who had a child affected by Angelman syndrome. The questionnaire requested general medical information and information about the epilepsy, its treatment, and treatment responses. RESULTS: One hundred and fifty questionnaires were sent out with an ASSERT routine mailing and 78 completed questionnaires were returned. Forty three patients were boys and 35 were girls; ages ranged from 1.7 to 25 years (mean 7.5 years). The overall general clinical and cytogenetic data were mostly consistent with previous reports. Epilepsy was reported in 68 children, most of whom had a detectable cytogenetic deletion. The most common seizure types reported by the families were absence seizures, tonic clonic seizures, drop attacks, and myoclonic seizures; in four patients only febrile seizures occurred. The age at onset of the seizures was < 2 years in more than half of the patients. Anti-epileptic drug treatment with valproate (VPA), clonazepam (CZP), and lamotrigine (LTG) as monotherapy or a combination of VPA and CZP or VPA and LTG was more often viewed favourably and considered effective with fewer side effects on the child's behaviour and alertness, versus more frequent adverse effects and increased frequency and severity of seizures with carbamazepine (CBZ) and vigabatrin (VGB) in monotherapy or in combination with other anti-epileptic drugs. Seizures did tend to improve with age but were still present and disabling at older ages. CONCLUSIONS: This is the first study to record parents' opinions about seizures, anti-epileptic drugs, and treatment responses in children with Angelman syndrome, and it is one of the largest series on epilepsy and Angelman syndrome to be reported to date.     

Management of childhood epilepsy

Epilepsy is a common childhood neurological morbidity needs careful evaluation, relevant investigations and precise therapy with appropriate antiepileptic drugs for optimal duration. Majority of childhood epilepsy remits with antiepileptic drugs and should be managed in the community. Practising Pediatricians must counsel the family for possible etiology of epilepsy, compliance during treatment and possible outcome. It is important to distinguish pseudo seizures and nonepileptic events from true epilepsy, as this would reduce the burden of unwanted medication. It is also equally important to enable ourselves to recognize early predictors of intractability and refer them to appropriate referral units. Epilepsy is a social and an economic burden for the family. Preventive strategies by improved perinatal care, prevention and managemnt of neuro infections and infestations which will mitigate epilepsy burden in the community are essential. In this context it is important to familarize appropriate and relevant use of investigations. Inappropriate antiepileptic drug usage is common in the community and drug therapy should be rationalized. Epilepsy management requires a close interaction between the patient, family and the treating physician and a concerted effort is essential.     

When to start drug treatment for childhood epilepsy: The clinical–epidemiological evidence

IntroductionMany data on the course and prognosis after provoked and unprovoked single and multiple seizures in childhood have been collected in the past decennia by prospective, large-scale, long-term observational cohort studies. These data may serve to guide treatment decisions and help to design controlled trials investigating treatment strategies in childhood epilepsy.MethodsThe results of the Dutch study of epilepsy in childhood will be compared with those of other studies. We will also discuss the potential consequences of these results for the “why” and “when” of the decision to start treatment.ResultsRecurrence after a solitary unprovoked seizure in childhood is about 50%. Those with a recurrence have a similar outcome of their epilepsy compared to children presenting with multiple seizures, regardless whether they were treated after the first seizure or not. This argues in favour of postponing anti-epileptic drug (AED) treatment until at least a second seizure has occurred. After an unprovoked status epilepticus (SE), later outcome is not worse than after presentation with a short seizure. Therefore, long-term AED treatment after a single unprovoked SE may not be necessary either. The same holds true for children presenting with a short (less than one week) burst of unprovoked seizures. One quarter of them do not have recurrences and the final prognosis of children with recurrences does again not differ from the prognosis of the entire cohort. Findings in new-onset epilepsy further indicate that AED treatment can be safely omitted or at least postponed in about 15%, especially those with only a small number of seizures before presentation, those with benign partial epilepsy and those with sporadic generalised tonic-clonic seizures.On the reverse side, three considerations might lead to the decision to start early and aggressive treatment: the dangers of the seizures, the chance of intractability and the possibility of intellectual decline caused by recurrent seizures or epileptic activity. In idiopathic generalised absence epilepsy, the risks of accidents and learning problems indeed prompt early AED treatment. A self-propagating mechanism of seizures promoting the occurrence of more seizures, in the end causing intractable epilepsy (Gowers), occurs only rarely. Real intractability is seen in only 5–15% of the children with new-onset epilepsy. The chance of intractability is increased by variables like symptomatic aetiology, localisation-related epilepsy, and an early unfavourable course. Landau–Kleffner or continuous spikes and waves during sleep (CSWS) syndrome cause cognitive decline and syndromes like West, Lennox–Gastaut or Dravet's induce both psychomotor regression and intractability. In such cases, early aggressive treatment is indicated, including early consideration of the ketogenic diet, immunotherapy, vagus nerve stimulation and, if possible, referral for epilepsy surgery.ConclusionsOmitting or postponing treatment after a solitary seizure, an unprovoked SE, a single burst of seizures or multiple infrequent seizures usually does not worsen the prognosis. A poor prognosis and the consequent indication for early and aggressive treatment are dependent mainly upon the presence of variables like symptomatic aetiology, certain epilepsy types and syndromes, and the early evolution of the epilepsy in that particular child. Intellectual decline caused by seizures or epilepsy is rare and may be confined to certain specific and readily recognizable syndromes.