Childhood epilepsy and sleep

Sleep and epilepsy are two well recognized conditions that interact with each other in a complex bi-directional way. Some types of epilepsies have increased activity during sleep disturbing it; while sleep deprivation aggravates epilepsy due to decreased seizure threshold. Epilepsy can deteriorate the sleep-related disorders and at the same time; the parasomnias can worsen the epilepsy. The secretion of sleep-related hormones can also be affected by the occurrence of seizures and supplementation of epileptic patients with some of these sleep-related hormones may have a beneficial role in controlling epilepsy.     

癫癎儿童发作间期全夜睡眠状况初步分析

目的：随着睡眠医学的发展,癫癎患者的睡眠质量作为生活质量的一个重要内容已日益受到重视。由于儿童睡眠研究的复杂性,目前关于癫癎儿童睡眠状态的研究较少。该研究旨在初步探讨癫癎对儿童发作间期睡眠的影响。方法：采用多导睡眠生理脑电记录仪对48名癫癎儿童及12名正常健康对照儿童进行全夜多导睡眠脑电记录仪检查,按照癫癎发作类型、发作好发时间将患儿分为全面性癫癎组、局灶性癫癎组以及清醒发作组、睡眠发作组,评价各组患儿与健康对照儿童之间各项睡眠参数的差异。结果：与健康对照儿童比较,癫癎儿童睡眠效率(SE)下降（85.4±8.6）% vs （90.9±5.8）% ,(P<0.05)。局灶性癫癎儿童睡眠总记录时间(TRT)延长、SE下降;全面性癫癎非快速动眼S1期睡眠比例（S1%）增加,快速动眼睡眠比例(REM%)减少;易在睡眠期发作的癫癎S1%延长,REM%、SE较对照组减少,其中全面性癫癎组REM%、SE缩短,局灶性癫癎组TRT、S1%延长,SE下降;易在觉醒期发作的癫癎与对照组各项睡眠参数无统计学差异。结论：癫癎可影响儿童的睡眠结构,不同类型的癫癎对儿童睡眠结构影响不同。全面性癫癎患儿浅睡眠延长、快动眼睡眠缩短,其中易在觉醒期发作的儿童以浅睡眠延长为主,易在睡眠期发作的儿童以快动眼睡眠缩短、睡眠效率降低为主。局灶性癫癎患儿睡眠效率降低,其中易在觉醒期发作的儿童睡眠结构无异常,易在睡眠期发作的儿童以浅睡期延长、睡眠效率降低为主。［中国当代儿科杂志,2007,9（1）：6-10］     

视频脑电图在睡眠相关发作性疾病中的诊断价值

目的探讨视频脑电图在睡眠相关发作性疾病的诊断与鉴别诊断中的应用价值。方法收集2005年1月～2006年6月在我院儿科病房和门诊就诊的具有与睡眠相关发作性症状患儿65例。其中拟诊癫痫35例,非性发作性疾病30例。全部行常规脑电图（EEG）和视频脑电图（VEEG）检查。结果拟诊癫痫35例,性波阳性率VEEG为62.86%（22/35）,EEG为17.14%（6/35）,有显著性差异（χ^2=15.23P〈0.01）;经EEG监测,结合其临床表现,确诊癫痫27例中,额叶癫痫2例,颞叶癫痫1例,儿童良性癫痫伴中央中颞区棘波7例;VEEG检查其中样放电22例（81.48%）,睡眠期18例（81.82%）,清醒期11例（50%）,睡眠相关性癫痫睡眠期异常放电率高于清醒期（χ^2=4.96P〈0.05）;共监测到发作22例,其中非癫痫性发作15例（睡眠肌阵挛10例、睡眠障碍3例、阵发性肌张力不全,生长痛各1例）。结论VEEG是鉴别发作性质及类型的最有效检查方法,也是鉴别癫痫与非癫痫性发作的有效方法。     

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).     

视频脑电图诊断非癫癎性发作的价值

为了解视频脑电图对非癫Jian性发作的诊断价值，对252例拟诊或排除癫Jian的患儿进行视频脑电图检查。结果：共监测到发作52例，非癫Jian性发作35例，其中睡眠期肌阵挛10例，睡眠障碍6例，习惯性抽动5例，歇斯底里2例，非癫Jian性发作性强直4例，行为异常4例，头痛、腹痛发作2例，百日咳发作、婴儿拥抱反射各1例；癫Jian性发作17例，其中强直和阵挛性发作4例，强直性发作4例，阵挛性发作3例，局限运动性发作2例，复杂部分性发作、肌阵挛性发作、婴儿痉挛、大田原综合征各1例。结果表明，视频脑电图是鉴别发作性质及类型的最有效的检查方法，也是鉴别癫Jian与非癫Jian性发作的有效方法。     

Landau-Kleffner 综合征的临床特征和远期预后

目的研究Landau-Kleffner综合征（LKS）的临床、脑电图特征、治疗反应和远期预后。方法对10例LKS患儿的临床及脑电图资料进行分析,并随访治疗效果及远期预后。结果起病年龄2～10、5岁。10例患儿均有获得性失语,表现为听觉失认。均有癫癎发作,8例有睡眠中部分运动性发作,其他发作形式还有不典型失神发作和全身强直一阵挛发作。9例伴有精神行为异常。10例均无听力异常、神经影像学异常及家族史。10例均有脑电图异常,9例有颞区限局性棘慢波发放,8例进行录像脑电图（VEEG）监测,4例发现睡眠中癫疴性电持续状态。10例癫疴发作均可用抗癫疴药物控制。经皮质激素治疗,10例失语均有改善。随访8例,癫癎发作控制均良好,5例语言较正常差。6例随访VEEG,2例停用激素后再次出现睡眠中持续放电。结论LKS是一个以获得性失语和癫疴发作为主要临床表现的儿童时期癫癎脑病。失语表现为听觉失认,多伴有其他精神行为异常。EEG常见颞区的限局性放电,睡眠期常泛化全导,并可呈持续发放。抗癫疴药物可控制癫疴发作,但对失语疗效不佳。早期应用足量皮质激素治疗可明显改善失语情况。EEG及癫癎发作转归良好,但常遗留语言障碍。     

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目的总结青少年肌阵挛性癫疒间(JME)的临床及脑电图特点。方法对2000年11月至2004年11月就诊于复旦大学儿科医院的44例JME患儿的临床资料进行回顾性分析。结果44例JME患儿100%出现全身性肌阵挛发作,16例(36·4%)伴有失神发作,33例(75%)伴有全身性强直阵挛性发作,11例(25%)3种发作均有,6例(13·6%)只有全身性肌阵挛发作。首次就诊即确诊为JME13例,有31例曾误诊。清醒脑电图阳性率为50%,睡眠脑电图阳性率为95·5%,3例动态脑电图检查均阳性。JME患儿43例给予药物治疗,除1例死亡外,其中3例偶有肌阵挛性发作,余39例发作均控制。治疗过程中出现肌阵挛持续状态(JMES)者有7例,占15·9%。结论JME是儿童时期较常见的癫疒间综合征,临床医生在诊断中要多询问病史,结合规范的睡眠及动态脑电图检查,以提高检出率,并合理选择抗癫疒间药物。     

癫癎发作生物节律的研究进展

随着对癫癎研究的不断深入,癫癎发作的时相性越来越受到关注。癫癎发作存在生物节律,同一类型的癫癎在不同时间段、不同状态(睡眠/觉醒、昼夜更替)发作频率不同。癫癎生物节律的分子机制、内分泌机制相当复杂,目前存在多种假说。明确癫癎发作的生物节律,根据其发作节律予以预防及治疗,既可以有效控制癫癎发作,也可以减少药物不良反应。癫癎发作生物节律的研究将为癫癎治疗提供新的思路。     

Early identification of epileptic encephalopathy with continuous spikes-and-waves during sleep: A case-control study

Epileptic encephalopathy with continuous spikes-and-waves during sleep (EE-CSWS) is a rare childhood epilepsy syndrome characterized by a regression in cognitive, behavioral and psychiatric functioning, seizures and a specific electroencephalographic pattern. An early recognition and an appropriate treatment might play a key role in the outcome of this epileptic encephalopathy. We conducted a case-control study to evaluate if there is any clinical or electroencephalographic sign suggestive of EE-CSWS after the first seizure. We retrospectively identified 10 EE-CSWS patients with available EEG recordings at time of the first seizure. We matched them with 10 controls from our first seizure clinics. All EEG recording were analyzed for the study. We did not find any clinical or EEG features that would suggest later development of EE-CSWS. As reported by others, the occurrence of multiple seizures types and a seizure worsening during the follow-up is more frequent in the cases than in the controls. These clinical criteria might be used as a red flag in clinical practice to identify the very few patients with EE-CSWS among the frequent patients with BECTS.     

Topiramate as a new antiepileptic drug in epileptic children in Iran

Objective It is known that current antiepileptic drugs cannot control seizures in 20–30% of patients. The aim of this study was to evaluate the efficacy and safety of topiramate (TPM) as add-on therapy in intractable epileptic children in Iran. Methods As a quasi- experimental (before and after) study, 42 iranian children aged 1–15 years, 28 boys and 14 girls with refractory seizures seeking treatment were recruited to be subjects of this study. Results Type of seizures of those 42 epileptic children were as follows: L.G.S. (n=14), idiopathic epilepsy (n=8), symptomatic epilepsy (n=16) and progressive myoclonic epilepsy (n=4). At the end of three months of treatment in which topiramate was used concomitantly with previous AED, 17% became seizure free, 26% had more than 50% reduction of seizure frequency and 5% of them had increasing seizures. Therefore, the drug is statistically significant in seizures reduction. The efficacy of the drug was statistically significant in idiopathic and symptomatic epilepsy. The author’s did not notice any serious side effects such as: hematologic abnormality, hepatotoxicity and nephrotoxicity. Conclusion This study supports efficacy and safety of TPM in controlling of intractable epilepsy in children and indicates the drug should be considered as an add-on therapy in the management of refractory epileptic syndromes.     

Epileptic seizures in childhood: from seizure type to diagnosis]

Epileptic seizures can be difficult to recognize in infancy and childhood because the semeiology can be misleading. Already, in the acute phase, precise assessment of the seizure is required, with active questioning about circumstances of occurrence, clinical manifestations and postictal symptoms. Laboratory tests and toxicologic screening should only be performed according to the circumstances and clinical examination in order to distinguish between symptomatic seizure and epilepsy at the beginning. Epilepsy consists in repetition of several unprovoked epileptic seizure. Assessment of the age of onset, type of seizures, interictal EEG and the neuropsychological profile are instrumental for both the diagnosis of epileptic syndrome and the choice of the right treatment. Epileptic seizures cause distress to parents and the fear they experience of death must always be taken into account.     

Late onset epileptic spasms is frequent in MECP2 gene duplication: Electroclinical features and long-term follow-up of 8 epilepsy patients

Mutation of the X-linked methyl CpG binding protein 2 (MECP2) has been first identified as the cause of Rett syndrome. More recently, MECP2 gene duplication syndrome has been identified in males. The MECP2 duplication syndrome is characterized by severe mental retardation, infantile hypotonia, progressive spasticity and recurrent infections. Epileptic seizures are inconstant but poorly described. The aim of the study is to describe the electroclinical features of epilepsy in MECP2 duplication patients in order to refine the epilepsy phenotype and its evolution.MethodsWe conducted a retrospective study in four child neurology departments in France. Eight boys with a MECP2 gene duplication and epilepsy were retrospectively studied. We evaluated both clinical and electroencephalographic data before seizure onset, at seizure onset and during the follow-up.ResultsThe patients started seizures at the median age of 6 years (range: 2.5–17 years). Half exhibits late onset epileptic spasms while the other exhibit either focal epilepsy or unclassified generalized epilepsy. Before seizure onset, EEGs were abnormal in all patients showing a slowing of the background or a normal background with fast activities, while EEG performed in epileptic patients, showed a slowing of the background in 6/8 and localized slow or sharp waves in 7/8. Most patients (6/8) have evolved to drug resistant epilepsy.ConclusionAlthough late onset epileptic spasms are common in patients with MECP2 duplication, no specific electroclinical phenotype emerges, probably due to genetic heterogeneity of the syndrome. Further studies are needed to individualize specific epileptic subtype in larger cohort of patients.     

伴有非癫癎发作的322例脑性瘫痪临床分析

目的：了解伴有非癫癎发作的脑性瘫痪（脑瘫）的临床特点,以提高与癫癎发作的鉴别能力。方法：1 198例9个月至6岁的脑瘫患儿纳入研究,对伴随发作性症状的患儿进行24 h视频脑电图监测,明确伴随的发作是否为癫癎发作,并观察伴随非癫癎发作患儿的发作症状、年龄、脑瘫型别及脑电图特征。结果：共578例（48.24%）患儿伴随发作性症状,其中伴随癫癎发作者231例（19.28%）,非癫癎发作322例（26.88%）。322例伴随非癫癎发作的脑瘫患儿中,发作性症状包括非癫癎性强直发作、发作性摇头、耸肩或头后仰、发作性哭闹、惊恐发作、睡眠肌阵挛、刻板性运动等;158例（49.1%）显示脑电图有非特异性异常;111例（34.5%）在基层医院误诊为癫癎;1岁以内患儿非癫癎发作频率高于1岁~和3~6岁组患儿;痉挛型脑瘫患儿非癫癎发作频率最高（168例,52.2%）,其次为不随意运动型（69例,21.4%）和混合型(65例,20.2%)。结论：脑瘫患儿伴随的发作性症状部分为非癫癎发作,注意与癫癎发作相鉴别。非癫癎发作发生频率与患儿年龄及脑瘫型别有一定关系。［中国当代儿科杂志,2010,12(12)：933-935］     

伴高热惊厥史的儿童癫痌病例分析

分析伴高热惊厥史的癫痌患儿的临床特点,探讨高热惊厥脑损伤及其与颞叶癫痌的关系。 方法对1996～1999年本院儿科神经病房480例住院癫痌患儿进行回顾性分析,包括首发年龄、家族史、持续时 间、癫痌发作类型、神经影像学及脑电图改变等。结果115例(23.9％)患儿有前期高热惊厥史。伴高热惊厥史 的患儿癫痌发作早且易于出现癫痌持续状态。与无高热惊厥史的患儿相比,伴高热惊厥史的患儿强直-阵挛发作 较多,复杂部分性发作较少。408例患儿曾行影像学检查,4例提示有海马硬化者均无高热惊厥史。在伴高热惊厥史 的癫痌患儿中脑电图局灶起源的异常放电显著低于无高热惊厥史的癫痌患儿。有6.08％(7/115)伴高热惊厥史的癫 痌惠儿和6.84％(25/365)无高热惊厥史的癫痌患儿脑电图表现为单纯颞叶异常放电,二组相比无明显差异。结论 在癫痌患儿中,高热惊厥可能伴有脑损伤,且可能与后期的癫痌发生有关,伴高热惊厥史者不一定发展为颞叶癫痌。     

Evaluation of children for epilepsy surgery

There are three main components to the evaluation of children who are candidates for epilepsy surgery: (1) confirmation of the epileptic nature of the seizures, (2) establishing the medical intractability of the child's epilepsy and (3) localization of the epileptogenic zone. Differentiating epileptic from nonepileptic events requires careful review of the history and the electroencephalogram. Electroencephalographic recording of the habitual seizures usually suffices to confirm the epileptic or nonepileptic nature of the events. Medical intractability is established by assuring that antiepileptic medications appropriate for the child's particular epilepsy syndrome have been used and have been titrated to maximum tolerance before being deemed ineffective. Confident localization of the epileptogenic zone requires that multiple findings such as behavior during the seizure, ictal and interictal EEG, and cerebral imaging studies provide data that are convergent with respect to a likely area of seizure onset.     

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?? Diagnostic value of video??EEG in infants with non??epileptic seizures. Shen Nanping,Hua Ying.Wuxi Children??s Hospital,Wuxi 214002,China AbstractObjectiveTo evaluate the diagnostic value of video??EEG in infants with non??epileptic seizure. MethodsFrom Jan.2004 to Jan.2005,152 infants suspected to have or need to weep out the possibility of epilepsy were examined with video??EEG in our clinics. ResultsNon??epileptic seizures 51:including non??epileptic tonic seizures 10 ??benign nocturnal myoclonus 8?? breath??holding spell 7??mashturbstion??like episodes 6??cerebral palsy 5??benign myoclonus of early infancy 5??benign infantile convulsions associated with mild gastroenteritis 4??other nonepileptic episodes 6. ConclusionThe results indicate that the video??EEG is not only the most sensitive way to differentiate the seizure characteristics and patterns,but also the most effective way in differenting epileptic seizures from the non??epileptic seizures.     

The clinical use of dynamic EEG in childhood]

A-EEG is an important recent technologic innovation in EEG recording that facilitates long-term monitoring. The system consists of a miniature cassette tape recorder and a video play-back unit, which permits the taped EEG to be reviewed (Brain Spy CH24, Micromed). Because it is extremely lightweight and portable, the system permits unrestricted activity during recording. On the other hand, this predisposes the recording to more artifacts than are seen in routine recordings. We examined 103 patients, aged 3 months-24 years, between July 1988 and July 1990. Patients were divided into three groups: group 1 included 61 subjects with evidence of epilepsy and clinically definite seizures; group 2 included 29 patients with recurrent episodes that were not clearly epileptic (suspected "pseudo epileptic"); group 3 included 13 subjects with psychiatric disorders. We found that the clinical utility of A-EEG in epileptic children was: 1) obtain better clinical and EEG characterization and circadian distribution of seizures in 17 cases (28%); 2) quantify epileptiform generalized abnormalities and their variations during the sleep in 6 cases (10%); 3) verify the efficacy of specific drug treatment such as Bzd and ACTH in 12 cases (20%). The role of A-EEG in non-epileptic children with pseudoseizures was to establish the epileptic or non epileptic nature of some ictal events by detecting EEG seizure patterns in 11 cases (38%). As to regard the group 3, A-EEG has permitted to study sleep architecture and REM sleep measures, especially in depressed children compared to normal children. We discuss advantages, drawbacks and clinical applications of A-EEG in child neurology and psychiatry vs conventional EEG.     

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.     

儿童额叶癫(癎)的临床与脑电图特征

目的 总结儿童额叶癫癇(FLE)的临床表现、脑电图(EEG)特征和预后.方法 对28例FEE患儿的临床资料进行回顾性分析.结果 起病年龄21个月～17岁,癫癇发作表现:躯体运动性自动症20例,偏转性强直18例,姿势性强直19例,植物神经症状10例,局部阵挛11例,发声9例,额叶失神3例,简单自动症3例,感觉异常1例,发笑发作1例.头颅影像学异常13例,长时间视频脑电图(V-EEG)检查78.6%(22/28例)记录到疴样放电,67.9%(19/28例)记录到临床发作.26例额叶癫癇随访1～6年,抗癫癇药物治疗效果良好15例.结论 额叶癫癇是一组具有特征性的癫癇综合征,V-EEG是临床无创伤性诊断额叶癫癇最佳方法,口服抗癫癇药物疗效良好.     

婴儿癫发作特征的录像脑电图分析

目的　分析婴儿癫发作的录像脑电图 (Video EEG)特征。方法　根据癫发作分类法对 45例婴儿 10 6次癫发作的Video EEG资料进行分析。结果　 (1)全身性发作包括 :①全身性粗大肌阵挛 8例 32次发作 ,发作期EEG为阵发性全导棘慢波、多棘慢波或弥漫性低电压 ,肌阵挛与EEG相关性良好 ;②散发游走性肌阵挛 3例 ,面部及肢体远端频发间断肌阵挛 ,EEG为广泛持续慢波及多灶性棘波、尖波 ,肌阵挛与EEG无相关性 ;③婴儿痉挛 10例 17次发作 ,背景EEG为高峰节律紊乱 ,发作期为高波幅爆发和 (或 )低波幅抑制。全身性发作中缺乏完整的全身强直 阵挛性发作及失神发作。(2 )部分性发作 16例 42次发作 ,突出表现为无动性凝视 ,其次为简单自动症、植物神经症状及轻微的惊厥性症状。多数意识状态难以准确判断。EEG提示发作起源于额区、中央区、颞区或枕区。部分性继发全身性发作 5例 7次发作 ,EEG提示阵发性放电分别起源于颞区 2例 ,枕区 2例及多灶性 1例。(3)不能分类的发作 3例 5次发作 ,Video EEG主要表现为在 1次发作中出现多种发作类型。结论婴儿期癫发作在识别和分类上都比较困难。Video EEG监测同步分析有助于对婴儿癫发作的准确观察与分类。