Vigabatrin in pediatric patients with refractory epilepsy

New generation antiepileptic medications have improved seizure outcome in patients with intractable epilepsy. We studied the efficacy and side effect profile of vigabatrin (VGB) in pediatric patients with intractable seizure disorder. We reviewed the database of our short-term video-EEG monitoring laboratory to screen patients with intractable epilepsy who were on VGB either alone or in combination for three months or more. We subsequently reviewed the medical records of these patients to abstract clinical information regarding age, sex, seizure type, epilepsy syndrome, efficacy and side effects of VGB. Of 111 patients, 75 (68%) were male and 36 (32%) female. Seizure onset was during the newborn period in 12 patients (11%), during the first year of life beyond the newborn period in 47 patients (42%), between 1-5 years in 23 patients (21%), and above five years in the remaining 29 patients (26%). Fifty-four patients (48.6%) had partial onset seizures with or without secondary generalization; 49 patients (44.1%) had primary generalized seizures; 8 patients (7.2%) had two or more types of seizure. Fifty-three percent of patients had mental retardation, and 35% had abnormal findings on physical/ neurological examination. Of 98 patients, 70 (71.4%) had abnormal magnetic resonance imaging (MRI) findings. Ninety-seven percent of patients had been on polytherapy before VGB was added to treatment. VGB reduced seizure frequency by at least 50% in 33.3% of patients with partial seizures, and in 30.6% of patients with primary generalized seizures. Six of the responders with partial seizures had complete resolution of their seizures. Most common side effects included visual field defects, increased appetite and obesity. Vigabatrin seems to be more effective in partial seizures in childhood intractable epilepsy. Patients should be closely monitored regarding side effects of VGB.     

Benign partial epilepsy in infancy

The aim was to examine the occurrence of benign partial epilepsy in infancy (BPEI). BPEI was defined as epilepsies with complex partial seizures (CPS) or secondary generalised seizures (SGS), or both, compatible with the following characteristics: normal development before and after onset, no underlying disorders, normal interictal electroencephalograms (EEGs), and good response to treatment. All 75 patients who developed epilepsy within the first 2 years of age between 1987 and 1993 were evaluated: 22 patients fulfilled the definition completely; eight had CPS only, four SGS only, and 10 had both CPS and SGS; 17 had clusters of seizures. Eight patients had a positive family history. The average age of onset of seizures was 5.9 months. Interictal EEGs were all normal. Response to treatment was excellent and the average period of seizure persistence was 3.0 months. All had normal psychomotor development. Patients with BPEI were more common in this study than previously reported.     

Add-on levetiracetam in children and adolescents with refractory epilepsy: Results of an open-label multi-centre study

PURPOSE: To study the efficacy and tolerability of add-on levetiracetam in children and adolescents with refractory epilepsy. METHODS: In this prospective multi-centre, open-label, add-on study, 33 children aged 4-16 years (median 8.5 years) with epilepsy refractory to at least two antiepileptic drugs were treated with levetiracetam in addition to their present treatment regimen with a follow-up of 26 weeks. The starting dose of 10 mg/kg/day was increased with 2-week steps of 10 mg/kg/day, if necessary, up to a maximum dose of 60 mg/kg/day. RESULTS: Retention rate was 69.7% after 26 weeks on a median levetiracetam dosage of 22 mg/kg/day. Four children dropped-out because levetiracetam was ineffective, four because seizure frequency increased and/or seizures became more severe, and two because they developed aggressive behaviour. Compared to their baseline seizure frequency, 13 children (39.4%) had a >50% seizure reduction 12 weeks after initiation of levetiracetam, and 17 children (51.5%) at 26 weeks. At 26 weeks, nine children (27.3%) had been seizure-free for at least the last 4 weeks, terminal remission ranged from 0 to 187 days (mean 46 days). Levetiracetam was effective in both partial and primary generalized seizures, but had most effect in partial seizures. Most reported side effects were hyperactivity (48.5%), somnolence (36.4%), irritability (33.3%) and aggressive behaviour (27.3%). Severity of most side effects was mild. Five children had a serious adverse event, which all concerned hospital admissions that were not related to levetiracetam use. CONCLUSION: Levetiracetam proved to be an effective and well-tolerated add-on treatment in this group of children with refractory epilepsy.     

Vagus nerve stimulation for children and adolescents with intractable epilepsies

BACKGROUND: Vagus nerve stimulation (VNS) has been shown to be efficacious in the treatment of patients > 12 years of age with refractory partial epilepsies and it is suggested that VNS should be considered as one of the treatment options for these patients. METHODS: Four patients had partial epilepsies and one had symptomatic generalized epilepsy. After observation of the baseline seizure frequency and the average seizure frequency for 3 months, the VNS system was implanted. Thereafter, seizure frequency, average seizure frequency of each seizure type during the month just before the evaluation, seizure severity, side effects and quality of life were recorded. RESULTS: In four of five patients, overall seizure frequency was reduced > 50% after VNS treatment. The seizure types that showed a > 50% reduction in frequency were auras, focal clonic, generalized tonic clonic seizures, astatic, versive, hypomotor, generalized tonic and generalized clonic seizures according to Lüders' classification. In two patients, as major convulsive seizures were reduced in number after VNS treatment, dialeptic seizures (non-convulsive seizure with lapse of consciousness) gradually appeared. In one patient without significant seizure reduction, quick recovery from postictal periods after generalized tonic seizure was seen after treatment. In one patient with generalized epilepsy, improvement of cognitive function was reported by his guardians. After VNS, the number of antiepileptic drugs was reduced from three to one in one patient. No significant adverse effects were noted in any patients. CONCLUSIONS: Our results suggest that VNS is well tolerated in young patients with intractable epilepsies and it may be an important non-pharmacologic treatment option for children with severe epilepsies who cannot tolerate medical therapy and/or are not candidates for epilepsy surgery.     

Factors predicting the risk of relapse after antiepileptic drug discontinuation in children with partial seizures

The purpose of this study is to identify possible factors which could influence the seizure recurrence after anti-epileptic drug (AED) withdrawal in children with partial epilepsy. AED was discontinued in 82 children who had been free of partial epileptic seizures for 2.0–11.0 years (mean 4.7 years). Twenty-four patients (29.3%) had a relapse from a few days to 6.1 years (mean 1.2 years) after AED discontinuation. Significantly more common in children who relapsed were: younger age at beginning of AED withdrawal, occurrence of complicated febrile convulsions (5/24 vs 1/58,P<0.01), abnormal neurological examination (8/24 vs 8/58,P<0.05), delayed psychomotor development (7/24 vs 7/58,P<0.05), focal slowing (6/24 vs 3/58,P<0.01) and focal epileptiform discharges (7/24 vs 6/58,P<0.05) in the last EEG before AED discontinuation. Between the two groups no statistical significant differences no statistical significant differences concerning the age at onset of seizures, the duration at AED therapy after the last seizure, the familial occurrence of epilepsy and background EEG abnormalities in the last EEG before AED discontinuation were found. On the basis of EEG, occurrence of febrile convulsions, and neurological and developmental examination it may be possible to predict which children have the best chance to remain free of recurrence after AED discontinuation.Presented in part at the European Congress of Epileptology, Oporto, Portugal, September 6–10, 1994     

Prognosis of partial epilepsy.

The prognosis of partial epilepsy in childhood (excluding cases of benign partial epilepsy) was studied; the average follow up period was 7.4 years. Improvement rate of seizure status was 82.3%. We studied favourable prognostic factors and found that those most often associated with seizure improvement were familial convulsions and idiopathic forms, no generalised seizures before partial onset, low frequency of seizures after 12 months of treatment, short duration of epilepsy, and no background activity abnormalities on electroencephalography. We also observed such factors as mental retardation, neurological abnormalities, and behaviour and cognitive disorders. Factors that determined the prognosis for social adjustment were similar to those for seizure improvement. We discuss the favourable prognosis of partial seizures in childhood and the predictive factors.     

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.     

左乙拉西坦单药治疗小儿癫癎的疗效和安全性随访研究

目的：评价左乙拉西坦(LEV)单药治疗小儿癫癎的疗效和安全性。方法：对该院2007年3月至2008年3月LEV单药治疗的32例癫癎患儿进行开放性自身对照随访研究,起始量每日10 mg/kg,每1周增加上述剂量1次,3~4周增加至维持剂量每日20～60 mg/kg,平均剂量为每日35 mg/kg。结果：LEV 单药治疗32例,失访1例,其余随访均在3个月以上。 80.6%（25/31）患儿发作减少≥50%,70.9%（22/31）患儿无发作,16.1%（5/31）患儿因发作控制不满意或者加重而停药。不良反应包括情绪异常19.4%（6/31）,乏力6.5%（2/31）,嗜睡6.5%（2/31）,皮疹3.2%（1/31）。上述不良反应均为一过性,在1～4 周内自然消失或者减量后消失,未导致停药,未发现过敏以及血液、肝、肾功能异常等严重不良反应。结论：LEV用于癫癎患儿（包括<4岁的婴幼儿）的部分性发作及全身性发作的单药治疗,疗效肯定、安全性好,是一个很有希望的用于儿童单药治疗的广谱抗癫癎药。     

Recurrence risk after withdrawal of antiepileptic drugs in children with epilepsy: A prospective study

AimTo study recurrence risk after withdrawal of antiepileptic drugs in children with epilepsy.MethodsAll children younger than 14 with two or more unprovoked seizures 24 h apart who were seen at our Hospital between 1994 and 2004 were included consecutively and prospectively followed. Patients previously examined in other centres were excluded. All patients who entered a remission were proposed to stop medication and were followed.ResultsThree hundred and fifty three children with two or more unprovoked seizures were attended. A total of 238 entered a remission period and were proposed to stop medication, 216 accept. Mean seizure-free time before medication withdrawal was 2.2 years. Kaplan-Meier estimate of recurrence risk was 23% at 2 years (95% CI: 17–29) and 28% at 5 years (95% CI: 22–34). A remote symptomatic etiology, various seizure types and a history of prior febrile seizures or prior neonatal seizures were associated with a significant increase in recurrence risk in univariable and multivariable analyses using Cox proportional hazards model. Recurrence risk at 2 years was 17% (95% CI: 11–23) for idiopathic/cryptogenic epilepsies and 41% (85% CI: 28–54) for remote symptomatic epilepsies. Recurrence risks at 2 years by epileptic syndrome were West syndrome (0%), benign rolandic epilepsy (10%), epilepsy without unequivocal partial or generalized seizures (11%), benign infantile seizures (13%), absence epilepsy (16%), cryptogenic partial epilepsies (20%), symptomatic partial epilepsies (45%), symptomatic generalized epilepsies (54%).ConclusionsRecurrence risk after withdrawal of antiepileptic treatment in children is low. Etiology and syndromic diagnosis are the main predictive factors.     

Vagus nerve stimulation for partial and generalized epilepsy from infancy to adolescence

Object Vagus nerve stimulation (VNS) is approved by the FDA for the treatment of partial epilepsy in patients older than 12 years. Authors of the current study performed a large retrospective analysis and comparison of VNS outcomes in children with an age ≥ and < 12 years, including those with partial and generalized epilepsy. Methods A retrospective review of the records of pediatric patients (age < 18 years) who had undergone primary VNS system implantation between 2001 and 2010 by a single pediatric neurosurgeon was undertaken. Considered data included demographics, epilepsy type (partial vs generalized), seizure frequency, seizure duration, postictal period duration, and antiepileptic medication use. Results One hundred forty-six patients (49% female) were followed up for a mean of 41 months after VNS implantation. Thirty-two percent of patients had partial epilepsy and 68% had generalized epilepsy. After VNS system implantation, seizure frequency was reduced in 91% of patients, seizure duration in 50%, postictal period in 49%, and antiepileptic medication use in 75%. There was no significant difference in age, sex, or duration of follow-up according to epilepsy type. Neither was there any significant difference in seizure frequency reduction, seizure duration, postictal period, medication use, overall clinical improvement, or improvement in quality of life based on an age ≥ or < 12 years or epilepsy type. Conclusions Vagus nerve stimulation reduced both seizure frequency and antiepileptic medication use in the majority of pediatric patients regardless of sex, age cohort, or epilepsy type. Vagus nerve stimulation also reduced seizure duration and postictal period in approximately half of the pediatric patients. Contrary to expectation, children with partial epilepsy do not benefit from VNS at higher rates than those with generalized epilepsy.     

孤独症合并癫患儿的临床及脑电图特征

目的 探讨孤独症合并癫患儿的临床、脑电图(EEG)特征及治疗效果.方法 对56例孤独症合并癫患儿应用抗癫药物治疗,治疗前后行EEG检查,观察其预后,并对临床特点及EEG进行分析.结果 孤独症合并癫患儿中,癫发作类型以局限性发作最为常见(75%),其中63%是复杂局限性发作.其他较常见的发作形式是强直性发作(36%)和热性惊厥(27%),强直-阵挛性发作、失张力发作、不典型失神发作等发作形式也可出现.EEG主要表现为阵发性异常,包括背景活动减慢、阵发性快活动、阵发性δ活动、阵发性θ活动等非特异改变10例,以及局灶性、多灶性、弥散性尖(棘)波、尖(棘)慢波活动等性放电46例,其中位于颞叶26例(46%),额叶10例(18%),中央区9例(16%),枕叶6例(10%).病变部位双侧局灶性29例(52%),单侧局灶性19例(34%),弥散性异常改变8例(14%).经正规抗癫治疗,77%的病例获得1 a以上无发作控制效果,其中63%单药治疗有效,37%的患儿联合用药有效,23%的患儿未能获得控制.结论 孤独症合并癫患儿存在不同类型的癫发作形式,局限性发作为其最常见的发作形式,大部分为复杂局限性发作.EEG主要表现为阵发性异常,部分出现癫样放电,EEG异常多起源于颞叶,多双侧出现.抗癫药物对于这类患者的癫发作有很好的控制效果,与其他原因所致癫治疗效果类似.     

Gelastische Anfälle, Pubertas praecox und Verhaltensstörung bei hypothalamischem Hamartom

Hypothalamic hamartomas are one possible reason for drug-resistant focal epilepsy with gelastic, complex focal, and secondary generalized tonic-clonic seizures. Resective surgery carries a high risk of complications because of the difficult anatomical connections. We report about one patient who was treated successfully by interstitial radiosurgery after presurgical monitoring including noninvasive and invasive video EEG monitoring. After the first seed implantation he had a reduction of seizure frequency by more than 50% After a second seed implantation he was seizure free for 6 months. There were no complications, and social interactions clearly improved. As far as we know, this is a new treatment of symptomatic epilepsy associated with hypothalamic hamartomas.     

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。     

16例Rasmussen综合征的临床特征和治疗

目的总结Rasmussen综合征（RS）的临床特征和治疗方法。方法对16例RS患儿的临床资料进行分析,并对患儿的预后进行随访。结果起病年龄1岁11个月～11岁6个月。均以癫癎发作为首发症状,主要的发作形式均为部分运动性发作。16例均进展为持续性部分性癫癎。13例就诊时已有偏瘫,出现固定偏瘫的时间为起病后2个月～3年。14例出现不同程度的认知障碍。EEG背景双侧不对称性慢波;发作间期均有异常放电,其中一侧半球放电11例,双侧半球放电5例;16例均记录到发作期EEG,其中5例发作与EEG放电不完全同步。16例头颅MRI均显示一侧大脑半球或局部皮层萎缩,出现皮层萎缩时的病程为5个月～3年4个月。16例对抗癫癎药疗效均不佳,大剂量丙种球蛋白或皮质类固醇可使部分患儿癫癎发作短期减少。6例行功能性半球切除者,4例发作停止,偏瘫及认知功能逐渐改善。结论RS的主要临床特征为难治性部分性癫癎、进行性偏瘫和认知倒退。EEG特点为背景双侧不对称慢波,发作间期放电可为一侧性或双侧性,临床发作同期与EEG放电可无明确相关性。头颅影像学特点为逐渐进展的一侧半球萎缩。该病药物治疗难以控制癫癎发作,功能性半球切除可改善发作并防止进一步脑功能恶化。     

病灶多变的家族性部分性癫(癎)家系的临床研究

目的 探讨病灶多变的家族性部分性癫(FPEVF)家系的临床特征.方法 收集2008-2010年就诊于本院小儿神经专科门诊的2个FPEVF家系资料,建立家系系谱图,对先证者家系成员的临床特征、脑电图(EEG)、头颅MRI等进行总结分析.结果 1.FPEVF 2个家系共48名成员,存活44名,受累患者达21例(1例死亡).男12例,女9例;平均发病年龄5岁,起病年龄:家系A:1～7岁,家系B:2～10岁.2.发作前无明显诱发因素,白天夜间均有发作,临床表现为单纯部分性发作10例,复杂部分性发作6例,局灶继发全面性发作3例,亚临床发作2例,其中1例伴热性惊厥史.3.EEG检查19例,均有样放电,表现为频发尖波、棘波、尖慢波或棘慢波,其中起源于颞区、额颞区各5例,额区、额顶区各4例,颞枕区1例.4.存活患者20例的神经系统检查均正常,1例双侧海马异常,余MRI均正常.5.自行缓解6例,2例先证者经丙戊酸钠、托吡酯治疗,发作和EEG均有明显改善.结论 1.FPEVF是一种少见的家族性部分性癫综合征,呈常染色体不完全显性遗传,昼夜均可发作,临床多见部分性发作.2.具有明显的表型异质性和遗传异质性,临床易误诊为其他家族性部分性癫,家族史调查是诊断FPEVF的关键.3.不同家系成员脑电图部分性样放电起源于不同脑区,多见于额颞区,且大脑结构无异常.     

Benign familial infantile convulsions: a clinical study of seven Dutch families.

Benign familial infantile convulsions (BFIC) is a recently identified partial epilepsy syndrome with onset between 3 and 12 months of age. We describe the clinical characteristics and outcome of 43 patients with BFIC from six Dutch families and one Dutch-Canadian family and the encountered difficulties in classifying the syndrome. Four families had a pure BFIC phenotype; in two families BFIC was accompanied by paroxysmal kinesigenic dyskinesias; in one family BFIC was associated with later onset focal epilepsy in older generations. Onset of seizures was between 6 weeks and 10 months, and seizures remitted before the age of 3 years in all patients with BFIC. In all, 29 (67%) of the 43 patients had been treated with anti-epileptic drugs for a certain period of time. BFIC is often not recognized as (hereditary) epilepsy by the treating physician. Seizures often remit shortly after the start of anti-epileptic drugs but, because of the benign course of the syndrome and the spontaneous remission of seizures, patients with low seizure frequency do not necessarily have to be treated. If prescribed, anti-epileptic drugs can probably be withdrawn after 1 or 2 years of seizure freedom.     

轻度胃肠炎伴良性婴幼儿惊厥的临床研究(英文)

目的：认识轻度胃肠炎伴良性婴幼儿惊厥(BICE)的临床特点,做到正确诊断和合理治疗。方法：对年龄在3～36个月的轻度胃肠炎伴惊厥的患儿进行临床观察和出院后18个月以上的随访。结果：12例诊断为BICE,发病年龄(16.0±6.5)个月,6例(50%)冬季发病,9例(75%)3d内发生惊厥,为全身性或部分性发作,7例(58%)有早期频繁发作,一次病程中惊厥平均发作2.1次。发作间期EEG、脑影像学、血生化及脑脊液检查正常。惊厥停止后未行抗痫治疗,3例(25%)复发,复发≤2次,病程最长为8个月。所有病例精神运动发育正常。结论：本组BICE具有以下特点:1～2岁高发,冬季多发,无家族史;无热惊厥多出现于病程的早期,全身性或部分性发作,早期频繁发作多见;血电解质、血生化、脑脊液、脑影像学和发作间期脑电图正常;部分病例可复发,病程少于1年,预后好。惊厥停止后不推荐应用抗癫痫药。     

奥卡西平联合其他抗癫(癎)药治疗儿童癫(癎)的疗效及安全性

目的 探讨奥卡西平(OXC)联合用药治疗儿童癫(癎)的疗效、剂量及药物安全性.方法 收集在本院神经内科门诊就诊的41例癫(癎)患儿.男23例,女18例;就诊年龄6个月～l5岁,平均7.70岁;病程15 d-5 a,平均9个月;随访时间6个月～6a,平均2a6个月.简单局限性发作(SPS)3例,复杂局限性发作(CPS)8例,局限性发作继发全面性发作(sGTCS) 14例,SPS和sGTCS 2种类型均有12例,CPS和sGTCS均有4例.根据应用OXC的先后顺序分为A组与B组:A组为开始应用一种非OXC抗癫(癎)药治疗,因疗效不好,在原用药剂量不变的情况下加用OXC;B组为首选OXC治疗后因仍有发作加用其他抗癫(癎)药物.回顾性分析41例OXC联合用药治疗癫(癎)患儿的临床资料,评估其疗效、药物不良反应及患儿的耐受性.结果 A组29例,经OXC添加治疗完全控制发作者占48.3％,发作频率减少≥50％(即总有效率)82.7％;OXC所用剂量为(26.70±6.75) ng·kg-1·d-1.其中12例成功转换为OXC单药治疗,10例单药治疗无发作.B组OXC加小剂量硝西泮药物组合治疗,发作频率减少均≥50％,疗效最高.对发作类型比较,OXC对各种局限性发作类型发作频率减少均≥50％.本研究中8例(19.5％)出现OXC相关药物不良反应,依次为困倦3例,皮疹2例,视物不清、无症状血钠减低和膝关节痛各l例,分别经缓慢加量、略减量或未进行任何处理,上述症状自行消失.结论 OXC联合用药治疗癫(癎)疗效高而稳定,耐药性小,安全性高,是适合于长期应用的新型高效抗癫(癎)药物之一.     

婴儿严重肌阵挛癫痫的临床特征和基因突变分析

目的 探讨婴儿严重肌阵挛癫癎(SMEI)的临床特点和基因诊断.方法分析13例SMEI患儿的临床和脑电图(EEG)特点及钠离子通道SCN1A基因突变筛查结果.结果男10例,女3例.8例有热性惊厥和癫痫家族史.惊厥起病年龄2～9个月,平均5.6个月.首次发作为热性惊厥9例.13例在病程早期均以反复发热诱发的全面性或一侧性阵挛或强直阵挛发作为主,其中8例有热性惊厥持续状态.出现无热惊厥的年龄为2～21个月.病程中均出现多种发作类型.发作均有热敏感的特点,诱发因素包括发热、洗热水澡和疫苗接种.起病后出现智力发育落后11例.共济失调5例,锥体束征阳性2例.EEG在1岁前多数正常,1岁后出现全导或局灶放电.头颅MBI检查异常2例.13例均应用多种抗癫痫药治疗,发作均未完全控制.卡马西平和拉莫三嗪使部分患儿发作加重.10例发现有SCN1A基因突变.结论 SMEI的临床特点是:1岁以内起病,首次发作常为热性惊厥;1岁以后出现多种发作形式和智力发育落后;发作具有热敏感的特点;EEG早期正常,以后出现全导或局灶放电.筛查SCN1A基因突变有助于早期明确诊断,指导选择抗癫癎药物.     

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。