托吡酯单药治疗全身强直-阵挛发作癫癎48例的疗效观察

目的研究托吡酯(TPM)单药治疗全身强直-阵挛发作(GTCS)癫癎的疗效和安全性.方法对48例GTCS患者(成人31例,儿童17例;特发性GTCS 32例,症状性GTCS 16例)给予TPM单药治疗20周.以基础期平均每月发作频率分别与加量期和稳定期平均每月发作频率进行比较,并观察治疗前后脑电图(EEG)的变化和药物的安全性.结果单药治疗20周后,与基础期比较,85.42%(41例)患者发作频率降低≥50%;66.67%(32例)患者发作频率降低≥75%;37.50%(18例)患者完全不发作.特发性GTCS总有效率(93.75%)高于症状性GTCS(68.75%)(P<0.01).治疗后EEG异常率(35.42%)较治疗前(64.60%)明显下降(P＜0.01).无明显不良反应.结论 TPM单药治疗GTCS的总有效率与丙戊酸钠、苯妥英钠相近,治疗后EEG有显著性改善,有良好的耐受性.TPM为GTCS单药治疗的有效药物之一.     

托吡酯单药治疗全身强直-阵挛发作癫痫48例的疗效观察

目的研究托吡酯(TPM)单药治疗全身强直-阵挛发作(GTCS)癫癎的疗效和安全性.方法对48例GTCS患者(成人31例,儿童17例;特发性GTCS 32例,症状性GTCS 16例)给予TPM单药治疗20周.以基础期平均每月发作频率分别与加量期和稳定期平均每月发作频率进行比较,并观察治疗前后脑电图(EEG)的变化和药物的安全性.结果单药治疗20周后,与基础期比较,85.42%(41例)患者发作频率降低≥50%;66.67%(32例)患者发作频率降低≥75%;37.50%(18例)患者完全不发作.特发性GTCS总有效率(93.75%)高于症状性GTCS(68.75%)(P<0.01).治疗后EEG异常率(35.42%)较治疗前(64.60%)明显下降(P＜0.01).无明显不良反应.结论 TPM单药治疗GTCS的总有效率与丙戊酸钠、苯妥英钠相近,治疗后EEG有显著性改善,有良好的耐受性.TPM为GTCS单药治疗的有效药物之一.     

妥泰单药治疗全身强直—阵挛性发作癫痫34例的疗效观察

目的 研究妥泰单药治疗全身强直－阵挛性发作癫痫的疗效和耐受性。方法 对34例全身强直－阵挛性发作患者予以妥泰单药治疗24周,以治疗前3月的平均每月癫痫发作频率与治疗后9－24周平均每月癫痫发作频率进行比较,观察其脑电图的改变和药物耐受性。结果 单药治疗24周后有20例（58．9％）癫痫发作频率较治疗前减少75％以上,其中9例（26．5％）在观察期间停止发作,8例（23．5％）发作频率减少74％－50％,总有效率为82．4％;治疗后脑电图有明显改善者占56．5％,与治疗前比较有显著性差异（P＜0．001）;无明显不良反应。结论 妥泰单药治疗全身强直－阵挛性发作的总有效率与丙戊酸钠、苯妥英钠相近,治疗后脑电图有显著性改善,有良好耐受性。为全身强直－阵挛性发作单药治疗的有效药物之一。     

托吡酯单药治疗全身强直-阵挛发作癫48例的疗效观察

目的　研究托吡酯 (TPM)单药治疗全身强直 阵挛发作 (GTCS)癫疒间 的疗效和安全性。方法　对4 8例GTCS患者 (成人 31例 ,儿童 17例 ;特发性GTCS 32例 ,症状性GTCS 16例 )给予TPM单药治疗 2 0周。以基础期平均每月发作频率分别与加量期和稳定期平均每月发作频率进行比较 ,并观察治疗前后脑电图 (EEG)的变化和药物的安全性。结果　单药治疗 2 0周后 ,与基础期比较 ,85 4 2 % (4 1例 )患者发作频率降低≥ 5 0 % ;6 6 6 7% (32例 )患者发作频率降低≥ 75 % ;37 5 0 % (18例 )患者完全不发作。特发性GTCS总有效率 (93 75 % )高于症状性GTCS(6 8 75 % ) (P <0 0 1)。治疗后EEG异常率 (35 4 2 % )较治疗前 (6 4 6 0 % )明显下降 (P <0 0 1)。无明显不良反应。结论　TPM单药治疗GTCS的总有效率与丙戊酸钠、苯妥英钠相近 ,治疗后EEG有显著性改善 ,有良好的耐受性。TPM为GTCS单药治疗的有效药物之一。     

妥泰添加治疗难治性癫(癇)的临床观察

目的 观察妥泰作为治疗难治性癫(癇)的添加剂的疗效及不良反应.方法 临床选取13例难治性癫(癇)患者,其中3例全面性强直阵挛发作,9例复杂部分性发作,1例单纯部分性发作.病程均在2年以上;经一线抗癫(癇)药物正规治疗1年以上仍频繁发作,每月发作频率均为4次以上;影响日常工作、生活;妥泰用药方法:成人用25mg开始,以后每周加25mg或50mg至发作控制或达到200mg/d;儿童1mg/(kg·d),以后每周增加1mg/(kg·d)至发作控制或达到5mg/(kg·d),以后维持观察12周,共20周.结果 经治疗5例发作减少75%以上(显效),3例发作减少50%(有效),2例发作减少25%以下(无效),1例发作增加25%以上(恶化),2例退出.总有效率61.5%.主要不良反应有厌食、恶心、呕吐、体重减轻、记忆力下降.结论 妥泰作为添加药物对难治性癫(癇)疗效较好,特别是对复杂部分性发作,有效率达77.8%.     

妥泰单药治疗部分性癫痫的疗效观察

目的 观察妥泰单药治疗部分性癫痫病人的疗效及安全性。方法 对30例部分性癫痫患者应用妥泰单药治疗20周,于治疗前观察并记录基础发作频率,剂量从25mg/d开始,每周增加25mg,共8周,达有效剂量或200mg/d后维持治疗12周,并观察癫痫发作频率变化及不良反应等。结果 发作完全控制16例（53．3％）,发作减少≥75％6例（20％）,发作减少≥50％2例（6．7％）,发作减少＜50％6例（20％）。病程短者治疗效果较好。首次接受抗癫痫药物治疗者发作完全控制比例明显高于经治过的病人。治疗过程中无严重不良反应。结论 妥泰单药治疗对控制单纯部分发作及复杂部分性发作均有良好的效果,且耐受性、安全性好。     

应用妥泰后大鼠癫痫模型血清NSE水平变化的研究

目的观察应用妥泰后发育期大鼠癫痫模型血清神经元特异性烯醇化酶(NSE)水平变化，探讨妥泰减少发育期大鼠癫痫发作引起的脑神经元损伤。方法戊四唑点燃发育期大鼠癫痫模型，随机分为正常对照组、点燃模型组、妥泰治疗组，观察大鼠血清NSE水平变化、惊厥发作频率和发作程度。结果正常对照组大鼠无惊厥发作．血清NSE水平在正常范围；癫痫模型组大鼠惊厥出现时间早，发作程度重，血清NSE水平明显高于其它两组；妥泰治疗组大鼠惊厥出现时间晚．发作程度轻，血清NSE水平略高于正常对照组而低于癫痫模型组。结论妥泰应用后血清NSE水平降低．考虑是由于其减少癫痫发作引起的神经元损伤。     

托吡酯治疗32例小儿部分性发作癫痫

目的探讨托吡酯(妥泰)添加及单一治疗小儿部分性发作癫的疗效及不良反应.方法对确诊为部分性发作癫的l9例患儿添加妥泰,13例单一治疗,均进行自身对照的开放性研究.妥泰剂量由1 mg·kg-1·d-1开始,分每日2次,每周增加1 mg·kg-1d-1,直至出现疗效或不能再耐受的不良反应.结果对上述病例妥泰治疗平均疗程(5.3±1.9)个月;32例中总的有效率占81.3%,完全控制46.9%.不良反应轻.结论妥泰对部分性发作癫单一和添加治疗,是一安全而有效的抗癫药物.     

托吡酯治疗32例小儿部分性发作癫

目的:探讨托吡酯(妥泰)添加及单一治疗小儿部分性发作癫的疗效及不良反应.方法:对确诊为部分性发作癫的l9例患儿添加妥泰,13例单一治疗,均进行自身对照的开放性研究.妥泰剂量由1 mg·kg-1·d-1开始,分每日2次,每周增加1 mg·kg-1d-1,直至出现疗效或不能再耐受的不良反应.结果:对上述病例妥泰治疗平均疗程(5.3±1.9)个月;32例中总的有效率占81.3%,完全控制46.9%.不良反应轻.结论:妥泰对部分性发作癫单一和添加治疗,是一安全而有效的抗癫药物.     

妥泰添加治疗难治性癫的临床观察

目的观察妥泰作为治疗难治性癫的添加剂的疗效及不良反应。方法临床选取13例难治性癫患者,其中3例全面性强直阵挛发作,9例复杂部分性发作,1例单纯部分性发作。病程均在2年以上;经一线抗癫药物正规治疗1年以上仍频繁发作,每月发作频率均为4次以上;影响日常工作、生活;妥泰用药方法:成人用25mg开始,以后每周加25mg或50mg至发作控制或达到200mg/d;儿童1mg/(kg.d),以后每周增加1mg/(kg.d)至发作控制或达到5mg/(kg.d),以后维持观察12周,共20周。结果经治疗5例发作减少75%以上(显效),3例发作减少50%(有效),2例发作减少25%以下(无效),1例发作增加25%以上(恶化),2例退出。总有效率61.5%。主要不良反应有厌食、恶心、呕吐、体重减轻、记忆力下降。结论妥泰作为添加药物对难治性癫疗效较好,特别是对复杂部分性发作,有效率达77.8%。     

Clinical and electroencephalographic effects of topiramate in patients with epilepsy and healthy volunteers

Although topiramate, one of the newer drugs used in treating epilepsy, is effective in reducing seizure frequency and has a wide spectrum of action, it often induces intolerable adverse effects, predominantly related to the central nervous system. Information that would help document adverse reactions early, thus allowing topiramate doses to be adjusted during the drug titration and maintenance phases, could be obtained from electroencephalogram (EEG) studies. We studied the clinical effects and EEG changes induced by topiramate in patients with refractory partial epilepsy receiving the drug as add-on therapy. To exclude effects related to the other drugs and to epilepsy itself, we compared data from patients and healthy volunteers. After receiving topiramate, 22.6% of patients became seizure free and 29% had their seizures reduced by 50% or more. Topiramate nevertheless induced noteworthy adverse reactions, the main problems being sedative and cognitive changes. Also, in healthy volunteers, a single 100-mg dose of topiramate induced mild adverse reactions, mainly affecting concentration and attention, with difficulties in speech and writing. In patients with epilepsy, the EEG changes induced by topiramate consisted of increased delta and theta activities and decreased activity in the rapid bands. This recognizable topiramate-induced EEG pattern was again evident in the healthy volunteers, in whom we also detected a significant reduction in the alpha frequency rhythm. Our results confirm that topiramate needs to be introduced gradually while patients undergo close neuropsychologic and neurophysiologic monitoring to detect adverse sedative and cognitive reactions early. The EEG correlate of these events seems to be increased activity in the slower frequency bands.     

左乙拉西坦治疗60例小儿癫痫的疗效和安全性分析

目的探讨左乙拉西坦在小儿癫痫治疗中的疗效和安全性。方法从我院2013-06—2014-06小儿神经内科专科门诊部收治的癫痫患儿中随机性抽取60例作为研究对象,采用开放性自对照随访研究方法。60例患儿均给予左乙拉西坦口服治疗,随访6~10个月,观察治疗前后癫痫发作频率变化、脑电图改变情况以及患儿治疗期间的不良反应,评价左乙拉西坦治疗小儿癫痫的疗效和安全性。结果本组患儿均成功获得随访,治疗后完全控制26例,有效20例,无效12例,加重2例,总有效率76.67%,且不同类型癫痫患儿治疗后的发作次数明显低于治疗前(P0.01)。脑电图检查痫样放电消失31例,痫样放电减少50%以上10例,痫样放电减少25%~49%9例,痫样放电无变化7例,痫样放电增加3例。本组治疗期间18例发生不良反应,不良反应发生率30.00%,主要表现为情绪异常、嗜睡乏力、皮疹等症状,给予对症治疗后均得到缓解,无严重影响治疗的不良反应。结论左乙拉西坦治疗儿童癫痫的疗效确切,不良反应少,是一种安全有效的药物。     

妥泰添加治疗Lennox—Gastaut综合征的临床观察

目的：研究妥泰辅助治疗Lennox-Gastaut综合征的安全性。方法：C地13例Lennox-Gastaut综合征患者进行妥泰进行的添加、开放性自身对照研究，以治疗前3个月的发作频度为基础，治疗后平均观察12个月（6－20月）。结果应用妥泰后，3例患者（23．1％）癫痫发作停止，6例（46．2％），癫痫发作频度减少≥50％，总有效率69．3％，妥泰对Lennox-Gastaut综合征的各型癫痫发作均有较好效果。脑电图有改善的倾,妥泰受性较好，治疗前后各项实验室检查未见有临床意义的异常改变。结论：妥泰是辅助Lennox-Gastaut综合征的一种实安全，有效的新型抗癫痫药。     

Topiramate monotherapy for childhood absence seizures: an open label pilot study.

This open-label, single-site, pilot study evaluated the therapeutic usefulness of topiramate in five children with typical absence seizures defined as loss of awareness associated with 3 Hz spike-wave activity on 24 hour ambulatory electroencephalogram (EEG). The children were previously untreated or treated unsuccessfully using other antiepileptic medication. Topiramate was initiated at a dose of 1 mg x kg (-1)day (-1), titrated twice weekly in 1 mg x kg (-1)day (-1)increments to 12 mg x kg (-1)day (-1)or individual maximally tolerated dose. Response was assessed after 6 weeks with ambulatory EEG monitoring and patient/parent record of seizure counts. All children completed the study. One previously untreated child became seizure-free on 5 mg x kg (-1)day(-1) topiramate, with no residual spike-wave activity at the final visit. In two patients, the frequency of seizures decreased in the early phases of titration, but rose to baseline levels as the topiramate dose was increased. With a reduction in dose to 6 mg x kg (-1)day (-1), seizure control improved, with substantial reductions in spike-wave activity. Seizure counts were not improved in the two remaining patients. Transient mood changes were noted in two patients. No child was withdrawn secondary to adverse effects. The results suggest that topiramate may be effective in childhood absence epilepsy. Controlled studies are now required to identify the clinically optimal dose.     

Efficacy and safety of topiramate in refractory epilepsy of childhood: long-term follow-up study

This study aimed to evaluate the long-term efficacy and safety of topiramate in treating children with drug-resistant epilepsy. A multicentric, retrospective, open-label, add-on study was undertaken of 277 children (mean age 8.4 years; range 12 months to 16 years) affected by drug-resistant epilepsy. The efficacy was rated according to the seizure types and epilepsy syndrome. After a mean period of 27.5 months of treatment (range 24-61 months), 11 patients (4%) were seizure free and 56 (20%) had more than 50% reduction in seizure frequency. The efficacy of topiramate treatment was noted in localization-related epilepsy and in generalized epilepsy. In addition, in a group of 114 patients, we compared the initial efficacy (evaluated after a mean of 9 months of follow-up) and the retention at a mean of 30 months of topiramate with regard to loss of efficacy (defined as the return to the baseline seizure frequency). Fifty-five (48%) of 114 patients were initial responders. The retention at a mean of 30 months was 23 of 114 patients (20%), 4 of whom (3.5%) were still seizure free. A loss of efficacy occurred in 32 of the 55 initial responders (58%). It was prominent in patients with generalized epilepsy, such as symptomatic infantile spasms and Lennox-Gastaut syndrome, as well as in those with Dravet syndrome. By contrast, a well-sustained topiramate efficacy was noted among patients with localization-related epilepsy. Globally, adverse events were observed in 161 patients (58%) and were mainly represented by weight loss, hyperthermia, sedation, and nervousness, which, in most cases, disappeared after slowing titration or reducing the dosage of the drug. In conclusion, the present long-term study confirms that topiramate represents a useful drug effective in a wide range of seizures and epilepsy syndromes. Moreover, preliminary data seem to suggest that the efficacy of topiramate, when evaluated in the long-term perspective, is more sustained in localization-related epilepsy than in generalized epilepsy.     

Recent developments in the diagnosis and treatment of status epilepticus

Despite increased understanding of its potential complications, status epilepticus (SE) frequently remains difficult to diagnose and treat. Advances in continuous electroencephalogram (EEG) monitoring facilitate more rapid identification of SE, even without visible clinical signs of seizures. EEG monitoring assists in modifying SE treatment and in making a prognosis. Despite the improved recognition of SE, some patients continue to seize after treatment with intravenous benzodiazepines and other medications. There are new uses for valproic acid, levetiracetam, and topiramate, and they have evidence of efficacy in treating different forms of SE. If medical treatments do not terminate SE, other interventions, such as surgery and stimulation procedures, may promote seizure cessation. This article reviews recent studies evaluating the use of continuous EEG monitoring in the setting of SE, new uses of anticonvulsants, and nonpharmacologic interventions for SE.     

Effect of γ-Vinyl GABA on Interictal Spikes and Sharp Waves in Patients with Intractable Complex Partial Seizures

The effect of gamma-vinyl GABA (GVG) on the interictal electroencephalogram (EEG) was studied in 13 patients with intractable complex partial seizures who participated in a single-blind, add-on, multicenter clinical trial of GVG. Precise operational definitions of epileptiform paroxysms were used to evaluate records before and after 3 months and 1 year of treatment with GVG. After 3 months of treatment, six patients exhibited reduction of both epileptiform paroxysms and seizure frequency, four had no change in seizure frequency nor in the EEG, and three had a reduction in seizure frequency but no concomitant reduction of epileptiform paroxysms in the EEG. Ten patients remained in the study after 1 year of treatment. In 4 patients both seizure frequency and EEG epileptiform paroxysms continued to decrease, in 1 patient both seizure frequency and number of EEG paroxysms increased, and in the remaining 3 there was no correlation between seizure frequency and EEG changes.     

Low glycemic index treatment for seizures in Angelman syndrome

Purpose: The low glycemic index treatment (LGIT) is a high fat, limited carbohydrate diet used in the treatment of epilepsy. The purpose of this study was to assess the efficacy and tolerability of the LGIT for the treatment of refractory seizures in pediatric patients with Angelman syndrome. Methods: A pediatric Angelman syndrome cohort with refractory epilepsy was treated with the LGIT and followed prospectively over 4 months. Parents recorded a daily seizure log for a minimum of 1 month prior to the start of treatment as well as throughout the LGIT trial. Electroencephalography (EEG) and neuropsychological assessments (Scales of Independent Behavior‐Revised and the Vineland Adaptive Behavior Scales‐2nd Edition were obtained for each subject at both baseline and 4‐month follow‐up time points. Clinical evaluations of subjects were completed by a neurologist and dietitian at the time of enrollment, as well as following both the first and fourth months of dietary therapy. At each time point, blood for laboratory chemistries was drawn and anthropometric measures were obtained. Key Findings: Six children (mean age 3.3 years, range 1.1–4.8) with genetically confirmed Angelman syndrome initiated the LGIT, and completed the trial with no significant adverse events. Cohort averages for indices of seizure severity were as follows: age of 1.6 years at seizure onset, 3 lifetime antiepileptic drugs tried (range 1–6), and baseline seizure frequency of 10.1 events/week (range: 0.4–30.9). All subjects had a decrease in seizure frequency on the LGIT, with five of six exhibiting >80% seizure frequency reduction. All posttrial EEG studies showed improvement and three of four children with epileptiform activity on his or her baseline EEG had no discharges present on follow‐up EEG. Developmental gains were noted by parents in all cases, although few of these neurocognitive gains were statistically significant on neuropsychological assessment. Significance: This is the first prospective study assessing the LGIT for epilepsy. Our results indicate that this dietary therapy is highly effective in treating Angelman syndrome–related seizures. The diet was well tolerated by subjects as evidenced by five of six subjects remaining on the LGIT after completion of the trial. Beyond the prospective trial window, all five subjects who remained on the diet had >90% seizure reduction after 1 year of LGIT therapy. Despite the small sample size in this prospective study, the results indicate a potentially higher degree of efficacy of the LGIT for the Angelman syndrome population than that observed in the general epilepsy population. Although this study is too small to make definitive recommendations, these results suggest that the LGIT is a promising treatment option for Angelman syndrome–related epilepsy.     

Topiramate in children with west syndrome: a retrospective multicenter evaluation of 100 patients

The aim of this study is to investigate the efficacy and tolerability of topiramate in a large number of children with West syndrome. The authors performed a retrospective, questionnaire-based data collection in specialized epilepsy units in Germany. Patients with West syndrome and hypsarrhythmia could be included if topiramate treatment had started at an age of < or =3 years. Data of 100 patients were evaluated. Nearly all patients were severely affected and had been treated with multiple antiepileptic drugs with insufficient effect. Topiramate was introduced at a median age of 11.9 months. The median starting dosage was 1.6 mg/kg body weight per day, increased to a median maximum dosage of 12.0 mg/kg. Sixty-one patients received between 1 and 3 antiepileptic drugs in addition to topiramate. The median daily dose considered by the attending physicians to be most effective regarding seizure reduction was 10 mg/kg. A significant reduction in the number of seizures per week was achieved. A total of 17.5% of patients became free of seizures, and in 47%, the seizure frequency decreased by at least 50%. Hypsarrhythmia or status-like electroencephalography patterns remitted in 18 of 83 cases. Side effects were reported in 25% of children and included mostly sedation, loss of appetite, weight loss, and metabolic acidosis. These side effects were statistically related to the number of additional antiepileptic drugs but not to the topiramate dosage. In 17% of patients, topiramate treatment was discontinued because of side effects and in a further 4% because of worsening of seizures. In 44% of patients, treatment was continued for more than 3 months. In conclusion, the data suggest that topiramate is a useful drug in treating West syndrome. However, because of the inherent limitations of the retrospective study design, future prospective controlled studies should be performed.     

Nightly oral administration of topiramate for benign childhood epilepsy with centrotemporal spikes

Objective

The objective of this study was to explore the feasibility of nightly oral administration of topiramate for treating benign childhood epilepsy with centrotemporal spikes (BECTS).

Methods

Eighty-five children with BECTS receiving topiramate treatment were randomly divided into A group (44 patients) and B group (41 patients). In A group, topiramate was orally administrated once a night, with a final dose of 2 mg/kg/day. In B group, topiramate was orally administrated twice a day, with a final dose of 4 mg/kg/day. At the end of the 12-month follow-up period, clinical efficacy, changes in electroencephalographic (EEG) activity, and adverse reactions were analyzed.

Results

There was no significant difference in overall efficacy rate, percentages of patients achieving seizure free, or changes in EEG activity between the two groups (P > 0.05). The rate of adverse reactions for A group was 9.1 %, which was significantly lower than the 29.3 % for B group (χ ² = 4.262, P < 0.05).

Conclusion

Nightly oral administration of topiramate is a feasible strategy for the treatment of BECTS, with the advantages of comparable efficacy, convenience, and fewer adverse reactions.