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使用硫噻嗪作为肌阵挛失神癫痫患儿的附加治疗:35 例患者研究。

Use of sulthiame as add-on therapy in children with myoclonic atonic epilepsy: A study of 35 patients.

机构信息

Hospital de Pediatría "Prof. Dr. Juan P Garrahan", Buenos Aires, Argentina.

Hospital de Pediatría "Prof. Dr. Juan P Garrahan", Buenos Aires, Argentina.

出版信息

Epilepsy Behav. 2022 Jun;131(Pt A):108702. doi: 10.1016/j.yebeh.2022.108702. Epub 2022 May 5.

Abstract

PURPOSE

The aim of this retrospective study was to evaluate efficacy and tolerability of sulthiame (STM) as add-on treatment in 35 patients with myoclonic atonic epilepsy (MAE) resistant to other antiseizure medications (ASMs) and/or non-pharmacological treatment.

METHODS

Patients were selected according to the diagnostic definition of MAE and were resistant to at least four previous to ASM, alone or in combination. Neurologic examinations, brain magnetic resonance imaging, and repeated prolonged electroencephalography (EEG) or video-EEG studies as well as neurometabolic studies were performed in all cases. Genetic studies were performed in 15 patients. Data on school achievements and/or neuropsychological evaluations were obtained over a mean follow-up of 30 months. Sulthiame was added in doses ranging from 10 to 30 mg/kg/day. Efficacy was assessed by comparing seizure frequency before and after initiating STM therapy.

RESULTS

Twenty-one of 35 patients (60%) who received STM as add-on therapy had a greater than 50% seizure decrease after a mean follow-up of 30 months. Complete seizure freedom was achieved in two patients (5.8%). The remaining 14 patients (40%) had a 25-50% seizure reduction. Adverse effects, consisting of hyperpnea and dyspnea, decreased appetite, nausea, drowsiness, headache, and irritability, were observed in 11 (31.4%). The adverse effects were mild and transient in all cases. Discontinuation of STM was not necessary.

CONCLUSION

Add-on STM led to a more than 50% seizure reduction in 21 of 35 patients with MAE with only mild or moderate adverse effects.

摘要

目的

本回顾性研究旨在评估氨己烯酸(STM)作为附加治疗在 35 例对其他抗癫痫药物(ASM)和/或非药物治疗耐药的肌阵挛失神癫痫(MAE)患者中的疗效和耐受性。

方法

根据 MAE 的诊断定义选择患者,并对至少四种之前的 ASM 单独或联合治疗耐药。所有患者均进行神经检查、脑磁共振成像、重复长时间脑电图(EEG)或视频脑电图研究以及神经代谢研究。15 例患者进行了基因研究。平均随访 30 个月后获得了关于学业成绩和/或神经心理学评估的数据。氨己烯酸的剂量范围为 10 至 30mg/kg/天。通过比较开始 STM 治疗前后的癫痫发作频率来评估疗效。

结果

在接受 STM 作为附加治疗的 35 例患者中,21 例(60%)在平均 30 个月的随访后癫痫发作减少了 50%以上。2 例患者(5.8%)完全无癫痫发作。其余 14 例患者(40%)癫痫发作减少了 25-50%。在 11 例(31.4%)患者中观察到包括过度通气和呼吸困难、食欲减退、恶心、嗜睡、头痛和易怒在内的不良反应。所有情况下的不良反应均为轻度和短暂的。不需要停止使用 STM。

结论

在 35 例 MAE 患者中,21 例患者添加 STM 后癫痫发作减少了 50%以上,仅有轻度或中度不良反应。

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