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[氨己烯酸单药治疗韦斯特综合征]

[Monotherapy with vigabatrin in the treatment of West's syndrome].

作者信息

Rufo M, Santiago C, Castro E, Ocaña O

机构信息

Servicio de Neuropediatría, Hospital Infantil Universitario Virgen del Rocío, Sevilla, España.

出版信息

Rev Neurol. 1997 Sep;25(145):1365-8.

PMID:9377291
Abstract

INTRODUCTION

Vigabatrin (VGB) was specifically synthesized to enhance inhibitory GABAergic transmission by elevating GABA levels via irreversible inhibition of GABA transaminase.

MATERIAL AND METHODS

This study was conducted to determine the efficacy of VGB introduced as monotherapy in 26 children fulfilled the criteria for diagnosis of West syndrome. Duration of follow-up was 24 months.

RESULTS

Following the introduction of VGB, 13 patients became seizure free, a special efficacy in one case of tuberous sclerosis. Relapses of infantile spasms occurred in 8 infants, generalized seizures developed in 4 infants, and partial seizures in 3, of whom 2 were eventually rendered seizure-free by increasing the dose. EEG features and age affecting the response rate. VGB has been well tolerated in children with agitation being the most commonly reported side effects, and one case required discontinuation of therapy.

CONCLUSIONS

VGB seems to be an effective an safe antiepileptic drug as primary monotherapy for West syndrome.

摘要

引言

氨己烯酸(VGB)是通过不可逆抑制γ-氨基丁酸转氨酶来提高γ-氨基丁酸水平,从而专门合成以增强抑制性γ-氨基丁酸能传递的药物。

材料与方法

本研究旨在确定在26名符合韦斯特综合征诊断标准的儿童中,将VGB作为单一疗法使用的疗效。随访时间为24个月。

结果

引入VGB后,13名患者无癫痫发作,1例结节性硬化症患者有特殊疗效。8名婴儿出现婴儿痉挛复发,4名婴儿出现全身性癫痫发作,3名婴儿出现部分性癫痫发作,其中2名最终通过增加剂量实现无癫痫发作。脑电图特征和年龄影响缓解率。VGB在儿童中耐受性良好,最常报告的副作用是激越,1例需要停药。

结论

VGB似乎是一种有效且安全的抗癫痫药物,可作为韦斯特综合征的一线单一疗法。

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