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氨己烯酸治疗结节性硬化症患儿痉挛:文献综述

Vigabatrin in the treatment of infantile spasms in tuberous sclerosis: literature review.

作者信息

Hancock E, Osborne J P

机构信息

Bath Unit for Research into Paediatrics, The Children's Centre, Royal United Hospital, United Kingdom.

出版信息

J Child Neurol. 1999 Feb;14(2):71-4. doi: 10.1177/088307389901400201.

Abstract

The purpose of this report is to review the efficacy and safety of vigabatrin in the treatment of infantile spasms in infants suffering from tuberous sclerosis complex. We reviewed all studies published in the English-language literature investigating the use of vigabatrin in the treatment of infantile spasms. Ten studies gave results for the efficacy of vigabatrin in infantile spasms for infants both with and without underlying diagnoses of tuberous sclerosis. Of the 313 patients without tuberous sclerosis complex, 170 (54%) had complete cessation of their infantile spasms; of the 77 patients with tuberous sclerosis complex, 73 (95%) had complete cessation of their seizures. We conclude that vigabatrin should be considered as first-line monotherapy for the treatment of infantile spasms in infants with either a confirmed diagnosis of tuberous sclerosis or those at high risk, ie, those with a first-degree relative with tuberous sclerosis complex. Paradoxically, in those without tuberous sclerosis complex, vigabatrin might be less efficacious than suggested by studies including patients with tuberous sclerosis complex.

摘要

本报告旨在回顾氨己烯酸治疗患有结节性硬化症的婴儿痉挛症的疗效和安全性。我们查阅了英文文献中所有关于氨己烯酸治疗婴儿痉挛症的研究。十项研究给出了氨己烯酸对患有和未患有潜在结节性硬化症诊断的婴儿痉挛症的疗效结果。在313例无结节性硬化症的患者中,170例(54%)婴儿痉挛症完全停止;在77例患有结节性硬化症的患者中,73例(95%)癫痫发作完全停止。我们得出结论,对于确诊为结节性硬化症的婴儿或高危婴儿(即有结节性硬化症一级亲属的婴儿),氨己烯酸应被视为治疗婴儿痉挛症的一线单一疗法。矛盾的是,在无结节性硬化症的患者中,氨己烯酸的疗效可能不如纳入了结节性硬化症患者的研究所显示的那样好。

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