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大剂量泼尼松龙治疗氨己烯酸耐药婴儿痉挛症。

Treatment with High-Dose Prednisolone in Vigabatrin-Refractory Infantile Spasms.

机构信息

Epilepsy Program, Division of Neurology, Department of Pediatrics, Hospital for Sick Children, Toronto, ON, Canada.

出版信息

Can J Neurol Sci. 2022 Jul;49(4):532-539. doi: 10.1017/cjn.2021.156. Epub 2021 Jul 2.

DOI:10.1017/cjn.2021.156
PMID:34212837
Abstract

OBJECTIVES

This research aimed to study the short-term seizure outcomes following treatment with 8 mg/kg/day prednisolone in children with infantile spasms (IS) refractory to vigabatrin. We hypothesized that high-dose prednisolone may result in similar rates of electroclinical remission when compared to published ACTH rates.

METHODS

All consecutive children with hypsarrhythmia or hypsarrhythmia variant on EEG with/without IS, who had been treated with vigabatrin as first-line anti-seizure medication (ASM) followed by high-dose oral prednisolone (8 mg/kg/day; maximum 60 mg/day) in cases who did not respond to vigabatrin, were included. Clinical and electroclinical response (ECR) at 2 weeks following initiation of treatment and adverse effects were assessed.

RESULTS

Sixty-five children were included. A genetic etiology was seen in 38.5% cases. Complete ECR was seen in 30.8% (20/65) of the patients 2 weeks after vigabatrin. Complete ECR was noted in 77.8% (35/45) of the patients, 2 weeks after prednisolone initiation in children who failed vigabatrin, and this was sustained at 6 weeks in 66.7% (30/45) patients. Prednisolone was generally well tolerated.

CONCLUSIONS

High-dose (8 mg/kg/day) oral prednisolone resulted in sustained complete ECR (at 6 weeks) in two-thirds of the children with hypsarrhythmia or hypsarrhythmia variant on EEG with/without parentally reported IS. It was generally well tolerated and found to be safe.

摘要

目的

本研究旨在研究婴儿痉挛症(IS)患儿在加巴喷丁耐药后,接受 8mg/kg/天泼尼松龙治疗后的短期癫痫发作结局。我们假设高剂量泼尼松龙可能导致与已发表的 ACTH 率相似的电临床缓解率。

方法

所有脑电图显示高度不规则性或高度不规则性变异伴/不伴 IS 的连续患儿,在加巴喷丁作为一线抗癫痫药物(ASM)治疗后,如果加巴喷丁治疗无效,给予高剂量口服泼尼松龙(8mg/kg/天;最大剂量 60mg/天)。评估治疗开始后 2 周的临床和电临床反应(ECR)和不良反应。

结果

共纳入 65 例患儿。38.5%的患儿存在遗传病因。2 周后,20/65 例(30.8%)患儿完全缓解。在加巴喷丁治疗失败的患儿中,泼尼松龙治疗后 2 周内,77.8%(35/45)患儿完全缓解,6 周时,66.7%(30/45)患儿持续缓解。泼尼松龙通常耐受良好。

结论

在脑电图显示高度不规则性或高度不规则性变异伴/不伴家长报告的 IS 的患儿中,高剂量(8mg/kg/天)口服泼尼松龙可使三分之二的患儿获得持续完全 ECR(6 周时)。它通常耐受良好且安全。

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