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促肾上腺皮质激素治疗Lennox综合征:一项临床与脑电图研究。

Treatment of the Lennox syndrome with ACTH: a clinical and electroencephalographic study.

作者信息

Yamatogi Y, Ohtsuka Y, Ishida T, Ichiba N, Ishida S, Miyake S, Oka E, Ohtahara S

出版信息

Brain Dev. 1979;1(4):267-76. doi: 10.1016/s0387-7604(79)80041-8.

DOI:10.1016/s0387-7604(79)80041-8
PMID:233337
Abstract

In 45 cases of Lennox syndrome treated with ACTH, the immediate and long-term effects and the various factors affecting them were investigated by a follow-up study. 1) Regarding the immediate effect, 23 (51.1%) of the 45 cases became "seizure free" for over 10 days. 2) As to the long-term prognosis of these 23 cases, 10 cases relapsed into Lennox syndrome within 6 months and in the remaining 13 cases, seizures were suppressed for over 6 months; out of these 13, seizure relapse was observed in eight cases from 9 months to 7 years later, and the other five cases followed a very favorable course without relapse. 3) The favorable factors related to the effect of ACTH for Lennox syndrome are: a) age at ACTH treatment: up to 4 years old, b) time lag between onset of Lennox syndrome and initiation of ACTH treatment: the shorter the better; at least within 1 year, preferably within 3 months, c) presumptive causes and underlying diseases: idiopathic cases are best, d) mental defects before treatment: the slighter the better, e) seizure patterns: without tonic seizures, and f) EEG findings: disorganized diffuse slow spike-waves without asymmetry. 4) It is desirable to continue the ACTH treatment as long as possible, with the goal of the disappearance of seizure discharges, or at least the disappearance of diffuse seizure discharges.

摘要

对45例接受促肾上腺皮质激素(ACTH)治疗的Lennox综合征患者进行随访研究,调查其即刻和长期疗效以及影响疗效的各种因素。1)关于即刻疗效,45例中有23例(51.1%)“无癫痫发作”超过10天。2)对于这23例患者的长期预后,10例在6个月内复发为Lennox综合征,其余13例癫痫发作被抑制超过6个月;在这13例中,8例在9个月至7年后出现癫痫复发,另外5例病情发展良好未复发。3)与ACTH治疗Lennox综合征疗效相关的有利因素包括:a)ACTH治疗时的年龄:4岁及以下,b)Lennox综合征发病与开始ACTH治疗之间的时间间隔:越短越好;至少在1年内,最好在3个月内,c)推测病因和基础疾病:特发性病例最佳,d)治疗前的智力缺陷:越轻微越好,e)癫痫发作类型:无强直发作,f)脑电图表现:无不对称的紊乱弥漫性慢棘波。4)尽可能长时间持续ACTH治疗是可取的,目标是癫痫放电消失,或至少弥漫性癫痫放电消失。

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