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[无高峰节律紊乱的癫痫性痉挛。1例新病例报告及文献复习]

[Epileptic spasms without hypsarrhythmia. A new case report and review of the literature].

作者信息

Navas-Sánchez P, Martínez-Antón J, Bauzano-Poley E

机构信息

Hospital Universitario Carlos Haya, Malaga, España.

出版信息

Rev Neurol. 2010 Nov 16;51(10):592-6.

Abstract

INTRODUCTION

The term 'epileptic spasm' must be used to refer to a type of seizure that is typically found in childhood. Clinically, it is expressed as brief axial contractions, in flexion, extension or mixed, which can be symmetrical or asymmetrical and usually appear in clusters. The best-known epileptic syndrome associated with the appearance of grouped spasms is West's syndrome. They may also appear in other generalised epilepsies, epilepsies with periodic spasms or even seizures that are similar to grouped spasms in certain partial epilepsies.

CASE REPORT

A 6-month-old girl with paroxysmal episodes of cluster spasms, for whom no pattern of hypsarrhythmia was observed in the electroencephalogram. The physical examination, neuroimaging and metabolic studies did not offer any pathological findings of interest. The psychomotor development of the patient prior to the onset of the seizures was normal. Following treatment with several different antiepileptic drug regimes, finally control over the seizures was accomplished with a combination of valproic acid and vigabatrine.

CONCLUSIONS

There are cases like this, with cluster spasms, which fall within the age bracket at which West's syndrome typically occurs and which, nevertheless, do not present the electroencephalographic characteristics of hypsarrhythmia (neither typical nor atypical) or neuropsychological impairment. In these patients it is not clear whether we are dealing with a variant related with West's syndrome or not, since two of the three criteria required for its diagnosis are not fulfilled.

摘要

引言

术语“癫痫性痉挛”必须用于指代一种典型见于儿童期的癫痫发作类型。临床上,其表现为短暂的轴向收缩,呈屈曲、伸展或混合形式,可为对称或不对称,且通常成串出现。与成串痉挛出现相关的最知名癫痫综合征是韦斯特综合征。它们也可能出现在其他全身性癫痫、伴有周期性痉挛的癫痫中,甚至在某些局灶性癫痫中出现类似于成串痉挛的发作。

病例报告

一名6个月大的女童出现成串痉挛的阵发性发作,脑电图未观察到高峰失律模式。体格检查、神经影像学和代谢研究均未发现任何有意义的病理结果。该患者在癫痫发作前的精神运动发育正常。在使用几种不同的抗癫痫药物治疗方案后,最终通过丙戊酸和氨己烯酸联合使用实现了癫痫发作的控制。

结论

存在这样一些病例,有成串痉挛,处于韦斯特综合征通常发生的年龄范围内,但却没有高峰失律的脑电图特征(既非典型也非非典型)或神经心理损害。在这些患者中,尚不清楚我们面对的是否是与韦斯特综合征相关的变异型,因为其诊断所需的三个标准中有两个未满足。

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