West 综合征伴婴儿痉挛和迟发性癫痫性痉挛的临床研究。

Clinical study of West syndrome with PS and late-onset epileptic spasms.

机构信息

Department of Neurology, West China Hospital, Sichuan University, Chengdu 610041, Sichuan, People's Republic of China.

出版信息

Epilepsy Res. 2010 Mar;89(1):82-8. doi: 10.1016/j.eplepsyres.2009.10.010. Epub 2009 Nov 24.

DOI:10.1016/j.eplepsyres.2009.10.010

PMID:19939629

Abstract

OBJECTIVE

Some patients resemble West syndrome (WS) but they do not fit the typical pattern completely. The aim of this study was to determine whether these conditions represent variant patterns of classic WS or reveal specific features related with other pathogenesis.

METHODS

We investigated the electro-clinical features, neuroimaging and pathophysiological mechanism in 85 children of WS with or without partial seizure (PS) and late-onset epileptic spasms (LOES), followed up for 2-5 years.

RESULTS

WS with PS (21 cases), LOES (11 cases), and WS without PS (52 cases) were brought into this study, and their age onset was 5.7, 20.68 and 6.26 months, respectively. All cases shared some features of classic WS, but asymmetric spasms and EEG, frequency and duration of spasms, hemiparesis, neuroimaging findings of the former two groups were more common than those of the latter group. LOES exhibited prolonged tonic components and/or PS, associated with focal or multifocal spike and sharp-wave discharges of fronto-temporal or centro-temporal predominance and neuroimaging features distinct from those of WS with or without FS.

CONCLUSION

WS with PS and LOES seem to represent the specific types of age-related epileptic encephalopathy due to their electro-clinical and neuroimaging features distinct from the classic WS. The former exhibited the focal originated generalized epilepsy, while LOES might represent an intermediate condition between WS and LGS.

摘要

目的

有些患者表现类似于 West 综合征（WS），但不完全符合典型模式。本研究旨在确定这些情况是否代表经典 WS 的变异模式，或揭示与其他发病机制相关的特定特征。

方法

我们研究了 85 例伴有或不伴有部分性发作（PS）和迟发性癫痫性痉挛（LOES）的 WS 患儿的电临床特征、神经影像学和病理生理学机制，随访 2-5 年。

结果

将 WS 伴 PS（21 例）、LOES（11 例）和 WS 不伴 PS（52 例）纳入本研究，其发病年龄分别为 5.7、20.68 和 6.26 个月。所有病例均具有经典 WS 的一些特征，但不对称痉挛和 EEG、痉挛的频率和持续时间、偏瘫在前两组中更为常见。LOES 表现为痉挛持续时间延长和/或 PS，伴有额颞部或中央颞部局灶性或多灶性棘波和尖波放电，神经影像学特征与 WS 伴或不伴 FS 不同。