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年龄依赖性癫痫性脑病:一项纵向研究。

Age-dependent epileptic encephalopathy: a longitudinal study.

作者信息

Yamatogi Y, Ohtahara S

出版信息

Folia Psychiatr Neurol Jpn. 1981;35(3):321-32. doi: 10.1111/j.1440-1819.1981.tb00233.x.

DOI:10.1111/j.1440-1819.1981.tb00233.x
PMID:7327468
Abstract

A longitudinal clinico-electroencephalographic study was conducted on 484 cases of age-dependent epileptic encephalopathy; early-infantile epileptic encephalopathy with suppression-burst (Ohtahara) (EIEE), the West and Lennox syndromes. 1) The three types had the same kind of etiologic heterogeneity. 2) An evolutional change with age was noted from EIEE to the West and from the West to the Lennox syndromes, with the change from suppression-burst to hypsarhythmia, and from hypsarhythmia to the diffuse slow spike-waves. 3) The core ictal EEG pattern of tonic spasms was the desynchronization in EIEE and the West syndrome, with the rapid synchronization and recruiting rhythm in the Lennox syndrome. In the same cases, the changes from desynchronization to hypersynchronization were observed with age. These facts suggest that the three syndromes are based on the same pathophysiology, strongly related to the subcortical mechanism, and that their clinico-electrical manifestations are modified by the degree of brain maturation.

摘要

对484例年龄依赖性癫痫性脑病患者进行了一项纵向临床脑电图研究;早期婴儿癫痫性脑病伴暴发抑制(大田原综合征)(EIEE)、韦斯特综合征和伦诺克斯综合征。1) 这三种类型具有相同类型的病因异质性。2) 随着年龄的增长,观察到从EIEE到韦斯特综合征以及从韦斯特综合征到伦诺克斯综合征的演变变化,伴随着从暴发抑制到高度节律失调,以及从高度节律失调到弥漫性慢棘波的变化。3) 强直性痉挛的核心发作期脑电图模式在EIEE和韦斯特综合征中为去同步化,在伦诺克斯综合征中为快速同步化和募集节律。在同一病例中,随着年龄的增长,观察到从去同步化到高度同步化的变化。这些事实表明,这三种综合征基于相同的病理生理学,与皮质下机制密切相关,并且它们的临床电表现会因脑成熟程度而改变。

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