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早期婴儿良性肌阵挛或良性非癫痫性婴儿痉挛症。

Benign myoclonus of early infancy or benign non-epileptic infantile spasms.

作者信息

Dravet C, Giraud N, Bureau M, Roger J, Gobbi G, Dalla Bernardina B

出版信息

Neuropediatrics. 1986 Feb;17(1):33-8. doi: 10.1055/s-2008-1052496.

Abstract

Lombroso and Fejerman (1983) described a syndrome which shares with West syndrome the clinical features of flexion spasm with onset in early infancy. However the syndrome differs from West syndrome in the absence of mental and psycho-motor involvement and having a normal EEG during wakefulness and sleep. We report four cases of these benign spasms of early infancy with polygraphic recording of the spasms providing the following information: the spasms are clinically similar to those often observed in West syndrome; namely they are characterized by a short (2-4 sec.) tonic contraction, there are no significant changes of the EEG concomitant to the spasm, series of spasms can occur not only during the daytime but also during sleep and immediately after awakening. Differential diagnosis is discussed with West syndrome and benign myoclonic epilepsy in infancy and with non-epileptic syndromes. The authors propose to name this syndrome: Benign Non-Epileptic Infantile Spasms.

摘要

龙勃罗梭和费耶曼(1983年)描述了一种综合征,它与韦斯特综合征有共同的临床特征,即婴儿早期出现的屈曲痉挛。然而,该综合征与韦斯特综合征的不同之处在于,不存在智力和精神运动方面的问题,且清醒和睡眠期间脑电图正常。我们报告了4例婴儿早期良性痉挛病例,并对痉挛进行了多导记录,提供了以下信息:这些痉挛在临床上与韦斯特综合征中常见的痉挛相似;也就是说,它们的特点是短暂(2 - 4秒)的强直性收缩,痉挛发作时脑电图无明显变化,一系列痉挛不仅可在白天发作,也可在睡眠期间及刚醒来后发作。文中讨论了与韦斯特综合征、婴儿良性肌阵挛癫痫以及非癫痫性综合征的鉴别诊断。作者建议将此综合征命名为:婴儿良性非癫痫性痉挛。

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