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[伴有脑电图异常和癫痫的非言语听觉失认症;一例罕见的Landau-Kleffner综合征]

[Non-verbal auditory agnosia with EEG abnormalities and epilepsy; an unusual case of Landau-Kleffner syndrome].

作者信息

Koeda T, Kohno Y

机构信息

Department of Pediatrics, Kitakyushu City Sogo Ryoiku Center, Fukuoka.

出版信息

No To Hattatsu. 1992 May;24(3):262-7.

PMID:1375480
Abstract

A case of unusual Landau-Kleffner syndrome was reported. She was an 8 year-old girl and showed non-verbal agnosia, diffuse EEG abnormalities and convulsions. Her responses to both verbal and non-verbal sounds remained inconsistent and unstable. When a continuous spike-wave complexes on EEG was detected, she paid no attention to any sound in spite of her fair consciousness. Auditory brainstem response and magnetic resonance imaging of her brain were normal. Auditory agnosia was correlated well with EEG abnormalities, and valproic acid and clonazepam were effective for EEG improvement. After the EEG improvement, clinical responses to sounds recovered well; firstly she could pay attention to sounds and then she could distinguish between verbal and non-verbal sounds. Finally, she could speak a few words after the learning letters.

摘要

报告了一例罕见的Landau-Kleffner综合征病例。她是一名8岁女孩,表现出非言语失认症、弥漫性脑电图异常和惊厥。她对言语和非言语声音的反应一直不一致且不稳定。当脑电图检测到持续性棘慢复合波时,尽管她意识清醒,但对任何声音都不予理会。她的听觉脑干反应和脑部磁共振成像均正常。听觉失认症与脑电图异常密切相关,丙戊酸和氯硝西泮对改善脑电图有效。脑电图改善后,对声音的临床反应恢复良好;首先她能注意到声音,然后能区分言语和非言语声音。最后,在学习字母后她能说出几个单词。

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