围生期下丘脑出血后韦斯特综合征病例中的丛集性痴笑发作

Gelastic seizures in clusters in a case of West syndrome after perinatal hypothalamic hemorrhage.

作者信息

Kito M, Yabuta K, Kato S, Osawa M, Okuda H

机构信息

Department of Pediatrics, Minami Seikyo Hospital, Nagoya, Japan.

出版信息

J Perinat Med. 2001;29(4):357-9. doi: 10.1515/JPM.2001.051.

DOI:10.1515/JPM.2001.051

PMID:11565206

Abstract

We describe a 4-month-old Japanese infant with West syndrome with gelastic seizures in clusters. Smile developed around 3 months and gradually increased in frequency and intensity. Positron emission tomography showed hypoperfusion in bilateral hypothalamus. Interictal electroencephalogram (EEG) showed hypsarrythmia. Simultaneous video/EEG monitoring was performed. At first, a smile-like episode developed every several seconds, gradually increased to an abrupt flexion of the neck and extremities, and gradually decreased to a smile-like episode at the end. Ictal EEG revealed desynchronization. ACTH was effective. Smiles are common emotional responses in infancy. However, EEG and neuroimaging should be considered in a case of perinatal asphyxia and delayed development.

摘要

我们描述了一名4个月大的日本婴儿，患有伴有成串痴笑发作的韦斯特综合征。微笑在3个月左右出现，并逐渐在频率和强度上增加。正电子发射断层扫描显示双侧下丘脑灌注不足。发作间期脑电图（EEG）显示高峰节律紊乱。进行了同步视频/脑电图监测。起初，每隔几秒就会出现一次类似微笑的发作，逐渐发展为颈部和四肢突然屈曲，最后又逐渐减少为类似微笑的发作。发作期脑电图显示去同步化。促肾上腺皮质激素有效。微笑是婴儿期常见的情绪反应。然而，对于围产期窒息和发育迟缓的病例，应考虑进行脑电图和神经影像学检查。