晚期婴儿脑病变:一个独特的发育和脑病变综合征
Shawn Kacker1, Chalongchai Phitsanuwong1, Audrey Oetomo1
1The University of Chicago, The University of Chicago Medical Center, Chicago, Illinois, USA.
Epileptic disorders : international epilepsy journal with videotape
|December 15, 2023
概括
晚期婴儿性脑病变 (LIEE) 是一种独特的综合征,在两岁以下的婴儿中,其特点是特定的类型和EEG发现. 这种疾病经常表现为耐药性和发育迟缓,使其与其他性脑病不同.
科学领域:
- 神经学 神经学
- 儿科症 儿科症
- 临床神经科学 临床神经科学
背景情况:
- 发育性和性脑病变 (DEE) 包含各种综合征,但一些婴儿呈现出不符合既定类别的独特特征,如EIDEE,IESS或LGS.
- 这些婴儿的一小部分表现出暗示一种独特疾病的特征,称为晚期婴儿性脑病变 (LIEE).
研究的目的:
- 定义和描述婴儿晚期脑病变 (LIEE) 的独特电临床特征.
- 为了区分LIEE与其他公认的婴儿脑病综合征.
主要方法:
- 在2022年7月至2023年5月期间,芝加哥大学康默儿童医院对儿科患者进行了回顾性分析.
- 根据电临床特征确定了符合LIEE标准的30名患者.
主要成果:
- 晚期婴儿脑病变 (LIEE) 在3.5%的评估患者中被发现,发病平均在6.8个月,LIEE特征在18.1个月.
- 常见的病因包括遗传/代谢障碍 (50%) 和结构异常 (43%). 主要的发作类型是肌性-增强性 (70%) 和性-增强性 (50%).
- 脑电图发现显示了断续续,电,扩散减速和多焦点形放电. 几乎所有患者都患有耐药性和显著的发育迟缓.
结论:
- 晚期婴儿性脑病变 (LIEE) 是一种独特的电临床综合征,通常在生命的第二年表现出来.
- 关键特征包括肌性-增强性和性-增强性发作,不同于低节律的特定EEG模式,以及与症状性的关联.
- 在发育性和性脑病变的范围内,LIEE代表了一个独特的实体,需要认可才能进行适当的管理.
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