婴儿发作综合征:当发作突然出现时
Marta Conti1, Sara Matricardi2, Ludovica M Piscitello1
1Child Neurology, Epilepsy and Movement Disorders, Bambino Gesù, IRCCS Children's Hospital, Full Member of European Reference Network EpiCARE, Rome, Italy.
在以前正常的儿童中,婴儿发作综合征 (IESS) 在及时治疗的情况下显示出有利的结果. 早期的发作自由和正常发育预测了这个IESS亚组的良好长期预后.
科学领域:
- 神经学 神经学
- 儿科神经学 儿科神经学
- 发病学 (Epileptology) 是一个专业的学科.
背景情况:
- 婴儿发作综合征 (IESS) 可以在以前正常发育的儿童中出现.
- 在这种特定的IESS子组中确定结果的预测因素对于临床管理至关重要.
研究的目的:
- 评估IESS在正常发育前的儿童中的电临床特征.
- 描述临床结果并确定有利预后的独立预测因素.
主要方法:
- 来自意大利两个中心的43名同质IESS患者的回顾性分析,正常发育前发病.
- 排除具有危险因素,其他类型的发作,大脑异常或已知的遗传疾病的患者.
- 标准化EEG评估,使用BASED评分进行间触模式的评估.
主要成果:
- 大多数患者 (65.11%) 在发病时表现出轻微的行为变化.
- 在EEG上,性脑病 (EE) 的流行率为 (清醒率:69.76%,睡眠率:81.40%).
- 实现了迅速的自由 (83.72%在15天内),主要是使用ACTH储存;所有患者在六个月内都没有和EE.
- 在最后一次随访时,81.40%的人具有正常的认知功能,而其他人则有语言障碍.
结论:
- 一个独特的IESS患者小组具有正常的先前发展,表现出迅速的治疗反应和有利的长期结果.
- 缺乏严重的神经发育影响和持续的自由性是这一子组的特征.
- 在IESS发病前的正常发育和早期治疗反应是良好的预后的关键预测因素.
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