Abstract
Dravet综合征（DS）为婴儿期起病的药物难治性癫痫综合征，约80%的患儿由SCN1A基因杂合变异所致。DS的临床特点包括1岁内常以热性惊厥起病；随后出现多种形式的无热惊厥；发作具有热敏感的特点；易反复发生癫痫持续状态；多有智力、运动发育落后。DS治疗的一线药物包括丙戊酸、托吡酯和氯巴占，应避免使用钠离子通道阻断剂。DS患儿由于发热容易诱发癫痫持续状态，少数患儿可出现急性脑病，遗留神经系统后遗症甚至死亡，应引起高度重视。.
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Schedule a callMore From: Zhonghua er ke za zhi = Chinese journal of pediatrics
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