Pearl Phillip L
Continuum (Minneap Minn). 2018 Feb;24(1, Child Neurology):186-209. doi: 10.1212/CON.0000000000000568.
Epilepsy syndromes are an important clinical construct in pediatric epilepsy, as they encompass recognizable patterns seen in patients with epilepsies, whether of the more benign variety or associated with encephalopathy.
Syndromes may be organized by age of onset: neonatal, infantile, childhood, or adolescent. The assignment of a syndrome has specific implications for diagnosis, management, and prognostication. The 2010 revised classification of the epilepsies by the International League Against Epilepsy preserved the syndrome approach, while progress in genetics continues to advance our understanding of the pathophysiology and overlap of the epilepsy syndromes.
Given that mutations of the same gene may cause both encephalopathic and relatively benign epilepsies, an understanding of the pediatric epilepsy syndromes remains vital to patient care.
癫痫综合征是小儿癫痫中一个重要的临床概念,因为它们涵盖了癫痫患者中可识别的模式,无论病情是较为良性还是与脑病相关。
综合征可按发病年龄进行分类:新生儿期、婴儿期、儿童期或青少年期。综合征的确定对诊断、治疗和预后具有特定意义。2010年国际抗癫痫联盟修订的癫痫分类保留了综合征分类方法,同时遗传学的进展不断加深我们对癫痫综合征病理生理学及重叠情况的理解。
鉴于同一基因的突变可能导致脑病性癫痫和相对良性的癫痫,了解小儿癫痫综合征对患者的治疗仍然至关重要。