Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
J Clin Neurophysiol. 2012 Oct;29(5):425-40. doi: 10.1097/WNP.0b013e31826bd943.
Benign epilepsy with centrotemporal spikes, early-onset childhood occipital epilepsy (Panayiotopoulos syndrome [PS]) and late-onset childhood occipital epilepsy (Gastaut type [LOCE-G]) are the principal pediatric focal epilepsy syndromes. They share major common characteristics: the appearance and resolution of electroclinical features are age related, there is a strong genetic predisposition, the clinical course is often mild with infrequent and easy to control seizures, interictal epileptiform activity is disproportionately abundant when compared with the clinical correlate, and tends to potentiate and generalize during sleep. In this review, we outline the relevant pathophysiology underlying this electroclinical spectrum. Then, the initial description of individual syndromes is followed by a summary of overlapping features and intermediate presentations that question the boundaries between these entities and provide the basis for the concept of a childhood seizure susceptibility syndrome. Additionally, we outline the main features of the related epileptic encephalopathies. An outlook on potential future lines of research completes this review.
良性癫痫伴中央颞区棘波、儿童早发性枕叶癫痫(Panayiotopoulos 综合征[PS])和儿童晚发性枕叶癫痫(Gastaut 型[LOCE-G])是主要的儿童局灶性癫痫综合征。它们具有主要的共同特征:电临床特征的出现和消退与年龄有关,存在强烈的遗传易感性,临床病程通常较轻,发作频率低且易于控制,发作间期痫样放电与临床相关性不成比例地丰富,并在睡眠期间倾向于增强和泛化。在这篇综述中,我们概述了这一电临床谱的相关病理生理学。然后,在描述个别综合征的初始描述之后,总结了重叠的特征和中间表现,这些特征和表现对这些实体之间的界限提出了质疑,并为儿童癫痫易感性综合征的概念提供了依据。此外,我们概述了相关癫痫性脑病的主要特征。对潜在未来研究方向的展望完成了这篇综述。