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特发性罗兰多癫痫综合征和特发性枕叶癫痫的谱系:从良性到致残性。

The spectrum of idiopathic Rolandic epilepsy syndromes and idiopathic occipital epilepsies: from the benign to the disabling.

作者信息

Gobbi Giuseppe, Boni Antonella, Filippini Melissa

机构信息

Child Neurology Unit, Maggiore Hospital, Bologna, Italy.

出版信息

Epilepsia. 2006;47 Suppl 2:62-6. doi: 10.1111/j.1528-1167.2006.00693.x.

Abstract

Benign childhood epilepsy with centrotemporal spikes (BCECTS) is an idiopathic focal epilepsy syndrome, the benign characteristics of which are debated; in fact, stable or intermittent specific neuropsychological deficits are frequent and may affect scholastic or adaptive abilities. Cognitive dysfunction may be related to a marked increment of the interictal epileptic discharges in NREM sleep: different degrees of neuropsychological deficits depend on the extent of awake and sleep activity and the predominant discharge localization. The age of onset and duration of atypical sleep EEG patterns may correlate with permanent cognitive impairments. These atypical clinical and EEG evolutions have been reported in the literature as different syndromes that constitute the spectrum of idiopathic rolandic epilepsy. Moreover, a clinical link between BCECTS and early-onset benign childhood occipital epilepsy has been demonstrated. According to the neurobiological approach, the spectrum of Idiopathic Rolandic Epilepsy is based on an age-dependent, idiopathic predisposition to focal seizures and sharp-wave discharges, as an expression of nonlesional cortical excitability. The involvement of a given area of the cerebral cortex may depend on the brain maturational stage.

摘要

儿童良性中央颞区棘波癫痫(BCECTS)是一种特发性局灶性癫痫综合征,其良性特征存在争议;事实上,稳定或间歇性的特定神经心理缺陷很常见,可能会影响学业或适应能力。认知功能障碍可能与非快速眼动睡眠期间发作间期癫痫放电的显著增加有关:不同程度的神经心理缺陷取决于清醒和睡眠活动的程度以及主要放电部位。发病年龄和非典型睡眠脑电图模式的持续时间可能与永久性认知障碍相关。这些非典型的临床和脑电图演变在文献中被报道为构成特发性罗兰多癫痫谱的不同综合征。此外,BCECTS与早发性儿童良性枕叶癫痫之间的临床联系已得到证实。根据神经生物学方法,特发性罗兰多癫痫谱基于年龄依赖性、特发性的局灶性癫痫发作和尖波放电易感性,这是无病变皮质兴奋性的一种表现。大脑皮质特定区域的受累可能取决于大脑成熟阶段。

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