Department of Neuroscience, Pediatric Neurology Unit, Tor Vergata University of Rome, Viale Oxford 81, 00133 Rome, Italy.
Seizure. 2012 Jan;21(1):70-4. doi: 10.1016/j.seizure.2011.09.005. Epub 2011 Oct 14.
Childhood absence epilepsy (CAE) and benign childhood epilepsy with centrotemporal spikes (BCECTS), or benign rolandic epilepsy (BRE), are the most common forms of childhood epilepsy. CAE and BCECTS are well-known and clearly defined syndromes; although they are strongly dissimilar in terms of their pathophysiology, these functional epileptic disturbances share many features such as similar age at onset, overall good prognosis, and inheritance factors. Few reports are available on the concomitance of CAE and BCECTS in the same patients or the later occurrence of generalized epilepsy in patients with a history of partial epilepsy. In most cases described in the literature, absence seizures always started after the onset of benign focal epilepsy but the contrary has never occurred yet. We describe two patients affected by idiopathic generalized epileptic syndrome with typical absences, who experienced BCECTS after remission of seizures and normalization of EEG recordings. While the coexistence of different seizure types within an epileptic syndrome is not uncommon, the occurrence of childhood absence and BCECTS in the same child appears to be extremely rare, and this extraordinary event supports the hypothesis that CAE and BCECTS are two distinct epileptic conditions. However, recent interesting observations in animal models suggest that BCECTS and CAE could be pathophysiologically related and that genetic links could play a large role.
儿童失神癫痫(CAE)和良性儿童期中央颞区棘波灶癫痫(BECTS),也称为良性罗兰多区癫痫(BRE),是儿童癫痫中最常见的两种类型。CAE 和 BECTS 是众所周知且明确界定的综合征;尽管它们在病理生理学方面存在明显差异,但这些功能性癫痫障碍具有许多共同特征,如发病年龄相似、总体预后良好以及遗传因素。在同一患者中 CAE 和 BECTS 共存或既往部分性癫痫患者后来发生全身性癫痫的情况鲜有报道。在文献中描述的大多数病例中,失神发作总是在良性局灶性癫痫发作后开始,但反之尚未发生过。我们描述了两名患有特发性全面性癫痫综合征的患者,他们在癫痫发作缓解和脑电图记录正常后出现了 BECTS。虽然癫痫综合征中存在不同类型的癫痫发作并不罕见,但同一名儿童同时患有 CAE 和 BECTS 的情况似乎极为罕见,这种特殊事件支持了 CAE 和 BECTS 是两种不同癫痫状态的假说。然而,最近在动物模型中的有趣观察结果表明,BECTS 和 CAE 在病理生理学上可能相关,遗传因素可能起重要作用。