The epileptic network of Lennox-Gastaut syndrome Cortically driven and reproducible across age
The epileptic network of Lennox-Gastaut syndrome Cortically driven and reproducible across age
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DOI：
10.1212/wnl.0000000000007775
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发表时间：
2019-07-16
期刊：
影响因子：
9.9
通讯作者：
Archer, John S.
中科院分区：
文献类型：
--
作者：
Warren, Aaron E. L.;Harvey, A. Simon;Archer, John S.
目的确定Lennox-Gastaut综合征（LGS）儿童和成人发作间期全身性阵发性快速活动（GPFA）的脑区及其因果关系。10名儿童（平均年龄8.9岁）在异氟烷-瑞芬太尼麻醉下扫描，15名老年患者（平均年龄31.7岁）在无麻醉下扫描。全脑事件相关分析确定各组中GPFA相关激活。结果被用来作为先验的动态因果模型（DCM）分析比较证据不同的神经元的假设描述的启动和传播的GPFA之间的皮层，丘脑，和brainstem.ResultsA共1,045 GPFA事件进行了分析（累计持续时间1,433秒）。在儿童组和老年组中，激活发生在分布的联合皮质区，以及丘脑和脑干（p < 0.05，校正了家族错误）。在癫痫的结构性、遗传性和未知病因的个体患者中，激活是相似的，特别是在额顶叶皮层。在这两组中，DCM显示，GPFA最有可能是由前额叶皮质，传播发生第一脑干，然后从脑干到thalam.ConclusionsWe显示可重复的证据皮质驱动的过程中的癫痫网络LGS。该网络存在于综合征过程中的早期（儿童）和晚期（老年患者）以及癫痫的不同病因，表明LGS反映了共享的“二级网络”参与。一个皮质到皮质下层次的假设，GPFA迅速传播从前额皮质到脑干通过锥体外系皮质网状通路，而丘脑是从事次要的。
ObjectiveTo identify brain regions underlying interictal generalized paroxysmal fast activity (GPFA), and their causal interactions, in children and adults with Lennox-Gastaut syndrome (LGS).MethodsConcurrent scalp EEG-fMRI was performed in 2 separately analyzed patient groups with LGS: 10 children (mean age 8.9 years) scanned under isoflurane-remifentanil anesthesia and 15 older patients (mean age 31.7 years) scanned without anesthesia. Whole-brain event-related analysis determined GPFA-related activation in each group. Results were used as priors in a dynamic causal modeling (DCM) analysis comparing evidence for different neuronal hypotheses describing initiation and propagation of GPFA between cortex, thalamus, and brainstem.ResultsA total of 1,045 GPFA events were analyzed (cumulative duration 1,433 seconds). In both pediatric and older groups, activation occurred in distributed association cortical areas, as well as the thalamus and brainstem (p < 0.05, corrected for family-wise error). Activation was similar across individual patients with structural, genetic, and unknown etiologies of epilepsy, particularly in frontoparietal cortex. In both groups, DCM revealed that GPFA was most likely driven by prefrontal cortex, with propagation occurring first to the brainstem and then from brainstem to thalamus.ConclusionsWe show reproducible evidence of a cortically driven process within the epileptic network of LGS. This network is present early (in children) and late (in older patients) in the course of the syndrome and across diverse etiologies of epilepsy, suggesting that LGS reflects shared "secondary network" involvement. A cortical-to-subcortical hierarchy is postulated whereby GPFA rapidly propagates from prefrontal cortex to the brainstem via extrapyramidal corticoreticular pathways, whereas the thalamus is engaged secondarily.