EEG-fMRI in myoclonic astatic epilepsy (Doose syndrome)
EEG-fMRI in myoclonic astatic epilepsy (Doose syndrome)
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DOI：
10.1212/wnl.0000000000000359
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发表时间：
2014-04
期刊：
影响因子：
9.9
通讯作者：
F. Moeller;K. Groening;J. Moehring;H. Muhle;S. Wolff;O. Jansen;U. Stephani;M. Siniatchkin
中科院分区：
文献类型：
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作者：
F. Moeller;K. Groening;J. Moehring;H. Muhle;S. Wolff;O. Jansen;U. Stephani;M. Siniatchkin
目的：明确肌阵挛无张力性癫痫(MAE)患者全身性棘波放电(GSW)的神经元网络。方法：对13例MAE患儿同时进行EEG-fMRI检查。对每个患者的个体GSW相关血氧水平依赖(BOLD)信号变化进行分析。进行一组分析，以确定所有患者的常见症状特定的血流动力学变化。结果：在11例患者中记录到GSW，均显示GSW相关的BOLD信号改变。丘脑(所有患者)、运动前皮质(6例)和壳核(6例)均被激活。在默认模式区域(7例患者)发现失活。该小组的分析证实了丘脑、运动前皮质、壳核和小脑的激活以及默认模式网络中的失活。结论：除了在特发性全身性癫痫中常见的丘脑皮质网络外，MAE患者的GSW还具有与运动功能相关的脑结构的大胆信号改变的特征。这一结果与动物研究表明，躯体感觉皮层、壳核和小脑参与了肌阵挛发作的发生。这些结构的受累可能导致在MAE中观察到的典型的肌阵挛痉挛发作符号学。
Objective: To identify neuronal networks underlying generalized spike and wave discharges (GSW) in myoclonic astatic epilepsy (MAE). Methods: Simultaneous EEG-fMRI recordings were performed in 13 children with MAE. Individual GSW-associated blood oxygenation level–dependent (BOLD) signal changes were analyzed in every patient. A group analysis was performed to determine common syndrome-specific hemodynamic changes across all patients. Results: GSW were recorded in 11 patients, all showing GSW-associated BOLD signal changes. Activation was detected in the thalamus (all patients), premotor cortex (6 patients), and putamen (6 patients). Deactivation was found in the default mode areas (7 patients). The group analysis confirmed activations in the thalamus, premotor cortex, putamen, and cerebellum and deactivations in the default mode network. Conclusions: In addition to the thalamocortical network, which is commonly found in idiopathic generalized epilepsies, GSW in patients with MAE are characterized by BOLD signal changes in brain structures associated with motor function. The results are in line with animal studies demonstrating that somatosensory cortex, putamen, and cerebellum are involved in the generation of myoclonic seizures. The involvement of these structures might predispose to the typical seizure semiology of myoclonic jerks observed in MAE.