Comparative study of cellular and synaptic abnormalities in brain tissue samples from pediatric tuberous sclerosis complex and cortical dysplasia type II.
Comparative study of cellular and synaptic abnormalities in brain tissue samples from pediatric tuberous sclerosis complex and cortical dysplasia type II.
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DOI：
10.1111/j.1528-1167.2010.02633.x
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发表时间：
2010-07
期刊：
影响因子：
5.6
通讯作者：
Levine MS
中科院分区：
文献类型：
--
作者：
Cepeda C;André VM;Yamazaki I;Hauptman JS;Chen JY;Vinters HV;Mathern GW;Levine MS
多发性硬化症（TSC）和严重皮质发育不良（CD）或根据Palmini分类的CD II型，具有组织病理学相似性，特别是存在巨细胞神经元和气球细胞。在这项研究中，我们检查了皮质组织样本中的细胞的形态学和电生理特性，这些样本来自接受了药物耐药性癫痫手术的TSC和CD II型儿科病例。TSC和CD II型病例中正常出现的锥体神经元具有相似的被动膜特性。然而，兴奋性突触后电流（EPSC）的频率是较高的TSC神经元相比，严重的CD情况下，特别是中，大幅度突触事件的频率。此外，与严重CD病例相比，TSC的正常细胞中EPSC的上升和衰减时间较慢。气球细胞更频繁地发现TSC的情况下，而巨细胞锥体神经元更经常发生在CD II型病例。两种细胞类型在TSC和严重CD中在形态学和电生理学上相似。这些结果表明，尽管TSC和严重CD的组织病理学相似，但异常细胞的自发突触活动和拓扑分布存在细微差异。与CD II型相比，这些差异可能有助于TSC患者癫痫发生的可变机制。
Tuberous Sclerosis Complex (TSC) and severe cortical dysplasia (CD), or CD Type II according to Palmini’s classification, share histopathologic similarities, specifically the presence of cytomegalic neurons and balloon cells. In this study we examined the morphological and electrophysiological properties of cells in cortical tissue samples from pediatric cases with TSC and CD Type II that underwent surgery for pharmacoresistant epilepsy. Normal-appearing pyramidal neurons from TSC and CD Type II cases had similar passive membrane properties. However, the frequency of excitatory postsynaptic currents (EPSCs) was higher in neurons from TSC compared to severe CD cases, particularly the frequency of medium- and large-amplitude synaptic events. In addition, EPSCs rise and decay times were slower in normal cells from TSC compared to severe CD cases. Balloon cells were found more frequently in TSC cases, whereas cytomegalic pyramidal neurons occurred more often in CD Type II cases. Both cell types were similar morphologically and electrophysiologically in TSC and severe CD. These results suggest that even though the histopathology in TSC and severe CD is similar, there are subtle differences in spontaneous synaptic activity and topographic distribution of abnormal cells. These differences may contribute to variable mechanisms of epileptogenesis in patients with TSC compared with CD Type II.
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影响因子：
3
作者：
HORIKAWA, K;ARMSTRONG, WE
通讯作者：
ARMSTRONG, WE
DOI：
10.1097/01.jnen.0000240473.50661.d8
发表时间：
2007-06-01
影响因子：
3.2
作者：
Andre, Veronique M.;Wu, Nanping;Cepeda, Carlos
通讯作者：
Cepeda, Carlos
影响因子：
5.6
作者：
Mathern, GW;Cepeda, C;Levine, MS
通讯作者：
Levine, MS
影响因子：
5.6
作者：
Mathern, Gary W.;Andres, Marissa;Vinters, Harry V.
通讯作者：
Vinters, Harry V.
影响因子：
6.2
作者：
Zecevic, N
通讯作者：
Zecevic, N