Cytomegalic interneurons:: A new abnormal cell type in severe pediatric cortical dysplasia
Cytomegalic interneurons:: A new abnormal cell type in severe pediatric cortical dysplasia
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DOI：
10.1097/01.jnen.0000240473.50661.d8
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发表时间：
2007-06-01
影响因子：
3.2
通讯作者：
Cepeda, Carlos
中科院分区：
文献类型：
--
作者：
Andre, Veronique M.;Wu, Nanping;Cepeda, Carlos
泰勒型皮质发育不良（CD）的一个明确的组织病理学特征是巨细胞神经元和气球细胞的存在。大多数巨细胞神经元呈锥体状，并呈巨噬细胞能.目前的研究表明，在一个严重的儿童CD患者的子集中存在巨细胞GABA能中间神经元。采用形态学和电生理学技术检查了轻度、重度和非CD病变儿童的皮质组织样本。通过使用体外切片，在10例严重CD患者中的6例中发现了与中间神经元特征一致的巨细胞。生物胞素标记表明，巨细胞型中间神经元有更多的树突比正常出现的中间神经元。全细胞膜片钳记录显示，巨细胞中间神经元与正常出现的中间神经元相比，具有增加的膜电容和时间常数。它们还表现出细胞过度兴奋的迹象，表现为放电率增加、动作电位失活减少和自发性膜去极化的发生。单细胞逆转录聚合酶链反应和免疫组化的GABA能标记物提供了进一步的证据，这些细胞可能是巨细胞中间神经元。GABA Aergie巨细胞中间神经元在重度CD组织中的病理生理学意义尚不清楚，但它们可以抑制神经元能细胞负性锥体神经元，或有助于神经元网络的同步化和重度CD儿科患者亚组中发作活动的传播。
A defining histopathologic feature of Taylor-type cortical dysplasia (CD) is the presence of cytomegalic neurons and balloon cells. Most cytomegalic neurons appear to be pyramidal -shaped and glutamatergic. The present study demonstrates the presence of cytomegalic GABAergic interneurons in a subset of pediatric patients with severe CD. Cortical tissue samples from children with mild, severe, and non-CD pathologies were examined using morphologic and electrophysiologic techniques. By using in vitro slices, cytomegalic cells with characteristics consistent with interneurons were found in 6 of 10 patients with severe CD. Biocytin labeling demonstrated that cytomegalic interneurons had more dendrites than normal-appearing interneurons. Whole-cell patch clamp recordings showed that cytomegalic interneurons had increased membrane capacitance and time constant compared with normal-appearing interneurons. They also displayed signs of cellular hyperexcitability, evidenced by increased firing rates, decreased action potential inactivation, and the occurrence of spontaneous membrane depolarizations. Single-cell reverse transcription-polymerase chain reaction and immunohistochemistry for GABAergic markers provided further evidence that these cells were probably cytomegalic intemeurons. The pathophysiologic significance of GABAergie cytomegalic intemeurons in severe CD tissue is unknown, but they could inhibit glutamatergic cytornegalic pyramidal neurons, or contribute to the synchronization of neuronal networks and the propagation of ictal activity in a subset of pediatric patients with severe CD.