Department of Neurosurgery, Epilepsy Research Center of PLA, Xinqiao Hospital, Army Medical University, Chongqing, China.
Department of Neurology, Xinqiao Hospital, Army Medical University, Chongqing, China.
Brain Pathol. 2022 Sep;32(5):e13065. doi: 10.1111/bpa.13065. Epub 2022 Mar 8.
Emergence of dysmorphic neurons is the primary pathology in focal cortical dysplasia (FCD) associated pediatric intractable epilepsy; however, the etiologies related to the development and function of dysmorphic neurons are not fully understood. Our previous studies revealed that the expression of vascular endothelial growth factor-C (VEGF-C) and corresponding receptors VEGFR-2, VEGFR-3 was increased in the epileptic lesions of patients with tuberous sclerosis complex or mesial temporal lobe epilepsy. Here, we showed that the expression of VEGF-C, VEGFR-2, and VEGFR-3 was increased at both mRNA and protein levels in patients with cortical lesions of type I, IIa, and IIb FCD. The immunoreactivity of VEGF-C, VEGFR-2 and VEGFR-3 was located in the micro-columnar neurons in FCD type I lesions, dysplastic neurons (DNs) in FCD type IIa lesions, balloon cells (BCs) and astrocytes in FCD type IIb lesions. Additionally, the amplitude of evoked-EPSCs (eEPSC) mediated by NMDA receptor, the ratio of NMDA receptor- and AMPA receptor-mediated eEPSC were increased in the dysmorphic neurons of FCD rats established by prenatal X-ray radiation. Furthermore, NMDA receptor mediated current in dysmorphic neurons was further potentiated by exogenous administration of VEGF-C, however, could be antagonized by ki8751, the blocker of VEGFR-2. These results suggest that VEGF-C system participate in the pathogenesis of cortical lesions in patients with FCD in association with modulating NMDA receptor-mediated currents.
形态异常神经元的出现是与儿童难治性癫痫相关的局灶性皮质发育不良(FCD)的主要病理学改变;然而,与形态异常神经元的发育和功能相关的病因尚不完全清楚。我们之前的研究表明,血管内皮生长因子-C(VEGF-C)及其相应受体 VEGFR-2、VEGFR-3 的表达在结节性硬化症或内侧颞叶癫痫患者的癫痫病灶中增加。在这里,我们发现在 I 型、IIa 型和 IIb 型 FCD 的皮质病变患者中,VEGF-C、VEGFR-2 和 VEGFR-3 的表达在 mRNA 和蛋白质水平上均增加。VEGF-C、VEGFR-2 和 VEGFR-3 的免疫反应性位于 FCD Ⅰ型病变的微柱状神经元、FCD Ⅱa 型病变的发育不良神经元(DN)、FCD Ⅱb 型病变的气球细胞(BC)和星形胶质细胞中。此外,在产前 X 射线辐射建立的 FCD 大鼠的发育不良神经元中,NMDA 受体介导的诱发-EPSC(eEPSC)的振幅、NMDA 受体和 AMPA 受体介导的 eEPSC 的比值增加。此外,外源性给予 VEGF-C 可进一步增强发育不良神经元中的 NMDA 受体介导电流,而 VEGFR-2 的阻断剂 ki8751 可拮抗 NMDA 受体介导电流。这些结果表明,VEGF-C 系统参与了 FCD 患者皮质病变的发病机制,同时调节 NMDA 受体介导的电流。