A distinct variant of focal cortical dysplasia type I characterised by magnetic resonance imaging and neuropathological examination in children with severe epilepsies
A distinct variant of focal cortical dysplasia type I characterised by magnetic resonance imaging and neuropathological examination in children with severe epilepsies
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DOI：
10.1684/epd.2010.0321
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发表时间：
2010-09-01
影响因子：
2.3
通讯作者：
Holthausen, Hans
中科院分区：
文献类型：
--
作者：
Bluemcke, Ingmar;Pieper, Tom;Holthausen, Hans
局灶性皮质发育不良（FCD）具有广泛的临床病理学谱。FCD被认为与致癫痫状态直接相关，尽管通过手术切除控制癫痫发作是可变的。这尤其适用于患有多叶FCD、患有严重癫痫和精神发育迟滞的幼儿。在此，我们进行了术前可用的数据和显微镜检查切除皮质标本的比较分析，以进一步阐明FCD的病理形态学谱。对18名患有严重耐药性癫痫的幼儿（平均年龄7.6岁）进行了连续系列的多叶切除术，这些儿童在广泛的术前表面/侵入性视频EEG监测术中皮质电描记术（iECoG）以及高分辨率MRI后患有严重耐药性癫痫。在所有情况下，系统的神经病理学检查的手术标本进行了建筑异常和细胞密度测量。这些组织形态学数据与体积MRI分析进行了比较。组织学检查显示，所有病例中神经元密度增加与皮质厚度减少和神经元微柱丰富相关。有趣的是，在我们的患者系列中的16名儿童中，受影响的大脑半球相对于非致癫痫对侧明显较小。总之，发育不全的新皮质和柱状结构紊乱点受损的皮质发育，并出现不同的FCD I亚型的儿童患有严重癫痫和精神发育迟滞。
Focal Cortical Dysplasias (FCDs) present with a large clinicopathological spectrum. FCDs are believed to relate directly to an epileptogenic condition, although seizure control by surgical resection is variable. This applies in particular to young children with multilobar FCDs, suffering from severe epilepsies and psychomotor retardation. Herein, we performed a comparative analysis of presurgically available data and microscopic inspection of resected cortical specimens to further characterise the pathomorphological spectrum of FCD. Multilobar resection procedures were performed in a consecutive series of 18 young children (mean 7.6 years) with severe pharmaco-resistant epilepsies following extensive presurgical surface-/invasive video-EEG monitoring intraoperative electro-corticography (iECoG), as well as high resolution MRI. In all cases, systematic neuropathological examination of surgical specimens was performed with respect to architectural abnormalities and cell density measurements. These histomorphological data were compared with volumetric MRI analysis. Histopathological examination revealed increased neuronal densities correlating with decreased cortical thickness and abundance of neuronal microcolumns in all cases. Intriguingly, the affected cerebral hemisphere was significantly smaller, relative to the non-epileptogenic contralateral side, in 16 children of our patient series. In conclusion, hypoplastic neocortex and columnar architectural disorganisation point to compromised cortical development, and appear as distinct FCD I subtype in children suffering from severe epilepsies and psychomotor retardation.