Dept. of Neuropathology, University of Bonn Medical Center, Bonn, Germany.
Epilepsy Res. 2013 Nov;107(1-2):190-4. doi: 10.1016/j.eplepsyres.2013.07.006. Epub 2013 Aug 5.
The distinction of isolated malformations and combined lesions constitutes a major novelty in the 2011 consensus classification of the International League against Epilepsy (ILAE) for focal cortical dysplasias (FCD). Cortical lamination abnormalities together with vascular lesions are subsumed as FCD IIIc. Little is known regarding frequency and etiology of this entity. Here, we systematically evaluated biopsy specimens from 72 drug-refractory epilepsy patients with cerebral cavernous malformations (CCM) regarding presence of associated FCD. Due to a non-perpendicular orientation of the cortical structures or absence of sufficient cortical tissue adjacent to the vascular lesion 25 samples were not classifiable. In the remaining 47 cases FCD IIIc was rare (4.3%), but significantly increased in patients with multiple CCM (28.6%; p<0.05 vs. single CCM). Association of FCD IIIc with multiple CCM may argue against FCD IIIc as an acquired lesion.
孤立性畸形和合并性病变的区别是国际抗癫痫联盟（ILAE）2011 年局灶性皮质发育不良（FCD）共识分类的主要新内容。皮质层状结构异常伴血管病变被归入 FCD IIIc。对于这种病变的频率和病因知之甚少。在这里，我们系统地评估了 72 例药物难治性伴有脑海绵状血管畸形（CCM）的癫痫患者的活检标本，以确定是否存在相关的 FCD。由于皮质结构的非垂直取向或紧邻血管病变的皮质组织不足，25 个样本无法分类。在剩余的 47 例中，FCD IIIc 很少见（4.3%），但在多发性 CCM 患者中显著增加（28.6%；p<0.05 与单发 CCM 相比）。FCD IIIc 与多发性 CCM 的关联可能表明 FCD IIIc 不是获得性病变。
