Tassi L, Pasquier B, Minotti L, Garbelli R, Kahane P, Benabid A L, Battaglia G, Munari C, Spreafico R
Centro Regionale Chirurgia dell'Epilessia C. Munari, Ospedale Niguarda Ca' Granda, Milano, Italy.
Epilepsia. 2001 Sep;42(9):1112-23. doi: 10.1046/j.1528-1157.2001.00501.x.
The aim of this study was to correlate the electroclinical and radiologic data with the neuropathologic findings and surgical outcome in epileptic patients with epilepsy and Taylor's focal cortical dysplasia (TFCD) and to characterize further the abnormal intermediate filaments expression in the balloon cell present in the peculiar dysplasia.
We retrospectively selected 13 TFCD patients who underwent surgery for intractable epilepsy with the aim of removing the magnetic resonance (MR)-detectable lesion and/or the epileptogenic zone defined by stereoelectroencephalographic recordings. The surgical specimens were analyzed by means of routine neuropathologic and immunocytochemical studies. Antisera against different intermediate filaments also were used in serial adjacent sections to evaluate their coexpression in balloon cells.
Histopathologic abnormalities typical of TFCD were found not only within the MR-visible lesions but also in most of the epileptogenic zones with no MR signal alterations. Furthermore, the MR-visible lesions contained a high proportion of cells with an abnormal expression of intermediate filament proteins. After a long follow-up, 10 of the patients are now seizure free.
Our findings indicate that highly epileptogenic zones may correspond to tissue alterations not revealed by neuroimaging. Furthermore, the immunocytochemical data show that the dysplastic tissue detected by MR contained high concentrations of cells filled with abnormal intermediate filaments. The detected colocalization of neuronal and glial markers in balloon cells indicates a failure of cellular commitment during development.
本研究旨在将癫痫合并泰勒局灶性皮质发育不良(TFCD)患者的电临床和放射学数据与神经病理学发现及手术结果相关联,并进一步描述存在于特殊发育异常中的气球样细胞中异常中间丝的表达特征。
我们回顾性选择了13例因顽固性癫痫接受手术的TFCD患者,目的是切除磁共振(MR)可检测到的病变和/或立体脑电图记录所确定的致痫区。手术标本通过常规神经病理学和免疫细胞化学研究进行分析。针对不同中间丝的抗血清也用于连续相邻切片,以评估它们在气球样细胞中的共表达。
TFCD典型的组织病理学异常不仅在MR可见病变内发现,而且在大多数无MR信号改变的致痫区内也发现。此外,MR可见病变中含有高比例的中间丝蛋白表达异常的细胞。经过长期随访,10例患者目前已无癫痫发作。
我们的研究结果表明,高度致痫区可能对应于神经影像学未显示的组织改变。此外,免疫细胞化学数据显示,MR检测到的发育异常组织中含有高浓度的充满异常中间丝的细胞。在气球样细胞中检测到的神经元和胶质细胞标志物的共定位表明发育过程中细胞分化失败。