Garbelli R, Munari C, De Biasi S, Vitellaro-Zuccarello L, Galli C, Bramerio M, Mai R, Battaglia G, Spreafico R
Department of Experimental Neurophysiology and Epileptology, National Neurological Institute C. Besta, Milan, Italy.
Brain Pathol. 1999 Jul;9(3):445-61. doi: 10.1111/j.1750-3639.1999.tb00534.x.
In the present report we describe the neuropathological characteristics of tissue surgically resected from three patients affected by intractable epilepsy secondary to cortical dysplasia. Common features, suggestive of a focal cortical dysplasia of Taylor, were observed in all specimens. Immunocytochemical procedures were performed using neuronal and glial markers and the sections were observed at light traditional and confocal microscopes. This part of the investigation pointed out: 1. cortical laminar disruption; 2. very large neurons displaying a pyramidal or round shape; 3. ballooned cells; 4. decrease of calcium binding proteins immunoreactivity; 5. abnormal nets of parvalbumin- and glutamic acid decarboxylase-positive puncta around giant neurons but not around ballooned cells. Ultrastructural investigation on the same material provided evidence of a high concentration of neurofilaments in giant neurons and of glial intermediate filaments in ballooned cells. In addition, immunolabeled GABAergic terminals clustered around giant neurons were not found to establish synapses on their cell bodies. The present data, derived from a limited sample of patients but showing very consistent features, suggest that in Taylor's type of cortical dysplasia a disturbance of migratory events could be paralleled by a disruption of cell differentiation and maturation and by an impairment of synaptogenesis. This latter mechanism seemed to affect especially the inhibitory elements, and could account for the hyperexcitability of this tissue and thus for the high epileptogenicity of Taylor's dysplasia.
在本报告中，我们描述了从三名患有皮质发育异常继发难治性癫痫的患者手术切除组织的神经病理学特征。在所有标本中均观察到提示泰勒局灶性皮质发育异常的共同特征。使用神经元和胶质细胞标志物进行免疫细胞化学检测，并在传统光学显微镜和共聚焦显微镜下观察切片。这部分研究指出：1. 皮质层状破坏；2. 呈现金字塔形或圆形的非常大的神经元；3. 气球样细胞；4. 钙结合蛋白免疫反应性降低；5. 在巨型神经元周围但不在气球样细胞周围有异常的小白蛋白和谷氨酸脱羧酶阳性小点网络。对相同材料的超微结构研究提供了证据，表明巨型神经元中神经丝高度集中，气球样细胞中有胶质中间丝。此外，未发现聚集在巨型神经元周围的免疫标记GABA能终末在其细胞体上形成突触。本数据来自有限的患者样本，但显示出非常一致的特征，表明在泰勒型皮质发育异常中，迁移事件的紊乱可能与细胞分化和成熟的破坏以及突触发生的损害同时存在。后一种机制似乎特别影响抑制性元件，并可能解释该组织的过度兴奋性，从而解释泰勒发育异常的高致痫性。
