Judkins A R, Porter B E, Cook N, Clancy R R, Duhaime A C, Golden J A
Division of Pathology at the Children's Hospital of Philadelphia, Department of Pathology at the University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
Epilepsy Res. 2006 Jul;70(1):49-58. doi: 10.1016/j.eplepsyres.2006.03.006. Epub 2006 May 2.
Cortical dysplasia is a frequent finding in cortical resections from children with refractory epilepsy. Diagnostic criteria and a classification scheme for cortical dysplasia has been proposed, though the relationship between specific cortical dysplasia features and their causal relationship with epilepsy is poorly understood. We reviewed 28 surgical resections from children and identified a common and easily recognized feature of cortical dysplasia: maloriented, misshapen and occasionally coarse neurofilament stained process forming a dystrophic neuritic background. The dystrophic neuritic background was associated with other features of cortical dysplasia in all 28 patients with cortical dysplasia, 26 with refractory epilepsy and 2 patients with other neurologic diagnoses. In seven children with refractory epilepsy due to other pathologic diagnosis such as vascular or glial lesions, the dystrophic neuritic background was only found in one patient with a ganglioglioma and other features suggestive of an associated cortical dysplasia. Our data indicate that a dystrophic neuritic background is a common and relatively specific neuropathologic finding in cortical dysplasia.
皮质发育异常是难治性癫痫患儿皮质切除术常见的发现。虽然已经提出了皮质发育异常的诊断标准和分类方案,但具体的皮质发育异常特征与其与癫痫的因果关系仍知之甚少。我们回顾了28例儿童的手术切除病例,发现了皮质发育异常一个常见且易于识别的特征:神经丝染色过程方向紊乱、形态异常且偶尔粗糙,形成营养不良性神经突背景。在所有28例皮质发育异常患者、26例难治性癫痫患者和2例其他神经疾病诊断患者中,营养不良性神经突背景均与皮质发育异常的其他特征相关。在7例因血管或胶质病变等其他病理诊断导致难治性癫痫的儿童中,仅在1例患有神经节细胞胶质瘤且有其他提示相关皮质发育异常特征的患者中发现了营养不良性神经突背景。我们的数据表明,营养不良性神经突背景是皮质发育异常中常见且相对特异的神经病理学发现。