Three children with profound mental retardation and intractable seizures died at ages 10 months, 3 years, and 7 years, respectively. Complete examination of their brains showed generalized cortical dysplasia, without any major malformation of the external gyral pattern. The neuropathologic features of cortical dysplasia include abnormally thickened cortex with indistinct demarcation of the gray-white matter junction. In many areas, the cortex contained increased numbers of large neurons with disordered cortical lamination. Heterotopic neurons were scattered throughout the white matter with decreased myelination of the underlying white matter. To our knowledge, these cases represent the first fully detailed neuropathologic study of diffuse cortical dysplasia--a newly recognized entity of abnormal neuronal migration.

译文

:三名患有严重智力障碍和顽固性癫痫发作的儿童分别在10个月,3岁和7岁时死亡。对他们的大脑进行全面检查,发现其大脑皮层发育异常,没有外部回旋模式的任何重大畸形。皮质发育异常的神经病理学特征包括皮质异常增厚,灰白质交界处分界不清。在许多地区,皮层中皮质层合异常的大型神经元数量增加。异位神经元散布在整个白质中,而基础白质的髓鞘减少。据我们所知,这些病例代表了弥漫性皮层发育不良的新的,完整的神经病理学研究,该研究是新发现的异常神经元迁移的实体。

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