Della Giustina E, Goffinet A M, Landrieu P, Lyon G
Acta Neuropathol. 1981;55(1):23-8. doi: 10.1007/BF00691526.
Characteristic neuronal heterotopias in two cases of Zellweger's cerebro-hepato-renal disease were studied with the Golgi method. In the corona radiata, heterotopias consist of large fields of small or medium-sized radial pyramids, and of dense clusters containing larger, randomly oriented pyramidal cells and multipolar neurons, some of which resemble granule cells. The latter type of heterotopia could result from a focal destructive process at a relatively early stage of neuronal migration. In the cerebellar white matter, heterotopic masses contain Purkinje cells and possibly Golgi neurons but no granule or basket cells. The mispositioned Purkinje cells resemble the subcortical and intragranular Purkinje cells of the reeler mutant mouse and those of the weaver mutant. The morphology of neurons in the abnormally convoluted olivary nucleus is normal.
采用高尔基方法对两例齐-韦氏脑肝肾综合征患者特征性的神经元异位进行了研究。在放射冠中，异位由小或中等大小的放射状锥体组成的大片区域以及包含较大的、随机取向的锥体细胞和多极神经元的密集簇组成，其中一些类似于颗粒细胞。后一种类型的异位可能是由于神经元迁移相对早期的局灶性破坏过程所致。在小脑白质中，异位团块包含浦肯野细胞以及可能的高尔基神经元，但没有颗粒细胞或篮状细胞。位置异常的浦肯野细胞类似于reeler突变小鼠和weaver突变小鼠的皮质下和颗粒内浦肯野细胞。异常卷曲的橄榄核中神经元的形态是正常的。
