Watanabe M, Tanaka R, Takeda N, Ikuta F, Oyanagi K
Department of Neurosurgery, Niigata University, Japan.
Neuroradiology. 1990;32(3):237-40. doi: 10.1007/BF00589120.
A case of focal pachygyria with an unusual vascular anomaly is reported. Preoperative magnetic resonance imaging demonstrated few and broad gyri, and an abnormally thickened cortex of the right frontal lobe. In addition, T2-weighted imaging showed a high intensity lesion beneath the thickened cortex. In the pachygyric cortex, the peripheral portions of the arteries were tortuous and irregularly dilated, and prominent deep medullary veins were found draining into the subependymal veins. Histological examination revealed a decreased number of neurons with no tendency towards lamination, and degenerative changes with gliosis in the white matter. These findings suggest that the etiology of this anomaly may be gradual perfusion failure restricted to the territory of the anomalous vessels through the period of neuronal migration to the post-migratory, perinatal stage.
报告了一例伴有异常血管异常的局灶性巨脑回病例。术前磁共振成像显示脑回少且宽，右侧额叶皮质异常增厚。此外，T2加权成像显示增厚皮质下方有高强度病变。在巨脑回皮质中，动脉外周部分迂曲且不规则扩张，发现有突出的深髓静脉汇入室管膜下静脉。组织学检查显示神经元数量减少，无分层倾向，白质有退行性改变伴胶质增生。这些发现表明，这种异常的病因可能是在神经元迁移至迁移后围产期阶段的整个过程中，仅限于异常血管区域的逐渐灌注衰竭。
