Smith M T, Huntington H W
Neurology. 1977 Mar;27(3):246-51. doi: 10.1212/wnl.27.3.246.
The malformation of inverse cerebellum and occipital encephalocele is situated morphologically between the Arnold-Chiari and Dandy-Walker malformations. In the three reported cases, hydrocephalus was not present, concomitant malformations of the lamina terminalis were present in two, and polymicrogyria was found in all three. The primary defect in the malformation is a complex occipital encephalocele composed of miniature hemispheres connected to the brain stem by an extension of the midbrain tectum. The cerebellar folia extend ventrally and cover the basilar artery. We propose that the encephalocele arises through the processes of overgrowth and dysraphism and thus falls into the organogenetic malformations of Yakovlev. Supporting this theory is the consistent observation of the duplicate hemispheres in the encephalocele and the hydromyelia in the examined spinal cords.
逆小脑和枕部脑膨出畸形在形态学上介于阿诺德-基亚里畸形和丹迪-沃克畸形之间。在报道的3例病例中，均未出现脑积水，2例伴有终板畸形，3例均发现多小脑回。该畸形的主要缺陷是一个复杂的枕部脑膨出，由通过中脑顶盖延伸与脑干相连的微型半球组成。小脑小叶向腹侧延伸并覆盖基底动脉。我们提出脑膨出是通过过度生长和神经管闭合不全的过程产生的，因此属于雅科夫列夫的器官发生畸形。支持这一理论的是在脑膨出中一致观察到的重复半球以及在所检查脊髓中的脊髓积水。
