Mytilinaios Dimitrios G, Tsamis Konstantinos I, Njau Samuel N, Polyzoides Konstantinos, Baloyannis Stavros J
Laboratory of Neuropathology, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Dev Neurorehabil. 2010 Feb;13(1):64-7. doi: 10.3109/17518420903236254.
Dandy-Walker malformation is a congenital malformation that is characterized by hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle and enlargement of the posterior fossa. The aim of the study is to describe the morphological and morphometric alterations of neurons (in various regions of the brain) in a case of Dandy-Walker Variant.
By Golgi method and routine techniques, the alterations in the brain of a 23 years old case of Dandy-Walker Variant who died accidentally were studied. Furthermore, a detailed morphometric analysis of neuronal parameters and a statistical comparison with an age-matched control were applied.
In the cerebellum the morphological study, apart from gliosis, revealed reduction of the size of cell body and poverty of dendritic arborization with loss of dendritic branches and spines. In the cerebral cortex and the hippocampus the most prominent findings were the tortuous configuration of the apical dendrites of the pyramidal neurons, the focal swellings of the axons and the dendrites and significant gliosis.
Although the gross anatomical examination of the brain demonstrated only mild neurodevelopmental anomalies (except the key features of the syndrome), the microscopic examination revealed significant morphological alterations of neurons and dendrites.
丹迪-沃克畸形是一种先天性畸形，其特征为小脑蚓部发育不全、第四脑室囊性扩张和后颅窝增大。本研究的目的是描述一例丹迪-沃克变异型病例中（大脑不同区域的）神经元的形态学和形态计量学改变。
采用高尔基方法和常规技术，对一名23岁意外死亡的丹迪-沃克变异型病例的大脑改变进行研究。此外，对神经元参数进行了详细的形态计量学分析，并与年龄匹配的对照组进行了统计学比较。
在小脑的形态学研究中，除了胶质增生外，还发现细胞体大小减小、树突分支减少以及树突分支和棘突缺失。在大脑皮质和海马中，最显著的发现是锥体细胞顶树突的扭曲形态、轴突和树突的局灶性肿胀以及明显的胶质增生。
尽管大脑的大体解剖检查仅显示轻度神经发育异常（除该综合征的关键特征外），但显微镜检查显示神经元和树突有明显的形态学改变。
