Gardner E, O'Rahilly R, Prolo D
Arch Neurol. 1975 Jun;32(6):393-407. doi: 10.1001/archneur.1975.00490480059007.
Five patients with the Dandy-Walker syndrome had dysgenesis of the cerebellar vermis, cystic dilatation of the fourth ventricle, and a high position of the tentorium cerebelli. When only these features are present, the patient may lead a normal life. Additional defects usually account for the prominent clinical and pathological features of this syndrome. In this series, one patient had aqueductal stenosis, four had agenesis of the corpus callosum, two had hydrocephalus, one had cerebral abiotrophy, and one (a 72-year-old man) had no additional defects and no symptoms from his Dandy-Walker syndrome. An analysis of development and teratological considerations indicates that the Dandy-Walker and Arnold-Chiari malformations are complex disorders that have different causes and mechanisms and begin at different times in the emryonic period. The causes are still unknown.
五例患有丹迪-沃克综合征的患者存在小脑蚓部发育不全、第四脑室囊性扩张以及小脑幕高位。当仅出现这些特征时，患者可能过着正常生活。其他缺陷通常是该综合征突出的临床和病理特征的原因。在这个系列中，一名患者有导水管狭窄，四名患者有胼胝体发育不全，两名患者有脑积水，一名患者有脑发育不全，还有一名（一名72岁男性）没有其他缺陷，其丹迪-沃克综合征也没有症状。对发育和致畸因素的分析表明，丹迪-沃克畸形和阿诺德-奇亚里畸形是复杂的疾病，它们有不同的病因和机制，在胚胎期的不同时间开始。病因仍然不明。
