Georgy B A, Snow R D, Brogdon B G, Wertelecki W
Department of Radiology, University of South Alabama College of Medicine, Mobile, USA.
AJNR Am J Neuroradiol. 1998 Feb;19(2):281-3.
Our purpose was to determine the neuroradiologic findings of Marinesco-Sjögren syndrome on plain skull radiographs, CT, and MR images.
Eight patients with proved Marinesco-Sjögren syndrome (age range, 4 to 56 years) had a total of nine CT scans, seven MR imaging studies, and two plain radiographic examinations of the skull. The findings were reviewed retrospectively, with particular attention to the size of the posterior fossa and cerebellum.
All patients had hypoplastic cerebellar hemispheres and a hypoplastic vermis in a small posterior fossa. One patient had a midline posterior fossa cyst and another had agenesis of the corpus callosum.
Hypoplasia of the cerebellar hemispheres and the vermis and a small posterior fossa are the most prominent neuroradiologic findings in Marinesco-Sjögren syndrome.
我们的目的是确定在颅骨平片、CT和磁共振成像（MR）图像上，马里内斯科 - 舍格伦综合征（Marinesco-Sjögren syndrome）的神经放射学表现。
8例确诊为马里内斯科 - 舍格伦综合征的患者（年龄范围4至56岁），共进行了9次CT扫描、7次MR成像检查以及2次颅骨平片检查。对检查结果进行回顾性分析，特别关注后颅窝和小脑的大小。
所有患者均有小脑半球发育不全以及小后颅窝内的小脑蚓部发育不全。1例患者有后颅窝中线囊肿，另1例患者胼胝体发育不全。
小脑半球和蚓部发育不全以及小后颅窝是马里内斯科 - 舍格伦综合征最显著的神经放射学表现。
