Stark T A, McKinney A M, Palmer C S, Maisel R H, Truwit C L
Department of Radiology, University of Minnesota Medical School & Hennepin County Medical Center, Minneapolis, MN 55415, USA.
AJNR Am J Neuroradiol. 2009 Feb;30(2):434-6. doi: 10.3174/ajnr.A1283. Epub 2008 Oct 22.
Limited studies of brain MR imaging findings in Usher syndrome have reported atrophy with enlarged subarachnoid spaces. However, the specific appearance of the subarachnoid spaces surrounding the cranial nerves has not yet been described. Herein we describe the skull base MR imaging findings in an adult with Usher syndrome. Multiple cranial nerve exits were enlarged to the point of causing cephaloceles with bony remodeling. A combination of uncommon findings in this rare disorder raises the question of an etiologic association.
关于Usher综合征脑部磁共振成像（MR）表现的研究有限，报告显示存在蛛网膜下腔增宽伴萎缩。然而，围绕颅神经的蛛网膜下腔的具体表现尚未见描述。在此，我们描述一名成年Usher综合征患者的颅底MR成像表现。多个颅神经出口扩大，导致脑膨出并伴有骨质重塑。这种罕见疾病中多种不常见表现引发了病因关联的问题。
