Hallervorden-Spatz病的临床特征和头颅MRI表现  

Clinical and imaging presentations of Hallervorden-Spatz disease

作　　者：葛剑青[1] 管青山[1] 

机构地区：[1]南京医科大学附属脑科医院神经病科,210029

出　　处：《江苏医药》2011年第24期2926-2927,共2页Jiangsu Medical Journal

摘　　要：目的分析Hallervorden-Spatz病(HSD)的临床及影像学表现。方法从万方数据库中搜集国内文献报道及本科收治的HSD共27例资料,分析其临床表现及影像学特征。结果 HSD以锥体外系症状及智能障碍为主要临床表现,头颅MRI表现为双侧对称性苍白球的信号异常,T2加权成像显示两侧苍白球呈低信号,中心见斑片状高信号影,即典型的"虎眼征"。结论 HSD的MRI有特征性的形态学表现,结合临床资料是可以确诊的。Objective To analyse clinical and imaging appearances of Hallervorden-Spatz disease（HSD）.Methods The clinical data of CT and MRI images from 27 HSD cases were reviewed retrospectively,of whom 26 cases were reported in China and 1 case was treated in our hospital.Results HSD was characterised by extrapyramidal and cognitive disorders,while the brain MRI of HSD showed abnormalities confined to the pallidus,which presented a diffuse low signal intensity in T2-weighted images and a central area of high intensity（eye-of-the-tiger sign）.Conclusion HSD has unique changes on MRI,by which combined with clinical data the diagnosis of HSD can be made.

关 键 词：HALLERVORDEN-SPATZ病 苍白球 磁共振成像 

分 类 号：R741[医药卫生—神经病学与精神病学]