Hallervorden-Spatz病的脑代谢及影像学表现(附一例报告及文献复习)

目的 分析Hallervorden-Spatz病的脑代谢及影像学表现。方法 搜集了1例临床资料证实为Hallervorden-Spatz病的CT、MRI片，复习了近几年国内外献报告的Hallervorden-Spatz病的表现。结果 Hallervorden-Spatz病的头颅MRI表现为：双侧对称性苍白球的信号异,T1中权呈高信号，T2徊权呈低信号，且苍白球的头部呈高信号，即典型的“虎眼征”。NAA、NAA/Cr、Cho/Cr比正常分别下降25％、16％和8％。结论 Hallervorden-Spatz病的MRI有特殊性的形态学表现和脑代谢的变化，结合临床资料是可以诊断的。

Cerebral metabolism and imaging appearances of Hallervorden- Spatz disease (report of one case with literatures review)

Purpose To analyze cerebral metabolism and imaging appearances of Hallervorden - Spatz disease (HSD) . Methods CT and MRI images of one case with HSD proved by clinical data were studied with review of the literatures. Results The brain MRI of HSD was showed abnormalities confined to the pallidum, which presented a diffuse low signal intensity in T2 - weighted images, and an anteromedial area of high signal intensity ( eye - of - the - tiger sign) . Compared with the indexex of the normal brains, the NAA NAA/Cr and Cho/Cr decrease 25 % , 16% , 8% respectively. Conclusions Hallervorden- Spatz disease has special changes in MRI and cerebral metabolism. Combined with clinical data, MRI can be helpful to diagnose HSD.