Schneider S, Ott D, Sauer M
Neuroradiologie, Universität Freiburg.
Nervenarzt. 1992 Feb;63(2):105-7.
The case of a 15 year old male patient with progressive dystonia, dysarthria and dementia is presented. No diagnosis could be found on clinical grounds. Low field MRI (0.23 T) demonstrated bilateral necrosis in the globus pallidus of clinically unknown etiology. Only high field imaging (2.0 T) revealed a pattern of lesions which is supposed to be specific for Hallervorden-Spatz disease (HSD): bilateral symmetrical decreased signal intensity in the globus pallidus secondary to the T2* effect of paramagnetic substances, principally iron. The neuroradiological differential diagnosis and pathognomonic signs of HSD are discussed.
本文报告了一例15岁男性患者，患有进行性肌张力障碍、构音障碍和痴呆。临床检查未明确诊断。低场强MRI（0.23T）显示双侧苍白球坏死，病因不明。仅高场强成像（2.0T）显示出一种被认为是Hallervorden-Spatz病（HSD）特异性的病变模式：由于顺磁性物质（主要是铁）的T2*效应，双侧苍白球信号强度对称降低。文中讨论了HSD的神经放射学鉴别诊断和特征性体征。
