病例报告：泛酸激酶2相关神经变性中的磁共振波谱分析

Parashari Umesh C, Aga Pallavi, Parihar Anit, Singh Ragini, Joshi Vindhya

Department of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, India.

Indian J Radiol Imaging. 2010 Aug;20(3):188-91. doi: 10.4103/0971-3026.69353.

We report a case of a 13-year-old girl with Hallervorden-Spatz disease (HSD) or pantothenate kinase-2 associated neurodegeneration (PKAN). HSD is a rare neurodegenerative disorder, which is characterized by a rapidly progressive extrapyramidal syndrome, dementia with optic atrophy, and retinal degeneration. It is associated with accumulation of cysteine-iron complex in the globus pallidi and substantia nigra. The MRI "eye of the tiger" sign is the characteristic. MRI spectroscopy is also characteristic. It shows markedly decreased NAA/Cr values in the globus pallidi and substantia nigra with increased mI/Cr values that suggest of gliosis.

我们报告一例13岁患有哈勒沃登-施帕茨病（HSD）或泛酸激酶2相关神经变性（PKAN）的女孩。HSD是一种罕见的神经退行性疾病，其特征为快速进展的锥体外系综合征、伴有视神经萎缩的痴呆以及视网膜变性。它与苍白球和黑质中半胱氨酸-铁复合物的蓄积有关。MRI的“虎眼征”是其特征表现。磁共振波谱也具有特征性。它显示苍白球和黑质中NAA/Cr值明显降低，同时mI/Cr值升高，提示胶质增生。