Sener R Nuri
Department of Radiology, Ege University Hospital, Bornova, Izmir, Turkey.
AJNR Am J Neuroradiol. 2003 Sep;24(8):1690-3.
We herein report the case of a 15-year-old male patient with pantothenate kinase-associated neurodegeneration. The classic "eye-of-the-tiger" appearance was initially present on the globus pallidi on T2-weighted MR images and had disappeared by the time of the 10-month follow-up examination. Fluid-attenuated inversion recovery images revealed marked hypointensity in the globus pallidi and dentate nuclei and high signal intensity changes in the deep cerebral white matter. Proton MR spectroscopy revealed markedly decreased N-acetylaspartate in the globus pallidi, associated with decreased N-acetylaspartate and increased myoinositol in the deep cerebral white matter. Diffusion MR images (b=1000 s/mm(2)) were negative (normal appearing) for deep cerebral white matter lesions, whereas apparent diffusion coefficient values were slightly increased (1.08-1.12 x 10(-3) mm(2)/s), compared with the apparent diffusion coefficient values from the normal white matter regions. Apparent diffusion coefficient values in the globus pallidi were lower than those in the unaffected thalamus.
我们在此报告一例15岁患有泛酸激酶相关神经变性的男性患者。经典的“虎眼征”最初出现在T2加权磁共振成像的苍白球上，在10个月的随访检查时已消失。液体衰减反转恢复图像显示苍白球和齿状核明显低信号，深部脑白质有高信号强度改变。质子磁共振波谱显示苍白球中N-乙酰天门冬氨酸明显减少，同时深部脑白质中N-乙酰天门冬氨酸减少，肌醇增加。扩散磁共振图像（b = 1000 s/mm(2)）显示深部脑白质病变为阴性（外观正常），而表观扩散系数值略有增加（1.08 - 1.12 x 10(-3) mm(2)/s），与正常白质区域的表观扩散系数值相比。苍白球中的表观扩散系数值低于未受影响的丘脑。
