Looking Deep into the Eye-of-the-Tiger in Pantothenate Kinase-Associated Neurodegeneration
Looking Deep into the Eye-of-the-Tiger in Pantothenate Kinase-Associated Neurodegeneration
复制标题
DOI：
10.3174/ajnr.a5514
复制
发表时间：
2018-03-01
影响因子：
3.5
通讯作者：
Hayflick, S. J.
中科院分区：
文献类型：
--
作者：
Lee, J. -H.;Gregory, A.;Hayflick, S. J.
背景和目的：详细描述泛酸激酶相关性神经退行性变苍白球的MRI改变将有助于患者的诊断和监测。本研究的目的是确定的形态光谱的虎眼征和铁沉积的地形图模式在一组患者与泛酸激酶相关neurodegeneration.MATERIALS：74个磁共振成像扫描从54个人PANK 2突变进行了分析苍白球的信号模式。15例患者接受1.5T（n = 7）、3T（n = 7）和7T（n = 2）MR成像，收集16个SWI数据，以显示铁Topographic.Results：苍白球内侧边缘的线性高信号是最早的T2信号改变。这一发现在铁沉积引起的SWI变化变得可见之前是明显的。儿童早期T2WI多呈孤立高信号。在成人患者中，由于铁积累导致的早期高信号中心内的显著信号降低导致中心和周围区域之间的信号差异丧失。SWI上的信号低信号随着年龄的增长从苍白球内侧向外侧逐渐增强。内侧苍白球和黑质和丘脑底核的前内侧面之间的纤维连接显着低信号SWI.CONCLUSIONS：在泛酸激酶相关的神经变性，苍白球的MR成像变化，使用SWI开发区域特异性和年龄依赖性的现象。在泛酸激酶相关的神经变性中，在苍白球上观察到信号不均匀性，在确定铁浓度时应考虑到这一点。
BACKGROUND AND PURPOSE: A detailed delineation of the MR imaging changes in the globus pallidus in pantothenate kinase-associated neurodegeneration will be helpful for diagnosis and monitoring of patients. The aim of this study was to determine the morphologic spectrum of the eye-of-the-tiger sign and the topographic pattern of iron deposition in a group of patients with pantothenate kinase-associated neurodegeneration.MATERIALS AND METHODS: Seventy-four MR imaging scans from 54 individuals with PANK2 mutations were analyzed for signal patterns in the globus pallidus. Sixteen SWI data from 15 patients who underwent 1.5T (n = 7), 3T (n = 7), and 7T (n = 2) MR imaging were included to visualize the iron topography.RESULTS: The linear hyperintensity alongside the medial border of the globus pallidus was the earliest T2 signal change. This finding was evident before SWI changes from iron deposition became visible. T2WI performed in early childhood mostly showed isolated hyperintense signal. In adult patients, marked signal reduction within an earlier hyperintense center resulting from iron accumulation led to the loss of signal difference between the central and surrounding areas. Signal hypointensity on SWI progressed from the medial to the lateral portion of the globus pallidus with increasing age. The fiber connections between the medial globus pallidus and the anteromedial aspect of the substantia nigra and subthalamic nucleus were markedly hypointense on SWI.CONCLUSIONS: In pantothenate kinase-associated neurodegeneration, the globus pallidus MR imaging changes using SWI develop as region-specific and age-dependent phenomena. Signal inhomogeneity was observed across the globus pallidus in pantothenate kinase-associated neurodegeneration and should be considered when determining the concentration of iron.