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首页> 外文期刊>Acta neurologica Scandinavica. >Structural changes of central white matter tracts in Kennedy's disease - a diffusion tensor imaging and voxel-based morphometry study
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Structural changes of central white matter tracts in Kennedy's disease - a diffusion tensor imaging and voxel-based morphometry study

机译：肯尼迪病中中央白质束的结构变化-扩散张量成像和基于体素的形态学研究

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Objectives: Spinobulbar muscular atrophy [Kennedy's disease (KD)] is a rare X-linked neurodegenerative disorder of mainly spinal and bulbar motoneurons. Recent studies suggest a multisystem character of this disease. The aim of this study was to identify and characterize structural changes of gray (GM) and white matter (WM) in the central nervous system. Material and methods: Whole-brain-based voxel-based morphometry (VBM) and diffusion tensor imaging (DTI) analyses were applied to MRI data of eight genetically proven patients with KD and compared with 16 healthy age-matched controls. Results: Diffusion tensor imaging analysis showed not only decreased fractional anisotropy (FA) values in the brainstem, but also widespread changes in central WM tracts, whereas VBM analysis of the WM showed alterations primarily in the brainstem and cerebellum. There were no changes in GM volume. The FA value decrease in the brainstem correlated with the disease duration. Conclusion: Diffusion tensor imaging analysis revealed subtle changes of central WM tract integrity, while GM and WM volume remained unaffected. In our patient sample, KD had more extended effects than previously reported. These changes could either be attributed primarily to neurodegeneration or reflect secondary plastic changes due to atrophy of lower motor neurons and reorganization of cortical structures.
机译：目的：脊柱侧球肌萎缩症[肯尼迪病（KD）]是一种罕见的X连锁神经退行性疾病，主要是脊髓和延髓运动神经元。最近的研究表明该疾病的多系统特征。这项研究的目的是识别和表征中枢神经系统中灰色（GM）和白质（WM）的结构变化。材料和方法：将基于全脑体素的形态计量学（VBM）和扩散张量成像（DTI）分析应用于8位经过基因证明的KD患者的MRI数据，并与16位年龄匹配的健康对照者进行比较。结果：扩散张量成像分析不仅显示脑干的分数各向异性（FA）值降低，而且中央WM道也发生了广泛变化，而WM的VBM分析显示主要在脑干和小脑中发生了变化。 GM量没有变化。脑干的FA值降低与疾病持续时间有关。结论：扩散张量成像分析显示中央WM道完整性的细微变化，而GM和WM量仍未受影响。在我们的患者样本中，KD具有比以前报道的更大的扩展作用。这些变化要么主要归因于神经退行性变，要么反映由于下运动神经元萎缩和皮层结构重组而引起的继发性塑性变化。

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