Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan.
Department of Medical Imaging, National Taiwan University Hospital, Taipei, Taiwan.
Neuroimage. 2024 Oct 1;299:120829. doi: 10.1016/j.neuroimage.2024.120829. Epub 2024 Sep 2.
Quantitative susceptibility mapping (QSM) is a post-processing technique that creates brain susceptibility maps reflecting metal burden through tissue magnetic susceptibility. We assessed topographic differences in magnetic susceptibility between participants with and without Wilson's disease (WD), correlating these findings with clinical severity, brain volume, and biofluid copper and iron indices.
A total of 43 patients with WD and 20 unaffected controls, were recruited. QSM images were derived from a 3T MRI scanner. Clinical severity was defined using the minimal Unified Wilson's Disease Rating Scale (M-UWDRS) and Montreal Cognitive Assessment scoring. Differences in magnetic susceptibilities between groups were evaluated using general linear regression models, adjusting for age and sex. Correlations between the susceptibilities and clinical scores were analyzed using Spearman's method.
In age- and sex-adjusted analyses, magnetic susceptibility values were increased in WD patients compared with controls, including caudate nucleus, putamen, globus pallidus, and substantia nigra (all p < 0.01). Putaminal susceptibility was greater with an initial neuropsychiatric presentation (n = 25) than with initial hepatic dysfunction (n = 18; p = 0.04). Susceptibility changes correlated negatively with regional brain volume in almost all topographic regions. Serum ferritin, but not serum copper or ceruloplasmin, correlated positively with magnetic susceptibility level in the caudate nucleus (p = 0.04), putamen (p = 0.04) and the hippocampus (p = 0.03). The dominance of magnetic susceptibility in cortical over subcortical regions correlated with M-UWDRS scores (p < 0.01).
The magnetic susceptibility changes could serve as a surrogate marker for patients with WD.
定量磁敏感图（QSM）是一种通过组织磁化率创建反映金属负担的脑磁化率图的后处理技术。我们评估了有和无肝豆状核变性（WD）的参与者之间的磁化率的地形差异，并将这些发现与临床严重程度、脑容量以及生物流体铜和铁指数相关联。
共招募了 43 名 WD 患者和 20 名无影响对照者。QSM 图像来自 3T MRI 扫描仪。临床严重程度采用最小统一 WD 评分量表（M-UWDRS）和蒙特利尔认知评估评分定义。使用广义线性回归模型评估组间磁化率差异，调整年龄和性别。使用 Spearman 方法分析磁化率与临床评分之间的相关性。
在年龄和性别调整分析中，与对照组相比，WD 患者的磁化率值增加，包括尾状核、壳核、苍白球和黑质（均 p < 0.01）。与初始肝功能障碍（n = 18）相比，具有初始神经精神表现（n = 25）的患者的壳核磁化率更高（p = 0.04）。磁化率变化与几乎所有地形区域的区域脑容量呈负相关。血清铁蛋白，但不是血清铜或铜蓝蛋白，与尾状核（p = 0.04）、壳核（p = 0.04）和海马（p = 0.03）的磁化率水平呈正相关。皮质区域磁化率的主导性超过皮质下区域与 M-UWDRS 评分相关（p < 0.01）。
磁化率变化可作为 WD 患者的替代标志物。
