Jing Xiao-Zhong, Yuan Xiang-Zhen, Li Gai-Ying, Chen Jia-Lin, Wu Rong, Yang Ling-Li, Zhang Shu-Yun, Wang Xiao-Ping, Li Jian-Qi
Department of Neurology, Tongren Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Department of Neurology, Weifang People's Hospital, Weifang, China.
Front Neurosci. 2022 Jun 1;16:794375. doi: 10.3389/fnins.2022.794375. eCollection 2022.
Histopathological studies in Wilson's disease (WD) have revealed increased copper and iron concentrations in the deep gray matter nuclei. However, the commonly used mean bulk susceptibility only reflects the regional metal concentration rather than the total metal content, and regional atrophy may affect the assessment of mean bulk susceptibility. Our study aimed to quantitatively assess the changes of metal concentration and total metal content in deep gray matter nuclei by quantitative susceptibility mapping to distinguish patients with neurological and hepatic WD from healthy controls.
Quantitative susceptibility maps were obtained from 20 patients with neurological WD, 10 patients with hepatic WD, and 25 healthy controls on a 3T magnetic resonance imaging system. Mean bulk susceptibility, volumes, and total susceptibility of deep gray matter nuclei in different groups were compared using a linear regression model. The area under the curve (AUC) was calculated by receiver characteristic curve to analyze the diagnostic capability of mean bulk susceptibility and total susceptibility.
Mean bulk susceptibility and total susceptibility of multiple deep gray matter nuclei in patients with WD were higher than those in healthy controls. Compared with patients with hepatic WD, patients with neurological WD had higher mean bulk susceptibility but similar total susceptibility in the head of the caudate nuclei, globus pallidus, and putamen. Mean bulk susceptibility of putamen demonstrated the best diagnostic capability for patients with neurological WD, the AUC was 1, and the sensitivity and specificity were all equal to 1. Total susceptibility of pontine tegmentum was most significant for the diagnosis of patients with hepatic WD, the AUC was 0.848, and the sensitivity and specificity were 0.7 and 0.96, respectively.
Brain atrophy may affect the assessment of mean bulk susceptibility in the deep gray matter nuclei of patients with WD, and total susceptibility should be an additional metric for total metal content assessment. Mean bulk susceptibility and total susceptibility of deep gray matter nuclei may be helpful for the early diagnosis of WD.
肝豆状核变性（WD）的组织病理学研究显示，深部灰质核团中的铜和铁浓度升高。然而，常用的平均体磁化率仅反映区域金属浓度，而非总金属含量，且区域萎缩可能会影响平均体磁化率的评估。我们的研究旨在通过定量磁化率成像定量评估深部灰质核团中金属浓度和总金属含量的变化，以区分神经型和肝型WD患者与健康对照。
在3T磁共振成像系统上，对20例神经型WD患者、10例肝型WD患者和25名健康对照者进行定量磁化率成像。使用线性回归模型比较不同组深部灰质核团的平均体磁化率、体积和总磁化率。通过受试者工作特征曲线计算曲线下面积（AUC），以分析平均体磁化率和总磁化率的诊断能力。
WD患者多个深部灰质核团的平均体磁化率和总磁化率高于健康对照。与肝型WD患者相比，神经型WD患者在尾状核头部、苍白球和壳核的平均体磁化率较高，但总磁化率相似。壳核的平均体磁化率对神经型WD患者的诊断能力最佳，AUC为1，敏感性和特异性均等于1。脑桥被盖的总磁化率对肝型WD患者的诊断最显著，AUC为0.848，敏感性和特异性分别为0.7和0.96。
脑萎缩可能会影响WD患者深部灰质核团平均体磁化率的评估，总磁化率应作为总金属含量评估的一个补充指标。深部灰质核团的平均体磁化率和总磁化率可能有助于WD的早期诊断。
