Zhou Xiang-Xue, Li Xun-Hua, Qin Haolin, Li Gui-Dian, Huang Hai-Wei, Liang Ying-Ying, Liang Xiu-Ling, Pu Xiao-Yong
Department of Neurology, The East Area of the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510700, China.
Department of Neurology, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510700, China.
J Neurol Sci. 2016 Mar 15;362:292-8. doi: 10.1016/j.jns.2016.02.006. Epub 2016 Feb 4.
To evaluate damage to the extracorticospinal tract in Wilson disease (WD) patients using diffusion tensor imaging (DTI).
70 patients with WD, including 50 with cerebral type and 20 with hepatic type, and 20 age-matched healthy controls were enrolled. Neurological symptoms were scored using the modified Young Scale. Patients with cerebral type WD were divided into four subgroups: those with (1) hypokinesia, (2) parkinsonism, (3) mouth and throat dystonia, and (4) psychiatric symptoms. All study subjects underwent DTI of the brain. Five regions of interest (ROIs) were chosen. Fractional anisotropy (FA) and fiber volumes between ROIs were determined, and the relationships between DTI metrics and clinical status were evaluated.
FA values and fiber volumes between subcortical nuclei were lower in WD patients. Fiber volumes between the putamen (PU) and the globus pallidus (GP), substantia nigra (SN), and thalamus (TH); between the head of the caudate nucleus (CA) and the GP and TH; and between the TH and cerebellum were lower in group 1 than in the other groups of WD patients. Fiber volumes between the GP and the SN and TH were lower in group 2, and fiber volumes between the SN and TH were lower in group 3. DTI metrics differed between patients with the cerebral and hepatic types of WD.
DTI can reconstruct the network of the extracorticospinal tract. Fiber projection between subcortical nuclei was abnormal in WD patients. Damage to fiber connections may correlate with neurological symptoms in WD patients.
采用扩散张量成像（DTI）评估肝豆状核变性（WD）患者皮质脊髓外束的损伤情况。
纳入70例WD患者，其中50例为脑型，20例为肝型，并选取20例年龄匹配的健康对照者。采用改良杨氏量表对神经症状进行评分。脑型WD患者分为四个亚组：（1）运动减少型；（2）帕金森型；（3）口咽部肌张力障碍型；（4）精神症状型。所有研究对象均接受脑部DTI检查。选取五个感兴趣区（ROI）。测定ROI之间的分数各向异性（FA）和纤维束体积，并评估DTI指标与临床状态之间的关系。
WD患者皮质下核团之间的FA值和纤维束体积较低。在第1组中，壳核（PU）与苍白球（GP）、黑质（SN）和丘脑（TH）之间；尾状核头（CA）与GP和TH之间；以及TH与小脑之间的纤维束体积低于其他WD患者组。第2组中GP与SN和TH之间的纤维束体积较低，第3组中SN与TH之间的纤维束体积较低。脑型和肝型WD患者的DTI指标存在差异。
DTI可重建皮质脊髓外束网络。WD患者皮质下核团之间的纤维投射异常。纤维连接损伤可能与WD患者的神经症状相关。
