title: Diffusion tensor imaging of the extracorticospinal network in the brains of patients with Wilson disease.
abstract: OBJECTIVE
  To evaluate damage to the extracorticospinal tract in Wilson disease (WD) patients using diffusion tensor imaging (DTI).
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology, The East Area of the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510700, China.
authors.forename: Xiang-Xue
authors.initials: XX
authors.lastname: Zhou
authors.affiliation: Department of Neurology, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510700, China. Electronic address: 1397880617@qq.com.
authors.forename: Xun-Hua
authors.initials: XH
authors.lastname: Li
authors.affiliation: Department of Radiology, The East Area of the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510700, China.
authors.forename: Haolin
authors.initials: H
authors.lastname: Qin
authors.affiliation: Department of Radiology, The East Area of the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510700, China.
authors.forename: Gui-Dian
authors.initials: GD
authors.lastname: Li
authors.affiliation: Department of Neurology, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510700, China.
authors.forename: Hai-Wei
authors.initials: HW
authors.lastname: Huang
authors.affiliation: Department of Neurology, The East Area of the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510700, China.
authors.forename: Ying-Ying
authors.initials: YY
authors.lastname: Liang
authors.affiliation: Department of Neurology, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510700, China.
authors.forename: Xiu-Ling
authors.initials: XL
authors.lastname: Liang
authors.affiliation: Department of Reproductive Medicine and Urology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou 510080, China.
authors.forename: Xiao-Yong
authors.initials: XY
authors.lastname: Pu
mesh_terms: D000293:Adolescent; D000328:Adult; D000704:Analysis of Variance; D001921:Brain / Q000000981:diagnostic imaging*; D056324:Diffusion Tensor Imaging; D005260:Female; D006527:Hepatolenticular Degeneration / Q000000981:diagnostic imaging*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008297:Male; D009434:Neural Pathways / Q000000981:diagnostic imaging*; D055815:Young Adult
keywords: Diffusion tensor imaging; Extracorticospinal tract; Network; Wilson disease