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• Clinical Article •
Clinical and MRI analysis of hepatic and cerebral hepatolenticular degeneration
ZHAO Tieniu  LIAN Yuanshu  HU Xuemei  TAN Wenting  DENG Guohong  LIU He  HU Xiaofei 

Cite this article as: Zhao TN, Lian YS, Hu XＭ, et al. Clinical and MRI analysis of hepatic and cerebral hepatolenticular degeneration [J]. Chin J Magn Reson Imaging, 2021, 12(3): 1-5. DOI:10.12015/issn.1674-8034.2021.03.001.


[Abstract] Objective To investigate the disparity of the imaging features, fundus examination and laboratory examination between hepatic and cerebral hepatolenticular degeneration [Wilson disease (WD)]. Materials andMethods The imaging features and clinical data, presentation, laboratory examinations of 89 patients with hepatolenticular degeneration diagnosed by gene test in the first affiliated hospital of army medical university hospital between 2009—2019 were collected and analyzed retrospectively. MRI abnormalities were rated using standardized methods. Comparison of clinical measures and imaging scores between different types of hepatolenticular degeneration patients were performed.Results There was no significant difference in the positive rate of KF between brain type and liver type (P=0.946＞0.05), but the content of copper and ceruloplasmin in patients of brain type were significant lower than those in patients of liver type (P＜0.001). The abnormal signal rate of brain MRI except pallidum, thalamus and cerebellum in patients of brain type was higher than that in patients of liver type (P=0.043, 0.013＜0.05). There was no significant difference between the two types of patients in the abnormal signal range of globus pallidus and thalamus (P＞0.05), while the cumulative score of patients with brain type was significantly higher than that of patients with liver type (P＜0.05); there was no statistical significance between the two types of patients in the abnormal signal range and cumulative score of cerebellar region (P=0.779＞0.05). The T2 sequence range and degree total score of brain MRI in patients with brain type hepatolenticular degeneration were positively correlated with the onset time (r=0.315, P=0.038), but not in patients with liver type.Conclusions Brain images of WD patients with simultaneous involvement of basal ganglia and brainstem are characteristic imaging findings both in patients of liver and brain types. The intracranial abnormal signal ratio, onset time, and the range and extent of intracranial lesions in patients brain types were higher than those in patients with liver type. Quantitative evaluation of T2 sequences is a potential evaluation method for the pathological stages and extent of the brain in WD patients.
[Keywords] hepatolenticular degeneration；clinical types；imaging features；disparity；magnetic resonance imaging

ZHAO Tieniu1, 2   LIAN Yuanshu3   HU Xuemei4   TAN Wenting5   DENG Guohong5   LIU He1   HU Xiaofei1*  

1 Department of Radiology, the First Affiliated Hospital of Army Medical University, Chongqing 400000, China

2 Department of Radiology, 924 th Hospital of the Joint Service Support Force of the People's Liberation Army, Guilin 541002, China

3 Department of Forensic surgery, 924 th Hospital of the Joint Service Support Force of the People's Liberation Army, Guilin 541002, China

4 Department of Ultrasound, 924 th Hospital of the Joint Service Support Force of the People's Liberation Army, Guilin 541002, China

5 Department of Infection, the First Affiliated Hospital of Army Medical University, Chongqing 400000, China

Hu XF, E-mail: harryzonetmmu@163.com

Conflicts of interest   None.

ACKNOWLEDGMENTS  National Science and Technology Major Project During the Thirteenth Five-year Plan Period No. 2018ZX10732202 Major Technical Innovation Project of Southwest Hospital No. SWH2016ZDCX1007
Received  2020-10-20
Accepted  2021-01-21
DOI: 10.12015/issn.1674-8034.2021.03.001
Cite this article as: Zhao TN, Lian YS, Hu XＭ, et al. Clinical and MRI analysis of hepatic and cerebral hepatolenticular degeneration [J]. Chin J Magn Reson Imaging, 2021, 12(3): 1-5. DOI:10.12015/issn.1674-8034.2021.03.001.

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