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Clinical Article
Impairments of cerebral cortex and subcortical nucleus in cerebral hepatolenticular degeneration: magnetic resonance imaging study
KANG Taishan  YANG Tianhe  LIN Jianzhong  CAI Congbo  ZHANG Jiaxing 

Cite this article as: Kang TS, Yang TH, Lin JZ, et al. Impairments of cerebral cortex and subcortical nucleus in cerebral hepatolenticular degeneration: magnetic resonance imaging study. Chin J Magn Reson Imaging, 2019, 10(5): 337-341. DOI:10.12015/issn.1674-8034.2019.05.004.


[Abstract] Objective: To explore the damages of brain gray matter in patients with cerebral hepatolenticular degeneration.Materials and Methods: Brain 3D T1 weighted images were obtained from 30 patients with untreated cerebral hepatolenticular degeneration. One-way ANOVA was used to analyze group difference. Cortical gray matter and deep nucli volumes were analyzed using FMRIB Software Library (FSL) software and voxel based morphometry (VBM). Thirty healthy volunteers were enrolled as controls.Results: Compared with controls, the percentages of each cortical lobe atrophy and the comparison of bilateral cortical lobes were as follows: cerebellum (left 16.48%<right 16.54%, P>0.05), frontal cortex (left 23.6%>right 19.5%, P<0.001), temporal cortex (left 16.9%<right 25.4%, P<0.001), parietal cortex (left 15.5%<right 16.1%, P<0.001), occipital cortex (left 21.0%<right 27.2%, P<0.001), and insular cortex (left 58.7%>right 49.2%, P<0.001). The volumes of the brainstem and deep gray matter nuclei except the amygdale were significant atrophy. Atrophy percentage arranged in descending order was the accumbens nuclei, putamen nuclei, globus pallidus, thalamus, caudate nuclus, and brain-stem. There were no significant difference in the percentage of atrophy in each nucleus between the hemispheres.Conclusions: Cerebral hepatolenticular degeneration was characterized by bilaterally non-symmetrical cortical atrophy and symmetrical deep gray matter atrophy.
[Keywords] hepatolenticular degeneration;brain;magnetic resonance imaging

KANG Taishan Department of MRI, Zhongshan Affiliated Hospital of Xiamen University, Xiamen 361004, China

YANG Tianhe* Department of MRI, Zhongshan Affiliated Hospital of Xiamen University, Xiamen 361004, China

LIN Jianzhong Department of MRI, Zhongshan Affiliated Hospital of Xiamen University, Xiamen 361004, China

CAI Congbo College of information science and technology, Xiamen University, Xiamen 361005, China

ZHANG Jiaxing Institute of Brain Diseases and Cognition, Medical College of Xiamen University, Xiamen 361102, China

*Correspondence to: Yang TH, E-mail: yth13606916211@163.com

Conflicts of interest   None.

ACKNOWLEDGMENTS  This research was funded by National Natural Science Foundation of China No. 81671674, 81871519
Received  2018-12-27
Accepted  2019-03-20
DOI: 10.12015/issn.1674-8034.2019.05.004
Cite this article as: Kang TS, Yang TH, Lin JZ, et al. Impairments of cerebral cortex and subcortical nucleus in cerebral hepatolenticular degeneration: magnetic resonance imaging study. Chin J Magn Reson Imaging, 2019, 10(5): 337-341. DOI:10.12015/issn.1674-8034.2019.05.004.

[1]
中华医学会神经病学分会帕金森病及运动障碍学组,中华医学会神经病学分会神经遗传病学组. 肝豆状核变性的诊断与治疗指南. 中华神经科杂志, 2008, 41(8): 566-569.
[2]
Caca K, Loudianos G, Mieli-Vergani G, et al. Diagnosis and phenotypic classification of Wilson disease. Liver Int, 2003, 23: 139-142.
[3]
Lorincz MT. Neurologic Wilson's disease. Ann N Y Acad Sci, 2010, 1184(1): 173-187.
[4]
Neal AP, Guilarte TR. Mechanisms of lead and manganese neurotoxicity. Toxicol Res, 2013, 2(2): 99-114.
[5]
Rouault TA. Iron metabolism in the CNS: implications for neurodegenerative diseases. Nat Rev Neurosci, 2013, 14(8): 551-564.
[6]
Donovan A, Andrews NC. The molecular regulation of iron metabolism. Hematology, 2013, 5(1): 373-380.
[7]
Ott P, Vilstrup H. Cerebral effects of ammonia in liver disease: current hypotheses. Metab Brain Dis, 2014, 29(4): 901-911.
[8]
Sureka J, Jakkani RK, Panwar S. MRI findings in acute hyperammonemic encephalopathy resulting from decompensated chronic liver disease. Acta Neurol Belg, 2012, 112(2): 221-223.
[9]
Braissant O, McLin VA, Cudalbu C. Ammonia toxicity to the brain. J Inherit Metab dis, 2013, 36(4): 595-612.
[10]
Butterworth RF. Metal toxicity, liver disease and neurodegeneration. Neurotox Res, 2010, 18(1): 100-105.
[11]
Hermann W. Morphological and functional imaging in neurological and non-neurological Wilson's patients. Ann N Y Acad Sci, 2014, 1315(1): 24-29.
[12]
郑红伟,张伶,祁佩红, 等. 3.0 T MR对肝豆状核变性颅脑病变的诊断与临床意义. 影像诊断与介入放射学, 2011, 20(4): 252-255.
[13]
Llansola M, Montoliu C, Cauli O, et al. Chronic hyperammonemia, glutamatergic neurotransmission and neurological alterations. Metab Brain Dis, 2013, 28(2): 151-154.
[14]
Kim TJ, Kim IO, Kim WS, et al. MR imaging of the brain in Wilson disease of childhood: findings before and after treatment with clinical correlation. Am J Neuroradiol, 2006, 27(6): 1373-1378.
[15]
Piga M, Murru A, Satta L, et al. Brain MRI and SPECT in the diagnosis of early neurological involvement in Wilson's disease. Eur J Nucl Med Mol Imaging, 2008, 35(4): 716-724.
[16]
Lee JH, Yang TI, Cho M, et al. Widespread cerebral cortical mineralization in Wilson's disease detected by susceptibility-weighted imaging. J Neurol Sci, 2012, 313(1): 54-56.
[17]
周香雪,李洵桦,蒲小勇, 等. 肝、脑型肝豆状核变性患者影像学及金属代谢的对比分析. 中华医学杂志, 2017, 97(3): 176-181
[18]
张春芸,王安琴,武红利, 等. 肝豆状核变性患者灰质结构MRI形态学初步研究. 中国中西医结合影像学杂志, 2016, 14(3): 239-242, 245.
[19]
Sureka J, Jakkani RK, Panwar S. MRI findings in acute hyperammonemic encephalopathy resulting from decompensated chronic liver disease. Acta Neurol Belg, 2012, 112(2): 221-223.
[20]
Ecevit C, Ozgenç F, Gökçay F, et al. The diagnostic value of multimodal evoked potentials in the determination of subclinical neurological involvement of Wilson's disease. Eur J Gastroenterol Hepatol, 2012, 24(6): 627-632.
[21]
Zhai Z, Feng J. Left-right asymmetry influenced the infarct volume and neurological dysfunction following focal middle cerebral artery occlusion in rats. Brain Behav. 2018, 8(12): e01166.
[22]
Sugawara N, Ikeda T, Sugawara C, et al. Regional distribution of copper, zinc and iron in the brain in Long-Evans Cinnamon (LEC) rats with a new mutation causing hereditary hepatitis. Brain Res, 1992, 588(2): 287-290.

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