Structural and functional brain changes in hepatic and neurological Wilson disease.


Journal

Brain imaging and behavior
ISSN: 1931-7565
Titre abrégé: Brain Imaging Behav
Pays: United States
ID NLM: 101300405

Informations de publication

Date de publication:
Oct 2021
Historique:
accepted: 09 11 2020
pubmed: 28 11 2020
medline: 13 10 2021
entrez: 27 11 2020
Statut: ppublish

Résumé

Wilson disease (WD) can manifest with hepatic or neuropsychiatric symptoms. Our understanding of the in vivo brain changes in WD, particularly in the hepatic phenotype, is limited. Thirty subjects with WD and 30 age- and gender-matched controls participated. WD group underwent neuropsychiatric assessment. Unified WD Rating Scale neurological exam scores were used to determine neurological (WDN, score > 0) and hepatic-only (WDH, score 0) subgroups. All subjects underwent 3 Tesla anatomical and resting-state functional MRI. Diffusion tensor imaging (DTI) and susceptibility-weighted imaging (SWI) were performed only in the WD group. Volumetric, DTI, and functional connectivity analyses were performed to determine between-group differences. WDN and WDH groups were matched in demographic and psychiatric profiles. The entire WD group compared to controls showed significant thinning in the bilateral superior frontal cortex. The WDN group compared to control and WDH groups showed prominent structural brain changes including significant striatal and thalamic atrophy, more subcortical hypointense lesions on SWI, and diminished white matter integrity in the bilateral anterior corona radiata and corpus callosum. However, the WDH group also showed significant white matter volume loss compared to controls. The functional connectivity between the frontostriatal nodes was significantly reduced in the WDN group, whereas that of the hippocampus was significantly increased in the WDH group compared to controls. In summary, structural and functional brain changes were present even in neurologically non-manifesting WD patients in this cross-sectional study. Longitudinal brain MRI scans may be useful as biomarkers for prognostication and optimization of treatment strategies in WD.

Identifiants

pubmed: 33244627
doi: 10.1007/s11682-020-00420-5
pii: 10.1007/s11682-020-00420-5
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

2269-2282

Subventions

Organisme : Albert Family
ID : N/A
Organisme : Jack Levin Foundation
ID : N/A
Organisme : Rachel and Drew Katz Foundation
ID : N/A

Informations de copyright

© 2020. Springer Science+Business Media, LLC, part of Springer Nature.

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Auteurs

Sule Tinaz (S)

Department of Neurology, Yale University School of Medicine, 15 York St, LCI Suite 710, New Haven, CT, 06510, USA. sule.tinaz@yale.edu.
Clinical Neurosciences Imaging Center, Yale University School of Medicine, New Haven, CT, USA. sule.tinaz@yale.edu.

Jagriti Arora (J)

Department of Radiology and Biomedical Imaging, Yale University School of Medicine, New Haven, CT, USA.

Keerthana Nalamada (K)

Department of Neurology, Yale University School of Medicine, 15 York St, LCI Suite 710, New Haven, CT, 06510, USA.

Ana Vives-Rodriguez (A)

Department of Neurology, Yale University School of Medicine, 15 York St, LCI Suite 710, New Haven, CT, 06510, USA.

Mine Sezgin (M)

Department of Neurology, Yale University School of Medicine, 15 York St, LCI Suite 710, New Haven, CT, 06510, USA.
Istanbul Faculty of Medicine, Department of Neurology, Istanbul University, Istanbul, Turkey.

Daphne Robakis (D)

Department of Neurology, Yale University School of Medicine, 15 York St, LCI Suite 710, New Haven, CT, 06510, USA.
Department of Neurology, State University of New York Downstate College of Medicine, Brooklyn, NY, USA.

Amar Patel (A)

Department of Neurology, Yale University School of Medicine, 15 York St, LCI Suite 710, New Haven, CT, 06510, USA.

R Todd Constable (RT)

Department of Radiology and Biomedical Imaging, Yale University School of Medicine, New Haven, CT, USA.

Michael L Schilsky (ML)

Departments of Medicine and Surgery, Sections of Digestive Diseases and Transplant and Immunology, Yale University School of Medicine, New Haven, CT, USA.

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