Associations of Brain Atrophy and Cerebral Iron Accumulation at MRI with Clinical Severity in Wilson Disease

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023001%3A_____%2F21%3A00081039" target="_blank" >RIV/00023001:_____/21:00081039 - isvavai.cz</a>

Alternative codes found

RIV/00216208:11110/21:10428553 RIV/00064165:_____/21:10428553 RIV/68407700:21230/21:00350904

Result on the web

<a href="https://pubs.rsna.org/doi/pdf/10.1148/radiol.2021202846" target="_blank" >https://pubs.rsna.org/doi/pdf/10.1148/radiol.2021202846</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1148/radiol.2021202846" target="_blank" >10.1148/radiol.2021202846</a>

Result language

angličtina

Original language name

Associations of Brain Atrophy and Cerebral Iron Accumulation at MRI with Clinical Severity in Wilson Disease

Original language description

Background: Abnormal findings at brain MRI in patients with neurologic Wilson disease (WD) are characterized by signal intensity changes and cerebral atrophy. T2 signal hypointensities and atrophy are largely irreversible with treatment; their relationship with -permanent disability has not been systematically investigated. Purpose: To investigate associations of regional brain atrophy and iron accumulation at MRI with clinical severity in participants with neurologic WD who are undergoing long-term anti-copper treatment. Materials and Methods: Participants with WD and controls were compared in a prospective study performed from 2015 to 2019. MRI at 3.0 T included three-dimensional T1-weighted and six-echo multigradient-echo pulse sequences for morphometry and quantitative susceptibility mapping, respectively. Neurologic severity was assessed with the Unified WD Rating Scale (UWDRS). Automated multi-atlas segmentation pipeline with dual contrast (susceptibility and T1) was used for the calculation of volumes and mean susceptibilities in deep gray matter nuclei. Additionally, whole-brain analysis using deformation and surface-based morphometry was performed. Least absolute shrinkage and selection operator regression was used to assess the association of regional volumes and susceptibilities with the UWDRS score. Results: Twenty-nine participants with WD (mean age, 47 years. 9 [standard deviation]; 15 women) and 26 controls (mean age, 45 years. 12; 14 women) were evaluated. Whole-brain analysis demonstrated atrophy of the deep gray matter nuclei, brainstem, internal capsule, motor cortex and corticospinal pathway, and visual cortex and optic radiation in participants with WD (P &gt; .05 at voxel level, corrected for family-wise error). The UWDRS score was negatively correlated with volumes of putamen (r = .0.63, P &gt; .001), red nucleus (r = .0.58, P = .001), globus pallidus (r = .0.53, P = .003), and substantia nigra (r = .0.50, P = .006) but not with susceptibilities. Only the putaminal volume was identified as a stable factor associated with the UWDRS score (R-2 = 0.38, P &gt; .001) using least absolute shrinkage and selection operator regression. Conclusion: Individuals with Wilson disease (WD) had widespread brain atrophy most pronounced in the central structures. The putaminal volume was associated with the Unified WD Rating Scale score and can be used as a surrogate imaging marker of clinical severity. (C) RSNA, 2021

Czech name

—

Czech description

—

Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30224 - Radiology, nuclear medicine and medical imaging

Project

<a href="/en/project/NV15-25602A" target="_blank" >NV15-25602A: Biomarkers of progression and treatment response in neurodegenerative disorders</a><br>

Continuities

P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Publication year

2021

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Radiology

ISSN

0033-8419

e-ISSN

—

Volume of the periodical

299

Issue of the periodical within the volume

3

Country of publishing house

US - UNITED STATES

Number of pages

11

Pages from-to

662-672

UT code for WoS article

000655251600035

EID of the result in the Scopus database

2-s2.0-85107091641