Semiquantitative Scale for Assessing Brain MRI Abnormalities in Wilson Disease: A Validation Study

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F20%3A10412330" target="_blank" >RIV/00216208:11110/20:10412330 - isvavai.cz</a>

Alternative codes found

RIV/00064165:_____/20:10412330

Result on the web

<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=jKrkOIpa7x" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=jKrkOIpa7x</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1002/mds.28018" target="_blank" >10.1002/mds.28018</a>

Result language

angličtina

Original language name

Semiquantitative Scale for Assessing Brain MRI Abnormalities in Wilson Disease: A Validation Study

Original language description

Background: MRI is a sensitive method for the assessment of brain abnormalities in Wilson disease, that is, T2 hyperintensities, T2 hypointensities, and atrophy, but a validated scoring system for the classification of radiological severity is lacking. The objective of this study was to develop and validate a brain MRI visual rating scale for Wilson disease. Methods: The proposed Wilson disease brain MRI severity scale consists of acute toxicity and chronic damage subscores from predefined structures. The former, calculated by summing scores of T2 hyperintensities (excluding cavitation), is likely to be partially reversible with treatment. The latter, representing the sum of scores of T2 hypointensities and brain atrophy, reflects pathology that is not readily reversible. Validation was performed on MRI scans acquired using 1.5T system from 39 Wilson disease patients examined at baseline and after 24 months on anticopper treatment. Intraclass correlation coefficients of 5 ratings from 3 raters were calculated. Temporal evolution of the MRI severity score and its association with clinical severity, assessed using the Unified Wilson Disease Rating Scale part III, was calculated. Results: Intrarater and interrater agreement were good (r &gt; 0.93; P &lt; 0.001; and r &gt; 0.74; P &lt; 0.001, respectively). In neurologic Wilson disease patients, the total MRI severity score improved over 2 years (P = 0.032), mainly because of reduced acute toxicity (P = 0.0015), whereas the chronic damage score deteriorated (P = 0.035). Unified Wilson Disease Rating Scale part III score was positively associated with chronic damage and total score at baseline (P = 0.005 and P = 0.003, respectively) and in month 24 (P &lt; 0.001 and P = 0.001, respectively). Conclusions: The Wilson disease brain MRI severity scale is a simple, reliable, and valid instrument that allows semiquantitative assessment of radiological Wilson disease severity.

Czech name

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Czech description

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Type

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

CEP classification

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OECD FORD branch

30103 - Neurosciences (including psychophysiology)

Project

Result was created during the realization of more than one project. More information in the Projects tab.

Continuities

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

Publication year

2020

Confidentiality

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

Name of the periodical

Movement Disorders

ISSN

0885-3185

e-ISSN

—

Volume of the periodical

35

Issue of the periodical within the volume

6

Country of publishing house

US - UNITED STATES

Number of pages

8

Pages from-to

994-1001

UT code for WoS article

000541254900016

EID of the result in the Scopus database

2-s2.0-85081897680