Modified Unified Wilson's Disease Rating Scale - scale presentation and pilot clinimetric testing

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064165%3A_____%2F25%3A10505913" target="_blank" >RIV/00064165:_____/25:10505913 - isvavai.cz</a>

Alternative codes found

RIV/00216208:11110/25:10505913

Result on the web

<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=0RUd8IpIZu" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=0RUd8IpIZu</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.5603/pjnns.101652" target="_blank" >10.5603/pjnns.101652</a>

Result language

angličtina

Original language name

Modified Unified Wilson's Disease Rating Scale - scale presentation and pilot clinimetric testing

Original language description

Aim of the study. The Unified Wilson&apos;s Disease Rating Scale (UWDRS) was first introduced in 2007 to assess neurologic status and impairment in patients with Wilson&apos;s disease (WD). However, major issues have been raised, including the lack of assessment of mental and psychiatric status, the lack of instructions for the assessment of certain motor functions, difficulties with gait and posture assessment, and heterogenous response options for questions across the scale. Clinical rationale for the study. The development of a modified version of the UWDRS (mUWDRS) was proposed. Materials and methods. A new version of UWDRS with revised wording and options was created, incorporating items from the Movement Disorder Society Unified Parkinson&apos;s Disease Rating Scale (MDS-UPDRS), the Unified Dyskinesia Rating Scale, and the Pantothenate Kinase Associated Neurodegeneration Disease Rating Scale (PKAN-DRS). As in the original, part I of the mUWDRS assesses the level of consciousness. Part II is now divided into two partly self-reported subscales: part IIa assesses 10 daily living activities and a new part IIb addresses cognitive functioning, mental and behavioral status. Part III assesses neurologic status as before, but with modifications for gait and posture items. After 2 rounds of cognitive pretesting, mUWDRS was validated in 7 patients by 8 neurologists for interrater agreement (Fleiss kappa or Kuder Richardson Coefficient) and for internal consistency (Cronbach&apos;s alpha). Results. In the pilot test, interrater agreement with mUWDRS was almost perfect or substantial, with index values of 1 for part I, 0.90 for part IIa, 0.93 for cognitive functioning and 0.86 for behavioral status in part IIb, and 0.826 for part III. A high level of internal consistency was observed, with Cronbach&apos;s alpha of 0.82 for part II and 0.84 for part III. Conclusions and clinical implications. The mUWDRS, designed to assess both motor and nonmotor symptoms of WD and functional impairment, performed well in pilot testing. Since the modified version of the WD rating scale has a good interrater agreement, a proper validity study is planned to be conducted. The scale is available on: https://www.movementdisorders.org/MDS/MDS-Clinical-Outcome-Assessment.htm.

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

—

OECD FORD branch

30103 - Neurosciences (including psychophysiology)

Project

—

Continuities

V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Publication year

2025

Confidentiality

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

Name of the periodical

Neurologia i Neurochirurgia Polska

ISSN

0028-3843

e-ISSN

1897-4260

Volume of the periodical

59

Issue of the periodical within the volume

6

Country of publishing house

PL - POLAND

Number of pages

9

Pages from-to

524-532

UT code for WoS article

001619183800001

EID of the result in the Scopus database

2-s2.0-105025206656