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

Area of Science:

• Neurology
• Clinical Assessment Tools
• Rare Diseases

Background:

• The Unified Wilson's Disease Rating Scale (UWDRS), established in 2007, has limitations in assessing mental/psychiatric status and specific motor functions in Wilson's disease (WD).
• Existing scale issues include unclear instructions for motor assessments, gait/posture evaluation challenges, and inconsistent response options.
• These limitations necessitate the development of a more comprehensive and refined assessment tool for WD patients.

Purpose of the Study:

• To develop and validate a modified version of the UWDRS (mUWDRS) that addresses the shortcomings of the original scale.
• The mUWDRS aims to provide a more thorough assessment of both motor and non-motor symptoms, including cognitive and behavioral aspects, in individuals with WD.
• To improve the reliability and consistency of WD patient evaluations.

Main Methods:

• The modified UWDRS (mUWDRS) was created by revising the original scale and integrating items from other established scales like the MDS-UPDRS and PKAN-DRS.
• The mUWDRS includes revised sections for consciousness, daily living activities (part IIa), cognitive/mental/behavioral status (part IIb), and neurologic status with gait/posture modifications (part III).
• Cognitive pretesting was conducted, followed by validation in 7 patients by 8 neurologists to assess interrater agreement and internal consistency.

Main Results:

• Pilot testing of the mUWDRS demonstrated nearly perfect to substantial interrater agreement across all parts (index values ranging from 0.826 to 1.0).
• Specific agreement indices included 1 for part I, 0.90 for part IIa, 0.93 for cognitive functioning, 0.86 for behavioral status (part IIb), and 0.826 for part III.
• High internal consistency was observed, with Cronbach's alpha values of 0.82 for part II and 0.84 for part III, indicating reliability of the scale's components.

Conclusions:

• The mUWDRS is a well-performing, modified rating scale designed for comprehensive assessment of motor and non-motor symptoms and functional impairment in Wilson's disease.
• The scale exhibits good interrater agreement, supporting its utility in clinical practice for WD patient evaluation.
• A formal validity study is planned, and the mUWDRS is available for use via the provided link.