Minimal Unified Wilson’s Disease Rating Scale (M-UWDRS) Score Compared to UWDRS Neurological Subscore in Wilson’s Disease: A Cross-sectional Study
Introduction: Wilson’s Disease (WD) is a copper metabolic disorder that affects the nervous system and liver. The Unified Wilson’s Disease Rating Scale (UWDRS) is used for diagnosing WD, but it is time-consuming. Therefore, a less exhaustive scale is needed as a screening tool, such as the minim...
Main Authors: | , , , , , , |
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Format: | Article |
Language: | English |
Published: |
JCDR Research and Publications Private Limited
2023-09-01
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Series: | Journal of Clinical and Diagnostic Research |
Subjects: | |
Online Access: | https://www.jcdr.net/articles/PDF/18375/62092_CE[Ra1]_F(IS)_QC(AN_IS)_PF1(AG_KM)_PFA(AG_KM)_PN(KM).pdf |
Summary: | Introduction: Wilson’s Disease (WD) is a copper metabolic
disorder that affects the nervous system and liver. The Unified
Wilson’s Disease Rating Scale (UWDRS) is used for diagnosing
WD, but it is time-consuming. Therefore, a less exhaustive
scale is needed as a screening tool, such as the minimal Unified
Wilson’s Disease Rating Scale (M-UWDRS).
Aim: To evaluate the utility of M-UWDRS in assessing signs and
symptoms associated with WD and compare it with the UWDRS
(Neurological) subscore.
Materials and Methods: This prospective, observational, non
interventional, cross-sectional study was conducted in the
Department of Neurology, Srirama Chandra Bhanja Medical
College and Hospital, Cuttack, Odisha, India from November
2017 to October 2020. A total of 42 patients with WD, based
on the European Association for the Study of the Liver (EASL)
guidelines, were enrolled. They were assessed using both
M-UWDRS and UWDRS (Neurological) scoring before treatment
and at a three-month follow-up.
Results: A total of 42 cases were enrolled, of which 28 were
males and 14 were females. The mean age of presentation was
15.4±5.1 years. Both the neurological subscore of UWDRS
and M-UWDRS were used pre and post-treatment, revealing a
significant improvement in the majority of subjects. Both scores
fared similarly in predicting disease severity, treatment outcome,
and follow-up. The score of the “minimal UWDRS” correlated
with the scores of the UWDRS for neurological subscores (r
value: 0.6, p-value<0.001).
Conclusion: M-UWDRS is a user-friendly, quick, and practical
prescreening scoring scale for evaluating disorder severity and
quantification of the outcomes in comparison to the UWDRS
score, especially in resource-constrained and busy neurology
departments. Scoring in WD will help in better prognostication
of the disease. |
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ISSN: | 2249-782X 0973-709X |