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...
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JCDR Research and Publications Private Limited
2023-09-01
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| Series: | Journal of Clinical and Diagnostic Research |
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| 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 |
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| author | M Mishra SD Nayak AK Mallick BD More G Mohanty M Samanta AK Sahu |
| author_facet | M Mishra SD Nayak AK Mallick BD More G Mohanty M Samanta AK Sahu |
| author_sort | M Mishra |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-11T21:43:39Z |
| format | Article |
| id | doaj.art-6df1d2b872df44a6ac4f8a8cd01be8cf |
| institution | Directory Open Access Journal |
| issn | 2249-782X 0973-709X |
| language | English |
| last_indexed | 2024-03-11T21:43:39Z |
| publishDate | 2023-09-01 |
| publisher | JCDR Research and Publications Private Limited |
| record_format | Article |
| series | Journal of Clinical and Diagnostic Research |
| spelling | doaj.art-6df1d2b872df44a6ac4f8a8cd01be8cf2023-09-26T11:21:31ZengJCDR Research and Publications Private LimitedJournal of Clinical and Diagnostic Research2249-782X0973-709X2023-09-011709050910.7860/JCDR/2023/62092.18375Minimal Unified Wilson’s Disease Rating Scale (M-UWDRS) Score Compared to UWDRS Neurological Subscore in Wilson’s Disease: A Cross-sectional StudyM Mishra0SD Nayak1AK Mallick2BD More3G Mohanty4M Samanta5AK Sahu6Senior Resident, Department of Neurology, Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India.Associate Professor, Department of Neurology, MKCG Medical College, Berhampur, Odisha, India.Professor, Department of Neurology, Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India.Associate Professor, Department of Pharmacology, Mahatma Gandhi Medical College and Research Institute, Puducherry, India.Professor, Department of Neurology, Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India.Assistant Professor, Department of Neurology, Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India.Assistant Professor, Department of Neurology, Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India.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. https://www.jcdr.net/articles/PDF/18375/62092_CE[Ra1]_F(IS)_QC(AN_IS)_PF1(AG_KM)_PFA(AG_KM)_PN(KM).pdfcopperhepatic symptomsprogressive lenticular degenerationpsychiatric symptoms |
| spellingShingle | M Mishra SD Nayak AK Mallick BD More G Mohanty M Samanta AK Sahu Minimal Unified Wilson’s Disease Rating Scale (M-UWDRS) Score Compared to UWDRS Neurological Subscore in Wilson’s Disease: A Cross-sectional Study Journal of Clinical and Diagnostic Research copper hepatic symptoms progressive lenticular degeneration psychiatric symptoms |
| title | Minimal Unified Wilson’s Disease Rating Scale (M-UWDRS) Score Compared to UWDRS Neurological Subscore in Wilson’s Disease: A Cross-sectional Study |
| title_full | Minimal Unified Wilson’s Disease Rating Scale (M-UWDRS) Score Compared to UWDRS Neurological Subscore in Wilson’s Disease: A Cross-sectional Study |
| title_fullStr | Minimal Unified Wilson’s Disease Rating Scale (M-UWDRS) Score Compared to UWDRS Neurological Subscore in Wilson’s Disease: A Cross-sectional Study |
| title_full_unstemmed | Minimal Unified Wilson’s Disease Rating Scale (M-UWDRS) Score Compared to UWDRS Neurological Subscore in Wilson’s Disease: A Cross-sectional Study |
| title_short | Minimal Unified Wilson’s Disease Rating Scale (M-UWDRS) Score Compared to UWDRS Neurological Subscore in Wilson’s Disease: A Cross-sectional Study |
| title_sort | minimal unified wilson s disease rating scale m uwdrs score compared to uwdrs neurological subscore in wilson s disease a cross sectional study |
| topic | copper hepatic symptoms progressive lenticular degeneration psychiatric symptoms |
| url | https://www.jcdr.net/articles/PDF/18375/62092_CE[Ra1]_F(IS)_QC(AN_IS)_PF1(AG_KM)_PFA(AG_KM)_PN(KM).pdf |
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