Minimal Unified Wilson’s Disease Rating Scale (M-UWDRS) Score Compared to UWDRS Neurological Subscore in Wilson’s Disease: A Cross-sectional Study
Published: September 1, 2023 | DOI: https://doi.org/10.7860/JCDR/2023/62092.18375
S Mishra, SD Nayak, AK Mallick, BD More, G Mohanty, M Samanta, AK Sahu
1. Senior Resident, Department of Neurology, Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India.
2. Associate Professor, Department of Neurology, MKCG Medical College, Berhampur, Odisha, India.
3. Professor, Department of Neurology, Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India.
4. Associate Professor, Department of Pharmacology, Mahatma Gandhi Medical College and Research Institute, Puducherry, India.
5. Professor, Department of Neurology, Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India.
6. Assistant Professor, Department of Neurology, Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India.
7. Assistant Professor, Department of Neurology, Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India.
Correspondence
Dr. BD More,
Associate Professor, Department of Pharmacology, Mahatma Gandhi Medical College and Research Institute, Puducherry-607402, India.
E-mail: drbdmore@gmail.com
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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