Minimal Unified Wilson’s Disease Rating Scale (M-UWDRS) Score Compared to UWDRS Neurological Subscore in Wilson’s Disease: A Cross-sectional Study

Abstract

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.