Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German patients with treated Wilson's disease

✍ Scribed by Barbara Leinweber; J. Carsten Möller; Andre Scherag; Ulrike Reuner; Peter Günther; Christoph J.G. Lang; Hartmut H.J. Schmidt; Christoph Schrader; Oliver Bandmann; Anna Czlonkowska; Wolfgang H. Oertel; Harald Hefter

• Publisher: John Wiley and Sons
• Year: 2008
• Tongue: English
• Weight: 159 KB
• Volume: 23
• Category: Article
• ISSN: 0885-3185
• DOI: 10.1002/mds.21761

✦ Synopsis

Abstract

Wilson's disease (WD) is an inherited autosomal‐recessive disorder of copper metabolism characterized by a wide variety of neurological, hepatic, and psychiatric symptoms. The aim of the present study was the development and evaluation of a clinical rating scale, termed Unified Wilson's Disease Rating Scale (UWDRS), to assess the whole spectrum of clinical symptoms in WD. Altogether 107 patients (mean age 37.6 ± 11.9 years; 46 male, 61 female) with treated WD participated in the study. Cronbach's alpha as a measure of the internal consistency for the entire scale was 0.92, whereas the intraclass correlation coefficient (ICC) was 0.98 (confidence interval (CI~95%~) 0.97–0.99), indicating an excellent interrater reliability as determined in 32 patients. Besides the total score was significantly correlated with the earning capacity of the patients as indicated by an estimated Spearman's ρ ≈ 0.54 (CI~95%~ 0.40–0.69, P < 0.001). In summary, the UWDRS appears to be a promising tool to assess the disease severity in WD. Its usefulness in clinical research and drug trials should be further addressed. © 2007 Movement Disorder Society