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Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German patients with treated Wilson's disease
Author(s) -
Leinweber Barbara,
Möller J. Carsten,
Scherag Andre,
Reuner Ulrike,
Günther Peter,
Lang Christoph J.G.,
Schmidt Hartmut H.J.,
Schrader Christoph,
Bandmann Oliver,
Czlonkowska Anna,
Oertel Wolfgang H.,
Hefter Harald
Publication year - 2008
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.21761
Subject(s) - cronbach's alpha , rating scale , intraclass correlation , inter rater reliability , wilson's disease , medicine , concordance , disease , confidence interval , psychology , psychiatry , psychometrics , physical therapy , clinical psychology , developmental psychology
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