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

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

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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

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