Evaluation of the unified Wilson's disease rating scale (UWDRS) in German patients with treated Wilson's disease

被引：97

作者：

Leinweber, Barbara ^{[1
]}

Moeller, J. Carsten ^{[1
]}

Scherag, Andre ^{[2
]}

Reuner, Ulrike ^{[3
]}

Guenther, Peter ^{[4
]}

Lang, Christoph J. G. ^{[5
,7
]}

Schmidt, Hartmut H. J. ^{[6
]}

Schrader, Christoph

Bandmann, Oliver ^{[8
]}

Czlonkowska, Anna ^{[9
,10
]}

Oertel, Wolfgang H. ^{[1
]}

Hefter, Harald ^{[11
]}

机构：

[1] Univ Marburg, Dept Neurol, D-35039 Marburg, Germany

[2] Univ Marburg, Inst Med Biometry & Epidemiol, D-35039 Marburg, Germany

[3] Tech Univ Dresden, Dept Neurol, Dresden, Germany

[4] Univ Leipzig, Dept Neurol, Leipzig, Germany

[5] Univ Erlangen Nurnberg, Dept Neurol, D-8520 Erlangen, Germany

[6] Univ Munster, Dept Gastroenterol & Gen Surg Transplant Hepatol, D-4400 Munster, Germany

[7] Hannover Med Sch, Dept Neurol, Hannover, Germany

[8] Univ Sheffield, Acad Neurol Unit, Sheffield, S Yorkshire, England

[9] Med Univ, Dept Neurol, Inst Psychiat & Neurol, Warsaw, Poland

[10] Med Univ, Dept Pharmacol, Warsaw, Poland

[11] Univ Dusseldorf, Dept Neurol, D-4000 Dusseldorf, Germany

来源：

MOVEMENT DISORDERS | 2008年 / 23卷 / 01期

关键词：

Wilson's disease; clinical assessment; rating scale; GeNeMove; EuroWilson;

D O I：

10.1002/mds.21761

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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 (CI95%) 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 rho approximate to 0.54 (CI95% 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. (c) 2007 Movement Disorder Society.

引用

页码：54 / 62

页数：9

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