Unified Wilson's Disease Rating Scale - a proposal for the neurological scoring of Wilson's disease patients

Background and purpose: The clinical forms of Wilson's disease (WD) neurological manifestations can be divided into three movement disorder syndromes: a) dystonic, b) ataxic, c) parkinsonian syndrome. These syndromes in WD seldom occur in isolation. Clinical rating scales such as the Unified Parkinson's Disease Rating Scale (UPDRS), the International Cooperative Ataxia Rating Scale (ICARS) and the Rating Scale for Dystonia (RSD), focusing on either parkinsonism or ataxia or dystonia alone, are not sufficient to reflect accurately the motor impairment of WD patients. The aim of the study was to develop a novel rating scale for WD, because as far as we know no scale for the clinical rating in WD has been designed before. Material and methods: In 2004 the EuroWilson consortium was founded, to create a European WD database. Members of the consortium from Poland, Germany, and France prepared a new scale using clinical rating scales as the UPDRS, ICARS, and RSD. Prepared drafts were discussed several times in detail at the first international neurological EuroWilson meeting in September 2004 in Paris and in November in Warsaw. Results and conclusions: The novel scale for WD consists of 3 parts, including: consciousness, a historical review based on the Barthel scale (2-11 items), and neurological examination (12-35, items). The maximum score for the first part is 3, for the second 39 points, and for the last 143 points. The initial reliability of the scale on the basis of 6 patients (on DVD) and 8 investigators was assessed. Inter-rater agreement was high. Now the scale is used by the EuroWilson and GeNeMove consortia.