Wilson’s Disease Scoring System Wilson's Disease Scoring System Scoring system developed at the 8th International Meeting on Wilson’s disease, Leipzig 2001 **EASL Clinical Practice Guidelines: Wilson's disease. J Hepatol. 2012 Mar;56(3):671-85. Kayser-Fleischer rings(Required) Present Absent Neurologic symptoms**(Required) Severe Mild Absent **or typical abnormalities at brain magnetic resonance imaging.Serum ceruloplasmin(Required) Normal (>0.2 g/L) 0.1-0.2 g/L <0.1 g/L Coombs-negative hemolytic anemia(Required) Present Absent Liver copper (in the absence of cholestasis)(Required) >5x ULN (>4 μmol/g) 0.8-4 μmol/g Normal (<0.8 μmol/g) Rhodanine-positive granules* *If no quantitative liver copper availableUrinary copper (in the absence of acute hepatitis)(Required) Normal 1-2x ULN >2x ULN Normal, but >5x ULN after D-penicillamine Mutation analysis(Required) On both chromosomes detected On 1 chromosome detected No mutations detected Score
