Table 2.

Scoring system developed at the 8th International Meeting on Wilson's disease, Leipzig 2001.³³

Symptoms
Kayser–Fleischer rings
Present	2
Absent	0
Neurologic symptoms or typical imaging at brain magnetic resonance imaging
Severe	2
Mild	1
Absent	0
Serum ceruloplasmin
Normal (>0.2 g/L)	0
0.1–0.2 g/L	1
<0.1 g/L	2
Coombs negative hemolytic anemia
Present	1
Absent	0
Other tests
Liver copper (in the absence of cholestasis)
>5x ULN (>4 μmol/g)	2
0.8–4 μmol/g	1
Normal (<0.8 μmol/g)	−1
Rhodanine-positive granulesa	1
Urinary copper (in the absence of acute hepatitis)
Normal	0
1-2x ULN	1
>2 ULN	2
Normal, but >5x ULN after D-Penicillamine	2
Mutation analysis
On both chromosome detected	4
On 1 chromosome detected	1
No mutations detected	0
Total score	Evaluation:
4 or more	Diagnosis established
3	Diagnosis possible, more tests needed
2 or less	Diagnosis very unlikely

ULN: upper limit of normal.

^aIf no quantitative liver copper available.

Reproduced with permission from Elsevier Publications J Hepatol. 2012; 56:671–85.