Table 3.
Leipzig scoring for Wilson’s disease
|
Typical clinical signs and symptoms
| |
| Kayser-Fleischer ring | |
| Present | 2 |
| Absent | 0 |
| Neurologic symptoms or typical abnormalities on MRI | |
| 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 copper1 | |
| > 4 μmol/g | 2 |
| 0.8-4 μmol/g | 1 |
| < 0.8 μmol/g | -1 |
| Rhodamine positive granules2 | 1 |
| Urinary copper excretion3 | |
| Normal | 0 |
| 1-2 times ULN | 1 |
| > 2 times ULN | 2 |
| 5 times ULN after penicillamine | 2 |
| Mutation analysis detected | |
| Both chromosomes | 4 |
| One chromosome | 1 |
| No mutations | 0 |
| Total Leipzig score | |
| Score | Evaluation |
| ≥ 4 | Diagnosis established |
| 3 | Diagnosis possible |
| ≤ 2 | Diagnosis very unlikely |
1
In the absence of cholestasis.
2
If no quantitative liver copper available.
3
In the absence of acute hepatitis. MRI: Magnetic resonance imaging; ULN: Upper limit of normal.