Table 3.
Modified Leipzig Scoring System for Diagnosis of Wilson's Disease.
| KF rings | |
| Present | 2 |
| Absent | 0 |
| Serum ceruloplasmin | |
| Normal (>20 mg/dl) | 0 |
| 0–5 mg/dl | 3 |
| 6–11 mg/dl | 2 |
| 11–20 mg/dl | 1 |
| 24-h urinary copper (in the absence of acute hepatitis) | |
| >100 mcg | 2 |
| 40–100 | 1 |
| <40 mcg | 0 |
| Coomb's-negative hemolytic anemia with liver disease | |
| Present | 1 |
| Absent | 0 |
| Mutational analysis | |
| On both chromosomes detected | 4 |
| On one chromosome detected | 1 |
| No mutation detected/test not done | 0 |
| Liver biopsy for histology S/O WD with | |
| Orcein- or rhodanine-positive granules | 1 |
| Neurobehavioral symptoms | 2 |
| Present | 2 |
| Absent | 0 |
| Typical features on MRI brain | 1 |
| Present | 1 |
| Absent | 0 |
| History of Wilson disease in a family member | |
| Sibling death from liver disease/neurological disease suggestive of WD | 1 |
| Total score | Evaluation |
| ≥4 or more | Diagnosis established |
| 3 | Diagnosis possible, more tests needed |
| ≤2 | Diagnosis very unlikely |
KF, Kayser-Fleischer; MRI, Magnetic Resonance Imaging; S/O, suggestive of; WD, Wilson's Disease.