Leipzig Score (Wilson Disease diagnosis)
Scores the Leipzig (Ferenci) system for diagnosing Wilson disease.
Open the calculator → Runs in your browser. Data leaves only if you deliberately send a problem report from the interactive tool.
Example
- Kayser-Fleischer rings
- Present
- Neurologic symptoms or MRI changes
- Severe
- Serum ceruloplasmin
- Below 0.1 g/L
- Quantitative liver copper
- Not available
- 24-hour urinary copper
- Normal
- ATP7B mutation analysis
- No disease-causing variant found
- Coombs-negative hemolytic anemia
- Absent
Result: Leipzig score 6
The tool opens with these values already filled in. Replace them with your own.
What the result means
- 4 or more
- Diagnosis of Wilson disease established.
- Exactly 3
- Diagnosis possible; more tests needed.
- 2 or fewer
- Diagnosis very unlikely.
- Mutation
- Deleterious ATP7B variants on both chromosomes score 4 - enough on their own to establish the diagnosis. One chromosome scores 1.
- Minus one
- A NORMAL quantitative liver copper scores MINUS 1, not zero. It is evidence against the diagnosis, so an add-only reading of the table overstates the total.
- Rhodanine
- Rhodanine-positive granules score 1 only when no quantitative liver copper is available. They stand in for it; counting both double-counts the same histology.
What you enter
- Kayser-Fleischer rings
- Neurologic symptoms or MRI changes
- Serum ceruloplasmin
- Coombs-negative hemolytic anemia
- Quantitative liver copper
- Rhodanine-positive granules
- 24-hour urinary copper
- ATP7B mutation analysis
How this is calculated
- Weights
- KF rings 2. Neurologic severe 2, mild 1. Ceruloplasmin 0.1-0.2 g/L 1, below 0.1 2. Coombs-negative hemolysis 1. Urinary copper 1-2x ULN 1, above 2x 2, or 2 if above 5x after D-penicillamine.
Ferenci P, Caca K, Loudianos G, et al. Diagnosis and phenotypic classification of Wilson disease. Liver Int. 2003;23(3):139-142. Agreed by the Working Party at the 8th International Meeting on Wilson Disease, Leipzig 2001. Read the source ↗
A reference and educational tool. Not medical, legal, or financial advice, and not a substitute for clinician judgment.