Leipzig score for Wilson’s disease

  Hepatology

The Leipzig score for Wilson’s disease is a diagnostic tool that combines various clinical, biochemical, and genetic criteria to assess the likelihood of the disease. This scoring system was developed at the 8th International Meeting on Wilson’s Disease in Leipzig in 2001 and has been incorporated into clinical practice guidelines by the European Association for the Study of the Liver (EASL).

As a table to copy-paste in word

CategoryDescriptionScore
Kayser-Fleischer RingsPresent2
Absent0
Neurologic SymptomsSevere2
Mild1
Absent0
Serum CeruloplasminNormal (>0.2 g/L)0
0.1-0.2 g/L1
<0.1 g/L2
Coombs-Negative Hemolytic AnemiaPresent1
Absent0
Liver Copper (in the absence of cholestasis)>5x ULN (>4 μmol/g)2
0.8-4 μmol/g1
Normal (<0.8 μmol/g)0
Rhodanine-positive granules1
Urinary Copper (in the absence of acute hepatitis)Normal0
1-2x ULN1
>2x ULN2
Normal, but >5x ULN after D-penicillamine2
Mutation AnalysisOn both chromosomes detected4
On 1 chromosome detected1
No mutations detected0

A score of 4 or more confirms the diagnosis of Wilson’s disease. A score of 3 indicates that the diagnosis is possible but requires further testing, while a score of 2 or lower suggests that Wilson’s disease is unlikely​

References:

EASL guideline Wilson 2012