Wilson’s disease: scoring system

Evidence-based neurology checklist on wilson’s disease: scoring system: Wilson’s disease severity scoring system: items Neurological impairment: 1-2 points Serum ceruloplasmin 0.1-0.2g/L: 1 point Serum ceruloplasmin <0.1g/L: 2 points Kayser-Fleischer rings: 1 point Coombe’s positive hemolytic…

Wilson’s disease severity scoring system: items

  • Neurological impairment: 1-2 points
  • Serum ceruloplasmin 0.1-0.2g/L: 1 point
  • Serum ceruloplasmin <0.1g/L: 2 points
  • Kayser-Fleischer rings: 1 point
  • Coombe’s positive hemolytic anemia: 1 point

Wilson’s disease severity scoring system: application

References

  1. Nicastro E, Ranucci G, Vajro P, Vegnente A, Iorio R. Re-evaluation of the diagnostic criteria for Wilson disease in children with mild liver disease. Hepatology 2010; 52:1948-1956.
  2. Prashanth LK, Taly AB, Sinha S, et al. Prognostic factors in patients presenting with severe neurological forms of Wilson’s disease. QJM 2005; 98:557-563. 
  3. Kumar N, Gross JB, Ahlskog JE. Copper deficiency myelopathy produces a clinical picture like subacute combined degeneration. Neurology 2004; 63:33-39.
  4. Nations SP, Boyer PJ, Love LA, et al. Denture cream: an unusual source of excess zinc, leading to hypocupremia and neurologic disease. Neurology 2008; 71:639-643.
  5. Rowin J, Lewis SL. Copper deficiency myeloneuropathy and pancytopenia secondary to overuse of zinc supplementation. JNNP 2005; 76:750-751.
  6. And 3 more. Subscribe to see the full list

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