A 24-year-old man presents with tremor, dysarthria, psychiatric symptoms, and Kayser-Fleischer rings. Serum ceruloplasmin is 8 mg/dL (low). 24-hour urinary copper is 180 μg/day (elevated). He is diagnosed with Wilson's disease. The PREFERRED treatment for the neurological presentation is:

• A D-penicillamine as first-line chelation therapy
• B Liver transplantation immediately
• C High-dose zinc alone as initial therapy
• D Trientine plus zinc maintenance (zinc acetate as adjunct) ✓

Explanation

For neurological Wilson's disease, trientine is preferred over D-penicillamine because penicillamine carries a 20–50% risk of initial neurological deterioration (paradoxical worsening) due to mobilisation of hepatic copper into the bloodstream with redistribution to the brain. Trientine is better tolerated with lower risk of neurological aggravation. Zinc acetate inhibits intestinal copper absorption and is used for maintenance or mild disease. Combination of trientine with zinc is the preferred strategy for neurological WD in current EASL 2012 guidelines. Liver transplantation corrects the metabolic defect but is not indicated for neurological disease without hepatic failure.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.