Wilson Disease – Treatment — SCE Neurology MCQ

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ModerateMovement DisordersWilson Disease – TreatmentSCE Neurology

A 22-year-old man with Wilson disease confirmed by low ceruloplasmin, high 24-hour urinary copper, and homozygous ATP7B mutation presents with tremor, dystonia, and psychiatric symptoms. Kayser-Fleischer rings are present. What is the first-line treatment for neurological Wilson disease?

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Correct answer: D — D-penicillamine

D-penicillamine is the traditional first-line chelation agent for Wilson disease. It chelates copper and promotes urinary copper excretion. However, it can cause initial neurological deterioration in ~15–20% of patients with neurological WD (paradoxical worsening), which has led some experts to prefer trientine (an alternative chelator with fewer side effects) as first-line. Current guidelines (EASL 2012, AASLD) support either penicillamine or trientine as first-line. Zinc acetate reduces intestinal copper absorption and is used for maintenance or mild disease. B: Trientine is an accepted alternative first-line with fewer side effects. C: Zinc is for maintenance therapy. D: Liver transplant is for fulminant hepatic failure or decompensated cirrhosis. E: Tetrathiomolybdate is experimental.

Reference: EASL Wilson Disease Guidelines (2012); ABN Movement Disorders Guidelines