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General Medicine · Neurology: Wilson disease
Sample itemA 17-year-old has tremor, dysarthria, Kayser-Fleischer rings and low serum ceruloplasmin with raised 24-hour urinary copper. What is the treatment?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Copper chelation with penicillamine or trientine, followed by zinc
Wilson disease is autosomal recessive ATP7B mutation causing copper accumulation in liver and basal ganglia. Chelation with penicillamine or trientine removes copper, and zinc blocks gut absorption for maintenance. Transplantation is reserved for acute liver failure or decompensation.
Option by option
A. Liver transplant first for all
Transplant is not first-line in neurological disease.
B. Copper chelation with penicillamine or trientine, followed by zinc (correct)
Correct. Chelators and zinc form the treatment.
C. Iron chelation
Iron chelation is for iron overload.
D. Levodopa
Levodopa does not treat the underlying disorder.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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