2026 New Edition Neurology RITE Sample Question

Did you get it right? The correct answer is D.

Answer Explanation

This patient is presenting with signs and symptoms suspicious for Wilson disease. This is also known as hepatolenticular degeneration and is a rare inherited disorder that causes copper accumulation.

Nearly all (98%) of patients with Wilson disease with neurologic manifestations have Kayser-Fleischer rings. These are formed by the deposition of copper in the Descemet membrane in the limbus of the cornea and may appear a greenish-gold to brown color. Slit-lamp examination may be necessary for diagnosis. Kayser-Fleischer rings are no longer considered to be pathognomonic of Wilson disease unless the rings are accompanied by neurologic manifestations. These rings can also be seen in patients with chronic cholestatic disorders.

Mid-teens and older patients with Wilson disease are more likely to present with neurologic manifestations (compared with younger ages presenting with hepatic manifestations), with the mean age at presentation being in the upper teens.

Neurologic symptoms may be very subtle or may be rapidly progressive, leading to severe disability over the course of months. Some of the more common neurologic manifestations include dysarthria, gait abnormalities/ataxia, dystonia, tremor, parkinsonism, and drooling. Behavioral and psychiatric symptoms are also more common in patients with neurologic involvement and include depression, personality change, incongruous behavior, and irritability.

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Incorrect Answer Explanations

Answer A – Coombs-positive hemolytic anemia: Although not a common finding in isolation, Coombs-negative hemolytic anemia may be the initial symptom of Wilson disease. It results from the effects of excess copper ions on the red blood cell membrane in the circulation due to hepatic copper release after cellular necrosis. Thrombocytopenia may also be seen, which can indicate hypersplenism due to portal hypertension.

Answer B – Elevated alanine transaminase to aspartate transaminase ratio: The aspartate transaminase to alanine transaminase ratio is often > 2 in patients with Wilson disease.

Answer C – Elevated ceruloplasmin and copper concentration: Approximately 90% of patients with Wilson disease have low serum ceruloplasmin levels, which is the major carrier of copper in the blood.

Answer E – Decreased urinary copper excretion: Kayser-Fleischer rings are a characteristic feature of Wilson disease and are seen in approximately 98% of patients with neurologic manifestations and approximately 50% of patients with hepatic manifestations.

Bottom Line

Kayser-Fleischer rings are a characteristic feature of Wilson disease and are seen in approximately 98% of patients with neurologic manifestations and approximately 50% of patients with hepatic manifestations.