2026 New Edition Neurology RITE Sample Question

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

Answer Explanation

This patient presents with a new-onset headache after age 65. This should be considered a red flag for secondary headaches. The presence of localized temporal pain, jaw claudication, and ipsilateral visual loss make giant cell arteritis (GCA) very likely. Inflammatory markers such as erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and platelets may be elevated and should be checked in these patients. GCA is considered a medium and large vessel vasculitis, and when untreated, ischemic lesions of the eye and permanent visual loss can occur. For this reason, treatment should not be delayed in patients with suspected GCA.

First-line therapy consists of high-dose steroids. In cases where there is toxicity or side effects due to steroids, in refractory patients, or for chronic therapy, steroid-sparing agents, such as methotrexate
and tocilizumab, are available.

The use of tocilizumab, an interleukin 6 (IL-6) receptor antagonist, for the treatment of GCA was suggested by evidence that IL-6 is important in disease pathogenesis due to typically increased IL-6 levels in patients with GCA. IL-6 can be induced by inflammatory conditions leading to a variety of immunological responses. Tocilizumab binds to IL-6 receptors and inhibits IL-6 signaling which leads to a decrease in cytokine and acute phase reactions as depicted in the image below. A growing number of case reports, small case series, and phase 2 trials reported the efficacy of tocilizumab for the management of GCA patients with refractory disease or glucocorticoid-related toxicities. It was FDA approved for GCA in 2017.

Illustration © TrueLearn, LLC

Incorrect Answer Explanations

Answer A – Steroids do not provide benefit: High-dose systemic glucocorticoids with Prednisone or Methylprednisolone should be initiated immediately if GCA is suspected. If administered early in the course of the disease, the use of steroids has been shown to improve systemic symptoms and signs of GCA.

Answer B – Biopsy must be done before starting steroids: If there is a high clinical suspicion for GCA, treatment should not be delayed and steroids (if no contraindication) should be started promptly. A temporal artery biopsy could still be obtained whether or not steroids have been started.

Answer C – Nonsteroid antiinflammatory drugs are a treatment option in this disease: Nonsteroidal anti-inflammatory drugs (NSAIDs) do not play a role in the treatment of GCA.

Answer E – Aspirin is the treatment of choice: Aspirin has shown conflicting results in the treatment of GCA.

Bottom Line

Tocilizumab is an interleukin 6 (IL-6) receptor antagonist used for the treatment of GCA.

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.

Illustration © TrueLearn, LLC

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.