2026 New Edition Neurology RITE Sample Question
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Try This RITE Sample Question from the 2026 New Edition
A 55-year-old woman presents to the clinic because of facial pain. She describes episodes of sharp, electric, shock-like pains on the right side of her face for the past month. The pains will last seconds to minutes at a time and resolve on their own. She will experience the symptoms throughout the day. She says that the pain is worsened anytime she brushes her teeth or if her bed sheets touch her face at night. She has tried acetaminophen for the pain without much relief. She is of Asian ancestry. Her neurologic examination and general physical examination are both normal. What should this patient be screened for before initiating therapy?
A. HLA-B1502
B. HLA-B27
C. HLA-DR2
D. HLA-DR3
E. HLA-DR4
The Answer and Explanation
Did you get it right? The correct answer is: A
Carbamazepine includes a warning that patients with ancestry in genetically at-risk populations should be screened for the HLA-B*1502 allele before starting treatment with carbamazepine. Patients with Asian ancestry are an at-risk population. Patients who test positive should not be treated with carbamazepine unless the benefit clearly outweighs the risk. The presence of this allele increases the risk of hypersensitivity reactions including Stevens-Johnson syndrome and toxic epidermal necrolysis, which can be fatal.
This patient is suffering from trigeminal neuralgia. Trigeminal neuralgia occurs in 4 to 13 per 100,000 people and is more common in women than men with a greater incidence as age increases. It is most commonly caused by compression of the trigeminal nerve root (long white arrow) near the entry into the pons or from an aberrant loop of an artery or vein. It is clinically defined by paroxysmal, attacks of sharp, superficial, stabbing pain in the distribution of trigeminal nerve (V1, V2, or V3). It usually lasts from seconds to at most 2 minutes. It is most commonly unilateral and can be associated with autonomic symptoms such as conjunctival injection or tearing. Pain can be precipitated by minimal stimulation to the affected area or from brushing teeth, cold air, smiling, or talking.
MRI is usually done to evaluate for structural lesions in the cerebellopontine angle and MR angiography can be used to identify vascular compressive causes of trigeminal neuralgia.
Depicted in this T2-weighted MRI is the right CN V being compressed by the superior cerebellar artery (long white arrow), compared to the unaffected left side (short white arrow).
First-line treatment for trigeminal neuralgia is with carbamazepine, which acts at the sodium channels. The main side effects of this medication include hyponatremia and blood dyscrasias.
Incorrect Answer Explanations
Answer B: HLA-B27 is associated with multiple autoimmune conditions. These conditions include ankylosing spondylitis, postgonococcal arthritis, and anterior uveitis. The patient’s clinical presentation is not consistent with any of these conditions.
Answer C: HLA-DR2 is associated with systemic lupus erythematosus (SLE). This patient did not have any clinical signs or symptoms suggestive of SLE. Patients with SLE typically do not present with severe lancinating pain as described above.
Answer D: HLA-DR3 is associated with autoimmune hepatitis, Sjogren syndrome, diabetes mellitus type 1, and systemic lupus erythematous. It is not associated with trigeminal neuralgia or the treatment used for this condition.
Answer E: HLA-DR4 is associated with diabetes mellitus type 1 and rheumatoid arthritis. Patients with diabetes mellitus can have irritation of nerves leading to a painful peripheral neuropathy, however, the treatment of choice would not be carbamazepine. Usual treatment is with gabapentin or pregabalin.
Bottom Line
Trigeminal neuralgia treatment of choice is with carbamazepine. Patients of Asian ancestry should have genetic testing for the HLA-B1502 allele prior to starting this medication due to the high risk of Stevens-Johnson syndrome and toxic epidermal necrolysis, which can be fatal.
For more information, see:
Trigeminal Neuralgia on UpToDate
Carbamazepine: Drug information on UpToDate
Human leukocyte antigen on Wikipedia
Cheng-Ching E, Baron EP, Chahine L, Rae-Grant A. Comprehensive Review in Clinical Neurology. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2011.
FDA Tegretol.
Image sources:
Try Another RITE Sample Question from the 2026 New Edition
A 19-year-old male presents with dysarthria and difficulty walking. On exam, he has parkinsonism and is drooling. He has a history of depression and irritability. You are told by your attending physician that he is suspected of having a copper accumulation disorder. Which of the following is most commonly seen in patients with this suspected diagnosis?.
A. Coombs-positive hemolytic anemia
B. Elevated alanine transaminase to aspartate transaminase ratio
C. Elevated ceruloplasmin and copper concentration
D. Abnormal color in the limbus of the cornea
E. Decreased urinary copper excretion
The Answer and Explanation
Did you get it right? The correct answer is: D
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.
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