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Neurosarcoidosis — SCE Neurology MCQ

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HardCNS inflammationNeurosarcoidosisSCE Neurology

A patient has unilateral painful ophthalmoplegia involving the third, fourth and sixth cranial nerves. Contrast MRI shows inflammatory tissue at the orbital apex, but infection, malignancy, thrombosis and sarcoidosis have not yet been excluded. How should Tolosa–Hunt syndrome be approached?

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Correct answer: C — Diagnosis of exclusion after structural and inflammatory assessment

The best answer is “Diagnosis of exclusion after structural and inflammatory assessment”. Tolosa–Hunt is a rare granulomatous painful ophthalmoplegia diagnosis that requires compatible imaging and systematic exclusion of neoplasm, infection, vascular disease and other inflammation; steroid response is neither unique nor sufficiently diagnostic. “Diagnosis based principally on a rapid corticosteroid response” is less appropriate because lymphoma and other inflammatory disorders can also improve transiently with steroids “Benign inflammatory diagnosis from a pupil-sparing third-nerve palsy” is less appropriate because aneurysm, diabetes, cavernous disease and orbital lesions require distinct urgent pathways “Lumbar puncture before reviewing the orbital-apex abnormality” is less appropriate because CSF may contribute but should be directed by the demonstrated anatomical lesion and safety assessment “Deferred imaging because pupil function remains preserved” is less appropriate because pupil sparing does not negate an orbital-apex or cavernous structural process

Reference: NICE NG127: Suspected neurological conditions — recommendations. https://www.nice.org.uk/guidance/ng127/chapter/Recommendations