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Learning disability and epilepsy — SCE Neurology MCQ

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HardSpecial GroupsLearning disability and epilepsySCE Neurology

A 29-year-old from Turkey with recurrent oral and genital ulceration and previous uveitis develops painful monocular visual loss. MRI shows circumferential optic-nerve-sheath enhancement with relative sparing of the nerve substance. Which diagnosis best integrates the systemic and imaging findings?

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Correct answer: B — Neuro-Behcet-associated optic perineuritis

Explanation lettering: B = shown as A · E = shown as B · D = shown as C · C = shown as D · A = shown as E

E is correct: Behcet disease can cause neurological disease including optic perineuritis, where the optic sheath rather than the nerve substance enhances. Recurrent oral and genital ulceration with uveitis supplies the systemic discriminator. MOG disease can also cause perineural enhancement but does not explain this multisystem syndrome. AQP4 disease favours severe posterior optic neuritis or chiasmal involvement. Sarcoidosis can involve the optic sheath but would require different systemic evidence. Leber disease is painless, hereditary and non-inflammatory.

Reference: Neurological involvement in Behcet disease: https://pn.bmj.com/content/23/5/386