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A 19-year-old with obesity presented with 4 days of progressive vision loss preceded by headache, nausea, and vomiting. He had been receiving acitretin, a retinoid medication, for acne vulgaris. Examination showed bilateral optic disc edema (see ), severely decreased visual acuity in both eyes, and a right relative afferent pupillary defect (RAPD). Serum inflammatory markers were elevated. MRI showed bilateral optic nerve and chiasmal enhancement; there was normal opening pressure on lumbar puncture. Early empirical high-dose glucocorticoids led to visual recovery.
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Comment
The final diagnosis here was only described relatively recently, but many patients previously labeled as having multiple sclerosis, acute disseminated encephalomyelitis (ADEM), or neuromyelitis optica (NMO) should be reevaluated for this disease. Since I work at an ophthalmologic hospital, I have seen many cases and remain impressed by the prominent involvement of the optic nerve (including the chiasm) and younger age of patients. I’ve learned not to be blindsided by imaging that doesn’t correlate clinically: dramatic lesions without symptoms, or imaging that lags behind findings or resolves spontaneously.