Neuromyelitis optica in Marfan syndrome

Neuromyelitis optica [NMO] is one of the differential diagnoses that should be considered in a patient with unilateral or bilateral loss of vision. It should be evaluated by history, examination, serological testing and neuroimaging studies. We report a case of a 39-year-old gentleman who was known to have Marfan's syndrome and presented with progressive loss of vision in one eye followed by the other one within one month. Neurological examination showed bilateral optic neuritis [ON] with optic atrophy and unilateral upper motor neuron signs. CSF analysis was positive for NMO-IgG; MRI of the brain and spine showed enhancement in both optic nerves pathways and the optic chiasm with normal spine appearance