Unilateral Scotoma as Inaugural Sign in Moyamoya Disease

Purpose: To report a case of Moyamoya Disease presenting with unilateral scotoma caused by unilateral sectorial optic nerve atrophy, associated with macular microaneurysms. Methods: Observational case report. Results: A 38-year-old patient presented with unilateral scotoma in his right eye. Optic nerve atrophy in the corresponding sector was observed, as well as unilateral macular microaneurysms and peri-foveal capillary dropout. Brain magnetic resonance imaging, magnetic resonance angiography and conventional angiography showed supra-clinoid, anterior, middle, and posterior cerebral Moyamoya disease, with vascular clutter around the right optic canal. Relative stenosis of the left cavernous carotid artery was also present. Conclusion: This report describes a patient whose inaugural sign of Moyamoya Disease was unilateral scotoma, that was associated with macular vascular malformations.