Deborah A Lipski, Simon-Pierre Docquier, Florence Rasquin
This report describes a patient whose inaugural sign of Moyamoya Disease was unilateral scotoma, that was associated with macular vascular malformations.
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.