Junctional scotoma in moyamoya disease

Abstract

Junctional scotoma in moyamoya disease Case presentation A 66-year-old Hispanic woman presented to the eye clinic with chronic, progressively worsening vision in the right eye over 2 years associated with right-sided temporal headaches, cutaneous allodynia of the scalp, and jaw claudication. She denied fevers, chills, joint pains, nausea, photophobia, phonophobia, personal history of autoimmune disease, and family history of hereditary ophthalmological diseases. Her ocular history included pterygium removal from the OD. Seven years prior to presentation, she had an episode of light-headedness, blurry vision for 30 minutes, a severe headache, and mild weakness of the left hemiface and left body lasting 2 hours. She was evaluated by her primary care provider, the emergency department, and neurology; neuroimaging was recommended but never completed, and atypical migraine was considered. At this presentation, her best corrected visual acuity was 20/ 400 OD and 20/20 OS. She had a 2+ relative afferent pupillary defect OD and optic disc pallor in the right eye with optic atrophy seen on magnetic resonance imaging of the brain and orbits. Fundoscopy of the left eye was normal. Automated perimetry showed visual field defects in each eye (Fig. 1). Erythrocyte sedimentation rate, C-reactive protein, and platelets were within normal limits. Computerized tomography angiography (CTA) was performed along with 3-dimensional volume-rendered CTA reformatting and angiography (Fig. 2), which