A 43-year-old woman presented with progressive left visual loss, gait impairment, and urinary incontinence. Examination revealed paraparetic gait, pyramidal deep tendon reflexes, and bilateral Babinski sign. Her erythrocyte sedimentation rate was raised. Extensive diagnostic workup (including measurement of antinuclear antibody, extractable nuclear antigen, antineutrophil cytoplasmic antibody, antiphospholipid antibodies, angiotensin-converting enzyme, and antibodies to aquaporin-4) ruled out systemic or infectious vasculitis. The results of cerebrospinal fluid biochemical and microbiological analysis were negative. Oligoclonal bands were absent. Spinal magnetic resonance imaging (MRI) disclosed a cervicodorsal T2-hyperintense lesion with cervical tract swelling (Figure, A); brain MRI showed acute ischemic lesions in watershed areas of the left centrum semiovale (Figure, B). Magnetic resonance angiography revealed bilateral middle cerebral artery occlusion. Catheter angiography confirmed these findings (Figure, C) and evidenced narrowing of the left ophthalmic artery (Figure, D), posterior cerebral artery (Figure, E), and right anterior cerebral artery, with activation of leptomeningeal collateral circulation.
Manara R, Schiavon F, Carraro V, Cagnin A, Briani C. Angiographic Diagnosis of Primary Central Nervous System Vasculitis With Spinal Cord Involvement. Arch Neurol. 2009;66(4):532–533. doi:10.1001/archneurol.2009.21
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