Angiographic Diagnosis of Primary Central Nervous System Vasculitis With Spinal Cord Involvement

Abstract

A43-YEAR-OLDWOMAN presented with progressive left visual loss, gait impairment, and urinary incontinence. Examination revealed paraparetic gait, pyramidal deep tendon reflexes, and bilateral Babinskisign.Hererythrocytesedimentation rate was raised. Extensive diagnostic workup (including measurement of antinuclear antibody, extractable nuclear antigen, antineutrophil cytoplasmic antibody, antiphospholipid antibodies, angiotensin-convertingenzyme,and antibodiestoaquaporin-4)ruledout systemicorinfectiousvasculitis.The results of cerebrospinal fluid biochemicalandmicrobiologicalanalysiswerenegative.Oligoclonalbands were absent. Spinal magnetic resonanceimaging(MRI)disclosedacervicodorsal T2-hyperintense lesion withcervicaltractswelling(Figure, A); brain MRI showed acute ischemiclesionsinwatershedareasofthe left centrum semiovale (Figure, B). Magneticresonanceangiographyrevealed 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 cerebralartery,withactivationofleptomeningeal collateral circulation. Althoughneuromyelitisopticaor multiple sclerosis are common in young women with this clinical onset, laboratory results, characteristicmultifocalintracranialvesselnarrowing or occlusion, and ischemic parenchymalinvolvementintheabsenceofknownvasculiticcausesled to the diagnosis of primary central nervous system (PCNS) vasculitis. After treatment with high-dose intravenous methylprednisolone sodium succinate followed by oral cyclophosphamide, the spinal lesion recovered within 2 weeks, whereas vascular MRI findings also persisted, unchanged, at 8-month follow-up. The patient regained the ability to walk independently, but mild visual and bladder dysfunctions persisted. COMMENT