Abstract TP444: CNS Vasculitis: A Tertiary Care Center’s Experience

Abstract

OBJECTIVE: CNS Vasculitis (CNSV) is a rare and ill-defined cause of stroke with limited available case series in the modern era of stroke management. The objective of this study was to evaluate CNS Vasculitis in a tertiary care cerebrovascular center DESIGN/METHODS/RESULTS: Between March 2009 and July 2012, 14 cases were coded as having cerebral vasculitis and fulfilled our criteria for CNSV which included acute onset of neurologic abnormalities in setting of angiographic guided multiple segmental narrowing in two or more CNS vessels. Patients included 5 males and 9 females between 11 and 88 years of which 3 were African-American and 11 were Caucasian. Presentations ranged from seizures in 28.6%(4/14) and stroke in 71.4% (10/14) with 40% (4/10) being hemorrhagic and 60% (6/10) ischemic, with a 33%(2/6)hemorrhagic conversion rate. 78.6% (11/14) were found to have Primary CNSV and 14.3% (2/14) had systemic vasculitis. Diagnostic modalities included cerebralangiography in 64.2% (9/14), MRA in 85% (12/14), computerized-tomographic angiography in 64%(9/14), and brain/leptomeningeal biopsies in 28% (4/14) of cases. Treatments were along the lines of acute reversal of vasoconstriction induced neurologic symptoms and immunomodulation. 71.4% (10/14) received systemic steroids,7%(1/14) received Mycophenolate mofetil and 57% (8/14) received Cyclophosphamide. Methotrexate was given in 7% (1 /14) and calcium channel blockers were given in 28% (4/14) with 14% administered in the cerebrovascular angio suite intraarterially. 57% (8/14) were transferred to acute rehabilitation facilities, 35.7% (5/14) discharged home, and there was a 7%(1/14)mortality rate. CONCLUSIONS: We found that primary CNSV was more common than systemic vasculitis and stroke was the most common presentation followed by seizures. Individuals younger than 20 who presented with seizures were less likely to have concomitant strokes. Despite our limited understanding of this rare condition 92%(13/14)of our patients were discharged to either home or acute rehabilitation, while the mortality rate was 7%(1/14)as was the recurrence rate. Further prospective studies are needed to allow for more systematic data collection and to better evaluate and manage CNSV patients.