A Tertiary Care Center's Experience with Central Nervous System Vasculitis (CNSV) (P07.016)

Abstract

Objective: To evaluate CNS Vasculitis in a tertiary care cerebrovascular center serving a population of 1.5 million. Background CNSV is a rare and ill-defined cause of stroke(3-5%) with limited available case series in the modern era of stroke management. Design/Methods: Case series included cases coded as cerebral arteritis admitted to Stony Brook University Hospital between March 2009 and October 2011. Inclusion criteria-Acute onset of neurologic abnormalities in setting of angiographic guided multiple segmental narrowing in two or more CNS vessels. Results: 11 cases coded as cerebral arteritis, 4 males and 7 females between 13 to 88 years, satisfied our criteria for CNSV. 2 patients African-American and 9 Caucasian. Comorbid conditions included smoking, drug-abuse, hepatitis, DM, HTN, CKD,COPD and cyclical-vomiting-syndrome. Presentations ranged from seizures-36%(4/11)and stroke-63%(7/11) with 28%(2/7) being hemorrhagic and 71%(5/7) ischemic, none of which developed hemorrhagic conversion during the observation period. Primary CNSV accounted for 54%(6/11), 27%(4/11) had systemic vasculitis(2-SLE and 1-Monoclonal-gammopathy and 1-infectious arteritis), and 9%(1/11) had Reversible-Cerebral-Vasoconstriction Syndrome. Diagnostic modalities included cerebral-angiography in 54%(6/11), MRA in 63%(7/11), computerized-tomographic angiography in 27%(3/11), and brain/leptomeningeal biopsies in 18%(2/11) of cases. Bilateral cerebrovascular involvement was noted in 54%(6/11), of which 66% were primary and 33% systemic. Treatments were along the lines of acute reversal of vasoconstriction induced neurologic symptoms and immunomodulation. 45%(5/11) were transferred to acute rehabilitation facilities, 36%(4/11) discharged home, with a mortality of 18%(2/11). Outcome: Recurrence-18%(2/11)and complete resolution of angiographic vasculitis pattern-18%(2/11). Conclusions: Primary CNSV was more common than systemic CNSV, with stroke being the most common presentation followed by seizures. MRA and cerebral angiography were the most common diagnostic modalities. Our review points out the need for further prospective studies to allow for more systematic data collection and to better evaluate and manage CNSV patients. Supported by: Stony Brook University Hospital. Disclosure: Dr. Al-Mufti has nothing to disclose. Dr. Gupta has nothing to disclose. Dr. Coyle has received personal compensation for activities with Acorda Therapeutics, Avanir Pharmaceuticals, Bayer Pharmaceuticals Corporation, Biogen Idec, Genzyme Corporation, Novartis, Questcor, Roche Diagnostics Corporation, Sanofi-Aventis Pharmaceuticals, Inc., and Teva Neuroscience. Dr. Coyle has received personal compensation in an editorial capacity for NEURA. Dr. Coyle has received research support from Serono, Inc., Novartis, and Sanofi-Aventis Pharmaceuticals, Inc. Dr. Harris has nothing to disclose. Dr. Medin has nothing to disclose. Dr. Kim has nothing to disclose.