Abstract WMP55: Vessel Wall Characteristics using High Resolution Magnetic Resonance Imaging in Reversible Cerebral Vasoconstriction Syndrome and Central Nervous System Vasculitis

Abstract

Introduction: Reversible cerebral vasoconstriction syndrome (RCVS) and central nervous system (CNS) vasculitis often have similar initial clinical presentation, laboratory findings and imaging features creating a diagnostic dilemma. High-resolution-3-Tesla Magnetic Resonance Imaging with Gadolinium contrast (HR-MRI) is a non-invasive method to look at intracranial vessel wall characteristics. Methods: A retrospective analysis of all patients with a diagnosis of RCVS or CNS vasculitis that underwent HR-MRI at our institution was performed. Inclusion criteria for RCVS were clinical presentation, no aneurysmal subarachnoid hemorrhage, normal cerebrospinal fluid and reversible multifocal intracranial vessel stenosis whereas criteria for CNS vasculitis were cases with positive brain biopsy or typical clinical presentation, course and laboratory markers with rheumatology and stroke neurology agreement in diagnosis. Demographics, clinical presentation, laboratory testing, imaging studies and outcomes were collected. Results: Eleven patients with RCVS [10 (90.9%) females, mean age 45.2] and 8 with CNS vasculitis [6 primary CNS vasculitis, 6 (75%) males, mean age 43.5] were included in the study. No abnormal vessel wall enhancement or thickening was seen in any of the RCVS patients in areas of vessel stenosis. Six (75%) of CNS vasculitis patients had vessel wall thickening or enhancement (p=0.001 for comparison to RCVS) and the remaining 2 patients had HR-MRI performed 6 and 10 years after diagnosis and chronic treatment. All RCVS patients who had follow up HR-MRI demonstrated resolution of the multifocal stenosis. Two out of 4 CNS vasculitis patients with subsequent HR-MRI imaging had decrease in vessel wall thickening and enhancement after immunosuppressive therapy. Conclusion: In acute stages of presentation, HR-MRI may be useful in differentiating RCVS from CNS vasculitis. It may also be useful in following the disease course to look for resolution of intracranial vessel stenosis in RCVS or treatment response in vasculitis. Further studies are needed to confirm the utility of HR-MRI in diagnosis and disease progression in RCVS and vasculitis.