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ObjectiveTo characterize lesion evolution and neurodegeneration in retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) using multimodal MRI.MethodsWe prospectively performed MRI and cognitive testing in RVCL-S and healthy control cohorts. Gray and white matter volume and disruption of white matter microstructure were quantified. Asymmetric spin echo acquisition permitted voxel-wise oxygen extraction fraction (OEF) calculation as an in vivo marker of microvascular ischemia. The RVCL-S cohort was included in a longitudinal analysis of lesion subtypes in which hyperintense lesions on FLAIR, T1-post-gadolinium, and diffusion-weighted imaging were delineated and quantified volumetrically.ResultsTwenty individuals with RVCL-S and 26 controls were enrolled. White matter volume and microstructure declined faster in RVCL–S compared to controls. White matter atrophy in RVCL-S was highly linear (ρ = −0.908, p < 0.0001). Normalized OEF was elevated in RVCL-S, and increased with disease duration. Multiple cognitive domains, specifically those measuring working memory and processing speed, were impaired in RVCL-S. Lesion volumes, regardless of subtype, progressed/regressed with high variability as a function of age, while FLAIR lesion burden increased near time-to-death (p < 0.001).ConclusionRVCL-S is a monogenic microvasculopathy predominantly affecting the white matter with regard to atrophy and cognitive impairment. White matter volumes in RVCL-S declined linearly, providing a potential metric against which to test efficacy of future therapies. Progressive elevation of white matter OEF suggests microvascular ischemia may underlie neurodegeneration in RVCL-S.

Introduction: The management of cerebral venous sinus thrombosis (CVST) is a common problem facing vascular neurologists. AHA/ASA guidelines suggest the use of heparin followed by vitamin K antagonists (VKA) for anticoagulation in CVST. In recent years, the evidence base has solidified for the use of novel oral anticoagulants (NOACs) in lower extremity DVT. Since data supporting their use in CVST is very limited, we sought to review our experience with NOACs in the treatment of CVST at a tertiary care center to address efficacy and safety. Methods: We retrospectively reviewed charts of all patients with CVST treated at our facility with a NOAC between 2014-2018. We collected data on demographics, risk factors for CVST, clinical features at presentation, imaging results, anticoagulation regimen, bleeding complications, and disability at follow up. We compared disability at follow up and major hemorrhagic events with historical controls. Results: We identified 27 patients with CVST treated with a NOAC, 22 of whom had follow up within our system. NOACs included apixaban (68.2%), rivaroxaban (27.3%) and dabigatran (4.5%). NOAC use was associated with stabilization of clot or partial recanalization in 81.8% of patients, and complete recanalization in 18.2%, at a median follow up of 5.5 months. Median mRS at follow up was 0, with one death. Four patients had bleeding complications, including two with symptomatic worsening of ICH. Comparison to historical controls showed no significant differences in terms of disability, death or major hemorrhagic events. Conclusion: Safety and efficacy of NOAC use for CVST were similar to historical controls anticoagulated with other agents. Analyses comparing NOAC-treated patients to 40 contemporary controls treated with vitamin K antagonists at our institution are underway, and prospective assessment of NOAC efficacy and safety in CVST in randomized controlled trials is warranted.