Gregoire Boulouis1, Andreas Charidimou2, Michael J Jessel2, Li Xiong2, Duangnapa Roongpiboonsopit2, Panagiotis Fotiadis2, Marco Pasi2, Alison Ayres2, M Emily Merrill2, Kristin M Schwab2, Jonathan Rosand2, M Edip Gurol2, Steven M Greenberg2, Anand Viswanathan2. 1. From the Hemorrhagic Stroke Research Program, Department of Neurology, Massachusetts General Hospital Stroke Research Center (G.B., A.C., M.J.J., L.X., D.R., P.F., M.P., A.A., K.M.S., J.R., M.E.G., S.M.G., A.V.), MIND Informatics, Massachusetts General Hospital Biomedical Informatics Core (M.E.M., J.R.), and Division of Neurocritical Care and Emergency Neurology, Massachusetts General Hospital (J.R.), Harvard Medical School, Boston, MA; Neuroradiology Department (G.B.), Université Paris Descartes, INSERM S894, Centre Hospitalier Sainte-Anne, Paris, France; and Faculty of Medicine (D.R.), Naresuan University, Phitsanulok, Thailand. gboulouis@mgh.harvard.edu. 2. From the Hemorrhagic Stroke Research Program, Department of Neurology, Massachusetts General Hospital Stroke Research Center (G.B., A.C., M.J.J., L.X., D.R., P.F., M.P., A.A., K.M.S., J.R., M.E.G., S.M.G., A.V.), MIND Informatics, Massachusetts General Hospital Biomedical Informatics Core (M.E.M., J.R.), and Division of Neurocritical Care and Emergency Neurology, Massachusetts General Hospital (J.R.), Harvard Medical School, Boston, MA; Neuroradiology Department (G.B.), Université Paris Descartes, INSERM S894, Centre Hospitalier Sainte-Anne, Paris, France; and Faculty of Medicine (D.R.), Naresuan University, Phitsanulok, Thailand.
Abstract
OBJECTIVE: Cerebral amyloid angiopathy (CAA) is a common age-related small vessel disease (SVD). Patients without intracerebral hemorrhage (ICH) typically present with transient focal neurologic episodes (TFNEs) or cognitive symptoms. We sought to determine if SVD lesion burden differed between patients with CAA first presenting with TFNEs vs cognitive symptoms. METHODS: A total of 647 patients presenting either to a stroke department (n = 205) or an outpatient memory clinic (n = 442) were screened for eligibility. Patients meeting modified Boston criteria for probable CAA were included and markers of SVD were quantified, including cerebral microbleeds (CMBs), perivascular spaces, cortical superficial siderosis (cSS), and white matter hyperintensities (WMHs). Patients were classified according to presentation symptoms (TFNEs vs cognitive). Total CAA-SVD burden was assessed using a validated summary score. Individual neuroimaging markers and total SVD burden were compared between groups using univariable and multivariable models. RESULTS: There were 261 patients with probable CAA included. After adjustment for confounders, patients first seen for TFNEs (n = 97) demonstrated a higher prevalence of cSS (p < 0.0001), higher WMH volumes (p = 0.03), and a trend toward higher CMB counts (p = 0.09). The total SVD summary score was higher in patients seen for TFNEs (adjusted odds ratio per additional score point 1.46, 95% confidence interval 1.16-1.84, p = 0.013). CONCLUSIONS: Patients with probable CAA without ICH first evaluated for TFNEs bear a higher burden of structural MRI SVD-related damage compared to those first seen for cognitive symptoms. This study sheds light on neuroimaging profile differences across clinical phenotypes of patients with CAA without ICH.
OBJECTIVE:Cerebral amyloid angiopathy (CAA) is a common age-related small vessel disease (SVD). Patients without intracerebral hemorrhage (ICH) typically present with transient focal neurologic episodes (TFNEs) or cognitive symptoms. We sought to determine if SVD lesion burden differed between patients with CAA first presenting with TFNEs vs cognitive symptoms. METHODS: A total of 647 patients presenting either to a stroke department (n = 205) or an outpatient memory clinic (n = 442) were screened for eligibility. Patients meeting modified Boston criteria for probable CAA were included and markers of SVD were quantified, including cerebral microbleeds (CMBs), perivascular spaces, cortical superficial siderosis (cSS), and white matter hyperintensities (WMHs). Patients were classified according to presentation symptoms (TFNEs vs cognitive). Total CAA-SVD burden was assessed using a validated summary score. Individual neuroimaging markers and total SVD burden were compared between groups using univariable and multivariable models. RESULTS: There were 261 patients with probable CAA included. After adjustment for confounders, patients first seen for TFNEs (n = 97) demonstrated a higher prevalence of cSS (p < 0.0001), higher WMH volumes (p = 0.03), and a trend toward higher CMB counts (p = 0.09). The total SVD summary score was higher in patients seen for TFNEs (adjusted odds ratio per additional score point 1.46, 95% confidence interval 1.16-1.84, p = 0.013). CONCLUSIONS:Patients with probable CAA without ICH first evaluated for TFNEs bear a higher burden of structural MRI SVD-related damage compared to those first seen for cognitive symptoms. This study sheds light on neuroimaging profile differences across clinical phenotypes of patients with CAA without ICH.
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