• Emma A Koemans, Ellis S van Etten, Anna M van Opstal, Gerda Labadie, Gisela M Terwindt, Wermer Marieke J H MJH From the Department of Neurology (E.A.K., E.S.v.E., G.M.T., M.J.H.W.)., Andrew G Webb, Edip M Gurol, Steven M Greenberg, Mark A van Buchem, Jeroen van der Grond, and Sanneke van Rooden.
• From the Department of Neurology (E.A.K., E.S.v.E., G.M.T., M.J.H.W.).
• Stroke. 2018 Jun 1; 49 (6): 1518-1520.

Background And PurposeThe aim of the present study is to explore whether using 7 Tesla magnetic resonance imaging, additional brain changes can be observed in hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D) patients as compared with the established magnetic resonance imaging features of sporadic cerebral amyloid angiopathy.MethodsThe local institutional review board approved this prospective cohort study. In all cases, informed consent was obtained. This prospective parallel cohort study was conducted between 2012 and 2014. We performed T2*-weighted magnetic resonance imaging performed at 7 Tesla in presymptomatic mutation carriers (n=11, mean age 35±12 years), symptomatic HCHWA-D patients (n=15, mean age 45±14 years), and in control subjects (n=29, mean age 45±14 years). Images were analyzed for the presence of changes that have not been reported before in sporadic cerebral amyloid angiopathy and HCHWA-D. Innovative observations comprised intragyral hemorrhaging and cortical changes. The presence of these changes was systematically assessed in all participants of the study.ResultsSymptomatic HCHWA-D-patients had a higher incidence of intragyral hemorrhage (47% [7/15], controls 0% [0/29], P<0.001), and a higher incidence of specific cortical changes (40% [6/15] versus 0% [0/29], P<0.005). In presymptomatic HCHWA-D-mutation carriers, the prevalence of none of these markers was increased compared with control subjects.ConclusionsThe presence of cortical changes and intragyral hemorrhage are imaging features of HCHWA-D that may help recognizing sporadic cerebral amyloid angiopathy in living patients.© 2018 American Heart Association, Inc.

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