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Neurology. 2019 Jan 23. pii: 10.1212/WNL.0000000000006956. doi: 10.1212/WNL.0000000000006956. [Epub ahead of print]

Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy.

Author information

1
From the Institute for Stroke and Dementia Research (F.A.W., C.O., C.C., R.M., M. Duering, M.J.K., M. Dichgans), University Hospital, LMU Munich; Klinik für Neurologie (C.O.), SLK-Kliniken Heilbronn GmbH, Heilbronn, Germany; Stroke Unit-Neurology Unit and Neuroradiology Unit (M.Z., R.P.), Arcispedale Santa Maria Nuova, Azienda Unità Sanitaria Locale-IRCCS Reggio Emilia, Italy; Serviço de Neurologia (R.S., M.C.), Centro Hospitalar do Porto, Hospital de Santo António, Portugal; Hospital de la Santa Creu i Sant Pau (J.M.-F.), Biomedical Research Institute Sant Pau, IIB Sant Pau, Spain; Institut und Poliklinik für Neuroradiologie (J.L.), Universitätsklinikum Carl Gustav Carus, Dresden; Department of Neuroradiology (J.L.), Klinikum der Universität München, Ludwig-Maximilians-Universität LMU, Munich; Munich Cluster for Systems Neurology (SyNergy) (M. Dichgans); and German Center for Neurodegenerative Diseases (DZNE, Munich) (M. Dichgans), Germany. frank.wollenweber@med.uni-munchen.de.
2
From the Institute for Stroke and Dementia Research (F.A.W., C.O., C.C., R.M., M. Duering, M.J.K., M. Dichgans), University Hospital, LMU Munich; Klinik für Neurologie (C.O.), SLK-Kliniken Heilbronn GmbH, Heilbronn, Germany; Stroke Unit-Neurology Unit and Neuroradiology Unit (M.Z., R.P.), Arcispedale Santa Maria Nuova, Azienda Unità Sanitaria Locale-IRCCS Reggio Emilia, Italy; Serviço de Neurologia (R.S., M.C.), Centro Hospitalar do Porto, Hospital de Santo António, Portugal; Hospital de la Santa Creu i Sant Pau (J.M.-F.), Biomedical Research Institute Sant Pau, IIB Sant Pau, Spain; Institut und Poliklinik für Neuroradiologie (J.L.), Universitätsklinikum Carl Gustav Carus, Dresden; Department of Neuroradiology (J.L.), Klinikum der Universität München, Ludwig-Maximilians-Universität LMU, Munich; Munich Cluster for Systems Neurology (SyNergy) (M. Dichgans); and German Center for Neurodegenerative Diseases (DZNE, Munich) (M. Dichgans), Germany.

Abstract

OBJECTIVE:

To investigate the prognostic relevance of cortical superficial siderosis (cSS) in patients with cerebral amyloid angiopathy (CAA).

METHODS:

A total of 302 patients fulfilling clinical and imaging criteria for probable or possible CAA were enrolled into a prospective, multicenter cohort study and followed for 12 months. cSS was assessed on T2*/susceptibility-weighted imaging MRI. The predefined primary composite endpoint was incident stroke or death in patients with cSS compared to those without. Secondary analyses included cerebrovascular events and functional outcome measured by the modified Rankin Scale (mRS). Multiple regression analysis was performed to adjust for possible confounders.

RESULTS:

cSS prevalence was 40%. The primary endpoint occurred more frequently in patients with cSS (22%, 27/121) compared to those without (8%, 15/181, p = 0.001). Rates of CAA-related incident intracranial hemorrhage were 17% (cSS) and 4% (no cSS, p = 0.0003). The proportion of patients being functionally independent (mRS 0-2) 12 months from baseline were 59% (cSS) and 82% (no cSS, p = 0.00002). Presence of cSS was associated with the primary endpoint (adjusted odds ratio [OR] 1.2, 95% confidence interval [CI] 1.1-1.3, p = 0.0005), incident intracranial hemorrhage (adjusted OR 1.2, 95% CI 1.1-1.3, p = 0.0003), and less favorable outcome as assessed by the mRS (common OR 1.9, 95% CI 1.2-3.1, p = 0.009). Similar results were obtained in analyses restricted to patients with probable CAA and to patients with disseminated cSS (all p < 0.005).

CONCLUSIONS:

Patients with cSS and suspected CAA are at high risk for CAA-related incident intracranial hemorrhage and poor functional outcome. Both the presence and extent of cSS have prognostic relevance and may influence clinical decision-making.

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