Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy

Wollenweber, Frank A.; Opherk, Christian; Zedde, Marialuisa; Catak, Cihan; Malik, Rainer; Duering, Marco; Konieczny, Marek J.; Pascarella, R.; Samões, R.; Correia, M.; Martí-Fàbregas, J.; Linn, Jennifer and Dichgans, Martin (19. February 2019): Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy. In: Neurology, Vol. 92, No. 8, e792-e801

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.

• Item Type: Journal article
• EU Funded Grant Agreement Number: 666881
• EU Projects: Horizon 2020 > RIA - Research and Innovation action > SVDs-at-target - Small Vessel Diseases from a therapeutic perspective: Targets for intervention. Affected pathways and mechanistic exploitation for prevention of stroke dementia; Horizon 2020 > RIA - Research and Innovation action > CoSTREAM - Common mechanisms and pathways in stroke and Alzheimer's disease
• Form of publication: Publisher's Version
• Faculties: Medicine > Institute for Stroke and Dementia Research (ISD); Medicine > Munich Cluster for Systems Neurology (SyNergy)
• Subjects: 600 Technology > 610 Medicine and health
• ISSN: 0028-3878
• Annotation: Study funding: Deutsche Forschungsgemeinschaft (German Research Foundation), Munich Cluster for Systems Neurology (EXC 1010 SyNergy), and European Union’s Horizon 2020 research and innovation programmes under grant agreements no. 666881 (SVDs@target) and 667375 (CoSTREAM)
• Language: English
• DFG: Gefördert durch die Deutsche Forschungsgemeinschaft (DFG) - 390857198