Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy

Frank Arne Wollenweber, Christian Opherk, Marialuisa Zedde, Cihan Catak, Rainer Malik, Marco Duering, Marek Janusz Konieczny, Rosario Pascarella, Raquel Samões, Manuel Correia, Joan Martí-Fàbregas, Jennifer Linn, Martin Dichgans

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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.

Original languageEnglish
Pages (from-to)e792-e801
JournalNeurology
Volume92
Issue number8
DOIs
Publication statusPublished - Feb 19 2019
Externally publishedYes

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Siderosis
Cerebral Amyloid Angiopathy
Intracranial Hemorrhages
Odds Ratio
Confidence Intervals

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Wollenweber, F. A., Opherk, C., Zedde, M., Catak, C., Malik, R., Duering, M., ... Dichgans, M. (2019). Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy. Neurology, 92(8), e792-e801. https://doi.org/10.1212/WNL.0000000000006956

Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy. / Wollenweber, Frank Arne; Opherk, Christian; Zedde, Marialuisa; Catak, Cihan; Malik, Rainer; Duering, Marco; Konieczny, Marek Janusz; Pascarella, Rosario; Samões, Raquel; Correia, Manuel; Martí-Fàbregas, Joan; Linn, Jennifer; Dichgans, Martin.

In: Neurology, Vol. 92, No. 8, 19.02.2019, p. e792-e801.

Research output: Contribution to journalArticle

Wollenweber, FA, Opherk, C, Zedde, M, Catak, C, Malik, R, Duering, M, Konieczny, MJ, Pascarella, R, Samões, R, Correia, M, Martí-Fàbregas, J, Linn, J & Dichgans, M 2019, 'Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy', Neurology, vol. 92, no. 8, pp. e792-e801. https://doi.org/10.1212/WNL.0000000000006956
Wollenweber FA, Opherk C, Zedde M, Catak C, Malik R, Duering M et al. Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy. Neurology. 2019 Feb 19;92(8):e792-e801. https://doi.org/10.1212/WNL.0000000000006956
Wollenweber, Frank Arne ; Opherk, Christian ; Zedde, Marialuisa ; Catak, Cihan ; Malik, Rainer ; Duering, Marco ; Konieczny, Marek Janusz ; Pascarella, Rosario ; Samões, Raquel ; Correia, Manuel ; Martí-Fàbregas, Joan ; Linn, Jennifer ; Dichgans, Martin. / Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy. In: Neurology. 2019 ; Vol. 92, No. 8. pp. e792-e801.
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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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AU - Opherk, Christian

AU - Zedde, Marialuisa

AU - Catak, Cihan

AU - Malik, Rainer

AU - Duering, Marco

AU - Konieczny, Marek Janusz

AU - Pascarella, Rosario

AU - Samões, Raquel

AU - Correia, Manuel

AU - Martí-Fàbregas, Joan

AU - Linn, Jennifer

AU - Dichgans, Martin

PY - 2019/2/19

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N2 - 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.

AB - 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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