Prevalence and mechanisms of cortical superficial siderosis in cerebral amyloid angiopathy

Charidimou, Andreas; Jäger, Hans Rolf; Fox, Zoë; Peeters, André; Vandermeeren, Yves; Laloux, Patrice; Baron, Jean‐Claude; Werring, David J.

doi:10.1212/wnl.0b013e3182a08f2c

Cited by 114 publications

(128 citation statements)

References 26 publications

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“…13 Another recent study found cSS in 40% of patients with a clinic-radiologic diagnosis of probable CAArelated ICH, but only 5% of patients with purely deep ICH, presumed to be due to hypertensive arteriopathy. 25 Increasing data support the hypothesis that cSS might precede lobar ICH in patients with CAA. 15,[26][27][28] In a recent retrospective study, 51 patients with cSS and no apparent cause other than CAA were identified through a single-center systematic database search and followed up for a median of 35.3 months.…”

Section: Resultsmentioning

confidence: 80%

“…29 The finding of cSS without previous ICH, and its tendency to occur distantly from previous ICH, favor this "primary" mechanism, 13,15 rather than leakage of blood into the subarachnoid space secondary to previous lobar "macro" ICH. 25 Consequently, cSS may be a marker of increased cortical and leptomeningeal small-vessel Figure 2 Time to symptomatic lobar ICH during follow-up…”

Section: Resultsmentioning

confidence: 99%

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Cortical superficial siderosis and intracerebral hemorrhage risk in cerebral amyloid angiopathy

et al. 2013

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Objective: To investigate whether cortical superficial siderosis (cSS) on MRI, especially if disseminated (involving more than 3 sulci), increases the risk of future symptomatic lobar intracerebral hemorrhage (ICH) in cerebral amyloid angiopathy (CAA).Methods: European multicenter cohort study of 118 patients with CAA (104 with baseline symptomatic lobar ICH) diagnosed according to the Boston criteria. We obtained baseline clinical, MRI, and follow-up data on symptomatic lobar ICH. Using Kaplan-Meier and Cox regression analyses, we investigated cSS and ICH risk, adjusting for known confounders.Results: During a median follow-up time of 24 months (interquartile range 9-44 months), 23 of 118 patients (19.5%, 95% confidence interval [CI]: 12.8%-27.8%) experienced symptomatic lobar ICH. Any cSS and disseminated cSS were predictors of time until first or recurrent ICH (logrank test: p 5 0.0045 and p 5 0.0009, respectively). ICH risk at 4 years was 25% (95% CI: 7.6%-28.3%) for patients without siderosis; 28.9% (95% CI: 7.7%-76.7%) for patients with focal siderosis; and 74% (95% CI: 44.1%-95.7%) for patients with disseminated cSS (log-rank test: p 5 0.0031). In Cox regression models, any cSS and disseminated cSS were both independently associated with increased lobar ICH risk, after adjusting for $2 microbleeds and age (hazard ratio: 2.53; 95% CI: 1.05-6.15; p 5 0.040 and hazard ratio: 3.16; 95% CI: 1.35-7.43; p 5 0.008, respectively). These results remained consistent in sensitivity analyses including only patients with symptomatic lobar ICH at baseline. Conclusions:Our findings indicate that cSS, particularly if disseminated, is associated with an increased risk of symptomatic lobar ICH in CAA. cSS may help stratify future bleeding risk in CAA, with implications for prognosis and treatment. Neurology ® 2013;81:1666-1673 GLOSSARY CAA 5 cerebral amyloid angiopathy; CI 5 confidence interval; CMB 5 cerebral microbleed; cSS 5 cortical superficial siderosis; FLAIR 5 fluid-attenuated inversion recovery; GRE 5 gradient-recalled echo; HR 5 hazard ratio; ICH 5 intracerebral hemorrhage.Sporadic cerebral amyloid angiopathy (CAA) is a highly prevalent, age-related small-vessel disease 1 caused by amyloid-b deposition in cortical and leptomeningeal vessel walls. 2 CAA is a major cause of lobar intracerebral hemorrhage (ICH), particularly in elderly patients.2-5 Spontaneous ICH is one of the most catastrophic forms of stroke, with a high risk of recurrence [6][7][8] ; CAA-related lobar ICH may carry a greater risk than deep ICH presumed to be due to hypertensive arteriopathy, 8,9 but this is currently difficult to predict. Predisposing factors for lobar ICH and lobar ICH recurrence in CAA include APOE e4 and e2 alleles, 10 hemorrhagic neuroimaging markers of CAA such as lobar cerebral microbleeds (CMBs), 11 and anticoagulant or antiplatelet use. 12 Little is known about cortical superficial siderosis (cSS), a recently identified neuroimaging marker of CAA, 13 and the risk of subsequent

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Section: Resultsmentioning

confidence: 80%

Section: Resultsmentioning

confidence: 99%

Cortical superficial siderosis and intracerebral hemorrhage risk in cerebral amyloid angiopathy

et al. 2013

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“…Larger studies could examine the influence of biomarkers of CAA 82,83 and other causes of ICH 84 on outcome and recurrent ICH. Because survivors of ICH have higher mortality in comparison with the general population 85 and given the apparently higher risk of ICH recurrence after lobar ICH ( Figure I in the online-only Data Supplement), 21 treatments directed at the specific small vessel diseases underlying ICH, such as CAA, may improve outcome (Study Evaluating the Safety, Tolerability and Efficacy of PF-04360365 in Adults With Probable Cerebral Amyloid Angiopathy, NCT01821118).…”

Section: Discussionmentioning

confidence: 99%

Influence of Intracerebral Hemorrhage Location on Incidence, Characteristics, and Outcome

Samarasekera

Fonville

Lerpiniere

et al. 2015

Stroke

155

106

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Background and Purpose-The characteristics of intracerebral hemorrhage (ICH) may vary by ICH location because of differences in the distribution of underlying cerebral small vessel diseases. Therefore, we investigated the incidence, characteristics, and outcome of lobar and nonlobar ICH. Methods-In a population-based, prospective inception cohort study of ICH, we used multiple overlapping sources of case ascertainment and follow-up to identify and validate ICH diagnoses in 2010 to 2011 in an adult population of 695 335. Results-There were 128 participants with first-ever primary ICH. The overall incidence of lobar ICH was similar to nonlobar ICH (9.

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“…This prevalence was higher than that in the general population (0.7%) but lower than in patients with cognitive impairment (6.1%) and patients with ICH because of probable CAA (≈40%). [19][20][21][22] CAA is proposed to be a cause of TFNE, which mimics the symptoms of TIA but is associated with high hemorrhage risk rather than the risk of recurrent ischemic stroke. Therefore, recognizing this syndrome is important to avoid escalating antithrombotic treatment in patients at a risk of hemorrhage.…”

Section: Discussionmentioning

confidence: 99%

Cerebral Microbleeds and Cortical Superficial Siderosis in Patients Presenting With Minor Cerebrovascular Events

Zerna

Modi

Bilston

et al. 2016

Stroke

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Background and Purpose-Transient focal neurological episodes occur in cerebral amyloid angiopathy (CAA) and can mimic transient ischemic attack (TIA). Risk factors and outcomes of minor ischemic stroke or TIA might differ in patients with and without cerebral microbleeds (CMBs), including CAA-consistent lobar CMB. Methods-Baseline magnetic resonance imaging (MRI) was analyzed for CMBs and cortical superficial siderosis in 416 patients in the prospective computed tomography and MRI in the CATCH study (Triage of TIA and Minor Cerebrovascular Events to Identify High Risk Patients). Clinical symptoms, baseline characteristics, recurrence, and 90-day modified Rankin Scale were prospectively collected. MRI white-matter hyperintensity was measured using the Fazekas scale. Results-CMBs were detected in 65 (15.6%) and cortical superficial siderosis in 11 patients (2.6%). Lobar CMBs were present in 49 (11.8% Cerebral Microbleeds and Cortical Superficial Siderosis in Patients Presenting With Minor Cerebrovascular Events

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Prevalence and mechanisms of cortical superficial siderosis in cerebral amyloid angiopathy

Cited by 114 publications

References 26 publications

Cortical superficial siderosis and intracerebral hemorrhage risk in cerebral amyloid angiopathy

Cortical superficial siderosis and intracerebral hemorrhage risk in cerebral amyloid angiopathy

Influence of Intracerebral Hemorrhage Location on Incidence, Characteristics, and Outcome

Cerebral Microbleeds and Cortical Superficial Siderosis in Patients Presenting With Minor Cerebrovascular Events

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