scholarly journals Cortical superficial siderosis multifocality in cerebral amyloid angiopathy

Neurology ◽  
2017 ◽  
Vol 89 (21) ◽  
pp. 2128-2135 ◽  
Author(s):  
Andreas Charidimou ◽  
Gregoire Boulouis ◽  
Duangnapa Roongpiboonsopit ◽  
Eitan Auriel ◽  
Marco Pasi ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Interrater Reliability ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Recurrence Rates ◽  
Rating Method ◽  
Cerebral Amyloid ◽  
Annual Risk ◽  
Cortical Superficial Siderosis

Objective:In order to explore the mechanisms of cortical superficial siderosis (cSS) multifocality and its clinical implications for recurrent intracerebral hemorrhage (ICH) risk in patients with cerebral amyloid angiopathy (CAA), we used a new rating method that we developed specifically to evaluate cSS extent at spatially separated foci.Methods:Consecutive patients with CAA-related ICH according to Boston criteria from a single-center prospective cohort were analyzed. The new score that assesses cSS multifocality (total range 0–4) showed excellent interrater reliability (k = 0.87). The association of cSS with markers of CAA and acute ICH was investigated. Patients were followed prospectively for recurrent symptomatic ICH.Results:The cohort included 313 patients with CAA. Multifocal cSS prevalence was 21.1%. APOE ε2 allele prevalence was higher in patients with multifocal cSS. In probable/definite CAA, cSS multifocality was independently associated with neuroimaging markers of CAA severity, including lobar microbleeds, but not with acute ICH features, which conversely, were determinants of cSS in possible CAA. During a median follow-up of 2.6 years (interquartile range 0.9–5.1 years), the annual ICH recurrence rates per cSS scores (0–4) were 5%, 6.5%, 13.5%, 16.2%, and 26.9%, respectively. cSS multifocality (presence and spread) was the only independent predictor of increased symptomatic ICH risk (hazard ratio 3.19; 95% confidence interval 1.77–5.75; p < 0.0001).Conclusions:The multifocality of cSS correlates with disease severity in probable CAA; therefore cSS is likely to be caused by discrete hemorrhagic foci. The new cSS scoring system might be valuable for clinicians in determining annual risk of ICH recurrence.

scholarly journals Cortical superficial siderosis and bleeding risk in cerebral amyloid angiopathy

Neurology ◽  
2019 ◽  
Vol 93 (24) ◽  
pp. e2192-e2202 ◽  
Author(s):  
Andreas Charidimou ◽  
Gregoire Boulouis ◽  
Steven M. Greenberg ◽  
Anand Viswanathan
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Meta Analysis ◽  
Bleeding Risk ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Class Iii ◽  
Cerebral Amyloid ◽  
T2 Mri ◽  
Cortical Superficial Siderosis

ObjectiveTo assess the association of cortical superficial siderosis (cSS) presence and extent with future bleeding risk in cerebral amyloid angiopathy (CAA).MethodsThis was a meta-analysis of clinical cohorts of symptomatic patients with CAA who had T2*-MRI at baseline and clinical follow-up for future intracerebral hemorrhage (ICH). We pooled data in a 2-stage meta-analysis using random effects models. Covariate-adjusted hazard ratios (adjHR) from multivariable Cox proportional hazard models were used.ResultsWe included data from 6 eligible studies (n = 1,239). cSS pooled prevalence was 34% (95% confidence interval [CI] 26%–41%; I2 87.94%; p < 0.001): focal cSS prevalence was 14% (95% CI 12%–16%; I2 6.75%; p = 0.37), and disseminated cSS prevalence was 20% (95% CI 13%–26%; I2 90.39%; p < 0.001). During a mean follow-up of 3.1 years (range 1–4 years), 162/1,239 patients experienced a symptomatic ICH-pooled incidence rate 6.9% per year (95% CI 3.9%–9.8% per year; I2 83%; p < 0.001). ICH incidence rates per year according to cSS status were 3.9% (95% CI 1.7%–6.1%; I2 70%; p = 0.018) for patients without cSS, 11.1% (95% CI 7%–15.2%; I2 56.8%; p = 0.074) for cSS presence, 9.1% (95% CI 5.5%–12.8%; I2 0%; p = 0.994) for focal cSS, and 12.5% (95% CI 5.3%–19.7%; I2 73.2%; p = 0.011) for disseminated cSS. In adjusted pooled analysis, any cSS presence was independently associated with increased future ICH risk (adjHR 2.14; 95% CI 1.19–3.85; p < 0.0001). Focal cSS was linked with ICH risk (adjHR 2.11; 95% CI 1.31–2.41; p = 0.002), while disseminated cSS conferred the strongest bleeding risk (adjHR 4.28; 95% CI 2.91–6.30; p < 0.0001).ConclusionIn patients with CAA, cSS presence and extent are the most important MRI prognostic risk factors for future ICH, likely useful in treatment planning.Classification of evidenceThis study provides Class III evidence that in symptomatic CAA survivors with baseline T2*-MRI, cSS (particularly if disseminated, i.e., affecting >3 sulci) increases the risk of future ICH.

Abstract 212: Cortical Superficial Siderosis and Risk of Recurrent Intracerebral Haemorrhage in Cerebral Amyloid Angiopathy: A Meta-analysis

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Andreas Charidimou ◽  
Gregoire Boulouis ◽  
Solene Moulin ◽  
Duangnapa Roongpiboonsopit ◽  
Nicolas Raposo ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Meta Analysis ◽  
Large Population ◽  
Recurrence Risk ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Increased Risk ◽  
Cerebral Amyloid ◽  
Cortical Superficial Siderosis

Background: Cerebral amyloid angiopathy (CAA) is a major cause of spontaneous lobar intracerebral hemorrhage (ICH) in the elderly. CAA-related ICH survivors are at substantial risk for recurrent ICH, accounting for the significant morbidity of the disease. Identifying predictors of recurrence is therefore crucial. Recent data have implicated cortical superficial siderosis (cSS) as a key hemorrhagic MRI signature of CAA, and a possible marker of increased risk for CAA-ICH recurrence. However, data remain limited. We obtained precise estimates on cSS as an independent predictor of ICH recurrence risk in CAA cohorts from a systematic review of published studies pooled with data from our centre. Methods: We included cohort studies of consecutive CAA-related ICH patients based on the original Boston criteria, with available blood-sensitive MRI sequences at baseline for cSS assessment, and adequate follow-up for recurrent symptomatic ICH. The strength of the association between cSS and recurrent ICH was quantified using random effects models. Covariate-adjusted hazard rations (adj-HR) as provided from pre-specified Cox proportional hazard models were used for a two-stage meta-analysis. Results: Three cohorts including 443 CAA-ICH patients were eligible for analysis. The pooled prevalence of cSS presence and disseminated cSS (>3 affected sulci) was 32% (95%CI: 32%-41%) and 21% (95%CI: 18%-25%) respectively. During a mean follow-up of 2.5 years (range: 2-3 years) 92 patients experienced recurrent ICH, a pooled risk ratio of 6.9% per year (I 2 : 63%, p=0.07). In adjusted pooled analysis, any cSS and disseminated cSS were both independently associated with increased lobar ICH recurrence risk (adj-HR: 2.4; 95%CI: 1.5-3.8; p<0.0001, I 2 : 0% and adj-HR: 4.1; 95%CI: 2.6-6.6; p<0.0001, I 2 : 47%), after adjusting for multiple strictly lobar microbleeds presence and increasing age. Conclusions: Our findings in a large population of CAA patients with ICH and a large number of recurrence events, indicate that cSS, particularly if disseminated, is the single most important prognostic risk factor on MRI for future recurrent lobar ICH. The provided estimates may help stratify future bleeding risk in CAA, with clinical implications for prognosis and treatment.

scholarly journals Evolution of DWI lesions in cerebral amyloid angiopathy

Neurology ◽  
2017 ◽  
Vol 89 (21) ◽  
pp. 2136-2142 ◽  
Author(s):  
Susanne J. van Veluw ◽  
Arne Lauer ◽  
Andreas Charidimou ◽  
Narimene Bounemia ◽  
Li Xiong ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Small Vessel Disease ◽  
Hemorrhagic Transformation ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Cross Sectional ◽  
Small Diffusion ◽  
Cerebral Amyloid ◽  
Cortical Superficial Siderosis

Objective:To address the pathophysiologic nature of small diffusion-weighted imaging (DWI) lesions in patients with cerebral amyloid angiopathy (CAA) who underwent serial MRI. Specifically, we tested (1) whether DWI lesions occurred preferentially in individuals with prior DWI lesions, (2) the cross-sectional association with chronic cortical cerebral microinfarcts (CMIs), and (3) the evolution of DWI lesions over time.Methods:Patients with probable CAA (n = 79) who underwent at least 2 MRI sessions were included. DWI lesions were assessed at each available time point. Lesion appearance and characteristics were assessed on available structural follow-up images. Presence and burden of other neuroimaging markers of small vessel disease (white matter hyperintensities, cerebral microbleeds, cortical superficial siderosis, and chronic cortical CMIs) were assessed as well.Results:Among 221 DWI scans (79 patients with 2 DWI scans; 40 with ≥3), 60 DWI lesions were found in 28 patients. Patients with DWI lesions at baseline were not more likely to have additional DWI lesions on follow-up compared to patients without DWI lesions at baseline. DWI lesions were associated with chronic cortical CMIs and cortical superficial siderosis, but not with other markers. For 39/60 DWI lesions, >1 MRI sequence was available at follow-up to determine lesion evolution. Twenty-four (62%) were demarcated as chronic lesions on follow-up MRI. Five appeared as cavitations, 18 as noncavitated infarcts, and 1 underwent hemorrhagic transformation.Conclusions:Based on their neuroimaging signature as well as their association with chronic cortical CMIs, DWI lesions appear to have an ischemic origin and represent one part of the CMI spectrum.

scholarly journals Cortical superficial siderosis and first-ever cerebral hemorrhage in cerebral amyloid angiopathy

Neurology ◽  
2017 ◽  
Vol 88 (17) ◽  
pp. 1607-1614 ◽  
Author(s):  
Andreas Charidimou ◽  
Gregoire Boulouis ◽  
Li Xiong ◽  
Michel J. Jessel ◽  
Duangnapa Roongpiboonsopit ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Cox Regression ◽  
Small Vessel Disease ◽  
Rank Test ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Increased Risk ◽  
Cerebral Amyloid ◽  
Cortical Superficial Siderosis

Objective:To investigate whether cortical superficial siderosis (cSS) is associated with increased risk of future first-ever symptomatic lobar intracerebral hemorrhage (ICH) in patients with cerebral amyloid angiopathy (CAA) presenting with neurologic symptoms and without ICH.Methods:Consecutive patients meeting modified Boston criteria for probable CAA in the absence of ICH from a single-center cohort were analyzed. cSS and other small vessel disease MRI markers were assessed according to recent consensus recommendations. Patients were followed prospectively for future incident symptomatic lobar ICH. Prespecified Cox proportional hazard models were used to investigate cSS and first-ever lobar ICH risk adjusting for potential confounders.Results:The cohort included 236 patients with probable CAA without lobar ICH at baseline. cSS prevalence was 34%. During a median follow-up of 3.26 years (interquartile range 1.42–5.50 years), 27 of 236 patients (11.4%) experienced a first-ever symptomatic lobar ICH. cSS was a predictor of time until first ICH (p = 0.0007, log-rank test). The risk of symptomatic ICH at 5 years of follow-up was 19% (95% confidence interval [CI] 11%–32%) for patients with cSS at baseline vs 6% (95% CI 3%–12%) for patients without cSS. In multivariable Cox regression models, cSS presence was the only independent predictor of increased symptomatic ICH risk during follow-up (HR 4.04; 95% CI 1.73–9.44, p = 0.001), after adjusting for age, lobar cerebral microbleeds burden, and white matter hyperintensities.Conclusions:cSS is consistently associated with an increased risk of future lobar ICH in CAA with potentially important clinical implications for patient care decisions such as antithrombotic use.

scholarly journals Cortical Superficial Siderosis Evolution

Stroke ◽  
2019 ◽  
Vol 50 (4) ◽  
pp. 954-962 ◽  
Author(s):  
Andreas Charidimou ◽  
Gregoire Boulouis ◽  
Li Xiong ◽  
Marco Pasi ◽  
Duangnapa Roongpiboonsopit ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Odds Ratio ◽  
Interquartile Range ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Cox Proportional Hazard Models ◽  
Cerebral Amyloid ◽  
Magnetic Resonance Imaging Mri ◽  
Cortical Superficial Siderosis

Background and Purpose— We investigated cortical superficial siderosis (cSS) progression and its clinical relevance for incident lobar intracerebral hemorrhage (ICH) risk, in probable cerebral amyloid angiopathy presenting with neurological symptoms and without ICH at baseline. Methods— Consecutive patients meeting modified Boston criteria for probable cerebral amyloid angiopathy from a single-center cohort who underwent magnetic resonance imaging (MRI) at baseline and during follow-up were analyzed. cSS progression was assessed by comparison of the baseline and follow-up images. Patients were followed prospectively for incident symptomatic ICH. cSS progression and first-ever ICH risk were investigated in Cox proportional hazard models adjusting for confounders. Results— The cohort included 118 probable cerebral amyloid angiopathy patients: 72 (61%) presented with transient focal neurological episodes and 46 (39%) with cognitive complaints prompting the baseline MRI investigation. Fifty-two patients (44.1%) had cSS at baseline. During a median scan interval of 2.2 years (interquartile range, 1.2–4.4 years) between the baseline (ie, first) MRI and the latest MRI, cSS progression was detected in 33 (28%) patients. In multivariable logistic regression, baseline cSS presence (odds ratio, 4.04; 95% CI, 1.53–10.70; P =0.005), especially disseminated cSS (odds ratio, 9.12; 95% CI, 2.85–29.18; P <0.0001) and appearance of new lobar microbleeds (odds ratio, 4.24; 95% CI, 1.29–13.9; P =0.017) were independent predictors of cSS progression. For patients without an ICH during the interscan interval (n=105) and subsequent follow-up (median postfinal MRI time, 1.34; interquartile range, 0.3–3 years), cSS progression independently predicted increased symptomatic ICH risk (hazard ratio, 3.76; 95% CI, 1.37–10.35; P =0.010). Conclusions— Our results suggest that cSS evolution may be a useful biomarker for assessing disease progression and ICH risk in cerebral amyloid angiopathy patients and a candidate biomarker for clinical studies and trials.

P4-196: Clinical Impacts of Cortical Superficial Siderosis in Patients With Clinically Diagnosed Cerebral Amyloid Angiopathy

2016 ◽  
Vol 12 ◽  
pp. P1099-P1099
Author(s):  
Young Kyoung Jang ◽  
Hee Jin Kim ◽  
Yeo Jin Kim ◽  
Jin San Lee ◽  
Juyoun Lee ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Cerebral Amyloid ◽  
Cortical Superficial Siderosis

scholarly journals Clinical and radiological differences between patients with probable cerebral amyloid angiopathy and mixed cerebral microbleeds

2020 ◽  
Vol 267 (12) ◽  
pp. 3602-3608 ◽  
Author(s):  
Ulf R. Jensen-Kondering ◽  
Caroline Weiler ◽  
Patrick Langguth ◽  
Naomi Larsen ◽  
Charlotte Flüh ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Cerebral Microbleeds ◽  
Superficial Siderosis ◽  
Periventricular White Matter ◽  
Amyloid Angiopathy ◽  
Imaging Features ◽  
Significant Difference ◽  
Comprehensive Comparison ◽  
Cerebral Amyloid ◽  
Cortical Superficial Siderosis

Abstract Background The key imaging features of cerebral amyloid angiopathy (CAA) are lobar, cortical, or cortico-subcortical microbleeds, macrohaemorrhages and cortical superficial siderosis (cSS). In contrast, hypertensive angiopathy is characterized by (micro) haemorrhages in the basal ganglia, thalami, periventricular white matter or the brain stem. Another distinct form of haemorrhagic microangiopathy is mixed cerebral microbleeds (mixed CMB) with features of both CAA and hypertensive angiopathy. The distinction between the two entities (CAA and mixed CMB) is clinically relevant because the risk of haemorrhage and stroke should be well balanced if oral anticoagulation is indicated in CAA patients. We aimed to comprehensively compare these two entities. Methods Patients with probable CAA according to the modified Boston criteria and mixed CMB without macrohaemorrhage were retrospectively identified from our database. Comprehensive comparison regarding clinical and radiological parameters was performed between the two cohorts. Results Patients with CAA were older (78 ± 8 vs. 74 ± 9 years, p = 0.036) and had a higher prevalence of cSS (19% vs. 4%, p = 0.027) but a lower prevalence of lacunes (73% vs. 50%, p = 0.018) and deep lacunes (23% vs. 51%, p = 0.0003) compared to patients with mixed CMB. Logistic regression revealed an association between the presence of deep lacunes and mixed CMB. The other collected parameters did not reveal a significant difference between the two groups. Conclusions CAA and mixed CMB demonstrate radiological differences in the absence of macrohaemorrhages. However, more clinically available biomarkers are needed to elucidate the contribution of CAA and hypertensive angiopathy in mixed CMB patients.

scholarly journals Cortical superficial siderosis progression in cerebral amyloid angiopathy

Neurology ◽  
2020 ◽  
Vol 94 (17) ◽  
pp. e1853-e1865 ◽  
Author(s):  
Thanakit Pongpitakmetha ◽  
Panagiotis Fotiadis ◽  
Marco Pasi ◽  
Gregoire Boulouis ◽  
Li Xiong ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Cerebral Amyloid Angiopathy ◽  
Ordinal Scale ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Cox Regression Analysis ◽  
Multivariable Logistic Regression ◽  
Potential Biomarker ◽  
Cerebral Amyloid ◽  
Cortical Superficial Siderosis

ObjectiveTo investigate the prevalence, predictors, and clinical relevance of cortical superficial siderosis (cSS) progression in cerebral amyloid angiopathy (CAA).MethodsConsecutive patients with symptomatic CAA meeting Boston criteria in a prospective cohort underwent baseline and follow-up MRI within 1 year. cSS progression was evaluated on an ordinal scale and categorized into mild (score 1–2 = cSS extension within an already present cSS focus or appearance of 1 new cSS focus) and severe progression (score 3–4 = appearance of ≥2 new cSS foci). Binominal and ordinal multivariable logistic regression were used to determine cSS progression predictors. We investigated future lobar intracerebral hemorrhage (ICH) risk in survival analysis models.ResultsWe included 79 patients with CAA (mean age, 69.2 years), 56 (71%) with lobar ICH at baseline. cSS progression was detected in 23 (29%) patients: 15 (19%) patients had mild and 8 (10%) severe progression. In binominal multivariable logistic regression, ICH presence (odds ratio [OR], 7.54; 95% confidence interval [CI], 1.75–53.52; p = 0.016) and baseline cSS (OR, 10.41; 95% CI, 2.84–52.83; p = 0.001) were independent predictors of cSS progression. In similar models, presence of disseminated (but not focal) cSS at baseline (OR, 5.58; 95% CI, 1.81–19.41; p = 0.004) was an independent predictor of cSS progression. Results were similar in ordinal multivariable logistic regression models. In multivariable Cox regression analysis, severe cSS progression was independently associated with increased future ICH risk (HR, 5.90; 95% CI, 1.30–26.68; p = 0.021).ConclusionscSS evolution on MRI is common in patients with symptomatic CAA and might be a potential biomarker for assessing disease severity and future ICH risk. External validation of these findings is warranted.

scholarly journals Early MRI imaging and follow-up study in cerebral amyloid angiopathy

Open Medicine ◽  
2021 ◽  
Vol 16 (1) ◽  
pp. 257-263
Author(s):  
Shan-chun Zhang ◽  
Jian-jun Jia ◽  
Heng-li Zhao ◽  
Bo Zhou ◽  
Wei Wang ◽  
...  
Keyword(s):  
Recurrence Rate ◽  
Cerebral Amyloid Angiopathy ◽  
Superficial Siderosis ◽  
Control Group ◽  
Amyloid Angiopathy ◽  
Imaging Features ◽  
Follow Up Study ◽  
Imaging Characteristics ◽  
Cerebral Amyloid

Abstract Aim To study the imaging features of leukoaraiosis (LA) and hemorrhage in cerebral amyloid angiopathy (CAA) patients. Methods The earliest MRI images of probable CAA patients and non-CAA patients were collected. The characteristics of LA in the two groups were analyzed. Cerebral micro bleeding (CMB), superficial siderosis (SS), and intracranial hemorrhage (ICH) were recorded in the follow-up study. The space relationship between CMB or SS and ICH was assessed. Results We found that 10/21 (47.6%) patients had occipital prominent LA and 14/21 (66.7%) patients had subcortical punctate LA before the ICH, which was higher than that of the ones in the control group (p = 0.015 and 0.038, respectively). The recurrence rate of ICH was 100% (3/3) in patients with diffuse SS and 36.4% (4/11) in patients without. The recurrence rate of ICH was 60% (3/5) in patients with multiple-lobe CMBs and 44.4% (4/9) in those without. The location of the ICH and CMB was inconsistent. ICH occurred in the ipsilateral cerebral hemisphere of SS in three patients with diffuse SS. Conclusion LA, diffuse SS, and multiple-lobe CMBs are important imaging characteristics of CAA, which may help make early diagnosis and predict the recurrence of ICH.

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