Cortical superficial siderosis

Neurology ◽  
2018 ◽  
Vol 91 (2) ◽  
pp. e132-e138 ◽  
Author(s):  
Solène Moulin ◽  
Barbara Casolla ◽  
Grégory Kuchcinski ◽  
Gregoire Boulouis ◽  
Costanza Rossi ◽  
...  
Keyword(s):  
White Matter Hyperintensities ◽  
Multivariable Analysis ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Clinical Factors ◽  
Cox Models ◽  
History Of ◽  
Observational Cohort ◽  
Cortical Superficial Siderosis

ObjectiveTo determine the prevalence of cortical superficial siderosis (cSS), its clinical and neuroimaging associated markers, and its influence on the risk of recurrent intracerebral hemorrhage (ICH) in a prospective observational ICH cohort.MethodsWe investigated clinical and radiologic markers associated with cSS using multivariable analysis. In survival analyses, we used Cox models to identify predictors of recurrent ICH after adjusting for potential confounders.ResultsOf the 258 patients included in the study, 49 (19%; 95% confidence interval [CI] 14%–24%) had cSS at baseline. Clinical factors independently associated with the presence of cSS were increasing age (odds ratio [OR] 1.03 per 1-year increase, 95% CI 1.001–1.06, p = 0.044), preexisting dementia (OR 2.62, 95% CI 1.05–6.51, p = 0.039), and history of ICH (OR 4.02, 95% CI 1.24–12.95, p = 0.02). Among radiologic biomarkers, factors independently associated with the presence of cSS were ICH lobar location (OR 24.841, 95% CI 3.2–14.47, p < 0.001), severe white matter hyperintensities score (OR 5.51, 95% CI 1.17–5.78, p = 0.019), and absence of lacune (OR 4.46, 95% CI 1.06–5.22, p = 0.035). During a median follow-up of 6.4 (interquartile range 2.9–8.4) years, recurrent ICH occurred in 19 patients. Only disseminated cSS (hazard ratio 4.69, 95% CI 1.49–14.71, p = 0.008), not the presence or absence of cSS or focal cSS on baseline MRI, was associated with recurrent symptomatic ICH.ConclusionIn a prospective observational cohort of spontaneous ICH, clinical and radiologic markers associated with cSS suggest the implication of underlying cerebral amyloid angiopathy. Disseminated cSS may become a key prognostic neuroimaging marker of recurrent ICH that could be monitored in future clinical trials dedicated to patients with ICH.

scholarly journals CT-visible convexity subarachnoid hemorrhage is associated with cortical superficial siderosis and predicts recurrent ICH

Neurology ◽  
2020 ◽  
pp. 10.1212/WNL.0000000000011052
Author(s):  
Qi Li ◽  
Maria Clara Zanon Zotin ◽  
Andrew D. Warren ◽  
Yuan Ma ◽  
Edip Gurol ◽  
...  
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Recurrent Stroke ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Cox Proportional Hazard ◽  
Increased Risk ◽  
History Of ◽  
Cox Proportional Hazard Models ◽  
Cortical Superficial Siderosis

Objective:To investigate whether acute convexity subarachnoid hemorrhage (cSAH) detected on CT in lobar intracerebral hemorrhage (ICH) related to cerebral amyloid angiopathy (CAA) is associated with recurrent ICH.Methods:We analyzed data from a prospective cohort of consecutive acute lobar ICH survivors fulfilling the Boston criteria for possible or probable CAA who had both brain CT and MRI at index ICH. Presence of cSAH was assessed on CT blinded to MRI data. Cortical superficial siderosis (cSS), cerebral microbleeds and white matter hyperintensities were evaluated on MRI. Cox proportional hazard models were used to assess the association between cSAH and the risk of recurrent symptomatic ICH during follow-up.Results:A total of 244 ICH survivors (76.4 ± 8.7 years; 54.5% female) were included. cSAH was observed on baseline CT in 99 patients (40.5%). Presence of cSAH was independently associated with cSS, hematoma volume and pre-existing dementia. During a median follow-up of 2.66 years, 49 patients (20.0%) had a recurrent symptomatic ICH. Presence of cSAH was associated with recurrent ICH (hazard ratio [HR] 2.64; 95% CI 1.46-4.79; p=0.001), after adjusting for age, antiplatelet use, warfarin use, history of previous ICH.Conclusion:cSAH was detected on CT in 40.5% of patients with acute lobar ICH related to CAA and heralds an increased risk of recurrent ICH. This CT marker may be widely used to stratify the ICH risk in patients with CAA.Classification of evidence:This study provides Class II evidence that cSAH accurately predicts recurrent stroke in patients with CAA.

Abstract WMP102: Prevalence and Natural History of Superficial Siderosis: A Population-based Study

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Michael Pichler ◽  
Kelly Flemming ◽  
Alejandro Rabinstein ◽  
Robert Brown ◽  
Kejal Kantarci ◽  
...  
Keyword(s):  
Population Based ◽  
Superficial Siderosis ◽  
Mri Findings ◽  
Population Based Study ◽  
Mri Scans ◽  
History Of ◽  
Lobar Hemorrhage ◽  
General Elderly Population ◽  
Cortical Superficial Siderosis

Introduction: Cortical superficial siderosis (cSS) refers to deposition of blood breakdown products along the cerebral cortex, causing characteristic staining patterns seen with iron-sensitive MRI techniques. Cortical superficial siderosis is a relatively rare disorder, but has been linked to cerebral amyloid angiopathy and Alzheimer’s disease. The objective of this study was to determine the frequency and natural history of cSS in the general elderly population. Methods: MRI scans from the Mayo Clinic Study of Aging (MCSA), an ongoing population-based study of elderly residents in Olmsted County, Minnesota, were reviewed by neuroradiologists. Participants with cSS were identified based on linear pattern of hypointensity on gradient recalled echo imaging consistent with cSS. Exclusion criteria were: 1) MRI findings not consistent with cSS or 2) alternative explanation for MRI findings (such as aneurysmal subarachnoid hemorrhage, intracranial surgery, or trauma). Additional data abstracted included extent of cSS, presence of cerebral microbleeds, and clinical outcome. Results: Eleven out of 1,441 participants had MRI scans showing cSS (0.8%). When stratified by age, the frequency was 0.4% in those 50 to 70 years old and 1.1% in those over 70 years old. Six participants had only focal involvement of cSS (restricted to three or fewer sulci) and five had disseminated involvement (affecting more than three sulci). Microbleeds were seen in four of five (80%) participants with disseminated cSS, but none with focal cSS. Five participants (2 focal, 3 disseminated cSS) had follow up MRI scans, with an average follow up of 25 months. There was no further hemorrhage in those with focal cSS. However, all three participants with disseminated cSS experienced additional hemorrhage: one with new microbleeds, one with new microbleeds and lobar hemorrhage, and one with sulcal subarachnoid hemorrhage and lobar hemorrhage. Conclusion: Although rare, cSS may be encountered in the general elderly population. Extent of involvement of cSS and concomitant microbleeds may be important risk factors for progression of disease and intracerebral hemorrhage. The clinical significance of focal cSS occurring in the absence of microbleeds requires further investigation.

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.

scholarly journals Convexity subarachnoid hemorrhage in lobar intracerebral hemorrhage

Neurology ◽  
2020 ◽  
Vol 94 (9) ◽  
pp. e968-e977 ◽  
Author(s):  
Nicolas Raposo ◽  
Andreas Charidimou ◽  
Duangnapa Roongpiboonsopit ◽  
Michelle Onyekaba ◽  
M. Edip Gurol ◽  
...  
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Intracerebral Hemorrhage ◽  
Regression Models ◽  
Cox Regression ◽  
Bleeding Risk ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Increased Risk ◽  
Cortical Superficial Siderosis

ObjectiveTo investigate whether acute convexity subarachnoid hemorrhage (cSAH) associated with acute lobar intracerebral hemorrhage (ICH) increases the risk of ICH recurrence in patients with cerebral amyloid angiopathy (CAA).MethodsWe analyzed data from a prospective cohort of consecutive survivors of acute spontaneous lobar ICH fulfilling the Boston criteria for possible or probable CAA (CAA-ICH). We analyzed baseline clinical and MRI data, including cSAH (categorized as adjacent or remote from ICH on a standardized scale), cortical superficial siderosis (cSS), and other CAA MRI markers. Multivariable Cox regression models were used to assess the association between cSAH and recurrent symptomatic ICH during follow-up.ResultsWe included 261 CAA-ICH survivors (mean age 76.2 ± 8.7 years). Of them, 166 (63.6%, 95% confidence interval [CI] 57.7%–69.5%) had cSAH on baseline MRI. During a median follow-up of 28.3 (interquartile range 7.2–57.0) months, 54 (20.7%) patients experienced a recurrent lobar ICH. In Cox regression, any cSAH, adjacent cSAH, and remote cSAH were independent predictors of recurrent ICH after adjustment for other confounders, including cSS. Incidence rate of recurrent ICH in patients with cSAH was 9.9 per 100 person-years (95% CI 7.3–13.0) compared with 1.2 per 100 person-years (95% CI 0.3–3.2) in those without cSAH (adjusted hazard ratio 7.5, 95% CI 2.6–21.1).ConclusionIn patients with CAA-related acute ICH, cSAH (adjacent or remote from lobar ICH) is commonly observed and heralds an increased risk of recurrent ICH. cSAH may help stratify bleeding risk and should be assessed along with cSS for prognosis and clinical management.

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 MRI predicts intracranial hemorrhage in patients who receive long-term oral anticoagulation

Neurology ◽  
2019 ◽  
Vol 92 (21) ◽  
pp. e2432-e2443 ◽  
Author(s):  
Joan Martí-Fàbregas ◽  
Santiago Medrano-Martorell ◽  
Elisa Merino ◽  
Luis Prats-Sánchez ◽  
Rebeca Marín ◽  
...  
Keyword(s):  
Ischemic Stroke ◽  
White Matter ◽  
Intracranial Hemorrhage ◽  
White Matter Hyperintensities ◽  
Cox Regression ◽  
Multivariable Analysis ◽  
Hazard Ratio ◽  
Superficial Siderosis ◽  
Increased Risk

ObjectiveWe tested the hypothesis that the risk of intracranial hemorrhage (ICH) in patients with cardioembolic ischemic stroke who are treated with oral anticoagulants (OAs) can be predicted by evaluating surrogate markers of hemorrhagic-prone cerebral angiopathies using a baseline MRI.MethodsPatients were participants in a multicenter and prospective observational study. They were older than 64 years, had a recent cardioembolic ischemic stroke, and were new users of OAs. They underwent a baseline MRI analysis to evaluate microbleeds, white matter hyperintensities, and cortical superficial siderosis. We collected demographic variables, clinical characteristics, risk scores, and therapeutic data. The primary endpoint was ICH that occurred during follow-up. We performed bivariate and multivariate Cox regression analyses.ResultsWe recruited 937 patients (aged 77.6 ± 6.5 years; 47.9% were men). Microbleeds were detected in 207 patients (22.5%), moderate/severe white matter hyperintensities in 419 (45.1%), and superficial siderosis in 28 patients (3%). After a mean follow-up of 23.1 ± 6.8 months, 18 patients (1.9%) experienced an ICH. In multivariable analysis, microbleeds (hazard ratio 2.7, 95% confidence interval [CI] 1.1–7, p = 0.034) and moderate/severe white matter hyperintensities (hazard ratio 5.7, 95% CI 1.6–20, p = 0.006) were associated with ICH (C index 0.76, 95% CI 0.66–0.85). Rate of ICH was highest in patients with both microbleed and moderate/severe WMH (3.76 per 100 patient-years, 95% CI 1.62–7.4).ConclusionPatients taking OAs who have advanced cerebral small vessel disease, evidenced by microbleeds and moderate to severe white matter hyperintensities, had an increased risk of ICH. Our results should help to determine the risk of prescribing OA for a patient with cardioembolic stroke.ClinicalTrials.gov identifierNCT02238470.

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.

Cortical superficial siderosis and recurrent intracerebral hemorrhage risk in cerebral amyloid angiopathy: Large prospective cohort and preliminary meta-analysis

2019 ◽  
Vol 14 (7) ◽  
pp. 723-733 ◽  
Author(s):  
Andreas Charidimou ◽  
Gregoire Boulouis ◽  
Duangnapa Roongpiboonsopit ◽  
Li Xiong ◽  
Marco Pasi ◽  
...  
Keyword(s):  
Intracerebral Hemorrhage ◽  
Prospective Cohort ◽  
Cox Regression ◽  
Meta Analysis ◽  
Recurrence Risk ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Cerebral Amyloid ◽  
Cortical Superficial Siderosis

Background We aimed to investigate cortical superficial siderosis as an MRI predictor of lobar intracerebral hemorrhage (ICH) recurrence risk in cerebral amyloid angiopathy (CAA), in a large prospective MRI cohort and a systematic review. Methods We analyzed a single-center MRI prospective cohort of consecutive CAA-related ICH survivors. Using Kaplan–Meier and Cox regression analyses, we investigated cortical superficial siderosis and ICH risk, adjusting for known confounders. We pooled data with eligible published cohorts in a two-stage meta-analysis using random effects models. Covariate-adjusted hazard rations (adj-HR) from pre-specified multivariable Cox proportional hazard models were used. Results The cohort included 240 CAA-ICH survivors (cortical superficial siderosis prevalence: 36%). During a median follow-up of 2.6 years (IQR: 0.9–5.1 years) recurrent ICH occurred in 58 patients (24%). In prespecified multivariable Cox regression models, cortical superficial siderosis presence and disseminated cortical superficial siderosis were independent predictors of increased symptomatic ICH risk at follow-up (HR: 2.26; 95% CI: 1.31–3.87, p = 0.003 and HR: 3.59; 95% CI: 1.96–6.57, p < 0.0001, respectively). Three cohorts including 443 CAA-ICH patients in total were eligible for meta-analysis. During a mean follow-up of 2.5 years (range: 2–3 years) 92 patients experienced recurrent ICH (pooled risk ratio: 6.9% per year, 95% CI: 4.2%–9.7% per year). In adjusted pooled analysis, any cortical superficial siderosis and disseminated cortical superficial siderosis were the only independent predictors associated with increased lobar ICH recurrence risk (adj-HR: 2.4; 95% CI: 1.5–3.7; p < 0.0001, and adj-HR: 4.4; 95% CI: 2–9.9; p < 0.0001, respectively). Conclusions In CAA-ICH patients, cortical superficial siderosis presence and extent are the most important MRI prognostic risk factors for lobar ICH recurrence. These results can help guide clinical decision making in patients with CAA.

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 in Memory Clinic Patients: Further Evidence for Underlying Cerebral Amyloid Angiopathy

2016 ◽  
Vol 41 (3-4) ◽  
pp. 156-162 ◽  
Author(s):  
Andreas Charidimou ◽  
Jun Ni ◽  
Sergi Martinez-Ramirez ◽  
Anastasia Vashkevich ◽  
Alison Ayres ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
White Matter ◽  
Cerebral Amyloid Angiopathy ◽  
White Matter Hyperintensities ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Perivascular Spaces ◽  
Memory Clinic ◽  
Cerebral Amyloid ◽  
Cortical Superficial Siderosis

Background: Cerebral amyloid angiopathy (CAA) is associated with many cases of spontaneous symptomatic lobar intracerebral haemorrhage in older individuals and is emerging as an important contributor to cognitive impairment. Cortical superficial siderosis (cSS) is an increasingly recognized haemorrhagic neuroimaging manifestation of CAA. We sought to investigate its prevalence and its association with underlying CAA among memory clinic patients. Methods: We included consecutive eligible patients who presented to the out-patient memory clinic at the Massachusetts General Hospital from 2007 to 2010 and had appropriate MRI, including blood-sensitive sequences. We analyzed the prevalence and topography of cSS according to demographic, clinical, APOE and MRI data. Results: Our cohort consisted of 339 memory clinic patients: Alzheimer's disease (n = 86); mild cognitive impairment (n = 162); vascular dementia/mixed dementia (n = 18); other dementia/undetermined (n = 42); and subjective cognitive complains (n = 31). cSS was detected in 10 patients (3%; 95% CI 1.4-5.4): in 7 cases cSS was focal and in 3 cases, it was disseminated. In multivariable logistic regression analysis, the presence of cSS was associated with lobar microbleeds (OR 1.08; 95% CI 1.03-1.13; p = 0.001, per each additional microbleed) and severe white matter hyperintensities (Fazekas score 5-6, OR 4.43; 95% CI 1.21-26.28; p = 0.028) after adjusting for age. These associations were not influenced by the clinical diagnosis. In patients with APOE data, the APOE ε4/ε4 genotype was overrepresented among subjects with vs. without cSS. In the subgroup of patients with probable CAA (n = 68; 9 with cSS) based on the presence of strictly lobar microbleeds, cSS was also associated with a higher prevalence of severe white matter hyperintensities (66.7 vs. 10.2%; p = 0.001), high centrum semiovale perivascular spaces burden (88.9 vs. 52.4%; p = 0.041) and higher counts of lobar microbleeds (median 13; IQR 10-36 vs. median 1; IQR 1-2; p < 0.00001), compared to patients without cSS. Conclusions: Our data provide further evidence supporting the hypothesis that cSS is a manifestation of advanced CAA in memory clinic populations. Future longitudinal studies should explore any direct effect of cSS on cognition or haemorrhage risk and disease progression.

Sign in / Sign up

Export Citation Format

Share Document