White Matter Perivascular Spaces Are Related to Cortical Superficial Siderosis in Cerebral Amyloid Angiopathy

Stroke ◽  
2014 ◽  
Vol 45 (10) ◽  
pp. 2930-2935 ◽  
Author(s):  
Andreas Charidimou ◽  
Rolf H. Jäger ◽  
Andre Peeters ◽  
Yves Vandermeeren ◽  
Patrice Laloux ◽  
...  
Keyword(s):  
White Matter ◽  
Cerebral Amyloid Angiopathy ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Perivascular Spaces ◽  
Cerebral Amyloid ◽  
Cortical Superficial Siderosis

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.

scholarly journals Stopping “transient ischemic attacks” by antiplatelet withdrawal

2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Lina Palaiodimou ◽  
Aikaterini Theodorou ◽  
Stefanos Lachanis ◽  
George P. Paraskevas ◽  
Matilda Papathanasiou ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Cerebral Amyloid Angiopathy ◽  
Brain Mri ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Perivascular Spaces ◽  
The Past ◽  
Cerebral Amyloid ◽  
Ischemic Attack ◽  
Cortical Superficial Siderosis

Abstract Introduction Transient ischemic attack (TIA) is considered to be an important risk factor for the development of ischemic stroke and requires complete etiopathogenic evaluation and prompt initiation of secondary prevention treatment. In addition, an accurate differential diagnosis should be performed in order to exclude other disorders mimicking TIA. Methods In this case report, we describe the clinical and neuroimaging evaluation and the differential diagnosis of a patient with suspected crescendo TIAs. Results A 79-year-old man presented with recurrent episodes of right-sided numbness over the past 7 months, despite different single and dual antiplatelet therapies that were sequentially prescribed for suspected TIAs. Brain MRI revealed cortical superficial siderosis, symmetrical periventricular leukoencephalopathy and enlarged perivascular spaces. Cerebral amyloid angiopathy was considered in the differential diagnosis of the patient. Antiplatelet withdrawal was recommended and led to complete remission of the patient’s transient focal neurological episodes (TFNE) that were initially misdiagnosed as TIAs. Discussion Cortical superficial siderosis has been implicated as a key neuroimaging feature of cerebral amyloid angiopathy, a diagnosis which can be supported by the additional radiological findings of symmetrical white matter hyperintensities and enlarged perivascular spaces. Antiplatelet treatment in patients with cortical superficial siderosis may increase the frequency and severity of TFNE, while it increases exponentially the risk of intracerebral hemorrhage. The present case highlights that recognition of cortical superficial siderosis is crucial in the management of patients presenting with transient focal neurological symptoms that can be misdiagnosed as recurrent TIAs.

Abstract WP371: Cortical Superficial Siderosis and Mortality in Cerebral Amyloid Angiopathy

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Duangnapa Roongpiboonsopit ◽  
Andreas Charidimou ◽  
Gregoire Boulouis ◽  
Li Xiong ◽  
Arne Lauer ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Mortality Risk ◽  
Older Age ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Perivascular Spaces ◽  
Increased Risk ◽  
Cerebral Amyloid ◽  
Cortical Superficial Siderosis ◽  
Overall Mortality

Objective: To investigate whether magnetic resonance imaging (MRI) markers of cerebral small vessel disease predict overall mortality in cerebral amyloid angiopathy (CAA) patients. Methods: Subjects were consecutive survivors (age≥55) of spontaneous symptomatic CAA-related-lobar ICH and CAA presenting without lobar ICH, diagnosed according to the Boston criteria drawn from an ongoing longitudinal cohort study and Memory Disorder Unit. All subjects had brain MRI at presentation. Baseline clinical, imaging, laboratory data and mortality information were collected. Neuroimaging markers including focal (≤3 sulci) or disseminated (>3 sulci) cortical superficial siderosis (cSS), cortical subarachnoid hemorrhage (cSAH), cerebral microbleeds (CMBs), enlarged perivascular spaces (EPVS) and white matter hyperintensities (WMH) were evaluated. Overall mortality risk was assessed using Cox proportional hazards models adjusting for potential confounders. Results: A total of 335 patients with probable CAA were enrolled, 196 presenting with lobar ICH and 139 without lobar ICH. During a median follow-up time of 3.44 years (interquartile range 1.61- 5.52 years), 181 of 335 patients (54.0%) died, 37.3% were patients with lobar ICH and 16.7% were those without. In univariable analysis, disseminated cSS, moderate to severe WMH, higher age and CAA related-lobar ICH group were predictors of overall mortality (p<0.05 for all comparisons). After adjusting for moderate to severe WMH and multiple CMBs (CMBs ≥5 foci), disseminated cSS remained as an independent neuroimaging predictor of overall mortality (HR 1.66; 95% CI 1.05-2.64, p = 0.030). Other predictors of mortality were older age (HR 1.08; 95% CI 1.06-1.11, p < 0.001) and presence of lobar ICH (HR 1.87; 95% CI 1.34-2.61, p < 0.001). The mortality risk was even greater in patients with both disseminated cSS and lobar ICH (HR 2.28; 95% CI 1.41-3.69, p = 0.001) and as well as in older patients (age>75 years) with disseminated cSS (HR 1.86; 95% CI 1.08-3.23, p = 0.026). Conclusion: Disseminated cSS is an independent neuroimaging biomarker of increased risk of overall mortality in probable CAA, particular in those patients with lobar ICH and older age. These findings may serve identify important markers of CAA severity.

Abstract 121: Mechanisms of Cortical Superficial Siderosis in Cerebral Amyloid Angiopathy

Stroke ◽  
2016 ◽  
Vol 47 (suppl_1) ◽  
Author(s):  
Andreas Charidimou ◽  
Gregoire Boulouis ◽  
Duangnapa Roongpiboonsopit ◽  
Kellen Haley ◽  
Eitan Auriel ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Quantitative Methods ◽  
Cox Regression ◽  
Secondary Analysis ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Perivascular Spaces ◽  
Cerebral Amyloid ◽  
Subarachnoid Blood ◽  
Cortical Superficial Siderosis

Introduction: Mechanisms of cortical superficial siderosis (cSS) in cerebral amyloid angiopathy (CAA) are unknown, but might include in situ bleeding from amyloid laden superficial vessels or blood products redistribution from acute lobar intracerebral hemorrhage (ICH). Hypothesis: We aimed to identify factors related to the extent and multifocality of cSS. Methods: We analyzed consecutive CAA-related ICH patients diagnosed with Boston criteria from a prospective cohort. cSS multifocality, was rated for spatially distinct foci involvement and bi-hemispheric distribution on a 0-4 scale (1 point: 1 sulcus or focal immediately adjacent sulci, 2 points: 2 or more non-adjacent sulci OR more than 3 adjacent sulci, in each hemisphere) with excellent interrater reliability (k=0.87). Radiological markers of CAA and ICH characteristics were assessed using quantitative methods or validated visual scales, and their association with cSS multifocality was investigated in multivariable regression. Results: The cohort included 312 CAA patients (53% male; mean age 71.7). cSS was found in 34.6%: unifocal in 42 patients (13.5%), multifocal in 66 (23.1%). Presence and multifocality of cSS were not related with characteristics of the acute ICH such as ICH volume, ICH side or areas of acute subarachnoid blood on initial CT. ICH side did not predict cSS side or multifocality at that side. cSS multifocality was related to the presence of IVH in patients who had a single ICH without microbleeds (MB), but not in patients with multiple hemorrhagic lesions (ICH and/or MBs). In ordinal logistic regression, cSS multifocality was related to lobar microbleeds burden (OR: 1.57; 95%CI: 1.27-1.94; p<0.0001) and MRI centrum semiovale perivascular spaces (PVS, OR: 2.05; 95%CI: 1.26-3.33; p=0.004) but not basal ganglia PVS or leukoaraiosis volume. A secondary analysis using Cox regression showed that cSS multifocality was an independent risk factor for recurrent ICH (HR: 2.21; 95%CI: 1.09-4.52 and 3.91; 95%CI: 1.40-10.9 for multifocality presence and severity) after adjusting for other confounders. Conclusion: Extent and multifocality of cSS appear to be markers of CAA severity and independent predictors of ICH recurrence, and can provide insights potential into pathomechanisms.

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

Abstract 36: The Boston Criteria V2.0 for Cerebral Amyloid Angiopathy: Updated Criteria and Multicenter MRI-Neuropathology Validation

Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Andreas Charidimou ◽  
Gregoire Boulouis ◽  
Matthew Frosch ◽  
Jean-Claude Baron ◽  
Marco Pasi ◽  
...  
Keyword(s):  
White Matter ◽  
Intracerebral Hemorrhage ◽  
Diagnostic Accuracy ◽  
Cerebral Amyloid Angiopathy ◽  
Brain Mri ◽  
Amyloid Angiopathy ◽  
Perivascular Spaces ◽  
Validation Sample ◽  
Temporal Validation ◽  
Cerebral Amyloid

Introduction: The Boston criteria are used worldwide for in vivo diagnosis of cerebral amyloid angiopathy (CAA). Given substantial advances in CAA research, we aimed to update the Boston criteria and externally validate their diagnostic accuracy across the spectrum of CAA-related presentations and across international sites. Methods: As part of an International CAA Association multicenter study, we identified patients age 50 or older with potential CAA-related clinical presentations (spontaneous intracerebral hemorrhage, cognitive impairment, or transient focal neurological episodes), available brain MRI, and histopathologic assessment for the diagnosis of CAA. We derived Boston criteria v2.0 by selecting MRI features to optimize diagnostic specificity and sensitivity in a pre-specified derivation sample (Boston cases 1994 to 2012, n=159), then externally validated in pre-specified temporal (Boston cases 2012-2018, n=59) and geographical (non-Boston cases 2004-2018; n=123) validation samples and compared their diagnostic accuracy to the currently used modified Boston criteria. Results: Based on exploratory analyses in the derivation sample, we derived provisional criteria for probable CAA requiring presence of at least 2 strictly lobar hemorrhagic lesions (intracerebral hemorrhage, cerebral microbleed, or cortical superficial siderosis focus) or at least 1 strictly lobar hemorrhagic lesion and 1 white matter characteristic (severe degree of visible perivascular spaces in centrum semiovale or white matter hyperintensities multispot pattern). Sensitivity/specificity of the criteria were 74.8/84.6% in the derivation sample, 92.5/89.5% in the temporal validation sample, 80.2/81.5% in the geographic validation sample, and 74.5/95.0% in cases across all samples with autopsy as the diagnostic gold standard. The v2.0 criteria for probable CAA had superior accuracy to the currently modified Boston criteria (p<0.005) in the autopsied cases. Conclusion: The Boston criteria v.2.0 incorporate emerging MRI markers of CAA to enhance sensitivity without compromising their high specificity. Validation of the criteria across independent patient settings firmly supports their adoption into clinical practice and research.

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 Advances in cerebral amyloid angiopathy imaging

2019 ◽  
Vol 12 ◽  
pp. 175628641984411 ◽  
Author(s):  
Szu-Ju Chen ◽  
Hsin-Hsi Tsai ◽  
Li-Kai Tsai ◽  
Sung-Chun Tang ◽  
Bo-Chin Lee ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Small Vessel Disease ◽  
Imaging Techniques ◽  
White Matter Hyperintensity ◽  
Superficial Siderosis ◽  
Clinical Aspects ◽  
Amyloid Angiopathy ◽  
Perivascular Spaces ◽  
Imaging Studies ◽  
Cerebral Amyloid

Cerebral amyloid angiopathy (CAA) is a cerebral small vessel disease caused by β -amyloid (Aβ) deposition at the leptomeningeal vessel walls. It is a common cause of spontaneous intracerebral hemorrhage and a frequent comorbidity in Alzheimer’s disease. The high recurrent hemorrhage rate in CAA makes it very important to recognize this disease to avoid potential harmful medication. Imaging studies play an important role in diagnosis and research of CAA. Conventional computed tomography and magnetic resonance imaging (MRI) methods reveal anatomical alterations, and remains as the most reliable tool in identifying CAA according to modified Boston criteria. The vascular injuries of CAA result in both hemorrhagic and ischemic manifestations and related structural changes on MRI, including cerebral microbleeds, cortical superficial siderosis, white matter hyperintensity, MRI-visible perivascular spaces, and cortical microinfarcts. As imaging techniques advance, not only does the resolution of conventional imaging improve, but novel skills in functional and molecular imaging studies also enable in vivo analysis of vessel physiological changes and underlying pathology. These modern tools help in early detection of CAA and may potentially serve as sensitive outcome markers in future clinical trials. In this article, we reviewed past studies of CAA focusing on utilization of various conventional and novel imaging techniques in both research and clinical aspects.

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.

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