A Clinico-Radiological Study of Cerebral Amyloid Angiopathy-Related Inflammation

2019 ◽  
Vol 48 (1-2) ◽  
pp. 38-44 ◽  
Author(s):  
Sarah Coulette ◽  
Dimitri Renard ◽  
Sylvain Lehmann ◽  
Nicolas Raposo ◽  
Caroline Arquizan ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Clinical Symptoms ◽  
Biological Data ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Leptomeningeal Involvement ◽  
Cerebral Mri ◽  
Initial Evolution ◽  
Cerebral Amyloid

Objective: To describe the clinico-radiological features and long-term prognosis in patients with cerebral amyloid angiopathy-related inflammation (CAA-ri). Methods: Twenty-eight CAA-ri patients were recruited retrospectively from 6 neurological centers. We recorded the clinico-radiological and biological data, at baseline and during follow-up. Baseline characteristics associated with relapse risk and prognosis were assessed. Results: Five patients had pathologically confirmed CAA-ri whereas 23 had probable (n = 21) or possible (n = 2) CAA-ri. The mean age was 72 years; main clinical symptoms included confusion (54%), hemiparesis (36%), and aphasia (29%). Cerebral MRI disclosed a brain parenchymal lesion (89%), which was usually multifocal (82%) and bilateral (89%). It was associated with gadolinium enhancement (84%), small ischemic lesions (39%), cortical superficial siderosis (CSS; 50%), and a high number of microbleeds (mean 240 ± 277). An isolated leptomeningeal involvement was observed in 3 patients with pathological confirmation. Despite a favorable initial evolution after treatment, we observed a 42% risk of relapse, mostly within the first year (83%). After a mean follow-up of 2 years, 29% died and 25% had a marked disability. Disseminated CSS was associated with death. Conclusion: Despite an apparently favorable initial evolution, CAA-ri is characterized by a poor prognosis. Diagnostic criteria should consider patients with isolated leptomeningeal involvement.

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 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 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.

scholarly journals β-Amyloid in CSF

Neurology ◽  
2016 ◽  
Vol 88 (2) ◽  
pp. 169-176 ◽  
Author(s):  
Ellis S. van Etten ◽  
Marcel M. Verbeek ◽  
Jeroen van der Grond ◽  
Ronald Zielman ◽  
Sanneke van Rooden ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Clinical Symptoms ◽  
White Matter Hyperintensity ◽  
Superficial Siderosis ◽  
Csf Biomarkers ◽  
Amyloid Angiopathy ◽  
Cross Sectional ◽  
Mutation Carriers ◽  
Β Amyloid ◽  
Cerebral Amyloid

Objective:To investigate CSF biomarkers in presymptomatic and symptomatic mutation carriers with hereditary cerebral hemorrhage with amyloidosis–Dutch type (HCHWA-D), a model for sporadic cerebral amyloid angiopathy, and to determine the earliest deposited form of β-amyloid (Aβ).Methods:HCHWA-D mutation carriers and controls were enrolled in the cross-sectional EDAN (Early Diagnosis of Amyloid Angiopathy Network) study. The HCHWA-D group was divided into symptomatic carriers with a previous intracerebral hemorrhage and presymptomatic carriers. CSF concentrations of Aβ40, Aβ42, total tau, and phosphorylated tau181 proteins were compared to those of controls of a similar age. Correlations between CSF biomarkers, MRI markers, and age were investigated with multivariate linear regression analyses.Results:We included 10 symptomatic patients with HCHWA-D (mean age 55 ± 6 years), 5 presymptomatic HCHWA-D carriers (mean age 36 ± 13 years), 31 controls <50 years old (mean age 31 ± 7 years), and 50 controls ≥50 years old (mean age 61 ± 8 years). After correction for age, CSF Aβ40 and Aβ42 were significantly decreased in symptomatic carriers vs controls (median Aβ40 1,386 vs 3,867 ng/L, p < 0.001; median Aβ42 289 vs 839 ng/L, p < 0.001) and in presymptomatic carriers vs controls (median Aβ40 3,501 vs 4,684 ng/L, p = 0.011; median Aβ42 581 vs 1,058 ng/L, p < 0.001). Among mutation carriers, decreasing CSF Aβ40 was associated with higher lobar microbleed count (p = 0.010), increasing white matter hyperintensity volume (p = 0.008), and presence of cortical superficial siderosis (p = 0.02).Conclusions:Decreased levels of CSF Aβ40 and Aβ42 occur before HCHWA-D mutation carriers develop clinical symptoms, implicating vascular deposition of both Aβ species as early steps in cerebral amyloid angiopathy pathogenesis. CSF Aβ40 and Aβ42 may serve as preclinical biomarkers of cerebral amyloid angiopathy pathology.

scholarly journals The Characteristics of Superficial Siderosis and Convexity Subarachnoid Hemorrhage and Clinical Relevance in Suspected Cerebral Amyloid Angiopathy

2015 ◽  
Vol 39 (5-6) ◽  
pp. 278-286 ◽  
Author(s):  
Jun Ni ◽  
Eitan Auriel ◽  
Jenelle Jindal ◽  
Alison Ayres ◽  
Kristin M. Schwab ◽  
...  
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Intracerebral Hemorrhage ◽  
Cerebral Amyloid Angiopathy ◽  
Epileptiform Activity ◽  
Brain Mri ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Cerebral Bleeding ◽  
Cerebral Amyloid

Background and Aims: Systematic studies of superficial siderosis (SS) and convexity subarachnoid hemorrhage (cSAH) in patients with suspected cerebral amyloid angiopathy (CAA) without lobar intracerebral hemorrhage (ICH) are lacking. We sought to determine the potential anatomic correlation between SS/cSAH and transient focal neurological episodes (TFNE) and whether SS/cSAH is predictor of future cerebral hemorrhagic events in these patients. Methods: We enrolled 90 consecutive patients with suspected CAA (due to the presence of strictly lobar microbleeds (CMBs) and/or SS/cSAH) but without the history of symptomatic lobar ICH who underwent brain MRI including T2*-weighted, diffusion-weighted imaging and fluid-attenuated inversion recovery sequences from an ongoing single center CAA cohort from 1998 to 2012. Evaluation of SS, cSAH and CMBs was performed. Medical records and follow-up information were obtained from prospective databases and medical charts. TFNE was defined according to published criteria and electroencephalogram reports were reviewed. Results: Forty-one patients (46%) presented with SS and/or cSAH. The prevalence of TFNE was significantly higher in those with SS/cSAH (61 vs. 10%; p < 0.001) and anatomically correlated with the location of cSAH, but not SS. The majority of TFNE in patients with SS/cSAH presented with spreading sensory symptoms. Intermittent focal slowing on electroencephalogram was present in the same area as SS/cSAH in 6 patients, but no epileptiform activity was found in any patients. Among those with available clinical follow-up (76/90 patients, 84%), ten patients with SS/cSAH (29%, median time from the scan for all patients with SS/cSAH: 21 months) had a symptomatic cerebral bleeding event on follow up (average time to events: 34 months) compared with only 1 event (2.4%, 25 months from the scan) in patients without SS/​cSAH (time to event: 25 months) (p = 0.001). The location of hemorrhages on follow-up scan was not in the same location of previously noted SS/cSAH in 9 of 10 patients. Follow-up imaging was obtained in 9 of 17 patients with cSAH and showed evidence of SS in the same location as initial cSAH in all these 9 cases. Conclusions: SS/cSAH is common in patients with suspected CAA without lobar intracerebral hemorrhage and may have a significantly higher risk of future cerebral bleeding events, regardless of the severity of the baseline CMB burden. The findings further highlight a precise anatomical correlation between TFNE and cSAH, but not SS. Distinct from transient ischemic attack or seizure, the majority of TFNE caused by SS/cSAH appear to present with spreading sensory symptoms.

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.

Abstract TP432: Predictors for Dementia Conversion in Cerebral Amyloid Angiopathy From a Stroke Unit

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Li Xiong ◽  
Raffaella Valenti ◽  
Andreas Charidimou ◽  
Gregoire Boulouis ◽  
Duangnapa Roongpiboonsopit ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Stroke Unit ◽  
Cox Regression ◽  
Superficial Siderosis ◽  
P Value ◽  
Amyloid Angiopathy ◽  
Perivascular Spaces ◽  
Cox Regression Analysis ◽  
Cerebral Amyloid

Objective: Cerebral amyloid angiopathy (CAA) is increasing recognized as a cause of cognitive impairment and dementia in older individuals. This study aimed to investigate predictors of dementia, including imaging markers, in CAA patients from a stroke unit. Methods: A total of 71 non-demented patients from a stroke unit were included according to modified Boston Criteria for probable CAA with available cognitive follow up. These CAA patients included both patients with and patients without previous intracerebral hemorrhage (ICH). At baseline, neuroimaging markers, including lobar microbleeds (CMBs), white matter hyperintensities (WMH), cortical superficial siderosis (cSS) and MRI-visible centrum semiovale perivascular spaces (CSO-PVS) were assessed. The small vessel disease (SVD) score for CAA was calculated by the scores of CMBs, WMH, cSS and CSO-PVS. The association between these neuroimaging markers and dementia conversion was analyzed. Results: The median follow up time is 1.91 years (quartiles 1.14-4.23 years). Fourteen (19.72%) CAA patients developed dementia during follow up period. Thirty-seven CAA patients (52.11%) had previous symptomatic ICH. Age, lobar CMBs≥20 and SVD score were selected from the univariate Cox-regression analysis with p value less than 0.1 (Table1). In a backward stepwise multivariabte analysis including age, previous ICH history and either SVD score or number of CMBs, age and SVD score independently predicted dementia conversion (Table 1). The individual neuroimaging markers for SVD related brain damage (CSO-PVS, cSS, lobar MBs and WMH) did not predict dementia conversion for probable CAA patients. Conclusion: Our results demonstrate that cognitive deterioration of CAA patients appears attributed to cumulative CAA related vasculopathic changes.

Abstract 2491: Cerebral Amyloid Angiopathy without Hemorrhagic Stroke: MRI Findings Associated with Transient Focal Symptoms

Stroke ◽  
2012 ◽  
Vol 43 (suppl_1) ◽  
Author(s):  
Jenelle A Jindal ◽  
Alison M Ayres ◽  
Mahmut E Gurol ◽  
Kristin Schwab ◽  
Jonathan Rosand ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Hemorrhagic Stroke ◽  
Clinical Presentation ◽  
Clinical Symptoms ◽  
Massachusetts General Hospital ◽  
The Other ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Neurologic Symptoms ◽  
Cerebral Amyloid

Background: The clinical presentation of cerebral amyloid angiopathy (CAA) includes not only hemorrhagic stroke, but also diverse syndromes such as transient focal neurologic symptoms, progressive cognitive dysfunction, subacute confusion, and seizures. We performed a systematic analysis of the neuroimaging features of these presentations to help elucidate their underlying pathophysiologies. Methods: We performed a retrospective review of all patients seen at Massachusetts General Hospital from 2000-2011 with 1) diagnosis of probable/definite CAA by Boston criteria, 2) clinical presentation other than hemorrhagic stroke, and 3) available MR images (including T2*-weighted, diffusion-weighted, and FLAIR). Review of clinical data for 44 subjects meeting these criteria (performed blinded to neuroimaging) yielded 3 categories of presentation: transient focal motor, sensory, or language symptoms (n=15; mean±SD age 74.4±8.1), memory/cognitive impairment over months-years (n=15; age 71.2±10.7), and subacute headache, confusion, generalized seizure, or syncope (n=14; age 73.6±9.8). Images were analyzed without knowledge of clinical symptoms for hemorrhagic lesions, acute infarcts, and regional T2-hyperintensities. Results: Superficial siderosis in cortical sulci (Panel A) was present in 10 of 15 (67%) patients presenting with transient focal symptoms versus 7 of 29 (24%) in the other subgroups (p<0.01). Most locations of superficial siderosis corresponded with the localization of the patient’s transient symptoms. Conversely, a pattern of T2-hyperintensities extending to subcortical white matter and overlying cortex (Panel B) was present in 8 of 14 (57%) patients presenting with headache, confusion, or seizure/syncope versus 3 of 30 (10%) in the other subgroups (p<0.005). Conclusions: These results suggest that the underlying trigger for CAA-related transient focal neurologic symptoms may often be superficial siderosis. They also support an association between the alternative presentation of headache, confusion, or seizure/syncope and T2-hyperintensities suggestive of the inflammatory subtype of CAA.

Acute convexity subarachnoid haemorrhage and cortical superficial siderosis in probable cerebral amyloid angiopathy without lobar haemorrhage

2017 ◽  
Vol 89 (4) ◽  
pp. 397-403 ◽  
Author(s):  
Andreas Charidimou ◽  
Grégoire Boulouis ◽  
Panagiotis Fotiadis ◽  
Li Xiong ◽  
Alison M Ayres ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Subarachnoid Haemorrhage ◽  
Cerebral Amyloid Angiopathy ◽  
Clinical Symptoms ◽  
Small Vessel Disease ◽  
Superficial Siderosis ◽  
Amyloid Angiopathy ◽  
Multivariable Logistic Regression ◽  
Cerebral Amyloid ◽  
Cortical Superficial Siderosis

IntroductionAcute non-traumatic convexity subarachnoid haemorrhage (cSAH) is increasingly recognised in cerebral amyloid angiopathy (CAA). We investigated: (a) the overlap between acute cSAH and cortical superficial siderosis—a new CAA haemorrhagic imaging signature and (b) whether acute cSAH presents with particular clinical symptoms in patients with probable CAA without lobar intracerebral haemorrhage.MethodsMRI scans of 130 consecutive patients meeting modified Boston criteria for probable CAA were analysed for cortical superficial siderosis (focal, ≤3 sulci; disseminated, ≥4 sulci), and key small vessel disease markers. We compared clinical, imaging and cortical superficial siderosis topographical mapping data between subjects with versus without acute cSAH, using multivariable logistic regression.ResultsWe included 33 patients with probable CAA presenting with acute cSAH and 97 without cSAH at presentation. Patients with acute cSAH were more commonly presenting with transient focal neurological episodes (76% vs 34%; p<0.0001) compared with patients with CAA without cSAH. Patients with acute cSAH were also more often clinically presenting with transient focal neurological episodes compared with cortical superficial siderosis-positive, but cSAH-negative subjects with CAA (76% vs 30%; p<0.0001). Cortical superficial siderosis prevalence (but no other CAA severity markers) was higher among patients with cSAH versus those without, especially disseminated cortical superficial siderosis (49% vs 19%; p<0.0001). In multivariable logistic regression, cortical superficial siderosis burden (OR 5.53; 95% CI 2.82 to 10.8, p<0.0001) and transient focal neurological episodes (OR 11.7; 95% CI 2.70 to 50.6, p=0.001) were independently associated with acute cSAH.ConclusionsThis probable CAA cohort provides additional evidence for distinct disease phenotypes, determined by the presence of cSAH and cortical superficial siderosis.

scholarly journals Cerebral Amyloid Angiopathy-Related Atraumatic Convexal Subarachnoid Hemorrhage: An ARIA before the Tsunami

2015 ◽  
Vol 35 (5) ◽  
pp. 710-717 ◽  
Author(s):  
Eva Martínez-Lizana ◽  
María Carmona-Iragui ◽  
Daniel Alcolea ◽  
Manuel Gómez-Choco ◽  
Eduard Vilaplana ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Subarachnoid Hemorrhage ◽  
Cerebral Amyloid Angiopathy ◽  
Functional Disability ◽  
Superficial Siderosis ◽  
Csf Biomarkers ◽  
Amyloid Angiopathy ◽  
Cerebral Amyloid ◽  
Convexal Subarachnoid Hemorrhage

Atraumatic convexal subarachnoid hemorrhage (cSAH) in elderly patients is a rare entity that has been associated with cerebral amyloid angiopathy (CAA) and intracerebral hematomas (ICH). To characterize this entity and to study these associations, 22 patients over 60 with cSAH were included in a multicenter ambispective cohort study. Clinical data, magnetic resonance imaging (MRI) studies, APOE genotyping, and cerebrospinal fluid (CSF) biomarkers were evaluated. Results were compared with data from healthy controls (HC), non-cSAH CAA patients (CAAo), and Alzheimer disease patients. Convexal subarachnoid hemorrhage presented with transient sensory or motor symptoms. At follow-up (median 30.7 months), 5 patients had died, 6 survivors showed functional disability (modified Rankins Scale (mRS) > 2), and 12 cognitive impairment. Four patients had prior ICH and six had an ICH during follow-up. CSF-Aβ40 and Aβ42 levels were lower in cSAH and CAAo compared with HC. Convexal subarachnoid hemorrhage presented an APOE-ε2 overrepresentation and CAAo had an APOE-ε4 overrepresentation. On MRI, all patients fulfilled CAA-modified Boston criteria and 9 showed cortical ischemia in the surrounding cortex or the vicinity of superficial siderosis. The neuropathologic study, available in one patient, showed severe CAA and advanced Alzheimer-type pathology. Convexal subarachnoid hemorrhage in the elderly is associated with cognitive impairment and lobar ICH occurrence. Our findings support the existence of an underlying CAA pathology.

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