scholarly journals Index event of cerebral amyloid angiopathy (CAA) determines long-term prognosis and recurrent events (retrospective analysis and clinical follow-up)

2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Andrea Wagner ◽  
Christiane Groetsch ◽  
Sibylle Wilfling ◽  
Karl-Michael Schebesch ◽  
Mustafa Kilic ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Recurrent Events ◽  
Clinical Presentation ◽  
Secondary Prophylaxis ◽  
Amyloid Angiopathy ◽  
Index Event ◽  
Asymptomatic Patients ◽  
Cerebral Amyloid

Abstract Background The modified Boston criteria (mBC) define the probability for the diagnosis of cerebral amyloid angiopathy (CAA). Its initial clinical presentation differs from asymptomatic cerebral microbleedings (cMBs), acute ischemic stroke (AIS), cortical hemosiderosis (cSS), to lobar ICH (lICH). Methods Retrospective analyses and clinical follow-ups of individuals with at least mBC “possible” CAA from 2005 to 2018. Results 149 patients were classified in subgroups due to the index event: lICH (n = 91), AIS (n = 32), > 3 cMBs only (n = 16) and cSS (n = 10). Patients in the lICH subgroup had a significantly higher percentage of single new lICHs compared to other groups, whereas patients in the AIS-group had a significantly higher percentage of multiple new AIS. cMBs as index event predisposed for AIS during follow up (p < 0.0016). Patients of the cMBs- or cSS-group showed significantly more TFNEs (transient focal-neurological episodes) and lower numbers of asymptomatic patients (for epilepsy and TFNEs) at the index event than patients with lICH or AIS (p < 0.0013). At long-term follow-up, the cMBs- and cSS-group were characterized by more TFNEs and fewer asymptomatic patients. Conclusions A new classification system of CAA should add subgroups according to the initial clinical presentation to the mBCs allowing individual prognosis, acute treatment and secondary prophylaxis.

scholarly journals 10-Year Follow-Up of a Patient with Cerebral Amyloid Angiopathy

2020 ◽  
Vol 12 (Suppl. 1) ◽  
pp. 202-206
Author(s):  
Min Kyoung Kang ◽  
Byung-Woo Yoon
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
The Elderly ◽  
Amyloid Angiopathy ◽  
Spontaneous Intracerebral Hemorrhage ◽  
Radiological Features ◽  
Magnetic Imaging ◽  
Long Term Follow Up ◽  
Cerebral Amyloid

We report the case of long-term follow-up of brain magnetic imaging of cerebral amyloid angiopathy. Cerebral amyloid angiopathy is often considered a major cause of spontaneous intracerebral hemorrhage in the elderly. This case illustrates the markedly progressive clinical and radiological features of the vasculopathic process in 10 years.

Cerebral amyloid angiopathy in streptozotocin rat model of sporadic Alzheimer’s disease: a long-term follow up study

2011 ◽  
Vol 118 (5) ◽  
pp. 765-772 ◽  
Author(s):  
Melita Salkovic-Petrisic ◽  
Jelena Osmanovic-Barilar ◽  
Martina K. Brückner ◽  
Siegfried Hoyer ◽  
Thomas Arendt ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Rat Model ◽  
Amyloid Angiopathy ◽  
Sporadic Alzheimer’S Disease ◽  
Follow Up Study ◽  
Long Term Follow Up ◽  
Streptozotocin Rat Model ◽  
Cerebral Amyloid

Abstract WMP95: Cerebral Amyloid Angiopathy Patients With Convexity Subarachnoid Hemorrhage Present Higher Risk of Subsequent Hemorrhage Than Those With Lobar Intracranial Hemorrhage

Stroke ◽  
2016 ◽  
Vol 47 (suppl_1) ◽  
Author(s):  
Lionel Calviere ◽  
Nicolas Raposo ◽  
Vanessa Cazolla ◽  
Sofia Pastoura ◽  
Jean François Albucher ◽  
...  
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Cerebral Amyloid Angiopathy ◽  
Intracranial Hemorrhage ◽  
Clinical Presentation ◽  
The Other ◽  
Amyloid Angiopathy ◽  
The Mean ◽  
Cerebral Amyloid ◽  
Mean Time

Background: Acute convexity subarachnoid hemorrhage (cSAH) is increasingly recognized as a marker of cerebral amyloid angiopathy (CAA). Little is known about the risk of subsequent hemorrhage in such CAA presentation, whereas CAA-related lobar intracranial hemorrhage (ICH) is associated with a high risk of recurrence. The aim of this study was to compare clinical outcome among patients with an acute CAA related- cSAH to those with an acute CAA-related lobar ICH. Hypothesis: We hypothesized that the risk of subsequent hemorrhage is different between patients with acute cSAH and lobar ICH related to CAA. Methods: We retrospectively reviewed the clinical outcomes (death, subsequent transient focal neurological episodes (TFNE), rates of ICH and acute cSAH) of 45 consecutive patients (75 ± 7 years) with an acute cSAH related to probable CAA compared to 70 consecutive patients (78 ± 7 years) with an acute lobar ICH meeting the Boston criteria for probable CAA. Results: cSAH-patients presented essentially with TFNE (84.4% vs 0%; p<0.001) whereas ICH-patients had a persistent neurological deficit (98.6% vs 15.6%; p<0.001). Five patients with lobar ICH died in the first days. Thirty nine cSAH-patients and 60 lobar ICH-patients had available follow-up data. The mean time of follow-up (± SD) was 364 ± 358 days. Mortality did not differ between cSAH-patients and ICH-patients who survived (10.2 % vs 16.7%; p = 0.38). Patients with cSAH had a higher rate of TFNE (48.7 % vs 0 %; p<0.001) and acute cSAH recurrence (20.5 % vs 1.7 %; p = 0.002). In the other hand, 20.5 % of cSAH-patients presented a subsequent ICH, not different from patients with acute ICH (15.0 %; p = 0.45). Conclusions: In the context of CAA, patients with acute cSAH present more clinical recurrences than lobar ICH-patients, due to higher risk of subsequent TFNE and acute cSAH. Although the clinical presentation of cSAH-patients may appear benign, their outcome regarding the risk of incident ICH and mortality, does not seem different from CAA-related lobar ICH survivors.

Left Ventricle Hypertrophy is Common in Patients with Cerebral Amyloid Angiopathy-Related Intracerebral Hemorrhage and Is Independently Associated with Long-Term Mortality.

Stroke ◽  
2013 ◽  
Vol 44 (suppl_1) ◽  
Author(s):  
Octavio M Pontes-Neto ◽  
Sergi Martinez-Ramirez ◽  
Anand Viswanathan ◽  
Eitan Auriel ◽  
Kristen M Schwab ◽  
...  
Keyword(s):  
Left Ventricle ◽  
Intracerebral Hemorrhage ◽  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Lv Mass ◽  
The Mean ◽  
Cerebral Amyloid ◽  
Long Term Mortality

Background: A post-hoc analysis of the PROGRESS trial suggested that long-term anti-hypertensive therapy prevents intracerebral hemorrhage (ICH) in patients with cerebral amyloid angiopathy (CAA). However, the burden of underlying hypertension in patients with CAA is unclear, and it is also unclear whether this hypertensive burden contributes to long-term outcome in survivors of CAA-related ICH. Left ventricle (LV) hypertrophy is a measure of the chronicity and severity of hypertension and could be used to assess hypertensive end-organ damage in patients with CAA. Objective: To test the hypothesis that LV hypertrophy is common in patients with CAA-related ICH and is associated with increased long-term mortality and shorter survival in those patients. Methods: This was a retrospective analysis of a prospectively collected cohort of consecutive patients with primary ICH presenting to a single academic center. We included patients presenting between January/2000 to December/2010, age > 55 years, who received a transthoracic echocardiogram (echo) during follow-up and were diagnosed with definitive, probable or possible CAA according to the Boston criteria. LV mass index (10g/m2) was calculated according to Penn convention. Ninety-day survivors were followed prospectively for long-term mortality or censoring at January/2012. Cox proportional hazards models were used to identify predictors of mortality as time-dependent variables adjusting for potential confounders. Results: Among 211 patients who met inclusion criteria, the mean time to follow-up was 4.28 ± 2.7 years; the median time to echocardiogram was 3 days (IQR:49). The mean age was 75.7 ± 9.1 years; 103 (49%) were male. LV hypertrophy was present in 55 (31.8%) patients and 152 (72%) patients survived more than 90 days. In multivariate analysis, after adjusting for baseline characteristics, LV mass index (10g/m2) was associated with higher long-term mortality (HR: 1.20; 95%CI: 1.01-1.4; p=0.039). On Cox-regression, LV hypertrophy was independently associated with shorter long-term survival (HR 1.91; 95%CI 1.05-3.47; p=0.034). Conclusions: LV hypertrophy is common in patients with CAA-related ICH and is associated with increased risk of subsequent mortality among 90-day survivors.

Cerebrovascular and Neurodegenerative Pathologies in Long-Term Stable Mild Cognitive Impairment

2021 ◽  
pp. 1-15
Author(s):  
Manu J. Sharma ◽  
Brandy L. Callahan
Keyword(s):  
Cognitive Impairment ◽  
Mild Cognitive Impairment ◽  
Cerebral Amyloid Angiopathy ◽  
Neurofibrillary Tangles ◽  
Small Vessel Disease ◽  
Significant Proportion ◽  
Amyloid Angiopathy ◽  
Prodromal Phase ◽  
Cerebral Amyloid

Background: Mild cognitive impairment (MCI) is considered by some to be a prodromal phase of a progressive disease (i.e., neurodegeneration) resulting in dementia; however, a substantial portion of individuals (ranging from 5–30%) remain cognitively stable over the long term (sMCI). The etiology of sMCI is unclear but may be linked to cerebrovascular disease (CVD), as evidence from longitudinal studies suggest a significant proportion of individuals with vasculopathy remain stable over time. Objective: To quantify the presence of neurodegenerative and vascular pathologies in individuals with long-term (>5-year) sMCI, in a preliminary test of the hypothesis that CVD may be a contributor to non-degenerative cognitive impairment. We expect frequent vasculopathy at autopsy in sMCI relative to neurodegenerative disease, and relative to individuals who convert to dementia. Methods: In this retrospective study, using data from the National Alzheimer’s Coordinating Center, individuals with sMCI (n = 28) were compared to those with MCI who declined over a 5 to 9-year period (dMCI; n = 139) on measures of neurodegenerative pathology (i.e., Aβ plaques, neurofibrillary tangles, TDP-43, and cerebral amyloid angiopathy) and CVD (infarcts, lacunes, microinfarcts, hemorrhages, and microbleeds). Results: Alzheimer’s disease pathology (Aβ plaques, neurofibrillary tangles, and cerebral amyloid angiopathy) was significantly higher in the dMCI group than the sMCI group. Microinfarcts were the only vasculopathy associated with group membership; these were more frequent in sMCI. Conclusion: The most frequent neuropathology in this sample of long-term sMCI was microinfarcts, tentatively suggesting that silent small vessel disease may characterize non-worsening cognitive impairment.

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.

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