scholarly journals Cerebral amyloid angiopathy: a cross-sectional study in a single center in Northeastern Brazil

2020 ◽  
Vol 78 (5) ◽  
pp. 277-281
Author(s):  
Marx Lima de BARROS-ARAÚJO ◽  
Irapuá Ferreira RICARTE ◽  
Edward MONTALVERNE FILHO ◽  
Guilherme Marconi Guimarães Martins HOLANDA ◽  
Ícaro Araújo de SOUSA ◽  
...  

ABSTRACT Background: Cerebral amyloid angiopathy (CAA) is a cerebrovascular disorder caused by progressive deposition of β-amyloid peptides in the walls of small and medium-sized cortical and leptomeningeal vessels. Until today, the prevalence of CAA is unknown in our region. Objective: This study aims to analyze the prevalence of this entity in a specific elderly population in a tertiary hospital in Northeastern Brazil. Methods: A cross-sectional, retrospective study with the enrollment of patients aged 65 or older followed in the neurological outpatient service of the Universidade Federal do Piauí, Brazil, who underwent brain magnetic resonance imaging (MRI) from July 2016 to June 2018. Results: One hundred and seventy-four patients were enrolled, of whom 100 were women (57.4%) and 74, men (42.6%), aged from 65 to 91 years old (median age 73.27). Nine patients were excluded from the study due to unavailability of MRI sequences needed for an appropriate analysis. Out of the 165 remaining patients, 12 (7.2%) had established the diagnosis of CAA, according to the modified Boston criteria. Conclusion: The prevalence of CAA in our study was like those of medical literature, with a progressive age-related increase.

Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Urvish K Patel ◽  
Nandakumar Nagaraja

Background/Objective: There is limited knowledge of the nationwide prevalence of cerebral amyloid angiopathy (CAA) diagnosis and disparity amongst US hospitalization. The aim of this study is to assess the prevalence of CAA diagnosis and identify hospital-level disparity in the CAA diagnosis amongst US hospitalizations. Methods: A cross-sectional study was performed using the National Inpatient Sample [2016-2017] for adult hospitalizations. We extracted a cohort of patients with a diagnosis of CAA using ICD 10 code. Age was categorized as <50 years, 50-59, 60-69, 70-79, and ≥80 years. Weighted analysis using chi-square and multivariable survey logistic regression was performed to identify the prevalence of CAA and evaluate the diagnostic disparity of CAA amongst USA hospitalization. Results: Out of total 60,609,519 US hospitalizations, 16040 (0.027%) had a diagnosis of CAA. Patients with CAA were of higher age 71-80 years (39.9% vs 16.4%), ≥81 years (36.4% vs 14.9%), men (48.1% vs 42.1%), white (71.5% vs 67.5%), and more likely admitted to urban-teaching hospitals (83.9% vs 66.0%), Northeast region hospitals (26.5% vs 18.8%), and hospitals with large bed-size (65.7% vs 51.2%) compared to patients without CAA (p<0.0001). On regression analysis, urban non-teaching (aOR 2.1; 95%CI 1.6-2.9; ref=rural), urban-teaching hospitals [5.4 (4.1-7.2); ref=rural], median size hospital [1.3 (1.1-1.5); ref=small], and large bed size hospitals [2.3 (2-2.7); ref=small] had higher odds of diagnosis of CAA. Compared to the Northeast region, Midwest [0.8 (0.7-0.97)] and South [0.7 (0.6-0.8)] region hospitals had lower odds of a diagnosis of CAA. Conclusion: CAA was present in 0.03% of hospitalized patients in 2016-17. It was more commonly diagnosed in urban teaching, urban non-teaching, large and medium bed size hospitals compared to rural and small bed size hospitals. Lack of awareness of CAA diagnosis in rural and small hospitals could be a potential factor for these disparities.


2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Syuichi Tetsuka ◽  
Ritsuo Hashimoto

Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare variant of CAA with autoimmune inflammation. A 77-year-old female experienced light-headedness during walking and mild ataxic gait without any other objective neuropsychological deficits. Brain magnetic resonance imaging (MRI) revealed an area of abnormal signal and mild parenchymal swelling in the right parietal lobe, indicating vasogenic edema. T2⁎-weighted gradient echo imaging revealed some subcortical microbleeds in the same lesion. Based on the proposed criteria for CAA-ri, she was diagnosed with probable CAA-ri. After 4 months, the spontaneous improvement was noted in the patient’s clinical and radiological findings. This report presents a rare and atypical case of CAA-ri in which the diagnosis was established after the patient underwent neuroimaging for only mild neurological symptoms, and the patient’s clinical and radiological findings displayed spontaneous improvement. Despite typical and striking MRI findings of CAA-ri, this patient only presented a minimal symptom; this dissociation could highlight the significance of not misinterpreting any new neurological symptoms. Thus, increased availability of MRI and growing awareness of CAA-ri might result in more incidentally diagnosed cases in the future. Furthermore, this case suggests that it would be better to strictly monitor the clinical-radiological findings of patients with probable CAA-ri who only present with minimal symptoms without the initiation of immunosuppressive therapy.


2021 ◽  
pp. 174749302110439
Author(s):  
Alvin S Das ◽  
Robert W Regenhardt ◽  
Elif Gokcal ◽  
Mitchell J Horn ◽  
Nader Daoud ◽  
...  

Background Although primary intraventricular hemorrhage is frequently due to trauma or vascular lesions, the etiology of idiopathic primary intraventricular hemorrhage (IP-IVH) is not defined. Aims Herein, we test the hypothesis that cerebral small vessel diseases (cSVD) including hypertensive cSVD (HTN-cSVD) and cerebral amyloid angiopathy are associated with IP-IVH. Methods Brain magnetic resonance imaging from consecutive patients (January 2011 to September 2019) with non-traumatic intracerebral hemorrhage from a single referral center were reviewed for the presence of HTN-cSVD (defined by strictly deep or mixed-location intracerebral hemorrhage/cerebral microbleeds) and cerebral amyloid angiopathy (applying modified Boston criteria). Results Forty-six (4%) out of 1276 patients were identified as having IP-IVH. Among these, the mean age was 74.4 ± 12.2 years and 18 (39%) were females. Forty (87%) had hypertension, and the mean initial blood pressure was 169.2 ± 40.4/88.8 ± 22.2 mmHg. Of the 35 (76%) patients who received a brain magnetic resonance imaging, two (6%) fulfilled the modified Boston criteria for possible cerebral amyloid angiopathy and 10 (29%) for probable cerebral amyloid angiopathy. Probable cerebral amyloid angiopathy was found at a similar frequency when comparing IP-IVH patients to the remaining patients with primary intraparenchymal hemorrhage (P-IPH) (27%, p = 0.85). Furthermore, imaging evidence for HTN-cSVD was found in 8 (24%) patients with IP-IVH compared to 209 (28%, p = 0.52) patients with P-IPH. Conclusions Among IP-IVH patients, cerebral amyloid angiopathy was found in approximately one-third of patients, whereas HTN-cSVD was detected in 23%—both similar rates to P-IPH patients. Our results suggest that both cSVD subtypes may be associated with IP-IVH.


Stroke ◽  
2019 ◽  
Vol 50 (Suppl_1) ◽  
Author(s):  
Pedram Honarpisheh ◽  
Robert M Bryan ◽  
Louise D McCullough ◽  
Bhanu P Ganesh

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 ◽  
...  

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.


2018 ◽  
Vol 89 (6) ◽  
pp. A33.3-A34
Author(s):  
Jasmin Tilling ◽  
Benjamin Trewin ◽  
Stanley Levy

IntroductionCerebral amyloid angiopathy (CAA) is a common age-related condition characterised by amyloid beta-peptide deposition affecting the medium sized cortical and leptomeningeal arteries, arterioles and capillaries. CAA-related Inflammation (CAA-I) is an increasingly recognised variant of CAA, which is thought to be due to perivascular auto-inflammation in response to amyloid deposition. We describe the clinical course of two cases of probable CAA-I.CasesA 71 year old man presented with new-onset seizures, headaches and subacute cognitive decline. MRI of the brain demonstrated confluent subcortical T2 white matter hyperintensities and cerebral oedema, with predominantly superimposed widespread cortico-subcortical micro-haemorrhages, in keeping with the diagnosis of CAA-I. A course of immunosuppresive therapy was commenced with five days of intravenous methylprednisolone, resulting in marked radiological and clinical improvement within two weeks.A 76 year old female presented with subacute cognitive dysfunction and apraxia, and transient left-sided weakness. MRI scan of the brain initially demonstrated a right temporo-occipital infarct, leading to primary treatment for stroke, but subsequently evolved to reveal diffuse multi-lobar T2 white matter hyperintensities with leptomeningeal involvement. A provisional diagnosis of CAA-I was made and following a poor clinical response to a trial of corticosteroid therapy, treatment with intravenous cyclophosphamide was commenced.ConclusionThese cases emphasise the importance of CAA-I as part of the differential diagnosis in patients presenting with symptoms of subacute cognitive decline, seizures, headaches and focal neurological deficits, given the potential for dramatic improvement with readily accessible immunosuppressive therapies.


2017 ◽  
Vol 2 (4) ◽  
Author(s):  
Saleh Mohammad Sulaiman Harun ◽  
Mounkaila Noma ◽  
Zeinab Yahia El Roubi

Introduction: Age-Related Macular Degeneration (AMD or ARMD), also known as Macular Degeneration, is an eye disorder that slowly destroys sharp, central vision. The causal factors of ARMD remain a controversy. The proposed study aimed to participate in controversy in focusing on the magnitude of ARMD in tertiary hospital in Khartoum state.  Methods: A hospital-based retrospective cross sectional study design was implemented in Makkah Eye Complex. Data on ARMD was derived from an electronic data based made available to the researcher. The data in excel format was uploaded in the SPSS version 21. All the 79 cases of ARMD was retained and summarized through descriptive statistics to produce graphics and frequency tables to address the research objectives and chi square test was performed to assess the association between ARMD and gender and age. A decision role was set prior to the test to reject the null hypothesis that there was no association whenever the p value is < 0.05.Results: During the period covered by the study a total of 86,788 patients attended Makkah Eye Complex, 79 patients among those was diagnosed as Age Related Macular Degeneration disease. This represents a prevalence of Age Related Macular Degeneration (ARMD) of 0.09 % (79/86,788).Discussion: Regarding age, our findings confirmed that the condition is related to aging. It occurred earlier in our research contrary to the commonly reported years of occurrence. However, the limited data available to us indicates the need to establish an electronic patient registry to enable to describe patients sociodemographic characteristics, the disease risk factors and patient management and related outcomes.


Neurology ◽  
2017 ◽  
Vol 88 (9) ◽  
pp. 878-884 ◽  
Author(s):  
Gregoire Boulouis ◽  
Andreas Charidimou ◽  
Michael J. Jessel ◽  
Li Xiong ◽  
Duangnapa Roongpiboonsopit ◽  
...  

Objective:Cerebral amyloid angiopathy (CAA) is a common age-related small vessel disease (SVD). Patients without intracerebral hemorrhage (ICH) typically present with transient focal neurologic episodes (TFNEs) or cognitive symptoms. We sought to determine if SVD lesion burden differed between patients with CAA first presenting with TFNEs vs cognitive symptoms.Methods:A total of 647 patients presenting either to a stroke department (n = 205) or an outpatient memory clinic (n = 442) were screened for eligibility. Patients meeting modified Boston criteria for probable CAA were included and markers of SVD were quantified, including cerebral microbleeds (CMBs), perivascular spaces, cortical superficial siderosis (cSS), and white matter hyperintensities (WMHs). Patients were classified according to presentation symptoms (TFNEs vs cognitive). Total CAA-SVD burden was assessed using a validated summary score. Individual neuroimaging markers and total SVD burden were compared between groups using univariable and multivariable models.Results:There were 261 patients with probable CAA included. After adjustment for confounders, patients first seen for TFNEs (n = 97) demonstrated a higher prevalence of cSS (p < 0.0001), higher WMH volumes (p = 0.03), and a trend toward higher CMB counts (p = 0.09). The total SVD summary score was higher in patients seen for TFNEs (adjusted odds ratio per additional score point 1.46, 95% confidence interval 1.16–1.84, p = 0.013).Conclusions:Patients with probable CAA without ICH first evaluated for TFNEs bear a higher burden of structural MRI SVD-related damage compared to those first seen for cognitive symptoms. This study sheds light on neuroimaging profile differences across clinical phenotypes of patients with CAA without ICH.


2020 ◽  
Vol 78 (4) ◽  
pp. 1765-1774
Author(s):  
Yuichiro Ii ◽  
Hidehiro Ishikawa ◽  
Hirofumi Matsuyama ◽  
Akihiro Shindo ◽  
Keita Matsuura ◽  
...  

Background: Hypertensive arteriopathy (HA) and cerebral amyloid angiopathy (CAA) may contribute to the development of mixed cerebral microbleeds (CMBs). Recently, the total small vessel disease (SVD) scores for HA and CAA were proposed, which are determined by a combination of MRI markers to reflect overall severity of these microangiopathies. Objective: We investigated whether or not total HA-SVD and CAA-SVD scores could be used to predict overlap of HA and CAA in patients with mixed CMBs. Methods: Fifty-three subjects with mixed CMBs were retrospectively analyzed. MRI markers (CMBs, lacunes, perivascular space, white matter hyperintensity [WMH] and cortical superficial siderosis [cSS]) were assessed. The HA-SVD score and CAA-SVD score were obtained for each subject. Anterior or posterior WMH was also assessed using the age-related white matter changes scale. Results: The two scores were positively correlated (ρ= 0.449, p < 0.001). The prevalence of lobar dominant CMB distribution (p < 0.001) and lacunes in the centrum semiovale (p < 0.001) and the severity of WMH in the parieto-occipital lobes (p = 0.004) were significantly higher in the high CAA-SVD score group. cSS was found in four patients with high CAA-SVD score who showed lobar-dominant CMB distribution and severe posterior WMH. Conclusion: Mixed CMBs are mainly due to HA. Assessing both two scores may predict the overlap of HA and CAA in individuals with mixed CMBs. Patients with a high CAA-SVD score may have some degree of advanced CAA, especially when lobar predominant CMBs, severe posterior WMH, lobar lacunes, or cSS are observed.


2020 ◽  
pp. 174749302097446
Author(s):  
Mariel G Kozberg ◽  
Valentina Perosa ◽  
M Edip Gurol ◽  
Susanne J van Veluw

Cerebral amyloid angiopathy is a common small vessel disease in the elderly involving vascular amyloid-β deposition. Cerebral amyloid angiopathy is one of the leading causes of intracerebral hemorrhage and a significant contributor to age-related cognitive decline. The awareness of a diagnosis of cerebral amyloid angiopathy is important in clinical practice as it impacts decisions to use lifelong anticoagulation or nonpharmacological alternatives to anticoagulation such as left atrial appendage closure in patients who have concurrent atrial fibrillation, another common condition in older adults. This review summarizes the latest literature regarding the management of patients with sporadic cerebral amyloid angiopathy, including diagnostic criteria, imaging biomarkers for cerebral amyloid angiopathy severity, and management strategies to decrease intracerebral hemorrhage risk. In a minority of patients, the presence of cerebral amyloid angiopathy triggers an autoimmune inflammatory reaction, referred to as cerebral amyloid angiopathy-related inflammation, which is often responsive to immunosuppressive treatment in the acute phase. Diagnosis and management of cerebral amyloid angiopathy-related inflammation will be presented separately. While there are currently no effective therapeutics available to cure or halt the progression of cerebral amyloid angiopathy, we discuss emerging avenues for potential future interventions.


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