Follow-up study of a patient with early onset cerebral amyloid angiopathy following childhood cadaveric dural graft

2021 ◽  
Author(s):  
Kenji Yoshiki ◽  
Genjiro Hirose ◽  
Kazuhiko Kumahashi ◽  
Yukihiko Kohda ◽  
Kazunori Ido ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Early Onset ◽  
Amyloid Angiopathy ◽  
Follow Up Study ◽  
Dural Graft ◽  
Cerebral Amyloid

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.

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

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.

Fatal Aβ cerebral amyloid angiopathy 4 decades after a dural graft at the age of 2 years

2018 ◽  
Vol 135 (5) ◽  
pp. 801-803 ◽  
Author(s):  
Dominique Hervé ◽  
Maximilien Porché ◽  
Lucie Cabrejo ◽  
Céline Guidoux ◽  
Elisabeth Tournier-Lasserve ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Dural Graft ◽  
Cerebral Amyloid

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 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 Spontaneous ARIA-like Events in Cerebral Amyloid Angiopathy–Related Inflammation: A Multicenter Prospective Longitudinal Cohort Study

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012778
Author(s):  
Laura Antolini ◽  
Jacopo C. DiFrancesco ◽  
Marialuisa Zedde ◽  
Gianpaolo Basso ◽  
Andrea Arighi ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Cerebral Amyloid Angiopathy ◽  
Pulse Therapy ◽  
Amyloid Angiopathy ◽  
Longitudinal Cohort ◽  
History Of ◽  
Corticosteroid Pulse Therapy ◽  
Cerebral Amyloid

Background and Objectives:To investigate the natural history and outcomes following treatment for spontaneous amyloid-related imaging abnormalities (ARIA)-like in cerebral amyloid angiopathy-related inflammation (CAA-ri).Methods:A multicenter, hospital-based, longitudinal, prospective observational study of inpatients meeting CAA-ri diagnostic criteria, recruited through the iCAβ International Network, in the period January 2013 - March 2017. A protocol for systematic data collection at first-ever presentation and at subsequent in-person visits, including T1-weighted, GRE-T2*, fluid-suppressed T2-weighted (FLAIR), and T1 post-gadolinium contrast-enhancement images aquired on 1.5T MRI, was employed at 3, 6, 12, 24-months follow-up. Centralized reads of MRI images were performed blinded to clinical, therapeutic, and time-points information. Main outcomes were survival, clinical and radiological recovery, intracerebral hemorrhage (ICH), and recurrence of CAA-ri.Results:The study enrolled 113 participants (10.6% definite, 71.7% probable, and 17.7% possible CAA-ri), mean age 72.9 years, 43.4% female, 37.1% APOEε4 carriers. 36.3% had a history of Alzheimer’s disease, 33.6% of ICH. A history of ICH, as well as the occurrence of new ICH at follow-up, was more common in patients with cortical superficial siderosis at baseline (52.6% vs 14.3%; p< 0.0001 and 19.3% vs 3.6%; p<0.009, respectively). After the first-ever presentation of CAA-ri, 70.3% (95% CI, 61.6-78.5) and 84.1% (95% CI, 76.2-90.6) clinically recovered within three and twelve months, followed by radiological recovery in 45.1% (95% CI, 36.4 - 54.8) and 77.4% (95% CI, 67.7 - 85.9), respectively. After clinicoradiological resolution of the first-ever episode, 38,3% (95% CI, 22.9 - 59.2) had at least one recurrence within the following 24 months. Recurrence was more likely if intravenous high dose corticosteroid pulse therapy was suddenly stopped compared to slow oral tapering-off (Hazard Ratio 4.68; 95% CI, 1.57-13.93; p=0.006).Discussion:These results from the largest longitudinal cohort registry of patients with CAA-ri support the transient and potentially relapsing inflammatory nature of the clinical-radiological acute manifestations of the disease and the effectiveness of slow oral tapering-off after intravenous corticosteroid pulse therapy in preventing recurrences. Our results highlight the importance of differential diagnosis for spontaneous ARIA-like events in Aβ-driven diseases, including treatment-related ARIA in Alzheimer’s disease patients exposed to immunotherapy drugs.

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