3,734 results match your criteria Cerebral Amyloid Angiopathy


Iatrogenic cerebral amyloid angiopathy: an emerging clinical phenomenon.

J Neurol Neurosurg Psychiatry 2022 May 16. Epub 2022 May 16.

Stroke Research Centre, Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, University College London Hospitals NHS Foundation Trust, London, UK.

In the last 6 years, following the first pathological description of presumed amyloid-beta (Aβ) transmission in humans (in 2015) and subsequent experimental confirmation (in 2018), clinical cases of iatrogenic cerebral amyloid angiopathy (CAA)-attributed to the transmission of Aβ seeds-have been increasingly recognised and reported. This newly described form of CAA is associated with early disease onset (typically in the third to fifth decade), and often presents with intracerebral haemorrhage, but also seizures and cognitive impairment. Although assumed to be rare, it is important that clinicians remain vigilant for potential cases, particularly as the optimal management, prognosis, true incidence and public health implications remain unknown. Read More

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Atypical Blepharospasm with Oromandibular Dystonia Associated with Cerebral Amyloid Angiopathy.

Neuroophthalmology 2022 10;46(3):178-181. Epub 2021 Aug 10.

Division of Neuro-Ophthalmology, Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA.

A case of atypical blepharospasm with oromandibular dystonia is presented in a patient found to have cerebral amyloid angiopathy on magnetic resonance imaging and a shared mechanism is discussed. Read More

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Reversible Periventricular Hyperintensity Lesions in Cerebral Amyloid Angiopathy: A Case Mimicking Cerebral Amyloid Angiopathy-related Inflammation.

Intern Med 2022 May 14. Epub 2022 May 14.

Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Japan.

A 59-year-old man with progressive cognitive decline and mood disturbances was admitted to the hospital. Brain magnetic resonance imaging revealed marked white matter hyperintensity (WMH) and widespread lobar cerebral microbleeds. Because he had untreated hypertension, we started antihypertensive treatment and found a significantly improved cognitive function and WMH regression. Read More

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Cerebral amyloid angiopathy related inflammation with leptomeningeal involvement: a case report and review of the literature.

Acta Neurol Belg 2022 May 15. Epub 2022 May 15.

Neurology Department, Centre Hospitalier Alpes-Léman, Contamine sur Arve, France.

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Impact of Cerebral Amyloid Angiopathy in Two Transgenic Mouse Models of Cerebral β-Amyloidosis: A Neuropathological Study.

Int J Mol Sci 2022 Apr 29;23(9). Epub 2022 Apr 29.

Neurovascular Research Laboratory, Vall d'Hebron Research Institute, Universitat Autònoma de Barcelona, 08035 Barcelona, Spain.

The pathological accumulation of parenchymal and vascular amyloid-beta (Aβ) are the main hallmarks of Alzheimer's disease (AD) and Cerebral Amyloid Angiopathy (CAA), respectively. Emerging evidence raises an important contribution of vascular dysfunction in AD pathology that could partially explain the failure of anti-Aβ therapies in this field. Transgenic mice models of cerebral β-amyloidosis are essential to a better understanding of the mechanisms underlying amyloid accumulation in the cerebrovasculature and its interactions with neuritic plaque deposition. Read More

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The relationship between amyloid-beta and brain capillary endothelial cells in Alzheimer's disease.

Neural Regen Res 2022 Nov;17(11):2355-2363

Department of Neurology, First Hospital of Shanxi Medical University, Taiyuan, Shanxi Province, China.

Neurovascular dysfunction, as an integral part of Alzheimer's disease, may have an important influence on the onset and progression of chronic neurodegenerative processes. The blood-brain barrier (BBB) pathway is one of the main pathways that mediates the clearance of amyloid-beta (Aβ) in the brain parenchyma. A large number of studies have shown that receptors and ATP-binding cassette transporters expressed on endothelial cells play an important role in Aβ transport across the BBB, but the specific mechanism is not clear. Read More

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November 2022

Cerebellar atrophy and its implications on gait in cerebral amyloid angiopathy.

J Neurol Neurosurg Psychiatry 2022 May 9. Epub 2022 May 9.

J Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital, Boston, MA, USA

Objective: Recent data suggest that cerebral amyloid angiopathy (CAA) causes haemorrhagic lesions in cerebellar cortex as well as subcortical cerebral atrophy. However, the potential effect of CAA on cerebellar tissue loss and its clinical implications have not been investigated.

Methods: Our study included 70 non-demented patients with probable CAA, 70 age-matched healthy controls (HCs) and 70 age-matched patients with Alzheimer's disease (AD). Read More

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Association Between Plasma Biomarkers of Amyloid, Tau, and Neurodegeneration with Cerebral Microbleeds.

J Alzheimers Dis 2022 May 2. Epub 2022 May 2.

Department of Neurology, Mayo Clinic, Rochester, MN, USA.

Background: Cerebral microbleeds (CMBs) are a common vascular pathology associated with future intracerebral hemorrhage. Plasma biomarkers of amyloid, tau, and neurodegeneration may provide a screening avenue to identify those with CMBs, but evidence is conflicting.

Objective: To determine the association between plasma biomarkers (Aβ40, Aβ42, t-tau, p-tau181, p-tau217, neurofilament light chain (NfL)) and CMBs in a population-based study of aging and whether these biomarkers predict higher signal on Aβ-PET imaging in patients with multiple CMBs. Read More

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Cerebrospinal Fluid Biomarkers for Cerebral Amyloid Angiopathy Diagnosis.

J Alzheimers Dis 2022 May 2. Epub 2022 May 2.

Department of Neurology, Boston University Medical Center and Boston University School of Medicine, Boston, MA, USA.

An accurate diagnosis of sporadic cerebral amyloid angiopathy (CAA) is critical for patient management and research (including clinical trials) for this common small vessel pathology of the brain. While the "big bang" of the CAA field has been the device and wide adoption of the clinico-radiological Boston criteria which allowed for CAA diagnosis during life, these criteria are not without major shortcoming. As it is now becoming evident that CAA is probably not a single disease, but rather represents divergent pathophysiological phenotypes and clinical trajectories, new biomarker-driven diagnostic approaches should be sought. Read More

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Soluble brain homogenates from diverse human and mouse sources preferentially seed diffuse Aβ plaque pathology when injected into newborn mouse hosts.

Free Neuropathol 2022 Jan 23;3(9). Epub 2022 Mar 23.

Department of Neuroscience, College of Medicine, University of Florida, Gainesville, FL 32610, USA.

Background: Seeding of pathology related to Alzheimer's disease (AD) and Lewy body disease (LBD) by tissue homogenates or purified protein aggregates in various model systems has revealed prion-like properties of these disorders. Typically, these homogenates are injected into adult mice stereotaxically. Injection of brain lysates into newborn mice represents an alternative approach of delivering seeds that could direct the evolution of amyloid-β (Aβ) pathology co-mixed with either tau or α-synuclein (αSyn) pathology in susceptible mouse models. Read More

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January 2022

Deep learning from multiple experts improves identification of amyloid neuropathologies.

Acta Neuropathol Commun 2022 04 28;10(1):66. Epub 2022 Apr 28.

Bakar Computational Health Sciences Institute, University of California, San Francisco, CA, 94158, USA.

Pathologists can label pathologies differently, making it challenging to yield consistent assessments in the absence of one ground truth. To address this problem, we present a deep learning (DL) approach that draws on a cohort of experts, weighs each contribution, and is robust to noisy labels. We collected 100,495 annotations on 20,099 candidate amyloid beta neuropathologies (cerebral amyloid angiopathy (CAA), and cored and diffuse plaques) from three institutions, independently annotated by five experts. Read More

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Mediators of cognitive impairment in cerebral amyloid angiopathy.

Int J Stroke 2022 May 19:17474930221099352. Epub 2022 May 19.

Department of Clinical Neurosciences, University of Calgary, Calgary, AB, Canada.

Background: Cerebral amyloid angiopathy (CAA) is associated with cognitive decline. CAA has diverse impacts on brain structure and function; however, the brain lesions that mediate the association of CAA with cognition are not understood well.

Aims: To determine the degree to which CAA neuroimaging biomarkers mediate the association of CAA with cognitive dysfunction. Read More

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Cerebral Amyloid Angiopathy-Related Inflammation in Down Syndrome-Related Alzheimer Disease.

Neurology 2022 Apr 25. Epub 2022 Apr 25.

Memory Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau - Biomedical Research Institute Sant Pau-Universitat Autònoma de Barcelona, Barcelona, Spain

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Unilateral cerebral amyloid angiopathy after neurointervention.

Neurologia (Engl Ed) 2022 Apr 22. Epub 2022 Apr 22.

Servicio de Neurología, Hospital Universitario Miguel Servet, Zaragoza, Spain.

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Prevalence of clinical manifestations and neuroimaging features in cerebral small vessel disease.

Clin Neurol Neurosurg 2022 Jun 12;217:107244. Epub 2022 Apr 12.

Department of Neurology, Cerrahpasa Medical Faculty, Istanbul University-Cerrahpasa, Istanbul, Turkey. Electronic address:

Background And Purpose: Cerebral small vessel disease (CSVD) may present with gradual onset, chronic parkinsonism and/or dementia. In this study, we aimed to identify the prevalence and clinical characteristics of apathy, dementia and parkinsonism in a cohort of patients with CSVD and to determine the neuroimaging features in these patients.

Methods: We included all patients with CSVD, who were admitted to the stroke outpatient clinic between February 2018 and February 2019. Read More

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Heterozygous and Homozygous Variants in Gene in Alzheimer's Disease Patients: Clinical, Neuroimaging and Neuropathological Findings.

Int J Mol Sci 2022 Apr 11;23(8). Epub 2022 Apr 11.

Genetic Service, Hospital Universitario 12 de Octubre, 28041 Madrid, Spain.

In the last few years, the gene has been strongly implicated in the development of Alzheimer's disease (AD). We performed whole-exome sequencing on 37 patients with early-onset dementia or family history suggestive of autosomal dominant dementia. Data analysis was based on a custom panel that included 46 genes related to AD and dementia. Read More

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Cerebral Amyloid Angiopathy-Related Inflammation following Multiple Cancers and Chemotherapies.

Case Rep Neurol 2022 Jan-Apr;14(1):149-156. Epub 2022 Mar 15.

Department of Neurology, Centre Hospitalier Universitaire de Liège, Liège, Belgium.

Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare autoimmune encephalopathy of aging caused by an autoantibody immune response against Aβ protein deposited in the brain of older adults affected by cerebral amyloid angiopathy (CAA) and Alzheimer's disease pathology. Its most common clinical manifestations are (sub)acute-onset cognitive and behavioral abnormalities, focal deficits, seizures, and headaches. Brain magnetic resonance imaging shows characteristic extensive and confluent white matter hyperintensities and CAA features. Read More

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Recent Advances in the Modeling of Alzheimer's Disease.

Front Neurosci 2022 31;16:807473. Epub 2022 Mar 31.

Laboratory for Proteolytic Neuroscience, RIKEN Center for Brain Science, Wako, Japan.

Since 1995, more than 100 transgenic (Tg) mouse models of Alzheimer's disease (AD) have been generated in which mutant amyloid precursor protein (APP) or APP/presenilin 1 (PS1) cDNA is overexpressed ( ). Although many of these models successfully recapitulate major pathological hallmarks of the disease such as amyloid β peptide (Aβ) deposition and neuroinflammation, they have suffered from artificial phenotypes in the form of overproduced or mislocalized APP/PS1 and their functional fragments, as well as calpastatin deficiency-induced early lethality, calpain activation, neuronal cell death without tau pathology, endoplasmic reticulum stresses, and inflammasome involvement. Such artifacts bring two important uncertainties into play, these being (1) why the artifacts arise, and (2) how they affect the interpretation of experimental results. Read More

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Retinal biomarkers of Cerebral Small Vessel Disease: A systematic review.

PLoS One 2022 14;17(4):e0266974. Epub 2022 Apr 14.

Henry and Allison McCance Center for Brain Health, Massachusetts General Hospital, Boston, MA, United States of America.

Introduction: Cerebral Small Vessel Disease (CSVD), a progressive degenerative disorder of small caliber cerebral vessels, represents a major contributor to stroke and vascular dementia incidence worldwide. We sought to conduct a systematic review of the role of retinal biomarkers in diagnosis and characterization of CSVD.

Methods: We conducted a systematic review of MEDLINE, PubMed, Scopus, the Cochrane Library Database, and Web of Science. Read More

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Cerebral Amyloid Angiopathy and Atrial Fibrillation: An up to Date Case Report.

Neurohospitalist 2022 Apr 11;12(2):391-394. Epub 2022 Feb 11.

Department of Neurology, Kings College Hospital Dubai, Dubai, UAE.

Concurrent Cerebral Amyloid Angiopathy (CAA) and Atrial Fibrillation are becoming an increasingly common dilemma in clinical practice due to the aging population and the comorbidities associated with it. In such patients, the physician must appreciate and strike the difficult balance between the risk of ischemic strokes from atrial fibrillation on one hand, and that of intracerebral hemorrhage from coexisting CAA on the other. Anticoagulation is necessary for the former but potentially deleterious for the latter. Read More

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Diffusion tensor tractography of the fornix in cerebral amyloid angiopathy, mild cognitive impairment and Alzheimer's disease.

Neuroimage Clin 2022 4;34:103002. Epub 2022 Apr 4.

Department of Medicine, Division of Neurology and Neuroscience and Mental Health Institute (NMHI), University of Alberta, Edmonton, AB, Canada. Electronic address:

Purpose: Cerebral amyloid angiopathy (CAA) is a common neuropathological finding and clinical entity that occurs independently and with co-existent Alzheimer's disease (AD) and small vessel disease. We compared diffusion tensor imaging (DTI) metrics of the fornix, the primary efferent tract of the hippocampus between CAA, AD and Mild Cognitive Impairment (MCI) and healthy controls.

Methods: Sixty-eight healthy controls, 32 CAA, 21 AD, and 26 MCI patients were recruited at two centers. Read More

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Views on the Desirability of Diagnosing Sporadic Cerebral Amyloid Angiopathy with Biological Evidence.

J Alzheimers Dis 2022 Apr 4. Epub 2022 Apr 4.

Radboud Alzheimer Centre, Radboud University Medical Center, The Netherlands.

Background: Sporadic cerebral amyloid angiopathy (sCAA) research of the past decade has increasingly focused on developing biomarkers that allow for an earlier and more accurate sCAA-diagnosis. Considering that sCAA does not have treatment options available (yet), more fundamental questions concerning the desirability of using such early-sCAA biomarkers in clinical practice need to be addressed.

Objective: In this qualitative interview study, we aim to explore the views of vascular neurologists on the purpose and possible consequences of an earlier and more accurate sCAA-diagnosis, using new biomarkers. Read More

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CT-Visible Convexity Subarachnoid Hemorrhage Predicts Early Recurrence of Lobar Hemorrhage.

Front Neurol 2022 23;13:843851. Epub 2022 Mar 23.

Department of Neurology, Peking University Third Hospital, Beijing, China.

Background And Purpose: Convexity subarachnoid hemorrhage (cSAH) may predict an increased recurrence risk in cerebral amyloid angiopathy (CAA)-related intracerebral hemorrhage (ICH) survivors. We aimed to investigate whether cSAH detected on CT was related to early recurrence in patients with ICH related to CAA.

Methods: We analyzed data from consecutive lobar ICH patients diagnosed as probable or possible CAA according to the Boston criteria using the method of cohort study. Read More

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Assessment of sex-related neuropathology and cognitive deficits in the Tg-SwDI mouse model of Alzheimer's disease.

Behav Brain Res 2022 Jun 6;428:113882. Epub 2022 Apr 6.

Division of Neurotoxicology, National Center for Toxicological Research/FDA, Jefferson, AR 72079, USA. Electronic address:

Cerebral amyloid angiopathy or CAA is a type of vascular dementia that can cause neuroinflammation, ischemia and hemorrhage, among other complications. CAA results from the deposition of amyloid beta (Aβ) in blood vessels and is frequently observed in individuals with Alzheimer's disease (AD). One functional output of those pathological changes is measurable cognitive decline. Read More

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Histopathology of Cerebral Microinfarcts and Microbleeds in Spontaneous Intracerebral Hemorrhage.

Transl Stroke Res 2022 Apr 6. Epub 2022 Apr 6.

Department of Neurology and Neurosurgery, University Medical Center Utrecht Brain Center, Utrecht University, G03.129, PO Box 85500, 3508 GA, Utrecht, The Netherlands.

In patients with spontaneous intracerebral hemorrhage caused by different vasculopathies, cerebral microinfarcts have the same aspect on MRI and the same applies to cerebral microbleeds. It is unclear what pathological changes underlie these cerebral microinfarcts and cerebral microbleeds. In the current study, we explored the histopathological substrate of these lesions by investigating the brain tissue of 20 patients (median age at death 77 years) who died from ICH (9 lobar, 11 non-lobar) with a combination of post-mortem 7-T MRI and histopathological analysis. Read More

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Endothelial BACE1 Impairs Cerebral Small Vessels via Tight Junctions and eNOS.

Circ Res 2022 Apr 6;130(9):1321-1341. Epub 2022 Apr 6.

Institute on Aging and Brain Disorders, The First Affiliated Hospital of USTC and Neurodegenerative Disorder Research Center (H.Z., F.G., X.Y., T.L., Z. Li, Q.W., H.B., Z. Long, Z.W., Y.S.), Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei.

Background: Cerebral small vessel injury, including loss of endothelial tight junctions, endothelial dysfunction, and blood-brain barrier breakdown, is an early and typical pathology for Alzheimer's disease, cerebral amyloid angiopathy, and hypertension-related cerebral small vessel disease. Whether there is a common mechanism contributing to these cerebrovascular alterations remains unclear. Studies have shown an elevation of BACE1 (β-site amyloid precursor protein cleaving enzyme 1) in cerebral vessels from cerebral amyloid angiopathy or Alzheimer's disease patients, suggesting that vascular BACE1 may involve in cerebral small vessel injury. Read More

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Association of Apolipoprotein E ɛ4 Allele with Enlarged Perivascular Spaces.

Ann Neurol 2022 Apr 3. Epub 2022 Apr 3.

NHLBI's Framingham Heart Study, Framingham, MA.

Objective: Enlarged perivascular spaces have emerged as markers of cerebral small vessel disease and are linked to perivascular drainage dysfunction. The apolipoprotein E-ɛ4 (APOE-ɛ4) allele is the strongest genetic risk factor for cerebral amyloid angiopathy and Alzheimer's neuropathology, but the underlying mechanisms remain unclear. We studied the relationship between APOE-ɛ4 and the topography and burden of enlarged perivascular spaces to elucidate underlying mechanisms between APOE-ɛ4 and adverse clinical outcomes. Read More

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Impact of Non-pharmacological Chronic Hypertension on a Transgenic Rat Model of Cerebral Amyloid Angiopathy.

Front Neurosci 2022 15;16:811371. Epub 2022 Mar 15.

Department of Biomedical and Pharmaceutical Sciences, University of Rhode Island, Kingston, RI, United States.

Cerebral amyloid angiopathy (CAA), a common comorbidity of Alzheimer's disease (AD), is a cerebral small vessel disease (CSVD) characterized by deposition of fibrillar amyloid β (Aβ) in blood vessels of the brain and promotes neuroinflammation and vascular cognitive impairment and dementia (VCID). Hypertension, a prominent non-amyloidal CSVD, has been found to increase risk of dementia, but clinical data regarding its effects in CAA patients is controversial. To understand the effects of hypertension on CAA, we bred rTg-DI transgenic rats, a model of CAA, with spontaneously hypertensive, stroke prone (SHR-SP) rats producing bigenic rTg-DI/SHR-SP and non-transgenic SHR-SP littermates. Read More

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Cerebrospinal Fluid Profile of Tau, Phosphorylated Tau, Aβ42, and Aβ40 in Probable Cerebral Amyloid Angiopathy.

J Alzheimers Dis 2022 Apr 1. Epub 2022 Apr 1.

Normandie Univ, UNIROUEN, Inserm U1245 and CHURouen, Department of Neurology and CNR-MAJ, Normandy Center for Genomic and Personalized Medicine, Rouen, France.

Background: There is no consensus regarding the diagnostic value of cerebrospinal fluid (CSF) Alzheimer's disease (AD) biomarkers in cerebral amyloid angiopathy (CAA).

Objective: To describe the CSF levels of Aβ 42, Aβ 40, total protein Tau, and phosphorylated-Tau (p-Tau) in a large series of probable CAA patients and to compare with AD patients in order to identify a specific pattern in CAA but also to look for correlations with the neuroimaging profile.

Methods: We retrospectively included from 2 French centers probable CAA patients according to modified Boston criteria who underwent lumbar puncture (LP) with CSF AD biomarker quantifications. Read More

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