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Cerebral amyloid angiopathy

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https://www.readbyqxmd.com/read/29331631/the-edinburgh-ct-and-genetic-diagnostic-criteria-for-lobar-intracerebral-haemorrhage-associated-with-cerebral-amyloid-angiopathy-model-development-and-diagnostic-test-accuracy-study
#1
Mark A Rodrigues, Neshika Samarasekera, Christine Lerpiniere, Catherine Humphreys, Mark O McCarron, Philip M White, James A R Nicoll, Cathie L M Sudlow, Charlotte Cordonnier, Joanna M Wardlaw, Colin Smith, Rustam Al-Shahi Salman
BACKGROUND: Identification of lobar spontaneous intracerebral haemorrhage associated with cerebral amyloid angiopathy (CAA) is important because it is associated with a higher risk of recurrent intracerebral haemorrhage than arteriolosclerosis-associated intracerebral haemorrhage. We aimed to develop a prediction model for the identification of CAA-associated lobar intracerebral haemorrhage using CT features and genotype. METHODS: We identified adults with first-ever intracerebral haemorrhage diagnosed by CT, who died and underwent research autopsy as part of the Lothian IntraCerebral Haemorrhage, Pathology, Imaging and Neurological Outcome (LINCHPIN) study, a prospective, population-based, inception cohort...
January 10, 2018: Lancet Neurology
https://www.readbyqxmd.com/read/29331207/cerebral-amyloid-angiopathy-related-inflammation-a-case-report-presenting-diagnostic-difficulties
#2
Cecylia Rajczewska-Oleszkiewicz, Agnieszka Cyganek, Anna Stadnik, Dorota Dziewulska
We describe an 86-year-old woman with a history of hypertension who presented sudden disturbances of consciousness and left hemiparesis. Brain magnetic resonance imaging (MRI) revealed diffused hyperintensive changes on T2-weighted images localized subcortically in the white matter of both cerebral hemispheres, corresponding to acute vasogenic edema, causing moderate mass effect. Posterior reversible encephalopathy syndrome was initially diagnosed. After implementation of anti-edema intravenous steroid treatment and hypotensive therapy the symptoms began to retire, till the total regression...
January 6, 2018: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/29311311/structural-heterogeneity-and-intersubject-variability-of-a%C3%AE-in-familial-and-sporadic-alzheimer-s-disease
#3
Carlo Condello, Thomas Lemmin, Jan Stöhr, Mimi Nick, Yibing Wu, Alison M Maxwell, Joel C Watts, Christoffer D Caro, Abby Oehler, C Dirk Keene, Thomas D Bird, Sjoerd G van Duinen, Lars Lannfelt, Martin Ingelsson, Caroline Graff, Kurt Giles, William F DeGrado, Stanley B Prusiner
Point mutations in the amyloid-β (Aβ) coding region produce a combination of mutant and WT Aβ isoforms that yield unique clinicopathologies in familial Alzheimer's disease (fAD) and cerebral amyloid angiopathy (fCAA) patients. Here, we report a method to investigate the structural variability of amyloid deposits found in fAD, fCAA, and sporadic AD (sAD). Using this approach, we demonstrate that mutant Aβ determines WT Aβ conformation through prion template-directed misfolding. Using principal component analysis of multiple structure-sensitive fluorescent amyloid-binding dyes, we assessed the conformational variability of Aβ deposits in fAD, fCAA, and sAD patients...
January 8, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29310723/iatrogenic-creutzfeldt-jakob-disease-with-amyloid-%C3%AE-pathology-an-international-study
#4
Ignazio Cali, Mark L Cohen, Stéphane Haїk, Piero Parchi, Giorgio Giaccone, Steven J Collins, Diane Kofskey, Han Wang, Catriona A McLean, Jean-Philippe Brandel, Nicolas Privat, Véronique Sazdovitch, Charles Duyckaerts, Tetsuyuki Kitamoto, Ermias D Belay, Ryan A Maddox, Fabrizio Tagliavini, Maurizio Pocchiari, Ellen Leschek, Brian S Appleby, Jiri G Safar, Lawrence B Schonberger, Pierluigi Gambetti
The presence of pathology related to the deposition of amyloid-β (Aβ) has been recently reported in iatrogenic Creutzfeldt-Jakob disease (iCJD) acquired from inoculation of growth hormone (GH) extracted from human cadaveric pituitary gland or use of cadaveric dura mater (DM) grafts.To investigate this phenomenon further, a cohort of 27 iCJD cases - 21 with adequate number of histopathological sections - originating from Australia, France, Italy, and the Unites States, were examined by immunohistochemistry, amyloid staining, and Western blot analysis of the scrapie prion protein (PrPSc), and compared with age-group matched cases of sporadic CJD (sCJD), Alzheimer disease (AD) or free of neurodegenerative diseases (non-ND)...
January 8, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29273521/the-pathology-and-pathophysiology-of-vascular-dementia
#5
REVIEW
Raj N Kalaria
Vascular dementia (VaD) is widely recognised as the second most common type of dementia. Consensus and accurate diagnosis of clinically suspected VaD relies on wide-ranging clinical, neuropsychological and neuroimaging measures in life but more importantly pathological confirmation. Factors defining subtypes of VaD include the nature and extent of vascular pathologies, degree of involvement of extra and intracranial vessels and the anatomical location of tissue changes as well as time after the initial vascular event...
December 19, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/29260245/-cerebral-amyloid-angiopathy-associated-with-inflammation
#6
J Schaumberg, M Trauscheid, B Eckert, D Petersen, W Schulz-Schaeffer, J Röther, W Heide
Cerebral amyloid angiopathy (CAA) associated with inflammation is a rare form of a potentially reversible encephalopathy in a subgroup of patients with CAA. The cerebral amyloid deposition can in isolated cases induce an inflammation predominantly of the cerebral blood vessels and a multifocal edema of the cerebral white matter. The courses can occur as monophasic, relapsing remitting and primarily progressive forms. We present seven cases with different courses of the disease and give an overview of the pathophysiology, clinical aspects and treatment of the disease with reference to the current literature...
December 19, 2017: Der Nervenarzt
https://www.readbyqxmd.com/read/29249378/cerebral-amyloid-angiopathy-the-modified-boston-criteria-in-clinical-practice
#7
André Caetano, Filipa Ladeira, Raquel Barbosa, Sofia Calado, Miguel Viana-Baptista
BACKGROUND: Early identification of patients with cerebral amyloid angiopathy (CAA) is relevant considering the increased risk for cerebral hemorrhage. A new set of diagnostic criteria for CAA was recently proposed, which include the presence of superficial siderosis. We aimed to assess the impact of applying these criteria regarding use of antithrombotic therapy. METHODS: Review of consecutive patients admitted to a Neurology Department from 2014 to 2016, with acute parenchymal or subarachnoid hemorrhage and/or atypical transient focal neurological episodes...
January 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29248380/clinical-pathologic-case-report-a-70-year-old-man-with-inflammatory-cerebral-amyloid-angiopathy-causing-headache-cognitive-impairment-and-aphasia
#8
Grace F Crotty, Kathleen McKee, Altaf Saadi, Andrew C Young, Isaac H Solomon, Jennifer L Lyons
A 70-year-old man presented with two months of worsening cognitive impairment, hallucinations, and difficulty speaking, with superimposed headaches. Cerebrospinal fluid analysis was notable for lymphocytic pleocytosis and elevated protein. Imaging studies revealed multiple acute and subacute infarcts with cortical microhemorrhages. The patient underwent a stereotactic brain biopsy. In this article, we discuss the patient's differential diagnosis, pathologic findings, ultimate diagnosis, and clinical outcome...
December 13, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29247143/cognitive-impairment-before-intracerebral-hemorrhage-is-associated-with-cerebral-amyloid-angiopathy
#9
Gargi Banerjee, Duncan Wilson, Gareth Ambler, Karen Osei-Bonsu Appiah, Clare Shakeshaft, Surabhika Lunawat, Hannah Cohen, Tarek Yousry, Gregory Y H Lip, Keith W Muir, Martin M Brown, Rustam Al-Shahi Salman, Hans Rolf Jäger, David J Werring
BACKGROUND AND PURPOSE: Although the association between cerebral amyloid angiopathy (CAA) and cognitive impairment is increasingly recognized, it is not clear whether this is because of the impact of recurrent intracerebral hemorrhage (ICH) events, disruptions caused by cerebral small vessel damage, or both. We investigated this by considering whether cognitive impairment before ICH was associated with neuroimaging features of CAA on magnetic resonance imaging. METHODS: We studied 166 patients with neuroimaging-confirmed ICH recruited to a prospective multicentre observational study...
December 15, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/29247070/mixed-location-cerebral-hemorrhage-microbleeds-underlying-microangiopathy-and-recurrence-risk
#10
Marco Pasi, Andreas Charidimou, Gregoire Boulouis, Eitan Auriel, Alison Ayres, Kristin M Schwab, Joshua N Goldstein, Jonathan Rosand, Anand Viswanathan, Leonardo Pantoni, Steven M Greenberg, M Edip Gurol
OBJECTIVE: To assess the predominant type of cerebral small vessel disease (SVD) and recurrence risk in patients who present with a combination of lobar and deep intracerebral hemorrhage (ICH)/microbleed locations (mixed ICH). METHODS: Of 391 consecutive patients with primary ICH enrolled in a prospective registry, 75 (19%) had mixed ICH. Their demographics, clinical/laboratory features, and SVD neuroimaging markers were compared to those of 191 patients with probable cerebral amyloid angiopathy (CAA-ICH) and 125 with hypertensive strictly deep microbleeds and ICH (HTN-ICH)...
December 15, 2017: Neurology
https://www.readbyqxmd.com/read/29246595/identification-of-post-translational-modifications-of-a%C3%AE-peptide-in-platelet-membranes-from-patients-with-cerebral-amyloid-angiopathy
#11
Lin Song, Rong Xue, Peng Ge, Man Li, Liang Wang, Fang Zheng, Lili Zhao, Zhexiang Wang, Zhuowei Wang, Qianqian Wang, Ning Liu, Xuguo Sun
Cerebral amyloid angiopathy (CAA) is characterized by cerebrovascular amyloid deposition. It contributes to the rate of cognitive decline in older individuals and is present in >90% of patients with Alzheimer's disease (AD), with no cure so far. Molecular modifications during CAA should be elucidated to improve its diagnosis and treatment. In this study, amyloid-β (Aβ) aggregates in platelet membranes from 65 patients with CAA and 66 healthy volunteers (controls) were confirmed through thioflavin T (ThT) assay and Western blot analysis...
December 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29237799/differential-associations-between-retinal-signs-and-cmbs-by-location-the-ages-reykjavik-study
#12
Chengxuan Qiu, Jie Ding, Sigurdur Sigurdsson, Diana E Fisher, Qian Zhang, Gudny Eiriksdottir, Ronald Klein, Mark A van Buchem, Vilmundur Gudnason, Mary Frances Cotch, Lenore J Launer
OBJECTIVE: To test the hypothesis that age-related macular degeneration (AMD) and retinal microvascular signs are differentially associated with lobar and deep cerebral microbleeds (CMBs). METHODS: CMBs in lobar regions indicate cerebral amyloid angiopathy (CAA). β-Amyloid deposits are implicated in both CAA and AMD. Deep CMBs are associated with hypertension, a major risk factor for retinal microvascular damage. This population-based cohort study included 2,502 participants in the Age, Gene/Environment Susceptibility (AGES)-Reykjavik Study who undertook binocular digital retinal photographs at baseline (2002-2006) to assess retinal microvascular signs and AMD and brain MRI scan at both baseline and follow-up (2007-2011) to assess CMBs...
December 13, 2017: Neurology
https://www.readbyqxmd.com/read/29236888/convexity-subarachnoid-hemorrhage-clinical-features-and-etiology-of-an-argentinian-cohort
#13
Aníbal Chertcoff, Lucrecia Bandeo, Fátima Pantiu, Luciana León Cejas, Sol Pacha, Claudia Uribe Roca, Manuel Fernández Pardal, Ricardo Reisin, Pablo Bonardo
Nontraumatic convexity subarachnoid hemorrhage is an increasingly recognized subtype of subarachnoid bleeding. OBJECTIVE: Our aim was to describe the etiology and clinical features of a cohort of patients with convexity subarachnoid hemorrhage. METHODS: We retrospectively analyzed all cases of convexity subarachnoid hemorrhage admitted to our hospital between January 2012 and April 2017. Demographic features, clinical characteristics, complementary investigations, etiology and mortality were assessed...
December 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/29222944/cerebral-microbleeds-an-mri-review-of-common-and-less-common-causes
#14
REVIEW
Dimitri Renard
Cerebral microbleeds (CMB) are small foci of (acute, subacute, or chronic) blood products, best seen using MRI techniques sensitive to iron deposits (i.e. gradient-echo T2*-weighted and susceptibility-weighted imaging), frequently encountered in small vessel disease (SVD, with hypertensive vasculopathy and cerebral amyloid angiopathy as most frequent conditions) and also in other disorders. In this review, we will mainly discuss the MRI characteristics of CMB and the associated MRI abnormalities encountered in common and less common SVD and non-SVD conditions...
December 9, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29204961/patterns-of-convexal-subarachnoid-haemorrhage-clinical-radiological-and-outcome-differences-between-cerebral-amyloid-angiopathy-and-other-causes
#15
Lionel Calviere, Nicolas Raposo, Victor Cuvinciuc, Christophe Cognard, Fabrice Bonneville, Alain Viguier
BACKGROUND: Cerebral amyloid angiopathy (CAA) is a common aetiology of convexal subarachnoid haemorrhage (cSAH) but little is known about its specific characteristics in comparison with cSAH from other causes. In this study we compared patients with CAA vs. non-CAA-related cSAH. METHODS: Retrospective review of baseline and follow-up data of consecutive patients admitted with a symptomatic acute cSAH. RESULTS: Sixty-two patients were included (mean age 66...
December 4, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/29201555/background-and-distribution-of-lobar-microbleeds-in-cognitive-dysfunction
#16
Hirofumi Matsuyama, Yuichiro Ii, Masayuki Maeda, Maki Umino, Yukito Ueda, Ken-Ichi Tabei, Hirotaka Kida, Masayuki Satoh, Akihiro Shindo, Akira Taniguchi, Ryosuke Takahashi, Hidekazu Tomimoto
Objectives: Cerebral microbleeds (CMBs) are often observed in memory clinic patients. It has been generally accepted that deep CMBs (D-CMBs) result from hypertensive vasculopathy (HV), whereas strictly lobar CMBs (SL-CMBs) result from cerebral amyloid angiopathy (CAA) which frequently coexists with Alzheimer's disease (AD). Mixed CMBs (M-CMBs) have been partially attributed to HV and also partially attributed to CAA. The aim of this study was to elucidate the differences between SL-CMBs and M-CMBs in terms of clinical features and regional distribution...
November 2017: Brain and Behavior
https://www.readbyqxmd.com/read/29195510/cerebrovascular-pathology-in-down-syndrome-and-alzheimer-disease
#17
Elizabeth Head, Michael J Phelan, Eric Doran, Ronald C Kim, Wayne W Poon, Frederick A Schmitt, Ira T Lott
People with Down syndrome (DS) are at high risk for developing Alzheimer disease (AD) with age. Typically, by age 40 years, most people with DS have sufficient neuropathology for an AD diagnosis. Interestingly, atherosclerosis and hypertension are atypical in DS with age, suggesting the lack of these vascular risk factors may be associated with reduced cerebrovascular pathology. However, because the extra copy of APP leads to increased beta-amyloid peptide (Aβ) accumulation in DS, we hypothesized that there would be more extensive and widespread cerebral amyloid angiopathy (CAA) with age in DS relative to sporadic AD...
December 1, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29183952/cerebellar-hematoma-location-implications-for-the-underlying-microangiopathy
#18
Marco Pasi, Sandro Marini, Andrea Morotti, Gregoire Boulouis, Li Xiong, Andreas Charidimou, Alison M Ayres, Myung Joo Lee, Alessandro Biffi, Joshua N Goldstein, Jonathan Rosand, M Edip Gurol, Steven M Greenberg, Anand Viswanathan
BACKGROUND AND PURPOSE: Spontaneous cerebellar intracerebral hemorrhage (ICH) has been reported to be mainly associated with vascular changes secondary to hypertension. However, a subgroup of cerebellar ICH seems related to vascular amyloid deposition (cerebral amyloid angiopathy). We sought to determine whether location of hematoma in the cerebellum (deep and superficial regions) was suggestive of a particular hemorrhage-prone small-vessel disease pathology (cerebral amyloid angiopathy or hypertensive vasculopathy)...
November 28, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/29168672/acute-diffusion-weighted-imaging-lesions-in-cerebral-amyloid-angiopathy-related-convexal-subarachnoid-hemorrhage
#19
Markus Beitzke, Christian Enzinger, Alexander Pichler, Gerit Wünsch, Franz Fazekas
Small acute diffusion-weighted imaging (DWI) lesions can accompany intracerebral hemorrhage due to cerebral amyloid angiopathy (CAA). We therefore examined the occurrence of such lesions in the context of CAA-related convexal subarachnoid hemorrhage (cSAH) both in a cross-sectional and longitudinal manner. DWI lesions were noted in 14/29 (48%) patients at their index cSAH and 12/21 patients (57%) showed acute small DWI lesions at follow-up MRI. Forty-four of 71 (62%) DWI lesions were spatially related to areas of cortical superficial siderosis...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/29167486/distinctive-clinical-effects-of-haemorrhagic-markers-in-cerebral-amyloid-angiopathy
#20
Young Kyoung Jang, Hee Jin Kim, Jin San Lee, Yeo Jin Kim, Ko Woon Kim, Yeshin Kim, Hyemin Jang, Juyoun Lee, Jong Min Lee, Seung-Joo Kim, Kyung-Ho Yu, Andreas Charidimou, David J Werring, Sung Tae Kim, Duk L Na, Sang Won Seo
Restricted lobar cerebral microbleeds (CMBs) and cortical superficial siderosis (CSS) are the characteristic markers of cerebral amyloid angiopathy (CAA). However, their effects on clinical features has not been evaluated well. The purpose of this study is to investigate the clinical implication of these markers in clinical-radiologically diagnosed CAA. A total of 372 patients with possible or probable CAA who met the modified Boston criteria were recruited in a memory clinic setting. Cortical thickness was measured using surface based methods...
November 22, 2017: Scientific Reports
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