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Brain Pathology

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https://www.readbyqxmd.com/read/30222900/granular-cell-astrocytoma-an-aggressive-idh-wildtype-diffuse-gliomarwith-molecular-genetic-features-of-primary-glioblastoma
#1
M Adelita Vizcaino, Doreen N Palsgrove, Ming Yuan, Caterina Giannini, Eibar Ernesto Cabrera-Aldana, Aparna Pallavajjala, Peter C Burger, Fausto J Rodriguez
Granular cell astrocytoma (GCA) is a rare adult infiltrating glioma subtype. We studied a series of 39 GCAs. Median age of presentation was 57.8 years and most cases developed in the frontal or temporal lobes. Tumors included grade II (n=14), grade III (n=11), and grade IV (n=14) by WHO criteria. Granular cell morphology was diffuse in 31 (79%) cases and partial in eight (21%). Immunohistochemistry showed frequent positivity for GFAP (28 of 31), OLIG2 (16 of16), and CD68 (27 of 30), but HAM56, CD163, and IBA-1 histiocytic markers were all negative (22 of 22)...
September 17, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30192999/deep-cervical-lymph-node-ligation-aggravates-ad-like-pathology-of-app-ps1-mice
#2
Linmei Wang, Yanli Zhang, Ying Zhao, Charles Marshall, Ting Wu, Ming Xiao
The imbalance between production and clearance of amyloid-beta (Aβ) is a key step in the onset and development of Alzheimer's disease (AD). Therefore, reducing Aβ accumulation in the brain is a promising therapeutic strategy for AD. The recently discovered glymphatic system and meningeal lymphatic vasculature have been shown to be critical for the elimination of interstitial waste products, especially Aβ, from the brain. In the present study, ligation of deep cervical lymph nodes was performed to block drainage of this system and explore the consequences on Aβ-related pathophysiology...
September 7, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30192422/alternative-lengthening-of-telomeres-atrx-loss-and-h3-k27m-mutations-in-histologically-defined-pilocytic-astrocytoma-with-anaplasia
#3
Fausto J Rodriguez, Jacqueline A Brosnan-Cashman, Sariah J Allen, M Adelita Vizcaino, Caterina Giannini, Sandra Camelo-Piragua, Milad Webb, Marcus Matsushita, Nitin Wadhwani, Abeer Tabbarah, Dima Hamideh, Liqun Jiang, Liam Chen, Leonidas D Arvanitis, Hussein H Alnajar, John R Barber, Alicia Rodríguez-Velasco, Brent Orr, Christopher M Heaphy
Anaplasia may be identified in a subset of tumors with a presumed pilocytic astrocytoma (PA) component or piloid features, which may be associated with aggressive behavior, but the biologic basis of this change remains unclear. Fifty-seven resections from 36 patients (23 M, 13 F, mean age 32 years, range 3-75) were included. A clinical diagnosis of NF1 was present in 8 (22%). Alternative lengthening of telomeres (ALT) was assessed by telomere-specific FISH and/or CISH. A combination of immunohistochemistry, DNA sequencing and FISH were used to study BRAF, ATRX, CDKN2A/p16, mutant IDH1 p...
September 7, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30106209/alzheimer-s-disease-and-type-2-diabetes-mellitus-are-distinct-diseases-with-potential-overlapping-metabolic-dysfunction-upstream-of-observed-cognitive-decline
#4
REVIEW
Yevgen Chornenkyy, Wang-Xia Wang, Angela Wei, Peter T Nelson
Alzheimer's disease (AD) and type-2 diabetes mellitus (T2DM) are highly prevalent aging-related diseases associated with significant morbidity and mortality. Some findings in human and animal models have linked T2DM to AD-type dementia. Despite epidemiological associations between the T2DM and cognitive impairment, the inter-relational mechanisms are unclear. The preponderance of evidence in longitudinal studies with autopsy confirmation have indicated that vascular mechanisms, rather than classic AD-type pathologies, underlie the cognitive decline often seen in self-reported T2DM...
August 14, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30062819/trex1-is-expressed-by-microglia-in-normal-human-brain-and-increases-in-regions-affected-by-ischemia
#5
Parul H Kothari, Grant R Kolar, Joanna C Jen, Rula Hajj-Ali, Paula Bertram, Robert E Schmidt, John P Atkinson
BACKGROUND: Mutations in the three-prime repair exonuclease 1 (TREX1) gene have been associated with neurological diseases, including Retinal Vasculopathy with Cerebral Leukoencephalopathy (RVCL). However, the endogenous expression of TREX1 in human brain has not been studied. METHODS: We produced a rabbit polyclonal antibody (pAb) to TREX1 to characterize TREX1 by Western blotting (WB) of cell lysates from normal controls and subjects carrying an RVCL frame-shift mutation...
July 30, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30062722/the-association-between-hypertensive-arteriopathy-and-cerebral-amyloid-angiopathy-in-spontaneously-hypertensive-stroke-prone-rats
#6
Solveig Jandke, Cornelia Garz, Daniel Schwanke, Michael Sendtner, Hans-Jochen Heinze, Roxana O Carare, Stefanie Schreiber
We aimed to test the hypothesis that in spontaneously hypertensive stroke-prone (SHRSP) rats, non-amyloid cerebral small vessel disease/hypertensive arteriopathy (HA) results in vessel wall injury that may promote cerebral amyloid angiopathy (CAA). Our study comprised 21 male SHRSP (age 17-44 weeks) and 10 age- and sex-matched Wistar control rats, that underwent two-photon (2PM) imaging of the arterioles in the parietal cortex using Methoxy-X04, Dextran and cerebral blood flow (CBF) measurements. Our data suggests that HA in SHRSP progresses in a temporal and age-dependent manner, starting from small vessel wall damage (stage 1A), proceeding to CBF reduction (stage 1B), non-occlusive (stage 2) and finally occlusive thrombi (stage 3)...
July 30, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30052311/knockdown-of-hif-1%C3%AE-impairs-post-ischemic-vascular-reconstruction-in-the-brain-via-deficient-homing-and-sprouting-bmepcs
#7
Yang Liu, Ran He, Yaping Xiao, Hongjin Wang, Yi Chen, Weihai Chen, Xiaoyu Xu
Although the critical role of hypoxia inducible factor-1α (HIF-1α) in cerebral neovascularization after stroke has been well characterized, the details regarding the regulation of endothelial progenitor cell (EPC)-dependent neovascularization by HIF-1α are not completely understood. Using lentiviral shRNA to knockdown HIF-1α, we showed that HIF-1α plays a central role in bone marrow-derived EPC (bmEPC) homing and sprouting in the post-acute stage of ischemic Sprague Dawley (SD) rat brains. First, knockdown of HIF-1α decreased the homing of both endogenous and exogenous bmEPCs to the ischemic brain...
July 27, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30051532/lysosomal-cathepsin-d-is-upregulated-in-alzheimer-s-disease-neocortex-and-may-be-a-marker-for-neurofibrillary-degeneration
#8
Yuek Ling Chai, Joyce R Chong, Jiaju Weng, David Howlett, Andrea Halsey, Jasinda H Lee, Johannes Attems, Dag Aarsland, Paul T Francis, Christopher P Chen, Mitchell K P Lai
Alzheimer's disease (AD) is characterized by accumulation of β-amyloid plaques (AP) and neurofibrillary tangles (NFT) in the cortex, together with synaptic loss and amyloid angiopathy. Perturbations in the brain lysosomal system, including the cathepsin family of proteases, have been implicated in AD where they may be involved in proteolytic clearance of misfolded and abnormally aggregated peptides. However, the status of cathepsin D (catD) is unclear in Lewy body dementia, the second most common form of neurodegenerative dementia after AD, and characterized by Lewy bodies (LB) containing aggregated α-synuclein...
July 27, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30051528/the-genetic-landscape-of-anaplastic-pleomorphic-xanthoastrocytoma
#9
Joanna J Phillips, Henry Gong, Katharine Chen, Nancy M Joseph, Jessica van Ziffle, Boris C Bastian, James P Grenert, Cassie N Kline, Sabine Mueller, Anuradha Banerjee, Theodore Nicolaides, Nalin Gupta, Mitchel S Berger, Han S Lee, Melike Pekmezci, Tarik Tihan, Andrew W Bollen, Arie Perry, Joseph T C Shieh, David A Solomon
Pleomorphic xanthoastrocytoma (PXA) is an astrocytic neoplasm that is typically well circumscribed and can have a relatively favorable prognosis. Tumor progression to anaplastic PXA (WHO grade III), however, is associated with a more aggressive biologic behavior and worse prognosis. The factors that drive anaplastic progression are largely unknown. We performed comprehensive genomic profiling on a set of twenty-three PXAs from 19 patients, including 15 with anaplastic PXA. Four patients had tumor tissue from multiple recurrences, including two with anaplastic progression...
July 27, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30051525/a-domain-responsible-for-spontaneous-conversion-of-bank-vole-prion-protein
#10
Atsushi Kobayashi, Yuichi Matsuura, Atsuko Takeuchi, Masahito Yamada, Ichiro Miyoshi, Shirou Mohri, Tetsuyuki Kitamoto
Bank vole is a small rodent that shows high susceptibility to infection with diverse prion strains. To determine whether the increased susceptibility of bank voles to prion diseases can be attributed to the intrinsic nature of bank vole prion protein (PrP) or to host factors other than PrP, we produced transgenic mice overexpressing bank vole PrP. These transgenic mice spontaneously developed neurological illness with spongiform changes and the accumulation of abnormal PrP in the brain. Then, we produced transgenic mice overexpressing chimeric mouse/bank vole PrP, which differs from mouse PrP only at two residues located at the C-terminus, to determine the minimum essential domain for the induction of spontaneous generation of abnormal PrP...
July 27, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30028551/neuronal-susceptibility-to-beta-amyloid-toxicity-and-ischemic-injury-involves-histone-deacetylase-2-regulation-of-endophilin-b1
#11
David B Wang, Chizuru Kinoshita, Yoshito Kinoshita, Bryce L Sopher, Takuma Uo, Rona J Lee, Joon Kyu Kim, Sean P Murphy, C Dirk Keene, Gwenn A Garden, Richard S Morrison
Histone deacetylases (HDACs) catalyze acetyl group removal from histone proteins, leading to altered chromatin structure and gene expression. HDAC2 is highly expressed in adult brain, and HDAC2 levels are elevated in Alzheimer's disease (AD) brain. We previously reported that neuron-specific splice isoforms of Endophilin-B1 (Endo-B1), promote neuronal survival, but are reduced in human AD brain and mouse models of AD and stroke. Here we demonstrate that HDAC2 suppresses Endo-B1 expression. HDAC2 knockdown or knockout enhances expression of Endo-B1...
July 20, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30021052/dissecting-the-neuronal-vulnerability-underpinning-alpers-syndrome-a-clinical-and-neuropathological-study
#12
Hannah Hayhurst, Maria-Eleni Anagnostou, Helen J Bogle, John P Grady, Robert W Taylor, Laurence A Bindoff, Robert McFarland, Doug M Turnbull, Nichola Z Lax
Alpers' syndrome is an early-onset neurodegenerative disorder often caused by biallelic pathogenic variants in the gene encoding the catalytic subunit of polymerase-gamma (POLG) which is essential for mitochondrial DNA (mtDNA) replication. Alpers' syndrome is characterised by intractable epilepsy, developmental regression and liver failure which typically affects children aged 6 months - 3 years. Although later onset variants are now recognised, they differ in that they are primarily an epileptic encephalopathy with ataxia...
July 18, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30020563/mri-of-cortical-lesions-and-its-use-in-studying-their-role-in-ms-pathogenesis-and-disease-course
#13
R Magliozzi, R Reynolds, M Calabrese
Cortical grey matter (GM) demyelination is present from the earliest stages of multiple sclerosis (MS) and is associated with physical deficits and cognitive impairment. In particular, the rate of disability progression in MS, both in the relapsing and progressive phases, appears to be strictly associated with degenerative GM demyelination and diffuse cortical atrophy. In the last decade, several histopathological studies and advanced radiological methodologies have contributed to better identify the exact involvement/load of cortical pathology in MS, even if the specific inflammatory features and the precise cell and molecular mechanisms of GM demyelination and neurodegeneration in MS remain still not fully understood...
July 18, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30020561/a-clinical-and-histopathological-study-of-malformations-observed-in-fetuses-infected-by-the-zika-virus
#14
A Beaufrère, B Bessières, M Bonnière, M Driessen, C Alfano, T Couderc, M Thiry, N Thelen, M Lecuit, T Attié-Bitach, M Vekemans, Y Ville, L Nguyen, M Leruez-Ville, F Encha-Razavi
BACKGROUND: The recent outbreak of Zika virus (ZIKV) infection and the associated increased prevalence of microcephaly in Brazil underline the impact of viral infections on embryo-fetal development. The aim of the present study is to provide a detailed clinical and histopathological study of the fetal disruption caused by the ZIKV, with a special focus on the associated neuropathological findings. METHODS: A detailed feto-placental examination, as well as neuropathological and neurobiological studies were performed on three fetuses collected after pregnancy termination between 22 to 25 weeks of gestation (WG), because brain malformations associated with a maternal and fetal ZIKV infection was diagnosed...
July 18, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30020560/exploring-the-heterogeneity-of-ms-lesions-using-positron-emission-tomography-a-reappraisal-of-their-contribution-to-disability
#15
Bruno Stankoff, Emilie Poirion, Matteo Tonietto, Benedetta Bodini
The biological mechanisms driving disability worsening in multiple sclerosis (MS) are only partly understood.. Monitoring changes in lesion load on MRI has a limited predictive value on the development of clinical disability, and there is an essential need for novel imaging markers specific for the main candidate mechanisms underlying neurodegeneration which include failing myelin repair, innate immune cell activation and grey matter neuronal damage. Positron Emission Tomography (PET) is an imaging technology based on the injection of radiotracers directed against specific molecular targets and has recently allowed the selective quantification in-vivo of the key biological mechanisms relevant to MS pathophysiology...
July 18, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30020556/iron-related-changes-in-ms-lesions-and-their-validity-to-characterize-ms-lesion-types-and-dynamics-with-ultra-high-field-magnetic-resonance-imaging
#16
Simon Hametner, Assunta Dal Bianco, Siegfried Trattnig, Hans Lassmann
Iron accumulates with age in the normal human brain. This process is altered at several levels in the brain of multiple sclerosis (MS) patients. Since iron is mainly stored in oligodendrocytes and myelin in the normal brain, its liberation in demyelinating lesions may amplify tissue damage in demyelinating lesions and its uptake in macrophages and microglia may help to more precisely define activity stages of the lesions. In addition, glia cells change their iron import, export and storage properties in MS lesions, which is reflected by alterations in the expression of iron transport molecules...
July 18, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30133867/a-56-year-old-woman-with-blindness-and-mental-status-changes
#17
Rachael A Vaubel, R Ross Reichard
No abstract text is available yet for this article.
July 2018: Brain Pathology
https://www.readbyqxmd.com/read/30133866/unexpected-death-of-a-9-year-old-girl
#18
Celeste I Herbst, John J Dempers, S Dan Zaharie
No abstract text is available yet for this article.
July 2018: Brain Pathology
https://www.readbyqxmd.com/read/30133865/a-72-year-old-female-with-an-intraventricular-mass
#19
Fenghua Zhang, Liam Chen
No abstract text is available yet for this article.
July 2018: Brain Pathology
https://www.readbyqxmd.com/read/30133864/traumatic-brain-injury-a-platform-for-studies-in-a%C3%AE-processing-commentary-on-rapid-a%C3%AE-oligomer-and-protofibril-accumulation-in-traumatic-brain-injury
#20
Douglas H Smith, William Stewart
No abstract text is available yet for this article.
July 2018: Brain Pathology
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