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Brain Pathology

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https://www.readbyqxmd.com/read/28481062/preclinical-transgenic-and-patient-derived-xenograft-models-recapitulate-the-radiological-features-of-human-adamantinomatous-craniopharyngioma
#1
Jessica K R Boult, John R Apps, Annett Hölsken, J Ciaran Hutchinson, Carreno Gabriela, Laura S Danielson, Laura M Smith, Tobias Bäuerle, Rolf Buslei, Michael Buchfelder, Alex K Virasami, Alexander Koers, Owen J Arthurs, Thomas S Jacques, Louis Chesler, Juan Pedro Martinez-Barbera, Simon P Robinson
AIM: To assess the clinical relevance of transgenic and patient-derived xenograft models of adamantinomatous craniopharyngioma (ACP) using serial magnetic resonance imaging (MRI) and high resolution post-mortem micro-computed tomography (μ-CT), with correlation with histology and human ACP imaging. METHODS: The growth patterns and radiological features of tumours arising in Hesx1(Cre/+) ;Ctnnb1(l) °(x(ex3)/+) transgenic mice, and of patient-derived ACP xenografts implanted in the cerebral cortex, were monitored longitudinally in vivo with anatomical and functional MRI, and by ex vivo μ-CT at study end...
May 8, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28474749/loss-of-smarce1-expression-is-a-specific-diagnostic-marker-of-clear-cell-meningioma-a-comprehensive-immunophenotypical-and-molecular-analysis
#2
Arnault Tauziede-Espariat, Béatrice Parfait, Aurore Besnard, Joëlle Lacombe, Johan Pallud, Sanaa Tazi, Stéphanie Puget, Guillaume Lot, Benoît Terris, Julie Cohen, Michel Vidaud, Dominique Figarella-Branger, Franck Monnien, Marc Polivka, Homa Adle-Biassette, Pascale Varlet
Clear cell meningioma (CCM) is a rare grade II histopathological subtype that usually occurs in young patients and displays high recurrence rate. Germline SMARCE1 mutations have been described in hereditary forms of this disease and more recently in small syndromic and sporadic CCM series. The diagnostic value of SMARCE1 in distinguishing between CCM and other meningioma variants has not been yet established. The aim of our study was to investigate the status of SMARCE1 in a series of CCMs and its morphological mimickers...
May 5, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28470822/pericyte-derived-bone-morphogenetic-protein-4-underlies-white-matter-damage-after-chronic-hypoperfusion
#3
Maiko T Uemura, Masafumi Ihara, Takakuni Maki, Takayuki Nakagomi, Seiji Kaji, Kengo Uemura, Tomohiro Matsuyama, Raj N Kalaria, Ayae Kinoshita, Ryosuke Takahashi
Subcortical small vessel disease (SVD) is characterized by white matter damage resulting from arteriolosclerosis and chronic hypoperfusion. Transforming growth factor beta 1 (TGFB1) is dysregulated in the hereditary SVD, CARASIL (cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy). However, very little is known about the role of the largest group in the TGFB superfamily - the bone morphogenetic proteins (BMPs) - in SVD pathogenesis. The aim of this study was to characterize signaling abnormalities of BMPs in sporadic SVD...
May 4, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28452087/cytotoxic-cd8-t-cells-ablation-enhances-the-capacity-of-regulatory-t-cells-to-delay-viral-elimination-in-theiler-s-murine-encephalomyelitis
#4
Malgorzata Ciurkiewicz, Vanessa Herder, Muhammad Akram Khan, Ann-Kathrin Uhde, René Teich, Stephan Floess, Wolfgang Baumgärtner, Jochen Huehn, Andreas Beineke
Theiler's murine encephalomyelitis (TME) of susceptible mouse strains is a commonly used infectious animal model for multiple sclerosis. The study aim was to test the hypothesis whether cytotoxic T cell responses account for the limited impact of regulatory T cells on antiviral immunity in TME virus-induced demyelinating disease (TMEV-IDD) resistant C57BL/6 mice. TME virus-infected C57BL/6 mice were treated with (i) interleukin-2/-anti-interleukin-2-antibody-complexes to expand regulatory T cells ('Treg-expansion'), (ii) anti-CD8-antibodies to deplete cytotoxic T cells ('CD8-depletion') or (iii) with a combination of Treg-expansion and CD8-depletion ('combined treatment') prior to infection...
April 27, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28401686/axonal-loss-in-the-multiple-sclerosis-spinal-cord-revisited
#5
Natalia Petrova, Daniele Carassiti, Daniel R Altmann, David Baker, Klaus Schmierer
Preventing chronic disease deterioration is an unmet need in people with multiple sclerosis, where axonal loss is considered a key substrate of disability. Clinically, chronic multiple sclerosis often presents as progressive myelopathy. Spinal cord cross-sectional area (CSA) assessed using MRI predicts increasing disability and has, by inference, been proposed as an indirect index of axonal degeneration. However, the association between CSA and axonal loss, and their correlation with demyelination, have never been systematically investigated using human post mortem tissue...
April 12, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28380661/characterising-subtypes-of-hippocampal-sclerosis-and-reorganization-correlation-with-pre-and-postoperative-memory-deficit
#6
Anaclara Prada Jardim, Joan Liu, Jack Baber, Zuzanna Michalak, Cheryl Reeves, Matthew Ellis, Jan Novy, Jane de Tisi, Andrew McEvoy, Anna Miserocchi, Elza Marcia Targas Yacubian, Sanjay Sisodiya, Pamela Thompson, Maria Thom
Neuropathological subtypes of hippocampal sclerosis (HS) in temporal lobe epilepsy (The 2013 International League Against Epilepsy classification) are based on the qualitative assessment of patterns of neuronal loss with NeuN. In practice, some cases appear indeterminate between type 1 (CA1 and CA4 loss) and type 2 HS (CA1 loss) and we predicted that MAP2 would enable a more stringent classification. HS subtypes, as well as the accompanying alteration of axonal networks, regenerative capacity and neurodegeneration have been previously correlated with outcome and memory deficits and may provide prognostic clinical information...
April 5, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28321951/transcriptional-network-analysis-in-frontal-cortex-in-lewy-body-diseases-with-focus-on-dementia-with-lewy-bodies
#7
Gabriel Santpere, Paula Garcia-Esparcia, Pol Andres-Benito, Belen Lorente-Galdos, Arcadi Navarro, Isidro Ferrer
The present study investigates global transcriptional changes in frontal cortex area 8 in incidental Lewy Body disease (iLBD), Parkinson disease (PD) and Dementia with Lewy bodies (DLB). We identified different co-expressed gene sets associated with disease stages, and gene ontology categories enriched in gene modules and differentially expressed genes including modules or gene clusters correlated to iLBD comprising upregulated dynein genes and taste receptors, and down-regulated innate inflammation. Focusing on DLB, we found modules with genes significantly enriched in functions related to RNA and DNA production, mitochondria and energy metabolism, purine metabolism, chaperone and protein folding system, and synapses and neurotransmission (particularly the GABAergic system)...
March 21, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28281318/intracranial-myxoid-mesenchymal-tumors-with-ewsr1-creb-family-gene-fusions-myxoid-variant-of-angiomatoid-fibrous-histiocytoma-or-novel-entity
#8
Tejus A Bale, Angelica Oviedo, Harry Kozakewich, Caterina Giannini, Phani K Davineni, Keith Ligon, Sanda Alexandrescu
Intracranial myxoid mesenchymal tumor harboring EWSR1 fusions with CREB family of genes was recently described, and it resembles the myxoid variant of angiomatoid fibrous histiocytoma. We present three pediatric patients with intracranial EWSR1-rearranged myxoid mesenchymal neoplasm and provide a molecular genetic characterization of these tumors. Clinical histories and imaging results were reviewed. Histology, immunohistochemistry, EWSR1, FUS, NR4A3 fluorescence in situ hybridization (FISH), and next-generation sequencing (NGS) were performed...
March 9, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28281308/overlapping-but-distinct-tdp-43-and-tau-pathologic-patterns-in-aged-hippocampi
#9
Vanessa D Smith, Adam D Bachstetter, Eseosa Ighodaro, Kelly Roberts, Erin L Abner, David W Fardo, Peter T Nelson
Intracellular proteinaceous aggregates (inclusion bodies) are almost always detectable at autopsy in brains of elderly individuals. Inclusion bodies composed of TDP-43 and tau proteins often coexist in the same brain, and each of these pathologic biomarkers is associated independently with cognitive impairment. However, uncertainties remain about how the presence and neuroanatomical distribution of inclusion bodies correlate with underlying diseases including Alzheimer's disease (AD). To address this knowledge gap, we analyzed data from the University of Kentucky AD Center autopsy series (n = 247); none of the brains had frontotemporal lobar degeneration...
March 9, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28268246/cerebellar-compartmentation-of-prion-pathogenesis
#10
Audrey Ragagnin, Juliette Ezpeleta, Aurélie Guillemain, François Boudet-Devaud, Anne-Marie Haeberlé, Valérie Demais, Catherine Vidal, Stanislas Demuth, Vincent Béringue, Odile Kellermann, Benoit Schneider, Nancy J Grant, Yannick Bailly
In prion diseases, the brain lesion profile is influenced by the prion "strain" properties, the invasion route to the brain, and still unknown host cell-specific parameters. To gain insight into those endogenous factors, we analyzed the histopathological alterations induced by distinct prion strains in the mouse cerebellum. We show that 22L and ME7 scrapie prion proteins (PrP(22L) , PrP(ME7) ), but not bovine spongiform encephalopathy PrP(6PB1) , accumulate in a reproducible parasagittal banding pattern in the cerebellar cortex of infected mice...
March 7, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28414892/a-19-year-old-male-with-a-well-demarcated-parieto-occipital-mass
#11
Aurélie Beaufrère, Mathilde Fouet, Onorina Bruno, Dominique Cazals-Hatem, Stéphane Goutagny
No abstract text is available yet for this article.
May 2017: Brain Pathology
https://www.readbyqxmd.com/read/28414891/genetically-engineered-mouse-models-of-craniopharyngioma-an-opportunity-for-therapy-development-and-understanding-of-tumor-biology
#12
REVIEW
John Richard Apps, Juan Pedro Martinez-Barbera
Adamantinomatous craniopharyngioma (ACP) is the commonest tumor of the sellar region in childhood. Two genetically engineered mouse models have been developed and are giving valuable insights into ACP biology. These models have identified novel pathways activated in tumors, revealed an important function of paracrine signalling and extended conventional theories about the role of organ-specific stem cells in tumorigenesis. In this review, we summarize these mouse models, what has been learnt, their limitations and open questions for future research...
May 2017: Brain Pathology
https://www.readbyqxmd.com/read/28414890/corrigendum
#13
(no author information available yet)
No abstract text is available yet for this article.
May 2017: Brain Pathology
https://www.readbyqxmd.com/read/28414889/proteomics-in-pediatric-cystic-craniopharyngioma
#14
Luca Massimi, Claudia Martelli, Massimo Caldarelli, Massimo Castagnola, Claudia Desiderio
Adamantinomatous craniopharyngioma (ACP) is still often burdened by a poor prognosis in children as far as the risk of recurrence and the quality of life are concerned. Therefore, many efforts are now dedicated to investigate the molecular characteristics of this tumor aiming at finding new therapeutic options. ACP is prevalently a cystic lesion so that an increasing number of researches are focused on the analysis of its cystic content. In the present article, the main results of the current proteomic analysis (PA) on the ACP fluid are summarized...
May 2017: Brain Pathology
https://www.readbyqxmd.com/read/28414888/models-of-human-adamantinomatous-craniopharyngioma-tissue-steps-toward-an-effective-adjuvant-treatment
#15
Annett Hölsken, Rolf Buslei
Even though ACP is a benign tumor, treatment is challenging because of the tumor's eloquent location. Today, with the exception of surgical intervention and irradiation, further treatment options are limited. However, ongoing molecular research in this field provides insights into the pathways involved in ACP pathogenesis and reveal a plethora of druggable targets. In the next step, appropriate models are essential to identify the most suitable and effective substances for clinical practice. Primary cell cultures in low passages provide a proper and rapid tool for initial drug potency testing...
May 2017: Brain Pathology
https://www.readbyqxmd.com/read/28414887/61-year-old-man-with-chronic-expansile-sellar-mass
#16
Negar Khanlou, Randy Tashjian, Hrayr K Shahinian, Harry V Vinters
No abstract text is available yet for this article.
May 2017: Brain Pathology
https://www.readbyqxmd.com/read/28414886/50-year-old-man-with-a-falcine-mass
#17
A Tauziede-Espariat, H Adle-Biassette, A Simonneau, J M Guinebretiere, M Polivka
No abstract text is available yet for this article.
May 2017: Brain Pathology
https://www.readbyqxmd.com/read/28414885/nasopharyngeal-tumor-developing-in-a-36-year-old-man-following-radiation-for-a-childhood-meningioma
#18
Marwah M Abdulkader, Isaac C Wu, James C Miller, Don-John Summerlin, Eyas M Hattab
No abstract text is available yet for this article.
May 2017: Brain Pathology
https://www.readbyqxmd.com/read/28236350/review-of-xanthomatous-lesions-of-the-sella
#19
B K Kleinschmidt-DeMasters, Kevin O Lillehei, Todd C Hankinson
Xanthomatous lesions of the sellar region have traditionally been divided into two separate categories, xanthomatous hypophysitis (XH) and xanthogranuloma (XG) of the sellar region. The seminal article on XH, a condition typified by foamy histiocytes and lymphoplasmacytic infiltrates in the pituitary gland/sellar region, but usually little or no hemosiderin pigment, detailed three patients. However, most reports since that time have been single cases, making understanding of the entity difficult. In contrast, the seminal report on XG, characterized by sellar region cholesterol clefts, lymphoplasmacytic infiltrates, marked hemosiderin deposits, fibrosis, multinucleated giant cells around cholesterol clefts, eosinophilic granular necrotic debris, and accumulation of macrophages, included 37 patients, allowing more insights into etiology...
May 2017: Brain Pathology
https://www.readbyqxmd.com/read/28236347/adamantinomatous-craniopharyngioma-and-xanthomatous-lesions-of-the-sella
#20
Todd C Hankinson, B K Kleinschmidt-DeMasters
No abstract text is available yet for this article.
May 2017: Brain Pathology
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