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Brain Pathology

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https://www.readbyqxmd.com/read/28401686/axonal-loss-in-the-multiple-sclerosis-spinal-cord-revisited
#1
N Petrova, D Carassiti, D R Altmann, D Baker, K 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 assessed using MRI predicts increasing disability and has, by inference, been proposed as an indirect index of axonal degeneration. However, the association between cross-sectional area 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-reorganisation-correlation-with-pre-and-post-operative-memory-deficit
#2
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 (2013 ILAE 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
#3
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
#4
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
#5
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
#6
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/28236345/unusual-tau-pathology-of-the-cerebellum-in-patients-with-amyotrophic-lateral-sclerosis-parkinsonism-dementia-complex-from-the-kii-peninsula-japan
#7
LETTER
Satoru Morimoto, Hiroyuki Hatsuta, Yasumasa Kokubo, Yuta Nakano, Masato Hasegawa, Misao Yoneda, Yoshifumi Hirokawa, Shigeki Kuzuhara, Taizo Shiraishi, Shigeo Murayama
No abstract text is available yet for this article.
February 25, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28213912/diagnostic-red-flags-steroid-treated-malignant-cns-lymphoma-mimicking-autoimmune-inflammatory-demyelination
#8
Alonso Barrantes-Freer, Aylin Sophie Engel, Odir Antonio Rodríguez-Villagra, Anne Winkler, Markus Bergmann, Christian Mawrin, Tania Kuempfel, Hannah Pellkofer, Imke Metz, Annalen Bleckmann, Silvia Hernández-Durán, Sven Schippling, Elisabeth J Rushing, Stephan Frank, Markus Glatzel, Jakob Matschke, Christian Hartmann, Guido Reifenberger, Wolf Müller, Hans-Ulrich Schildhaus, Wolfgang Brück, Christine Stadelmann
The presence of inflammation and demyelination in a central nervous system (CNS) biopsy points towards a limited, yet heterogeneous group of pathologies, of which multiple sclerosis (MS) represents one of the principal considerations. Inflammatory demyelination has also been reported in patients with clinically suspected primary central nervous system lymphoma (PCNSL), especially when steroids had been administered prior to biopsy acquisition. The histopathological changes induced by corticosteroid treatment can range from mild reduction to complete disappearance of lymphoma cells...
February 18, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28181327/hereditary-spastic-paraplegia-type-8-neuropathological-findings
#9
LETTER
Caroline Pehrson, Jens Michael Hertz, Martin Wirenfeldt, Egon Stenager, Lene Wermuth, Bjarne Winther Kristensen
No abstract text is available yet for this article.
February 9, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28181325/recurrent-copy-number-alterations-in-low-grade-and-anaplastic-pleomorphic-xanthoastrocytoma-with-and-without-braf-v600e-mutation
#10
Rachael A Vaubel, Alissa A Caron, Seiji Yamada, Paul A Decker, Jeanette E Eckel Passow, Fausto J Rodriguez, Amulya A Nageswara Rao, Daniel Lachance, Ian Parney, Robert Jenkins, Caterina Giannini
Pleomorphic xanthoastrocytoma (PXA) is a rare localized glioma characterized by frequent BRAF V600E mutation and CDKN2A/B deletion. We explored the association of copy-number variants (CNVs) with BRAF mutations, tumor grade, and patient survival in a cohort of 41 PXA patients using OncoScan chromosomal microarray. Primary resection specimens were available in 38 cases, including 24 PXA and 14 anaplastic PXA (A-PXA), 23 BRAF V600E mutant tumors (61%). CNVs were identified in all cases and most frequently involved chromosome 9 with homozygous CDKN2A/B deletion (n = 33, 87%), a higher proportion than previously detected by comparative genomic hybridization (50%-60%) (37)...
February 9, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28160367/cerebral-amyloidoma-is-characterized-by-b-cell-clonality-and-a-stable-clinical-course
#11
Katharina Heß, Jan Purrucker, Ute Hegenbart, Benjamin Brokinkel, Rouven Berndt, Kathy Keyvani, Camelia M Monoranu, Mario Löhr, Guido Reifenberger, Christopher Munoz-Bendix, Jörg Kalla, Justus Groß, Uta Schick, Jennifer Kollmer, Wolfram Klapper, Christoph Röcken, Martin Hasselblatt, Werner Paulus
Amyloidomas are rare amyloid-containing lesions, which may also occur in the central nervous system. Etiology, pathogenesis and clinical course are poorly understood. To gain more insight into the biology of cerebral amyloidoma, we aimed to characterize its histopathological, molecular and clinical features in a retrospective series of seven patients. FFPE tissue specimens were examined using immunohistochemistry, chromogenic in situ hybridization (CISH) for light chains kappa and lambda as well as an IgH gene clonality analysis...
February 4, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28414892/a-19-year-old-male-with-a-well-demarcated-parieto-occipital-mass
#12
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
#13
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
#14
(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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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