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Brain Pathology

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https://www.readbyqxmd.com/read/30417460/clinicopathologic-features-of-anaplastic-myxopapillary-ependymomas
#1
Julieann C Lee, Nima Sharifai, Sonika Dahiya, B K Kleinschmidt-DeMasters, Marc K Rosenblum, Gerald F Reis, David Samuel, Aleli M Siongco, Mariarita Santi, Phillip B Storm, Sean P Ferris, Andrew W Bollen, Melike Pekmezci, David A Solomon, Tarik Tihan, Arie Perry
Myxopapillary ependymomas (MPE) are considered benign (WHO grade I) neoplasms with favorable prognosis. However, malignant behavior occurs in a small subset. To our knowledge, only five anaplastic MPEs have been reported without consensus on diagnostic criteria. We retrieved fourteen anaplastic MPEs from the pathology archives of six institutions. Each tumor included at least two of the following features: > 5 mitoses per 10 high power fields, Ki-67 labeling index (LI) > 10%, microvascular proliferation (MVP), and spontaneous necrosis...
November 12, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30403311/regulation-of-glioma-cell-invasion-by-3q26-gene-products-pik3ca-sox2-and-opa1
#2
Thorsten Schaefer, Archana Ramadoss, Severina Leu, Lionel Tintignac, Cristobal Tostado, Andrea Bink, Christoph Schürch, Joelle Müller, Jonas Schärer, Giusi Moffa, Philippe Demougin, Suzette Moes, Christoph Stippich, Simona Falbo, Heike Neddersen, Heiner Bucher, Stephan Frank, Paul Jenö, Claudia Lengerke, Marie-Françoise Ritz, Luigi Mariani, Jean-Louis Boulay
Diffuse gliomas progress by invading neighboring brain tissue to promote post-operative relapse. Transcription factor SOX2 is highly expressed in invasive gliomas and maps to chromosome region 3q26 together with the genes for PI3K/AKT signaling activator PIK3CA and effector molecules of mitochondria fusion and cell invasion, MFN1 and OPA1. Gene copy number analysis at 3q26 from 129 glioma patient biopsies revealed mutually exclusive SOX2 amplifications (26%) and OPA1 losses (19%). Both forced SOX2 expression and OPA1 inactivation increased LN319 glioma cell invasion in vitro and promoted cell dispersion in vivo in xenotransplanted D...
November 7, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30368965/axonal-swellings-and-spheroids-a-new-insight-into-the-pathology-of-neurocysticercosis
#3
Alan J Mejia Maza, Rogger P Carmen-Orozco, Emma Carter, Danitza G Dávila, Gino Castillo, Jemina D Morales, Javier Mamani, Cesar Gavídia, Joseph Alroy, Charles R Sterling, Armando E Gonzalez, Héctor H García, Randy L Woltjer, Manuela R Verástegui, Robert H Gilman
Neurocysticercosis is a parasitic brain disease caused by the larval form (Cysticercus cellulosae) of the Taenia solium and is the leading cause of preventable epilepsy world-wide. However, the pathophysiology and relation to the wide range of clinical features remains poorly understood. Axonal swelling is emerging as an important early pathological finding in multiple neurodegenerative diseases and causes of brain injury, but has not been well-described in neurocysticercosis. Histological analysis was performed on human, rat and porcine NCC brain specimens to identify axonal pathology...
October 28, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30325080/subicular-hypotrophy-in-fetuses-with-down-syndrome-and-in-the-ts65dn-model-of-down-syndrome
#4
Fiorenza Stagni, Andrea Giacomini, Marco Emili, Beatrice Uguagliati, Maria Paola Bonasoni, Renata Bartesaghi, Sandra Guidi
Intellectual disability in Down syndrome (DS) has been attributed to neurogenesis impairment during fetal brain development. Consistently with explicit memory alterations observed in children with DS, fetuses with DS exhibit neurogenesis impairment in the hippocampus, a key region involved in memory formation and consolidation. Recent evidence suggests that the subiculum plays a unique role in memory retrieval, a process that is also altered in DS. While much attention has been devoted to the hippocampus, there is a striking lack of information regarding the subiculum of individuals with DS and DS models...
October 16, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30325077/diagnostics-of-paediatric-supratentorial-rela-ependymomas-integration-of-information-from-histopathology-genetics-dna-methylation-and-imaging
#5
Mélanie Pagès, Kristian W Pajtler, Stéphanie Puget, David Castel, Nathalie Boddaert, Arnault Tauziède-Espariat, Stéphanie Picot, Debily Marie-Anne, Marcel Kool, David Capper, Christian Sainte-Rose, Fabrice Chrétien, Stefan M Pfister, Torsten Pietsch, Jacques Grill, Pascale Varlet, Felipe Andreiuolo
Ependymoma with RELA fusion has been defined as a novel entity of the revised WHO 2016 classification of tumours of the central nervous system (CNS), characterised by fusion transcripts of the RELA gene and consequent pathological activation of the NFkB pathway. These tumours represent the majority of supratentorial ependymomas in children. The validation of diagnostic tools to identify this clinically relevant ependymoma entity is essential. Here, we have used interphase fluorescent in situ hybridisation (FISH) for C11orf95 and RELA, immunohistochemistry (IHC) for p65-RelA and the recently developed DNA-methylation based classification besides conventional histopathology and compared the precision of the methods in 40 supratentorial paediatric brain tumours diagnosed as ependymomas in the past years...
October 16, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30303592/oxidative-stress-and-inflammation-in-a-spectrum-of-epileptogenic-cortical-malformations-molecular-insights-into-their-interdependence
#6
Andrea Arena, Till S Zimmer, Jackelien van Scheppingen, Anatoly Korotkov, Jasper J Anink, Angelika Mühlebner, Floor E Jansen, Wim van Hecke, Wim G Spliet, Peter C van Rijen, Annamaria Vezzani, Johannes C Baayen, Sander Idema, Anand M Iyer, Marzia Perluigi, James D Mills, Erwin A van Vliet, Eleonora Aronica
Oxidative stress (OS) occurs in brains of patients with epilepsy and coincides with brain inflammation, and both phenomena contribute to seizure generation in animal models. We investigated whether expression of OS and brain inflammation markers co-occurred also in resected brain tissue of patients with epileptogenic cortical malformations: hemimegalencephaly (HME), focal cortical dysplasia (FCD) and cortical tubers in tuberous sclerosis complex (TSC). Moreover, we studied molecular mechanisms linking OS and inflammation in an in vitro model of neuronal function...
October 10, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30295351/clusterin-contributes-to-early-stage-of-alzheimer-s-disease-pathogenesis
#7
Shin-Bi Oh, Min Sun Kim, SuJi Park, HyunJu Son, Seog-Young Kim, Min-Seon Kim, Dong-Gyu Jo, Eunyoung Tak, Joo-Yong Lee
While clusterin is reportedly involved in Alzheimer's disease (AD) pathogenesis, how clusterin interacts with amyloid-β (Aß) to cause Aß neurotoxicity remains unclear in vivo. Using 5×FAD transgenic mice, which develop robust AD pathology and memory deficits when very young, we detected interactions between clusterin and Aß in the mouse brains. The two proteins were concurrently upregulated and bound or colocalized with each other in the same complexes or in amyloid plaques. Neuropathology and cognitive performance were assessed in the progeny of clusterin-null mice crossed with 5×FAD mice, yielding clu-/- ;5×FAD and clu+/+ ;5×FAD...
October 8, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30246434/childhood-supratentorial-ependymomas-with-yap1-mamld1-fusion-an-entity-with-characteristic-clinical-radiological-cytogenetic-and-histopathological-features
#8
Felipe Andreiuolo, Pascale Varlet, Arnault Tauziède-Espariat, Stephanie T Jünger, Evelyn Dörner, Verena Dreschmann, Klaus Kuchelmeister, Andreas Waha, Christine Haberler, Irene Slavc, Selim Corbacioglu, Markus J Riemenschneider, Alfred Leipold, Thomas Rüdiger, Dieter Körholz, Till Acker, Alexandra Russo, Jörg Faber, Clemens Sommer, Sven Armbrust, Martina Rose, Bernhard Erdlenbruch, Volkmar H Hans, Benedikt Bernbeck, Dominik Schneider, Johann Lorenzen, Martin Ebinger, Rupert Handgretinger, Manuela Neumann, Miriam van Buiren, Marco Prinz, Jelena Roganovic, Antonia Jakovcevic, Sung-Hye Park, Jacques Grill, Stéphanie Puget, Martina Messing-Jünger, Harald Reinhard, Markus Bergmann, Elke Hattingen, Torsten Pietsch
Ependymoma with YAP1-MAMLD1 fusion is a rare, recently described supratentorial neoplasm of childhood, with few cases published so far. We report on 15 pediatric patients with ependymomas carrying YAP1-MAMLD1 fusions, with their characteristic histopathology, immunophenotype and molecular/cytogenetic, radiological and clinical features. The YAP1-MAMLD1 fusion was documented by RT-PCR /Sanger sequencing, and tumor genomes were studied by molecular inversion probe (MIP) analysis. Significant copy number alterations were identified by GISTIC (Genomic Identification of Significant Targets in Cancer) analysis...
September 23, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30222900/granular-cell-astrocytoma-an-aggressive-idh-wildtype-diffuse-glioma-with-molecular-genetic-features-of-primary-glioblastoma
#9
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 of 16), 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
#10
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
#11
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/30375119/myelin-water-imaging-to-detect-demyelination-and-remyelination-and-its-validation-in-pathology
#12
Cornelia Laule, G R Wayne Moore
Damage to myelin is a key feature of multiple sclerosis (MS) pathology. Magnetic resonance imaging (MRI) has revolutionized our ability to detect and monitor MS pathology in vivo. Proton density, T1 and T2 can provide qualitative contrast weightings that yield superb in vivo visualization of central nervous system tissue and have proved invaluable as diagnostic and patient management tools in MS. However, standard clinical MR methods are not specific to the types of tissue damage they visualize, and they cannot detect subtle abnormalities in tissue that appears otherwise normal on conventional MRIs...
September 2018: Brain Pathology
https://www.readbyqxmd.com/read/30375118/66-year-old-woman-with-muscle-weakness-and-respiratory-failure
#13
J Bernardo-Cofiño, R Gómez-Illán, C Nicolás, A Astudillo, I Fernandez-Vega
No abstract text is available yet for this article.
September 2018: Brain Pathology
https://www.readbyqxmd.com/read/30375117/a-77-year-old-man-with-parkinsonism-and-rapidly-progressive-dementia
#14
Henrique Soares Dutra Oliveira, Pedro Sudbrack de Oliveira, Victor Calil, Philippe Joaquim Oliveira Menezes Macêdo, Nathalie Canedo, Luiz Felipe Rocha Vasconcellos
No abstract text is available yet for this article.
September 2018: Brain Pathology
https://www.readbyqxmd.com/read/30375115/a-66-year-old-woman-with-progressive-dementia
#15
Sheng Chen, Meng-Sha Yao, Fei Yuan
No abstract text is available yet for this article.
September 2018: Brain Pathology
https://www.readbyqxmd.com/read/30375114/magnetic-resonance-imaging-correlates-of-neuro-axonal-pathology-in-the-ms-spinal-cord
#16
Klaus Schmierer, Marc E Miquel
In people with multiple sclerosis (MS), the spinal cord is the structure most commonly affected by clinically detectable pathology at presentation, and a key part of the central nervous system involved in chronic disease deterioration. Indices, such as the spinal cord cross-sectional area at the level C2 have been developed as tools to predict future disability, and-by inference-axonal loss. However, this and other histo-pathological correlates of spinal cord magnetic resonance imaging (MRI) changes in MS remain incompletely understood...
September 2018: Brain Pathology
https://www.readbyqxmd.com/read/30375113/a-3-month-old-boy-with-progressive-weakness
#17
Soo Yeon Kim, Jung Min Ko, Sun Ah Choi, Anna Cho, Jin Sook Lee, Byung Chan Lim, Ki Joong Kim, Jong-Hee Chae
No abstract text is available yet for this article.
September 2018: Brain Pathology
https://www.readbyqxmd.com/read/30375112/multiple-sclerosis-pathology-and-its-reflection-by-imaging-technologies-introduction
#18
Hans Lassmann
No abstract text is available yet for this article.
September 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
#19
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...
September 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
#20
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 progression 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 gray matter neuronal damage. Positron Emission Tomography (PET) is an imaging technology based on the injection of radiotracers directed against specific molecular targets, which has recently allowed the selective quantification in-vivo of the key biological mechanisms relevant to MS pathophysiology...
September 2018: Brain Pathology
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