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Neuropathology and Applied Neurobiology

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https://www.readbyqxmd.com/read/28075491/review-integrated-classification-of-inflammatory-myopathies
#1
Y Allenbach, O Benveniste, H-H Goebel, W Stenzel
Inflammatory myopathies comprise a multitude of diverse diseases, most often occurring in complex clinical settings. In order to ensure accurate diagnosis, multidisciplinary expertise is required. Here, we propose a comprehensive myositis classification that incorporates clinical, morphological and molecular data as well as autoantibody profile. This review focuses on recent advances in myositis research, in particular, the correlation between autoantibodies and morphological or clinical phenotypes that can be used as the basis for an 'integrated' classification system...
January 11, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28054381/rhabdoid-component-emerging-as-a-subclonal-evolution-of-paediatric-glioneuronal-tumours
#2
A Bertrand, C Rondenet, J Masliah-Planchon, P Leblond, A de la Fourchardière, D Pissaloux, K Aït-Raïs, D Lequin, A Jouvet, P Freneaux, H Sevestre, D Ranchere-Vince, A Tauziede-Espariat, C-A Maurage, K Silva, G Pierron, O Delattre, P Varlet, D Frappaz, F Bourdeaut
Atypical teratoid/rhabdoid tumors (AT/RT) are high-grade tumors partially composed of rhabdoid cells (1). The 1-year overall survival rate is 41% (2). Rhabdoid cells have large eccentric nuclei, a single prominent nucleolus, and abundant cytoplasm with eosinophilic inclusions. The immunohistochemical profile of these cells frequently includes loss of nuclear BAF47 expression due to loss of the SMARCB1 locus combined with a mutation of the other allele (3). This article is protected by copyright. All rights reserved...
January 5, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28054371/review-tau-in-biofluids-relation-to-pathology-imaging-and-clinical-features
#3
Henrik Zetterberg
Tau is a microtubule-binding protein that is important for the stability of neuronal axons. It is normally expressed within neurons and is also secreted into the brain interstitial fluid that communicates freely with cerebrospinal fluid (CSF) and, in a more restricted manner, blood via the glymphatic clearance system of the brain. In Alzheimer's disease (AD), neuroaxonal degeneration results in increased release of tau from neurons. Further, tau is truncated and phosphorylated, which leads to aggregation of tau in neurofibrillary tangles of the proximal axoplasm...
January 5, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28039950/ageing-causes-prominent-neurovascular-dysfunction-associated-with-loss-of-astrocytic-contacts-and-gliosis
#4
Jessica Duncombe, Ross J Lennen, Maurits A Jansen, Ian Marshall, Joanna M Wardlaw, Karen Horsburgh
AIMS: Normal neurovascular coupling, mediated by the fine interplay and communication of cells within the neurovascular unit, is critical for maintaining normal brain activity and cognitive function. This study investigated whether, with advancing age there is disruption of neurovascular coupling and specific cellular components of the neurovascular unit, and whether the effects of increasing amyloid (a key feature of Alzheimer's disease) would exacerbate these changes. METHODS: Wild-type mice, in which amyloid deposition is absent, were compared to transgenic APP littermates (TgSwDI) which develop age-dependent increases in amyloid...
December 31, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28039900/evidence-of-early-defects-in-cajal-retzius-cell-localisation-during-brain-development-in-a-mouse-model-of-dystroglycanopathy
#5
H S Booler, V Pagalday-Vergara, J L Williams, M Hopkinson, S C Brown
AIMS: The secondary dystroglycanopathies represent a heterogeneous group of congenital muscular dystrophies characterised by the defective glycosylation of alpha dystroglycan. These disorders are associated with mutations in at least 17 genes, including Fukutin-related protein (FKRP). At the severe end of the clinical spectrum there is substantial brain involvement, and cobblestone lissencephaly is highly suggestive of these disorders. The precise pathogenesis of this phenotype has however, remained unclear with most attention focused on the disruption to the radial glial scaffold...
December 31, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28039872/commentary-on-human-adult-neurogenesis-across-the-ages-an-immunohistochemical-study
#6
LETTER
Gianluca Marucci
I have read with great interest the paper entitled "Human adult neurogenesis across the ages: An immunohistochemical study" by Dennis et al [1]. The Authors evaluated cell proliferation and neurogenesis in the subventricular zone (SVZ) and subgranular zone (SGZ) of 23 individuals aged 0.2-59 years, using immunohistochemistry and immunofluorescence. The Authors evidenced a marked decline in neurogenesis in both neurogenic niches in early childhood and that after 3 years of age, microglia were the only proliferating cells found in either niche or in the adjacent parenchyma...
December 31, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28009443/review-myopathology-in-the-times-of-modern-genetics
#7
Markus Schuelke, Nancy Christine Øien, Anders Oldfors
The advent of Next Generation Sequencing (NGS) technologies has accelerated the rate of novel disease gene discovery. Analysis of the large datasets generated by whole exome sequencing, whole genome sequencing, and other NGS approaches poses a challenge to physicians and pathologists searching for disease causing variants amongst the 50,000-3 million polymorphisms typically seen in these datasets. This review describes strategies that successfully combine classical neuropathological investigation (e.g. histology, immunostaining, and electron microscopy) with modern NGS technologies to pinpoint the underlying genetic cause of a disease...
December 23, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28009439/sudan-black-a-fast-easy-and-non-toxic-method-to-assess-myelin-repair-in-demyelinating-diseases
#8
Benjamin V Ineichen, Oliver Weinmann, Nicolas Good, Patricia Plattner, Carla Wicki, Elisabeth J Rushing, Michael Linnebank, Martin E Schwab
AIMS: The search for novel drugs that enhance myelin repair in entities such as multiple sclerosis has top priority in neurological research, not least because remyelination can hinder further neurodegeneration in neuro-inflammatory conditions. Recently, several new compounds with the potential to boost remyelination have been identified using high-throughput in vitro screening methods. However, assessing their potential to enhance remyelination in vivo using plastic embedded semi-thin sections or electron microscopy, even though being the gold standard for assessing remyelination, is toxic, extremely time-consuming, and expensive...
December 23, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27997032/k27m-midline-gliomas-display-malignant-progression-by-imaging-and-histology
#9
Franziska J Vettermann, Julia E Neumann, Bogdana Suchorska, Peter Bartenstein, Armin Giese, Mario M Dorostkar, Nathalie L Albert, Ulrich Schüller
K27M midline gliomas are diffuse gliomas that usually occur in children and young adults and arise in midline structures of the brain, the brain stem and the spinal cord. They are defined by point mutations within the chromatin modifiers H3 and are usually associated with a bad clinical outcome [1,2,3]. As such, they are represented as a new separate entity in the new version of the WHO classification for tumors of the central nervous system. Interestingly, these tumors display a huge histological variability [4] and diagnoses currently range from low grade astrocytoma (WHO-grade II) to glioblastoma (WHO-grade IV)...
December 20, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27992965/globular-glial-inclusions-unveil-enigmas-of-mapt-mutations
#10
EDITORIAL
Gabor G Kovacs
Neurodegenerative diseases are characterised by progressive dysfunction and loss of neurons leading to distinct involvement of functional systems defining clinical presentations [1]. In the last two decades proteins were placed in the centre of the pathogenesis of these disorders; thus current molecular pathological classification of the most frequent adult-onset neurodegenerative diseases is protein-based [1]. One of these proteins is tau, which is related to a group of disorders called tauopathies. This article is protected by copyright...
December 19, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27976420/myopathology-in-congenital-myopathies
#11
Caroline A Sewry, Carina Wallgren-Pettersson
Congenital myopathies are clinically and genetically a heterogeneous group of early onset neuromuscular disorders, characterised by hypotonia and muscle weakness. Clinical severity and age of onset are variable. Many patients are severely affected at birth whilst others have a milder, moderately progressive or non-progressive phenotype. Respiratory weakness is a major clinical aspect that requires regular monitoring. Causative mutations in several genes have been identified that are inherited in a dominant, recessive or X-linked manner or arise de novo...
December 15, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27886392/deregulated-expression-of-ezh2-in-congenital-brainstem-disconnection
#12
P G Barth, E Aronica, S Fox, K Fluiter, M A J Weterman, A Poretti, D C Miller, E Boltshauser, B Harding, M Santi, F Baas
Congenital brainstem disconnection (CBSD) is an enigmatic embryo-fetal defect presenting as (sub)total absence of a segment between mesencephalon and lower brainstem. Rostro-caudal limits of the defect vary while the basal pons is always involved and the cerebellum is globally hypoplastic. A recent update and review[1] lists 14 cases, including 3 brain autopsy studies[1-3]. Necrosis and glial- or inflammatory reactions were absent. Inferior olivary nuclei were small or absent, pontine nuclei depleted, and the cerebellar dentate nuclei dysplastic...
November 25, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27859539/clinicopathologic-heterogeneity-in-ftdp-17-due-to-mapt-p-p301l-mutation-including-a-patient-with-globular-glial-tauopathy
#13
Pawel Tacik, Monica Sanchez-Contreras, Michael DeTure, Melissa E Murray, Rosa Rademakers, Owen A Ross, Zbigniew K Wszolek, Joseph E Parisi, David S Knopman, Ronald C Petersen, Dennis W Dickson
AIM: The p.P301L mutation in microtubule-associated protein tau (MAPT) is a common cause of frontotemporal dementia and Parkinsonism linked to chromosome 17 (FTDP-17). We compare clinicopathologic features of five unrelated and three related (brother, sister and cousin) patients with FTDP-17 due to p.P301L mutation. METHODS: Genealogical, clinical, neuropathologic, and genetic data were reviewed from eight individuals RESULTS: The series consisted of five men and three women with an average age of death of 58 years (52-65 years) and average disease duration of 9 years (3-14 years)...
November 8, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27783888/association-of-cortical-microinfarcts-and-cerebral-small-vessel-pathology-in-the-ageing-brain
#14
Enikö Kövari, François R Herrmann, Gabriel Gold, Patrick R Hof, Andreas Charidimou
AIMS: Cortical microinfarcts (CMI) are frequently observed in the ageing brain independent of cognitive decline, but their etiology is not fully elucidated. To examine the potential role of different vessel pathologies, including cerebral amyloid angiopathy (CAA), arteriolosclerosis-hyalinosis, and thromboembolism in the development of CMI, we examined 80 autopsy cases with more than one CMI on routine neuropathological examination. METHODS: Pial and intracortical vessels around CMI were assessed for their integrity with hematoxylin-eosin staining and antibodies against amyloid-β protein and fibrinogen using a semiquantitative 4-level rating scale (absent to severe) in the hippocampus, and the frontal, temporal, and occipital cortex...
October 26, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27891697/in-this-issue
#15
(no author information available yet)
No abstract text is available yet for this article.
December 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27424496/human-adult-neurogenesis-across-the-ages-an-immunohistochemical-study
#16
C V Dennis, L S Suh, M L Rodriguez, J J Kril, G T Sutherland
AIMS: Neurogenesis in the postnatal human brain occurs in two neurogenic niches; the subventricular zone (SVZ) in the wall of the lateral ventricles and the subgranular zone (SGZ) of the hippocampus. The extent to which this physiological process continues into adulthood is an area of ongoing research. This study aimed to characterize markers of cell proliferation and assess the efficacy of antibodies used to identify neurogenesis in both neurogenic niches of the human brain. METHODS: Cell proliferation and neurogenesis were simultaneously examined in the SVZ and SGZ of 23 individuals aged 0...
December 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27732747/meningeal-swi-snf-related-matrix-associated-actin-dependent-regulator-of-chromatin-subfamily-b-member-1-smarcb1-deficient-tumours-an-emerging-group-of-meningeal-tumours
#17
B Dadone, D Fontaine, L Mondot, G Cristofari, A Jouvet, C Godfraind, P Varlet, D Ranchère-Vince, J-M Coindre, L Gastaud, C Baudoin, A-C Peyron, A Thyss, M Coutts, J-F Michiels, F Pedeutour, F Burel-Vandenbos
AIMS: Bi-allelic inactivation of SWI/SNF related, matrix-associated, actin-dependent regulator of chromatin, subfamily B member 1 (SMARCB1; also known as INI1) and loss of immunohistochemical expression of SMARCB1 define the group of SMARCB1-deficient tumours. Initially highlighted in malignant rhabdoid tumours, this inactivation has subsequently been observed in several intra and extracranial tumours. To date, primary meningeal SMARCB1-deficient tumours have not been described. We report two cases of meningeal SMARCB1-deficient tumours occurring in adults...
October 12, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27543695/systems-proteomic-analysis-reveals-that-clusterin-and-tissue-inhibitor-of-metalloproteinases-3-increase-in-leptomeningeal-arteries-affected-by-cerebral-amyloid-angiopathy
#18
A Manousopoulou, M Gatherer, C Smith, J A R Nicoll, C H Woelk, M Johnson, R Kalaria, J Attems, S D Garbis, R O Carare
AIMS: Amyloid beta (Aβ) accumulation in the walls of leptomeningeal arteries as cerebral amyloid angiopathy (CAA) is a major feature of Alzheimer's disease. In this study, we used global quantitative proteomic analysis to examine the hypothesis that the leptomeningeal arteries derived from patients with CAA have a distinct endophenotypic profile compared to those from young and elderly controls. METHODS: Freshly dissected leptomeningeal arteries from the Newcastle Brain Tissue Resource and Edinburgh Sudden Death Brain Bank from seven elderly (82...
October 5, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27683244/in-this-issue
#19
(no author information available yet)
No abstract text is available yet for this article.
October 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27291591/review-induced-pluripotent-stem-cell-models-of-frontotemporal-dementia
#20
REVIEW
E Preza, J Hardy, T Warner, S Wray
The increasing prevalence of dementia in the ageing population combined with the lack of treatments and the burden on national health care systems globally make dementia a public health priority. Despite the plethora of important research findings published over the past two decades, the mechanisms underlying dementia are still poorly understood and the progress in pharmacological interventions is limited. Recent advances in cellular reprogramming and genome engineering technologies offer an unprecedented new paradigm in disease modeling...
October 2016: Neuropathology and Applied Neurobiology
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