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Neuropathology and Applied Neurobiology

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https://www.readbyqxmd.com/read/28644906/cholinesterases-in-normal-and-alzheimer-s-disease-primary-olfactory-gyrus
#1
Hayam Hamodat, Meghan K Cash, John D Fisk, Sultan Darvesh
AIMS: Alzheimer's disease (AD) is characterized by cholinergic dysfunction and deposition of β-amyloid (Aβ) plaques and tau neurofibrillary tangles (NFTs) in the brain. Olfactory abnormalities often precede cognitive symptoms in AD, indicating early involvement of pathology in olfactory structures. The cholinergic system is important not only in cognition but also in modulation of the olfactory system. The primary olfactory gyrus (POG) is comprised of the olfactory tract, anterior olfactory nucleus (AON) and olfactory area (OA)...
June 23, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28637099/aggregation-and-phosphorylation-of-%C3%AE-synuclein-with-proteinase-k-resistance-in-focal-%C3%AE-synucleinopathy-predominantly-localized-to-the-cardiac-sympathetic-nervous-system
#2
Nei Fukasawa, Takahiro Fukuda, Masato Nagaoka, Tohru Harada, Hiroyuki Takahashi, Masahiro Ikegami
Aggregates of α-synuclein, a major component of Lewy bodies (LBs) and Lewy neurites (LNs), are distributed throughout the nervous system, including the central nervous system (CNS), sympathetic ganglia, enteric nervous system (ENS), cardiac and pelvic plexuses, submandibular gland, adrenal medulla and skin, in incidental Lewy body disease (ILBD), Parkinson's disease (PD), dementia with Lewy bodies (DLB), and pure autonomic failure (PAF) [1-3]. Here we report focal α-synucleinopathy predominantly localized to the cardiac sympathetic nervous system (SNS)...
June 21, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28627088/astrogliosis-and-impaired-aquaporin-4-and-dystrophin-systems-in-idiopathic-normal-pressure-hydrocephalus
#3
Per Kristian Eide, Hans-Arne Hansson
AIMS: Idiopathic normal pressure hydrocephalus (iNPH) is one subtype of dementia that may improve following drainage of cerebrospinal fluid (CSF). This prospective observational study explored whether expression of the water channel aquaporin-4 (AQP4) and the anchoring molecule dystrophin 71 (Dp71) are altered at astrocytic perivascular endfeet and in adjacent neuropil of iNPH patient. Observations were related to measurements of pulsatile and static intracranial pressure (ICP). METHODS: The study included iNPH patients undergoing overnight monitoring of the pulsatile/static ICP, in whom a biopsy was taken from the frontal cerebral cortex during placement of the ICP sensor...
June 19, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28626918/marinesco-bodies-and-substantia-nigra-neuron-density-in-parkinson-s-disease
#4
Robert D Abbott, James S Nelson, G Webster Ross, Jane H Uyehara-Lock, Caroline M Tanner, Kamal H Masaki, Lenore J Launer, Lon R White, Helen Petrovitch
AIM: Marinesco bodies (MB) are intranuclear inclusions in pigmented neurons of the substantia nigra (SN). While rare in children, frequency increases with normal aging and is high in Alzheimer's disease, dementia with Lewy bodies, and other neurodegenerative disorders. Coinciding with the age-related rise in MB frequency is initiation of cell death among SN neurons. Whether MB have a role in this process is unknown. Our aim is to examine the association of MB with SN neuron density in Parkinson's disease (PD) in the Honolulu-Asia Aging Study...
June 19, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28603852/unusual-neuropathological-features-and-increased-brain-aluminium-in-a-resident-of-camelford-uk
#5
Andrew King, Claire Troakes, Miren Aizpurua, Ambreen Mirza, Angela Hodges, Safa Al-Sarraj, Christopher Exley
The possible role of aluminium in the pathogenesis of Alzheimer's disease (AD) has been hotly debated over the past few decades. Whereas the so-called "aluminium hypothesis" was popular in the 1970s and 1980s it has gradually fallen out of favour in the past few years possibly following a number of inconclusive and contradictory human environmental/clinical studies. Nevertheless, there have from time to time been reminders in the media of environmental accidents; these have prevented the topic from disappearing completely from public memory...
June 12, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28574618/effects-of-short-to-long-term-enzyme-replacement-therapy-ert-on-skeletal-muscle-tissue-in-late-onset-pompe-disease-lopd
#6
Michela Ripolone, Raffaella Violano, Dario Ronchi, Stefania Mondello, Annachiara Nascimbeni, Irene Colombo, Gigliola Fagiolari, Andreina Bordoni, Francesco Fortunato, Valeria Lucchini, Simona Saredi, Massimiliano Filosto, Olimpia Musumeci, Paola Tonin, Tiziana Mongini, Stefano Previtali, Lucia Morandi, Corrado Angelini, Marina Mora, Marco Sandri, Monica Sciacco, Antonio Toscano, Giacomo P Comi, Maurizio Moggio
AIMS: Pompe disease is an autosomal recessive lysosomal storage disorder resulting from deficiency of acid α-glucosidase (GAA) enzyme. Histopathological hallmarks in skeletal muscle tissue are fiber vacuolization and autophagy. Since 2006, ERT is the only approved treatment with human recombinant GAA alglucosidase alfa. We designed a study to examine ERT-related skeletal muscle changes in 18 modestly to moderately affected LOPD patients along with the relationship between morphological/biochemical changes and clinical outcomes...
June 2, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28574607/prognostic-value-of-mgmt-protein-expression-in-glioblastoma-excluding-non-tumour-cells-from-the-analysis
#7
Rikke H Dahlrot, Joseph Dowsett, Sigurd Fosmark, Annika Malmström, Roger Henriksson, Henning Boldt, Karin de Stricker, Mia D Sørensen, Hans S Poulsen, Malgorzata Lysiak, Peter Söderkvist, Johan Rosell, Steinbjørn Hansen, Bjarne W Kristensen
AIMS: It is important to predict response to treatment with temozolomide (TMZ) in glioblastoma (GBM) patients. Both MGMT protein expression and MGMT promoter methylation have been reported to predict the response to TMZ. We investigated the prognostic value of quantified MGMT protein levels in tumour cells and the prognostic importance of combining information of MGMT protein level and MGMT promoter methylations status. METHODS: MGMT protein expression was quantified in tumour cells in 171 GBMs from the population-based Region of Southern Denmark (RSD)-cohort using a double immunofluorescence approach...
June 2, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28543916/hcv-rna-genomic-sequences-and-hcv-e2-glycoprotein-in-sural-nerve-biopsies-from-hcv-infected-patients-with-peripheral-neuropathy
#8
Sabino Russi, Domenico Sansonno, Salvatore Monaco, Sara Mariotto, Sergio Ferrari, Fabio Pavone, Gianfranco Lauletta, Franco Dammacco
AIMS: Peripheral neuropathy (PN), the major neurological complication of chronic HCV infection, is frequently associated with mixed cryoglobulinemia (MC) and small-vessel systemic vasculitis. While humoral and cell-mediated immune mechanisms are suspected to act together in an aberrant immune response that results in peripheral nerve damage, the role of HCV remains largely speculative. The possible demonstration of HCV in peripheral nerve tissue would obviously assume important pathogenic implications...
May 24, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28543098/brain-endothelial-cell-expression-of-sparcl-1-is-specific-to-chronic-multiple-sclerosis-lesions-and-is-regulated-by-inflammatory-mediators-in-vitro
#9
C Bridel, M J A Koel-Simmelink, L Peferoen, C Derada Troletti, S Durieux, R Gorter, E Nutma, P Gami, E Iacobeus, L Brundin, J Kuhle, H Vrenken, J Killestein, S R Piersma, T V Pham, H E De Vries, S Amor, C R Jimenez, C E Teunissen
AIMS: Cell matrix modulating protein SPARCL-1 is highly expressed by astrocytes during CNS development and following acute CNS damage. Applying NanoLC-MS/MS to CSF of RRMS and SPMS patients, we identified SPARCL-1 as differentially expressed between these two stages of MS, suggesting a potential as CSF biomarker to differentiate RRMS from SPMS and a role in MS pathogenesis. METHODS: This study examines the potential of SPARCL-1 as CSF biomarker discriminating RRMS from SPMS in 3 independent cohorts (n=249), analyses its expression pattern in MS lesions (n= 26), and studies its regulation in cultured human brain microvasculature endothelial cells (BEC) after exposure to MS-relevant inflammatory mediators...
May 24, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28489313/in-utero-seizures-revealing-dentato-olivary-dysplasia-caused-by-scn2a-mutation
#10
Fanny Sauvestre, Sébastien Moutton, Catherine Badens, Bernard Broussin, Dominique Carles, Nada Houcinat, Caroline Lacoste, Florent Marguet, Christophe Pecheux, Laurent Villard, Fanny Pelluard, Annie Laquerrière, Gwenaëlle André
Most early-onset epileptic encephalopathies (EOEE) are caused by genetic defects. In the past, mutations, especially in genes encoding sodium channels, have been identified using linkage studies, array-CGH and more recently next-generation sequencing (NGS) [1]. Mutations in SCN2A gene have been identified in a wide variety of early-onset epileptic syndromes including benign familial neonatal infantile seizures (BFNIS) [2] and more severe forms leading to encephalopathy such as Ohtahara or West syndromes [3], epilepsy of infancy with migrating focal seizures (EIMFS) [4] and autism spectrum disorders [5]...
May 10, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28489263/diagnostic-anoctamin-5-protein-defect-in-patients-with-ano5-mutated-muscular-dystrophy
#11
A Vihola, H Luque, M Savarese, S Penttilä, M Lindfors, F Leturcq, B Eymard, G Tasca, B Brais, T Conte, K Charton, I Richard, B Udd
AIMS: Previously, detection of ANO5 protein has been complicated by unspecific antibodies, most of which have not identified the correct protein. The aims of the study were to specify ANO5 protein expression in human skeletal muscle, and to investigate if the ANO5 protein levels are affected by different ANO5 mutations in anoctaminopathy patients. METHODS: Four different antibodies were tested for ANO5 specificity. A sample preparation method compatible with membrane proteins, combined with tissue fractionation was used to determine ANO5 expression in cell cultures expressing ANO5, in normal muscles and eight patient biopsies with six different ANO5 mutations in homozygous or compound heterozygous states, and in other dystrophies...
May 10, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28455903/the-physiological-phosphorylation-of-tau-is-critically-changed-in-fetal-brains-of-individuals-with-down-syndrome
#12
I Milenkovic, J Jarc, E Dassler, E Aronica, A Iyer, H Adle-Biassette, A Scharrer, T Reischer, J A Hainfellner, G G Kovacs
AIMS: Down syndrome (DS) is a common cause of mental retardation accompanied by cognitive impairment. Comprehensive studies suggested a link between development and ageing, as nearly all individuals with DS develop Alzheimer disease (AD)-like pathology. However, there is still a paucity of data on tau in early DS to support this notion. METHODS: Using morphometric immunohistochemistry we compared tau phosphorylation in normal brains and in brains of individuals with DS from early development until early postnatal life...
April 28, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28453876/age-associated-changes-in-the-blood-brain-barrier-comparative-studies-in-human-and-mouse
#13
Emily F Goodall, Chunfang Wang, Julie E Simpson, David J Baker, David R Drew, Paul R Heath, M Jill Saffrey, Ignacio A Romero, Stephen B Wharton
AIMS: While vascular pathology is a common feature of a range of neurodegenerative diseases, we hypothesised that vascular changes occur in association with normal ageing. Therefore we aimed to characterise age-associated changes in the blood brain barrier (BBB) in human and mouse cohorts. METHODS: Immunohistochemistry and Evans Blue assays were used to characterise BBB dysfunction (tight junction protein expression and serum plasma protein accumulation), vascular pathology (pericyte loss and vascular density) and glial pathology (astrocyte and microglial density) in ageing neurological control human pre-frontal cortex (a total of 23 cases from 5 age groups representing the spectrum of young adult to old age: 20-30yrs, 31-45yrs, 46-60yrs, 61-75yrs and 75+) and C57BL/6 mice (3 month, 12 month, 18 month and 24 month, n=5/6 per group)...
April 28, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28419506/neuronal-loss-demyelination-and-volume-change-in-the-multiple-sclerosis-neocortex
#14
D Carassiti, D R Altmann, N Petrova, B Pakkenberg, F Scaravilli, K Schmierer
AIMS: Indices of brain volume (grey matter, white matter, lesions) are being used as outcomes in clinical trials of patients with multiple sclerosis (MS). We investigated the relationship between cortical volume, the number of neocortical neurons estimated using stereology, and demyelination. METHODS: Nine MS and seven control hemispheres were dissected into coronal slices. On sections stained for Giemsa, the cortex was outlined and optical disectors applied using systematic uniform random sampling...
April 18, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28403515/an-aggressive-multifocal-primary-cns-histiocytosis-with-ptpn11-shp2-mutation
#15
Qi Zhang, Areej Shibani, Bekim Sadikovic, Christopher J Howlett, Lee-Cyn Ang
Primary histiocytic tumors of the CNS are rare. The current WHO classification (2016) included 5 entities: Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), Rosai-Dorfman disease (RDD), juvenile xanthogranuloma (JXG), and histiocytic sarcoma (HS) (1). The diagnosis usually is made based on the tumor differentiation as to the counterpart in normal histiocyte development. This article is protected by copyright. All rights reserved.
April 12, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28398599/hippocampal-sclerosis-and-mesial-temporal-lobe-epilepsy-in-chorea-acanthocytosis-a-case-with-clinical-pathologic-and-genetic-evaluation
#16
Karin Mente, Sun A Kim, Christopher Grunseich, Marco M Hefti, John F Crary, Adrian Danek, Barbara I Karp, Ruth H Walker
Chorea-acanthocytosis (ChAc) is an autosomal recessive neurodegenerative disease associated with mutations in VPS13A that encodes the protein chorein. ChAc is characterized by progressive chorea, dystonia, and psychiatric symptoms, developing in young adulthood, often with acanthocytosis in peripheral blood. Tongue protrusion, or feeding dystonia, is common, as are seizures and neuropathy [1]. On neuropathology, there is basal ganglia atrophy, neuronal loss, and gliosis, especially in the caudate nucleus [2]...
April 11, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28386933/novel-conformation-selective-alpha-synuclein-antibodies-raised-against-different-in-vitro-fibril-forms-show-distinct-patterns-of-lewy-pathology-in-parkinson-s-disease
#17
D J Covell, J L Robinson, R S Akhtar, M Grossman, D Weintraub, H M Bucklin, R M Pitkin, D Riddle, A Yousef, J Q Trojanowski, V M-Y Lee
AIMS: The aim of this study was to test the hypothesis that different conformations of misfolded α-synuclein (α-syn) are present in Parkinson's disease (PD) brain. METHODS: Using two previously characterized conformations of α-syn fibrils, we generated new conformation-selective α-syn monoclonal antibodies (mAbs). We then interrogated multiple brain regions in a well-characterized autopsy cohort of PD patients (n = 49) with these mAbs, Syn7015 and Syn9029. RESULTS: Syn7015 detects Lewy bodies (LBs) and Lewy neurites (LNs) formed by pathological α-syn in all brain regions tested, and is particularly sensitive to LNs and small Lewy dots, inclusions believed to form early in the disease...
April 7, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28370398/tau-aggregates-where-when-why-and-what-consequences
#18
EDITORIAL
Matthew P Frosch
Neuropathologists have been aware of neurofibrillary tangles for more than a century following their description as part of Alois Alzheimer's initial report. Since those early days, our understanding of the relationship of this particular type of cellular inclusion associated with neurodegeneration has continually broadened. Textbooks, now a partially antiquated concept, commonly list a range of disorders as being associated with tangles -NDASH- including typical neurodegenerative diseases (Alzheimer disease [AD], forms of frontotemporal lobar degenerations [FTLD-tau], progressive supranuclear palsy [PSP], corticobasal degeneration [CBD], primary aged related tauopathy [PART]), secondary degenerative diseases such as chronic traumatic encephalopathy, metabolic disease (Niemann-Pick disease type C) and infections (SSPE)...
April 2, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28370167/tau-aggregates-where-when-why-and-what-consequences
#19
EDITORIAL
Matthew P Frosch
Neuropathologists have been aware of neurofibrillary tangles for more than a century following their description as part of Alois Alzheimer's initial report. Since those early days, our understanding of the relationship of this particular type of cellular inclusion associated with neurodegeneration has continually broadened. Textbooks, now a partially antiquated concept, commonly list a range of disorders as being associated with tangles -NDASH- including typical neurodegenerative diseases (Alzheimer disease [AD], forms of frontotemporal lobar degenerations [FTLD-tau], progressive supranuclear palsy [PSP], corticobasal degeneration [CBD], primary aged related tauopathy [PART]), secondary degenerative diseases such as chronic traumatic encephalopathy, metabolic disease (Niemann-Pick disease type C) and infections (SSPE)...
March 30, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28039900/evidence-of-early-defects-in-cajal-retzius-cell-localization-during-brain-development-in-a-mouse-model-of-dystroglycanopathy
#20
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 characterized 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...
June 2017: Neuropathology and Applied Neurobiology
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