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Journal of Neuropathology and Experimental Neurology

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https://www.readbyqxmd.com/read/28591867/multisite-assessment-of-aging-related-tau-astrogliopathy-artag
#1
Gabor G Kovacs, Sharon X Xie, Edward B Lee, John L Robinson, Carrie Caswell, David J Irwin, Jon B Toledo, Victoria E Johnson, Douglas H Smith, Irina Alafuzoff, Johannes Attems, Janos Bencze, Kevin F Bieniek, Eileen H Bigio, Istvan Bodi, Herbert Budka, Dennis W Dickson, Brittany N Dugger, Charles Duyckaerts, Isidro Ferrer, Shelley L Forrest, Ellen Gelpi, Stephen M Gentleman, Giorgio Giaccone, Lea T Grinberg, Glenda M Halliday, Kimmo J Hatanpaa, Patrick R Hof, Monika Hofer, Tibor Hortobágyi, James W Ironside, Andrew King, Julia Kofler, Enikö Kövari, Jillian J Kril, Seth Love, Ian R Mackenzie, Qinwen Mao, Radoslav Matej, Catriona McLean, David G Munoz, Melissa E Murray, Janna Neltner, Peter T Nelson, Diane Ritchie, Roberta D Rodriguez, Zdenek Rohan, Annemieke Rozemuller, Kenji Sakai, Christian Schultz, Danielle Seilhean, Vanessa Smith, Pawel Tacik, Hitoshi Takahashi, Masaki Takao, Dietmar Rudolf Thal, Serge Weis, Stephen B Wharton, Charles L White, John M Woulfe, Masahito Yamada, John Q Trojanowski
Aging-related tau astrogliopathy (ARTAG) is a recently introduced terminology. To facilitate the consistent identification of ARTAG and to distinguish it from astroglial tau pathologies observed in the primary frontotemporal lobar degeneration tauopathies we evaluated how consistently neuropathologists recognize (1) different astroglial tau immunoreactivities, including those of ARTAG and those associated with primary tauopathies (Study 1); (2) ARTAG types (Study 2A); and (3) ARTAG severity (Study 2B). Microphotographs and scanned sections immunostained for phosphorylated tau (AT8) were made available for download and preview...
June 7, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28535304/the-interplay-of-micrornas-in-the-inflammatory-mechanisms-following-ischemic-stroke
#2
Seyed Esmaeil Khoshnam, William Winlow, Maryam Farzaneh
Immunity and inflammation are important parameters of the pathophysiology of stroke, a destructive illness that is the second most common cause of death worldwide. Following ischemic stroke, neuroinflammation plays a critical role in neurodegeneration and brain injury. MicroRNAs (miRNAs) are a class of endogenously expressed, noncoding RNA molecules that function to inhibit mRNA translation. Recent studies demonstrate that miRNAs are key regulators of inflammatory processes contributing to ischemic stroke injuries...
May 23, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28535250/evidence-for-the-deregulation-of-protein-turnover-pathways-in-atm-deficient-mouse-cerebellum-an-organotypic-study
#3
Catherine D Kim, Ryan E Reed, Meredith A Juncker, Zhide Fang, Shyamal D Desai
Interferon-stimulated gene 15 (ISG15), an antagonist of the ubiquitin pathway, is elevated in cells and brain tissues obtained from ataxia telangiectasia (A-T) patients. Previous studies reveal that an elevated ISG15 pathway inhibits ubiquitin-dependent protein degradation, leading to activation of basal autophagy as a compensatory mechanism for protein turnover in A-T cells. Also, genotoxic stress (ultraviolet [UV] radiation) deregulates autophagy and induces aberrant degradation of ubiquitylated proteins in A-T cells...
May 23, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28525615/embolic-foreign-material-in-the-central-nervous-system-of-pediatric-autopsy-patients-with-instrumented-heart-disease
#4
Matthew Torre, Mirna Lechpammer, Vera Paulson, Sanjay Prabhu, Audrey C Marshall, Amy L Juraszek, Robert F Padera, Elizabeth A Bundock, Sara O Vargas, Rebecca D Folkerth
Upon detection of foreign-body embolization to the central nervous system (CNS) following a specific invasive cardiovascular procedure in 1 autopsied child, we undertook a quality assurance analysis to determine whether other patients had had similar events. Autopsies of all infants and children with history of cardiac catheterization, heart surgery on cardiopulmonary bypass, and/or extracorporeal membrane oxygenation over a 5-year period at a single tertiary care institution were reviewed for light-microscopic evidence of foreign material...
May 19, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28398505/cortical-remyelination-is-heterogeneous-in-multiple-sclerosis
#5
Eva M M Strijbis, Evert-Jan Kooi, Paul van der Valk, Jeroen J G Geurts
Cortical lesions (CLs) are an important component of multiple sclerosis (MS) pathology; they correlate better with physical disability and cognitive impairment than white matter lesions (WMLs). Because remyelination can be extensive in CLs, we quantified remyelination in gray matter (GM) and white matter (WM), addressing oligodendrocyte (OGD) maturation state and clinical relevance of remyelination. Brain tissue samples from 21 chronic MS patients were immunohistochemically stained for myelin proteolipid protein, Olig2, which is strongly expressed in OGD precursor cells (OPCs), but weakly expressed in mature OGDs and other OGD markers...
April 7, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28505333/multiregional-age-associated-reduction-of-brain-neuronal-reserve-without-association-with-neurofibrillary-degeneration-or-%C3%AE-amyloidosis
#6
Jerzy Wegiel, Michael Flory, Izabela Kuchna, Krzysztof Nowicki, Shuang Yong Ma, Jarek Wegiel, Eulalia Badmaev, Wayne P Silverman, Mony de Leon, Barry Reisberg, Thomas Wisniewski
Increase in human life expectancy has resulted in the rapid growth of the elderly population with minimal or no intellectual deterioration. The aim of this stereological study of 10 structures and 5 subdivisions with and without neurofibrillary degeneration in the brains of 28 individuals 25-102-years-old was to establish the pattern of age-associated neurodegeneration and neuronal loss in the brains of nondemented adults and elderly. The study revealed the absence of significant neuronal loss in 7 regions and topographically selective reduction of neuronal reserve over 77 years in 8 brain structures including the entorhinal cortex (EC) (-33...
June 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28505283/oxidative-injury-and-iron-redistribution-are-pathological-hallmarks-of-marmoset-experimental-autoimmune-encephalomyelitis
#7
Jordon Dunham, Jan Bauer, Graham R Campbell, Don J Mahad, Nikki van Driel, Susanne M A van der Pol, Bert A 't Hart, Hans Lassmann, Jon D Laman, Jack van Horssen, Yolanda S Kap
Oxidative damage and iron redistribution are associated with the pathogenesis and progression of multiple sclerosis (MS), but these aspects are not entirely replicated in rodent experimental autoimmune encephalomyelitis (EAE) models. Here, we report that oxidative burst and injury as well as redistribution of iron are hallmarks of the MS-like pathology in the EAE model in the common marmoset. Active lesions in the marmoset EAE brain display increased expression of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase (p22phox, p47phox, and gp91phox) and inducible nitric oxide synthase immunoreactivity within lesions with active inflammation and demyelination, coinciding with enhanced expression of mitochondrial heat-shock protein 70 and superoxide dismutase 1 and 2...
June 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28499012/resistance-to-alzheimer-disease-neuropathologic-changes-and-apparent-cognitive-resilience-in-the-nun-and-honolulu-asia-aging-studies
#8
Caitlin S Latimer, C Dirk Keene, Margaret E Flanagan, Laura S Hemmy, Kelvin O Lim, Lon R White, Kathleen S Montine, Thomas J Montine
Two population-based studies key to advancing knowledge of brain aging are the Honolulu-Asia Aging Study (HAAS) and the Nun Study. Harmonization of their neuropathologic data allows cross comparison, with findings common to both studies likely generalizable, while distinct observations may point to aging brain changes that are dependent on sex, ethnicity, environment, or lifestyle factors. Here, we expanded the neuropathologic evaluation of these 2 studies using revised NIA-Alzheimer's Association guidelines and compared directly the neuropathologic features of resistance and apparent cognitive resilience...
June 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28498973/chordoma-occurs-in-young-children-with-tuberous-sclerosis
#9
Nathan A Dahl, Timothy Luebbert, Michele Loi, Ilana Neuberger, Michael H Handler, Bette Kay Kleinschmidt-DeMasters, Jean M Mulcahy Levy
Chordomas are rare bony neoplasms usually unassociated with a familial tumor predisposition syndrome. The peak incidence of this midline axial skeletal tumor is in adulthood but when very young children are affected, consideration should be given to occurrence within the tuberous sclerosis (TS) complex, especially when presenting in neonates <3 months of age. To call attention to this association, we present a brachyury-immunopositive chordoma occurring in the skull base of a 2-month-old male infant who was later realized to have metastases to the subcutaneous tissues and lungs, as well as rhabdomyoma of the heart and renal cysts/angiomyolipomas, that is, characteristic features of the TS complex...
June 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28498956/the-lateral-temporal-lobe-in-early-human-life
#10
Isabel S Goldstein, Drexel J Erickson, Lynn A Sleeper, Robin L Haynes, Hannah C Kinney
Abnormalities of lateral temporal lobe development are associated with a spectrum of genetic and environmental pathologic processes, but more normative data are needed for a better understanding of gyrification in this brain region. Here, we begin to establish guidelines for the analysis of the lateral temporal lobe in humans in early life. We present quantitative methods for measuring gyrification at autopsy using photographs of the gross brain and simple computer-based quantitative tools in a cohort of 28 brains ranging in age from 27 to 70 postconceptional weeks (end of infancy)...
June 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28521040/cortical-remyelination-is-heterogeneous-in-multiple-sclerosis
#11
Eva M M Strijbis, Evert-Jan Kooi, Paul van der Valk, Jeroen J G Geurts
Cortical lesions (CLs) are an important component of multiple sclerosis (MS) pathology; they correlate better with physical disability and cognitive impairment than white matter lesions (WMLs). Because remyelination can be extensive in CLs, we quantified remyelination in gray matter (GM) and white matter (WM), addressing oligodendrocyte (OGD) maturation state and clinical relevance of remyelination. Brain tissue samples from 21 chronic MS patients were immunohistochemically stained for myelin proteolipid protein, Olig2, which is strongly expressed in OGD precursor cells (OPCs), but weakly expressed in mature OGDs and other OGD markers...
May 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28521039/rhabdoid-meningioma-grading-and-prognostic-significance-of-this-uncommon-variant
#12
Valeria Barresi, Maria Caffo
No abstract text is available yet for this article.
May 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28521038/ventricular-zone-disruption-in-human-neonates-with-intraventricular-hemorrhage
#13
James P McAllister, Maria Montserrat Guerra, Leandro Castaneyra Ruiz, Antonio J Jimenez, Dolores Dominguez-Pinos, Deborah Sival, Wilfred den Dunnen, Diego M Morales, Robert E Schmidt, Esteban M Rodriguez, David D Limbrick
To determine if ventricular zone (VZ) and subventricular zone (SVZ) alterations are associated with intraventricular hemorrhage (IVH) and posthemorrhagic hydrocephalus, we compared postmortem frontal and subcortical brain samples from 12 infants with IVH and 3 nonneurological disease controls without hemorrhages or ventriculomegaly. Birth and expiration estimated gestational ages were 23.0-39.1 and 23.7-44.1 weeks, respectively; survival ranges were 0-42 days (median, 2.0 days). Routine histology and immunohistochemistry for neural stem cells (NSCs), neural progenitors (NPs), multiciliated ependymal cells (ECs), astrocytes (AS), and cell adhesion molecules were performed...
May 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28521037/clinical-significance-of-tdp-43-neuropathology-in-amyotrophic-lateral-sclerosis
#14
Matthew D Cykowski, Suzanne Z Powell, Leif E Peterson, Joan W Appel, Andreana L Rivera, Hidehiro Takei, Ellen Chang, Stanley H Appel
To determine the significance of TAR DNA binding protein 43 kDa (TDP-43) pathology in amyotrophic lateral sclerosis (ALS), we examined the whole brains and spinal cords of 57 patients (35 men; 22 women; mean age 63.3 years; 15 patients with c9orf72-associated ALS [c9ALS]). TDP-43 pathologic burden was determined relative to symptom onset site, disease duration, progression rate, cognitive status, and c9ALS status. There was a trend for greater TDP-43 pathologic burden in cognitively impaired patients (p = 0...
May 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28419288/editor-s-note-transitions-at-the-jnen
#15
Raymond A Sobel
No abstract text is available yet for this article.
May 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28419269/dual-genotype-diffuse-low-grade-glioma-is-it-really-time-to-abandon-oligoastrocytoma-as-a-distinct-entity
#16
Valeria Barresi, Simona Lionti, Laura Valori, Giovanna Gallina, Maria Caffo, Sabrina Rossi
We report a unique case of dual-genotype oligoastrocytoma characterized by IDH2 gene mutation. The tumor was resected from the temporal lobe of a 25-year-old man. At histological examination with hematoxylin and eosin stain, it showed distinct oligodendroglial and astrocytic areas. The former retained alpha-thalassaemia/mental retardation X-linked (ATRX) immuno-expression and had absent staining for p53, while the latter had ATRX loss and p53 over-expression. Molecular analyses were separately assessed in the 2 tumor components...
May 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28402459/spine-topographical-distribution-of-skin-%C3%AE-synuclein-deposits-in-idiopathic-parkinson-disease
#17
Vincenzo Donadio, Alex Incensi, Giovanni Rizzo, Cesa Scaglione, Sabina Capellari, Enrico Fileccia, Patrizia Avoni, Rocco Liguori
Phosphorylated α-synuclein (p-syn) in skin nerves mainly in the proximal sites is a promising neurodegenerative biomarker for idiopathic Parkinson disease (IPD). However, the p-syn spine distribution particularly in patients with unilateral motor dysfunctions remains undefined. This study aimed to investigate in IPD p-syn differences between left and right cervical spine sites in patients with prevalent unilateral motor symptoms, and cervical and thoracic spine sites in patients with bilateral motor symptoms...
May 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28379416/absence-of-alzheimer-disease-neuropathologic-changes-in-eyes-of-subjects-with-alzheimer-disease
#18
Erik A Williams, Declan McGuone, Matthew P Frosch, Bradley T Hyman, Nora Laver, Anat Stemmer-Rachamimov
Alzheimer disease (AD) is the most common cause of dementia in the elderly, and is characterized by extracellular deposition of β-amyloid and intracellular accumulation of hyperphosphorylated tau protein in the brain. These pathologic findings are identified postmortem. Various visual deficits in AD have been reported and there have been conflicting reports, through imaging and pathology studies, regarding the presence of changes in the globe that mirror Alzheimer changes in the brain. Moreover, both macular degeneration and glaucoma have been variously characterized as having AD-related features...
May 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28371804/muscle-pathology-as-a-diagnostic-clue-to-allgrove-syndrome
#19
Jens Reimann, Nicolai Kohlschmidt, Karen Tolksdorf, Joachim Weis, Klaus Kuchelmeister, Andreas Roos
Allgrove or triple A syndrome is a rare autosomal recessive disorder that can present with a variable range of multi-system manifestations, including optic atrophy, cerebellar ataxia, upper and lower motoneuron signs and various neuropathic abnormalities. These cases are a diagnostic challenge, particularly when the eponymous combination of achalasia, Addisonianism and alacrima is incomplete. Therefore, it is in the differential diagnosis for multisystem conditions and should be known to pathologists who diagnose disorders of skeletal muscle...
May 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28340257/cd8-positive-t-cell-leukoencephalitis-with-astrocytopathy-clinically-presenting-as-neuromyelitis-optica
#20
Diana L Thomas, Jody Manners, Daniel Marker, Joseph Mettenburg, Geoffrey Murdoch, Bryan Stevens, Guoji Wang, Clayton Wiley
We describe a novel disease entity with the clinical and radiologic presentation of neuromyelitis optica (NMO) and widespread CD8-positive T-cell leukoencephalitis and astrocytopathy. The 59-year-old female patient had a complex 2-year neurological history that included early changes in cognition and memory, progressive lower extremity motor dysfunction, and multimodal sensory involvement. MRI of the spinal cord showed increased T2 signal in the central cord extending from C2 through T4. MRI of the brain showed symmetric radial enhancement in periventricular deep white matter without evidence of demyelinating lesions...
May 1, 2017: Journal of Neuropathology and Experimental Neurology
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