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Neuropathology and Applied Neurobiology

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https://www.readbyqxmd.com/read/29319907/aberrant-accumulation-of-erbb4-in-progressive-supranuclear-palsy
#1
Aya Murakami, Masataka Nakamura, Satoshi Kaneko, Wen-Lang Lin, Dennis W Dickson, Hirofumi Kusaka
The human epidermal growth factor receptor family consists of 4 members that belong to the ErbB lineage of proteins (ErbB1-4). Neuregulin-1 (NRG1)/ErbB signalling regulates brain development and function. Abnormalities in this signalling have been implicated in the aetiology or development of neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis. So, we aimed at investigating whether the expression of NRG1 or ErbB proteins are altered in progressive supranuclear palsy (PSP) METHODS: The brains of 10 PSP and 6 control patients were investigated by immunohistochemical analysis RESULTS: Whereas C-terminal ErbB4 immunoreacitivity was partially but distinctly present in the cytoplasm and/or in the nucleus of neurons in control patients, it was rarely observed in the neuronal nuclei in PSP patients...
January 10, 2018: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29315734/molecular-characteristics-of-long-term-epilepsy-associated-tumours-leats-and-mechanisms-for-tumour-related-epilepsy-tre
#2
Thomas J Stone, Rachel Rowell, Bodiabaduge Ashan Prasanna Jayasekera, Mark O Cunningham, Thomas S Jacques
Brain tumours are the second most common cause of seizures identified in epilepsy surgical series. While any tumour involving the brain has the potential to cause seizures, specific subtypes are more frequently associated with epilepsy. Tumour-related epilepsy has a profound impact on patients with brain tumours and these seizures are often refractory to anti-epileptic treatments, resulting in long-term disability and patient morbidity. Despite the drastic impact epilepsy-associated tumours have on patients, they have not traditionally enjoyed as much attention as more malignant neoplasms...
January 6, 2018: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29288503/neurodegenerative-processes-in-temporal-lobe-epilepsy-with-hippocampal-sclerosis-clinical-pathological-and-neuroimaging-evidence
#3
X Y Tai, B Bernhardt, M Thom, P Thompson, S Baxendale, M Koepp, N Bernasconi
Cognitive decline is increasingly described as a co-morbidity of temporal lobe epilepsy (TLE). Mechanisms underlying cognitive impairment are not fully understood despite examining clinical factors, such as seizure frequency, and cellular mechanisms of excitotoxicity. We review the neuropsychometry evidence for progressive cognitive decline and examine the pathology and neuroimaging evidence supporting a neurodegenerative process in hippocampal sclerosis (HS)-related TLE. Accelerated cognitive decline is described in groups of adult HS-related TLE patients...
December 30, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29231968/value-of-magnetic-resonance-imaging-for-evaluating-muscle-inflammation-insights-from-a-new-mouse-model-of-myositis
#4
Gwladys Bourdenet, Benjamin Dubourg, Lionel Nicol, Paul Mulder, Jérémie Martinet, Yves Allenbach, Christian Boitard, Olivier Boyer
Inflammatory myopathies or myositides represent a group of severe skeletal muscle diseases characterized by muscle weakness, elevation of serum creatine kinase levels and muscle inflammatory cell infiltrates. Despite the contribution of a growing number of myositis-specific autoantibodies and the existence of characteristic dermatological features in dermatomyositis, the definitive diagnosis of myositis requires pathological examination of a muscle biopsy [1, 2]. To limit false negatives, this biopsy should be performed in an area of active disease [2]...
December 12, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29215752/familial-tauopathy-with-p364s-mapt-mutation-clinical-course-neuropathology-and-ultrastructure-of-neuronal-tau-inclusions
#5
Peter Štrafela, Jerica Pleško, Jožef Magdič, Blaž Koritnik, Andrej Zupan, Damjan Glavač, Mara Bresjanac, Mara Popović
AIMS: This report presents the clinical course, neuropathology and ultrastructure of neuronal tau inclusions of four Slovene relatives with P364S MAPT mutation. METHODS: The clinical history of three out of four P364S MAPT mutation carriers was taken. After formalin fixation, thorough sampling of the central nervous system was followed by paraffin embedding, H&E, Gallyas, Bielschowsky, and immunostaining with AT8, anti-3R, anti-4R tau, anti-Amyloid-β, anti-TDP43, and anti-alpha-synuclein antibodies...
December 7, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29215728/multiple-neuronal-pathologies-are-common-in-young-patients-with-pathologically-proven-frontotemporal-lobar-degeneration
#6
Rachel H Tan, Yue Yang, Glenda M Halliday
AIMS: The past decade has seen a surge in studies identifying mixed pathologies in elderly populations. Importantly however, few studies have focussed on mixed pathology in Frontotemporal Lobar Degeneration (FTLD), particularly in younger cases. METHODS: The present study study examined concomitant pathological neuronal inclusions of TDP-43, hyperphosphorylated tau and α-synuclein protein in the anterior cingulate, hippocampus and entorhinal cortex in young (≤65 years at death) versus elderly (≥80 years at death) cases with pathologically-confirmed FTLD (n=52) or Alzheimer's disease (AD) (n=47)...
December 7, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29210089/aicardi-gouti%C3%A3-res-syndrome-with-muscle-involvement-in-early-infancy
#7
Nikolaus Deigendesch, Susanne Moralez-Gonzalez, Bernhard Weschke, Hans-Hilmar Goebel, Markus Schuelke, Werner Stenzel
Aicardi-Goutières-syndrome (AGS) comprises a group of monogenetic disorders characterized by aberrant immune activation (1). The spectrum of neurological features includes acquired microcephaly with cerebral atrophy, basal ganglia calcification, leukodystrophy, chronic cerebrospinal lymphocytosis, and/or elevated concentration of type I interferons (IFNs) resulting in high morbidity and mortality (2). AGS can be caused by mutations in several genes involved in sensing or modifying nucleic acids. This elevates the α-interferon response and activates the innate and adaptive immune system...
December 5, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29181857/whole-exome-sequencing-of-the-bdr-cohort-evidence-to-support-the-role-of-the-pilra-gene-in-alzheimer-s-disease
#8
Tulsi Patel, Keeley J Brookes, James Turton, Sultan Chaudhury, Tamar Guetta-Baranes, Rita Guerreiro, Jose Bras, Dena Hernandez, Andrew Singleton, Paul T Francis, John Hardy, Kevin Morgan
AIM: Late-onset Alzheimer's disease (LOAD) accounts for 95% of all Alzheimer's cases and is genetically complex in nature. Overlapping clinical and neuropathological features between AD, FTD and Parkinson's disease highlight the potential role of genetic pleiotropy across diseases. Recent GWAS have uncovered 20 new loci for AD risk, however these exhibit small effect sizes. Using NGS, here we perform association analyses using exome-wide and candidate-gene driven approaches. METHODS: Whole-exome sequencing was performed on 132 AD cases and 53 control samples...
November 27, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29178443/the-past-present-and-future-challenges-in-epilepsy-related-and-sudden-deaths-and-biobanking
#9
Maria Thom, Maura Boldrini, Elizabeth Bundock, Mary N Sheppard, Orrin Devinsky
Awareness and research on epilepsy-related deaths (ERD), in particular Sudden Unexpected Death in Epilepsy (SUDEP), have exponentially increased over the last two decades. Most publications have focused on guidelines that inform clinicians dealing with these deaths, educating patients, potential risk factors and mechanisms. There is a relative paucity of information available for pathologists who conduct these autopsies regarding appropriate post-mortem practice and investigations. As we move from recognizing SUDEP as the most common form of ERD toward in-depth investigations into its causes and prevention, health professionals involved with these autopsies and post-mortem procedure must remain fully informed...
November 27, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29130549/concomitant-idh-wildtype-glioblastoma-and-idh1-mutant-anaplastic-astrocytoma-in-a-patient-with-constitutional-mismatch-repair-deficiency-syndrome
#10
Francesca Galuppini, Enrico Opocher, Uri Tabori, Isabella Mammi, Melissa Edwards, Britany Campbell, Jacalyn Kelly, Alessandra Viel, Michele Quaia, Francesca Rivieri, Domenico D'Avella, Antonella Arcella, Felice Giangaspero, Matteo Fassan, Marina Paola Gardiman
Constitutional mismatch repair deficiency (CMMRD) is a rare and often under-recognized tumour predisposition syndrome, presenting with both extracranial and malignant brain tumours that occur in children and/or young adults [1]. The genetic defects underlying this disease are biallelic germline mutations in one of the DNA mismatch repair (MMR) genes leading to a constitutional DNA repair defect that causes a cancer predisposition syndrome with early onset [2]. This mechanism is different from Lynch syndrome (LS) where a heterozygous germline loss-of-function mutation is observed and the patients are more prone to develop colon and genitourinary cancers as adults [1]...
November 12, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29130506/animal-models-of-acquired-epilepsy-insights-into-mechanisms-of-human-epileptogenesis
#11
Albert J Becker
In many patients who suffer from epilepsies, recurrent epileptic seizures do not start at birth but develop later in life. This holds particularly true for epilepsies with a focal seizure origin including focal cortical dysplasias (FCDs) and temporal lobe epilepsy (TLE). TLE most frequently has its seizure onset in the hippocampal formation. Hippocampal biopsies of pharmacoresistant TLE patients undergoing epilepsy surgery for seizure control most frequently reveal the damage pattern of hippocampal sclerosis, i...
November 12, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29086434/myoblots-dystrophin-quantification-by-in-cell-western-assay-for-a-streamlined-development-of-dmd-treatments
#12
Estíbaliz Ruiz-Del-Yerro, Iker Garcia-Jimenez, Kamel Mamchaoui, Virginia Arechavala-Gomeza
AIMS: New therapies for neuromuscular disorders are often mutation-specific and require to be studied in patient's cell cultures. In Duchenne muscular dystrophy (DMD) dystrophin restoration drugs are being developed but, as muscle cell cultures from DMD patients are scarce and do not grow or differentiate well, only a limited number of candidate drugs are tested. Moreover, dystrophin quantification by western blotting requires a large number of cultured cells; so fewer compounds are as thoroughly screened as is desirable...
October 31, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28626918/marinesco-bodies-and-substantia-nigra-neuron-density-in-parkinson-s-disease
#13
R D Abbott, J S Nelson, G W Ross, J H Uyehara-Lock, C M Tanner, K H Masaki, L J Launer, L R White, H Petrovitch
AIM: Marinesco bodies (MB) are intranuclear inclusions in pigmented neurons of the substantia nigra (SN). While rare in children, frequency increases with normal ageing 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...
December 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
#14
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...
December 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29053887/low-foxg1-and-high-olig-2-labelling-indices-define-a-prognostically-favorable-subset-in-idh-mutant-gliomas
#15
Sarah Schäfer, Felix Behling, Marco Skardelly, Marilin Koch, Ines Ott, Frank Paulsen, Ghazaleh Tabatabai, Jens Schittenhelm
AIMS: Previous data suggest that expression of transcription factors FoxG1 and Olig-2 can separate hotspot H3F3A-mutant tumors in pediatric glioma. We evaluated their prognostic potential and feasibility for identifying H3F3A-mutant tumors among IDH-mutant/wildtype gliomas. METHODS: Immunohistochemistry of FoxG1/Olig-2 and ATRX in 471 cases of diffuse gliomas and molecular determination of IDH, H3F3A, MGMT and 1p/19 codeletion status. RESULTS: Mean percentage of FoxG1 positive tumor cells increased from 17% in WHO grade II to over 21% in grade III to 37% in grade IV tumors, while mean Olig-2 indices decreased from 29% to 28% to 17% respectively...
October 20, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29044639/inflammatory-pathology-markers-activated-microglia-and-reactive-astrocytes-in-early-and-late-onset-alzheimer-disease-a-post-mortem-study
#16
R Taipa, V Ferreira, P Brochado, A Robinson, I Reis, F Marques, D M Mann, M Melo-Pires, N Sousa
AIMS: The association between the pathological features of AD and dementia is stronger in younger old persons than in older old persons suggesting that additional factors are involved in the clinical expression of dementia in the oldest old. Cumulative data suggests that neuroinflammation plays a prominent role in Alzheimer's disease (AD) and different studies reported an age-associated dysregulation of the neuroimmune system. Consequently, we sought to characterize the pattern of microglial cell activation and astrogliosis in brain post mortem tissue of pathologically confirmed cases of early and late onset AD (EOAD and LOAD) and determine their relation to age...
October 17, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28977690/neuroinflammatory-pathways-as-treatment-targets-and-biomarker-candidates-in%C3%A2-epilepsy-emerging-evidence-from-preclinical-and-clinical-studies
#17
Erwin A van Vliet, Eleonora Aronica, Annamaria Vezzani, Teresa Ravizza
Accumulating evidence indicates an important pathophysiological role of brain inflammation in epilepsy. In this review, we will provide an update of specific inflammatory pathways that have been proposed to be crucial in the underlying molecular mechanisms of epilepsy, including the interleukin-1 receptor/toll-like receptor signaling, cyclooxygenase-2, tumor necrosis factor-alpha, complement signaling and chemokines. Furthermore, by drawing on evidence from preclinical and clinical studies we will discuss the potential of these signaling pathways targets for novel therapeutic interventions that control drug-resistant seizures or have disease-modifying effects...
October 4, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28901022/in-this-issue-october-2017
#18
(no author information available yet)
No abstract text is available yet for this article.
October 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
#19
LETTER
K Mente, S A Kim, C Grunseich, M M Hefti, J F Crary, A Danek, B I Karp, R H Walker
No abstract text is available yet for this article.
October 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28039950/ageing-causes-prominent-neurovascular-dysfunction-associated-with-loss-of-astrocytic-contacts-and-gliosis
#20
J Duncombe, R J Lennen, M A Jansen, I Marshall, J M Wardlaw, K 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 amyloid precursor protein (APP) littermates (TgSwDI) which develop age-dependent increases in amyloid...
October 2017: Neuropathology and Applied Neurobiology
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