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Demyelinating disease

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https://www.readbyqxmd.com/read/29648443/shedding-light-on-the-molecular-pathology-of-amyloid-plaques-in-transgenic-alzheimer-s-disease-mice-using-multimodal-maldi-imaging-mass-spectrometry
#1
Ibrahim Kaya, Henrik Zetterberg, Kaj Blennow, Jörg Hanrieder
Senile plaques formed by aggregated amyloid β peptides are one of the major pathological hallmarks of Alzheimer's disease (AD) which have been suggested to be the primary influence triggering the AD pathogenesis and the rest of the disease process. However, neurotoxic Aβ aggregation and progression are associated with a wide range of enigmatic biochemical, biophysical and genetic processes. MALDI imaging mass spectrometry (IMS) is a label-free method to elucidate the spatial distribution patterns of intact molecules in biological tissue sections...
April 12, 2018: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/29629942/paediatric-multiple-sclerosis-and-other-acute-demyelinating-diseases
#2
Kevin Rostásy, Barbara Bajer-Kornek
PURPOSE OF REVIEW: Neuroimmunological diseases encompass a wide spectrum of diseases in children. Apart from the discovery of autoantibodies affecting primarily grey matter structures and the improved clinical characterization of rare entities such as N-methyl D-aspartate receptor-R- encephalitis, important strides have also been made in autoimmune-mediated white matter diseases, including paediatric multiple sclerosis (pedMS) and other acute demyelinating syndromes (ADS) often associated with antibodies (abs) against myelin-oligodendrocyte-glycoprotein (MOG)...
April 7, 2018: Current Opinion in Neurology
https://www.readbyqxmd.com/read/29629601/occipital-neuralgia-associates-with-high-cervical-spinal-cord-lesions-in-idiopathic-inflammatory-demyelinating-disease
#3
Narayan R Kissoon, James C Watson, Christopher J Boes, Orhun H Kantarci
Background The association of trigeminal neuralgia with pontine lesions has been well documented in multiple sclerosis, and we tested the hypothesis that occipital neuralgia in multiple sclerosis is associated with high cervical spinal cord lesions. Methods We retrospectively reviewed the records of 29 patients diagnosed with both occipital neuralgia and demyelinating disease by a neurologist from January 2001 to December 2014. We collected data on demographics, clinical findings, presence of C2-3 demyelinating lesions, and treatment responses...
January 1, 2018: Cephalalgia: An International Journal of Headache
https://www.readbyqxmd.com/read/29629528/prevalence-of-multiple-sclerosis-in-a-turkish-city-bordering-an-iron-and-steel-factory
#4
Ülkü Türk Börü, Adnan Burak Bilgiç, Cansu Köseoğlu Toksoy, Abdullah Yasir Yılmaz, Mustafa Tasdemir, Nilay Padir Sensöz, Özgür Öztop Çakmak, Arda Duman, Cem Bölük
BACKGROUND AND PURPOSE: Multiple sclerosis (MS) is an autoimmune disease characterized by inflammatory demyelination. Recent studies have shown that long-term exposure to air pollutants (including PM10 particulates) is potentially an environmental risk factor for MS. We aimed to determine the prevalence rates of MS in two cities with different levels of air pollution. METHODS: This door-to-door population-based study was conducted between April 2014 and June 2015...
April 2018: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/29628374/myelination-of-neuronal-cell-bodies-when-myelin-supply-exceeds-axonal-demand
#5
Rafael G Almeida, Simon Pan, Katy L H Cole, Jill M Williamson, Jason J Early, Tim Czopka, Anna Klingseisen, Jonah R Chan, David A Lyons
The correct targeting of myelin is essential for nervous system formation and function. Oligodendrocytes in the CNS myelinate some axons, but not others, and do not myelinate structures including cell bodies and dendrites [1]. Recent studies indicate that extrinsic signals, such as neuronal activity [2, 3] and cell adhesion molecules [4], can bias myelination toward some axons and away from cell bodies and dendrites, indicating that, in vivo, neuronal and axonal cues regulate myelin targeting. In vitro, however, oligodendrocytes have an intrinsic propensity to myelinate [5-7] and can promiscuously wrap inert synthetic structures resembling neuronal processes [8, 9] or cell bodies [4]...
March 31, 2018: Current Biology: CB
https://www.readbyqxmd.com/read/29627579/genetic-detection-of-sonic-hedgehog-shh-expression-and-cellular-response-in-the-progression-of-acute-through-chronic-demyelination-and-remyelination
#6
Maria A Sanchez, Genevieve M Sullivan, Regina C Armstrong
Multiple sclerosis is a demyelinating disease in which neurological deficits result from damage to myelin, axons, and neuron cell bodies. Prolonged or repeated episodes of demyelination impair remyelination. We hypothesized that augmenting Sonic hedgehog (Shh) signaling in chronically demyelinated lesions could enhance oligodendrogenesis and remyelination. Shh regulates oligodendrocyte development during postnatal myelination, and maintains adult neural stem cells. We used genetic approaches to detect Shh expression and Shh responding cells in vivo...
April 5, 2018: Neurobiology of Disease
https://www.readbyqxmd.com/read/29627036/nocebo-in-chronic-inflammatory-demyelinating-polyneuropathy-a-systematic-review-and-meta-analysis-of-placebo-controlled-clinical-trials
#7
Panagiotis Zis, Marios Hadjivassiliou, Ptolemaios G Sarrigiannis, Thomas M Jenkins, Dimos-Dimitrios Mitsikostas
INTRODUCTION: Nocebo is very prevalent among neurological disorders, resulting in low adherence and treatment outcome. We sought to examine the adverse events (AE) following placebo administration in placebo-controlled randomized clinical trials (RCTs) for chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: After a systematic literature search for RCTs for CIDP pharmacotherapy treatments, we assessed the number of AE in the placebo groups and the number discontinuations because of placebo intolerance...
May 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29626178/unique-clinical-and-neurophysiologic-profile-of-a-cohort-of-children-with-cmtx3
#8
Manoj Kanhangad, Kayla Cornett, Megan H Brewer, Garth A Nicholson, Monique M Ryan, Robert L Smith, Gopinath M Subramanian, Helen K Young, Stephan Züchner, Marina L Kennerson, Joshua Burns, Manoj P Menezes
OBJECTIVE: To describe in detail the clinical profile of Charcot-Marie-Tooth disease subtype 3 (CMTX3) to aid appropriate genetic testing and rehabilitative therapy. METHODS: We reviewed the clinical and neurophysiologic profile and CMT Pediatric Scale (CMTPedS) assessments of 11 children with CMTX3. RESULTS: Compared with the more common forms of CMT, CMT1A and CMTX, CMTX3 was characterized by early onset with early and progressive hand weakness...
April 6, 2018: Neurology
https://www.readbyqxmd.com/read/29624648/cns-langerhans-cell-histiocytosis-common-hematopoietic-origin-for-lch-associated-neurodegeneration-and-mass-lesions
#9
Kenneth L McClain, Jennifer Picarsic, Rikhia Chakraborty, Daniel Zinn, Howard Lin, Harshal Abhyankar, Brooks Scull, Albert Shih, Karen Phaik Har Lim, Olive Eckstein, Joseph Lubega, Tricia L Peters, Walter Olea, Thomas Burke, Nabil Ahmed, M John Hicks, Brandon Tran, Jeremy Jones, Robert Dauser, Michael Jeng, Robert Baiocchi, Deborah Schiff, Stanton Goldman, Kenneth M Heym, Harry Wilson, Benjamin Carcamo, Ashish Kumar, Carlos Rodriguez-Galindo, Nicholas S Whipple, Patrick Campbell, Geoffrey Murdoch, Julia Kofler, Simon Heales, Marian Malone, Randy Woltjer, Joseph F Quinn, Paul Orchard, Michael C Kruer, Ronald Jaffe, Markus G Manz, Sergio A Lira, D Williams Parsons, Miriam Merad, Tsz-Kwong Man, Carl E Allen
BACKGROUND: Central nervous system Langerhans cell histiocytosis (CNS-LCH) brain involvement may include mass lesions and/or a neurodegenerative disease (LCH-ND) of unknown etiology. The goal of this study was to define the mechanisms of pathogenesis that drive CNS-LCH. METHODS: Cerebrospinal fluid (CSF) biomarkers including CSF proteins and extracellular BRAFV600E DNA were analyzed in CSF from patients with CNS-LCH lesions compared with patients with brain tumors and other neurodegenerative conditions...
April 6, 2018: Cancer
https://www.readbyqxmd.com/read/29623298/a-murine-model-of-charcot-marie-tooth-disease-4f-reveals-a-role-for-the-c-terminus-of-periaxin-in-the-formation-and-stabilization-of-cajal-bands
#10
Diane L Sherman, Peter J Brophy
Charcot-Marie-Tooth (CMT) disease comprises up to 80 monogenic inherited neuropathies of the peripheral nervous system (PNS) that collectively result in demyelination and axon degeneration. The majority of CMT disease is primarily either dysmyelinating or demyelinating in which mutations affect the ability of Schwann cells to either assemble or stabilize peripheral nerve myelin. CMT4F is a recessive demyelinating form of the disease caused by mutations in the Periaxin ( PRX ) gene . Periaxin (Prx) interacts with Dystrophin Related Protein 2 (Drp2) in an adhesion complex with the laminin receptor Dystroglycan (Dag)...
2018: Wellcome Open Research
https://www.readbyqxmd.com/read/29623106/defining-distinct-features-of-anti-mog-antibody-associated-central-nervous-system-demyelination
#11
REVIEW
Martin S Weber, Tobias Derfuss, Imke Metz, Wolfgang Brück
Extensive research over the last decades basically failed to identify a common cause of noninfectious inflammatory central nervous system (CNS) demyelinating disease. To a great extent, this may reflect that the group of inflammatory CNS demyelinating disorders likely contains multiple pathogenetically distinct disease entities. Indeed, the greatest success so far in deciphering the pathogenesis of a CNS demyelinating disorder resulted from the discovery of anti-aquaporin (AQP)-4 antibodies (ab), which allowed progressive delineation of neuromyelitis optica (NMO), formerly considered a variant of the most common CNS demyelinating disorder, multiple sclerosis (MS), as a distinct disease...
2018: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/29621743/b-cell-depleting-therapy-for-multiple-sclerosis-overlapping-with-neuromyelitis-optica-spectrum-disorder
#12
Tingting Lu, Yaqing Shu, Yongqiang Dai, Xu Liu, Yanyu Chang, Qiao Huang, Allan G Kermode, Wei Qiu
Multiple sclerosis and neuromyelitis optica spectrum disorder are currently thought to be independent entities. Some patients display intermediate manifestations that fit the criteria for both diseases without positive relevant serobiomarkers. An overall standard and consensus for the diagnosis and treatment of these overlapping patients have not been reached. We describe a patient with frequently relapsing demyelinating episodes and repeatedly adjusted treatment regimens due to diagnostic difficulties. This case did not respond adequately to glucocorticoid plus azathioprine or to interferon...
March 26, 2018: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29619025/mast-cells-and-innate-lymphoid-cells-underappreciated-players-in-cns-autoimmune-demyelinating-disease
#13
REVIEW
Melissa A Brown, Rebecca B Weinberg
Multiple sclerosis (MS) and its mouse model, experimental autoimmune encephalomyelitis, are autoimmune CNS inflammatory diseases. As a result of a breakdown in the relatively impermeable blood-brain barrier (BBB) in affected individuals, myelin-specific CD4+ and CD8+ T cells gain entry into the immune privileged CNS and initiate myelin, oligodendrocyte, and nerve axon destruction. However, despite the absolute requirement for T cells, there is increasing evidence that innate immune cells also play critical amplifying roles in disease pathogenesis...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29618802/integrative-analysis-of-multiple-sclerosis-using-a-systems-biology-approach
#14
Karla Cervantes-Gracia, Holger Husi
Multiple sclerosis (MS) is a chronic autoimmune disorder characterized by inflammatory-demyelinating events in the central nervous system. Despite more than 40 years of MS research its aetiology remains unknown. This study aims to identify the most frequently reported and consistently regulated molecules in MS in order to generate molecular interaction networks and thereby leading to the identification of deregulated processes and pathways which could give an insight of the underlying molecular mechanisms of MS...
April 4, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29618748/fingolimod-therapy-is-not-effective-in-a-mouse-model-of-spontaneous-autoimmune-peripheral-polyneuropathy
#15
Petra Huehnchen, Wolfgang Boehmerle, Matthias Endres
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disorder, which causes progressive sensory and motor deficits and often results in severe disability. Knockout of the co-stimulatory protein CD86 in mice of the non-obese diabetic background (NoD.129S4-Cd86tm1Shr /JbsJ) results in the development of a spontaneous autoimmune peripheral polyneuropathy (SAPP). We used this previously described transgenic model to study the effects of the sphingosine-1-phosphate receptor agonist fingolimod on SAPP symptoms, functional and electrophysiological characteristics...
April 4, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29615960/reported-changes-in-dietary-behavior-following-a-first-clinical-diagnosis-of-central-nervous-system-demyelination
#16
Rebecca D Russell, Robyn M Lucas, Vanessa Brennan, Jill L Sherriff, Andrea Begley, Lucinda J Black
Background/objectives: Although the current evidence is insufficient to recommend a special diet for people with multiple sclerosis (MS), dietary advice for people with MS is prolific online and in the media. This study aimed to describe dietary changes made in the year following a first clinical diagnosis of central nervous system demyelination (FCD), a common precursor to MS. Subjects/methods: We used follow-up data from the Ausimmune Study, a multicentre matched case-control study examining the environmental risk factors for a FCD...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29615658/systemic-igf-1-gene-delivery-by-raav9-improves-spontaneous-autoimmune-peripheral-polyneuropathy-sapp
#17
Tong Gao, Nataliia Bogdanova, Sameera Ghauri, Gang Zhang, Jianxin Lin, Kazim Sheikh
Spontaneous autoimmune peripheral polyneuropathy (SAPP) is a mouse model of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in non-obese diabetic (NOD) mice null for costimulatory molecule, B7-2 gene (B7-2-/- ). SAPP is a chronic progressive and multifocal inflammatory and demyelinating polyneuropathy of spontaneous onset with secondary axonal degeneration. Insulin-like growth factor 1(IGF-1) is a pleiotropic factor with neuroprotective, regenerative, and anti-inflammatory effects with extensive experience in its preclinical and clinical use...
April 3, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29613792/nutshell-extracts-of-xanthoceras-sorbifolia-a-new-potential-source-of-bioactive-phenolic-compounds-as-natural-antioxidant-and-immunomodulator
#18
Li Zhao, Xing Li, Fei Zhang, Juan-Juan Han, Ting Yang, Ze-Qing Ye, Zhe-Zhi Wang, Yuan Zhang
Nutshell of Xanthoceras sorbifolia, a waste product in the production of edible oil, is rich in health-promoting phenolic acids. However, the individual constituents, bioactivities, and mechanism of action are largely unknown. In this study, 20 phenolic compounds were characterized in nutshell extracts (NE) of X. sorbifolia by GC-MS. Four established in vitro studies showed that NE has significant antioxidant potential. Results in vivo indicated that oral administration of NE effectively ameliorated clinical disease severity of experimental autoimmune encephalomyelitis (EAE) and reduced central nervous system (CNS) inflammation and demyelination...
April 3, 2018: Journal of Agricultural and Food Chemistry
https://www.readbyqxmd.com/read/29610440/sustained-expression-of-negative-regulators-of-myelination-protects-schwann-cells-from-dysmyelination-in-a-charcot-marie-tooth-1b-mouse-model
#19
Francesca Florio, Cinzia Ferri, Cristina Scapin, M Laura Feltri, Lawrence Wrabetz, Maurizio D'Antonio
Schwann cell differentiation and myelination in the peripheral nervous system are the result of fine-tuning of positive and negative transcriptional regulators. As myelination starts, negative regulators are down-regulated whereas positive ones are up-regulated. Fully differentiated Schwann cells maintain an extraordinary plasticity, and can transdifferentiate into "repair" Schwann cells after nerve injury. Reactivation of negative regulators of myelination is essential to generate repair Schwann cells...
April 2, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29606615/promoting-myelin-repair-through-in-vivo-neuroblast-reprogramming
#20
Bilal El Waly, Myriam Cayre, Pascale Durbec
Demyelination is frequently observed in a variety of CNS insults and neurodegenerative diseases. In rodents, adult neural stem cells can generate oligodendrocytes and participate to myelin repair. However, these cells mainly produce migratory neuroblasts that differentiate in the olfactory bulb. Here, we show that, in the demyelination context, a small subset of these neuroblasts can spontaneously convert into myelinating oligodendrocytes. Furthermore, we demonstrate that the contribution of neuroblasts to myelin repair can be improved by in vivo forced expression of two transcription factors: OLIG2 and SOX10...
March 27, 2018: Stem Cell Reports
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