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https://www.readbyqxmd.com/read/28634078/astrocytes-regulate-the-expression-of-sp1r3-on-oligodendrocyte-progenitor-cells-through-cx47-and-promote-their-proliferation
#1
Dan Xu, Zhaoyu Liu, Shang Wang, Yan Peng, Xiaochuan Sun
Many degenerative diseases of the central nervous system are associated with demyelination. Oligodendrocyte progenitor cells (OPCs) are potential stem cells that can differentiate into various cell types, including oligodendrocytes (OLs). Promoting the proliferation and differentiation of OPCs into mature OLs that can myelinate axons is the key to stimulate remyelination. Here, we report that astrocytes (ASTs) increase the number of sphingosine-1-phosphate receptors 3 (S1pR3) on OPCs and promote OPCs proliferation through a direct contact via connexin 47 (Cx47)...
June 17, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28633435/mcm3ap-in-recessive-charcot-marie-tooth-neuropathy-and-mild-intellectual-disability
#2
Emil Ylikallio, Rosa Woldegebriel, Manuela Tumiati, Pirjo Isohanni, Monique M Ryan, Zornitza Stark, Maie Walsh, Sarah L Sawyer, Katrina M Bell, Alicia Oshlack, Paul J Lockhart, Mariia Shcherbii, Alejandro Estrada-Cuzcano, Derek Atkinson, Taila Hartley, Martine Tetreault, Inge Cuppen, W Ludo van der Pol, Ayse Candayan, Esra Battaloglu, Yesim Parman, Koen L I van Gassen, Marie-José H van den Boogaard, Kym M Boycott, Liisa Kauppi, Albena Jordanova, Tuula Lönnqvist, Henna Tyynismaa
Defects in mRNA export from the nucleus have been linked to various neurodegenerative disorders. We report mutations in the gene MCM3AP, encoding the germinal center associated nuclear protein (GANP), in nine affected individuals from five unrelated families. The variants were associated with severe childhood onset primarily axonal (four families) or demyelinating (one family) Charcot-Marie-Tooth neuropathy. Mild to moderate intellectual disability was present in seven of nine affected individuals. The affected individuals were either compound heterozygous or homozygous for different MCM3AP variants, which were predicted to cause depletion of GANP or affect conserved amino acids with likely importance for its function...
June 19, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28631805/drug-therapy-for-chronic-idiopathic-axonal-polyneuropathy
#3
REVIEW
Janna Warendorf, Alexander Fje Vrancken, Ivo N van Schaik, Richard Ac Hughes, Nicolette C Notermans
BACKGROUND: Chronic idiopathic axonal polyneuropathy (CIAP) is an insidiously progressive sensory or sensorimotor polyneuropathy that affects elderly people. Although severe disability or handicap does not occur, CIAP reduces quality of life. CIAP is diagnosed in 10% to 25% of people referred for evaluation of polyneuropathy. There is a need to gather and review emerging evidence on treatments, as the number of people affected is likely to increase in ageing populations. This is an update of a review first published in 2004 and previously updated in 2006, 2008, 2011 and 2013...
June 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28631093/myelin-regulatory-factor-drives-remyelination-in-multiple-sclerosis
#4
Greg J Duncan, Jason R Plemel, Peggy Assinck, Sohrab B Manesh, Fraser G W Muir, Ryan Hirata, Matan Berson, Jie Liu, Michael Wegner, Ben Emery, G R Wayne Moore, Wolfram Tetzlaff
Remyelination is limited in the majority of multiple sclerosis (MS) lesions despite the presence of oligodendrocyte precursor cells (OPCs) in most lesions. This observation has led to the view that a failure of OPCs to fully differentiate underlies remyelination failure. OPC differentiation requires intricate transcriptional regulation, which may be disrupted in chronic MS lesions. The expression of few transcription factors has been differentially compared between remyelinating lesions and lesions refractory to remyelination...
June 19, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28629631/probenecid-treatment-reduces-demyelination-induced-by-cuprizone-feeding
#5
Nadine Hainz, Philipp Becker, Daniel Rapp, Stefan Wagenpfeil, Bodo Wonnenberg, Christoph Beisswenger, Thomas Tschernig, Carola Meier
Recent experiments showed that a pannexin-1 inhibitor, probenecid, reduced clinical symptoms in the murine experimental autoimmune encephalomyelitis when applied during the initial phase of neuronal inflammation. An inflammatory component is also present in a toxically induced inflammation and demyelination using cuprizone diet. Probenecid is a pannexin-1 antagonist and a probenecid therapy was investigated. Mice were fed for 10days with a cuprizone diet. In the following, the diet was continued but combined with a daily injection of a low dose of probenecid or solvent for 10days...
June 16, 2017: Journal of Chemical Neuroanatomy
https://www.readbyqxmd.com/read/28629158/contribution-of-the-degeneration-of-the-neuro-axonal-unit-to-the-pathogenesis-of-multiple-sclerosis
#6
REVIEW
Hannah E Salapa, Sangmin Lee, Yoojin Shin, Michael C Levin
Multiple sclerosis (MS) is a demyelinating, autoimmune disease of the central nervous system. In recent years, it has become more evident that neurodegeneration, including neuronal damage and axonal injury, underlies permanent disability in MS. This manuscript reviews some of the mechanisms that could be responsible for neurodegeneration and axonal damage in MS and highlights the potential role that dysfunctional heterogeneous nuclear ribonucleoprotein A1 (hnRNP A1) and antibodies to hnRNP A1 may play in MS pathogenesis...
June 18, 2017: Brain Sciences
https://www.readbyqxmd.com/read/28627931/hyperacoustic-hypoacusis-a-new-pontine-syndrome-case-report
#7
Mohammad Hassan A Noureldine, Raghid Kikano, Naji Riachi, Rechdi Ahdab
We herein report a unique case of a lateral pontine demyelinating lesion presenting with unilateral sensorineural hearing loss and paradoxical ipsilateral hyperacusis. The association of unilateral hearing loss and ipsilateral hyperacusis is a rare manifestation of a central nervous system lesion. The paradoxical combination of these symptoms strongly suggests pontine dysfunction and prompts urgent neurological evaluation.
June 19, 2017: Brain Injury: [BI]
https://www.readbyqxmd.com/read/28625589/acute-disseminated-encephalomyelitis-in-dengue-viral-infection
#8
REVIEW
Wan Aliaa Wan Sulaiman, Liyana Najwa Inche Mat, Hasnur Zaman Hashim, Fan Kee Hoo, Siew Mooi Ching, Ramachandran Vasudevan, Mohd Hazmi Mohamed, Hamidon Basri
Dengue is the most common arboviral disease affecting many countries worldwide. An RNA virus from the flaviviridae family, dengue has four antigenically distinct serotypes (DEN-1-DEN-4). Neurological involvement in dengue can be classified into dengue encephalopathy immune-mediated syndromes, encephalitis, neuromuscular or dengue muscle dysfunction and neuro-ophthalmic involvement. Acute disseminated encephalomyelitis (ADEM) is an immune mediated acute demyelinating disorder of the central nervous system following recent infection or vaccination...
June 15, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28625338/acute-diplopia-in-the-pediatric-emergency-department-a-cohort-multicenter-italian-study
#9
Umberto Raucci, Pasquale Parisi, Nicola Vanacore, Francesco La Penna, Valentina Ferro, Lucia Calistri, Claudia Bondone, Fabio Midulla, Agnese Suppiej, Raffaele Falsaperla, Duccio Maria Cordelli, Antonella Palmieri, Alberto Verrotti, Sabrina Becciani, Sonia Aguzzi, Mario Mastrangelo, Federica Pelizza, Filippo Greco, Giulia Carbonari, Ramona Tallone, Gabriella Bottone, Italo Trenta, Stefano Masi, Maria Pia Villa, Antonino Reale
BACKGROUND: Acute diplopia (AD) is an uncommon and distressing symptom of numerous ocular and neurological conditions, with potentially serious sequelaes. No data are present in pediatrics on the presentation and management of AD. AIM: This study investigated characteristics, etiology and health care utilization of the pediatric population with AD accessed to pediatric Emergency Departments (ED), trying to identify "red flags" associated with potentially life-threatening (LT) conditions...
June 3, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28623673/a-prominent-lactate-peak-as-a-potential-key-magnetic-resonance-spectroscopy-mrs-feature-of-progressive-multifocal-leukoencephalopathy-pml-spectrum-pattern-observed-in-three-patients
#10
Duško Kozić, Mladen Bjelan, Jasmina Boban, Jelena Ostojić, Vesna Turkulov, Aleksandar Todorović, Slobodanka Lemajić-Komazec, Snežana Brkić
Progressive multifocal leukoencephalopathy (PML) is a rare, often fatal, opportunistic infection, associated with demyelinating process. PML is caused by John Cunningham (JC) polyomavirus, and predominantly involves patients with human immunodeficiency virus (HIV) disease or other immunocompromised conditions. The purpose of this report was to determine the role of magnetic resonance spectroscopy (MRS) in establishing the diagnosis of PML. MRS with long and short echo time was performed in two patients with PML associated with HIV infection and in one PML patient associated with chronic lymphocytic leukemia...
June 17, 2017: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28622911/demyelination-strokes-and-eculizumab-lessons-from-the-congenital-cd59-gene-mutations
#11
Adi Tabib, Netanel Karbian, Dror Mevorach
Neurological symptoms of patients with p.Cys89Tyr mutation in the CD59 gene include recurrent peripheral neuropathy resembling Guillain-Barré syndrome, characterized by sensory-motor demyelinating neuropathy with secondary axonal damage and moderate enhancement of the nerve roots on spine MRI, together with recurrent strokes and retinal involvement. Three additional mutations in CD59, leading to loss of function, have been described, and overall, 12/12 (100%) of patients with any mutation presented with neurological symptoms; 11/12 (92%) patients presented with recurrent peripheral neuropathy, 6/12 (50%) with recurrent strokes, and 1/12 (8%) with retinal involvement...
June 13, 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28620719/large-coverage-mr-neurography-in-cidp-diagnostic-accuracy-and-electrophysiological-correlation
#12
Moritz Kronlage, Philipp Bäumer, Kalliopi Pitarokoili, Daniel Schwarz, Véronique Schwehr, Tim Godel, Sabine Heiland, Ralf Gold, Martin Bendszus, Min-Suk Yoon
The objective of this study was to evaluate large coverage magnetic resonance neurography (MRN) in chronic inflammatory demyelinating polyneuropathy (CIDP). In this prospective study, 18 patients with CIDP and 18 healthy controls were examined by a standardized MRN protocol at 3 T. Lumbosacral plexus was imaged by a T2-weighted 3D sequence and peripheral nerves of the upper and lower extremity by axial T2-weighted turbo spin-echo sequences. Lesions were characterized by nerve cross-sectional area (CSA) and T2-weighted signal (nT2)...
June 15, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28619436/tumefactive-demyelinating-lesions-a-comprehensive-review
#13
REVIEW
Hussein Algahtani, Bader Shirah, Ali Alassiri
Tumefactive multiple sclerosis or tumefactive demyelinating lesion (TDL) is one of the rare variants of multiple sclerosis (MS) posing a diagnostic challenge and a therapeutic enigma since it is difficult to distinguish from a true central nervous system (CNS) neoplasm or other CNS lesions on magnetic resonance imaging (MRI). The prevalence of TDL is estimated to be 1-3/1000 cases of MS with an annual incidence of 0.3/100,000. This could be an underestimate due to unavailability of a global MS registry and under-reporting of this condition...
May 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28619434/terminalia-chebula-attenuates-quinolinate-induced-oxidative-pc12-and-oln-93-cell-death
#14
Hamid R Sadeghnia, Roya Jamshidi, Amir R Afshari, Hamid Mollazadeh, Fatemeh Forouzanfar, Hasan Rakhshandeh
BACKGROUND: Quinolinic acid (QA) is a product of tryptophan degradation and its pathologic accumulation has been found to induce neuroinflammatory and demyelinating diseases such as multiple sclerosis via excessive free radicals generation. Recent studies showed that Terminalia chebula has several pharmacological effects such as antioxidant, anti-inflammatory and neuroprotective properties. The aim of this study was evaluation of the protective effect of T. chebula alcoholic extract (TCAE) on oxidative PC12 and OLN-93 cells death induced by QA...
May 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28619428/neuromyelitis-optica-presenting-with-horner-syndrome-a-case-report-and-review-of-literature
#15
İrem Fatma Uludağ, Alp Sarıteke, Levent Öcek, Yaşar Zorlu, Ufuk Şener, Figen Tokuçoğlu, Burhanettin Uludağ
Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. We describe a 19 years old woman with left Horner syndrome (HS), who was diagnosed as NMO with characteristic longitudinally extensive myelitis and positive serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Our report describes one of the very rare ocular motor symptoms in NMO patients.
May 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28619424/cerebrospinal-fluid-and-serum-levels-of-interleukin-8-in-patients-with-multiple-sclerosis-and-its-correlation-with-q-albumin
#16
Z Matejčíková, J Mareš, V Sládková, T Svrčinová, J Vysloužilová, J Zapletalová, P Kaňovský
INTRODUCTION: Multiple sclerosis (MS) is an inflammatory-demyelinating disease of the central nervous system (CNS). Autoimmune inflammation is common in the early stages of MS and is followed by neurodegenerative processes. The result of these changes is axon and myelin breakdown. The paraclinical examination methods are an important part of the diagnostic process. Magnetic resonance imaging of the brain and the cervical spinal cord and an examination of cerebrospinal fluid (CSF) are common paraclinical examinations...
May 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28619074/novel-oligodendroglial-alpha-synuclein-viral-vector-models-of-multiple-system-atrophy-studies-in-rodents-and-nonhuman-primates
#17
Ronald J Mandel, David J Marmion, Deniz Kirik, Yaping Chu, Clifford Heindel, Thomas McCown, Steven J Gray, Jeffrey H Kordower
Multiple system atrophy (MSA) is a horrible and unrelenting neurodegenerative disorder with an uncertain etiology and pathophysiology. MSA is a unique proteinopathy in which alpha-synuclein (α-syn) accumulates preferentially in oligodendroglia rather than neurons. Glial cytoplasmic inclusions (GCIs) of α-syn are thought to elicit changes in oligodendrocyte function, such as reduced neurotrophic support and demyelination, leading to neurodegeneration. To date, only a murine model using one of three promoters exist to study this disease...
June 16, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28618115/iron-sulfur-glutaredoxin-2-protects-oligodendrocytes-against-damage-induced-by-nitric-oxide-release-from-activated-microglia
#18
Klaudia Lepka, Katrin Volbracht, Eckhard Bill, Reiner Schneider, Natalia Rios, Thomas Hildebrandt, Jens Ingwersen, Timur Prozorovski, Christopher Horst Lillig, Jack van Horssen, Lawrence Steinman, Hans-Peter Hartung, Rafael Radi, Arne Holmgren, Orhan Aktas, Carsten Berndt
Demyelinated brain lesions, a hallmark of autoimmune neuroinflammatory diseases like multiple sclerosis, result from oligodendroglial cell damage. Activated microglia are considered a major source of nitric oxide and subsequent peroxynitrite-mediated damage of myelin. Here, we provide biochemical and biophysical evidence that the oxidoreductase glutaredoxin 2 inhibits peroxynitrite formation by transforming nitric oxide into dinitrosyl-diglutathionyl-iron-complexes. Glutaredoxin 2 levels influence both survival rates of primary oligodendrocyte progenitor cells and preservation of myelin structure in cerebellar organotypic slice cultures challenged with activated microglia or nitric oxide donors...
June 15, 2017: Glia
https://www.readbyqxmd.com/read/28618073/drug-discovery-for-remyelination-and-treatment-of-ms
#19
REVIEW
Katy L H Cole, Jason J Early, David A Lyons
Glia constitute the majority of the cells in our nervous system, yet there are currently no drugs that target glia for the treatment of disease. Given ongoing discoveries of the many roles of glia in numerous diseases of the nervous system, this is likely to change in years to come. Here we focus on the possibility that targeting the oligodendrocyte lineage to promote regeneration of myelin (remyelination) represents a therapeutic strategy for the treatment of the demyelinating disease multiple sclerosis, MS...
June 15, 2017: Glia
https://www.readbyqxmd.com/read/28617998/schwann-cell-specific-deletion-of-the-endosomal-pi-3-kinase-vps34-leads-to-delayed-radial-sorting-of-axons-arrested-myelination-and-abnormal-erbb2-erbb3-tyrosine-kinase-signaling
#20
Anne M Logan, Anna E Mammel, Danielle C Robinson, Andrea L Chin, Alec F Condon, Fred L Robinson
The PI 3-kinase Vps34 (Pik3c3) synthesizes phosphatidylinositol 3-phosphate (PI3P), a lipid critical for both endosomal membrane traffic and macroautophagy. Human genetics have implicated PI3P dysregulation, and endosomal trafficking in general, as a recurring cause of demyelinating Charcot-Marie-Tooth (CMT) peripheral neuropathy. Here, we investigated the role of Vps34, and PI3P, in mouse Schwann cells by selectively deleting Vps34 in this cell type. Vps34-Schwann cell knockout (Vps34(SCKO) ) mice show severe hypomyelination in peripheral nerves...
June 15, 2017: Glia
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