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Marlene Thorsen Mørch, Sofie Forsberg Sørensen, Reza Khorooshi, Nasrin Asgari, Trevor Owens
BACKGROUND: Encounter of autoantibodies with specific antigens can lead to hypersensitivity reactions and pathology. In multiple sclerosis and neuromyelitis optica spectrum disease (NMOSD), immunoglobulin-G (IgG) deposition has been observed in pathological lesions in the central nervous system. The paradigmatic autoantibodies in NMOSD are specific for the water channel aquaporin-4, localized to astrocytic end-feet at the blood-brain barrier and ependymal cells at the cerebrospinal fluid-brain barrier...
April 17, 2018: Journal of Neuroinflammation
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
Timothy J Zerk, Peter W Moore, Joshua S Harbort, Sharon Chow, Lindsay Byrne, George A Koutsantonis, Jeffrey R Harmer, Manuel Martínez, Craig M Williams, Paul V Bernhardt
The Ley-Griffith reaction is utilized extensively in the selective oxidation of alcohols to aldehydes or ketones. The central catalyst is commercially available tetra- n -propylammonium perruthenate (TPAP, n -Pr4 N[RuO4 ]) which is used in combination with the co-oxidant N -methylmorpholine N -oxide (NMO). Although this reaction has been employed for more than 30 years, the mechanism remains unknown. Herein we report a comprehensive study of the oxidation of diphenylmethanol using the Ley-Griffith reagents to show that the rate determining step involves a single alcohol molecule, which is oxidised by a single perruthenate anion; NMO does not appear in rate law...
December 1, 2017: Chemical Science
Zhiguo Li, Jinrui Han, Honglei Ren, Cun-Gen Ma, Fu-Dong Shi, Qiang Liu, Minshu Li
Astrocyte loss induced by neuromyelitis optica (NMO)-IgG and complement-dependent cytotoxicity (CDC) is the hallmark of NMO pathology. The survival of astrocytes is thought to reflect astrocyte exposure to environmental factors in the CNS and the response of astrocytes to these factors. However, still unclear are how astrocytes respond to NMO-IgG and CDC, and what CNS environmental factors may impact the survival of astrocytes. In a murine model of NMO induced by intracerebral injection of NMO-IgG and human complement, we found dramatic upregulation of IL-15 in astrocytes...
2018: Frontiers in Immunology
J Ciron, B Audoin, B Bourre, D Brassat, F Durand-Dubief, D Laplaud, E Maillart, C Papeix, S Vukusic, H Zephir, R Marignier, N Collongues
There is growing evidence of a preventive effect of Rituximab (RTX) in neuromyelitis optica spectrum disorders (NMO-SD). This monoclonal antibody against CD20 is becoming the most widely used preventive therapy in NMO-SD, as a first-line therapy or as a rescue therapy. Nevertheless, considerable heterogeneity still exists concerning the pre-treatment work-up, the vaccinations required before and under treatment, the number and dosage of infusions, prevention of the risk of infusion-related reactions, prevention of infections under treatment, and frequency of therapeutic cycles...
March 29, 2018: Revue Neurologique
Yerbolat Tashenov, Mathias Daniels, Koen Robeyns, Luc Van Meervelt, Wim Dehaen, Yerlan M Suleimen, Zsolt Szakonyi
A library of bidentate diols, as well as tridentate triols and aminodiols, derived from (+)-sabinol, was synthesized in a stereoselective manner. Sabinol was transformed into allylic trichloroacetamide via Overman rearrangement of the corresponding trichloroacetimidate. After changing the protecting group to Boc, the enamine was subjected to stereospecific dihydroxylation with OsO₄/NMO, resulting in the (1R,2R,3R,5R)-aminodiol diastereomer. The obtained primary aminodiol was transformed to a secondary analogue...
March 27, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
Tetsuya Akaishi, Toshiyuki Takahashi, Noriko Himori, Takayuki Takeshita, Toru Nakazawa, Masashi Aoki, Ichiro Nakashima
Chloride imbalance between the serum and the cerebrospinal fluid (CSF) has been recently shown to exist in the acute phase of neuromyelitis optica (NMO). In this report, we studied the relation between the quotient of chloride (QCl ) and the severity of optic neuritis (ON) in NMO patients. There was a positive correlation (R = 0.67; p < 0.05) between QCl and the length of ON-lesion. The visual prognosis also showed a positive correlation with QCl in the acute phase (R = 0.58; p < 0.05)...
March 16, 2018: Journal of Neuroimmunology
Min Li, Jia Liu, Huan Yi, Li Xu, Xiufeng Zhong, Fuhua Peng
BACKGROUND: Wolfram syndrome (WS), caused by mutations of the Wolfram syndrome 1 (WFS1) gene on chromosome 4p16.1, is an autosomal recessive disorder characterized by diabetes insipidus (DI), neuro-psychiatric disorders, hearing deficit, and urinary tract anomalies. CASE PRESENTATION: Here we report a 11-year-old Chinese boy who presented with visual loss, was suspected with optic neuritis (ON) or neuromyelitis optica (NMO) and referred to our department for further diagnosis...
March 17, 2018: BMC Pediatrics
L Monge Galindo, A L Martínez de Morentín, V Pueyo Royo, J P García Iñiguez, S Sánchez Marco, J López-Pisón, J L Peña-Segura
INTRODUCTION AND OBJECTIVE: In this article, we present our experience on optic neuritis (ON) and provide a diagnostic/therapeutic protocol, intended to rule out other aetiologies (particularly infection), and a fact sheet for parents. MATERIAL AND METHODS: We conducted a descriptive, retrospective study of patients with ON over a 27-year period (1990-2017). A review of the available scientific evidence was performed in order to draft the protocol and fact sheet...
March 8, 2018: Neurología: Publicación Oficial de la Sociedad Española de Neurología
Christopher Hollen, Omer Suhaib, Aaron Farrow, Evgeny Sidorov
We present a case of an 82-year-old man with new-onset neuromyelitis optica (NMO) spectrum disorder, the treatment of which was complicated by a severe pre-existing prednisone allergy. His age caused much initial doubt about his diagnosis, and his corticosteroid allergy altered our management as we attempted to minimize risk to the patient. Our patient was a healthy 82-year-old, right-handed man who presented with sensory loss of the bilateral lower extremities and progressive, painless vision loss. MRI showed bilateral pre-chiasmatic optic nerve and optic chiasm enhancement, along with enhancement within the thoracic spinal cord from T3 to T7...
January 2018: Case Reports in Neurology
Ami Schattner, Shilo Voichanski, Livnat Uliel
A healthy 38-year-old woman developed sudden unilateral vision loss due to retrobulbar optic neuritis in the wake of varicella-zoster virus infection. She had no further central nervous system (CNS) lesions. Antinuclear antibodies (ANA) and anti-aquaporin 4 antibodies were found, consistent with neuromyelitis optica (NMO). Later, serial MRIs showed dynamic short-segment and long-segment myelitis lesions, ANA titre increased and additional autoantibodies were found including anti-dsDNA, anti-chromatin/nucleosome and antiphospholipid antibodies...
March 5, 2018: BMJ Case Reports
Seyed Mohammad Baghbanian, Nasrin Asgari, Mohammad Ali Sahraian, Abdorreza Naser Moghadasi
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disorder of the central nervous system (CNS). Serum immunoglobulin G autoantibodies (NMO-IgG) are identified in the majority of NMOSD patients. The Pediatric form presents before 18 years. Based on the similarity of clinical, neuroimaging, and laboratory characteristics of pediatric NMOSD to those of the adult form, the international panel suggested that adult criteria of NMOSD also are appropriate in pediatric patients. However, the proposed criteria need validation in pediatric patients...
May 15, 2018: Journal of the Neurological Sciences
J Al Abdullah, Abdul G Al Lafi, Yusr Amin, Tasneem Alnama
Nano-composites were synthesized by the reaction of waste polystyrene (PS) and KMnO4 . The structure of the composite was controlled by the solvent/non-solvent system and the concentration of KMnO4 . The FTIR spectra indicated the functionalization of PS and the attachment of NMO with the polymer chains. The maximum adsorption capacities (qmax ) were 10,000 and 5000 Bq g-1 , for U and Th respectively. Different but controllable sorption/desorption behaviours were noted between Th and U, which could be promising in the separation of Th and U from their mixture...
February 16, 2018: Applied Radiation and Isotopes
Fernanda C Rueda-Lopes, Fernanda M C Pessôa, Gustavo Tukamoto, Fabíola Rachid Malfetano, Simone Batista Scherpenhuijzen, Soniza Alves-Leon, Emerson L Gasparetto
OBJECTIVE: The aim of our study was to detect functional changes in default-mode network of neuromyelitis optica (NMO) patients using resting-state functional magnetic resonance images and the evaluation of subcortical grey-matter structures volumes. MATERIALS AND METHODS: NMO patients (n = 28) and controls patients (N=19) were enrolled. We used the integrated registration and segmentation tool, part of FMRIB's Software Library (FSL) to segment subcortical structures including the thalamus, caudate nucleus, putamen, hippocampus and amygdalae...
February 19, 2018: Journal of Neuroradiology. Journal de Neuroradiologie
Philippe Cabre, M Mejdoubi, S Jeannin, H Merle, Y Plumelle, G Cavillon, D Smadja, R Marignier
OBJECTIVE: Neuromyelitis optica (NMO) is a very severe autoimmune disorder of the central nervous system. It affects young subjects and has a poor prognosis both on a functional and vital level. Therefore, it is imperative to reduce the frequency of relapses. The purpose of this study was to evaluate the clinical and neuroradiological effectiveness of rituximab (RTX) on active forms of NMO. METHODS: We conducted a 2-year open prospective multicenter study that included 32 patients treated with RTX at a dose of 375 mg/m2 /week for 1 month...
February 17, 2018: Journal of Neurology
Krystian Obara, Marta Waliszewska-Prosół, Sławomir Budrewicz, Paweł Szewczyk, Maria Ejma
Neuromyelitis optica (NMO) is a rare, disabling, recurring inflammatory demyelinating disease affecting the spinal cord and optic nerves with predominance in women. We present the case of a female patient with chronic C hepatitis, who, despite treatment, developed severe symptoms of NMO during pregnancy and postpartum.
February 6, 2018: Neurologia i Neurochirurgia Polska
Elisabeth Maillart, Anaïs Lippi, Catherine Lubetzki, Céline Louapre, Caroline Papeix
BACKGROUND: We report a dramatic clinical and radiological worsening within two months after rituximab initiation in a patient with NeuroMyelitis Optica/Multiple Sclerosis (NMO/MS) overlap syndrome. METHODS: Case study. RESULTS: A 45-year-old Caucasian woman with NMO/MS overlap syndrome experienced a severe myelitis nine weeks after first rituximab infusion, with extensive new gadolinium-enhanced spinal cord lesions. CONCLUSION: This case report illustrates the limits of MS and NMO-Spectrum Disorder classification and challenges the criteria of therapeutic failure within the 6 months after rituximab initiation...
February 6, 2018: Multiple Sclerosis and related Disorders
Lukmanee Tradtrantip, Christian M Felix, Rolf Spirig, Adriana Baz Morelli, A S Verkman
Intravenous human immunoglobulin G (IVIG) may have therapeutic benefit in neuromyelitis optica spectrum disorders (herein called NMO), in part because of the anti-inflammatory properties of the IgG Fc region. Here, we evaluated recombinant Fc hexamers consisting of the IgM μ-tailpiece fused with the Fc region of human IgG1. In vitro, the Fc hexamers prevented cytotoxicity in aquaporin-4 (AQP4) expressing cells and in rat spinal cord slice cultures exposed to NMO anti-AQP4 autoantibody (AQP4-IgG) and complement, with >500-fold greater potency than IVIG or monomeric Fc fragments...
February 8, 2018: Neuropharmacology
Brett Alldredge, Allison Jordan, Jaime Imitola, Michael K Racke
OBJECTIVES: Multiple sclerosis (MS) is an immune-mediated disease of the central nervous system. B cells play an important pathogenic role in MS. Rituximab (RTX), a B-cell depleting drug, has been used to treat MS and neuromyelitis optica (NMO). Patient characteristics, safety, and efficacy measures are reviewed to ascertain the therapeutic benefit and safety of RTX in a real-world setting with long-term follow-up. METHODS: This is a retrospective chart review of patients who received RTX at The Ohio State University's MS clinic from January 2005 to October 2016...
March 2018: Clinical Neuropharmacology
Johnson Agniswamy, Renata A G Reis, Yuan-Fang Wang, Crystal Smitherman, Dan Su, Irene Weber, Giovanni Gadda
Nitronate monooxygenase (NMO) is an FMN-dependent enzyme that oxidizes the neurotoxin propionate 3-nitronate (P3N) and represents the best-known system for P3N detoxification in different organisms. The crystal structure of the first eukaryotic Class I NMO from Cyberlindnera saturnus (CsNMO) has been solved at 1.65 Å resolution and refined to an R-factor of 14.0%. The three-dimensional structures of yeast CsNMO and bacterial PaNMO are highly conserved with the exception of three additional loops on the surface in the CsNMO enzyme and differences in four active sites residues...
January 31, 2018: Proteins
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