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https://www.readbyqxmd.com/read/29901009/a-case-of-primary-sj%C3%A3-gren-s-syndrome-presenting-as-mass-like-encephalitis-with-progression-to-neuromyelitis-optica-spectrum-disorder
#1
Hyun-Jung Lee, Sung Hae Chang, Eun Ha Kang, Yun Jong Lee, Yeong Wook Song, You-Jung Ha
Neuromyelitis optica is an idiopathic inflammatory demyelinating disease of the central nervous system (CNS) that predominantly affects the optic nerves and spinal cord. With the discovery of the pathogenic anti-aquaporin-4 (AQP4) antibody, the disease was recognized as part of a spectrum of autoimmune diseases that target AQP4, collectively referred to as neuromyelitis optica spectrum disorder (NMOSD). NMOSD consists of conditions that affect various parts of the CNS with the AQP4 antibody. In this article, we report a 43-year-old female patient who was initially diagnosed with primary Sjögren's syndrome (pSS) with CNS involvement, but was later diagnosed with overlapping pSS and NMOSD, which required more intensive treatment...
December 2017: Archives of Rheumatology
https://www.readbyqxmd.com/read/29881979/only-follow-up-of-memory-b-cells-helps-monitor-rituximab-administration-to-patients-with-neuromyelitis-optica-spectrum-disorders
#2
Christine Lebrun, Mikael Cohen, Maria Alessandra Rosenthal-Allieri, Saskia Bresch, Sylvia Benzaken, Romain Marignier, Barbara Seitz-Polski, Michel Ticchioni
INTRODUCTION: Neuromyelitis optica spectrum disorders (NMOSD) are identified as a spectrum of inflammatory demyelinating disorders involving the brain, spinal cord and optic nerves. These disorders require early diagnosis and highly active immunosuppressive treatment. Rituximab (RTX) has demonstrated efficacy in limiting relapse in NMOSD when using several administration schedules. We questioned if the CD19+ CD27+ memory B cell count was a more reliable marker to monitor RTX administration than the RTX plasma level and CD19+ B cell count...
June 7, 2018: Neurology and Therapy
https://www.readbyqxmd.com/read/29872456/tryptophan-immunoadsorption-during-pregnancy-and-breastfeeding-in-patients-with-acute-relapse-of-multiple-sclerosis-and-neuromyelitis-optica
#3
Frank Hoffmann, Andrea Kraft, Franz Heigl, Erich Mauch, Jürgen Koehler, Lutz Harms, Tania Kümpfel, Wolfgang Köhler, Sven Ehrlich, Antonios Bayas, Julia Weinmann-Menke, Carolin Beuker, Karl-Heinz Henn, Ilya Ayzenberg, Gisa Ellrichmann, Kerstin Hellwig, Reinhard Klingel, Cordula Marie Fassbender, Harald Fritz, Torsten Slowinski, Horst Weihprecht, Marcus Brand, Thomas Stiegler, Jan Galle, Sebastian Schimrigk
Background: Up to every fourth woman with multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD) suffers a clinically relevant relapse during pregnancy. High doses of steroids bear some serious risks, especially within the first trimester of pregnancy. Immunoadsorption (IA) is an effective and more selective treatment option in disabling MS relapse than plasma exchange. Data on the use of IA during pregnancy and breastfeeding are scarce. Methods: In this retrospective multicenter study, we analyzed the safety and efficacy of IA treatment in acute relapses during pregnancy or breastfeeding...
2018: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/29806512/intractable-nausea-and-vomiting-as-an-uncommon-presentation-in-an-anti-aquaporin-4-positive-patient
#4
Lu Wang, Hong-Jun Su, Jin-Long Qi, Rui-Fang E, Kun Zhao
Autoantibodies targeting aquaporin 4 (AQP4) water channels are a sensitive and specific biomarker for neuromyelitis optica spectrum disorder (NMOSD). Presence of AQP4 antibodies distinguishes NMOSD from multiple sclerosis. We present our experience with an anti-AQP4 antibody-positive patient diagnosed with NMOSD who complained of intractable nausea and vomiting, not restricted to optic neuritis or acute myelitis during the first attack. Her symptoms partially resolved after appropriate therapy with intravenous methylprednisolone and oral prednisolone...
January 1, 2018: Journal of International Medical Research
https://www.readbyqxmd.com/read/29802557/short-term-effect-of-additional-apheresis-on-visual-acuity-changes-in-patients-with-steroid-resistant-optic-neuritis-in-neuromyelitis-optica-spectrum-disorders
#5
Sotaro Mori, Takuji Kurimoto, Kaori Ueda, Makoto Nakamura
PURPOSE: To evaluate temporal changes in visual acuity in patients with steroid-resistant optic neuritis (ON) in neuromyelitis optica spectrum disorders (NMOSD) after apheresis. DESIGN: Retrospective observational study, clinical case series SUBJECTS AND METHODS: We reviewed the medical charts of 15 eyes of 9 consecutive patients with ON in NMOSD who underwent apheresis between March 2010 and September 2017. All patients were seropositive for anti-aquaporin 4 (AQP4) antibody and resistant to steroid pulse therapy...
May 25, 2018: Japanese Journal of Ophthalmology
https://www.readbyqxmd.com/read/29798830/neuromyelitis-optica-in-sub-saharan-africa-the-first-case-report-from-togo
#6
K Apetse, K Assogba, J E Diatewa, J J Dongmo Tajeuna, N Maneh, F M Tassa Kayem, D Kombate, K A Balogou
Neuromyelitis optica (NMO) appears to affect blacks disproportionately in mixed populations. We report the first case of documented NMO in Togo (West Africa). A 26-year-old Togolese woman was admitted for progressive tetraplegia, acute urine retention, and dyspnea. Magnetic resonance imaging of the cervical spinal cord demonstrated an extensive longitudinal lesion, and an ophthalmologic examination found bilateral optic neuritis. Antibody testing for AQP4-IgG was positive. The patient died of severe respiratory distress despite high doses of intravenous corticosteroids...
May 25, 2018: Médecine et Santé Tropicales
https://www.readbyqxmd.com/read/29787495/differentiating-neuromyelitis-optica-related-and-multiple-sclerosis-related-acute-optic-neuritis-using-conventional-magnetic-resonance-imaging-combined-with-readout-segmented-echo-planar-diffusion-weighted-imaging
#7
Ping Lu, Guohong Tian, Xilan Liu, Feng Wang, Zhongshuai Zhang, Yan Sha
PURPOSE: In clinical practice, acute optic neuritis (ON) associated with the development of neuromyelitis optica (NMO) after the first attack is often indistinguishable from that associated with multiple sclerosis (MS). We aimed to determine the optimal combination of features derived from conventional magnetic resonance imaging (MRI) and diffusion-weighted imaging using readout-segmented echo-planar imaging (RESOLVE-DWI) for the differentiation of these conditions. MATERIALS AND METHODS: Orbital conventional MRI and RESOLVE-DWI were performed using a 3...
May 21, 2018: Journal of Computer Assisted Tomography
https://www.readbyqxmd.com/read/29771186/cross-modal-plasticity-among-sensory-networks-in-neuromyelitis-optica-spectrum-disorders
#8
Maria Assunta Rocca, Filippo Savoldi, Paola Valsasina, Marta Radaelli, Paolo Preziosa, Giancarlo Comi, Andrea Falini, Massimo Filippi
OBJECTIVE: To explore resting-state (RS) functional connectivity (FC) of the main sensory/motor networks of patients with neuromyelitis optica spectrum disorders (NMOSDs), clinically isolated optic neuritis (ON), and myelitis. METHODS: Clinical evaluation and RS fMRI were obtained from 28 NMOSD, 11 recurrent ON, and 12 recurrent myelitis patients and 30 healthy controls. Between-group RS FC comparisons and correlations with motor performance were assessed (SPM12) on the main sensory/motor RS networks (RSNs) identified by independent component analysis...
May 1, 2018: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/29769526/a-whole-genome-sequence-study-identifies-genetic-risk-factors-for-neuromyelitis-optica
#9
Karol Estrada, Christopher W Whelan, Fengmei Zhao, Paola Bronson, Robert E Handsaker, Chao Sun, John P Carulli, Tim Harris, Richard M Ransohoff, Steven A McCarroll, Aaron G Day-Williams, Benjamin M Greenberg, Daniel G MacArthur
Neuromyelitis optica (NMO) is a rare autoimmune disease that affects the optic nerve and spinal cord. Most NMO patients ( > 70%) are seropositive for circulating autoantibodies against aquaporin 4 (NMO-IgG+). Here, we meta-analyze whole-genome sequences from 86 NMO cases and 460 controls with genome-wide SNP array from 129 NMO cases and 784 controls to test for association with SNPs and copy number variation (total N = 215 NMO cases, 1244 controls). We identify two independent signals in the major histocompatibility complex (MHC) region associated with NMO-IgG+, one of which may be explained by structural variation in the complement component 4 genes...
May 16, 2018: Nature Communications
https://www.readbyqxmd.com/read/29766684/neuromyelitis-optica-review-and-utility-of-testing-aquaporin-4-antibody-in-typical-optic-neuritis
#10
Meagan Seay, Janet C Rucker
Neuromyelitis optica (NMO) is an autoimmune, inflammatory demyelinating disorder often leading to severe vision impairment and disability. The discovery of a diagnostic biomarker, the aquaporin-4 antibody (AQP4-IgG), transformed the clinical diagnosis and treatment of NMO and broadened the spectrum of disease [NMO spectrum disorders (NMOSD)]. Though the antibody is highly sensitive and specific to NMOSD, routine testing in patients with typical optic neuritis is considered controversial. This article will provide a brief review of NMOSD and highlight the pros and cons of routine testing in typical optic neuritis...
May 16, 2018: Asia-Pacific Journal of Ophthalmology
https://www.readbyqxmd.com/read/29750948/altered-macular-microvasculature-in-neuromyelitis-optica-spectrum-disorders
#11
William Robert Kwapong, Chenlei Peng, Zhiyong He, Xiran Zhuang, Meixiao Shen, Fan Lu
PURPOSE: To evaluate macular microvascular changes in neuromyelitis optica spectrum disorders (NMOSD) by using optical coherence tomography angiography (OCT-A) and investigate their correlations with neuroaxonal structural damage evaluated with Spectral domain OCT (SD-OCT). DESIGN: Cross-sectional study. METHODS: Twenty eyes of 20 patients with NMOSD and 21 eyes from 21 healthy controls were enrolled. OCT-A was used to obtain microvascular network images of the whole, superficial, and deep retinal capillary plexuses (WRCP, SRCP, and DRCP) in a 3-mm diameter area around the macula...
May 8, 2018: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/29747362/-research-progression-of-animal-models-of-idiopathic-optic-neuritis
#12
Y X Zhang, H Yang
Idiopathic optic neuritis (ON) is an idiopathic inflammatory disease of the optic nerve. The two most common types of ON are multiple sclerosis related ON and neuromyelitis optica related ON. But so far the precise mechanisms of different types of ON have been unclear and treatments are not satisfactory. Animal disease models play critical roles in the disease mechanism research. On the other hand, with more understanding about the etiology and mechanism of ON, new models mimicking different types of ON are under development...
April 11, 2018: [Zhonghua Yan Ke za Zhi] Chinese Journal of Ophthalmology
https://www.readbyqxmd.com/read/29724765/imaging-differences-between-neuromyelitis-optica-spectrum-disorders-and-multiple-sclerosis-a-multi-institutional-study-in-japan
#13
H Tatekawa, S Sakamoto, M Hori, Y Kaichi, A Kunimatsu, K Akazawa, T Miyasaka, H Oba, T Okubo, K Hasuo, K Yamada, T Taoka, S Doishita, T Shimono, Y Miki
BACKGROUND AND PURPOSE: Both clinical and imaging criteria must be met to diagnose neuromyelitis optica spectrum disorders and multiple sclerosis. However, neuromyelitis optica spectrum disorders are often misdiagnosed as MS because of an overlap in MR imaging features. The purpose of this study was to confirm imaging differences between neuromyelitis optica spectrum disorders and MS with visually detailed quantitative analyses of large-sample data. MATERIALS AND METHODS: We retrospectively examined 89 consecutive patients with neuromyelitis optica spectrum disorders (median age, 51 years; range, 16-85 years; females, 77; aquaporin 4 immunoglobulin G-positive, 93%) and 89 with MS (median age, 36 years; range, 18-67 years; females, 68; relapsing-remitting MS, 89%; primary-progressive MS, 7%; secondary-progressive MS, 2%) from 9 institutions across Japan (April 2008 to December 2012)...
May 3, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29724224/mog-encephalomyelitis-international-recommendations-on-diagnosis-and-antibody-testing
#14
REVIEW
S Jarius, F Paul, O Aktas, N Asgari, R C Dale, J de Seze, D Franciotta, K Fujihara, A Jacob, H J Kim, I Kleiter, T Kümpfel, M Levy, J Palace, K Ruprecht, A Saiz, C Trebst, B G Weinshenker, B Wildemann
Over the past few years, new-generation cell-based assays have demonstrated a robust association of autoantibodies to full-length human myelin oligodendrocyte glycoprotein (MOG-IgG) with (mostly recurrent) optic neuritis, myelitis and brainstem encephalitis, as well as with acute disseminated encephalomyelitis (ADEM)-like presentations. Most experts now consider MOG-IgG-associated encephalomyelitis (MOG-EM) a disease entity in its own right, immunopathogenetically distinct from both classic multiple sclerosis (MS) and aquaporin-4 (AQP4)-IgG-positive neuromyelitis optica spectrum disorders (NMOSD)...
May 3, 2018: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29721683/pediatric-neuromyelitis-optica-spectrum-disorders
#15
REVIEW
Grace Y Gombolay, Tanuja Chitnis
PURPOSE OF REVIEW: Neuromyelitis optica spectrum disorders (NMOSDs) are a group of inflammatory and demyelinating disorders of the central nervous system that can occur in children and adults. The classic presentation of NMOSD is characterized by optic neuritis and transverse myelitis, but other presentations are also recognized, expanding the disease as NMO spectrum disorders. The purpose of this review is to discuss the clinical features, along with management and treatment options, including potential future therapeutic options, in pediatric NMOSD...
May 2, 2018: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/29716388/alice-in-wonderland-syndrome-secondary-to-optical-neuromyelitis-caused-by-varicella
#16
Alisson Paulino Trevizol, Raphael Cerqueira, Elisa Brietzke, Quirino Cordeiro
No abstract text is available yet for this article.
April 1, 2018: Australian and New Zealand Journal of Psychiatry
https://www.readbyqxmd.com/read/29709797/trident-sign-trumps-aquaporin-4-igg-elisa-in-diagnostic-value-in-a-case-of-longitudinally-extensive-transverse-myelitis
#17
Evan A Jolliffe, B Mark Keegan, Eoin P Flanagan
Longitudinally-extensive T2-hyperintense spinal cord lesions (≥3 vertebral segments) are associated with neuromyelitis optical spectrum disorder but occur with other disorders including spinal cord sarcoidosis. When linear dorsal subpial enhancement is accompanied by central cord/canal enhancement the axial post-gadolinium sequences may reveal a "trident" pattern that has previously been shown to be strongly suggestive of spinal cord sarcoidosis. We report a case in which the patient was initially diagnosed with neuromyelitis optical spectrum disorder, but where the "trident" sign ultimately led to the correct diagnosis of spinal cord sarcoidosis...
April 21, 2018: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29692631/pediatric-acquired-demyelinating-syndromes-a-nationwide-validation-study-of-the-danish-national-patient-register
#18
Magnus Spangsberg Boesen, Melinda Magyari, Alfred Peter Born, Lau Caspar Thygesen
Objective: To validate the Danish National Patient Register's (NPR) diagnoses of pediatric acquired demyelinating syndromes (ADS) including multiple sclerosis (MS). Study design and setting: We identified ADS diagnostic groups using International Classification of Diseases (ICD) codes and reviewed medical records to validate the NPR diagnoses during 2008-2015. Results: Among 409 children in the study, 184 children had a validated and final ADS diagnosis after reviewing medical records as follows: optic neuritis (ON; n=46), transverse myelitis (TM; n=16), acute disseminated encephalomyelitis (ADEM; n=50), clinically isolated syndrome (CIS) including dissemination in space (CIS [DIS]) but not dissemination in time (n=6), neuromyelitis optica spectrum disorder (NMOsd; n=5), and MS (n=61)...
2018: Clinical Epidemiology
https://www.readbyqxmd.com/read/29682349/multiple-sclerosis-patients-with-markedly-low-intrathecal-antibody-response-in-sri-lanka
#19
S M K Gamage, I Wijeweera, S B Adikari, Katharina Fink, Jan Hillert, Anna Fogdell-Hahn, H M A Sominanda
Multiple sclerosis (MS) is a heterogeneous disease which is poorly studied in Asia, where the disease is known to be rare with significant differences in clinical and radiological presentations and intrathecal antibody response. Therefore the objective of this study was to determine clinical presentation, radiological and neurophysiological characteristics, and oligoclonal band status in Sri Lankan MS patients, following careful exclusion of patients with neuromyelitis optica spectrum disorders and other conditions mimicking multiple sclerosis...
2018: Multiple Sclerosis International
https://www.readbyqxmd.com/read/29676196/contiguous-and-symmetrical-disease-expansion-and-massive-necrosis-of-the-cerebral-white-matter-in-a-patient-with-neuromyelitis-optica
#20
Chiho Ishida, Tokuhei Ikeda, Kiyonobu Komai, Kazuya Takahashi, Moeko Noguchi-Shinohara, Masahito Yamada
The present report discusses the case of a woman with neuromyelitis optica (NMO) who exhibited bilateral optic neuritis, longitudinally extensive myelitis, serum anti-aquaporin-4 antibodies, and a unique pattern of white matter involvement. The disease duration was 26 years, and the patient died at the age of 65 years. Sequential magnetic resonance images obtained during the last 6 years of life revealed leukoencephalopathy-like lesions extending symmetrically and contiguously from the periventricular regions, which had begun to transform into multiple cavities with semi-annular partitions...
April 1, 2018: Multiple Sclerosis: Clinical and Laboratory Research
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