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Neuromyelitis optica

Yuxin Zhang, Yiqin Bao, Wei Qiu, Lisheng Peng, Ling Fang, Ying Xu, Hui Yang
Neuromyelitis optica spectrum disorders (NMOSD) are a group of autoimmune astrocytopathies in the central nervous system, which are mainly caused by immunoglobulin G (IgG) against astrocyte water channel aquaporin-4 (AQP4). In this study, we aimed to establish a model of NMOSD-related optic neuritis (NMOSD-ON) and to evaluate the progressive changes of the optic nerve and visual function. AQP4 IgG-positive serum from NMOSD patients was injected into the subarachnoid space of the rat optic nerve to induce the NMOSD-ON model (AQP4 + group), and healthy serum was injected as the control...
June 15, 2018: Experimental Eye Research
Georgina Flórez-Grau, Irati Zubizarreta, Raquel Cabezón, Pablo Villoslada, Daniel Benitez-Ribas
The identification of activated T-lymphocytes restricted to myelin-derived immunogenic peptides in multiple sclerosis (MS) and aquaporin-4 water channel in neuromyelitis optica (NMO) in the blood of patients opened the possibility for developing highly selective and disease-specific therapeutic approaches. Antigen presenting cells and in particular dendritic cells (DCs) represent a strategy to inhibit pro-inflammatory T helper cells. DCs are located in peripheral and lymphoid tissues and are essential for homeostasis of T cell-dependent immune responses...
2018: Frontiers in Immunology
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
Sarah Kariv, Kathleen Bateman
A 17-year-old girl presented with intractable vomiting due to area postrema involvement in the first presentation of seronegative neuromyelitis optica (NMO). During the course of her illness, she developed mild hyponatremia, and magnetic resonance imaging revealed abnormalities consistent with the co-occurrence of osmotic demyelination syndrome (ODS). This combination of imaging features is novel, and this case expands the spectrum of brain abnormalities seen in NMO and NMO spectrum disorders. It was suspected that NMO may predispose to ODS by causing astrocyte dysfunction involving aquaporin 4 water channels, which are implicated in both conditions...
May 2018: International Journal of MS Care
Maureen A Mealy, Remi A Kessler, Zoe Rimler, Allyson Reid, Lauren Totonis, Gary Cutter, Ilya Kister, Michael Levy
No abstract text is available yet for this article.
July 2018: Neurology® Neuroimmunology & Neuroinflammation
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
O Aktas, M P Wattjes, M Stangel, H-P Hartung
Multiple sclerosis (MS) is the most common chronic autoimmune disorder of the central nervous system (CNS) largely affecting young adults. The diagnosis of MS is based on two pillars: 1) detection of the spatial and temporal dissemination of focal neurological deficits and 2) exclusion of important differential diagnoses. The current revision of the diagnostic criteria (McDonald 2017) also follows these principles, takes new data on magnetic resonance imaging (MRI) into account and reintroduces the role of cerebrospinal fluid (CSF) diagnostics for relapsing-remitting forms...
June 6, 2018: Der Nervenarzt
Kimihiko Kaneko, Douglas Kazutoshi Sato, Ichiro Nakashima, Ryo Ogawa, Tetsuya Akaishi, Yoshiki Takai, Shuhei Nishiyama, Toshiyuki Takahashi, Tatsuro Misu, Hiroshi Kuroda, Satoru Tanaka, Kyoichi Nomura, Yuji Hashimoto, Dagoberto Callegaro, Lawrence Steinman, Kazuo Fujihara, Masashi Aoki
OBJECTIVE: To evaluate cerebrospinal fluid (CSF) cytokine profiles in myelin oligodendrocyte glycoprotein IgG-positive (MOG-IgG+) disease in adult and paediatric patients. METHODS: In this cross-sectional study, we measured 27 cytokines in the CSF of MOG-IgG+ disease in acute phase before treatment (n=29). The data were directly compared with those in aquaporin-4 antibody-positive (AQP4-IgG+) neuromyelitis optica spectrum disorder (NMOSD) (n=20), multiple sclerosis (MS) (n=20) and non-inflammatory controls (n=14)...
June 6, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
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
Sara Gil-Perotin, Jéssica Castillo-Villalba, Joan Carreres-Polo, Arantxa Navarré-Gimeno, Javier Mallada-Frechín, Francisco Pérez-Miralles, Francisco Gascón, Carmen Alcalá-Vicente, Laura Cubas-Nuñez, Bonaventura Casanova-Estruch
The clinical diagnosis of patients with autoantibodies directed to conformational myelin oligodendrocyte glycoprotein MOG-IgG, can be challenging because of atypical clinical presentation. MOG-IgG seropositivity has been reported in several demyelinating diseases, including relapsing opticospinal syndromes [in the neuromyelitis optica spectrum disorders (NMOSD) and less frequently, in multiple sclerosis (MS)], but it has rarely been associated with the progressive course of disease. To contribute to the characterization of MOG-related demyelination, we describe the case of a patient with progressive demyelinating opticospinal disease, IgG-oligoclonal bands (OCB), and serum MOG-IgG...
2018: Frontiers in Neurology
Inês Furtado, Guiomar Pinheiro, Ana Campar, Teresa Mendonça
Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder. Neuromyelitis optica (NMO) is an infrequent neuroinflammatory disorder, whose association with SLE remains rare. The authors report the case of an 18-year-old woman, with SLE refractory to multiple immunosuppressive therapies and novel biological agents. Under immunosuppressive therapy, the patient presented with transverse myelitis with contiguous spinal cord lesions and urinary incontinence, having been diagnosed with seropositive NMO, which was also proven to be refractory to common treatments...
June 4, 2018: BMJ Case Reports
Sara Gerhardt, M Hasan Mohajeri
In recent years evidence has emerged that neurodegenerative diseases (NDs) are strongly associated with the microbiome composition in the gut. Parkinson's disease (PD) is the most intensively studied neurodegenerative disease in this context. In this review, we performed a systematic evaluation of the published literature comparing changes in colonic microbiome in PD to the ones observed in other NDs including Alzheimer's disease (AD), multiple system atrophy (MSA), multiple sclerosis (MS), neuromyelitis optica (NMO) and amyotrophic lateral sclerosis (ALS)...
June 1, 2018: Nutrients
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
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
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
Maria Stavrou, Lucy Francis, Nomathamsanqa Tshuma, Klaus Schmierer
Rhomboencephalitis, at least in its acute phase, is often a severely disabling syndrome, and can be life threatening. A range of underlying conditions can lead to this clinical syndrome. Rapid diagnosis to initiate treatment early is key to a beneficial outcome. We report the case of a 22 year old Afro-Caribbean woman, who presented with a two -week history of walking difficulties, upper limb incoordination and slurred speech. Her brainstem function deteriorated at pace, and she developed hypersomnia. A broad diagnostic approach led to prophylactic treatment for the most common infectious causes...
May 23, 2018: BMJ Case Reports
Edgar Carnero Contentti, Vanessa Daccach Marques, Ibis Soto de Castillo, Verónica Tkachuk, Amilton Antunes Barreira, Elizabeth Armas, Edson Chiganer, Camila de Aquino Cruz, José Luis Di Pace, Javier Pablo Hryb, Carolina Lavigne Moreira, Carmen Lessa, Omaira Molina, Mónica Perassolo, Arnoldo Soto, Alejandro Caride
STUDY DESIGN: Multicenter retrospective study. OBJECTIVES: The aim was to determine the frequency and magnetic resonance imaging (MRI) features of short-segment transverse myelitis (STM) in patients with neuromyelitis optica spectrum disorders (NMOSD) during a myelitis attack. SETTING: Latin American diagnostic centres (Neuroimmunology Unit). A multicenter study from Argentina, Brazil and Venezuela was performed. METHODS: Seventy-six patients with NMOSD were included...
May 22, 2018: Spinal Cord
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
Maureen A Mealy, Lawrence J Cook, Florence Pache, Diego L Velez, Nadja Borisow, Daniel Becker, Jorge A Jimenez Arango, Friedemann Paul, Michael Levy
BACKGROUND: It is unknown if vaccines cause non-specific immune activation in patients with neuromyelitis optica spectrum disorder and no consensus on the use of vaccines exists for this population. We investigated the temporal association of vaccinations with relapses in patients with neuromyelitis optica spectrum disorder. METHODS: This is a multi-center retrospective analysis of patients with neuromyelitis optica spectrum disorder for whom immunization history and clinical records from disease onset were available...
May 7, 2018: Multiple Sclerosis and related Disorders
Sabrina Gmuca, Rui Xiao, Pamela F Weiss, Amy T Waldman, Jeffrey S Gerber
Background: Treatment algorithms for neuromyelitis optica spectrum disorder (NMOSD) vary, and sparse data exist regarding the impact of initial treatments on disease course. We aimed to determine whether administration of rituximab during first hospitalization reduces 1-year readmission rates. Methods: We conducted a retrospective cohort study of subjects with NMOSD using the Pediatric Health Information System database from 2005-2015. Subjects were ages 1 to 21 years who received glucocorticoids and an ICD-9-CM code indicating neuromyelitis optica (NMO) during first hospitalization...
April 2018: Multiple Sclerosis and Demyelinating Disorders
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