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https://www.readbyqxmd.com/read/28226075/neuromyelitis-optica-phenotypic-characteristics-in-a-brazilian-case-series
#1
Maria Cristina Del Negro, Patricia Beatriz Christino Marinho, Regina Maria Papais-Alvarenga
Objective: To describe the characteristics of 34 Brazilian NMO patients. To evaluate the contribution of the 2015 criteria to the diagnosis of NMO spectrum disorders (NMOSD) in 40 patients with longitudinal extensive transverse myelitis (LEMT). Methods: This is a retrospective, descriptive and analytic study. Results: Among NMO patients, there was a predominance of women, with onset in the fourth decade of life, and AQP4-IgG seropositivity in 73...
February 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/28212662/marked-central-nervous-system-pathology-in-cd59-knockout-rats-following-passive-transfer-of-neuromyelitis-optica-immunoglobulin-g
#2
Xiaoming Yao, Alan S Verkman
Neuromyelitis optica spectrum disorders (herein called NMO) is an inflammatory demyelinating disease of the central nervous system in which pathogenesis involves complement-dependent cytotoxicity (CDC) produced by immunoglobulin G autoantibodies targeting aquaporin-4 (AQP4-IgG) on astrocytes. We reported evidence previously, using CD59(-/-) mice, that the membrane-associated complement inhibitor CD59 modulates CDC in NMO (Zhang and Verkman, J. Autoimmun. 53:67-77, 2014). Motivated by the observation that rats, unlike mice, have human-like complement activity, here we generated CD59(-/-) rats to investigate the role of CD59 in NMO and to create NMO pathology by passive transfer of AQP4-IgG under conditions in which minimal pathology is produced in normal rats...
February 17, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28203460/the-prevalence-of-anti-aquaporin-4-antibody-in-patients-with-idiopathic-inflammatory-demyelinating-diseases-presented-to-a-tertiary-hospital-in-malaysia-presentation-and-prognosis
#3
S Abdullah, W F Wong, C T Tan
Background. There have been inconsistent reports on the prevalence and pathogenicity of anti-Aquaporin 4 (AQP4) in patients presented with idiopathic inflammatory demyelinating diseases (IIDDs). Objective. To estimate the prevalence of anti-AQP4 antibody in patients with IIDDs presented to University Malaya Medical Centre in terms of patients' clinical and radiological presentations and prognoses. Methods. Retrospective data review of IIDDs patients presented from 2005 to 2015. Patients were classified into classical multiple sclerosis (CMS), opticospinal (OS) presentation, optic neuritis (ON), transverse myelitis (TM), brainstem syndrome (BS), and tumefactive MS...
2017: Multiple Sclerosis International
https://www.readbyqxmd.com/read/28184993/pathogenic-implications-of-cerebrospinal-fluid-barrier-pathology-in-neuromyelitis-optica
#4
Yong Guo, Stephen D Weigand, Bogdan F Popescu, Vanda A Lennon, Joseph E Parisi, Sean J Pittock, Natalie E Parks, Stacey L Clardy, Charles L Howe, Claudia F Lucchinetti
Pathogenic autoantibodies associated with neuromyelitis optica (NMO) induce disease by targeting aquaporin-4 (AQP4) water channels enriched on astrocytic endfeet at blood-brain interfaces. AQP4 is also expressed at cerebrospinal fluid (CSF)-brain interfaces, such as the pial glia limitans and the ependyma and at the choroid plexus blood-CSF barrier. However, little is known regarding pathology at these sites in NMO. Therefore, we evaluated AQP4 expression, microglial reactivity, and complement deposition at pial and ependymal surfaces and in the fourth ventricle choroid plexus in 23 autopsy cases with clinically and/or pathologically confirmed NMO or NMO spectrum disorder...
February 9, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28174515/upregulation-of-bcl-2-and-its-promoter-signals-in-cd4-t-cells-during-neuromyelitis-optica-remission
#5
Tao Yang, Su Wang, Xiao Yang, Qi Zheng, Lei Wang, Qian Li, Mingyan Wei, Zongpan Du, Yongping Fan
The homeostatic balance between production and elimination of CD4+ T cells in peripheral blood plays an important role in patients with neuromyelitis optica (NMO). The objective of the present study was to evaluate the anti-apoptosis genes Bcl-2 and its promoter signal (nuclear factor kappa-light-chain-enhancer of activated B cells, NFκB) in CD4+ T cells. Healthy subjects (HS, n = 25) and patients with multiple sclerosis (MS) (n = 25) and NMO (n = 30) in remission were consecutively enrolled in this prospective study between May and December 2015...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28173728/metabolic-changes-in-normal-appearing-white-matter-in-patients-with-neuromyelitis-optica-and-multiple-sclerosis-a-comparative-magnetic-resonance-spectroscopy-study
#6
Yunyun Duan, Zheng Liu, Yaou Liu, Jing Huang, Zhuoqiong Ren, Zheng Sun, Hai Chen, Huiqing Dong, Jing Ye, Kuncheng Li
Background Previous studies with a small sample size have not reported metabolic changes in neuromyelitis optica (NMO). Metabolic changes, such as decreased N-acetylaspartate (NAA), are well-established in patients with multiple sclerosis (MS). It remains unknown whether different patterns of metabolic changes occur in NMO and MS. Purpose To investigate the metabolic changes in normal-appearing white matter (NAWM) in NMO, compared with MS patients and healthy controls (HC), and correlate these changes with clinical disability...
January 1, 2017: Acta Radiologica
https://www.readbyqxmd.com/read/28159705/demyelinating-syndrome-in-sle-encompasses-different-subtypes-do-we-need-new-classification-criteria-pooled-results-from-systematic-literature-review-and-monocentric-cohort-analysis
#7
Matteo Piga, Elisabetta Chessa, Maria Teresa Peltz, Alberto Floris, Alessandro Mathieu, Alberto Cauli
OBJECTIVE: To describe features of demyelinating syndrome (DS) in systemic lupus erythematosus (SLE). METHODS: A systematic review using a combination of Mesh terms in PubMed and a retrospective analysis of 343 adult patients with SLE were carried out to identify patients with DS. Retrieved cases were classified as affected with DS according to 1999 ACR nomenclature and attributed to SLE by applying the 2015 algorithm. DS defined according to the clinical but not temporal 1999 ACR criteria was classified as clinically isolated syndrome (CIS)...
January 31, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28157198/subregional-structural-and-connectivity-damage-in-the-visual-cortex-in-neuromyelitis-optica
#8
Huanhuan Cai, Jiajia Zhu, Ningnannan Zhang, Qiuhui Wang, Chao Zhang, Chunsheng Yang, Jie Sun, Xianting Sun, Li Yang, Chunshui Yu
Patients with neuromyelitis optica (NMO) have shown structural and functional impairments in the visual cortex. We aimed to characterize subregional grey matter volume (GMV) and resting-state functional connectivity (rsFC) changes in the visual cortex in NMO. Thirty-seven NMO patients and forty-two controls underwent structural and functional MRI scans. The GMV and rsFC of each visual subregion were compared between the groups. Compared with controls, NMO patients had GMV reductions in the bilateral V1, V2, V3d, VP, and LO and in the left V3A...
February 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28149102/magnetic-resonance-imaging-brain-findings-in-a-case-of-aquaporin-4-antibody-positive-neuromyelitis-optica-spectrum-disorder-presenting-with-intractable-vomiting-and-hiccups
#9
Prerna Garg, Muthusubramanian Rajasekaran, Salil Pandey, Gnanashanmugam Gurusamy, Devanand Balalakshmoji, Rajakumar Rathinasamy
Neuromyelitisoptica (NMO) and multiple sclerosis (MS) were once considered to be differing manifestation of same auto immune disease, NMO predominantly involving the optic nerve and cord. Now with discovery of NMO antibody the concept has changed and a spectrum of disorders with lesions in brain has been identified. Occasionally, brain may be the first or the only site of involvement in these disorders hence it is essential to be aware of this spectrum. The brain lesions in NMO/NMOSD may be located in characteristic regions and present with symptoms mimicking non neurological disease...
January 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28131216/monitoring-cd27-memory-b-cells-in-neuromyelitis-optica-spectrum-disorders-patients-treated-with-rituximab-results-from-a-bicentric-study
#10
M Cohen, G Romero, J Bas, M Ticchioni, M Rosenthal, R Lacroix, C Brunet, A Rico, J Pelletier, B Audoin, C Lebrun
BACKGROUND: Rituximab (RTX) is increasingly used in the treatment of neuromyelitis optica spectrum disorder (NMO-SD). Administration regimen is not consensual as there is no reliable biomarker of RTX efficacy. In most cases, after induction, RTX is administered systematically every 6months. OBJECTIVE: To assess efficacy and safety of a maintenance regimen based on CD19+ CD27+ memory B-cell (mBc) detection. METHODS: We conducted a study in two French centers, including patients with NMO-SD who received an induction therapy with RTX...
February 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28129686/a-novel-approach-to-discriminate-subgroups-in-multiple-sclerosis
#11
Mehrdad Farrokhi, Zahra Saadatpour, Elyas Fadaee, Leila Saadatpour, Ali Rezaei, Pedram Moeini, Ali Amani Beni
Multiple sclerosis (MS) is an autoimmune disease of central nervous system. Since different types of immune cells are involved in MS pathogenesis, in this study we aimed to evaluate serum levels of several immunological components including soluble CD4 (sCD4), sCD8, sCD163, and immunoglobulins as markers of activity of T-cells, macrophages, and B-cells in different types of MS. Serum levels of sCD4, sCD8, and sCD163 of patients with relapsing-remitting MS (RRMS, n=61), primary progressive MS (PRMS, n=31), secondary progressive MS (SPMS, n=31), clinical isolated syndrome (CIS, n=31) and neuromyelitis optica (NMO, n=31), and healthy controls (n=49) were measured using enzyme-linked immunosorbent assay (ELISA)...
December 2016: Iranian Journal of Allergy, Asthma, and Immunology
https://www.readbyqxmd.com/read/28109254/letm-presented-with-causalgia-and-ensued-by-sudden-death
#12
Rana Alnasser Alsukhni, Yasmin Aboras, Ziena Jriekh, Mahmoud Almalla, Ahmad Sheikh El-Kahwateya
BACKGROUND: Longitudinally Extensive Transverse Myelitis LETM is a specific pattern of myelitis wherein at least three continuous vertebral segments are involved. Characteristically, it is a defining feature of neuromyelitis optica NMO. However, it is described in many other etiologies. CASE PRESENTATION: We present a case of 60 year old male who presented with symptoms and signs of regional sympathetic dystrophy RSD followed by symptoms of myelitis. Spinal cord MRI revealed cervical LETM extending to the brainstem...
January 21, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28106565/neuromyelitis-optica-in-a-patient-from-family-with-both-myotonic-dystrophy-type%C3%A2-1-and-2
#13
V Rakocevic-Stojanovic, S Peric, I Dujmovic, J Drulovic, J Pesovic, D Savic-Pavicevic
INTRODUCTION: The aim of this study was to present a family co-segregating myotonic dystrophy type 1 (DM1) and 2 (DM2), and one member affected with neuromyelitis optica (NMO). CASE REPORT: Index case underwent cataract surgery at age 39. Although she had no muscle symptoms, genetic testing revealed a DM2 mutation and a DM1 protomutation. The patient noticed difficulties in climbing stairs at age 47. Clinical examination showed mild muscle weakness, calf hypertrophy, mild myotonia and several multisystem signs...
January 20, 2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/28092989/therapeutic-complement-inhibition-a-promising-approach-for-treatment-of-neuroimmunological-diseases
#14
Kjara S Pilch, Peter Spaeth, Yuki Nobuhiro, Benjamin R Wakerley
Autoimmunity is an important cause of disease both in the central and peripheral nervous systems. Aetiologies and clinical manifestations are complex and heterogeneous. Inappropriate control of complement activation at inappropriate sites has been recognized as a major determinant in several neurological conditions, including Guillain-Barré syndrome and neuromyelitis optica. In each case pathogenesis is thought to be associated with generation of autoantibodies which upon binding guide activation of the complement system to self-tissue...
January 17, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/28071581/role-of-aqp4-antibody-serostatus-and-its-prediction-of-visual-outcome-in-neuromyelitis-optica-a-systematic-review-and-meta-analysis
#15
Nan Lin, Qing Liu, Xiaoyu Wang, Jianmei Ma, Yuyuan Li
: Backgroud: Neuromyelitis optica (NMO) is an autoimmune inflammatory disorder, which is characterized by severe attacks of optic neuritis and myelitis. Antibodies (Ab) to aquaporin-4 (AQP4) (or NMO-IgG) as a serological biomarker of NMO have been widespread used. Nevertheless, some NMO patients remain seronegative for AQP4-Ab and/or have no detected optic nerve involvement. In addition, no consensus exists on the association between AQP4-Ab serostatus and visual outcome in NMO. To drive a more precise estimate of this postulated relationship, a meta-analysis was performed based on existing relevant studies...
January 10, 2017: Protein and Peptide Letters
https://www.readbyqxmd.com/read/28060988/-neuromyelitis-optica-presenting-concomitantly-with-systemic-lupus-erythematosus-report-of-one-case
#16
Felipe Suárez H, Daniela Urrutia E, Felipe Canales P, Camila Gutiérrez O
Neuromyelitis optica (NMO) is a severe demyelinating disease of the central nervous system, which preferentially attacks the optic nerve and spinal cord. It is associated with antibodies against aquaporin 4. Morbidity and mortality are higher than in multiple sclerosis and its treatment focuses on immunosuppressive drugs. Immunomodulators are contraindicated. We report a previously healthy 35-year-old man, presenting with NMO concomitantly with systemic lupus erythematosus. His evolution was torpid with three outbreaks in the 10 months after the diagnosis, requiring a first-line therapy with methylprednisolone and cyclophosphamide and then a second-line therapy with rituximab...
September 2016: Revista Médica de Chile
https://www.readbyqxmd.com/read/28058717/deletional-tolerance-prevents-aqp4-directed-autoimmunity-in-mice
#17
Anna-Lena Vogel, Benjamin Knier, Katja Lammens, Sudhakar Reddy Kalluri, Tanja Kuhlmann, Jeffrey L Bennett, Thomas Korn
Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system (CNS) mediated by antibodies to the water channel protein AQP4 expressed in astrocytes. The contribution of AQP4-specific T cells to the class switch recombination of pathogenic AQP4-specific antibodies and the inflammation of the blood-brain barrier is incompletely understood, as immunogenic naturally processed T-cell epitopes of AQP4 are unknown. By immunizing Aqp4(-/-) mice with full-length murine AQP4 protein followed by recall with overlapping peptides, we here identify AQP4(201-220) as the major immunogenic IA(b) -restricted epitope of AQP4...
January 5, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28057643/retinal-segmented-layers-with-strong-aquaporin-4-expression-suffered-more-injuries-in-neuromyelitis-optica-spectrum-disorders-compared-with-optic-neuritis-with-aquaporin-4-antibody-seronegativity-detected-by-optical-coherence-tomography
#18
Chun Xia Peng, Hong Yang Li, Wei Wang, Jun Qing Wang, Lei Wang, Quan Gang Xu, Shan Shan Cao, Huan Fen Zhou, Shuo Zhao, Shi Hui Wei
PURPOSE: To evaluate retinal segmented layer alterations in optic neuritis (ON) in an AQP4-Ab seropositive (AQP4-Ab+/ON) cohort and in neuromyelitis optica (NMO) with ON eyes (NMO-ON) compared with an AQP4-Ab seronegative ON (AQP4-Ab-/ON) cohort using optical coherence tomography (OCT). METHODS: We recruited 109 patients with ON (161 eyes) and 47 healthy controls. All patients with ON were subdivided into three subcohorts: 37 patients (54 eyes) with AQP4-Ab+/ON, 45 patients (65 eyes) with AQP4-Ab-/ON and 27 patients (42 eyes) with NMO-ON...
January 5, 2017: British Journal of Ophthalmology
https://www.readbyqxmd.com/read/28056870/anti-n-methyl-d-aspartate-receptor-nmdar-antibody-encephalitis-presents-in-atypical-types-and-coexists-with-neuromyelitis-optica-spectrum-disorder-or-neurosyphilis
#19
Kaiyu Qin, Wenqing Wu, Yuming Huang, Dongmei Xu, Lei Zhang, Bowen Zheng, Meijuan Jiang, Cheng Kou, Junhua Gao, Wurong Li, Jinglin Zhang, Sumei Wang, Yanfei Luan, Chaoling Yan, Dan Xu, Xinmei Zheng
BACKGROUND: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a clinically heterogeneous disorder characterized by epileptic seizures, psychosis, dyskinesia, consciousness impairments, and autonomic instability. Symptoms are always various. Sometimes it presents in milder or incomplete forms. We report 4 cases of anti-NMDAR encephalitis with incomplete forms, 3 cases of which were accompanied by neuromyelitis optica spectrum disorder or neurosyphilis respectively. CASE PRESENTATION: A 33-year-old man presented with dysarthria, movement disorder and occasional seizures...
January 5, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28054001/aquaporin-4-antibody-titration-in-nmo-patients-treated-with-rituximab-a-retrospective-study
#20
Paola Valentino, Fabiana Marnetto, Letizia Granieri, Marco Capobianco, Antonio Bertolotto
OBJECTIVE: We undertook an observational retrospective study to investigate the usefulness of aquaporin-4 (AQP4) antibodies (Ab) titration in the management of patients with neuromyelitis optica (NMO) treated with rituximab (RTX) by studying (1) the correlation between AQP4-Ab titer and disease activity, (2) the influence of RTX on antibody levels, and (3) the association between AQP4-Ab levels and responsiveness to RTX. METHODS: A cell-based assay was used for AQP4-Ab titration in 322 serum samples from 7 patients with NMO treated with RTX (median follow-up 65 months), according to a treatment-to-target approach...
March 2017: Neurology® Neuroimmunology & Neuroinflammation
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