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https://www.readbyqxmd.com/read/28626410/neuromyelitis-optica-spectrum-disorder-coinciding-with-spinocerebellar-ataxia-type-31
#1
Yoshiaki Takahashi, Yasuhiro Manabe, Ryuta Morihara, Hisashi Narai, Toru Yamashita, Koji Abe
We report the unusual case of a 63-year-old man with spinocerebellar ataxia (SCA) type 31 who developed neuromyelitis optica spectrum disorder (NMOSD) 14 years after the onset of cerebellar symptoms. In addition to cerebellar atrophy, magnetic resonance imaging showed multiple high-intensity areas in the brain and a long thoracic cord lesion from Th1/2 to Th11. The combination of NMOSD and SCA31 is accidental. However, our case suggests that inflammatory processes could be involved in the pathogenesis of NMOSD and SCA31...
May 2017: Case Reports in Neurology
https://www.readbyqxmd.com/read/28615429/diagnostic-algorithm-for-relapsing-acquired-demyelinating-syndromes-in-children
#2
Yael Hacohen, Kshitij Mankad, W K Chong, Frederik Barkhof, Angela Vincent, Ming Lim, Evangeline Wassmer, Olga Ciccarelli, Cheryl Hemingway
OBJECTIVE: To establish whether children with relapsing acquired demyelinating syndromes (RDS) and myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) show distinctive clinical and radiologic features and to generate a diagnostic algorithm for the main RDS for clinical use. METHODS: A panel reviewed the clinical characteristics, MOG-Ab and aquaporin-4 (AQP4) Ab, intrathecal oligoclonal bands, and Epstein-Barr virus serology results of 110 children with RDS. A neuroradiologist blinded to the diagnosis scored the MRI scans...
June 14, 2017: Neurology
https://www.readbyqxmd.com/read/28608315/effectiveness-of-low-dose-of-rituximab-compared-with-azathioprine-in-chinese-patients-with-neuromyelitis-optica-an-over-2-year-follow-up-study
#3
Meini Zhang, Chuntao Zhang, Peng Bai, Huiru Xue, Guilian Wang
Neuromyelitis optical (NMO) and neuromyelitis optical spectrum disorder (NMOSD) are inflammatory autoimmune demyelination diseases affecting the central nervous system. We investigated the efficiency of low-dose rituximab treatment in 31 Chinese patients with NMO/NMOSD across a median period of 2.29 ± 0.97 years and azathioprine combined with corticosteroid treatment in 34 Chinese patients with NMO/NMOSD across a median period of 2.61 ± 0.94 years. Among the rituximab-treated patients, the mean Expanded Disability Status Scale (EDSS) was 5...
June 12, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/28601284/aquaporin-4-antibody-positive-neuromyelitis-optica-spectrum-disorder-associated-with-esophageal-cancer
#4
Tomoya Kon, Tatsuya Ueno, Chieko Suzuki, Jinichi Nunomura, Shohei Igarashi, Tsugumi Sato, Masahiko Tomiyama
Autoimmune diseases are sometimes associated with neoplasms. A 70-year-old Japanese woman with myelitis, seropositive for aquaporin-4 (AQP4) antibody, was diagnosed with neuromyelitis optica spectrum disorder (NMOSD); thereafter an esophageal squamous cell carcinoma was identified. Immunohistochemically, her cancer was anti-AQP4 antibody negative. Her symptoms, imaging findings and AQP4 titer markedly improved with corticosteroid and anti-cancer therapies. Although AQP4 may be a paraneoplastic antigen, paraneoplastic syndrome could not be definitively diagnosed in this case...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28601281/association-of-cd58-gene-polymorphisms-with-nmo-spectrum-disorders-in-a-han-chinese-population
#5
Ju Liu, Ziyan Shi, Zhiyun Lian, Hongxi Chen, Qin Zhang, Huiru Feng, Xiaohui Miao, Qin Du, Hongyu Zhou
This study aimed to perform a comprehensive assessment of the association between CD58 polymorphisms and the risk of neuromyelitis optica spectrum disorders (NMOSD) in a Han Chinese population. Nine single-nucleotide polymorphisms (SNPs) were genotyped in 230 NMOSD patients and 487 healthy controls. Five SNPs were significantly associated with an increased risk of NMOSD (rs2300747, rs1335532, rs56302466, rs1016140, and rs12044852). The haplotype TAGCCCAA significantly increased the risk of NMOSD, while TATTACGG reduced the risk...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28601277/late-onset-neuromyelitis-optica-mimicking-an-acute-stroke-in-an-elderly-patient
#6
Kushak Suchdev, Sara Razmjou, Praveen Venkatachalam, Omar A Khan, Wazim Mohamed, Mohammed S Ibrahim
Neuromyelitis optica spectrum disorder (NMOSD) was recently proposed as a unifying term for Neuromyelitis optica (NMO) and related syndromes to incorporate patients with NMO antibody but without the full clinical spectrum. NMO is a rare, demyelinating condition which predominantly affects females with a peak incidence in the third and fourth decade of life. We report a case of NMOSD in an elderly patient with extremely late onset (>80years) of disease. The patient presented with findings of sudden onset unilateral symptoms, which is extremely unusual for NMOSD...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28600575/altered-humoral-immunity-to-mycobacterial-antigens-in-japanese-patients-affected-by-inflammatory-demyelinating-diseases-of-the-central-nervous-system
#7
Davide Cossu, Kazumasa Yokoyama, Yuji Tomizawa, Eiichi Momotani, Nobutaka Hattori
Mycobacterium avium subsp. paratuberculosis (MAP) and Mycobacterium bovis (BCG) have been associated to several human autoimmune diseases such as multiple sclerosis (MS), but there are conflicting evidence on the issue. The objective of this study is to evaluate their role in Japanese patients affected by inflammatory demyelinating disorders of the central nervous system (IDDs). A total of 97 IDDs subjects including 51 MS and 46 neuromyelitis optica spectrum disorder (NMOSD) patients, and 34 healthy controls (HCs) were tested for the detection of IgG, IgM and IgA against mycobacterial antigens by indirect ELISA...
June 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28589164/cns-aquaporin-4-specific-b-cells-connect-with-multiple-b-cell-compartments-in-neuromyelitis-optica-spectrum-disorder
#8
Markus C Kowarik, David Astling, Christiane Gasperi, Scott Wemlinger, Hannah Schumann, Monika Dzieciatkowska, Alanna M Ritchie, Bernhard Hemmer, Gregory P Owens, Jeffrey L Bennett
OBJECTIVES: Neuromyelitis optica spectrum disorder (NMOSD) is a severe inflammatory disorder of the central nervous system (CNS) targeted against aquaporin-4 (AQP4). The origin and trafficking of AQP4-specific B cells in NMOSD remains unknown. METHODS: Peripheral (n = 7) and splenic B cells (n = 1) recovered from seven NMOSD patients were sorted into plasmablasts, naïve, memory, and CD27-IgD- double negative (DN) B cells, and variable heavy chain (VH) transcriptome sequences were generated by deep sequencing...
June 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28584663/clinical-pitfall-false-positive-aquaporin-4-igg-leading-to-misdiagnosis-of-neuromyelitis-optica-spectrum-disorder-in-patient-with-spinal-arteriovenous-fistula
#9
Suradech Suthiphosuwan, Jiwon Oh, Aditya Bharatha
INTRODUCTION: We report the cases of a 68-year-old male with a filum terminale arteriovenous fistula (AVF) who was initially misdiagnosed with neuromyelitis optica spectrum disorder (NMOSD) based on imaging findings and false-positive aquaporin-4 IgG (AQP4-IgG). CASE PRESENTATION: A 68-year-old male presented with slowly progressive weakness and numbness in his bilateral lower extremities. He was initially diagnosed with NMOSD and treated with immunosuppressive therapy based on findings of extensive spinal cord edema on magnetic resonance imaging (MRI) and initial negative spinal angiography as well as positive AQP4-IgG...
2017: Spinal Cord Series and Cases
https://www.readbyqxmd.com/read/28580873/aquaporin-4-igg-positive-neuromyelitis-optica-spectrum-disorder-with-recurrent-short-partial-transverse-myelitis-and-favorable-prognosis-two-new-cases
#10
Jinhua Zhang, Fang Liu, Yiqi Wang, Ying Yang, Yuehong Huang, Hongchen Zhao, Yong Bi, Tianming Shi, Shunyuan Guo, Meiping Wang
Understanding the characteristics of neuromyelitis optica spectrum disorder (NMOSD) with recurrent short partial transverse myelitis (SPTM), which is very rare, contributes to the differential diagnosis of multiple sclerosis (MS). We present two Chinese aquaporin-4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD cases who had at least twice SPTM during 4 and 6 years of follow-up, respectively. Their SPTMs have been mild and responded well to corticosteroids just like in the case of MS. The findings highlight the need of searching for serum AQP4-IgG (cell-based assay strongly recommended) in patients with recurrent SPTM and suggest that those patients may have a mild acute attack phase and favorable long-term prognosis...
April 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28572277/immunotherapies-in-neuromyelitis-optica-spectrum-disorder-efficacy-and-predictors-of-response
#11
Jan-Patrick Stellmann, Markus Krumbholz, Tim Friede, Anna Gahlen, Nadja Borisow, Katrin Fischer, Kerstin Hellwig, Florence Pache, Klemens Ruprecht, Joachim Havla, Tania Kümpfel, Orhan Aktas, Hans-Peter Hartung, Marius Ringelstein, Christian Geis, Christoph Kleinschnitz, Achim Berthele, Bernhard Hemmer, Klemens Angstwurm, Kim Lea Young, Simon Schuster, Martin Stangel, Florian Lauda, Hayrettin Tumani, Christoph Mayer, Lena Zeltner, Ulf Ziemann, Ralf Andreas Linker, Matthias Schwab, Martin Marziniak, Florian Then Bergh, Ulrich Hofstadt-van Oy, Oliver Neuhaus, Uwe Zettl, Jürgen Faiss, Brigitte Wildemann, Friedemann Paul, Sven Jarius, Corinna Trebst, Ingo Kleiter
OBJECTIVE: To analyse predictors for relapses and number of attacks under different immunotherapies in patients with neuromyelitis optica spectrum disorder (NMOSD). DESIGN: This is a retrospective cohort study conducted in neurology departments at 21 regional and university hospitals in Germany. Eligible participants were patients with aquaporin-4-antibody-positive or aquaporin-4-antibody-negative NMOSD. Main outcome measures were HRs from Cox proportional hazard regression models adjusted for centre effects, important prognostic factors and repeated treatment episodes...
June 1, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28571193/recurrent-longitudinally-extensive-myelitis-and-aquaporin-4-seronegativity-the-expanding-spectrum-of-neuromyelitis-optica
#12
Ravi Anadure, Coimbatore Sivasubramanian Narayanan, Govindaraj Varadraj
Neuromyelitis Optica Spectrum Disorder (NMOSD) is a recently proposed unifying term for Neuromyelitis Optica (NMO), also known as Devic's disease and related syndromes. It is a relapsing inflammatory demyelinating disease that most commonly affects optic nerves and the spinal cord, leading to sudden vision loss or weakness in one or both eyes, and loss of sensation and bladder function. Though inflammation may also involve the brain, the lesions seen in NMO are different from Multiple Sclerosis (MS) which has a similar clinical course...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28550707/micrornas-correlate-with-multiple-sclerosis-and-neuromyelitis-optica-spectrum-disorder-in-a-chinese-population
#13
Jianglong Chen, Jiting Zhu, Zeng Wang, Xiaoping Yao, Xuan Wu, Fang Liu, Weidong Zheng, Zhiwen Li, Aiyu Lin
BACKGROUND Recent studies identified a set of differentially expressed miRNAs in whole blood that may discriminate neuromyelitis optica spectrum disorders (NMOSD) from relapsing-remitting multiple sclerosis (RRMS). This study invalidated 9 known miRNAs in Chinese patients. MATERIAL AND METHODS The levels of miRNAs in whole blood were assayed in healthy controls (n=20) and patients with NMOSD (n=45), RRMS (n=17) by quantitative real-time polymerase chain reaction (qRT-PCR), and pairwise-compared between groups...
May 27, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28550069/incidence-and-prevalence-of-nmosd-in-australia-and-new-zealand
#14
Wajih Bukhari, Kerri M Prain, Patrick Waters, Mark Woodhall, Cullen M O'Gorman, Laura Clarke, Roger A Silvestrini, Christine S Bundell, David Abernethy, Sandeep Bhuta, Stefan Blum, Mike Boggild, Karyn Boundy, Bruce J Brew, Matthew Brown, Wallace J Brownlee, Helmut Butzkueven, William M Carroll, Celia Chen, Alan Coulthard, Russell C Dale, Chandi Das, Keith Dear, Marzena J Fabis-Pedrini, David Fulcher, David Gillis, Simon Hawke, Robert Heard, Andrew P D Henderson, Saman Heshmat, Suzanne Hodgkinson, Sofia Jimenez-Sanchez, Trevor Killpatrick, John King, Christopher Kneebone, Andrew J Kornberg, Jeannette Lechner-Scott, Ming-Wei Lin, Christpher Lynch, Richard Macdonell, Deborah F Mason, Pamela A McCombe, Michael P Pender, Jennifer A Pereira, John D Pollard, Stephen W Reddel, Cameron Shaw, Judith Spies, James Stankovich, Ian Sutton, Steve Vucic, Michael Walsh, Richard C Wong, Eppie M Yiu, Michael H Barnett, Allan G Kermode, Mark P Marriott, John D E Parratt, Mark Slee, Bruce V Taylor, Ernest Willoughby, Robert J Wilson, Angela Vincent, Simon A Broadley
OBJECTIVES: We have undertaken a clinic-based survey of neuromyelitis optica spectrum disorders (NMOSDs) in Australia and New Zealand to establish incidence and prevalence across the region and in populations of differing ancestry. BACKGROUND: NMOSD is a recently defined demyelinating disease of the central nervous system (CNS). The incidence and prevalence of NMOSD in Australia and New Zealand has not been established. METHODS: Centres managing patients with demyelinating disease of the CNS across Australia and New Zealand reported patients with clinical and laboratory features that were suspicious for NMOSD...
May 26, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28542592/fatigue-in-patients-with-neuromyelitis-optica-spectrum-disorder-and-its-impact-on-quality-of-life
#15
Jin Myoung Seok, Misong Choi, Eun Bin Cho, Hye Lim Lee, Byoung Joon Kim, Kwang Ho Lee, Pamela Song, Eun Yeon Joo, Ju-Hong Min
Fatigue is a prevalent symptom and major burden in neuroimmunological diseases. In neuromyelitis optica spectrum disorder (NMOSD), a severe autoimmune central nervous system (CNS) inflammatory disease with autoantibodies reactive to aquaporin-4, there are few reports about fatigue and quality of life (QOL). We aimed to evaluate the severity of fatigue and its relationship with QOL in patients with NMOSD. We prospectively studied patients with NMOSD who were in remission and seropositive for anti-aquaporin-4 antibody, and they were divided into 2 groups based on the presence of fatigue assessed using the Functional Assessment of Chronic Illness Therapy-fatigue score...
2017: PloS One
https://www.readbyqxmd.com/read/28533781/myelin-oligodendrocyte-glycoprotein-deciphering-a-target-in-inflammatory-demyelinating-diseases
#16
REVIEW
Patrick Peschl, Monika Bradl, Romana Höftberger, Thomas Berger, Markus Reindl
Myelin oligodendrocyte glycoprotein (MOG), a member of the immunoglobulin (Ig) superfamily, is a myelin protein solely expressed at the outermost surface of myelin sheaths and oligodendrocyte membranes. This makes MOG a potential target of cellular and humoral immune responses in inflammatory demyelinating diseases. Due to its late postnatal developmental expression, MOG is an important marker for oligodendrocyte maturation. Discovered about 30 years ago, it is one of the best-studied autoantigens for experimental autoimmune models for multiple sclerosis (MS)...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28508010/spontaneous-remission-lasting-more-than-a-decade-in-untreated-aqp4-antibody-positive-nmosd
#17
Lekha Pandit, Sharik Mustafa
No abstract text is available yet for this article.
July 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28495144/expansion-of-il-6-th17-like-cells-expressing-tlrs-correlates-with-microbial-translocation-and-neurological-disabilities-in-nmosd-patients
#18
Priscila O Barros, Aleida S O Dias, Taissa M Kasahara, Alice M M Ornelas, Renato S Aguiar, Soniza A Leon, Anne Ruiz, Romain Marignier, Ana Carolina R A Araújo, Regina Alvarenga, Cleonice A M Bento
Different microbial antigens, by signaling through toll-like receptors (TLR), may contribute to Th17-mediated autoimmune diseases, such as neuromyelitis optica spectrum disorder (NMOSD). The objective of this study was to determine the proportion of different Th17-like cell subsets that express TLR in NMOSD patients. For this study, the frequency of different Th17 cell subsets expressing TLR subsets in healthy individuals (n=20) and NMOSD patients (n=20) was evaluated by cytometry. The peripheral levels of soluble CD14 (sCD14) and cytokines were determined by ELISA...
June 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28476103/intracranial-lesion-as-onset-symptom-in-a-patient-with-early-undifferentiated-connective-tissue-disease-a-case-report
#19
Ying Du, Chuan Li, Dai-di Zhao, Jia-Rui Lu, Wei Zhang, Zhu-Yi Li
BACKGROUND: Undifferentiated connective tissue disease (UCTD) is widely considered to be a distinct clinical entity, and now divided into two subgroups: stable UCTD and early UCTD. The most frequent onset symptoms of UCTD include arthralgias, arthritis, Raynaud's phenomenon, mucocutaneous involvement, and sicca symptoms. However, Neurologic involvement is rare, and intracranial lesion as onset symptom in a patient with early UCTD has not yet been reported. CASE PRESENTATION: A 51-year-old Chinese female experienced progressive left leg weakness for 14 days before hospitalizing in our department...
May 5, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28465700/neuromyelitis-optica-spectrum-disease-characteristics-in-isfahan-iran-a-cross-sectional-study
#20
Fereshte Ashtari, Ali Safaei, Vahid Shaygannejad, Mohammad Amin Najafi, Sahar Vesal
BACKGROUND: Neuromyelitis optica spectrum disease (NMOSD) is a severe autoimmune demyelinating disorder of the central nervous system that throughout epidemiological data, it has not been completely determined. The aim of this study was to assess characteristics of NMOSD patients in Isfahan as one of the most prevalent cities for multiple sclerosis in Iran. MATERIALS AND METHODS: Forty-five patients diagnosed as neuromyelitis optica (NMO) disease through 5 years enrolled in this study...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
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