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Neuromyelitis optica

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https://www.readbyqxmd.com/read/29455925/differentiation-of-remitting-neuromyelitis-optica-spectrum-disorders-from-multiple-sclerosis-by-integrating-parameters-from-serum-proteins-and-lymphocyte-subsets
#1
Peng-Peng Ip, Chen-Yen Chung, Chien-Ching Chang, Yu-Fang Lee, Hui-Min Wang, Ie-Bin Lian, Cathy Shen-Jang Fann, Chih-Chao Yang, Fang Liao
Differential diagnosis for neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) is always doubtful. To differentiate these diseases, we studied the immune status in the blood of patients with MS (n = 45) or NMOSD (n = 23) at remission phase. Remitting NMOSD patients had increased levels of CXCL13 and memory B cells, while remitting MS patients had elevated levels of galectin-9 and Th1 cells. A diagnostic model with these four variables is built to distinguish remitting NMOSD from MS with a sensitivity of 91...
February 8, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29455827/paraneoplastic-neuromyelitis-optica-spectrum-disorder-as-presentation-of-esophageal-adenocarcinoma
#2
Daniel C Wiener, Tamara B Kaplan, Carlos E Bravo-Iñiguez, Jordan Miller, Aaron L Berkowitz, Michael T Jaklitsch
Neuromyelitis optica spectrum disorders are a group of relapsing, inflammatory, demyelinating neurologic syndromes involving the central nervous system associated with antibodies against aquaporin-4. Although most commonly an idiopathic autoimmune condition, neuromyelitis optica may occur as a paraneoplastic syndrome in rare instances. We report a case of transverse myelitis caused by paraneoplastic neuromyelitis optica as the presenting clinical syndrome in a patient with esophageal adenocarcinoma.
March 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29455361/treatment-of-neuromyelitis-optica-with-rituximab-a-2-year-prospective-multicenter-study
#3
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
https://www.readbyqxmd.com/read/29455184/csf-free-light-chain-identification-of-demyelinating-disease-comparison-with-oligoclonal-banding-and-other-csf-indexes
#4
Kari M Gurtner, Eslam Shosha, Sandra C Bryant, Bruna D Andreguetto, David L Murray, Sean J Pittock, Maria Alice V Willrich
BACKGROUND: Cerebrospinal fluid (CSF) used in immunoglobulin gamma (IgG) index testing and oligoclonal bands (OCBs) are common laboratory tests used in the diagnosis of multiple sclerosis. The measurement of CSF free light chains (FLC) could pose as an alternative to the labor-intensive isoelectric-focusing (IEF) gels used for OCBs. METHODS: A total of 325 residual paired CSF and serum specimens were obtained after physician-ordered OCB IEF testing. CSF kappa (cKFLC) and lambda FLC (cLFLC), albumin and total IgG were measured...
February 19, 2018: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/29454471/severe-course-of-neuromyelitis-optica-in-a-female-patient-with-chronic-c-hepatitis
#5
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
https://www.readbyqxmd.com/read/29449281/mri-features-of-aquaporin-4-antibody-positive-longitudinally-extensive-transverse-myelitis-insights-into-the-diagnosis-of-neuromyelitis-optica-spectrum-disorders
#6
C G Chee, K S Park, J W Lee, H W Ahn, E Lee, Y Kang, H S Kang
BACKGROUND AND PURPOSE: Longitudinally extensive transverse myelitis is a well-documented spinal manifestation of neuromyelitis optica spectrum disorders, however, other forms of nontumorous myelopathy can also manifest as longitudinally extensive transverse myelitis. Our aim was to evaluate the MR imaging features of aquaporin-4 antibody-positive longitudinally extensive transverse myelitis, which is strongly associated with neuromyelitis optica spectrum disorders. MATERIALS AND METHODS: We evaluated cervicomedullary junction involvement, cord expansion ratios, bright spotty lesions, the number of involved segments, skipped lesions, enhancement patterns, and axial distribution patterns using spinal MR imaging of 41 patients with longitudinally extensive transverse myelitis who underwent aquaporin-4 antibody testing...
February 15, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29447335/condition-dependent-generation-of-aquaporin-4-antibodies-from-circulating-b-cells-in-neuromyelitis-optica
#7
Robert Wilson, Mateusz Makuch, Anne-Kathrin Kienzler, James Varley, Jennifer Taylor, Mark Woodhall, Jacqueline Palace, M Isabel Leite, Patrick Waters, Sarosh R Irani
Autoantibodies to aquaporin-4 (AQP4) are pathogenic in neuromyelitis optica spectrum disorder (NMOSD). However, it is not known which B cells are the major contributors to circulating AQP4 antibodies nor which conditions promote their generation. Our experiments showed CD19+CD27++CD38++ circulating ex vivo antibody-secreting cells did not produce AQP4 antibodies under several culture conditions. To question whether other cells in circulation were capable of AQP4 antibody production, B cells were differentiated into antibody-secreting cells in vitro...
February 13, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29443442/paediatric-adem-followed-by-optic-neuritis-disease-course-treatment-response-and-outcome
#8
Y Y M Wong, Y Hacohen, T Armangue, E Wassmer, H Verhelst, C Hemingway, E D van Pelt, C E Catsman-Berrevoets, R Q Hintzen, K Deiva, M J Lim, K Rostásy, R F Neuteboom
BACKGROUND: Acute disseminated encephalomyelitis, followed by optic neuritis (ADEM-ON) is a rare demyelinating syndrome different than MS and neuromyelitis optica spectrum disorder. We aim to describe the disease course, treatment response and outcome of these children. METHODS: children <18 years were identified from 6 countries of the EU Paediatric Demyelinating Disease Consortium. Patients fulfilled the diagnostic criteria for ADEM, followed by at least one ON...
February 14, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29436488/worldwide-prevalence-of-neuromyelitis-optica-spectrum-disorders
#9
EDITORIAL
Masahiro Mori, Satoshi Kuwabara, Friedemann Paul
No abstract text is available yet for this article.
February 7, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29434480/a-rare-presentation-of-neuromyelitis-optica-spectrum-disorders
#10
Navneet K Singh, Alexander J Sweidan, Sarah Strube, Ignacio Carrillo-Nunez
Neuromyelitis optica spectrum disorders (NMOSDs) are a set of demyelinating disorders that primarily target the optic nerves and the spinal cord. Previously thought to be a subset of multiple sclerosis (MS), now is recognized as a distinct entity. We present a 59-year-old female patient who was admitted for acute upper and lower extremity weakness. The patient had woken up from sleep with sudden onset of weakness. Patient was initially diagnosed with a right hemispheric stroke; however, magnetic resonance imaging of the cervical spine later performed showed abnormal enhancement from C2-C4, representing transverse myelitis...
2018: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/29433095/early-relapse-after-rtx-initiation-in-a-patient-with-nmo-ms-overlap-syndrome-how-long-to-conclude-to-a-failure-treatment
#11
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
https://www.readbyqxmd.com/read/29428821/recombinant-igg1-fc-hexamers-block-cytotoxicity-and-pathological-changes-in-experimental-in-vitro-and-rat-models-of-neuromyelitis-optica
#12
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
https://www.readbyqxmd.com/read/29423614/mri-of-the-first-event-in-pediatric-acquired-demyelinating-syndromes-with-antibodies-to-myelin-oligodendrocyte-glycoprotein
#13
Matthias Baumann, Astrid Grams, Tanja Djurdjevic, Eva-Maria Wendel, Christian Lechner, Bettina Behring, Astrid Blaschek, Katharina Diepold, Astrid Eisenkölbl, Joel Fluss, Michael Karenfort, Johannes Koch, Bahadir Konuşkan, Steffen Leiz, Andreas Merkenschlager, Daniela Pohl, Mareike Schimmel, Charlotte Thiels, Barbara Kornek, Kathrin Schanda, Markus Reindl, Kevin Rostásy
Antibodies against the myelin oligodendrocyte glycoprotein (MOG-Ab) can be detected in various pediatric acquired demyelinating syndromes (ADS). Here, we analyze the spectrum of neuroradiologic findings in children with MOG-Ab and a first demyelinating event. The cerebral and spinal MRI of 69 children with different ADS was assessed in regard to the distribution and characteristics of lesions. Children with acute disseminated encephalomyelitis (n = 36) or neuromyelitis optica spectrum disorder (n = 5) presented an imaging pattern characterized predominantly by poorly demarcated lesions with a wide supra- and infratentorial distribution...
February 8, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29422845/myointimal-hyperplasia-in-a-patient-with-neuromyelitis-optica-devic-s-disease-after-the-creation-of-an-arteriovenous-graft
#14
Christina Rao, Stephanie Fox, Sapan S Desai
Neuromyelitis optica, also known as Devic's disease, is an autoimmune disorder that leads to the inflammation and demyelination of nerves. Devic's disease primarily affects the optic nerve and spinal cord, but can lead to a significant loss of function throughout the body if not treated with steroid therapy or plasmapheresis. We recently saw a 62-year-old patient who received plasmapheresis for Devic's disease through a PTFE arteriovenous graft in her left arm. Her graft clotted without warning, and percutaneous thrombolysis was not successful...
September 2017: Case Reports in Neurology
https://www.readbyqxmd.com/read/29421990/measurement-of-soluble-cd59-in-csf-in-demyelinating-disease-evidence-for-an-intrathecal-source-of-soluble-cd59
#15
Wioleta M Zelek, Lewis M Watkins, Owain W Howell, Rhian Evans, Sam Loveless, Neil P Robertson, Marijke Beenes, Loek Willems, Ricardo Brandwijk, B Paul Morgan
BACKGROUND: CD59, a broadly expressed glycosylphosphatidylinositol-anchored protein, is the principal cell inhibitor of complement membrane attack on cells. In the demyelinating disorders, multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD), elevated complement protein levels, including soluble CD59 (sCD59), were reported in cerebrospinal fluid (CSF). OBJECTIVES: We compared sCD59 levels in CSF and matched plasma in controls and patients with MS, NMOSD and clinically isolated syndrome (CIS) and investigated the source of CSF sCD59 and whether it was microparticle associated...
February 1, 2018: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/29414297/rituximab-induced-interstitial-lung-disease-in-a-patient-with-aquaporin-4-immunoglobulin-g-positive-neuromyelitis-optica-spectrum-disorder
#16
So Hyun Ahn, Sung-Min Kim, Jung-Joon Sung
Aquaporin-4 immunoglobulin G -positive neuromyelitis optica spectrum disorder (NMOSD-AQP4) is an inflammatory disease of the central nervous system that predominantly affects the optic nerves and spinal cord. Rituximab (RTX)-a monoclonal antibody to CD20 in B cells-is effective in the treatment of NMOSD. However, interstitial lung disease (ILD) is a very rare yet potentially fatal complication of RTX treatment. Here, we detail the first reported case of RTX-induced ILD in a patient with NMOSD-AQP4. ILD should be suspected in patients with NMOSD undergoing RTX treatment who present with dyspnea and/or cough without any signs or symptoms of infection...
February 2, 2018: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29414288/comparison-of-myelin-oligodendrocyte-glycoprotein-mog-antibody-disease-and-aqp4-igg-positive-neuromyelitis-optica-spectrum-disorder-nmosd-when-they-co-exist-with-anti-nmda-n-methyl-d-aspartate-receptor-encephalitis
#17
Siyuan Fan, Yan Xu, Haitao Ren, Hongzhi Guan, Feng Feng, Xuehui Gao, Ding Ding, Fang Fang, Guangliang Shan, Tianjia Guan, Yao Zhang, Yi Dai, Ming Yao, Bin Peng, Yicheng Zhu, Liying Cui
BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG)-antibody (ab) disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) can co-exist with anti-NMDA (N-methyl-D-aspartate) receptor encephalitis (NMDARe). OBJECTIVES: To characterize MOG-ab disease and AQP4-IgG-positive NMOSD during NMDARe. METHODS: We analyzed all the patients with overlapping MOG-ab disease and NMDARe (MNOS) and patients with AQP4-IgG-positive NMOSD and NMDARe (ANOS) in our hospital and compared those data with data from systematically review of previously published reports...
January 30, 2018: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29414283/beneficial-effect-of-plasma-exchange-in-acute-attack-of-neuromyelitis-optica-spectrum-disorders
#18
Tayanan Srisupa-Olan, Sasitorn Siritho, Kulvara Kittisares, Jiraporn Jitprapaikulsan, Chanjira Sathukitchai, Naraporn Prayoonwiwat
BACKGROUND: Plasma exchange (PLEX) is routinely performed in neuromyelitis optica spectrum disorders (NMOSD) patients with an acute attack who do not respond to corticosteroids treatment. OBJECTIVE: To compare treatment outcomes in NMOSD patients with an acute attack between the two groups. METHODS: We retrospectively studied 67 attacks from 52 NMOSD patients. Outcome measurements using Expanded Disability Status Scale (EDSS), modified Rankin Scale (mRS) were compared...
February 1, 2018: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29406915/validation-of-the-modified-fatigue-impact-scale-and-the-relationships-among-fatigue-pain-and-serum-interleukin-6-levels-in-patients-with-neuromyelitis-optica-spectrum-disorder
#19
Hiroki Masuda, Masahiro Mori, Akiyuki Uzawa, Tomohiko Uchida, Ryohei Ohtani, Shigeo Kobayashi, Satoshi Kuwabara
Fatigue and pain are disabling symptoms in patients with neuromyelitis optica spectrum disorder (NMOSD). The Modified Fatigue Impact Scale (MFIS) has not yet been validated in patients with NMOSD, and anti-interleukin-6 (IL-6) receptor antibody was reported to decrease pain and fatigue in patients with NMOSD. The aim of this study was to validate MFIS and to investigate the relationships among fatigue, pain and serum IL-6 levels in patients with NMOSD. MFIS and the Multidimensional Fatigue Inventory (MFI), an established scale for fatigue, were administered to patients with NMOSD and age- and sex-matched healthy controls (HCs)...
February 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29406904/comparison-of-efficacy-and-tolerability-of-azathioprine-mycophenolate-mofetil-and-lower-dosages-of-rituximab-among-patients-with-neuromyelitis-optica-spectrum-disorder
#20
Yang Yang, Chun-Juan Wang, Bao-Jie Wang, Zi-Ling Zeng, Shou-Gang Guo
OBJECTIVE: To observe and compare the efficacy and tolerability of azathioprine (AZA), mycophenolate mofetil (MMF) and lower dosages of rituximab (RTX) among patients with neuromyelitis optica spectrum disorder. METHODS: In this prospective cohort, AQP4-IgG-seropositive patients with neuromyelitis optica spectrum disorder (NMOSD) were enrolled and randomly divided into three groups, using AZA, MMF or lower dosages of RTX (defined as 100mg RTX intravenous injection, once per week for 4 consecutive weeks) respectively...
February 15, 2018: Journal of the Neurological Sciences
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