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Neuromyelitis Optica (NMO) Spectrum Disorders

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https://www.readbyqxmd.com/read/27806871/neuromyelitis-optica-unmasked-by-a-spinal-dural-arteriovenous-fistula
#1
Brin Freund, Ellen M Mowry, McR, Michael Levy, Scott D Newsome
Neuromyelitis optica spectrum disorder (NMO-SD) and spinal dural arteriovenous fistula (SDAVF) can both cause longitudinally extensive myelopathy. We describe a case of longitudinally extensive myelopathy attributed to a SDAVF. Despite treatment, myelopathy recurred and led to subsequent NMO-SD diagnosis. We consider a possible link between the two disorders which may shed light on the pathophysiology of NMO-SD. To our knowledge, this is the first published case of co-existent NMO-SD and SDAVF leading to myelopathy...
November 15, 2016: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/27804015/combined-brain-and-anterior-visual-pathways-mris-assist-in-early-identification-of-neuromyelitis-optica-spectrum-disorder-at-onset-of-optic-neuritis
#2
D Buch, J Savatovsky, O Gout, C Vignal, R Deschamps
Acute optic neuritis (ON) is the initial presentation in half of neuromyelitis optica spectrum disorder (NMO-SD) cases. Our objective was to evaluate accuracy of combined MRIs of the anterior visual pathways and of the brain to correctly identify NMO-SD among patients with acute ON. We performed a retrospective study on patients with acute ON in NMO-SD (16 episodes) and first-event non-NMO-SD (32 episodes). All MRIs included exams of the brain and anterior visual pathways using T2-weighted and post-gadolinium T1-weighted coronal thin slices...
November 1, 2016: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/27802824/mog-igg-in-nmo-and-related-disorders-a-multicenter-study-of-50-patients-part-4-afferent-visual-system-damage-after-optic-neuritis-in-mog-igg-seropositive-versus-aqp4-igg-seropositive-patients
#3
Florence Pache, Hanna Zimmermann, Janine Mikolajczak, Sophie Schumacher, Anna Lacheta, Frederike C Oertel, Judith Bellmann-Strobl, Sven Jarius, Brigitte Wildemann, Markus Reindl, Amy Waldman, Kerstin Soelberg, Nasrin Asgari, Marius Ringelstein, Orhan Aktas, Nikolai Gross, Mathias Buttmann, Thomas Ach, Klemens Ruprecht, Friedemann Paul, Alexander U Brandt
BACKGROUND: Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been reported in patients with aquaporin-4 antibody (AQP4-IgG)-negative neuromyelitis optica spectrum disorders (NMOSD). The objective of this study was to describe optic neuritis (ON)-induced neuro-axonal damage in the retina of MOG-IgG-positive patients in comparison with AQP4-IgG-positive NMOSD patients. METHODS: Afferent visual system damage following ON was bilaterally assessed in 16 MOG-IgG-positive patients with a history of ON and compared with that in 16 AQP4-IgG-positive NMOSD patients...
November 1, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27800532/insufficient-treatment-of-severe-depression-in-neuromyelitis-optica-spectrum-disorder
#4
Velina S Chavarro, Maureen A Mealy, Alexandra Simpson, Anna Lacheta, Florence Pache, Klemens Ruprecht, Stefan M Gold, Friedemann Paul, Alexander Ulrich Brandt, Michael Levy
OBJECTIVE: To investigate depression frequency, severity, current treatment, and interactions with somatic symptoms among patients with neuromyelitis optica spectrum disorder (NMOSD). METHODS: In this dual-center observational study, we included 71 patients diagnosed with NMOSD according to the International Panel for NMO Diagnosis 2015 criteria. The Beck Depression Inventory (BDI) was classified into severe, moderate, or minimal/no depressive state category. We used the Fatigue Severity Scale to evaluate fatigue...
December 2016: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/27793206/mog-igg-in-nmo-and-related-disorders-a-multicenter-study-of-50-patients-part-2-epidemiology-clinical-presentation-radiological-and-laboratory-features-treatment-responses-and-long-term-outcome
#5
Sven Jarius, Klemens Ruprecht, Ingo Kleiter, Nadja Borisow, Nasrin Asgari, Kalliopi Pitarokoili, Florence Pache, Oliver Stich, Lena-Alexandra Beume, Martin W Hümmert, Marius Ringelstein, Corinna Trebst, Alexander Winkelmann, Alexander Schwarz, Mathias Buttmann, Hanna Zimmermann, Joseph Kuchling, Diego Franciotta, Marco Capobianco, Eberhard Siebert, Carsten Lukas, Mirjam Korporal-Kuhnke, Jürgen Haas, Kai Fechner, Alexander U Brandt, Kathrin Schanda, Orhan Aktas, Friedemann Paul, Markus Reindl, Brigitte Wildemann
BACKGROUND: A subset of patients with neuromyelitis optica spectrum disorders (NMOSD) has been shown to be seropositive for myelin oligodendrocyte glycoprotein antibodies (MOG-IgG). OBJECTIVE: To describe the epidemiological, clinical, radiological, cerebrospinal fluid (CSF), and electrophysiological features of a large cohort of MOG-IgG-positive patients with optic neuritis (ON) and/or myelitis (n = 50) as well as attack and long-term treatment outcomes. METHODS: Retrospective multicenter study...
September 27, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27788675/mog-igg-in-nmo-and-related-disorders-a-multicenter-study-of-50-patients-part-1-frequency-syndrome-specificity-influence-of-disease-activity-long-term-course-association-with-aqp4-igg-and-origin
#6
Sven Jarius, Klemens Ruprecht, Ingo Kleiter, Nadja Borisow, Nasrin Asgari, Kalliopi Pitarokoili, Florence Pache, Oliver Stich, Lena-Alexandra Beume, Martin W Hümmert, Corinna Trebst, Marius Ringelstein, Orhan Aktas, Alexander Winkelmann, Mathias Buttmann, Alexander Schwarz, Hanna Zimmermann, Alexander U Brandt, Diego Franciotta, Marco Capobianco, Joseph Kuchling, Jürgen Haas, Mirjam Korporal-Kuhnke, Soeren Thue Lillevang, Kai Fechner, Kathrin Schanda, Friedemann Paul, Brigitte Wildemann, Markus Reindl
BACKGROUND: Antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) have been suggested to play a role in a subset of patients with neuromyelitis optica and related disorders. OBJECTIVE: To assess (i) the frequency of MOG-IgG in a large and predominantly Caucasian cohort of patients with optic neuritis (ON) and/or myelitis; (ii) the frequency of MOG-IgG among AQP4-IgG-positive patients and vice versa; (iii) the origin and frequency of MOG-IgG in the cerebrospinal fluid (CSF); (iv) the presence of MOG-IgG at disease onset; and (v) the influence of disease activity and treatment status on MOG-IgG titers...
September 26, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27783452/comparisons-of-the-efficacy-and-tolerability-of-mycophenolate-mofetil-and-azathioprine-as-treatments-for-neuromyelitis-optica-and-neuromyelitis-optica-spectrum-disorder
#7
H Chen, W Qiu, Q Zhang, J Wang, Z Shi, J Liu, Z Lian, H Feng, X Miao, H Zhou
BACKGROUND AND PURPOSE: To research and compare the efficacy and tolerability of mycophenolate mofetil (MMF) and azathioprine (AZA) in neuromyelitis optica (NMO) and NMO spectrum disorder (NMOSD). METHODS: In this observational study, we enrolled patients with NMO/NMOSD who received either MMF or AZA for 6 months or more. We compared the efficacy and tolerability of MMF and AZA as preventive treatments in patients with NMO/NMOSD. RESULTS: Baseline variables between groups were not significantly different...
October 26, 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/27772762/low-t3-syndrome-in-neuromyelitis-optica-spectrum-disorder-associations-with-disease-activity-and-disability
#8
Eun Bin Cho, Ju-Hong Min, Hye-Jin Cho, Jin Myoung Seok, Hye Lim Lee, Hee Young Shin, Kwang-Ho Lee, Byoung Joon Kim
BACKGROUND: Neuromyelitis optica (NMO) sometimes coexists with serological marker-positive, non-organ-specific autoimmune disorders. We evaluated the prevalence of thyroid dysfunction and anti-thyroid antibodies in patients with NMO spectrum disorder (NMOSD) and investigated the associations between thyroid dysfunction/autoimmunity and clinical features of NMOSD. METHODS: Forty-nine NMOSD patients with anti-aquaporin-4 antibody and 392 age- and sex-matched healthy controls were included...
November 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27758954/influence-of-female-sex-and-fertile-age-on-neuromyelitis-optica-spectrum-disorders
#9
Nadja Borisow, Ingo Kleiter, Anna Gahlen, Katrin Fischer, Klaus-Dieter Wernecke, Florence Pache, Klemens Ruprecht, Joachim Havla, Markus Krumbholz, Tania Kümpfel, Orhan Aktas, Marius Ringelstein, Christian Geis, Christoph Kleinschnitz, Achim Berthele, Bernhard Hemmer, Klemens Angstwurm, Robert Weissert, Jan-Patrick Stellmann, Simon Schuster, Martin Stangel, Florian Lauda, Hayrettin Tumani, Christoph Mayer, Lena Zeltner, Ulf Ziemann, Ralf A Linker, Matthias Schwab, Martin Marziniak, Florian Then Bergh, Ulrich Hofstadt-van Oy, Oliver Neuhaus, Alexander Winkelmann, Wael Marouf, Lioba Rückriem, Jürgen Faiss, Brigitte Wildemann, Friedemann Paul, Sven Jarius, Corinna Trebst, Kerstin Hellwig
BACKGROUND: Gender and age at onset are important epidemiological factors influencing prevalence, clinical presentation, and treatment response in autoimmune diseases. OBJECTIVE: To evaluate the impact of female sex and fertile age on aquaporin-4-antibody (AQP4-ab) status, attack localization, and response to attack treatment in patients with neuromyelitis optica (NMO) and its spectrum disorders (neuromyelitis optica spectrum disorder (NMOSD)). METHODS: Female-to-male ratios, diagnosis at last visit (NMO vs NMOSD), attack localization, attack treatment, and outcome were compared according to sex and age at disease or attack onset...
October 6, 2016: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/27740991/imaging-of-central-nervous-system-demyelinating-disorders
#10
Konstantin Balashov
PURPOSE OF REVIEW: This article focuses on neuroimaging in multiple sclerosis (MS), the most common central nervous system (CNS) demyelinating disorder encountered by practicing neurologists. Less common adult demyelinating disorders and incidental subclinical white matter abnormalities that are often considered in the differential diagnosis of MS are also reviewed. RECENT FINDINGS: Advancements in neuroimaging techniques, eg, the application of ultrahigh-field MRI, are rapidly expanding the use of neuroimaging in CNS demyelinating disorders...
October 2016: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/27648464/restoring-immune-tolerance-in-neuromyelitis-optica-part-ii
#11
REVIEW
Amit Bar-Or, Larry Steinman, Jacinta M Behne, Daniel Benitez-Ribas, Peter S Chin, Michael Clare-Salzler, Donald Healey, James I Kim, David M Kranz, Andreas Lutterotti, Roland Martin, Sven Schippling, Pablo Villoslada, Cheng-Hong Wei, Howard L Weiner, Scott S Zamvil, Terry J Smith, Michael R Yeaman
Neuromyelitis optica spectrum disorder (NMO/SD) and its clinical variants have at their core the loss of immune tolerance to aquaporin-4 and perhaps other autoantigens. The characteristic phenotype is disruption of astrocyte function and demyelination of spinal cord, optic nerves, and particular brain regions. In this second of a 2-part article, we present further perspectives regarding the pathogenesis of NMO/SD and how this disease might be amenable to emerging technologies aimed at restoring immune tolerance to disease-implicated self-antigens...
October 2016: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/27648463/restoring-immune-tolerance-in-neuromyelitis-optica-part-i
#12
REVIEW
Larry Steinman, Amit Bar-Or, Jacinta M Behne, Daniel Benitez-Ribas, Peter S Chin, Michael Clare-Salzler, Donald Healey, James I Kim, David M Kranz, Andreas Lutterotti, Roland Martin, Sven Schippling, Pablo Villoslada, Cheng-Hong Wei, Howard L Weiner, Scott S Zamvil, Michael R Yeaman, Terry J Smith
Neuromyelitis optica (NMO) and spectrum disorder (NMO/SD) represent a vexing process and its clinical variants appear to have at their pathogenic core the loss of immune tolerance to the aquaporin-4 water channel protein. This process results in a characteristic pattern of astrocyte dysfunction, loss, and demyelination that predominantly affects the spinal cord and optic nerves. Although several empirical therapies are currently used in the treatment of NMO/SD, none has been proven effective in prospective, adequately powered, randomized trials...
October 2016: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/27645353/neuromyelitis-optica-spectrum-disorder-coinciding-with-hematological-immune-disease-a-case-report
#13
R Patejdl, M Wittstock, U K Zettl, K Jost, A Grossmann, J Prudlo
INTRODUCTION: Recently defined consensus criteria for the diagnosis of neuromyelitis optica spectrum disorders (NMOSD) allow establishing the diagnosis in patients without elevated AQP4-Ab and optic nerve involvement. According to the new extended definition, NMOSD is closely associated with extensive spinal cord inflammation occurring in the course of systemic autoimmune diseases as sarcoidosis or lupus erythematodes. NMOSD occurring in the course of hematological disease have not yet been reported in the literature...
September 2016: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/27602543/application-of-diffusional-kurtosis-imaging-to-detect-occult-brain-damage-in-multiple-sclerosis-and-neuromyelitis-optica
#14
Wenshu Qian, Koon Ho Chan, Edward S Hui, Chi Yan Lee, Yong Hu, Henry Ka-Fung Mak
Multiple sclerosis (MS) and neuromyelitis optica (NMO) are two common types of inflammatory demyelinating disease of the central nervous system. Early distinction of NMO from MS is crucial but quite challenging. In this study, 13 NMO spectrum disorder patients (Expanded Disability Status Scale (EDSS) of 3.0 ± 1.7, ranging from 2 to 6.5; disease duration of 5.3 ± 4.7 years), 17 relapsing-remitting MS patients (EDSS of 2.6 ± 1.4, ranging from 1 to 5.5; disease duration of 7.9 ± 7.8 years) and 18 healthy volunteers were recruited...
November 2016: NMR in Biomedicine
https://www.readbyqxmd.com/read/27572863/neuromyelitis-optica-spectrum-disorders-in-children-and-adolescents
#15
Silvia Tenembaum, Tanuja Chitnis, Ichiro Nakashima, Nicolas Collongues, Andrew McKeon, Michael Levy, Kevin Rostasy
Neuromyelitis optica (NMO) is a severe autoimmune disease of the CNS characterized by recurrent inflammatory events primarily involving the optic nerves and spinal cord. NMO is infrequent in children, but early recognition is important to start adequate treatment. In this article, we review the evolving diagnostic criteria of NMO and provide an update on the clinical and neuroimaging spectrum of the disorder in pediatric patients, including current knowledge on immunopathogenesis and treatment recommendations for children with NMO...
August 30, 2016: Neurology
https://www.readbyqxmd.com/read/27571069/efficacy-of-polyvalent-human-immunoglobulins-in-an-animal-model-of-neuromyelitis-optica-evoked-by-intrathecal-anti-aquaporin-4-antibodies
#16
Benedikt Grünewald, Jeffrey L Bennett, Klaus V Toyka, Claudia Sommer, Christian Geis
Neuromyelitis Optica Spectrum Disorders (NMOSD) are associated with autoantibodies (ABs) targeting the astrocytic aquaporin-4 water channels (AQP4-ABs). These ABs have a direct pathogenic role by initiating a variety of immunological and inflammatory processes in the course of disease. In a recently-established animal model, chronic intrathecal passive-transfer of immunoglobulin G from NMOSD patients (NMO-IgG), or of recombinant human AQP4-ABs (rAB-AQP4), provided evidence for complementary and immune-cell independent effects of AQP4-ABs...
2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27553618/the-impact-of-2015-neuromyelitis-optica-spectrum-disorders-criteria-on-diagnostic-rates
#17
Shahd Hm Hamid, Liene Elsone, Kerry Mutch, Tom Solomon, Anu Jacob
BACKGROUND: The international panel for neuromyelitis optica (NMO) diagnosis has proposed diagnostic criteria for neuromyelitis optica spectrum disorders (NMOSD). OBJECTIVES: We assessed the impact of these criteria on diagnostic rates in a large cohort of patients. METHODS: We identified and applied the 2006 and 2015 criteria to all patients (n = 176) seen in the NMO and non-multiple sclerosis central nervous system demyelination clinic (part of the UK NMO service) from January 2013 to May 2015...
August 23, 2016: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/27538635/clinical-characteristics-of-disabling-attacks-at-onset-in-patients-with-neuromyelitis-optica-spectrum-disorder
#18
Jin Myoung Seok, Eun Bin Cho, Hye Lim Lee, Hye-Jin Cho, Ju-Hong Min, Kwang Ho Lee, Byoung Joon Kim
BACKGROUND: Individual attacks of neuromyelitis optica (NMO) are generally severe enough to cause disability even after the onset attack. We aimed to elucidate the clinical characteristics of disabling attacks at the onset of NMO. METHODS: We investigated the clinical characteristics at onset and at first relapse in patients with NMO or NMO spectrum disorder with seropositive for the anti-aquaporin-4 antibody. A disabling attack at onset (DAO) was defined as an onset attack in which, at best recovery (allowing up to one year), patients were unable to walk without assistance or were left functionally blind in at least one affected eye...
September 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27529327/finding-nmo-the-evolving-diagnostic-criteria-of-neuromyelitis-optica
#19
Jeffrey L Bennett
Neuromyelitis optica (NMO) is an autoimmune demyelinating disorder of the central nervous system (CNS) with predilection for the optic nerves and spinal cord. Since its emergence in the medical literature in the late 1800's, the diagnostic criteria for NMO has slowly evolved from the simultaneous presentation of neurologic and ophthalmic signs to a relapsing or monophasic CNS disorder defined by clinical, neuroimaging, and laboratory criteria. Due to the identification of a specific autoantibody response against the astrocyte water channel aquaporin-4 (AQP4) in the vast majority of affected individuals, the clinical spectrum of NMO has greatly expanded necessitating the development of new international criteria for the diagnosis of NMO spectrum disorder (NMOSD)...
September 2016: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/27503347/aquaporin-4-specific-t-cells-and-nmo-igg-cause-primary-retinal-damage-in-experimental-nmo-sd
#20
Bleranda Zeka, Maria Hastermann, Nathalie Kaufmann, Kathrin Schanda, Marko Pende, Tatsuro Misu, Paulus Rommer, Kazuo Fujihara, Ichiro Nakashima, Charlotte Dahle, Fritz Leutmezer, Markus Reindl, Hans Lassmann, Monika Bradl
Neuromyelitis optica/spectrum disorder (NMO/SD) is a severe, inflammatory disease of the central nervous system (CNS). In the majority of patients, it is associated with the presence of pathogenic serum autoantibodies (the so-called NMO-IgGs) directed against the water channel aquaporin 4 (AQP4), and with the formation of large, astrocyte-destructive lesions in spinal cord and optic nerves. A large number of recent studies using optical coherence tomography (OCT) demonstrated that damage to optic nerves in NMO/SD is also associated with retinal injury, as evidenced by retinal nerve fiber layer (RNFL) thinning and microcystic inner nuclear layer abnormalities...
2016: Acta Neuropathologica Communications
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