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Neuromyelitis Optica (NMO) Spectrum Disorders

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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/28184993/pathogenic-implications-of-cerebrospinal-fluid-barrier-pathology-in-neuromyelitis-optica
#3
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/28159705/demyelinating-syndrome-in-sle-encompasses-different-subtypes-do-we-need-new-classification-criteria-pooled-results-from-systematic-literature-review-and-monocentric-cohort-analysis
#4
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/28149102/magnetic-resonance-imaging-brain-findings-in-a-case-of-aquaporin-4-antibody-positive-neuromyelitis-optica-spectrum-disorder-presenting-with-intractable-vomiting-and-hiccups
#5
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
#6
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/28109254/letm-presented-with-causalgia-and-ensued-by-sudden-death
#7
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/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
#8
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
#9
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/28017256/treatment-of-neuromyelitis-optica-and-neuromyelitis-optica-spectrum-disorders-with-rituximab-using-a-maintenance-treatment-regimen-and-close-cd19-b-cell-monitoring-a-six-year-follow-up
#10
M E Evangelopoulos, E Andreadou, G Koutsis, V Koutoulidis, M Anagnostouli, P Katsika, D S Evangelopoulos, I Evdokimidis, C Kilidireas
Neuromyelitis optinca (NMO) represents a serious demyelinating disease of the central nervous system selectively attacking the spinal cord and optic nerve. Early differential diagnosis from multiple sclerosis is of vital importance, as NMO mandates immunosuppressive and not immunomodulatory treatment. Rituximab has been recently introduced as a treatment option for NMO. However, optimal surrogate measures and treatment intervals are still unclear. Five patients (females, mean age 54±10.21years) with NMO and NMO spectrum disorders (NMOSD) were evaluated with respect to disability and relapse rate...
January 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27806871/neuromyelitis-optica-unmasked-by-a-spinal-dural-arteriovenous-fistula
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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...
January 2017: 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
#18
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
#19
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
#20
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
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