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https://www.readbyqxmd.com/read/28920766/clinical-profile-of-patients-with-paraneoplastic-neuromyelitis-optica-spectrum-disorder-and-aquaporin-4-antibodies
#1
Maria Sepúlveda, Nuria Sola-Valls, Domingo Escudero, Bojan Rojc, Manuel Barón, Luis Hernández-Echebarría, Begoña Gómez, Josep Dalmau, Albert Saiz, Francesc Graus
BACKGROUND: In a minority of patients with neuromyelitis optica spectrum disorder (NMOSD) and aquaporin-4 antibodies (AQP4-IgG), the disease has a paraneoplastic origin. It is unknown whether these patients have distinctive clinical features. OBJECTIVE: To report the clinical features of a series of patients with paraneoplastic NMOSD and AQP4-IgG and to review previously reported cases. METHODS: Retrospective analysis of clinical records of 156 patients with NMOSD and AQP4-IgG and review of previously reported patients with paraneoplastic NMOSD and AQP4-IgG...
September 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28904454/rituximab-in-neuromyelitis-optica-spectrum-disorders-our-experience
#2
Jui Dilip Jade, Srishti Bansi, Bhim Singhal
BACKGROUND: Neuromyelitis optica (NMO) is an inflammatory demyelinating central nervous system disease, with recurrent attacks of severe bilateral optic neuritis and longitudinally extensive transverse myelitis. Aggressive immunosuppression is essential to prevent clinical relapses and permanent disability. Rituximab, a monoclonal antibody to CD20, has been found effective in several reports and small uncontrolled studies. There is a paucity of data regarding its use in Indian patients...
July 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28879451/prognostic-factors-and-disease-course-in-aquaporin-4-antibody-positive-chinese-patients-with-acute-optic-neuritis
#3
Hao Kang, Tingjun Chen, Hongyang Li, Quangang Xu, Shanshan Cao, Shihui Wei
The objective is to assess the frequency of AQP4-Ab in Chinese patients with optic neuritis (ON) and to investigate the prognostic implications of AQP4-Ab seropositivity in such patients. The presence of aquaporin-4 (AQP4) antibodies in human AQP4-transfected cells was determined by indirect immunofluorescence, and the diagnostic and prognostic relevance of AQP4 antibodies in 215 Chinese patients with optic neuritis was evaluated. The patients were enrolled from the Chinese People's Liberation Army General Hospital (PLAGH) and followed up to 1-228 months...
September 6, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28852993/stat4-polymorphisms-are-associated-with-neuromyelitis-optica-spectrum-disorders
#4
Ziyan Shi, Qin Zhang, Hongxi Chen, Zhiyun Lian, Ju Liu, Huiru Feng, Xiaohui Miao, Qin Du, Hongyu Zhou
STAT4 plays a crucial role in the functioning of the innate and adaptive immune cells and has been identified as a susceptibility gene in numerous autoimmune disorders. However, its association with neuromyelitis optica spectrum disorders (NMOSD) remains uncertain. Here, we performed a case-control study to determine whether STAT4 contributed to the risk of NMOSD. We tested five STAT4 SNPs in 233 patients with established NMOSD and 492 healthy controls. Chi-square tests and logistic regression analyses were performed with four genetic models, including allelic, additive, dominant, and recessive models, to identify associations with NMOSD...
August 29, 2017: Neuromolecular Medicine
https://www.readbyqxmd.com/read/28846745/comparison-of-cognitive-and-brain-grey-matter-volume-profiles-between-multiple-sclerosis-and-neuromyelitis-optica-spectrum-disorder
#5
Hiroki Masuda, Shigeki Hirano, Nobuyoshi Takahashi, Etsuko Hatsugano, Akiyuki Uzawa, Tomohiko Uchida, Ryohei Ohtani, Satoshi Kuwabara, Masahiro Mori
OBJECTIVE: Brain regions responsible for cognitive dysfunction in MS and neuromyelitis optica spectrum disorder (NMOSD) are not known. Our aim of this study was to investigate whether cognitive function and brain volume differed between MS and NMOSD in Japanese patients. METHODS: Brain MRI and neuropsychological tests including the Wechsler Adult Intelligence Scale-III (WAIS-III), Wechsler Memory Scale-Revised (WMS-R), Trail Making Test (TMT) and Clinical Assessment for Attention (CAT) were performed...
2017: PloS One
https://www.readbyqxmd.com/read/28840314/what-proportion-of-aqp4-igg-negative-nmo-spectrum-disorder-patients-are-mog-igg-positive-a-cross-sectional-study-of-132-patients
#6
Shahd H M Hamid, Daniel Whittam, Kerry Mutch, Samantha Linaker, Tom Solomon, Kumar Das, Maneesh Bhojak, Anu Jacob
Antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) have been described in patients with neuromyelitis optica spectrum disorders (NMOSD) without aquaporin-4 antibodies (AQP4-IgG). We aimed to identify the proportion of AQP4-IgG-negative NMOSD patients who are seropositive for MOG-IgG. In a cross sectional study, we reviewed all patients seen in the National NMO clinic over the last 4 years (after the availability of MOG-IgG testing), including clinical information, MRI, and antibody tests. 261 unique patients were identified...
August 24, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28835657/multimodal-magnetic-resonance-imaging-in-relation-to-cognitive-impairment-in-neuromyelitis-optica-spectrum-disorder
#7
Su-Hyun Kim, Eun Young Park, Boram Park, Jae-Won Hyun, Na Young Park, AeRan Joung, Sang Hyun Lee, Ho Jin Kim
Cognitive impairment (CI) is reported in 29-57% of patients with neuromyelitis optica spectrum disorder (NMOSD). However, the pathophysiology underlying CI in NMOSD is poorly understood. The present study aims to investigate the predictive values of various conventional and quantitative MRI parameters for cognitive performance in patients with NMOSD. Neurological assessment and conventional, diffusion tensor, and volumetric MRI sequences were collected form 73 patients with NMOSD and 44 healthy controls (HCs)...
August 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28834867/cortical-blindness-and-not-optic-neuritis-as-a-cause-of-vision-loss-in-a-sj%C3%A3-gren-s-syndrome-ss-patient-with-the-neuromyelitis-optica-spectrum-disorder-nmosd-challenges-of-ascribing-demyelinating-syndromes-to-ss-a-case-report
#8
Aliya Lalji, Izlem Izbudak, Julius Birnbaum
RATIONALE: The conception that multiple sclerosis may be challenging to distinguish from demyelinating manifestations of Sjögren's syndrome (SS) was introduced more than 30 years ago. However, it is now recognized that the neuromyelitis optica spectrum disorder (NMOSD) may occur more frequently in SS as opposed to multiple sclerosis. Characteristic NMOSD features can include severe attacks of optic neuritis, myelitis which is frequently longitudinally-extensive (spanning at least three vertebral segments on magnetic resonance imaging [MRI]), and an association with anti-aquaporin-4 antibodies...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28831548/comparison-of-the-efficacy-of-azathioprine-and-rituximab-in-neuromyelitis-optica-spectrum-disorder-a-randomized-clinical-trial
#9
Zahra Nikoo, Shervin Badihian, Vahid Shaygannejad, Nasrin Asgari, Fereshteh Ashtari
Neuromyelitis optica spectrum disorder (NMOSD) often follows a relapsing course. As disability in NMOSD is attack-related, effective treatments are needed. We aimed to compare the efficacy of azathioprine (AZA) and rituximab (RIT) as maintenance therapy in NMOSD patients. An open, randomized clinical trial was conducted during September 2015 to December 2016, in Isfahan, Iran. Initially, 100 NMOSD patients were approached, 86 entered the study, and 68 cases completed the trial. All patients had a relapsing-remitting course with expanded disability extended scale (EDSS) ≤7 (median 2...
August 22, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28814166/late-presentation-of-nmosd-as-rapidly-progressive-leukoencephalopathy-with-atypical-clinical-and-radiological-findings
#10
Elia Sechi, Alberto Addis, Lucia Batzu, Sara Mariotto, Sergio Ferrari, Maurizio Conti, GianPietro Sechi
Brain abnormalities in neuromyelitis optica spectrum disorder (NMOSD) are highly heterogeneous and often non-specific. Extensive white matter involvement has been described and frequently manifests with encephalopathy requiring prompt intervention. Rarely, this may represent the only manifestation at onset without concurrent suggestive features of the disease, thus making diagnosis challenging. NMOSD may potentially occur at any age, but it seems that this disorder has distinctive clinical features in the elderly...
August 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28803524/endocrinopathies-in-paediatric-onset-neuromyelitis-optica-spectrum-disorder-with-aquaporin-4-aqp4-antibody
#11
Yael Hacohen, Silvia Messina, Hoong-Wei Gan, Sukhvir Wright, Saleel Chandratre, Maria Isabel Leite, Penny Fallon, Angela Vincent, Olga Ciccarelli, Evangeline Wassmer, Ming Lim, Jacqueline Palace, Cheryl Hemingway
The involvement of the diencephalic regions in neuromyelitis optica spectrum disorder (NMOSD) may lead to endocrinopathies. In this study, we identified the following endocrinopathies in 60% (15/25) of young people with paediatric-onset aquaporin 4-Antibody (AQP4-Ab) NMOSD: morbid obesity ( n = 8), hyperinsulinaemia ( n = 5), hyperandrogenism ( n = 5), amenorrhoea ( n = 5), hyponatraemia ( n = 4), short stature ( n = 3) and central hypothyroidism ( n = 2) irrespective of hypothalamic lesions...
August 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28780848/-clinical-characteristics-of-neuromyelitis-optica-spectrum-disorders-associated-with-syringomyelia
#12
Y G Wang, Y Q Wang, W Qiu, X Q Hu, Z Z Lu
Objective: To analyze the clinical characteristics of neuromyelitis optica spectrum disorders (NMOSD) associated with syringomyelia (SML). Methods: The clinical manifestations, laboratory parameters and imaging findings of 7 patients with NMOSD associated with SML during June 2008 to August 2016 from The Third Affiliated Hospital of Sun Yat-sen University were retrospectively analyzed. Results: Six patients were female and 1 was male, with ages ranging from 27-67 years, the course of the disease was 3-12 years, recurrence was 2-7 times, all the patients were cerebrospinal fluid oligoclonal bands (CSF OCB) negative, 5 patients were positive for aquaporin-4-antibody/NMO-IgG...
August 1, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28770374/mog-antibody-related-disorders-common-features-and-uncommon-presentations
#13
Álvaro Cobo-Calvo, Anne Ruiz, Hyacintha D'Indy, Anne-Lise Poulat, Maryline Carneiro, Nicolas Philippe, Françoise Durand-Dubief, Kumaran Deiva, Sandra Vukusic, Vincent Desportes, Romain Marignier
Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) have been reported in acute demyelinating encephalomyelitis (ADEM), optic neuritis (ON), and neuromyelitis optica spectrum disorders (NMOSD) in adults and pediatrics. We aimed to delineate the common features of MOG-Ab-related disorders in children and adults, and report uncommon presentations. Twenty-seven consecutive pediatric and adult patients testing positive for MOG-Ab, with a minimum follow-up of 6 months, were included. Comprehensive epidemiological, clinical, radiological, and laboratory data were retrospectively analyzed...
August 2, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28768844/prognostic-relevance-of-mog-antibodies-in-children-with-an-acquired-demyelinating-syndrome
#14
MULTICENTER STUDY
Eva-Maria Hennes, Matthias Baumann, Kathrin Schanda, Banu Anlar, Barbara Bajer-Kornek, Astrid Blaschek, Sigrid Brantner-Inthaler, Katharina Diepold, Astrid Eisenkölbl, Thaddäus Gotwald, Georgi Kuchukhidze, Ursula Gruber-Sedlmayr, Martin Häusler, Romana Höftberger, Michael Karenfort, Andrea Klein, Johannes Koch, Verena Kraus, Christian Lechner, Steffen Leiz, Frank Leypoldt, Simone Mader, Klaus Marquard, Imke Poggenburg, Daniela Pohl, Martin Pritsch, Markus Raucherzauner, Mareike Schimmel, Charlotte Thiels, Daniel Tibussek, Silvia Vieker, Carolin Zeches, Thomas Berger, Markus Reindl, Kevin Rostásy
OBJECTIVE: To assess the prognostic value of MOG antibodies (abs) in the differential diagnosis of acquired demyelinating syndromes (ADS). METHODS: Clinical course, MRI, MOG-abs, AQP4-abs, and CSF cells and oligoclonal bands (OCB) in children with ADS and 24 months of follow-up were reviewed in this observational prospective multicenter hospital-based study. RESULTS: Two hundred ten children with ADS were included and diagnosed with acute disseminated encephalomyelitis (ADEM) (n = 60), neuromyelitis optica spectrum disorder (NMOSD) (n = 12), clinically isolated syndrome (CIS) (n = 101), and multiple sclerosis (MS) (n = 37) after the first episode...
August 29, 2017: Neurology
https://www.readbyqxmd.com/read/28756870/anti-c1q-autoantibodies-in-patients-with-neuromyelitis-optica-spectrum-disorders
#15
Nobuaki Yoshikura, Akio Kimura, Yuichi Hayashi, Takashi Inuzuka
We examined anti-complement C1q (C1q) autoantibody levels in serum and cerebrospinal fluid (CSF) samples of patients with neuromyelitis optica spectrum disorders (NMOSD). We analyzed the correlations between anti-C1q autoantibody levels and the clinical and other CSF characteristics of NMOSD. Serum and CSF anti-C1q autoantibody levels increased during the acute phase of NMOSD, reverting to the same levels as controls during remission. CSF anti-C1q autoantibody levels during the acute phase correlated with several markers reflecting disease severity, Expanded Disability Status Scale worsening, spinal cord lesion length in cases with myelitis, CSF protein and interleukin-6 levels, and CSF/serum albumin ratios...
September 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28748449/first-report-of-persistent-dengue-1-associated-autoimmune-neurological-disturbance-neuromyelitis-optica-spectrum-disorder
#16
Marzia Puccioni-Sohler, Alice M M Ornelas, Andrea S de Souza, Mauro Jorge Cabral-Castro, Jessyca T M A Ramos, Carolina Rosadas, Maria Cecilia F Salgado, Alexandre A Castiglione, Fernando Ferry, Jose Mauro Peralta, Carolina Moreira Voloch, Amilcar Tanuri, Fernanda Tovar-Moll, Renato Santana Aguiar
Dengue virus (DENV) causes immune-mediated diseases. Neurological involvement represents a severe condition that is rarely observed in DENV-1 infection. Neuromyelitis optica (NMO)/NMO spectrum disorders (NMOSD) are idiopathic immune-mediated demyelinating syndromes of the central nervous system. We report a 17-year-old female with oligosymptomatic DENV-1 viremia, diagnosed as NMOSD. Magnetic resonance imaging showed spinal cord and brainstem lesions. Antibody for aquaporin 4 was negative. DENV-1 RNA infection was detected by serial RT-PCR and confirmed by phylogenetic analysis in serum...
July 26, 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/28746356/metabolomic-profiling-of-csf-in-multiple-sclerosis-and-neuromyelitis-optica-spectrum-disorder-by-nuclear-magnetic-resonance
#17
Hyun-Hwi Kim, In Hye Jeong, Ja-Shil Hyun, Byung Soo Kong, Ho Jin Kim, Sung Jean Park
Multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) are inflammatory diseases of the central nervous system. Although several studies have characterized the metabolome in the cerebrospinal fluid (CSF) from MS and NMOSD patients, comparative analyses between them and between the relapse and the remission of each disease have not been performed. Both univariate and multivariate analyses were used to compare 1H-NMR spectra of CSF from MS, NMOSD, and healthy controls (HCs). The statistical analysis showed alterations of eight metabolites that were dependent on the disease...
2017: PloS One
https://www.readbyqxmd.com/read/28725551/cervical-cord-myelin-water-imaging-shows-degenerative-changes-over-one-year-in-multiple-sclerosis-but-not-neuromyelitis-optica-spectrum-disorder
#18
Anna J E Combes, Lucy Matthews, Jimmy S Lee, David K B Li, Robert Carruthers, Anthony L Traboulsee, Gareth J Barker, Jacqueline Palace, Shannon Kolind
Spinal cord pathology is a feature of both neuromyelitis optica spectrum disorder (NMOSD) and relapsing-remitting multiple sclerosis (MS). While subclinical disease activity has been described in MS using quantitative magnetic resonance imaging measures, current evidence suggests that neurodegeneration is absent between relapses in NMOSD, although most evidence comes from brain studies. We aimed to assess cross-sectional differences and longitudinal changes in myelin integrity in relapse-free MS and NMOSD subjects over one year...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28699327/acute-onset-severe-occipital-neuralgia-associated-with-high-cervical-lesion-in-patients-with-neuromyelitis-optica-spectrum-disorder
#19
Yuichi Hayashi, Akihiro Koumura, Megumi Yamada, Akio Kimura, Toshirou Shibata, Takashi Inuzuka
OBJECTIVE: To address occipital neuralgia in patients with neuromyelitis optica spectrum disorder (NMOSD). BACKGROUND: NMOSD is an inflammatory demyelinating disease that commonly presents with pain; however, headache symptoms have received little attention. METHODS: We presented three cases of NMOSD in which the patients experienced acute-onset, severe, and steroid-responsive occipital neuralgia. All patients provided consent to use their demographic and imaging data retrospectively...
July 2017: Headache
https://www.readbyqxmd.com/read/28684532/longitudinal-analysis-of-myelin-oligodendrocyte-glycoprotein-antibodies-in-cns-inflammatory-diseases
#20
Jae-Won Hyun, Mark R Woodhall, Su-Hyun Kim, In Hye Jeong, Byungsoo Kong, Gayoung Kim, Yeseul Kim, Min Su Park, Sarosh R Irani, Patrick Waters, Ho Jin Kim
BACKGROUND: We evaluated the seroprevalence of myelin oligodendrocyte glycoprotein immunoglobulin G1 (MOG-IgG) and associated clinical features of patients from a large adult-dominant unselected cohort with mainly relapsing central nervous system (CNS) inflammatory diseases. We also investigate the clinical relevance of MOG-IgG through a longitudinal analysis of serological status over a 2-year follow-up period. METHODS: Serum samples from 505 patients with CNS inflammatory diseases at the National Cancer Center were analysed using cell-based assays for MOG-IgG and aquaporin-4 immunoglobulin G (AQP4-IgG)...
July 6, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
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