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https://www.readbyqxmd.com/read/28780848/-clinical-characteristics-of-neuromyelitis-optica-spectrum-disorders-associated-with-syringomyelia
#1
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/28768356/-singultus-diagnostic-workup-and-therapy
#2
Ohlrich Marcus, Georg Royl
A hiccup is a reflex movement with diffusely distributed afferents and efferents in the thorax; its functional relevance is controversial. In its physiological form, it is mostly a minor complaint that stops spontaneously and rarely leads to medical consultation. However, prolonged agonizing hiccups represent serious deterioration of quality of life. Chronic hiccups by definition last for more than 48 h, with gastroesophageal reflux being the frequent underlying disease. Various other causes affect multiple organ systems, some with serious underlying diseases...
July 2017: Laryngo- Rhino- Otologie
https://www.readbyqxmd.com/read/28755680/capillary-leak-syndrome-in-neuromyelitis-optica-treated-with-rituximab
#3
Ignacio Fuentes Fernandez, Rocio Hernandez-Clares, Ester Carreón Guarnizo, Jose E Meca Lallana
61-year-old woman with Neuromyelitis optica (NMO) diagnosis treated with rituximab was referred to our hospital with severe hypovolemic shock and anasarca. The laboratory findings showed marked hemoconcentration and a decrease in total serum protein. She developed a multiple organ failure and died three hours later. We diagnosed the patient as having capillary leak syndrome (CLS). CLS is a very rare condition caused by unexplained episodic capillary hyperpermeability, which can be idiopathic or secondary to some conditions like infection, malignant disease and some drugs like monoclonal antibodies...
August 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28750658/complement-regulator-cd59-prevents-peripheral-organ-injury-in-rats-made-seropositive-for-neuromyelitis-optica-immunoglobulin-g
#4
Xiaoming Yao, Alan S Verkman
Pathogenesis in aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive neuromyelitis optica spectrum disorders (herein called NMO) involves complement-dependent cytotoxicity initiated by AQP4-IgG binding to astrocyte AQP4. We recently reported that rats lacking complement inhibitor protein CD59 were highly susceptible to development of NMO pathology in brain and spinal cord following direct AQP4-IgG administration (Yao and Verkman, Acta Neuropath Commun 2017, 5:15). Here, we report evidence that CD59 is responsible for protection of peripheral, AQP4-expressing tissues in seropositive NMO...
July 27, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28748851/insights-into-initial-demyelinating-episodes-of-central-nervous-system-during-puerperium
#5
Qian Wu, Bo Chen, Na Liu, Yang Hu, Chao Pan, Ping Zhang, Zhou-Ping Tang, Bi-Tao Bu
BACKGROUND: Inflammatory demyelinating disease of central nervous system (CNS) is an inflammatory disease characterized by a high childbearing female predominance. Labor-related alterations for postpartum demyelinating attacks are not entirely clear. This study aimed to summarize clinical features of female patients of reproductive age with initial CNS inflammatory demyelinating attacks during puerperium. METHODS: Fourteen female patients with initial demyelinating events during puerperium between January 2013 and December 2016 were retrospectively studied...
August 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28748449/first-report-of-persistent-dengue-1-associated-autoimmune-neurological-disturbance-neuromyelitis-optica-spectrum-disorder
#6
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/28748216/t-cells-targeting-neuromyelitis-optica-autoantigen-aquaporin-4-cause-paralysis-and-visual-system-injury
#7
COMMENT
Andrés Cruz-Herranz, Sharon A Sagan, Raymond A Sobel, Ari J Green, Scott S Zamvil
Aquaporin-4 (AQP4)-specific antibodies are instrumental in promoting central nervous system (CNS) tissue injury in neuromyelitis optica (NMO), yet evidence indicates that AQP4-specific T cells also have a pivotal role in NMO pathogenesis. Although considerable effort has been devoted to creation of animal models to study how AQP4-specific T cells and antibodies may cooperate in development of both clinical and histologic opticospinal inflammatory disease, the initial attempts were unsuccessful. Recently, it was discovered that T cells from AQP4-deficient (AQP4(-/-)) mice recognize distinct AQP4 epitopes that were not identified previously in wild-type (WT) mice, and that donor Th17 cells from AQP4(-/-) mice that target those novel epitopes could cause paralysis and visual system injury associated with opticospinal inflammation in WT recipient mice...
May 2017: Journal of Nature and Science
https://www.readbyqxmd.com/read/28741987/multimodal-characterization-of-gray-matter-alterations-in-neuromyelitis-optica
#8
Yaou Liu, Xueyan Jiang, Helmut Butzkueven, Yunyun Duan, Jing Huang, Zhuoqiong Ren, Huiqing Dong, Fu-Dong Shi, Frederik Barkhof, Kuncheng Li, Jinhui Wang
OBJECTIVE: To investigate structural and functional alterations of gray matter (GM) and examine their clinical relevance in neuromyelitis optica (NMO) using multimodal magnetic resonance imaging (MRI) techniques. METHODS: A total of 35 NMO and 36 healthy controls (HC) were recruited in this study. Cortical lesions were investigated by double inversion recovery technique. Five voxel-wise MRI measurements were obtained for each participant in the GM including gray matter volume (GMV), fractional anisotropy (FA), mean diffusivity (MD), amplitude of low-frequency fluctuation (ALFF), and weighted functional connectivity strength (wFCS)...
July 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28736276/b-cells-as-therapeutic-targets-in-neuro-inflammatory-diseases
#9
Reinhard Hohlfeld
B cells are an emerging therapeutic target in neuroinflammatory diseases. The anti-CD20 monoclonal antibody ocrelizumab was recently approved in the US as the first B-cell targeting immunomodulatory treatment for relapsing-remitting MS and primary progressive MS. In autoantibody-associated demyelinating syndromes such as neuromyelitis optica (NMO) and in myelin-oligodendrocyte-glycoprotein-(MOG)-autoantibody-associated encephalomyelitis, B-cells are a logical target based on the pathogenesis of these antibody-mediated disorders...
July 21, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28716289/devic-s-disease-before-devic-on-the-contribution-of-friedrich-albin-schanz-1863-1923
#10
S Jarius, B Wildemann
Neuromyelitis optica (NMO, Devic's disease) is an autoimmune disorder of the CNS that mainly affects the optic nerves and spinal cord and, if not treated, can leave patients blind and wheelchair-bound. NMO was long considered a clinical variant of multiple sclerosis (MS). While the history of MS has been studied extensively, relatively little is known about the early history of NMO. A dissertation written by Fernand Gault (1873-1936), a doctoral student to Eugène Devic (1858-1930), a Lyon-based neurologist after whom the disease would later be named, is considered by many the first review of NMO...
August 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28679661/glucose-regulated-protein-78-autoantibody-associates-with-blood-brain-barrier-disruption-in-neuromyelitis-optica
#11
Fumitaka Shimizu, Kristin L Schaller, Gregory P Owens, Anne C Cotleur, Debra Kellner, Yukio Takeshita, Birgit Obermeier, Thomas J Kryzer, Yasuteru Sano, Takashi Kanda, Vanda A Lennon, Richard M Ransohoff, Jeffrey L Bennett
Neuromyelitis optica (NMO) is an inflammatory disorder mediated by antibodies to aquaporin-4 (AQP4) with prominent blood-brain barrier (BBB) breakdown in the acute phase of the disease. Anti-AQP4 antibodies are produced mainly in the periphery, yet they target the astrocyte perivascular end feet behind the BBB. We reasoned that an endothelial cell-targeted autoantibody might promote BBB transit of AQP4 antibodies and facilitate NMO attacks. Using monoclonal recombinant antibodies (rAbs) from patients with NMO, we identified two that strongly bound to the brain microvascular endothelial cells (BMECs)...
July 5, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28667482/different-patterns-of-longitudinal-brain-and-spinal-cord-changes-and-their-associations-with-disability-progression-in-nmo-and-ms
#12
Yaou Liu, Yunyun Duan, Jing Huang, Zhuoqiong Ren, Zheng Liu, Huiqing Dong, Florian Weiler, Horst K Hahn, Fu-Dong Shi, Helmut Butzkueven, Frederik Barkhof, Kuncheng Li
OBJECTIVE: To investigate the longitudinal spinal cord and brain changes in neuromyelitis optica (NMO) and multiple sclerosis (MS) and their associations with disability progression. PATIENTS AND METHODS: We recruited 28 NMO, 22 MS, and 20 healthy controls (HC), who underwent both spinal cord and brain MRI at baseline. Twenty-five NMO and 20 MS completed 1-year follow-up. Baseline spinal cord and brain lesion loads, mean upper cervical cord area (MUCCA), brain, and thalamus volume and their changes during a 1-year follow-up were measured and compared between groups...
June 30, 2017: European Radiology
https://www.readbyqxmd.com/read/28642888/aquaporin-4-antibodies-in-patients-treated-with-natalizumab-for-suspected-ms
#13
Anna Gahlen, Anne-Kathrin Trampe, Steffen Haupeltshofer, Marius Ringelstein, Orhan Aktas, Achim Berthele, Brigitte Wildemann, Ralf Gold, Sven Jarius, Ingo Kleiter
OBJECTIVE: To evaluate (1) the frequency of aquaporin-4 antibody (AQP4-ab)-seropositive cases among patients treated with natalizumab (NAT) and previously diagnosed with MS (MS(NAT)) in a nationwide cohort, (2) the clinical course of NAT-treated AQP4-ab-seropositive neuromyelitis optica spectrum disorder (NMOSD) patients (NMO(NAT)), (3) AQP4-ab titers in NMO(NAT) and AQP4-ab-seropositive NMOSD treated with other immunotherapies (NMO(IT)), and (4) immune mechanisms influencing disease activity in NMO(NAT)...
July 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28640199/the-role-of-peripheral-cns-directed-antibodies-in-promoting-inflammatory-cns-demyelination
#14
REVIEW
Silke Kinzel, Martin S Weber
In central nervous system (CNS) demyelinating disorders, such as multiple sclerosis (MS), neuromyelitis optica (NMO) and related NMO-spectrum disorders (NMO-SD), a pathogenic role for antibodies is primarily projected into enhancing ongoing CNS inflammation by directly binding to target antigens within the CNS. This scenario is supported at least in part, by antibodies in conjunction with complement activation in the majority of MS lesions and by deposition of anti-aquaporin-4 (AQP-4) antibodies in areas of astrocyte loss in patients with classical NMO...
June 22, 2017: Brain Sciences
https://www.readbyqxmd.com/read/28639937/a-multicentre-randomised-controlled-trial-of-intravenous-immunoglobulin-compared-with-standard-therapy-for-the-treatment-of-transverse-myelitis-in-adults-and-children-strive
#15
Michael Absoud, Peter Brex, Olga Ciccarelli, Onyinye Diribe, Gavin Giovannoni, Jennifer Hellier, Rosemary Howe, Rachel Holland, Joanna Kelly, Paul McCrone, Caroline Murphy, Jackie Palace, Andrew Pickles, Michael Pike, Neil Robertson, Anu Jacob, Ming Lim
BACKGROUND: Transverse myelitis (TM) is an immune-mediated disorder of the spinal cord that affects adults and children and that causes motor, sensory and autonomic dysfunction. There is a prolonged recovery phase, which may continue for many years. Neuromyelitis optica (NMO) is an uncommon relapsing inflammatory central nervous system condition in which TM can be the first presenting symptom. As TM and NMO affect many patients in the prime of their working life, the disorder can impose a significant demand on health resources...
May 2017: Health Technology Assessment: HTA
https://www.readbyqxmd.com/read/28638708/citation-classics-in-central-nervous-system-inflammatory-demyelinating-disease
#16
Jee-Eun Kim, Kang M Park, Yerim Kim, Dae Y Yoon, Jong S Bae
OBJECTIVES: To identify and analyze the characteristics of the most influential articles about central nervous system (CNS) inflammatory demyelinating disease. MATERIALS AND METHODS: The Institute for Scientific Information (ISI) Web of Science database and the 2014 Journal Citation Reports Science Edition were used to retrieve the top 100 cited articles on CNS inflammatory demyelinating disease. The citation numbers, journals, years of publication, authorships, article types, subjects and main issues were analyzed...
June 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28637379/neuropsychological-outcomes-of-pediatric-demyelinating-diseases-a-review
#17
Alexander Tan, Cole Hague, Benjamin M Greenberg, Lana Harder
Immune-mediated central nervous system (CNS) demyelinating diseases impact various areas of the brain, optic nerves, and/or spinal cord and can result in a wide range of neurologic symptoms including adverse cognitive outcomes. Neuropsychological outcomes in adult multiple sclerosis (MS) are well documented, while literature on such outcomes in pediatric cohorts is more limited. Furthermore, literature on neuropsychological outcomes in pediatric acute disseminated encephalomyelitis (ADEM), neuromyelitis optica (NMO), and transverse myelitis (TM) is even more limited...
June 21, 2017: Child Neuropsychology: a Journal on Normal and Abnormal Development in Childhood and Adolescence
https://www.readbyqxmd.com/read/28619428/neuromyelitis-optica-presenting-with-horner-syndrome-a-case-report-and-review-of-literature
#18
İrem Fatma Uludağ, Alp Sarıteke, Levent Öcek, Yaşar Zorlu, Ufuk Şener, Figen Tokuçoğlu, Burhanettin Uludağ
Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. We describe a 19 years old woman with left Horner syndrome (HS), who was diagnosed as NMO with characteristic longitudinally extensive myelitis and positive serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Our report describes one of the very rare ocular motor symptoms in NMO patients.
May 2017: Multiple Sclerosis and related Disorders
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
#19
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/28601281/association-of-cd58-gene-polymorphisms-with-nmo-spectrum-disorders-in-a-han-chinese-population
#20
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
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