Imaging Surrogates of Disease Activity in Neuromyelitis Optica Allow Distinction from Multiple Sclerosis.

Science.gov (United States)

Matthews, Lucy; Kolind, Shannon; Brazier, Alix; Leite, Maria Isabel; Brooks, Jonathan; Traboulsee, Anthony; Jenkinson, Mark; Johansen-Berg, Heidi; Palace, Jacqueline

2015-01-01

Inflammatory demyelinating lesions of the central nervous system are a common feature of both neuromyelitis optica and multiple sclerosis. Despite this similarity, it is evident clinically that the accumulation of disability in patients with neuromyelitis optica is relapse related and that a progressive phase is very uncommon. This poses the question whether there is any pathological evidence of disease activity or neurodegeneration in neuromyelitis optica between relapses. To investigate this we conducted a longitudinal advanced MRI study of the brain and spinal cord in neuromyelitis optica patients, comparing to patients with multiple sclerosis and controls. We found both cross-sectional and longitudinal evidence of diffusely distributed neurodegenerative surrogates in the multiple sclerosis group (including thalamic atrophy, cervical cord atrophy and progressive widespread diffusion and myelin water imaging abnormalities in the normal appearing white matter) but not in those with neuromyelitis optica, where localised abnormalities in the optic radiations of those with severe visual impairment were noted. In addition, between relapses, there were no new silent brain lesions in the neuromyelitis optica group. These findings indicate that global central nervous system neurodegeneration is not a feature of neuromyelitis optica. The work also questions the theory that neurodegeneration in multiple sclerosis is a chronic sequela to prior inflammatory and demyelinating pathology, as this has not been found to be the case in neuromyelitis optica where the lesions are often more destructive.

Brain MRI lesions in neuromyelitis optica: clinical case

International Nuclear Information System (INIS)

Rosales Bravo, Luis Guillermo; Heyden Cordero, Marvin; Chinchilla Weinstok, Dennis; Mendelewicz Goldwaig, Isaias

2011-01-01

Many cases of patients with neuromyelitis optica have submitted without demyelinating lesions in the cerebral white matter, it has documented that this entity can cause from the onset of illness or through its natural evolution. Diagnostic methods currently as Magnetic Resonance Imaging (MRI) and specific antibodies in plasma (such as antiaquaporin-4) have been diagnosed of neuromyelitis optica cases that were initially confused with multiple sclerosis. Disease in Costa Rica has been little prevalent and is not exactly known what the prevalence and incidence. The degree of disorder is illustrated through a case study, both in the cerebral white matter as spinal cord, in a patient with neuromyelitis optica during a follow-up period of 4 years. This is the first case that has been reported in the scientific literature of Costa Rica. (author) [es

Imaging Surrogates of Disease Activity in Neuromyelitis Optica Allow Distinction from Multiple Sclerosis.

Directory of Open Access Journals (Sweden)

Lucy Matthews

Full Text Available Inflammatory demyelinating lesions of the central nervous system are a common feature of both neuromyelitis optica and multiple sclerosis. Despite this similarity, it is evident clinically that the accumulation of disability in patients with neuromyelitis optica is relapse related and that a progressive phase is very uncommon. This poses the question whether there is any pathological evidence of disease activity or neurodegeneration in neuromyelitis optica between relapses. To investigate this we conducted a longitudinal advanced MRI study of the brain and spinal cord in neuromyelitis optica patients, comparing to patients with multiple sclerosis and controls. We found both cross-sectional and longitudinal evidence of diffusely distributed neurodegenerative surrogates in the multiple sclerosis group (including thalamic atrophy, cervical cord atrophy and progressive widespread diffusion and myelin water imaging abnormalities in the normal appearing white matter but not in those with neuromyelitis optica, where localised abnormalities in the optic radiations of those with severe visual impairment were noted. In addition, between relapses, there were no new silent brain lesions in the neuromyelitis optica group. These findings indicate that global central nervous system neurodegeneration is not a feature of neuromyelitis optica. The work also questions the theory that neurodegeneration in multiple sclerosis is a chronic sequela to prior inflammatory and demyelinating pathology, as this has not been found to be the case in neuromyelitis optica where the lesions are often more destructive.

A population-based study of neuromyelitis optica in Caucasians

DEFF Research Database (Denmark)

Asgari, N; Lillevang, S T; Skejoe, H P B

2011-01-01

Epidemiologic studies have suggested different prevalence of neuromyelitis optica (NMO) in different ethnic groups. However, data on the incidence and prevalence of NMO in Caucasians are scarce.......Epidemiologic studies have suggested different prevalence of neuromyelitis optica (NMO) in different ethnic groups. However, data on the incidence and prevalence of NMO in Caucasians are scarce....

[A treatment of neuromyelitis optica (Devic's disease) during pregnancy].

Science.gov (United States)

Daouda, Moussa Toudou; Obenda, Norlin Samuel; Assadeck, Hamid; Camara, Diankanagbe; Djibo, Fatimata Hassane

2016-01-01

Neuromyelitis optica (Devic's disease) is an inflammatory demyelinating disease of the central nervous system that mainly affects spinal cord, optic nerve and brain regions with high aquaporin 4 antigen expression. This is a severe autoimmune disease caused by autoantibodies directed against aquaporin 4 and associated with high morbidity and mortality. Unlike other inflammatory conditions such as multiple sclerosis or rheumatoid polyarthritis, pregnancy does not seem to influence the activity of neuromyelitis optica, hence the need for a thorough treatment during pregnancy. Corticosteroid therapy is the treatment of choice for neuromyelitis optica during pregnancy. Other treatments may also be used including rituximab, some immunosuppressive agents and immunoglobulins. Immunosuppressive treatment or rituximab is recommended when the long-term corticosteroid treatment is contraindicated, in case of inefficiency or if side effects are intolerable. Immunoglobulins are administered to patients with serious outbreaks of neuromyelitis optica which do not respond to bolus methylprednisolone. Immunoglobulins alone can also be continued at a dose of 0.4 g/kg/day for 6-8 weeks until delivery. Plasmapheresis is also a good alternative to bolus methylprednisolone when outbreaks are extremely severe.

Clinical evaluation of rituximab treatment for neuromyelitis optica.

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Fernández-Megía, M J; Casanova-Estruch, B; Pérez-Miralles, F; Ruiz-Ramos, J; Alcalá-Vicente, C; Poveda-Andrés, J L

2015-10-01

Neuromyelitis optica is an inflammatory and usually relapsing demyelinating autoimmune disease of the central nervous system that targets the optic nerves and spinal cord. Rituximab has been used for different neurological diseases that are probably immune-mediated or involving humoural immunity. The objective of this study is to evaluate the efficacy and safety of rituximab as treatment for neuromyelitis optica in a tertiary hospital. Retrospective study of patients with neuromyelitis optica treated with rituximab 1000mg on days 1 and 15, repeated every 6 to 8 months. We recorded EDSS score, relapse rate, overall condition, CD19+ count, presence of anti-NMO antibodies, and possible adverse reactions. Six patients were treated; all were women with a median age of 46 years (range, 38-58). Anti-NMO antibodies were detected in 3 patients (50%). Baseline EDSS was 4 (range 2.0-5.5). Two patients had previously been treated with an immunomodulatory drug. Median time from the first rituximab infusion to first relapse was 3.7 years (range 1.7-6.9). Two patients had infusion reactions after the first dose of rituximab. Four patients remained relapse-free and their EDSS score did not progress during rituximab treatment, one patient showed no clinical improvement, and one patient could not be evaluated. Rituximab can be considered an attractive therapeutic alternative for patients with neuromyelitis optica as there are no approved treatments for this disease. Further studies with rituximab are needed to establish the role of this drug in treating neuromyelitis optica. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.

Neuromyelitis Optica and Neuromyelitis Optica Spectrum Disorder Patients in Turkish Cohort: Demographic, Clinical, and Laboratory Features.

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Altintas, Ayse; Karabudak, Rana; Balci, Belgin P; Terzi, Murat; Soysal, Aysun; Saip, Sabahattin; Tuncer Kurne, Asli; Uygunoglu, Ugur; Nalbantoglu, Mecbure; Gozubatik Celik, Gokcen; Isik, Nihal; Celik, Yahya; Gokcay, Figen; Duman, Taskin; Boz, Cavit; Yucesan, Canan; Mangan, Mehmet Serhat; Celebisoy, Nese; Diker, Sevda; Colpak Isikay, Ilksen; Kansu, Tulay; Siva, Aksel

2015-10-01

Neuromyelitis optica (NMO) is an immune-mediated, chronic relapsing, inflammatory disease characterized by severe attacks of optic neuritis and myelitis. To determine the demographic, clinical, and laboratory features; antibody status; and treatment modalities of patients with NMO and neuromyelitis optica spectrum disorders in a Turkish cohort from 11 centers. A total of 182 patients were included in this study. Data on age at disease onset, sex, type of attacks, clinical presentation, analysis of cerebrospinal fluid, serum antiaquaporin-4 antibody status, annual progression index, and medical and family histories were collected. Mean age was 38.43±12.40 years (range, 13 to 75 y), and mean age at disease onset was 31.29±12.40 years (median, 29 y; range, 10 to 74 y). In NMO group, the rate of NMO immunoglobulin (Ig)G positivity was 62.5%. The annual progression index was significantly higher in the longitudinally extending spinal cord lesion. The mean Expanded Disability Status Scale score was higher in the late than early-onset NMO group. Our results revealed a lower rate of NMO IgG positivity, more severe disability in patients with NMO/neuromyelitis optica spectrum disorders presenting with either transverse myelitis or late-onset NMO, and no correlation between disability and NMO IgG status.

Imaging Differences between Neuromyelitis Optica Spectrum Disorders and Multiple Sclerosis: A Multi-Institutional Study in Japan.

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Tatekawa, H; Sakamoto, S; Hori, M; Kaichi, Y; Kunimatsu, A; Akazawa, K; Miyasaka, T; Oba, H; Okubo, T; Hasuo, K; Yamada, K; Taoka, T; Doishita, S; Shimono, T; Miki, Y

2018-05-03

Both clinical and imaging criteria must be met to diagnose neuromyelitis optica spectrum disorders and multiple sclerosis. However, neuromyelitis optica spectrum disorders are often misdiagnosed as MS because of an overlap in MR imaging features. The purpose of this study was to confirm imaging differences between neuromyelitis optica spectrum disorders and MS with visually detailed quantitative analyses of large-sample data. We retrospectively examined 89 consecutive patients with neuromyelitis optica spectrum disorders (median age, 51 years; range, 16-85 years; females, 77; aquaporin 4 immunoglobulin G-positive, 93%) and 89 with MS (median age, 36 years; range, 18-67 years; females, 68; relapsing-remitting MS, 89%; primary-progressive MS, 7%; secondary-progressive MS, 2%) from 9 institutions across Japan (April 2008 to December 2012). Two neuroradiologists visually evaluated the number, location, and size of all lesions using the Mann-Whitney U test or the Fisher exact test. We enrolled 79 patients with neuromyelitis optica spectrum disorders and 87 with MS for brain analysis, 57 with neuromyelitis optica spectrum disorders and 55 with MS for spinal cord analysis, and 42 with neuromyelitis optica spectrum disorders and 14 with MS for optic nerve analysis. We identified 911 brain lesions in neuromyelitis optica spectrum disorders, 1659 brain lesions in MS, 86 spinal cord lesions in neuromyelitis optica spectrum disorders, and 102 spinal cord lesions in MS. The frequencies of periventricular white matter and deep white matter lesions were 17% and 68% in neuromyelitis optica spectrum disorders versus 41% and 42% in MS, respectively (location of brain lesions, P optica spectrum disorders (cervical versus thoracic, 29% versus 71%), whereas they were equally distributed in MS (46% versus 54%). Furthermore, thoracic lesions were significantly longer than cervical lesions in neuromyelitis optica spectrum disorders ( P = .001), but not in MS ( P = .80). Visually detailed

Neuromyelitis optica-like pathology is dependent on type I interferon response

DEFF Research Database (Denmark)

Khorooshi, Reza; Wlodarczyk, Agnieszka; Asgari, Nasrin

2013-01-01

Neuromyelitis optica is an antibody-mediated autoimmune inflammatory disease of the central nervous system. Reports have suggested that interferon beta which is beneficial for multiple sclerosis, exacerbates neuromyelitis optica. Our aim was to determine whether type I interferon plays a role in ...

Pediatric familial neuromyelitis optica in two sisters with long term follow-up.

Science.gov (United States)

Chuquilin, Miguel; Mullaguri, Naresh; Weinshenker, Brian

2016-07-01

Neuromyelitis optica causes bilateral optic neuritis and longitudinal extensive transverse myelitis. Although usually sporadic, 3% of cases of neuromyelitis optica are familial. The interval over which attacks continue and the long term prognosis for pediatric-onset neuromyelitis optica are not well defined. We describe two patients with pediatric familial neuromyelitis optica with the longest clinical follow-up of a pediatric case reported in the literature to our knowledge. One woman developed blindness with bilateral eye involvement within a few weeks at age 3. This was followed by transverse myelitis with paraparesis at age 19 leading to diagnosis of neuromyelitis optica. Her serum anti-aquaporin 4 antibody was later found to be positive. She continued with sporadic myelitis-related relapses but remained ambulant until age 40 when she had a more severe relapse. There was evidence of longitudinal extensive T2 hyperintensity in the thoracic spinal cord. Her sister also developed blindness at age 3.5 followed by myelitis 1year later with multiple relapses of gait impairment until her death from pneumonia at age 21. These patients represent the rare occurrence of neuromyelitis optica in children within the same family and show that this disease can have prolonged periods of remission but a continued tendency to relapse, supporting the need for lifelong immunosuppression. Copyright © 2016 Elsevier Ltd. All rights reserved.

Individualized rituximab treatment for relapsing neuromyelitis optica: a pediatric case report.

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He, Dian; Yu, YunLi; Yan, WeiBo; Dai, QingQing; Xu, Zhu; Chu, Lan

2014-08-01

Neuromyelitis optica is an autoimmune inflammatory disorder of the central nervous system. Current therapeutic approaches are based on small uncontrolled trials, case series, or case reports. There are only a few case reports describing rituximab for pediatric neuromyelitis optica. A 7-year-old girl with neuromyelitis optica had high disease activity with recurrent myelitis and steroid dependence. A remarkable increase of CD19(+) B-cell count in the peripheral blood mononuclear cells and seropositivity for anti-aquaporin 4 antibody were detected at each attack. After induction therapy with rituximab, the CD19(+) B-cell number was significantly reduced and sustained at low levels. The level of serum anti-aquaporin 4 antibody normalized. She was relapse-free over 1-year follow-up period. An individualized maintenance therapy scheme is underway. Treatment with rituximab for relapsing neuromyelitis optica requires an individualized regimen to optimize the frequency and dosage of administration to maximize efficacy yet minimize overtreatment and cost. Personal levels of CD19(+) B cells in peripheral blood mononuclear cells at previous attacks and responsiveness to rituximab in induction therapy may be two useful indicators in establishing individualized maintenance therapy schemes for relapsing neuromyelitis optica. Copyright © 2014 Elsevier Inc. All rights reserved.

Paucity of natural killer and cytotoxic T cells in human neuromyelitis optica lesions

Science.gov (United States)

Saadoun, Samira; Bridges, Leslie R.; Verkman, A. S.; Papadopoulos, Marios C.

2013-01-01

Neuromyelitis optica is a severe inflammatory demyelinating disease of the central nervous system. Most patients with neuromyelitis optica have circulating immunoglobulin G (IgG) antibodies against the astrocytic water channel protein aquaporin-4 (AQP4), which are pathogenic. Anti-AQP4 IgG-mediated complement-dependent astrocyte toxicity is a key mechanism of central nervous system damage in neuromyelitis optica, but the role of natural killer and cytotoxic T cells is unknown. Our objective was to determine whether natural killer and cytotoxic T cells play a role in human neuromyelitis optica lesions. We immunostained four actively demyelinating lesions, obtained from patients with anti-AQP4 IgG positive neuromyelitis optica, for Granzyme B and Perforin. The inflammatory cells were perivascular neutrophils, eosinophils and macrophages, with only occasional Granzyme B+ or Perforin + cells. Greater than 95% of inflamed vessels in each lesion had no surrounding Granzyme B+ or Perforin + cells. Granzyme B+ or Perforin+ cells were abundant in human spleen (positive control). Although natural killer cells produce central nervous system damage in mice injected with anti-AQP4 IgG, our findings here indicate that natural killer-mediated and T cell-mediated cytotoxicity are probably not involved in central nervous system damage in human neuromyelitis optica. PMID:23108041

Neuromyelitis optica and multiple sclerosis

DEFF Research Database (Denmark)

Bennett, J. L.; de Seze, J.; Lana-Peixoto, M.

2015-01-01

Neuromyelitis optica (NMO) is an inflammatory autoimmune disease of the central nervous system that preferentially targets the optic nerves and spinal cord. The clinical presentation may suggest multiple sclerosis (MS), but a highly specific serum autoantibody against the astrocytic water channel...

Neuromyelitis optica in an adolescent after bone marrow transplantation.

Science.gov (United States)

Baumer, Fiona M; Kamihara, Junne; Gorman, Mark P

2015-01-01

Central nervous system complications of bone marrow transplant are a common occurrence and the differential diagnosis is quite broad, including opportunistic infections, medications toxicities, graft versus host disease, and other autoimmune processes. We summarize previously reported cases of autoimmune myelitis in post-transplant patients and discuss a 17-year-old boy who presented with seronegative neuromyelitis optica after a bone marrow transplant for acute myeloid leukemia. Our patient had a marked improvement in symptoms after plasmapheresis. Including our patient, there have been at least eight cases of post-transplant autoimmune myelitis presented in the literature, and at least three of these are suspicious for neuromyelitis optica. Several of these patients had poor outcomes with persistent symptoms after the myelitis. Autoimmune processes such as neuromyelitis optica should be carefully considered in patients after transplant as aggressive treatment like early plasmapheresis may improve outcomes. Copyright © 2015 Elsevier Inc. All rights reserved.

Hypertrophic pachymeningitis accompanying neuromyelitis optica spectrum disorder: A case report.

Science.gov (United States)

Kon, Tomoya; Nishijima, Haruo; Haga, Rie; Funamizu, Yukihisa; Ueno, Tatsuya; Arai, Akira; Suzuki, Chieko; Nunomura, Jin-ichi; Baba, Masayuki; Takahashi, Toshiyuki; Tomiyama, Masahiko

2015-10-15

We report a case of idiopathic cerebral hypertrophic pachymeningitis accompanying neuromyelitis optica spectrum disorder. No other identifiable cause of pachymeningitis was detected. Corticosteroid therapy was effective for both diseases. Hypertrophic pachymeningitis is closely related to autoimmune inflammatory disease of the central nervous system. This case supports the hypothesis that hypertrophic pachymeningitis can be a rare comorbidity of neuromyelitis optica spectrum disorder. Copyright © 2015. Published by Elsevier B.V.

Immunology of neuromyelitis optica during pregnancy.

OpenAIRE

Bove, Riley; Davoudi, V; Keyhanian, K; Bove, RM; Chitnis, T

2016-01-01

Anti-aquaporin-4 (AQP4) autoantibody plays a key role in the pathogenesis of neuromyelitis optica (NMO). Studies have shown increased relapse rates in patients with NMO during pregnancy and postpartum. High estrogen levels during pregnancy can increase act

Imaging the Visual Pathway in Neuromyelitis Optica

OpenAIRE

Pfueller, Caspar F.; Paul, Friedemann

2011-01-01

The focus of this paper is to summarize the current knowledge on visual pathway damage in neuromyelitis optica (NMO) assessed by magnetic resonance imaging (MRI) and optical coherence tomography (OCT).

Cognitive impairment in Chinese neuromyelitis optica

NARCIS (Netherlands)

Zhang, N.; Li, Y.J.; Fu, Y.; Shao, J.H.; Luo, L.L.; Yang, L.; Shi, F.D.; Liu, Y.

2015-01-01

Background: Cognitive dysfunction is frequently seen in neuromyelitis optica (NMO). However, the features and influencing factors of cognitive impairment of Chinese NMO patients are unclear. Objective: To investigate the patterns of cognitive impairment in Chinese NMO patients, and correlate the

Application of unimodal optic fiber to communications among electric substations; Aplicacion de fibra optica unimodal a comunicaciones entre subestaciones electricas

Energy Technology Data Exchange (ETDEWEB)

Martinez Pinon, Fernando; Hernandez Juarez, Taide [Instituto de Investigaciones Electricas, Cuernavaca (Mexico)

1992-07-01

The utilization of the unimodal fiber optics technology in the electric power systems, represents one of the best communication options because of its multiple advantages, that results in a better coordination of the technical and administrative activities, needed to carry the electric energy from its generation site to the most remote locations. In this document a study, in accordance with the available options for the design of communication systems via unimodal optic fiber, is presented. With this technology, ties of more than 100 kilometers without the need of repeating stations, can be made. [Espanol] La utilizacion de la tecnologia de fibra optica unimodal en los sistemas electricos de potencia representa una de las mejores alternativas de comunicacion por sus multiples ventajas que se traducen en una mejor coordinacion de las acitividades tecnicas y administrativas necesarias para llevar la energia electrica desde el lugar de generacion hasta los puntos mas distantes. En este documento se presenta un estudio de acuerdo a las opciones disponibles para el diseno de sistemas de comunicacion por fibra optica unimodal. Con esta tecnologia se pueden realizar enlaces mayores que 100 km sin necesidad de repetidores.

Neuromyelitis optica antibody in Leber hereditary optic neuropathy: case report

Directory of Open Access Journals (Sweden)

Luciano Mesquita Simão

2012-08-01

Full Text Available Neuromyelitis optica antibody (or aquaporin-4 antibody is a well stablished serum marker associated to high-risk neuromyelitis optica syndrome that presents as an inflammatory demyelinating disease characterized by the occurrence of bilateral and simultaneous optic neuritis without complete visual recovery or it occurs as an isolated episode of transverse myelitis accompanied by longitudinally extensive spinal cord lesions. On the other hand, Leber hereditary optic neuropathy is a primarily hereditary disorder that affects all tissues of the body and its clinical presentation is tissue-specific for the optic nerve and, eventually, it might reach the spinal cord. Overlapping clinical features of neuromyelitis optica and Leber hereditary optic neuropathy may suggest common target organ diseases. The case report described herein emphasizes the coexistence of serum markers of both diseases, and suggests that further investigation of this challenging clinical presentation is warranted to confirm or rule out this association.

Diagnostik og behandling af neuromyelitis optica

DEFF Research Database (Denmark)

Nielsen, Helle Hvilsted; Ravnborg, Mads; Illes, Zsolt

2014-01-01

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease characterized by antibodies against aquaporin-4 in up to 80% of the cases and even less in the NMO spectrum disorders, which may be difficult to distinguish from early multiple sclerosis. While immunosuppressive therapy should...

Interferon alpha association with neuromyelitis optica

DEFF Research Database (Denmark)

Asgari, Nasrin; Voss, Anne; Steenstrup, Troels

2013-01-01

Interferon-alpha (IFN- α ) has immunoregulatory functions in autoimmune inflammatory diseases. The goal of this study was to determine occurrence and clinical consequences of IFN- α in neuromyelitis optica (NMO) patients. Thirty-six NMO and 41 multiple sclerosis (MS) patients from a population...

Subclinical primary retinal pathology in neuromyelitis optica spectrum disorder.

Science.gov (United States)

Jeong, In Hye; Kim, Ho Jin; Kim, Nam-Hee; Jeong, Kyoung Sook; Park, Choul Yong

2016-07-01

Foveal thickness may be a more sensitive indicator of primary retinal pathology than retinal nerve fiber layer thickness since the fovea contains no or sparse retinal nerve fiber layer, which coalesces into axons of the optic nerve. To our knowledge, few quantitative in vivo studies have investigated foveal thickness. By using optical coherence tomography, we measured foveal thickness to evaluate intrinsic retinal pathology. Seventy-two neuromyelitis optica spectrum disorder patients (99 eyes with optic neuritis and 45 eyes without optic neuritis) and 34 age-matched controls were included. Foveal thinning was observed both in eyes with non-optic neuritis (185.1 µm, p optica spectrum disorder, foveal thickness correlated with 2.5 % low contrast visual acuity, while retinal nerve fiber layer thickness correlated with high or low contrast visual acuity, extended disability status scale, and disease duration. In this study, we observed foveal thinning irrespective of optic neuritis; thus, we believe that subclinical primary retinal pathology, prior to retinal nerve fiber layer thinning, may exist in neuromyelitis optica spectrum disorder.

Hydrocephalus in neuromyelitis optica

OpenAIRE

Clardy, Stacey L.; Lucchinetti, Claudia F.; Krecke, Karl N.; Lennon, Vanda A.; O'Toole, Orna; Weinshenker, Brian G.; Boyd, Clara D.; Krieger, Stephen; McGraw, Corey; Guo, Yong; Pittock, Sean J.

2014-01-01

A majority of patients with neuromyelitis optica (NMO) spectrum disorders (NMOSD) have MRI brain abnormalities, some of which are “NMO-typical” with localization in aquaporin 4 (AQP4)–rich circumventricular and periaqueductal regions.1 Although uncommon in adult patients, symptomatic brain involvement occurs in approximately 50% of NMO–immunoglobulin G (IgG) seropositive children. Here we report the clinical characteristics, type, and frequency of hydrocephalus in NMOSD.

Imaging the Visual Pathway in Neuromyelitis Optica

Directory of Open Access Journals (Sweden)

Caspar F. Pfueller

2011-01-01

Full Text Available The focus of this paper is to summarize the current knowledge on visual pathway damage in neuromyelitis optica (NMO assessed by magnetic resonance imaging (MRI and optical coherence tomography (OCT.

Aquaporin-4 serostatus does not predict response to immunotherapy in neuromyelitis optica spectrum disorders.

Science.gov (United States)

Mealy, Maureen A; Kim, Su-Hyun; Schmidt, Felix; López, Reydmar; Jimenez Arango, Jorge A; Paul, Friedemann; Wingerchuk, Dean M; Greenberg, Benjamin M; Kim, Ho Jin; Levy, Michael

2017-08-01

Debate exists about whether neuromyelitis optica spectrum disorder seronegative disease represents the same immune-mediated attack on astrocytic aquaporin-4 as in seropositive disease. We investigated whether response to common treatments for neuromyelitis optica spectrum disorder differed by serostatus, as assessed by change in annualized relapse rate. We performed a multicenter retrospective analysis of 245 patients with neuromyelitis optica spectrum disorder who were treated with either rituximab or mycophenolate mofetil as their first-line immunosuppressive treatment for disease prevention. Patients were followed for a minimum of 6 months following treatment initiation. In those started on rituximab, the pre-treatment annualized relapse rates for seropositive and seronegative patients were 1.81 and 1.93, respectively. On-treatment annualized relapse rates significantly declined to 0.32 (seropositive; p optica spectrum disorder patients, treatment was effective regardless of serostatus. This suggests that treatment should not differ when considering these treatments.

Movement disorders in multiple sclerosis and neuromyelitis optica: A clinical marker of neurological disability.

Science.gov (United States)

Candeias da Silva, Carolina; Bichuetti, Denis Bernardi; Azevedo Silva, Sonia Maria Cesar de; Ferraz, Henrique Ballalai; Oliveira, Enedina Maria Lobato de; Borges, Vanderci

2018-03-03

Movement disorders are not rare in demyelinating diseases but there are few studies comparing their frequency between multiple sclerosis and neuromyelitis optica spectrum disorder. Our aim was to determine the frequency and the related features of movement disorders in a cohort of patients with multiple sclerosis and neuromyelitis optica spectrum disorder. It is a cross-sectional study of patients with multiple sclerosis and neuromyelitis optica spectrum disorder. Patients were evaluated by a movement disorder specialist. Data from a personal interview and neurological examination were collected. Fahn-Tolosa-Marin tremor rating scale was used for tremor evaluation. Health-related quality of life was assessed using EuroQol instrument. Two hundred fifty-three patients were included (mean [SD] age, 40 [12] years; 74.3% female; median [IQR] EDSS score 2.5 [1.0-6.0]); 26% presented with movement disorders. Paroxysmal dystonia (n = 32) and tremor (n = 27) were the most common movement disorders. Patients with multiple sclerosis and low Expanded Disability Status Scale score (below 4.0) have fewer movement disorders than patients with neuromyelitis optica spectrum disorder. The diagnosis of neuromyelitis optica spectrum disorder was strongly associated with paroxysmal dystonia (OR = 22.07, 95% CI = 2.56-189.78; p = 0.005). Patients with multiple sclerosis and patients without movement disorders have a slightly better quality of life. Paroxysmal dystonia was the most common movement disorder in demyelinating diseases and strongly associated with neuromyelitis optica spectrum disorder. Copyright © 2018 Elsevier Ltd. All rights reserved.

Suspected bacterial meningomyelitis: The first presenting clinical feature of neuromyelitis optica spectrum disorder.

Science.gov (United States)

Li, Xiang; Lin, Jie; Pan, Sipei; Weng, Yiyun; Li, Jia; Zhang, Xu; Xia, Junhui; Tong, Qiaowen

2017-08-15

A rare case of neuromyelitis optica spectrum disorder, suspected to be bacterial meningomyelitis as the initial manifestation, is reported. The patient presented with initial symptoms of meningomyelitis and fever. Cerebrospinal fluid analysis revealed pleocytosis (1280×10 6 /L [98% lymphocytes]) and glucose level of 1.8mmol/L. Magnetic resonance imaging revealed >3 vertebral, longitudinally extensive transverse myelitis and area postrema lesions. Right optic neuritis was experienced 20months after the first attack. Serum anti-aquaporin-4 antibody was positive, and a diagnosis of neuromyelitis optica spectrum disorder was made, supporting the hypothesis that the pathogenesis of neuromyelitis optica is triggered by infection. Copyright © 2017. Published by Elsevier B.V.

Transverse myelitis associated with an itchy rash and hyperckemia: neuromyelitis optica associated with dermatitis herpetiformis.

Science.gov (United States)

Iyer, Anand; Rathnasabapathi, Devipriya; Elsone, Liene; Mutch, Kerry; Terlizzo, Monica; Footitt, David; Jacob, Anu

2014-05-01

Neuromyelitis optica is associated with severe neurodisability if not recognized and treated promptly. Several autoimmune disorders are associated with this condition and may vary in their presentation. It is essential that clinicians are aware of the uncommon presenting features of neuromyelitis optica and associated autoimmune conditions. A 53-year-old woman presented with nausea and vomiting and was noted to have an asymptomatic elevated creatinine kinase level, which improved with conservative management. She had a history of iron-deficiency anemia due to long-standing celiac disease that was managed with a gluten-free diet. She then presented with recurrent transverse myelitis and a vesicobullous rash over her arms and feet that was pruritic and excoriating. Skin biopsy results confirmed a clinical diagnosis of dermatitis herpetiformis and antibody test findings against aquaporin-4 were positive, leading to a diagnosis of neuromyelitis optica spectrum disorder. She was treated with methylprednisolone sodium succinate, plasma exchange, and azathioprine and has remained in remission. This report highlights the association of neuromyelitis optica with dermatitis herpetiformis, which can present even without clinical features of celiac disease. Nausea, vomiting, and asymptomatic hyperCKemia should be recognized as rare presenting features of neuromyelitis optica.

Olfactory dysfunction in neuromyelitis optica spectrum disorders

NARCIS (Netherlands)

Zhang, L.J.; Zhao, N.; Fu, Y.; Zhang, D.Q.; Wang, J.; Qin, W.; Zhang, N.N.N.; Wood, K.; Liu, Y.; Yu, C.S.; Shi, F.D.; Yang, L.

2015-01-01

Few data were available for the understanding of olfactory function in neuromyelitis optica spectrum disorders (NMOSDs). The aims of our study were to investigate the incidence of olfactory dysfunction and characterize olfactory structures, using MRI, in patients with NMOSDs. Olfactory function was

Neuro-ophthalmic manifestation of neuromyelitis optica spectrum disorders

Directory of Open Access Journals (Sweden)

Xiao-jun ZHANG

2014-10-01

Full Text Available Neuromyelitis optica spectrum disorders (NMOSDs include classic neuromyelitis optica (NMO, opticospinal multiple sclerosis (OSMS, limited form of NMO and isolated optic neuritis or myelitis accompanied by either systemic autoimmune diseases or typical MRI findings of NMO. The common neuro-ophthalmic features of NMOSDs include simultaneous or consecutive bilateral optic neuritis, more commonly seen optic disk edema and surrounding exudate, poor visual recovery, steroid responsiveness and dependency. Combined with serum aquaporin 4 (AQP4 antibody and brain MRI examination, these clinical features can be helpful to the early differential diagnosis between NMOSDs and MS. Some types of eye movement abnormalities have been reported in patients with NMOSDs, but further investigation needs to be done before the specificity of these features are confirmed. doi: 10.3969/j.issn.1672-6731.2014.10.003

Serological prevalence of celiac disease in Brazilian population of multiple sclerosis, neuromyelitis optica and myelitis.

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de Oliveira, Pérola; de Carvalho, Daniel Rocha; Brandi, Ivar Viana; Pratesi, Riccardo

2016-09-01

Comorbidity of celiac disease with demyelinating diseases of the central nervous system has been reported since the 1960s. The objective of this study was to determine the serological prevalence of celiac disease in the largest series of patients diagnosed with multiple sclerosis, neuromyelitis optica, or myelitis. A prevalence study was conducted with patients evaluated at Sarah Network of Rehabilitation Hospitals between March 2012 and September 2013. They were previously diagnosed with multiple sclerosis, neuromyelitis optica, or idiopathic myelitis. The serum levels of antibodies against tissue transglutaminase and endomysium were assessed. Of the 379 patients evaluated, 249 (65.70%) were diagnosed with multiple sclerosis, 37 (9.56%) with neuromyelitis optica, and 96 (24.54%) with idiopathic myelitis. Two patients (0.53%), one with multiple sclerosis and other with myelitis, tested positive for both antibodies. Our study do not confirm the relationship between celiac serological antibodies with multiple sclerosis, neuromyelitis optica and inflammatory myelitis of an unknown etiology. Copyright © 2016 Elsevier B.V. All rights reserved.

Features of anti-aquaporin 4 antibody-seropositive Chinese patients with neuromyelitis optica spectrum optic neuritis.

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Li, Hongyang; Wang, Yanling; Xu, Quangang; Zhang, Aidi; Zhou, Huanfen; Zhao, Shuo; Kang, Hao; Peng, Chunxia; Cao, Shanshan; Wei, Shihui

2015-10-01

The detection of anti-aquaporin-4 autoantibody (AQP-4 Ab) is crucial to detect patients who will develop neuromyelitis optica (NMO); however, there are few studies on the AQP-4 Ab serostatus of patients with neuromyelitis optica spectrum ON. We analyzed the clinical and paraclinical features of neuromyelitis optica spectrum ON patients in China according to the patients' AQP4-Ab serostatus. 125 patients with recurrent and bilateral ON with simultaneous attacks were divided into AQP-4 Ab-seropositive and -seronegative groups. Demographic, clinical, serum autoantibody data, connective tissue disorders (CTDs), visual performance were compared. A Visual Acuity (VA) of less than 0.1 during acute ON attacks occurred more frequently in the seropositive group (p = 0.023); however, there was not a significant difference between groups on VA recovery after the first attack. The seropositive group experienced the worst outcome during the last attack (p = 0.017). Other co-existing autoimmunity antibodies (p optica spectrum ON.

Neuromyelitis optica (NMO) - an autoimmune disease of the central nervous system (CNS)

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Asgari, N; Owens, T; Frøkiaer, J

2010-01-01

Asgari N, Owens T, Frøkiaer J, Stenager E, Lillevang ST, Kyvik KO. Neuromyelitis optica (NMO) - an autoimmune disease of the central nervous system (CNS).
Acta Neurol Scand: DOI: 10.1111/j.1600-0404.2010.01416.x.
© 2010 John Wiley & Sons A/S. In the past 10 years, neuromyelitis optica (NMO) has...... or by intrathecal administration to naive mice. NMO may be characterized as a channelopathy of the central nervous system with autoimmune characteristics....

Neuromyelitis optica with linear enhancement of corpus callosum in brain magnetic resonance imaging with contrast: a case report.

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Sahraian, Mohammad Ali; Moghadasi, Abdorreza Naser; Owji, Mahsa; Naghshineh, Hoda; Minagar, Alireza

2015-06-10

Neuromyelitis optica is a demyelinating disease of the central nervous system with various patterns of brain lesions. Corpus callosum may be involved in both multiple sclerosis and neuromyelitis optica. Previous case reports have demonstrated that callosal lesions in neuromyelitis optica are usually large and edematous and have a heterogeneous intensity showing a "marbled pattern" in the acute phase. Their size and intensity may reduce with time or disappear in the chronic stages. In this report, we describe a case of a 25-year-old Caucasian man with neuromyelitis optica who presented clinically with optic neuritis and myelitis. His brain magnetic resonance imaging demonstrated linear enhancement of the corpus callosum. Brain images with contrast agent added also showed linear ependymal layer enhancement of the lateral ventricles, which has been reported in this disease previously. Linear enhancement of corpus callosum in magnetic resonance imaging with contrast agent could help in diagnosing neuromyelitis optica and differentiating it from other demyelinating disease, especially multiple sclerosis.

Immunotherapy of Neuromyelitis Optica

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2013-01-01

Neuromyelitis optica (NMO) is a chronic inflammatory disease of the central nervous system that affects the optic nerves and spinal cord resulting in visual impairment and myelopathy. There is a growing body of evidence that immunotherapeutic agents targeting T and B cell functions, as well as active elimination of proinflammatory molecules from the peripheral blood circulation, can attenuate disease progression. In this review, we discuss the immunotherapeutic options and the treatment strategies in NMO. We also analyze the pathogenic mechanisms of the disease in order to provide recommendations regarding treatments. PMID:24455211

Neuromyelitis optica, atypical hemophagocytic lymphohistiocytosis and heterozygous perforin A91V mutation.

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Palterer, Boaz; Brugnolo, Francesca; Sieni, Elena; Barilaro, Alessandro; Parronchi, Paola

2017-10-15

Neuromyelitis optica is an autoimmune demyelinating inflammatory disease characterized by optic neuritis and myelitis with anti-aquaporin 4 antibodies. Hemophagocytic lymphohistiocytosis is a severe systemic inflammatory syndrome that can present in a genetic primary form or secondarily to infective, neoplastic or autoimmune diseases. Our case discusses the first reported case of atypical late-onset hemophagocytic lymphohistiocytosis in a patient with neuromyelitis optica, with multiple triggering factors and carrying the common A91V hypomorphic perforin mutation, that blurs the distinction between primary and secondary forms. Copyright © 2017 Elsevier B.V. All rights reserved.

Neuromyelitis optica-- en vigtig differentialdiagnose til multipel sklerose

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Holbech, Jakob Vormstrup; Falah, Masoud

2009-01-01

Neuromyelitis optica (Devic's disease) is a rare autoimmune neuroinflammatory disease. The disease can often be difficult to distinguish from multiple sclerosis. It is important to be familiar with the disease because of the risk of rapid progression to disability or even death. We describe a 15...

Seronegative neuromyelitis optica after cardiac transplantation.

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Kim, Elecia; Van Vrancken, Michael; Shaji, Mohamed; Mir, Osman; Spak, Cedric W; Gupta, Manu; Shamim, Sadat A

2016-01-01

We report a case of a 42-year-old man who presented with progressive weakness and blindness over the course of several months and met criteria for seronegative neuromyelitis optica. This presentation was in the setting of immunosuppression following cardiac transplant. No infectious causes were found within the neuroaxis, and he ultimately died with complete blindness, quadriplegia, and respiratory failure attributed to panmyelitis and brain stem inflammation despite aggressive therapies.

EFNS guidelines on diagnosis and management of neuromyelitis optica

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Sellner, J; Boggild, M; Clanet, M

2010-01-01

Neuromyelitis optica (NMO) or Devic's disease is a rare inflammatory and demyelinating autoimmune disorder of the central nervous system (CNS) characterized by recurrent attacks of optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM), which is distinct from multiple sclerosis...

EFNS guidelines on diagnosis and management of neuromyelitis optica

NARCIS (Netherlands)

Sellner, J.; Boggild, M.; Clanet, M.; Hintzen, R.Q.; Illes, Z.; Montalban, X.; Du Pasquier, R.A.; Polman, C.H.; Sorensen, P.S.; Hemmer, B.

2010-01-01

Background and purpose: Neuromyelitis optica (NMO) or Devic's disease is a rare inflammatory and demyelinating autoimmune disorder of the central nervous system (CNS) characterized by recurrent attacks of optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM), which is distinct

Catálogo de Radio-Fuentes Opticas con Astrolabio Fotoeléctrico PAII

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Manrique, W. T.; Podestá, R. C.; Alonso, E.; Actis, E. V.; Pacheco, A. M.; Bustos, G.; Lizhi, L.; Zezhi, W.; Fanmiao, Z.; Hongqi, W.; Perdomo, R.

Usando los datos observados en San Juan con el Astrolabio Fotoeléctrico PAII desde Febrero de 1992 hasta Marzo de 1997, se ha realizado el Catálogo de Radio-Fuentes Opticas de San Juan conteniendo 69 estrellas. Las observaciones de las posiciones de las radio-fuentes están realizadas para la época y equinoccio J2000,0 y en un sistema cercano al del FK5. Las precisiones medias son ± 2,2 ms y ± 0,"035 en ascensión recta y declinación respectivamente. Las magnitudes de las estrellas son desde 0,9 a 10,7 . Las declinaciones son desde --2,5 grados a --60 grados. La época media es 1995,1. Se muestran también los resultados comparados con el Catálogo Hiparcos.

[Topics of neuromyelitis optica].

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Miyamoto, Katsuichi

2014-01-01

Neuromyelitis optica (NMO) has been revealed the difference in the pathology of multiple sclerosis since the anti-aquaporin 4 (AQP4) antibody associated with NMO has been found. The clinical epidemiological study has been reviewed, NMO-related patient number in Japan is estimated to be about 4400 people. The antibody-positive patients against myelin-oligodendrocyte glycoprotein (MOG) are present in anti-AQP4 antibody-negative NMO patients. These patients have a characteristic with high frequency of optic neuritis and good response to therapy. In addition, by research in recent years, a new treatment such as anti-IL-6 therapy or anti-complement therapy has been attempted to NMO.

Structural MRI substrates of cognitive impairment in neuromyelitis optica

NARCIS (Netherlands)

Liu, Y.; Fu, Y.; Schoonheim, M.M.; Zhang, N.; Fan, M.L.; Su, L.; Shen, Y.; Yan, Y.P.; Yang, L.; Wang, Q.H.; Zhang, N.N.N.; Yu, C.S.; Barkhof, F.; Shi, F.D.

2015-01-01

Objective: To identify the clinical and structural MRI markers for predicting cognitive impairment (CI) in patients with neuromyelitis optica (NMO). Methods: Fifty-four patients with NMO and 27 healthy controls underwent extensive neuropsychological testing and multimodal 3.0T MRI. The patient group

Multisystem involvement in neuromyelitis optica

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Megan M Langille

2015-01-01

Full Text Available We describe a case of pediatric neuromyelitis optica (NMO with muscle and lung involvement in addition to central nervous system disease. Our patient initially presented with features of area postrema syndrome, then subsequently with optic neuritis. The patient also had recurrent hyperCKemia that responded to corticosteroids. Finally, axillary and hilar adenopathy with pulmonary consolidation were noted as well and responded to immunomodulation. Our case highlights multisystem involvement in NMO including non-infectious pulmonary findings which have not been described in the pediatric population previously.

Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset.

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Collongues, N; Marignier, R; Jacob, A; Leite, M I; Siva, A; Paul, F; Zephir, H; Akman-Demir, G; Elsone, L; Jarius, S; Papeix, C; Mutch, K; Saip, S; Wildemann, B; Kitley, J; Karabudak, R; Aktas, O; Kuscu, D; Altintas, A; Palace, J; Confavreux, C; De Seze, J

2014-07-01

Few data are available for patients with a late onset (≥ 50 years) of neuromyelitis optica (LONMO) or neuromyelitis optica spectrum disease (LONMOSD), defined by an optic neuritis/longitudinally extensive transverse myelitis with aquaporin-4 antibodies (AQP4-Ab). To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death. We identified 430 patients from four cohorts of NMO/NMOSD in France, Germany, Turkey and UK. We extracted the late onset patients and analyzed them for predictive factors of disability and death, using the Cox proportional model. We followed up on 63 patients with LONMO and 45 with LONMOSD during a mean of 4.6 years. This LONMO/LONMOSD cohort was mainly of Caucasian origin (93%), women (80%), seropositive for AQP4-Ab (85%) and from 50 to 82.5 years of age at onset. No progressive course was noted. At last follow-up, the median Expanded Disability Status Scale (EDSS) scores were 5.5 and 6 in the LONMO and LONMOSD groups, respectively. Outcome was mainly characterized by motor disability and relatively good visual function. At last follow-up, 14 patients had died, including seven (50%) due to acute myelitis and six (43%) because of opportunistic infections. The EDSS 4 score was independently predicted by an older age at onset, as a continuous variable after 50 years of age. Death was predicted by two independent factors: an older age at onset and a high annualized relapse rate. LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death. © The Author(s) 2013.

Idiopathic Transverse Myelitis and Neuromyelitis Optica: Clinical Profiles, Pathophysiology and Therapeutic Choices

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Awad, Amer; Stüve, Olaf

2011-01-01

Transverse myelitis is a focal inflammatory disorder of the spinal cord which may arise due to different etiologies. Transverse myelitis may be idiopathic or related/secondary to other diseases including infections, connective tissue disorders and other autoimmune diseases. It may be also associated with optic neuritis (neuromyelitis optica), which may precede transverse myelitis. In this manuscript we review the pathophysiology of different types of transverse myelitis and neuromyelitis optica and discuss diagnostic criteria for idiopathic transverse myelitis and risk of development of multiple sclerosis after an episode of transverse myelitis. We also discuss treatment options including corticosteroids, immunosuppressives and monoclonal antibodies, plasma exchange and intravenous immunoglobulins. PMID:22379456

Seronegative neuromyelitis optica spectrum disorder patients diagnosed using new diagnostic criteria.

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Uzawa, Akiyuki; Mori, Masahiro; Uchida, Tomohiko; Masuda, Hiroki; Ohtani, Ryohei; Kuwabara, Satoshi

2016-09-01

Recently, new diagnostic criteria for neuromyelitis optica spectrum disorders (NMOSD) were published. Our primary aim was to evaluate the usefulness of the new diagnostic criteria in anti-aquaporin 4 (AQP4) antibody-negative cases. Consecutive 471 patients whose anti-AQP4 antibody was measured at Chiba University were reviewed. Four anti-AQP4 antibody negative-patients, who fulfilled the new diagnostic criteria for NMOSD but not 2006 diagnostic criteria for neuromyelitis optica (NMO), were identified. They showed high cerebrospinal fluid interleukin-6 and glial fibrillary acidic protein levels, an absence of oligoclonal bands and/or cloud-like enhancement on magnetic resonance imaging, which are compatible findings for NMO. The new diagnostic criteria are clinically useful in seronegative NMOSD. © The Author(s), 2015.

Neuromyelitis Optica: An Often Forgotten Cause of Intractable Nausea and Vomiting

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Chijioke Enweluzo

2013-07-01

Full Text Available Neuromyelitis optica, also known as Devic's disease, is a rare autoimmune disorder in which a patient's immune system affects the optic nerves and the spinal cord, leading to loss of vision and spinal cord dysfunction. We present our experience with a 38-year-old female who presented to our facility with complaints of intractable nausea and vomiting. After extensive evaluation, she was found to have neuromyelitis optica. Her symptoms completely resolved following institution of appropriate therapy. She made a significant recovery and has since been placed on chronic immunosuppressive therapy. Through this article we hope to bring attention to a significant cause of intractable nausea and vomiting that may often be forgotten in general medicine or gastroenterology services.

Systemic manifestations and pathogenesis exploration of neuromyelitis optica spectrum disorders

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Hong JIANG

2014-09-01

Full Text Available Objective To investigate the immunological abnormalities beyond central nervous system (CNS associated with neuromyelitis optica spectrum disorders (NMOSDs.  Methods Clinical data of 56 patients with NMOSDs from January 2010 to December 2013 enrolled in Department of Neurology at Peking University People's Hospital were analyzed retrospectively. All patients were divided into 2 groups: neuromyelitis optica (N = 33 and non-neuromyelitis optica (N = 23. Each patient underwent detailed physical examination including internal medicine and nervous system. Records of complicated autoimmune diseases as well as scoring of Expanded Disability Status Scale (EDSS were used to evaluate disease severity. Part of patients received detection of multiple immunological indicators.  Results In all patients with NMOSDs, there were 3 cases with Hashimoto thyroiditis, one case with systemic lupus erythematosus (SLE and Sjögren's syndrome (SS, asthma, hyperthyroidism, rheumatoid arthritis and iridocyclitis, respectively. In patients whose immunological indices were available, the first three abnormal immunological changes were abnormal thyroid function (10/17, positive anti-nuclear antibody (14/28 and positive complement 3 (8/19. In addition, NMOSDs got worsen in 2 cases after delivery.  Conclusions NMOSDs coexist with many kinds of autoimmune diseases and multiple autoantibodies. The above-mentioned autoimmunity may be related to the pathogenesis of NMOSDs. Besides, pregnancy or delivery may aggravate the disease severity of NMOSDs. doi: 10.3969/j.issn.1672-6731.2014.09.009

Severe optic neuritis in a patient with combined neuromyelitis optica spectrum disease and primary Sjögren’s syndrome: a case report

Directory of Open Access Journals (Sweden)

Tan Petrina

2012-11-01

Full Text Available Abstract Introduction Optic neuritis, although uncommon, can be the initial presentation of Sjögren’s syndrome. Coexisting Sjögren’s syndrome has also been reported with neuromyelitis optica spectrum disorder. This case report highlights the association between the two diseases and the importance of rheumatological and neurological evaluations in patients with such diagnoses. Distinction of neuromyelitis optica with coexisting connective tissue disease has both prognostic and therapeutic significance for the patient. Case presentation We report a case of a 56-year-old Chinese woman who presented with bilateral asymmetric visual loss secondary to optic neuritis. She was subsequently found to be seropositive for neuromyelitis optica immunoglobulin G (NMO-IgG (anti-aquaporin-4 antibody and was diagnosed with neuromyelitis optica spectrum disorder. She also fulfilled the international criteria for Sjögren’s syndrome. Despite initial high dose immunosuppressive therapy, she failed to regain vision in one eye. Conclusion Patients presenting with optic neuritis and severe visual loss should be screened for neuromyelitis optica and treated appropriately. Neuromyelitis optica has been associated with systemic autoimmune diseases, in particular Sjögren’s syndrome, and current evidence indicates that they are two distinct entities. We recommend that both diagnoses be considered in cases of optic neuritis with severe visual loss.

Update on neuromyelitis optica: natural history and management

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Jindahra, Panitha; Plant, T

2012-01-01

Neuromyelitis optica or Devic disease is an inflammatory disorder of the central nervous system. It is caused by antibodies that attack aquaporin 4 water channels in the cell membrane of astrocytic foot processes at the blood brain barrier. It can involve the optic nerve, the spinal cord and beyond. Here we review its pathophysiology, clinical features, and therapy. PMID:28539779

Higher frequency of brain abnormalities in neuromyelitis optica spectrum disorder patients without primary Sjögren's syndrome.

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Gu, Li-Na; Zhang, Min; Zhu, Hui; Liu, Jing-Yao

2016-10-01

Neuromyelitis optica spectrum disorder often co-exists with primary Sjögren's syndrome. We compared the clinical features of 16 neuromyelitis optica spectrum disorder patients with ( n = 6) or without primary Sjögren's syndrome ( n = 10). All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjögren's syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinuclear autoantibody, anti-Sjögren's-syndrome-related antigen A antibodies, anti-Sjögren's-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjögren's syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% (4/6) of patients with primary Sjögren's syndrome and in 60% (6/10) of patients without primary Sjögren's syndrome. More brain abnormalities were observed in patients without primary Sjögren's syndrome than in those with primary Sjögren's syndrome. Segments lesions (> 3 centrum) were noted in 50% (5/10) of patients without primary Sjögren's syndrome and in 67% (4/6) of patients with primary Sjögren's syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjögren's syndrome are similar. However, neuromyelitis optica spectrum disorder patients without primary Sjögren's syndrome have a high frequency of brain abnormalities.

Fiber-optic components for optical communicatios and sensing =

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Marques, Carlos Alberto Ferreira

Nos ultimos anos, a Optoelectronica tem sido estabelecida como um campo de investigacao capaz de conduzir a novas solucoes tecnologicas. As conquistas abundantes no campo da optica e lasers, bem como em comunicacoes opticas tem sido de grande importancia e desencadearam uma serie de inovacoes. Entre o grande numero de componentes opticos existentes, os componentes baseados em fibra optica sao principalmente relevantes devido a sua simplicidade e a elevada de transporte de dados da fibra optica. Neste trabalho foi focado um destes componentes opticos: as redes de difraccao em fibra optica, as quais tem propriedades opticas de processamento unicas. Esta classe de componentes opticos e extremamente atraente para o desenvolvimento de dispositivos de comunicacoes opticas e sensores. O trabalho comecou com uma analise teorica aplicada a redes em fibra e foram focados os metodos de fabricacao de redes em fibra mais utilizados. A inscricao de redes em fibra tambem foi abordado neste trabalho, onde um sistema de inscricao automatizada foi implementada para a fibra optica de silica, e os resultados experimentais mostraram uma boa aproximacao ao estudo de simulacao. Tambem foi desenvolvido um sistema de inscricao de redes de Bragg em fibra optica de plastico. Foi apresentado um estudo detalhado da modulacao acustico-optica em redes em fibra optica de silica e de plastico. Por meio de uma analise detalhada dos modos de excitacao mecanica aplicadas ao modulador acustico-optico, destacou-se que dois modos predominantes de excitacao acustica pode ser estabelecidos na fibra optica, dependendo da frequencia acustica aplicada. Atraves dessa caracterizacao, foi possivel desenvolver novas aplicacoes para comunicacoes opticas. Estudos e implementacao de diferentes dispositivos baseados em redes em fibra foram realizados, usando o efeito acustico-optico e o processo de regeneracao em fibra optica para varias aplicacoes tais como rapido multiplexador optico add-drop, atraso de grupo

OPTICA: Our Path Together Initiating Cultural Access. Final Report.

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Jackson, Susan, Comp.

This final report describes the activities and outcomes of OPTICA (Our Path Together Initiating Cultural Access) programs. For each program an information sheet indicates the goal, total participation, status of the goal, and activities of the program. Programs included: (1) Hands On: ASL Creative Story Telling, a program that used children's…

Neuromyelitis Optica Spectrum Disorders.

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Akaishi, Tetsuya; Nakashima, Ichiro; Sato, Douglas Kazutoshi; Takahashi, Toshiyuki; Fujihara, Kazuo

2017-05-01

Neuromyelitis optica (NMO) is clinically characterized by severe optic neuritis and transverse myelitis, but recent studies with anti-aquaporin-4-antibody specific to NMO have revealed that the clinical spectrum is wider than previously thought. International consensus diagnostic criteria propose NMO spectrum disorders (NMOSD) as the term to define the entire spectrum including typical NMO, optic neuritis, acute myelitis, brain syndrome, and their combinations. NMOSD is now divided into anti-aquaporin-4-antibody-seropositive NMOSD and -seronegative NMOSD (or unknown serostatus). MR imaging and optical coherence tomography are indispensable in the diagnosis and evaluation of NMOSD. This article reviews the clinical and MR imaging findings of anti-aquaporin-4-antibody-seropositive and anti-myelin oligodendrocyte glycoprotein-antibody-seropositive NMOSD. Copyright © 2017 Elsevier Inc. All rights reserved.

Neuromyelitis optica with clinical and histopathological involvement of the brain.

NARCIS (Netherlands)

Hengstman, G.J.D.; Wesseling, P.; Frenken, C.W.; Jongen, P.J.H.

2007-01-01

Diagnostic criteria for neuromyelitis optica (NMO) state that there should be no active disease outside the optic nerves and spinal cord. However, several cases have been described with symptomatic brain involvement. We describe an autopsy case of a patient with NMO and symptomatic involvement of

Neuromyelitis optica spectrum disorders in Algeria: A preliminary study in the region of Tizi Ouzou.

Science.gov (United States)

Daoudi, Smail; Bouzar, Melissa

2016-03-01

Neuromyelitis optica (NMO) is a disabling inflammatory condition that targets astrocytes in the optic nerves and spinal cord. Recent advances led to the individualization of a set of conditions now referred as NMO spectrum disorder (NMOSD). To describe the prevalence and characteristics of NMO SD in north Algeria. The present study is a retrospective and descriptive work which took place in Nedir Mohamed teaching hospital, Tizi-Ouzou, Algeria. 938 Medical files of patients with CNS inflammatory demyelinating diseases were reviewed then patients with optic neuritis and/or myelitis were preselected. Patients who met the 2015 neuromyelitis optica spectrum disorders criteria were selected and analyzed 08 Patients (3.4%) met the 2015 criteria for neuromyelitis optica spectrum disorders, 3/8 (37.5%) were positive to AQ4-IgG and 5/8 (62.5%) were negative. Mean age of onset was 29 years, female to male ratio was 3:1, cerebral MRI was normal in 75% of cases and longitudinally extensive transverse myelitis was present in 75% of cases. 37/232 Patients (15.9%) were considered at high risk of neuromyelitis optica spectrum disorders The present study suggests that the spectrum of NMO disorders is a rare entity among patients with optic nerve and spinal cord demyelinating lesions in north Algeria. However, the lack of accurate AQ4-IgG test certainly underestimates its real prevalence. Copyright © 2015 Elsevier B.V. All rights reserved.

Diagnosis and management of Neuromyelitis Optica Spectrum Disorder (NMOSD) in Iran

DEFF Research Database (Denmark)

Sahraian, Mohammad Ali; Moghadasi, Abdorreza Naser; Azimi, Amir Reza

2017-01-01

Neuromyelitis Optica Spectrum Disorder (NMOSD) is a relapsing neuro inflammatory disease of the central nervous system that typically presents with optic neuritis or myelitis and may cause severe disability. The diagnostic criteria have been updated and several immunosuppressive agents have been...

Quantifying visual pathway axonal and myelin loss in multiple sclerosis and neuromyelitis optica.

Science.gov (United States)

Manogaran, Praveena; Vavasour, Irene M; Lange, Alex P; Zhao, Yinshan; McMullen, Katrina; Rauscher, Alexander; Carruthers, Robert; Li, David K B; Traboulsee, Anthony L; Kolind, Shannon H

2016-01-01

The optic nerve is frequently injured in multiple sclerosis and neuromyelitis optica, resulting in visual dysfunction, which may be reflected by measures distant from the site of injury. To determine how retinal nerve fiber layer as a measure of axonal health, and macular volume as a measure of neuronal health are related to changes in myelin water fraction in the optic radiations of multiple sclerosis and neuromyelitis optica participants with and without optic neuritis and compared to healthy controls. 12 healthy controls, 42 multiple sclerosis (16 with optic neuritis), and 10 neuromyelitis optica participants (8 with optic neuritis) were included in this study. Optical coherence tomography assessment involved measurements of the segmented macular layers (total macular, ganglion cell layer, inner plexiform layer, and inner nuclear layer volume) and paripapillary retinal nerve fiber layer thickness. The MRI protocol included a 32-echo T2-relaxation GRASE sequence. Average myelin water fraction values were calculated within the optic radiations as a measure of myelin density. Multiple sclerosis and neuromyelitis optica eyes with optic neuritis history had lower retinal nerve fiber layer thickness, total macular, ganglion cell and inner plexiform layer volumes compared to eyes without optic neuritis history and controls. Inner nuclear layer volume increased in multiple sclerosis with optic neuritis history (mean = 0.99 mm(3), SD = 0.06) compared to those without (mean = 0.97 mm(3), SD = 0.06; p = 0.003). Mean myelin water fraction in the optic radiations was significantly lower in demyelinating diseases (neuromyelitis optica: mean = 0.098, SD = 0.01, multiple sclerosis with optic neuritis history: mean = 0.096, SD = 0.01, multiple sclerosis without optic neuritis history: mean = 0.098, SD = 0.02; F3,55 = 3.35, p = 0.03) compared to controls. Positive correlations between MRI and optical coherence tomography measures were also apparent

Use of advanced magnetic resonance imaging techniques in neuromyelitis optica spectrum disorder

NARCIS (Netherlands)

S. Kremer (Stephane); F. Renard (Felix); S. Achard (Sophie); M.A. Lana-Peixoto (Marco A.); J. Palace (Jacqueline); N. Asgari (Nasrin); E.C. Klawiter (Eric C.); S. Tenembaum (Silvia); B. Banwell (Brenda); B.M. Greenberg (Benjamin M.); J.L. Bennett (Jeffrey); M. Levy (Michael); P. Villoslada (Pablo); A. Saiz (Albert Abe); K. Fujihara (Kazuo); K.H. Chan (Koon Ho); S. Schippling (Sven); F. Paul (Friedemann); H.J. Kim (Ho Jin); J. De Seze (Jerome); J.T. Wuerfel (Jens T.); P. Cabre (Philippe); R. Marignier (Romain); T. Tedder (Thomas); E.D. van Pelt - Gravesteijn (Daniëlle); S. Broadley (Simon); T. Chitnis (Tanuja); D. Wingerchuk (Dean); L. Pandit (Lekha); M.I. Leite (M. Isabel); M. Apiwattanakul (Metha); I. Kleiter (Ingo); N. Prayoonwiwat (Naraporn); M. Han (May); K. Hellwig (Kerstin); K. Van Herle (Katja); G. John (Gareth); D.C. Hooper (D. Craig); I. Nakashima (Ichiro); D. Sato (Douglas); M.R. Yeaman (Michael R.); E. Waubant (Emmanuelle); S. Zamvil (Scott); O. Stüve (Olaf); O. Aktas (Orhan); T.J. Smith (Terry J.); A. Jacob (Anu); K. O'Connor (Kevin)

2015-01-01

textabstractBrain parenchymal lesions are frequently observed on conventional magnetic resonance imaging (MRI) scans of patients with neuromyelitis optica (NMO) spectrum disorder, but the specific morphological and temporal patterns distinguishing them unequivocally from lesions caused by other

Tonic spasms are a common clinical manifestation in patients with neuromyelitis optica Espasmos tônicos são manifestações clínicas frequentes em pacientes com neuromielite óptica

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Luz Abaroa

2013-05-01

Full Text Available Tonic spasms have been most commonly associated with multiple sclerosis. To date, few reports of series of patients with neuromyelitis optica and tonic spasms have been published. Methods: We analyzed the characteristics and frequency of tonic spasms in 19 subjects with neuromyelitis optica. Data was collected using a semi-structured questionnaire for tonic spasms, by both retrospectively reviewing medical records and performing clinical assessment. Results: All patients except one developed this symptom. The main triggering factors were sudden movements and emotional factors. Spasms were commonly associated to sensory disturbances and worsened during the acute phases of the disease. Carbamazepine was most commonly used to treat the symptom and patients showed good response to the drug. Conclusions: Tonic spasms are a common clinical manifestation in patients with neuromyelitis optica.Espasmos tônicos têm sido mais frequentemente associados com esclerose múltipla. Foram publicados até agora poucos relatos de série de pacientes com neuromielite óptica e espasmos tônicos. Métodos: Foram analisadas as características e a frequência de espasmos tônicos em 19 indivíduos com neuromielite óptica. Os dados foram coletados por meio de um questionário semiestruturado para espasmos tônicos, mediante a avaliação retrospectiva dos prontuários e a análise dos dados clínicos Resultados: Todos os pacientes com neuromielite óptica exceto um apresentaram espasmos tônicos. Os principais fatores desencadeantes foram movimentos bruscos e fatores emocionais. Espasmos foram frequentemente associados a perturbações sensoriais e se agravaram durante a fase aguda da doença. A carbamazepina foi utilizada frequentemente para tratar os sintomas, com boa resposta. Conclusões: Os espasmos tônicos são manifestações clínicas frequentes em pacientes com neuromielite óptica.

New onset neuromyelitis optica in a young Nigerian woman with possible antiphospholipid syndrome: a case report

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Komolafe Morenikeji A

2008-11-01

Full Text Available Abstract Introduction Devic's neuromyelitis optica is an inflammatory demyelinating disease that targets the optic nerves and spinal cord. It has a worldwide distribution and distinctive features that distinguish it from multiple sclerosis. There has been no previous report of neuromyelitis optica from our practice environment, and we are not aware of any case associated with antiphospholipid syndrome in an African person. Case presentation We report the case of a 28-year-old Nigerian woman who presented with neck pain, paroxysmal tonic spasms, a positive Lhermitte's sign and spastic quadriplegia. She later developed bilateral optic neuritis and had clinical and biochemical features of antiphospholipid syndrome. Her initial magnetic resonance imaging showed a central linear hyperintense focus in the intramedullary portion of C2 to C4. Repeat magnetic resonance imaging after treatment revealed resolution of the signal intensity noticed earlier. Conclusion Neuromyelitis optica should be considered in the differential diagnoses of acute myelopathy in Africans. We also highlight the unusual association with antiphospholipid syndrome. Physicians should screen such patients for autoimmune disorders.

A Rare Case of Neuromyelitis Optica Spectrum Disorder in Patient with Sjogren’s Syndrome

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Supat Thongpooswan

2014-01-01

Full Text Available We report a 48-year-old female with the history of Sjogren’s syndrome who presented with 3-week history of tingling, numbness, and shooting back, waist, and bilateral leg pain and numbness in the pelvic region with urinary and bowel incontinence. Physical examination was remarkable for reduced motor power in both lower extremities with spasticity. Sensory deficit was noted at the T6 level. Laboratory investigation revealed elevated ESR and CRP and positive serum antiaquaporin-4 IgG. Thoracic and lumbar magnetic resonance imaging revealed abnormal patchy areas, leptomeningeal enhancement through the thoracic cord extending from T3 through T6 levels, without evidence of cord compression. Impression of neuromyelitis optica spectrum disorder was made and patient was treated with methylprednisolone intravenously followed by tapering oral prednisone. Neurological symptoms gradually improved with resolution of bowel and urinary incontinence. In a patient with Sjogren’s syndrome who presents with neurological complaints, the possibility of neuromyelitis optica or neuromyelitis optica spectrum disorder should be considered. Awareness of the possibility of CNS disease is important due to the serious nature of CNS complications, some of which are treatable with immunosuppressants. Our patient with Sjogren’s syndrome who presented with myelopathy benefited from early recognition and institution of appropriate therapy.

Acquired Demyelinating Syndromes: Focus on Neuromyelitis Optica and childhood-onset Multiple Sclerosis

NARCIS (Netherlands)

E.D. van Pelt - Gravesteijn (Daniëlle)

2016-01-01

markdownabstractAcquired demyelinating syndromes (ADS) cover a broad spectrum of central nervous system (CNS) inflammatory demyelinating syndromes, of which multiple sclerosis (MS) is the most common subtype. This thesis focuses on two relatively rare clinical subtypes of ADS: neuromyelitis optica

The history of neuromyelitis optica

Science.gov (United States)

2013-01-01

The discovery of a novel serum autoantibody (termed NMO-IgG or AQP4-Ab) in a subset of patients in 2004 has revived interest in neuromyelitis optica (NMO). While the history of classical multiple sclerosis has been extensively studied, only little is known about the history of NMO. In the present article, we provide a comprehensive review of the early history of this rare but intriguing syndrome. We trace the origins of the concept of NMO in the 19th century medical literature and follow its evolution throughout the 20th and into the 21st century. Finally, we discuss recent proposals to revise the concept of NMO and explain why there is indeed a need for a more systematic and descriptive nomenclature. PMID:23320783

Neuromyelitis optica and neuromyelitis optica spectrum disorder: Natural history and long-term outcome, an Indian experience

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Sujit Abajirao Jagtap

2015-01-01

Full Text Available Background: Neuromyelitis optica (NMO has evolved from devic′s classical description to a broader disease spectrum, from monophasic illness to a polyphasic illness with multiple recurrences, disease confined to optic nerve and spinal cord to now brain stem, cerebrum and even endocrinopathy due to hypothalamic involvement. Objectives: To report, the epidemiological characteristics, clinical presentations, recurrence rate, treatment and response to therapy in 26 patients with NMO and NMO spectrum disorder among the Indian population. Methods: We performed observational, retrospective analysis of our prospectively maintained data base of patients with NMO, longitudinally extensive transverse myelitis during the period of January 2003-December 2012 who satisfied the national multiple sclerosis society (NMSS task force criteria for diagnosis of NMO and NMO spectrum disorder. Results: There were 26 patients (female: male, 21:5, the mean age of onset of symptom was 27 years (range 9-58, standard deviation = 12. Twenty-one patients (80% fulfilled NMSS criteria for NMO while rest 5 patients (20% were considered as NMO spectrum disorder. Seven patients (27% had a monophasic illness, 19 patients (73% had a polyphasic illness with recurrences. The Median recurrence rate was 4/patient in the polyphasic group. 13 (50% patient were tested for aquaporin 4 antibody, 8 (61% were positive while 5 patients (39% were negative. All patients received intravenous methyl prednisolone, 9 patients (35% required further treatment for acute illness in view of unresponsiveness to steroids. Thirteen patients (50% received disease-modifying agents for recurrences. Mean duration of follow-up was 5 years. All patients had a good outcome (modified Rankin scale, <3 except one who had poor visual recovery. Conclusion: Neuromyelitis optica/NMO spectrum disorder is demyelinating disorder with female predominance, polyphasic course, myelitis being most common event although brain

Iron and Non-Iron-Related Characteristics of Multiple Sclerosis and Neuromyelitis Optica Lesions at 7T MRI.

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Chawla, S; Kister, I; Wuerfel, J; Brisset, J-C; Liu, S; Sinnecker, T; Dusek, P; Haacke, E M; Paul, F; Ge, Y

2016-07-01

Characterization of iron deposition associated with demyelinating lesions of multiple sclerosis and neuromyelitis optica has not been well studied. Our aim was to investigate the potential of ultra-high-field MR imaging to distinguish MS from neuromyelitis optica and to characterize tissue injury associated with iron pathology within lesions. Twenty-one patients with MS and 21 patients with neuromyelitis optica underwent 7T high-resolution 2D-gradient-echo-T2* and 3D-susceptibility-weighted imaging. An in-house-developed algorithm was used to reconstruct quantitative susceptibility mapping from SWI. Lesions were classified as "iron-laden" if they demonstrated hypointensity on gradient-echo-T2*-weighted images and/or SWI and hyperintensity on quantitative susceptibility mapping. Lesions were considered "non-iron-laden" if they were hyperintense on gradient-echo-T2* and isointense or hyperintense on quantitative susceptibility mapping. Of 21 patients with MS, 19 (90.5%) demonstrated at least 1 quantitative susceptibility mapping-hyperintense lesion, and 11/21 (52.4%) had iron-laden lesions. No quantitative susceptibility mapping-hyperintense or iron-laden lesions were observed in any patients with neuromyelitis optica. Iron-laden and non-iron-laden lesions could each be further characterized into 2 distinct patterns based on lesion signal and morphology on gradient-echo-T2*/SWI and quantitative susceptibility mapping. In MS, most lesions (n = 262, 75.9% of all lesions) were hyperintense on gradient-echo T2* and isointense on quantitative susceptibility mapping (pattern A), while a small minority (n = 26, 7.5% of all lesions) were hyperintense on both gradient-echo-T2* and quantitative susceptibility mapping (pattern B). Iron-laden lesions (n = 57, 16.5% of all lesions) were further classified as nodular (n = 22, 6.4%, pattern C) or ringlike (n = 35, 10.1%, pattern D). Ultra-high-field MR imaging may be useful in distinguishing MS from neuromyelitis optica. Different

Painful tonic spasms and brainstem involvement in a patient with neuromyelitis optica spectrum disorder.

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Roman-Filip, Corina; Ungureanu, Aurelian; Cernuşcă-Miţaru, Mihaela

2016-01-01

Neuromyelitis optica (NMO) is an inflammatory-demyelinating disease of the central nervous system classically characterized by optic neuritis and severe myelitis. New diagnostic criteria defined neuromyelitis optica spectrum disorder as limited forms of NMO or diverse neurologic presentations in the presence of specific antiaquaporin-4 antibodies. We report the case of a 57-year-old woman admitted in our department for recurrent attacks of optic neuritis, tetraparesis with severe painful tonic spasms of the left limbs and brainstem involvement. Painful tonic spasms have been described as movement disorders associated with multiple sclerosis, but a growing number of reports describe them in cases of NMO. Copyright © 2015 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Neuromyelitis Optica (Devic's Syndrome): an Appraisal.

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Crout, Teresa M; Parks, Laura P; Majithia, Vikas

2016-08-01

Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD), previously known as Devic's syndrome, are a group of inflammatory disorders of the central nervous system (CNS) characterized by severe, immune-mediated demyelination and axonal damage, predominantly targeting optic nerves and the spinal cord typically associated with a disease-specific serum NMO-IgG antibody that selectively binds aquaporin-4 (AQP4). The classic and best-defined features of NMOSD include acute attacks of bilateral or rapidly sequential optic neuritis (leading to visual loss) or transverse myelitis (often causing limb weakness and bladder dysfunction) or both with a typically relapsing course. The diagnosis of NMO/NMOSD requires a consistent history and examination with typical clinical presentations, findings on spinal cord neuroimaging with MRI, cerebrospinal fluid analysis along with determination of AQP4-IgG serum autoantibody status, and exclusion of other disorders. Two major advances in this field has been the development of diagnostic criteria and treatment recommendations. Consensus diagnostic criteria have been established and were recently revised and published in 2015, enhancing the ability to make a diagnosis and appropriately evaluate these disorders. Expert recommendations and uncontrolled trials form the basis of treatment guidelines. All patients with suspected NMOSD should be treated for acute attacks as soon as possible with high-dose intravenous methylprednisolone -1 gram daily for three to five consecutive days and in some cases, plasma exchange should be used. It is recommended that every patient with NMOSD be started on an immunosuppressive agent, such as, azathioprine, methotrexate, or mycophenolate and in some cases, rituximab, soon after the acute attack and usually be treated for about 5 years after the attack. These advances have helped improve the prognosis and outcome in these disorders.

Association between TPMT*3C and decreased thiopurine S-methyltransferase activity in patients with neuromyelitis optica spectrum disorders in China.

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Gong, Xiaoqing; Mei, Shenghui; Li, Xindi; Li, Xingang; Zhou, Heng; Liu, Yonghong; Zhou, Anna; Yang, Li; Zhao, Zhigang; Zhang, Xinghu

2018-06-01

Thiopurines are effective drugs in treating neuromyelitis optica spectrum disorders and other diseases. Thiopurines' toxicity is mainly imputed to thiopurine S-methyltransferase activity. In Chinese population, the most common and important variation of thiopurine S-methyltransferase is TPMT*3C (rs1142345). This study aims to reveal the association between thiopurine S-methyltransferase activity and genetic polymorphisms of thiopurine S-methyltransferase in patients with neuromyelitis optica spectrum disorders in China. A liquid chromatography tandem mass/mass method was used to evaluate the thiopurine S-methyltransferase activity by using 6-mercapthioprine as the substrate in human erythrocyte haemolysate via 1 h incubation at 37 °C to form its methylated product 6-methylmercaptopurine. The amount of 6-methylmercaptopurine was adjusted by haematocrit and normalized to 8 × 10 8 erythrocytes. The selected polymorphisms of thiopurine S-methyltransferase were identified using MassARRAY system (Sequenom) and multiple SNaPshot technique. In 69 patients with neuromyelitis optica spectrum disorders, thiopurine S-methyltransferase activity was 80.29-154.53 (127.51 ± 16.83) pmol/h/8 × 10 8 erythrocytes. TPMT*3C (rs1142345) was associated with lower thiopurine S-methyltransferase activity (BETA = -25.37, P = 0.011). Other selected variants were not associated with thiopurine S-methyltransferase activity. TPMT*3C affects TPMT activity in Chinese patients with neuromyelitis optica spectrum disorders. Further studies are warranted to confirm the results. TPRs = thiopurines; NMOSD = neuromyelitis optica spectrum disorders; TPMT = thiopurine S-methyltransferase; LC-MS/MS = liquid chromatography tandem mass/mass; 6-MMP = 6-methylmercaptopurine; IS = internal standard; SNP = single nucleotide polymorphism; MAF = minor allele frequency; HWE = Hardy-Weinberg equilibrium; BETA = regression coefficients; UTR-3 = untranslated region 3.

The Urine Proteome Profile Is Different in Neuromyelitis Optica Compared to Multiple Sclerosis

DEFF Research Database (Denmark)

Nielsen, Helle H; Beck, Hans C; Kristensen, Lars P

2015-01-01

OBJECTIVES: Inflammatory demyelinating diseases of the CNS comprise a broad spectrum of diseases like neuromyelitis optica (NMO), NMO spectrum disorders (NMO-SD) and multiple sclerosis (MS). Despite clear classification criteria, differentiation can be difficult. We hypothesized that the urine...

Influence of female sex and fertile age on neuromyelitis optica spectrum disorders.

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Borisow, Nadja; Kleiter, Ingo; Gahlen, Anna; Fischer, Katrin; Wernecke, Klaus-Dieter; Pache, Florence; Ruprecht, Klemens; Havla, Joachim; Krumbholz, Markus; Kümpfel, Tania; Aktas, Orhan; Ringelstein, Marius; Geis, Christian; Kleinschnitz, Christoph; Berthele, Achim; Hemmer, Bernhard; Angstwurm, Klemens; Weissert, Robert; Stellmann, Jan-Patrick; Schuster, Simon; Stangel, Martin; Lauda, Florian; Tumani, Hayrettin; Mayer, Christoph; Zeltner, Lena; Ziemann, Ulf; Linker, Ralf A; Schwab, Matthias; Marziniak, Martin; Then Bergh, Florian; Hofstadt-van Oy, Ulrich; Neuhaus, Oliver; Winkelmann, Alexander; Marouf, Wael; Rückriem, Lioba; Faiss, Jürgen; Wildemann, Brigitte; Paul, Friedemann; Jarius, Sven; Trebst, Corinna; Hellwig, Kerstin

2017-07-01

Gender and age at onset are important epidemiological factors influencing prevalence, clinical presentation, and treatment response in autoimmune diseases. 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)). 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. A total of 186 NMO/SD patients (82% female) were included. In AQP4-ab-positive patients, female predominance was most pronounced during fertile age (female-to-male ratio 23:1). Female patients were more likely to be positive for AQP4-abs (92% vs 55%; p 40 years. Our data suggest an influence of sex and age on susceptibility to AQP4-ab-positive NMO/SD. Genetic and hormonal factors might contribute to pathophysiology of NMO/SD.

Müller cells and retinal axons can be primary targets in experimental neuromyelitis optica spectrum disorder.

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Zeka, Bleranda; Lassmann, Hans; Bradl, Monika

2017-01-01

Recent work from our laboratory, using different models of experimental neuromyelitis optica spectrum disorder (NMOSD), has led to a number of observations that might be highly relevant for NMOSD patients. For example: (i) in the presence of neuromyelitis optica immunoglobulin G, astrocyte-destructive lesions can be initiated by CD4+ T cells when these cells recognize aquaporin 4 (AQP4), but also when they recognize other antigens of the central nervous system. The only important prerequisite is that the T cells have to be activated within the central nervous system by "their" specific antigen. Recently activated CD4+ T cells with yet unknown antigen specificity are also found in human NMOSD lesions. (ii) The normal immune repertoire might contain AQP4-specific T cells, which are highly encephalitogenic on activation. (iii) The retina might be a primary target of AQP4-specific T cells and neuromyelitis optica immunoglobulin G: AQP4-specific T cells alone are sufficient to cause retinitis with low-grade axonal pathology in the retinal nerve fiber/ganglionic cell layer. A thinning of these layers is also observed in NMOSD patients, where it is thought to be a consequence of optic neuritis. Neuromyelitis optica immunoglobulin G might target cellular processes of Müller cells and cause their loss of AQP4 reactivity, when AQP4-specific T cells open the blood-retina barrier in the outer plexiform layer. Patchy loss of AQP4 reactivity on Müller cells of NMOSD patients has been recently described. Cumulatively, our findings in experimental NMOSD suggest that both CD4+ T cell and antibody responses directed against AQP4 might play an important role in the pathogenesis of tissue destruction seen in NMOSD.

Neuromyelitis optica spectrum disorders: beyond longitudinally extensive transverse myelitis

International Nuclear Information System (INIS)

Lemos, M.D.; Carvalho, G.B.S.; Carvalho, R.S.; Bichuetti, D.B.; Oliveira, E.M.L. de; Abdala, N.

2015-01-01

Aim: To describe the neuroradiological features and their prevalence in patients with neuromyelitis optica (NMO). Materials and methods: Two neuroradiologists independently reviewed 35 spinal cord and 37 brain MRI studies from patients with NMO. The examinations were analysed for the presence of lesion, topography, enhancement, and brain lesions suggestive of multiple sclerosis and/or NMO. Results: Seventy percent of the spinal cord lesions involved over three or more vertebral segments. Seventy-eight percent of brain scans were abnormal, and the most prevalent findings were non-specific foci of T2 hyperintensities in the cerebral white matter (55%) and brainstem lesions (52%). One patient had lesions disseminated in space compatible with multiple sclerosis according to 2010 revised McDonald criteria. Brain lesions suggestive of NMO occurred at least once in 17 (59%) patients. Conclusion: Spinal cord lesions were often longitudinally extensive and brain lesions were common, with the majority of patients having at least one distinctive NMO lesion. - Highlights: • Neuromyelitis optica (NMO) affects mainly the spinal cord but also the brain. • We accessed the radiological features of patients with NMO in brain and spinal cord. • We found a high prevalence of extensive myelitis and specific brain lesions. • Some brainstem lesions could be misdiagnosed if not actively searched. • A practical approach to evaluate suspected NMO images is presented

Membranous nephropathy with Neuromyelitis optica spectrum disorder.

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Li, Xiangling; Wang, YanQiang

2017-07-01

Membranous nephropathy (MN) accompanying Neuromyelitis optica spectrum disorders (NMOSD) has rarely been described previously. We recently presented a 45-year-old Chinese male presenting with recurrent lower extremity pitting edema, or eyelid edema, proteinuria and hyperlipidemia. especially intractable hiccup and vomiting, painful tonic spasm (PTS) as the revealing symptom of a demyelinating disorder of central nervous system. The kindey biopsy specimen showed MN stage 2. Serological testing revealed antibodies AQP4, MRI head and spine revealed medulla oblongata and C1-C2 cervical vertebra lesions. Treatment with methylprednisolone, cyclophosphamide, azathioprine resulted in consistent clinical improvement. Copyright © 2017 Elsevier B.V. All rights reserved.

Clinical characteristics of late-onset neuromyelitis optica spectrum disorder: A multicenter retrospective study in Korea.

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Seok, Jin Myoung; Cho, Hye-Jin; Ahn, Suk-Won; Cho, Eun Bin; Park, Min Su; Joo, In-Soo; Shin, Ha Young; Kim, Sun-Young; Kim, Byung-Jo; Kim, Jong Kuk; Cho, Joong-Yang; Huh, So-Young; Kwon, Ohyun; Lee, Kwang-Ho; Kim, Byoung Joon; Min, Ju-Hong

2017-11-01

There are currently few studies regarding late-onset neuromyelitis optica spectrum disorder (LO-NMOSD). We aimed to describe the characteristic features of patients with LO-NMOSD in Korea. Anti-aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder (NMOSD) from nine tertiary hospitals were reviewed retrospectively. The patients were divided into two groups based on age of onset: LO-NMOSD (⩾50 years of age at onset) versus early-onset neuromyelitis optica spectrum disorder (EO-NMOSD) (<50 years of age at onset). Clinical, laboratory, and magnetic resonance imaging (MRI) parameters were investigated. Among a total of 147 patients (125 female; age of onset, 39.4 ± 15.2 years), 45 patients (30.6%) had an age of onset of more than 50 years. Compared to patients with EO-NMOSD, patients with LO-NMOSD had more frequent isolated spinal cord involvement at onset (64.4% vs 37.2%, p = 0.002), less frequent involvement of the optic nerve (40.0% vs 67.7%, p = 0.002), and less frequent brain MRI lesions (31.1% vs 50.0%, p = 0.034). Furthermore, there was a significant positive correlation between age of onset and Expanded Disability Status Scale (EDSS) score at last follow-up ( r = 0.246, p = 0.003). Age of onset could be an important predictor of lesion location and clinical course of patients with NMOSD.

Brain perfusion SPECT analysis : New insights in mild cognitive impairment and neuromyelitis optica

NARCIS (Netherlands)

Sánchez Catasùs, Carlos Alfredo

2018-01-01

Het doel van dit proefschrift was om aan te tonen dat hersenperfusie SPECT kan helpen bij het verhelderen van belangrijke vragen met betrekking tot het prodromale “Mild Cognitive Impairment” (MCI) stadium van de ziekte van Alzheimer (AD) en recidiverende Neuromyelitis Optica (NMO). Dit is niet

Th17 cells in neuromyelitis optica spectrum disorder: a review.

Science.gov (United States)

Lin, Jie; Li, Xiang; Xia, Junhui

2016-12-01

Neuromyelitis optica spectrum disorder (NMOSD) has been identified as a central nervous system (CNS) autoimmune inflammatory disorder, which has been recognized as a B cell-mediated humoral immune disease. However, cases have been reported indicating that some of the neuromyelitis optica (NMO) patients have been resistant to B cell-related treatments. Recently, more and more evidence has shown that T cell-mediated immunity may take part in the pathogenesis of NMOSD, especially in the Th17 phenotype. In our PUBMED search, we used the following keywords: Th17 cell, Th17 cell-related cytokines, T cells, B cells, B cell-related productions, NMO, NMOSD, recurrent/bilateral optic neuritis, recurrent transverse myelitis and longitudinally extensive transverse myelitis. We systemically reviewed the role of Th17 cells and Th17 cell-related cytokines in NMOSD. We found that Th17 cells and Th17-related cytokines, such as IL-6, IL-1β, IL-17, IL-21, IL-22, IL-23 and TGF-β, are not only directly involved in the pathogenesis but also collaborated with B cells and B cell-related antibody production to induce CNS lesions. Th17 cell-related therapy has also been reviewed in this article, and the data suggested that Th17 may be a new therapeutic target of NMOSD.

High risk of postpartum relapses in neuromyelitis optica spectrum disorder.

Science.gov (United States)

Klawiter, Eric C; Bove, Riley; Elsone, Liene; Alvarez, Enrique; Borisow, Nadja; Cortez, Melissa; Mateen, Farrah; Mealy, Maureen A; Sorum, Jaime; Mutch, Kerry; Tobyne, Sean M; Ruprecht, Klemens; Buckle, Guy; Levy, Michael; Wingerchuk, Dean; Paul, Friedemann; Cross, Anne H; Jacobs, Anu; Chitnis, Tanuja; Weinshenker, Brian

2017-11-28

To study the effect of pregnancy on the frequency of neuromyelitis optica spectrum disorder (NMOSD) relapse and evaluate rates of pregnancy-related complications in an international multicenter setting. We administered a standardized survey to 217 women with NMOSD from 7 medical centers and reviewed their medical records. We compared the annualized relapse rate (ARR) during a baseline period 2 years prior to a participant's first pregnancy to that during pregnancy and to the 9 months postpartum. We also assessed pregnancy-related complications. There were 46 informative pregnancies following symptom onset in 31 women with NMOSD. Compared to baseline (0.17), ARR was increased both during pregnancy (0.44; p = 0.035) and during the postpartum period (0.69; p = 0.009). The highest ARR occurred during the first 3 months postpartum (ARR 1.33). A total of 8 of 76 (10.5%) with onset of NMOSD prior to age 40 experienced their initial symptom during the 3 months postpartum, 2.9 times higher than expected. The postpartum period is a particularly high-risk time for initial presentation of NMOSD. In contrast to published observations in multiple sclerosis, in neuromyelitis optica, relapse rate during pregnancy was also increased, although to a lesser extent than after delivery. © 2017 American Academy of Neurology.

Antibodies against aquaporin-4 in neuromyelitis optica: distinction between recurrent and monophasic patients

NARCIS (Netherlands)

Ketelslegers, I.A.; Modderman, P.W.; Vennegoor, A.; Killestein, J.; Hamann, D.; Hintzen, R.Q.

2011-01-01

The detection of antibodies against aquaporin-4 (AQP4) has improved the diagnosis of neuromyelitis optica (NMO). We evaluated a recently established cell-based anti-AQP4 assay in 273 patients with inflammatory CNS demyelination. The assay had a specificity of 99% and a sensitivity of 56% to detect

The Treatment of Neuromyelitis Optica

Science.gov (United States)

Kowarik, Markus C.; Soltys, John; Bennett, Jeffrey L.

2014-01-01

Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system directed against astrocytes. Initially diagnosed in individuals with monophasic or relapsing optic neuritis and transverse myelitis, NMO is now recognized as a demyelinating disorder with pleiotropic presentations due to the identification of a specific autoantibody response against the astrocyte water channel aquaporin-4 in the majority of individuals. As visual impairment and neurologic dysfunction in NMO are commonly severe, aggressive treatment of relapses and prophylactic immunomodulatory therapy are the focus of treatment. Although there are no approved treatments for NMO, medications and therapeutic interventions for acute and chronic treatment have been the subject of retrospective study and case reports. The goal of this review is to familiarize the reader with biologic and clinical data supporting current treatments in NMO and highlight future strategies based on advancements in our understanding of NMO pathogenesis. PMID:24531318

Treatment-resistant neuromyelitis optica spectrum disorders associated with Toxocara canis infection: A case report.

Science.gov (United States)

Kambe, Daisuke; Takeoka, Kayo; Ogawa, Kenta; Doi, Kosuke; Maruyama, Haruhiko; Yoshida, Ayako; Suenaga, Toshihiko; Kageyama, Takashi

2017-04-01

A 53-year-old woman was admitted to the department of neurology in Tenri Hospital because of progressive thoracic myelitis a month after she had eaten uncooked bovine liver. A previous episode of right optic neuritis and a positive test for serum anti-aquaporin-4 antibodies indicated a diagnosis of neuromyelitis optica spectrum disorders. Although the patient initially recovered with the reduction of anti-aquaporin-4 antibodies during treatment with intravenous methylprednisolone infusion and plasma exchange, her neurological symptoms deteriorated soon after the completion of plasma exchange. Western blotting analysis detected anti-Toxocara canis antibodies in the serum; thus, the patient underwent oral albendazole treatment. This resulted in the alleviation of her symptoms. We therefore consider that rigorous investigation should be encouraged to detect rare pathogens including parasites in cases of treatment-resistant neuromyelitis optica spectrum disorders. Copyright © 2017 Elsevier B.V. All rights reserved.

Neuromyelitis optica med udtalte cerebrale magnetisk resonans-skannings-forandringer hos en niårig pige

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Trautner, Simon; Pedersen, Hans Peder; Bendtson, Inger

2009-01-01

Neuromyelitis optica (NMO) is a rare inflammatory disorder characterised by optic neuritis and transverse myelitis. We report a severe pediatric case presenting with impaired vision, tetraparesis, bladder retention and lower extremity pain. Magnetic resonance imaging demonstrated longitudinally...

Gaze palsy, hypogeusia and a probable association with miscarriage of pregnancy--the expanding clinical spectrum of non-opticospinal neuromyelitis optica spectrum disorders: a case report.

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Chang, Thashi; Withana, Milinda

2015-02-10

Neuromyelitis optica is characterised by optic neuritis, longitudinally-extensive transverse myelitis and presence of anti-aquaporin-4 antibodies in the serum. However, non-opticospinal central nervous system manifestations have been increasingly recognised. Awareness of the widening clinical spectrum of neuromyelitis optica (unified within the nosology of 'neuromyelitis optica spectrum disorders') is key to earlier diagnosis and appropriate therapy. We report 2 patients to illustrate the varied clinical manifestations of neuromyelitis optica spectrum disorders while postulating an effect of anti-aquaporin-4 antibodies on the miscarriage of pregnancy. This is the first report of horizontal gaze palsy as a presenting symptom of neuromyelitis optica spectrum disorders. Patient 1: A 17-year-old Sri Lankan female presented with hypersomnolence, lateral gaze palsy and loss of taste of 1 week duration. Two years previously she had presented with intractable hiccups and vomiting followed by a brainstem syndrome. Magnetic resonance imaging showed a lesion in the left cerebellum extending into the pons while lesions in bilateral hypothalami and medulla noted 2 years ago had resolved. Autoimmune, vasculitis and infection screens were negative. Anti-aquaporin-4 antibodies were detected in serum. All her symptoms resolved with immunosuppressive therapy. Patient 2: A 47-Year-old Sri Lankan female presented with persistent vomiting lasting over 3 weeks. Three years previously, at 25-weeks of her 4(th) pregnancy, she had presented with quadriparesis and was found to have a longitudinally extensive transverse myelitis from C2 to T2 vertebral levels, which gradually improved following intravenous steroid therapy. Magnetic resonance imaging showed a hyper-intense lesion in the area postrema and longitudinally extensive atrophy of the cord corresponding to her previous myelitis. Autoimmune, vasculitis and infection screens were negative. Anti-aquaporin-4 antibodies were detected in

Neuromyelitis optica with Hashimoto′s thyroiditis: A new syndrome or just coincidence

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Abhishek Singhai

2015-01-01

Full Text Available Neuromyelitis optica (NMO is an uncommon disease syndrome of the central nervous system that affects the optic nerves and spinal cord. NMO with endocrinopathies has been described as being unique to black Antillean and Afro-Brazilian women. We describe one case of NMO with hashimoto′s thyroiditis in a young female, probably first case report in India.

Brainstem and limbic encephalitis with paraneoplastic neuromyelitis optica.

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Moussawi, Khaled; Lin, David J; Matiello, Marcelo; Chew, Sheena; Morganstern, Daniel; Vaitkevicius, Henrikas

2016-01-01

The spectrum of disorders associated with anti-neuromyelitis optica (NMO) antibody is being extended to include infrequent instances associated with cancer. We describe a patient with brainstem and limbic encephalitis from NMO-immunoglobulin G in serum and cerebrospinal fluid in the context of newly diagnosed breast cancer. The neurological features markedly improved with excision of her breast cancer and immune suppressive therapy. This case further broadens the NMO spectrum disorders (NMOSD) by an association between NMOSD and cancer and raises the question of coincidental occurrence and the appropriate circumstances to search for a tumor in certain instances of NMO. Copyright © 2015 Elsevier Ltd. All rights reserved.

Cognitive Impairment and Whole Brain Diffusion in Patients with Neuromyelitis Optica after Acute Relapse

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He, Diane; Wu, Qizhu; Chen, Xiuying; Zhao, Daidi; Gong, Qiyong; Zhou, Hongyu

2011-01-01

The objective of this study investigated cognitive impairments and their correlations with fractional anisotropy (FA) and mean diffusivity (MD) in patients with neuromyelitis optica (NMO) without visible lesions on conventional brain MRI during acute relapse. Twenty one patients with NMO and 21 normal control subjects received several cognitive…

Neuromyelitis optica spectrum disorder presenting with repeated hypersomnia due to involvement of the hypothalamus and hypothalamus-amygdala linkage.

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Kume, Kodai; Deguchi, Kazushi; Ikeda, Kazuyo; Takata, Tadayuki; Kokudo, Yohei; Kamada, Masaki; Touge, Tetsuo; Takahashi, Toshiyuki; Kanbayashi, Takashi; Masaki, Tsutomu

2015-06-01

We report the case of a 46-year-old Japanese woman with neuromyelitis optica spectrum disorder presenting with repeated hypersomnia accompanied by decreased CSF orexin level. First episode associated with hypothalamic-pituitary dysfunction showed bilateral hypothalamic lesions that can cause secondary damage to the orexin neurons. The second episode associated with impaired memory showed a left temporal lesion involving the amygdala. The mechanism remains unknown, but the reduced blood flow in the hypothalamus ipsilateral to the amygdala lesion suggested trans-synaptic hypothalamic dysfunction secondary to the impaired amygdala. A temporal lesion involving the amygdala and hypothalamus could be responsible for hypersomnia due to neuromyelitis optica spectrum disorder. © The Author(s), 2015.

An update on the evidence for the efficacy and safety of rituximab in the management of neuromyelitis optica

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Collongues, Nicolas; de Seze, Jérôme

2016-01-01

Neuromyelitis optica spectrum disorders (NMOSDs) is a new concept which includes classical neuromyelitis optica (NMO) and partial forms of NMO such as recurrent optic neuritis with positive aquaporin-4 antibodies (AQP4) or brainstem symptoms (intractable hiccups or vomiting). This disease is clearly distinguished from multiple sclerosis (MS) and the therapeutic approach is clearly different. Rituximab is actually considered to be one of the most efficient treatments of NMOSD, even if class I studies are clearly lacking. In the present review, we describe the state of the art about rituximab treatment in NMOSD, including adults and children, plus its efficacy and tolerance and we also underline the questions that should be addressed in the near future. PMID:27134673

An update on the evidence for the efficacy and safety of rituximab in the management of neuromyelitis optica.

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Collongues, Nicolas; de Seze, Jérôme

2016-05-01

Neuromyelitis optica spectrum disorders (NMOSDs) is a new concept which includes classical neuromyelitis optica (NMO) and partial forms of NMO such as recurrent optic neuritis with positive aquaporin-4 antibodies (AQP4) or brainstem symptoms (intractable hiccups or vomiting). This disease is clearly distinguished from multiple sclerosis (MS) and the therapeutic approach is clearly different. Rituximab is actually considered to be one of the most efficient treatments of NMOSD, even if class I studies are clearly lacking. In the present review, we describe the state of the art about rituximab treatment in NMOSD, including adults and children, plus its efficacy and tolerance and we also underline the questions that should be addressed in the near future.

Acute Respiratory Failure due to Neuromyelitis Optica Treated Successfully with Plasmapheresis

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Massa Zantah

2016-01-01

Full Text Available Neuromyelitis Optica (NMO is a demyelinating autoimmune disease involving the central nervous system. Acute respiratory failure from cervical myelitis due to NMO is known to occur but is uncommon in monophasic disease and is treated with high dose steroids. We report a case of a patient with NMO who developed acute respiratory failure related to cervical spinal cord involvement, refractory to pulse dose steroid therapy, which resolved with plasmapheresis.

Continuous Positive Airway Pressure (CPAP for prevention of recurrent pneumonia in the Neuromyelitis Optica patient

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James A. Welker

2014-01-01

Conclusions: Patients with Neuromyelitis Optica differ from those with traumatic spinal cord injury as they have a chronic progressive systemic illness that causes continued deterioration of their nervous system resulting in the need for routine monitoring that ensures the timely addition of CPAP for the prevention of pneumonia and its associated medical expenses.

A Case of Neuromyelitis Optica Masquerading as Miller Fisher Syndrome

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Yuka Furutani

2014-10-01

Full Text Available A 22-year-old woman presented with double vision that she had experienced since an infection 2 weeks previously. A neurological examination showed limited bilateral eye abduction, mimicking Miller Fisher syndrome. However, T2-weighted magnetic resonance imaging of her brain revealed hyperintense areas in the tegmentum of the pons, including the abducens nucleus, and her serum anti-aquaporin-4 antibody test was positive. She was finally diagnosed with neuromyelitis optica. Intravenous high-dose steroid therapy immediately improved the patient's abduction palsy, but bilateral optic neuritis manifested during the treatment. Subsequent treatment with plasma exchange improved her optic neuritis symptoms.

Neuromyelitis Optica: A Case Report

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Wei-Chia Chia

2010-12-01

Full Text Available Neuromyelitis optica (NMO, Devic's syndrome is a demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. It is often confused with multiple sclerosis. Early discrimination between NMO and multiple sclerosis is important because the two diseases have different natural histories and treatment regimens. Seropositivity for NMO-IgG and longitudinally extensive spinal cord lesions (3 or more spinal segments are characteristic of NMO. Despite the absence of a definitive therapeutic strategy for NMO syndrome, methylprednisolone pulse therapy is recommended in the acute phase. Treatment strategies in relapse phases are aimed at preventing relapses, and increasing evidence shows a better clinical response of immunosuppressive therapy than immuno-modulating therapy (a standard multiple sclerosis-modulating therapy. We describe a 10-year-old girl who had visual loss due to acute optic neuritis at 6 years old and suffered repetitive myelitis 2 years later. NMO was diagnosed because of characteristic longitudinal myelitis and positive NMO-IgG. After combining therapy with prednisolone and an immunosuppressant (cyclophosphamide, the patient's medical condition was stable and no relapse symptoms were observed.

Change in autoantibody and cytokine responses during the evolution of neuromyelitis optica in patients with systemic lupus erythematosus

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Kovacs, Katalin T; Kalluri, Sudhakar Reddy; Boza-Serrano, Antonio

2016-01-01

BACKGROUND: Neuromyelitis optica (NMO)-systemic lupus erythematosus (SLE) association is a rare condition characterized by multiple autoantibodies. OBJECTIVE: To examine if, during the evolution of NMO, anti-AQP4 responses are part of polyclonal B cell activation, and if T cell responses contribute...

Convergence spasm due to aquaporin-positive neuromyelitis optica spectrum disorder

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Pınar Özçelik

2017-06-01

Full Text Available A female 27 presented with nausea and diplopia for 1 week. On examination she had normal vertical gaze but would develop convergence with miosis whenever she made horizontal saccades. Pupils were 6 mm and unreactive to light. MRI showed extensive hyperintensity in the dorsal midbrain and thalamus. Spinal MRI and CSF were both normal. Serum aquaporin-4-antibody was positive. She was treated with steroids and plasmapheresis and after 3 months convergence spasm resolved but pupils remained unreactive. Neuromyelitis optica often presents with brainstem signs, rarely a dorsal midbrain syndrome. Convergence spasm is occasionally of organic neurologic origin.

Autoantibodies in patients with neuromyelitis optica

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Xin FAN

2016-10-01

Full Text Available Neuromyelitis optica (NMO is an autoimmune disease of the central nervous system (CNS which primarily involves the optic nerve and spinal cord. Aquaporin 4 (AQP4 is the main objective antigen, and its specific antibody was NMO-IgG. It was found in clinic that most of NMO-IgG-positive patients were female, whose clinical symptoms were more severe, bilateral optic neuritis (BON or optic neuritis (ON and myelitis were more likely to appear at the same time, and involved spinal segments were longer. Recent studies discovered that anti-aquaporin 1 (AQP1 and anti-myelin oligodendrocyte glycoprotein (MOG antibodies existed in the serum of patients with NMO-IgG-negative. It was discovered that low proportions of women, more cases of long-segment spinal cord lesion, and rare cases of ON appeared in anti-AQP1 antibody-positive patients. Most of anti-MOG antibody-positive patients were male. ON was common, especially bilateral optic nerves involved at the same time, and thoracolumbar involvement was common. DOI: 10.3969/j.issn.1672-6731.2016.10.004

Neuromyelitis optica presenting with horner syndrome: A case report and review of literature.

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Uludağ, İrem Fatma; Sarıteke, Alp; Öcek, Levent; Zorlu, Yaşar; Şener, Ufuk; Tokuçoğlu, Figen; Uludağ, Burhanettin

2017-05-01

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. Copyright © 2017 Elsevier B.V. All rights reserved.

Molecular Pathogenesis of Neuromyelitis Optica

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Bukhari, Wajih; Barnett, Michael H; Prain, Kerri; Broadley, Simon A

2012-01-01

Neuromyelitis optica (NMO) is a rare autoimmune disorder, distinct from multiple sclerosis, causing inflammatory lesions in the optic nerves and spinal cord. An autoantibody (NMO IgG) against aquaporin-4 (AQP4), a water channel expressed on astrocytes is thought to be causative. Peripheral production of the antibody is triggered by an unknown process in genetically susceptible individuals. Anti-AQP4 antibody enters the central nervous system (CNS) when the blood brain barrier is made permeable and has high affinity for orthogonal array particles of AQP4. Like other autoimmune diseases, Th17 cells and their effector cytokines (such as interleukin 6) have been implicated in pathogenesis. AQP4 expressing peripheral organs are not affected by NMO IgG, but the antibody causes extensive astrocytic loss in specific regions of the CNS through complement mediated cytotoxicity. Demyelination occurs during the inflammatory process and is probably secondary to oligodendrocyte apoptosis subsequent to loss of trophic support from astrocytes. Ultimately, extensive axonal injury leads to severe disability. Despite rapid advances in the understanding of NMO pathogenesis, unanswered questions remain, particularly with regards to disease mechanisms in NMO IgG seronegative cases. Increasing knowledge of the molecular pathology is leading to improved treatment strategies. PMID:23202933

A systematic analysis of the complement pathways in patients with neuromyelitis optica indicates alteration but no activation during remission

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Veszeli, Nóra; Füst, György; Csuka, Dorottya

2014-01-01

Neuromyelitis optica (NMO) is an autoimmune demyelinating inflammatory disorder, mediated by pathogenic autoantibodies against aquaporin 4 (AQP4), the main water channel of the central nervous system (CNS). NMO is characterized by local IgG deposition and complement activation within the CNS...

Sudden onset of sleep due to hypothalamic lesions in neuromyelitis optica spectrum disorder positive for anti-aquaporin-4 antibody.

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Okuma, H; Matsumura, K; Hatanaka, Y; Saito, F; Sonoo, M

2014-09-01

We report a patient with neuromyelitis optica spectrum disorders who presented with sudden onset of sleep as the sole manifestation. Magnetic resonance imaging investigation revealed lesions in the hypothalamus bilaterally, which vanished completely after methylprednisolone pulse therapy. © The Author(s) 2014.

Visual evoked potentials in neuromyelitis optica and its spectrum disorders.

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Ringelstein, Marius; Kleiter, Ingo; Ayzenberg, Ilya; Borisow, Nadja; Paul, Friedemann; Ruprecht, Klemens; Kraemer, Markus; Cohn, Eva; Wildemann, Brigitte; Jarius, Sven; Hartung, Hans-Peter; Aktas, Orhan; Albrecht, Philipp

2014-04-01

Optic neuritis (ON) is a key feature of neuromyelitis optica (NMO). Recently, NMO patients of predominantly Afro-Brazilian origin were evaluated by visual evoked potentials (VEPs) and showed marked amplitude reductions. Here, we analyzed VEPs in a predominantly Caucasian cohort, consisting of 43 patients with definite NMO, 18 with anti-aquaporin (AQP) 4 antibody-seropositive NMO spectrum disorders and 61 matched healthy controls. We found reduced amplitudes in only 12.3%, prolonged latencies in 41.9% and a lack of response in 14.0% of NMO eyes. Delayed P100 latencies in eyes without prior ON suggested this was a subclinical affection. The data indicate heterogenous patterns in NMO, warranting further investigation.

Protective effect of an elastase inhibitor in a neuromyelitis optica-like disease driven by a peptide of myelin oligodendroglial glycoprotein.

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Herges, K.; Jong, B.A. de; Kolkowitz, I.; Dunn, C.; Mandelbaum, G.; Ko, R.M.; Maini, A.; Han, M.H.; Killestein, J.; Polman, C.; Goodyear, A.L.; Dunn, J.; Steinman, L.; Axtell, R.C.

2012-01-01

BACKGROUND: The pathology of neuromyelitis optica (NMO), in contrast to multiple sclerosis, comprises granulocyte infiltrates along extensive lengths of spinal cord, as well as optic nerve. Furthermore, IFN-beta treatment worsens NMO. We recently found that experimental autoimmune encephalomyelitis

Seronegative Neuromyelitis Optica Spectrum Disorder following Exposure to Hepatitis B Vaccination

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Richard Heekin

2015-04-01

Full Text Available Controversy exists regarding a potential link between exposure to recombinant hepatitis B vaccine (HBV and central nervous system demyelinating diseases. Here, we present a case of seronegative neuromyelitis optica spectrum disorder (NMOSD following exposure to HBV. A 28-year-old man developed painful eye movements 11 days after exposure to HBV. Within 24 h, he experienced vision loss, ascending numbness, and ataxia. T-spine MRI showed a cord lesion spanning T6-T9. Brain MRI showed bilateral optic nerve contrast enhancement and a right-sided internal capsule lesion. Cerebrospinal fluid analysis was normal, including negative oligoclonal bands and normal IgG index. AQP4-IgG serology was negative. The patient's visual symptoms improved after treatment with steroids and plasma exchange. He received plasma exchange weekly for 4 weeks with decreased numbness and tingling as well as improved coordination. Treatment with mycophenolate mofetil was started, and the patient remains clinically stable with near resolution of his prior symptoms. Neuromyelitis optica is characterized by optic neuritis and/or longitudinally extensive transverse myelitis. While our patient tested seronegative for AQP4-IgG (which remains negative in 10-50% of NMOSD cases, despite testing with the most sensitive assays available, he did meet NMOSD diagnostic criteria. In a literature review, we found 7 cases of NMOSD onset or relapse associated with exposure to various vaccines, but to our knowledge this represents the first published report of NMOSD onset following exposure to HBV. While causality between vaccination and CNS demyelinating disease remains elusive, it is important to report these cases to help develop safer vaccinations and provoke further inquiry into the pathogenesis of NMOSD.

Depressive state and chronic fatigue in multiple sclerosis and neuromyelitis optica.

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Akaishi, Tetsuya; Nakashima, Ichiro; Misu, Tatsuro; Fujihara, Kazuo; Aoki, Masashi

2015-06-15

Depression and chronic fatigue are frequently present in multiple sclerosis (MS); however, the prevalence rates have not been investigated in neuromyelitis optica (NMO). Thirty-nine consecutive NMO and 75 MS patients were compared using self-rating questionnaires for depressive states, daily activity, and fatigue, as well as serum carnitine levels. A subgroup of patients with low carnitine levels were re-evaluated regarding depression and fatigue after levocarnitine treatment. Depression and fatigue were equally prevalent in MS and NMO and were strongly correlated with one another. Measurement of the serum carnitine levels and the administration of levocarnitine did not appear to be beneficial. Copyright © 2015 Elsevier B.V. All rights reserved.

Brain MRI abnormalities in neuromyelitis optica

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Wang Fei, E-mail: feiwang1973@gmail.com [Department of Radiology, Xuanwu Hospital, Capital University of Medical Sciences, 45 Chang-Chun St, Xuanwu District, Beijing 100053 (China); Liu Yaou, E-mail: asiaeurope80@gmail.com [Department of Radiology, Xuanwu Hospital, Capital University of Medical Sciences, 45 Chang-Chun St, Xuanwu District, Beijing 100053 (China); Duan Yunyun, E-mail: duanyun2003@sohu.com [Department of Radiology, Xuanwu Hospital, Capital University of Medical Sciences, 45 Chang-Chun St, Xuanwu District, Beijing 100053 (China); Li Kuncheng, E-mail: kunchengli@yahoo.com.cn [Department of Radiology, Xuanwu Hospital, Capital University of Medical Sciences, 45 Chang-Chun St, Xuanwu District, Beijing 100053 (China); Education Ministry Key Laboratory for Neurodegenerative Disease, Xuanwu Hospital, Capital University of Medical Sciences, 45 Chang-Chun St, Xuanwu District, Beijing 100053 (China)

2011-11-15

Objective: The purpose of this study was to explore brain MRI findings in neuromyelitis optica (NMO) and to investigate specific brain lesions with respect to the localization of aquaporin-4 (AQP-4). Materials and methods: Forty admitted patients (36 women) who satisfied the 2006 criteria of Wingerchuk et al. for NMO were included in this study. All patients received a neurological examination and MRI scanning including brain and spinal cord. MRIs were classified as normal, nonspecific, multiple sclerosis-like, typical abnormalities. MS-like lesions were too few to satisfy the Barkhof et al. criteria for MS. Confluent lesions involving high AQP-4 regions were considered typical. Non-enhancing deep white matter lesions other than MS-like lesions or typical lesions were classified as nonspecific. Results: Brain MRI lesions were delineated in 12 patients (25%). Four patients (10%) had hypothalamus, brainstem or periventricle lesions. Six (15%) patients were nonspecific, and 2 (5%) patients had multiple sclerosis-like lesions. Conclusion: Brain MRIs are negative in most NMO, and brain lesions do not exclude the diagnosis of NMO. Hypothalamus, brainstem or periventricle lesions, corresponding to high sites of AQP-4 in the brain, are indicative of lesions of NMO.

Brain MRI abnormalities in neuromyelitis optica

International Nuclear Information System (INIS)

Wang Fei; Liu Yaou; Duan Yunyun; Li Kuncheng

2011-01-01

Objective: The purpose of this study was to explore brain MRI findings in neuromyelitis optica (NMO) and to investigate specific brain lesions with respect to the localization of aquaporin-4 (AQP-4). Materials and methods: Forty admitted patients (36 women) who satisfied the 2006 criteria of Wingerchuk et al. for NMO were included in this study. All patients received a neurological examination and MRI scanning including brain and spinal cord. MRIs were classified as normal, nonspecific, multiple sclerosis-like, typical abnormalities. MS-like lesions were too few to satisfy the Barkhof et al. criteria for MS. Confluent lesions involving high AQP-4 regions were considered typical. Non-enhancing deep white matter lesions other than MS-like lesions or typical lesions were classified as nonspecific. Results: Brain MRI lesions were delineated in 12 patients (25%). Four patients (10%) had hypothalamus, brainstem or periventricle lesions. Six (15%) patients were nonspecific, and 2 (5%) patients had multiple sclerosis-like lesions. Conclusion: Brain MRIs are negative in most NMO, and brain lesions do not exclude the diagnosis of NMO. Hypothalamus, brainstem or periventricle lesions, corresponding to high sites of AQP-4 in the brain, are indicative of lesions of NMO.

Neuromyelitis optica spectrum disease characteristics in Isfahan, Iran: A cross-sectional study

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Fereshte Ashtari

2017-01-01

Full Text Available Background: Neuromyelitis optica spectrum disease (NMOSD is a severe autoimmune demyelinating disorder of the central nervous system that throughout epidemiological data, it has not been completely determined. The aim of this study was to assess characteristics of NMOSD patients in Isfahan as one of the most prevalent cities for multiple sclerosis in Iran. Materials and Methods: Forty-five patients diagnosed as neuromyelitis optica (NMO disease through 5 years enrolled in this study. Demographics and characteristics of disease such as Expanded Disability Status Scale (EDSS score, disease duration, clinical symptoms, laboratory data, and magnetic resonance imaging findings (including T1, T2, and flair protocols were recorded. NMO-immunoglobulin G serology assay was done in all of the patients by ELISA test. Results: Female to male ratio was 5.4:1. The mean age of disease onset was 29.8 ± 11.2 years. NMO antibody was positive in 24.4% of patients. The presenting symptoms were optic neuritis (55.5%, transverse myelitis (40%, and brainstem symptoms (4.5%. The interval between the first and second attack was 19.28 ± 31.27 months (range: 1 month to 17 years. The mean EDSS score of the patients was 2.8 ± 2.25. Frequency of long-extending cervical plaque was higher among men than women (85.7% vs. 57.9%. Conclusion: Based on this study, the mean age of NMOSD onset among Isfahan population was considerably lower than other studies, and there was higher frequency of long-extending cervical lesion among male patients which needs more consideration in further studies.

Enhancing Brain Lesions during Acute Optic Neuritis and/or Longitudinally Extensive Transverse Myelitis May Portend a Higher Relapse Rate in Neuromyelitis Optica Spectrum Disorders.

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Orman, G; Wang, K Y; Pekcevik, Y; Thompson, C B; Mealy, M; Levy, M; Izbudak, I

2017-05-01

Neuromyelitis optica spectrum disorders are inflammatory demyelinating disorders with optic neuritis and/or longitudinally extensive transverse myelitis episodes. We now know that neuromyelitis optica spectrum disorders are associated with antibodies to aquaporin-4, which are highly concentrated on astrocytic end-feet at the blood-brain barrier. Immune-mediated disruption of the blood-brain barrier may manifest as contrast enhancement on brain MR imaging. We aimed to delineate the extent and frequency of contrast enhancement on brain MR imaging within 1 month of optic neuritis and/or longitudinally extensive transverse myelitis attacks and to correlate contrast enhancement with outcome measures. Brain MRIs of patients with neuromyelitis optica spectrum disorders were evaluated for patterns of contrast enhancement (periependymal, cloudlike, leptomeningeal, and so forth). The Fisher exact test was used to evaluate differences between the proportion of contrast enhancement in patients who were seropositive and seronegative for aquaporin-4 antibodies. The Mann-Whitney test was used to compare the annualized relapse rate and disease duration between patients with and without contrast enhancement and with and without seropositivity. Brain MRIs of 77 patients were evaluated; 59 patients (10 males, 49 females) were scanned within 1 month of optic neuritis and/or longitudinally extensive transverse myelitis attacks and were included in the analysis. Forty-eight patients were seropositive, 9 were seronegative, and 2 were not tested for aquaporin-4 antibodies. Having brain contrast enhancement of any type during an acute attack was significantly associated with higher annualized relapse rates ( P = .03) and marginally associated with shorter disease duration ( P = .05). Having periependymal contrast enhancement was significantly associated with higher annualized relapse rates ( P = .03). Brain MRIs of patients with neuromyelitis optica spectrum disorders with contrast

Pulmonary tuberculosis with neuromyelitis optica: an uncommon association of a common disease

International Nuclear Information System (INIS)

Siddiqi, S.A.; Hashmi, M.; Azmat, Z.; Mustafa, S.; Siddiqui, K.A.

2012-01-01

Systemic tuberculosis has been reported with varying neurological manifestations like meningitis, tuberculomas, myositis and neuropathy. Neuromyelitis optica (NMO) is a well known neurological entity which has been described in association with several systemic disorders like systemic lupus erythematosis, diabetes mellitus, hypothyroidism, exposure to Mycobacterium tuberculosis. insecticides etc. However, only a few cases of NMO have been reported in association with Here, we report a case of pulmonary tuberculosis in association with NMO to highlight the under-reported association of NMO with pulmonary tuberculosis presenting in a peculiar anatomical fashion i.e. longitudinal myelitis with predominant posterior column involvement. (author)

Autoimmune thyroiditis associated with neuromyelitis optica (NMO

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Sudulagunta, Sreenivasa Rao

2015-11-01

Full Text Available Neuromyelitis optica (NMO or Devic’s syndrome is a rare relapsing demyelinating disease of the central nervous system (CNS that mainly affects the spinal cord and optic nerves and shares many clinical and radiological features with multiple sclerosis. The association of NMO with other autoimmune diseases was reported, but very few reports described association with autoimmune thyroid disease. Early differentiation between NMO and multiple sclerosis is very important as the natural course and treatment regimens differ significantly. We report a case of a 50-year-old woman who was admitted initially with vomiting, hiccups and paraesthesias but was not diagnosed with NMO and presented with a severe progression of the disease. The patient was also diagnosed to have autoimmune thyroiditis with lymphocytic infiltration of the thyroid which progressed from hyperthyroidism to hypothyroidism. NMO diagnosis was established with seropositivity for NMO-IgG and MRI showing longitudinally extensive spinal cord lesions (3 or more spinal segments. In spite of treatment, the response was poor due to lack of early diagnosis and aggressive immunosuppressant therapy.

Finding NMO: The Evolving Diagnostic Criteria of Neuromyelitis Optica

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Bennett, Jeffrey L.

2016-01-01

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). The routine application of new diagnostic criteria for NMOSD in clinical practice will be critical for future refinement and correlation with therapeutic outcomes. PMID:27529327

Seronegative neuromyelitis optica presenting with life-threatening respiratory failure.

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Nardone, Raffaele; Zuccoli, Giulio; Brigo, Francesco; Trinka, Eugen; Fitzgerald, Ryan T

2016-11-01

Dyspnea has rarely been reported as a presenting symptom in patients with neuromyelitis optica (NMO). We report an unusual case of NMO relapse presenting with rapidly progressive respiratory failure and briefly discuss the possible pathophysiological mechanisms of this potential life-threatening complication of NMO. The 58-year-old woman with a history of bilateral optic neuritis presented to the emergency department with rapidly worsening dyspnea. Cervical spine magnetic resonance imaging showed extensive abnormal signal with involvement of the medulla oblongata. Since in our patient chest radiography failed to disclose a diaphragmatic palsy that is commonly observed in patients with phrenic nerve involvement, this acute manifestation of the disease may be attributed to brainstem involvement instead of cervical myelitis. Clinicians should be aware of this atypical presentation of NMO, which needs to be promptly recognized and aggressively treated.

Plasma Exchange in Severe Attacks of Neuromyelitis Optica

Directory of Open Access Journals (Sweden)

Mickael Bonnan

2012-01-01

Full Text Available Background. Neuromyelitis optica (NMO attacks are poorly controlled by steroids and evolve in stepwise neurological impairments. Assuming the strong humoral response underlying NMO attacks, plasma exchange (PLEX is an appropriate technique in severe NMO attacks. Objective. Presenting an up-to-date review of the literature of PLEX in NMO. Methods. We summarize the rationale of PLEX in relation with the physiology of NMO, the main technical aspects, and the available studies. Results. PLEX in severe attacks from myelitis or optic neuritis are associated with a better outcome, depending on PLEX delay (“time is cord and eyes”. NMO-IgG status has no influence. Finally, we build up an original concept linking the inner dynamic of the lesion, the timing of PLEX onset and the expected clinical results. Conclusion. PLEX is a safe and efficient add-on therapy in NMO, in synergy with steroids. Large therapeutic trials are required to definitely assess the procedure and define the time opportunity window.

Interferon Alpha Association with Neuromyelitis Optica

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Nasrin Asgari

2013-01-01

Full Text Available Interferon-alpha (IFN-α has immunoregulatory functions in autoimmune inflammatory diseases. The goal of this study was to determine occurrence and clinical consequences of IFN-α in neuromyelitis optica (NMO patients. Thirty-six NMO and 41 multiple sclerosis (MS patients from a population-based retrospective case series were included. Expanded Disability Status Scale (EDSS score and MRI findings determined disease activity. Linear regression was used to assess the effects of the level of IFN-α on disability (EDSS. IFN-α was determined by sensitive ELISA assays. IFN-α was detectable in sera from 9/36 NMO patients, significantly more often than in the MS group (2/41 (P=0.0197. A higher frequency of IFN-α was observed in NMO patients with acute relapse compared to NMO patients in remission (P<0.001 and compared to the MS patients with relapse (P=0.010. In NMO patients, the levels of IFN-α were significantly associated with EDSS (P=0.0062. It may be concluded that IFN-α was detectable in a subgroup of NMO patients. Association of IFN-α levels with clinical disease activity and severity suggests a role for IFN-α in disease perpetuation and may provide a plausible explanation for a negative effect of IFN-1 treatment in NMO patients.

What do we know about brain contrast enhancement patterns in neuromyelitis optica?☆

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Pekcevik, Yeliz; Orman, Gunes; Lee, In Ho; Mealy, Maureen A.; Levy, Michael; Izbudak, Izlem

2016-01-01

Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system that usually presents with acute myelitis and/or optic neuritis. Recently, some brain magnetic resonance imaging findings have been described in NMO that are important in the differential diagnosis. Pencil-thin, leptomeningeal, and cloud-like enhancement may be specific to NMO. These patterns are usually seen during relapses. Recognizing these lesions and enhancement patterns may expedite the diagnosis and allows early effective treatment. The purpose of this article is to review the latest knowledge and to share our experience with the contrast enhancement patterns of NMO brain lesions. PMID:26615899

Comparison of the efficacy of azathioprine and rituximab in neuromyelitis optica spectrum disorder

DEFF Research Database (Denmark)

Nikoo, Zahra; Badihian, Shervin; Shaygannejad, Vahid

2017-01-01

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...... = 0–7). Patients were randomized into two groups, which did not differ according to age, gender distribution, and disease duration. In the AZA group, 35 patients [20 aquaporin-4 (AQP4)-IgG positive] were started on 50 mg/day oral AZA and increased to 2–3 mg/kg/day (with oral prednisolone as adjunctive...

Epidemiological, clinical and immunological aspects of neuromyelitis optica (NMO)

DEFF Research Database (Denmark)

Asgari, Nasrin

2013-01-01

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease (IDD) of the central nervous system (CNS) and probably the most common non-multiple sclerosis (MS) CNS IDD. Serum immunoglobulin G autoantibodies have been identified in the majority of NMO patients with the water channel aquaporin...... on either the Wingerchuk 2006 criteria or the United States National Multiple Sclerosis Society 2008 criteria could be made purely on clinical grounds in a high proportion (64%) of cases. Heterogeneity of clinical NMO manifestations including optic neuritis, longitudinal extensive transverse myelitis (LETM...... with specific clinical, immunogenetic and experimental perspectives. The yearly incidence rate of NMO in the population was estimated to be 0.4 per 105 person-years (95% CI 0.30-0.54) and the prevalence was 4.4 per 105 (95% CI 3.1-5.7). The results indicated that NMO is more common in a Caucasian population...

OPTIC NEURITIS IN NEUROMYELITIS OPTICA

Science.gov (United States)

Levin, Marc H.; Bennett, Jeffrey L.; Verkman, A.S.

2013-01-01

Neuromyelitis optica (NMO) is an autoimmune demyelinating disease associated with recurrent episodes of optic neuritis and transverse myelitis, often resulting in permanent blindness and/or paralysis. The discovery of autoantibodies (AQP4-IgG) that target aquaporin-4 (AQP4) has accelerated our understanding of the cellular mechanisms driving NMO pathogenesis. AQP4 is a bidirectional water channel expressed on the plasma membranes of astrocytes, retinal Müller cells, skeletal muscle, and some epithelial cells in kidney, lung and the gastrointestinal tract. AQP4 tetramers form regular supramolecular assemblies at the cell plasma membrane called orthogonal arrays of particles. The pathological features of NMO include perivascular deposition of immunoglobulin and activated complement, loss of astrocytic AQP4, inflammatory infiltration with granulocyte and macrophage accumulation, and demyelination with axon loss. Current evidence supports a causative role of AQP4-IgG in NMO, in which binding of AQP4-IgG to AQP4 orthogonal arrays on astrocytes initiates complement-dependent and antibody-dependent cell-mediated cytotoxicity and inflammation. Immunosuppression and plasma exchange are the mainstays of therapy for NMO optic neuritis. Novel therapeutics targeting specific steps in NMO pathogenesis are entering the development pipeline, including blockers of AQP4-IgG binding to AQP4 and inhibitors of granulocyte function. However, much work remains in understanding the unique susceptibility of the optic nerves in NMO, in developing animal models of NMO optic neuritis, and in improving therapies to preserve vision. PMID:23545439

Brain Abnormalities in Neuromyelitis Optica Spectrum Disorder

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Woojun Kim

2012-01-01

Full Text Available Neuromyelitis optica (NMO is an idiopathic inflammatory syndrome of the central nervous system that is characterized by severe attacks of optic neuritis (ON and myelitis. Until recently, NMO was considered a disease without brain involvement. However, since the discovery of NMO-IgG/antiaqaporin-4 antibody, the concept of NMO was broadened to NMO spectrum disorder (NMOSD, and brain lesions are commonly recognized. Furthermore, some patients present with brain symptoms as their first manifestation and develop recurrent brain symptoms without ON or myelitis. Brain lesions with characteristic locations and configurations can be helpful in the diagnosis of NMOSD. Due to the growing recognition of brain abnormalities in NMOSD, these have been included in the NMO and NMOSD diagnostic criteria or guidelines. Recent technical developments such as diffusion tensor imaging, MR spectroscopy, and voxel-based morphometry reveal new findings related to brain abnormalities in NMOSD that were not identified using conventional MRI. This paper focuses on the incidence and characteristics of the brain lesions found in NMOSD and the symptoms that they cause. Recent studies using advanced imaging techniques are also introduced.

Monoclonal antibody therapy for neuromyelitis optica spectrum disorder: current and future.

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Lin, Jie; Xue, Binbin; Li, Xiang; Xia, Junhui

2017-08-01

Monoclonal-antibody has been used for patients with autoimmune disorders for several years, and efficacy and safety were appreciated for these patients. Neuromyelitis optica specturm disorder (NMOSD) has been defined as an autoimmune demyelination disorder of the central nervous system (CNS) with a course of relapse-remission. Treatment of prevention is important for patients with NMOSD because of the increased disability after several attacks. Multiple factors were involved in the pathogenesis of NMOSD. Currently, targeting specific factor was favored in the research into the treatment for NMOSD. Previous studies reported the efficacy and tolerance in NMOSD for drugs such as rituximab, tocilizumab, and eculizumab. The aim of this article is to review the current monoclonal therapies for NMOSD patients, and also future alternative options.

Quantitative Susceptibility Mapping Indicates a Disturbed Brain Iron Homeostasis in Neuromyelitis Optica ? A Pilot Study

OpenAIRE

Doring, Thomas Martin; Granado, Vanessa; Rueda, Fernanda; Deistung, Andreas; Reichenbach, Juergen R.; Tukamoto, Gustavo; Gasparetto, Emerson Leandro; Schweser, Ferdinand

2016-01-01

Dysregulation of brain iron homeostasis is a hallmark of many neurodegenerative diseases and can be associated with oxidative stress. The objective of this study was to investigate brain iron in patients with Neuromyelitis Optica (NMO) using quantitative susceptibility mapping (QSM), a quantitative iron-sensitive MRI technique. 12 clinically confirmed NMO patients (6 female and 6 male; age 35.4y±14.2y) and 12 age- and sex-matched healthy controls (7 female and 5 male; age 33.9±11.3y) underwen...

Use of Advanced Magnetic Resonance Imaging Techniques in Neuromyelitis Optica Spectrum Disorder

DEFF Research Database (Denmark)

Kremer, S.; Renard, F.; Achard, S.

2015-01-01

Brain parenchymal lesions are frequently observed on conventional magnetic resonance imaging (MRI) scans of patients with neuromyelitis optica (NMO) spectrum disorder but the specific morphological and temporal patterns distinguishing them uneqtaivcally from lesions caused by other disorders have...... not been identified. This literature review summarizes the literature on advanced quantitative imaging measures reported for patients with NMO spectrum disorder, including proton MR spectroscopy, diffusion tensor imaging, magnetization transfer imaging, quantitative MR voltametry, and ultrahigh...... diffusion-weighted imaging and brain tissue volumetry indicate greater white matter than gray matter degradation. These findings could be confirmed by ultrahigh-field MRI. The use of nonconventional MR I techniques may further our understanding of the pathogenic processes hi NMO spectrum disorders and may...

Solitary pulmonary nodule: radiologic features and diagnostic approach; Nodulo pulmonar solitario: caracteristicas radiologicas y abordaje diagnostico

Energy Technology Data Exchange (ETDEWEB)

Rodriguez Cambronero, Luis Enrique

2012-07-01

A literature review is conducted on the solitary pulmonary nodule, to determine the diagnostic methods and specific characteristics. The diagnostic methods used have been: chest radiography, computed tomography, positron emission tomography and magnetic resonance imaging. The radiological features are defined: location, size, definition of contours or edges (margins), densitometric and attenuation characteristics, cavitation, air bronchogram, growth, doubling time, satellite nodules, nutrient vessels [Spanish] Una revision bibliografica es realizada sobre el nodulo pulmonar solitario, para determinar los metodos de diagnostico y caracteristicas especificas. Los metodos de diagnostico utilizados han sido: la radiografia de torax, tomografia computarizada, tomografia por emision de positrones y resonancia magnetica. Las caracteristicas radiologicas son definidas: localizacion, tamano, definicion de los bordes o contornos (margenes), caracteristicas densitometricas y de atenuacion, cavitacion, broncograma aereo, crecimiento, tiempo de duplicacion, nodulos satelite, vasos nutrientes.

Seronegative Neuromyelitis Optica Spectrum - The challenges on disease definition and pathogenesis

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Douglas Kazutoshi Sato

2014-06-01

Full Text Available Neuromyelitis optica spectrum disorders (NMOSD are characterized by severe optic neuritis and/or longitudinally extensive transverse myelitis, and some brain lesions are also unique to NMOSD. Serum autoantibodies against aquaporin-4 (AQP4 are detected in most cases of NMOSD. However, some patients with NMOSD remain seronegative despite repetitive testing during attacks with highly sensitive cell-based assays. The differential diagnosis of NMOSD is not restricted to multiple sclerosis and it includes many diseases that can produce longitudinally extensive myelitis and/or optic neuritis. We review the clinical features, imaging, and laboratory findings that can be helpful on the diagnostic work-up, discuss the differences between AQP4 antibody positive and negative patients with NMOSD, including features of NMOSD with antibodies against myelin oligodendrocyte glycoprotein.

MRI characteristics of neuromyelitis optica spectrum disorder

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Paul, Friedemann; Lana-Peixoto, Marco A.; Tenembaum, Silvia; Asgari, Nasrin; Palace, Jacqueline; Klawiter, Eric C.; Sato, Douglas K.; de Seze, Jérôme; Wuerfel, Jens; Banwell, Brenda L.; Villoslada, Pablo; Saiz, Albert; Fujihara, Kazuo; Kim, Su-Hyun

2015-01-01

Since its initial reports in the 19th century, neuromyelitis optica (NMO) had been thought to involve only the optic nerves and spinal cord. However, the discovery of highly specific anti–aquaporin-4 antibody diagnostic biomarker for NMO enabled recognition of more diverse clinical spectrum of manifestations. Brain MRI abnormalities in patients seropositive for anti–aquaporin-4 antibody are common and some may be relatively unique by virtue of localization and configuration. Some seropositive patients present with brain involvement during their first attack and/or continue to relapse in the same location without optic nerve and spinal cord involvement. Thus, characteristics of brain abnormalities in such patients have become of increased interest. In this regard, MRI has an increasingly important role in the differential diagnosis of NMO and its spectrum disorder (NMOSD), particularly from multiple sclerosis. Differentiating these conditions is of prime importance because early initiation of effective immunosuppressive therapy is the key to preventing attack-related disability in NMOSD, whereas some disease-modifying drugs for multiple sclerosis may exacerbate the disease. Therefore, identifying the MRI features suggestive of NMOSD has diagnostic and prognostic implications. We herein review the brain, optic nerve, and spinal cord MRI findings of NMOSD. PMID:25695963

Immunopathogenesis in Myasthenia Gravis and Neuromyelitis Optica

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Wang, Zhen; Yan, Yaping

2017-01-01

Myasthenia gravis (MG) and neuromyelitis optica (NMO) are autoimmune channelopathies of the peripheral neuromuscular junction (NMJ) and central nervous system (CNS) that are mainly mediated by humoral immunity against the acetylcholine receptor (AChR) and aquaporin-4 (AQP4), respectively. The diseases share some common features, including genetic predispositions, environmental factors, the breakdown of tolerance, the collaboration of T cells and B cells, imbalances in T helper 1 (Th1)/Th2/Th17/regulatory T cells, aberrant cytokine and antibody secretion, and complement system activation. However, some aspects of the immune mechanisms are unique. Both targets (AChR and AQP4) are expressed in the periphery and CNS, but MG mainly affects the NMJ in the periphery outside of CNS, whereas NMO preferentially involves the CNS. Inflammatory cells, including B cells and macrophages, often infiltrate the thymus but not the target—muscle in MG, whereas the infiltration of inflammatory cells, mainly polymorphonuclear leukocytes and macrophages, in NMO, is always observed in the target organ—the spinal cord. A review of the common and discrepant characteristics of these two autoimmune channelopathies may expand our understanding of the pathogenic mechanism of both disorders and assist in the development of proper treatments in the future. PMID:29312313

Anti-C1q autoantibodies in patients with neuromyelitis optica spectrum disorders.

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Yoshikura, Nobuaki; Kimura, Akio; Hayashi, Yuichi; Inuzuka, Takashi

2017-09-15

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. Copyright © 2017 Elsevier B.V. All rights reserved.

Experimental Neuromyelitis Optica Induces a Type I Interferon Signature in the Spinal Cord

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Kaufmann, Nathalie; Zeka, Bleranda; Schanda, Kathrin; Fujihara, Kazuo; Illes, Zsolt; Dahle, Charlotte; Reindl, Markus; Lassmann, Hans; Bradl, Monika

2016-01-01

Neuromyelitis optica (NMO) is an acute inflammatory disease of the central nervous system (CNS) which predominantly affects spinal cord and optic nerves. Most patients harbor pathogenic autoantibodies, the so-called NMO-IgGs, which are directed against the water channel aquaporin 4 (AQP4) on astrocytes. When these antibodies gain access to the CNS, they mediate astrocyte destruction by complement-dependent and by antibody-dependent cellular cytotoxicity. In contrast to multiple sclerosis (MS) patients who benefit from therapies involving type I interferons (I-IFN), NMO patients typically do not profit from such treatments. How is I-IFN involved in NMO pathogenesis? To address this question, we made gene expression profiles of spinal cords from Lewis rat models of experimental neuromyelitis optica (ENMO) and experimental autoimmune encephalomyelitis (EAE). We found an upregulation of I-IFN signature genes in EAE spinal cords, and a further upregulation of these genes in ENMO. To learn whether the local I-IFN signature is harmful or beneficial, we induced ENMO by transfer of CNS antigen-specific T cells and NMO-IgG, and treated the animals with I-IFN at the very onset of clinical symptoms, when the blood-brain barrier was open. With this treatment regimen, we could amplify possible effects of the I-IFN induced genes on the transmigration of infiltrating cells through the blood brain barrier, and on lesion formation and expansion, but could avoid effects of I-IFN on the differentiation of pathogenic T and B cells in the lymph nodes. We observed that I-IFN treated ENMO rats had spinal cord lesions with fewer T cells, macrophages/activated microglia and activated neutrophils, and less astrocyte damage than their vehicle treated counterparts, suggesting beneficial effects of I-IFN. PMID:26990978

Serial quantitative MR assessment of optic neuritis in a case of neuromyelitis optica, using gadolinium-'enhanced' STIR imaging

International Nuclear Information System (INIS)

Barkhof, F.; Scheltens, P.; Valk, J.; Waalewijn, C.; Uitdehaag, B.M.J.; Polman, C.H.

1991-01-01

A patient is presented with neuromyelitis optica. MR imaging, using a short inversion time inversion recovery (STIR) technique, clearly depicted the lesion in the left optic nerve. Subsequent serial STIR imaging, with and without Gadolinium-DTPA, allowed quantitative assessment of changes parallel to improved optic nerve function. STIR imaging is a sensitive technique to demonstrate optic nerve lesions, and enables quantitative assessment to be made of the effect of (steroid) medication. (orig.)

Treatment of neuromyelitis optica: an evidence based review

Directory of Open Access Journals (Sweden)

Douglas Sato

2012-01-01

Full Text Available Neuromyelitis optica (NMO is an inflammatory disease of the central nervous system characterized by severe optic neuritis and transverse myelitis, usually with a relapsing course. Aquaporin-4 antibody is positive in a high percentage of NMO patients and it is directed against this water channel richly expressed on foot processes of astrocytes. Due to the severity of NMO attacks and the high risk for disability, treatment should be instituted as soon as the diagnosis is confirmed. There is increasing evidence that NMO patients respond differently from patients with multiple sclerosis (MS, and, therefore, treatments for MS may not be suitable for NMO. Acute NMO attacks usually are treated with high dose intravenous corticosteroid pulse and plasmapheresis. Maintenance therapy is also required to avoid further attacks and it is based on low-dose oral corticosteroids and non-specific immunosuppressant drugs, like azathioprine and mycophenolate mofetil. New therapy strategies using monoclonal antibodies like rituximab have been tested in NMO, with positive results in open label studies. However, there is no controlled randomized trial to confirm the safety and efficacy for the drugs currently used in NMO.

International consensus diagnostic criteria for neuromyelitis optica spectrum disorders

Science.gov (United States)

Banwell, Brenda; Bennett, Jeffrey L.; Cabre, Philippe; Carroll, William; Chitnis, Tanuja; de Seze, Jérôme; Fujihara, Kazuo; Greenberg, Benjamin; Jacob, Anu; Jarius, Sven; Lana-Peixoto, Marco; Levy, Michael; Simon, Jack H.; Tenembaum, Silvia; Traboulsee, Anthony L.; Waters, Patrick; Wellik, Kay E.

2015-01-01

Neuromyelitis optica (NMO) is an inflammatory CNS syndrome distinct from multiple sclerosis (MS) that is associated with serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Prior NMO diagnostic criteria required optic nerve and spinal cord involvement but more restricted or more extensive CNS involvement may occur. The International Panel for NMO Diagnosis (IPND) was convened to develop revised diagnostic criteria using systematic literature reviews and electronic surveys to facilitate consensus. The new nomenclature defines the unifying term NMO spectrum disorders (NMOSD), which is stratified further by serologic testing (NMOSD with or without AQP4-IgG). The core clinical characteristics required for patients with NMOSD with AQP4-IgG include clinical syndromes or MRI findings related to optic nerve, spinal cord, area postrema, other brainstem, diencephalic, or cerebral presentations. More stringent clinical criteria, with additional neuroimaging findings, are required for diagnosis of NMOSD without AQP4-IgG or when serologic testing is unavailable. The IPND also proposed validation strategies and achieved consensus on pediatric NMOSD diagnosis and the concepts of monophasic NMOSD and opticospinal MS. PMID:26092914

Differential diagnosis of neuromyelitis optica spectrum disorders

Science.gov (United States)

Kim, Sung-Min; Kim, Seong-Joon; Lee, Haeng Jin; Kuroda, Hiroshi; Palace, Jacqueline; Fujihara, Kazuo

2017-01-01

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system (CNS) mostly manifesting as optic neuritis and/or myelitis, which are frequently recurrent/bilateral or longitudinally extensive, respectively. As the autoantibody to aquaporin-4 (AQP4-Ab) can mediate the pathogenesis of NMOSD, testing for the AQP4-Ab in serum of patients can play a crucial role in diagnosing NMOSD. Nevertheless, the differential diagnosis of NMOSD in clinical practice is often challenging despite the phenotypical and serological characteristics of the disease because: (1) diverse diseases with autoimmune, vascular, infectious, or neoplastic etiologies can mimic these phenotypes of NMOSD; (2) patients with NMOSD may only have limited clinical manifestations, especially in their early disease stages; (3) test results for AQP4-Ab can be affected by several factors such as assay methods, serologic status, disease stages, or types of treatment; (4) some patients with NMOSD do not have AQP4-Ab; and (5) test results for the AQP4-Ab may not be readily available for the acute management of patients. Despite some similarity in their phenotypes, these NMOSD and NMOSD-mimics are distinct from each other in their pathogenesis, prognosis, and most importantly treatment. Understanding the detailed clinical, serological, radiological, and prognostic differences of these diseases will improve the proper management as well as diagnosis of patients. PMID:28670343

Magnetic Resonance Imaging Features of Neuromyelitis Optica

Energy Technology Data Exchange (ETDEWEB)

You, Sun Kyung; Song, Chang June; Park, Woon Ju; Lee, In Ho; Son, Eun Hee [Chungnam National University College of Medicine, Chungnam National University Hospital, Daejeon (Korea, Republic of)

2013-03-15

To report the magnetic resonance (MR) imaging features of the spinal cord and brain in patients of neuromyelitis optica (NMO). Between January 2001 and March 2010, the MR images (spinal cord, brain, and orbit) and the clinical and serologic findings of 11 NMO patients were retrospectively reviewed. The contrast-enhancement of the spinal cord was performed (20/23). The presence and pattern of the contrast-enhancement in the spinal cord were classified into 5 types. Acute myelitis was monophasic in 8 patients (8/11, 72.7%); and optic neuritis preceded acute myelitis in most patients. Longitudinally extensive cord lesion (average, 7.3 vertebral segments) was involved. The most common type was the diffuse and subtle enhancement of the spinal cord with a multifocal nodular, linear or segmental intense enhancement (45%). Most of the brain lesions (5/11, 10 lesions) were located in the brain stem, thalamus and callososeptal interphase. Anti-Ro autoantibody was positive in 2 patients, and they showed a high relapse rate of acute myelitis. Anti-NMO IgG was positive in 4 patients (4/7, 66.7%). The imaging findings of acute myelitis in NMO may helpful in making an early diagnosis of NMO which can result in a severe damage to the spinal cord, and to make a differential diagnosis of multiple sclerosis and inflammatory diseases of the spinal cord such as toxocariasis.

Magnetic Resonance Imaging Features of Neuromyelitis Optica

International Nuclear Information System (INIS)

You, Sun Kyung; Song, Chang June; Park, Woon Ju; Lee, In Ho; Son, Eun Hee

2013-01-01

To report the magnetic resonance (MR) imaging features of the spinal cord and brain in patients of neuromyelitis optica (NMO). Between January 2001 and March 2010, the MR images (spinal cord, brain, and orbit) and the clinical and serologic findings of 11 NMO patients were retrospectively reviewed. The contrast-enhancement of the spinal cord was performed (20/23). The presence and pattern of the contrast-enhancement in the spinal cord were classified into 5 types. Acute myelitis was monophasic in 8 patients (8/11, 72.7%); and optic neuritis preceded acute myelitis in most patients. Longitudinally extensive cord lesion (average, 7.3 vertebral segments) was involved. The most common type was the diffuse and subtle enhancement of the spinal cord with a multifocal nodular, linear or segmental intense enhancement (45%). Most of the brain lesions (5/11, 10 lesions) were located in the brain stem, thalamus and callososeptal interphase. Anti-Ro autoantibody was positive in 2 patients, and they showed a high relapse rate of acute myelitis. Anti-NMO IgG was positive in 4 patients (4/7, 66.7%). The imaging findings of acute myelitis in NMO may helpful in making an early diagnosis of NMO which can result in a severe damage to the spinal cord, and to make a differential diagnosis of multiple sclerosis and inflammatory diseases of the spinal cord such as toxocariasis.

Neuromyelitis optica immunoglobulin G in Chinese patients detected by immunofluorescence assay on a monkey brain substrate.

Science.gov (United States)

Long, Youming; Hu, Xueqiang; Peng, Fuhua; Lu, Zhengqi; Wang, Yuge; Yang, Yu; Qiu, Wei

2012-01-01

Serum neuromyelitis optica immunoglobulin G (NMO-IgG) is used as a biomarker to differentiate between neuromyelitis optica (NMO) and multiple sclerosis (MS). However, the original assay is expensive and complex and shows low sensitivity. Here, we investigated the potential of NMO-IgG detection using an indirect immunofluorescence (IIF) assay on monkey brains. NMO-IgG seroprevalence was determined in 168 samples by an IIF assay on a monkey brain substrate. The data were compared with those from a standard mouse brain IIF assay using McNemar and kappa tests. Thirty-one of 50 (62%) NMO patients, 7 of 18 (38.9%) longitudinally extensive transverse myelitis patients, 6 of 57 (10.5%) MS patients, and 5 of 10 (50%) optic neuritis patients were seropositive for NMO-IgG. None of the acute partial transverse myelitis patients (n = 3) or healthy controls (n = 20) was positive. Thus, the sensitivity of the test was 62% for the patients with clinically definite NMO. The specificity was 89.5%, considering the 57 MS patients as the control group. The modified IIF assay on monkey brains and the standard IIF assay based on mouse brains were not significantly different (McNemar test; p = 1.000). The two assays were concordant in 39 seropositive samples and 100 seronegative samples (kappa test; kappa = 0.592, p monkey brain assay was no better than the standard mouse brain IIF assay, we affirmed that NMO-IgG is a sensitive and specific biomarker to differentiate between NMO and MS. Copyright © 2011 S. Karger AG, Basel.

Quantitative MRI analysis of the brain after twenty-two years of neuromyelitis optica indicates focal tissue damage

DEFF Research Database (Denmark)

Aradi, Mihaly; Koszegi, Edit; Orsi, Gergely

2013-01-01

). In such abnormal NAWM regions, biexponential diffusion analysis and quantitative spectroscopy indicated extracellular edema and axonal loss, respectively. Repeated analysis 6 months later identified the same alterations. Such patchy alterations were not detectable in the NAWM of the 3 cases with short-term NMO......BACKGROUND: The long-term effect of neuromyelitis optica (NMO) on the brain is not well established. METHODS: After 22 years of NMO, a patient's brain was examined by quantitative T1- and T2-weighted mono- and biexponential diffusion and proton spectroscopy. It was compared to 3 cases with short...

Clinical analysis of neuromyelitis optica presenting as intractable nausea, vomiting and hiccups.

Science.gov (United States)

Jin, Xuehong; Pei, Shaofang; Liu, Yi; Li, Xia

2017-10-01

Vomiting and hiccups can be the manifestations of numerous systemic and neurological illnesses. Intractable nausea, vomiting and hiccups (INH) are reported as possible initial manifestations of neuromyelitis optica (NMO), but not correctly identified. Awareness of these atypical presentations is conducive to NMO early diagnosis and proper treatment to prevent further disability. In this paper, 12 NMO were reported, whose intractable vomiting and hiccups were the sole manifestations of the first attack and other attacks involving spinal cord and optic nerves developed later. All the patients were women and serum aquaporin 4 antibody (AQP4-Ab) of 83% patients was positive. MRI of 50% patients showed T2-weighted imaging/fluid attenuated inversion recovery hyperintensity which were longitudinally extensive transverse myelitis or linear signal changes. Sixty-seven percent of patients had medulla lesions, in which dorsomedial and area postrema were involved.

Unexpected exacerbations following initiation of disease-modifying drugs in neuromyelitis optica spectrum disorder: Which factor is responsible, anti-aquaporin 4 antibodies, B cells, Th1 cells, Th2 cells, Th17 cells, or others?

Science.gov (United States)

Kira, Jun-Ichi

2017-08-01

Some disease-modifying drugs for multiple sclerosis, which mainly act on T cells, are ineffective for neuromyelitis optica spectrum disorder and induce unexpected relapses. These include interferon beta, glatiramer acetate, fingolimod, natalizumab, and alemtuzumab. The cases reported here suggest that dimethyl fumarate, which reduces the number of Th1 and Th17 cells and induces IL-4-producing Th2 cells, is also unsuitable for neuromyelitis optica spectrum disorder, irrespective of anti-aquaporin 4 IgG serostatus. Although oral dimethyl fumarate with manageable adverse effects is easy to initiate in the early course of multiple sclerosis, special attention should be paid for atypical demyelinating cases.

Non-linear absorption for concentrated solar energy transport

Energy Technology Data Exchange (ETDEWEB)

Jaramillo, O. A; Del Rio, J.A; Huelsz, G [Centro de Investigacion de Energia, UNAM, Temixco, Morelos (Mexico)

2000-07-01

In order to determine the maximum solar energy that can be transported using SiO{sub 2} optical fibers, analysis of non-linear absorption is required. In this work, we model the interaction between solar radiation and the SiO{sub 2} optical fiber core to determine the dependence of the absorption of the radioactive intensity. Using Maxwell's equations we obtain the relation between the refractive index and the electric susceptibility up to second order in terms of the electric field intensity. This is not enough to obtain an explicit expression for the non-linear absorption. Thus, to obtain the non-linear optical response, we develop a microscopic model of an harmonic driven oscillators with damp ing, based on the Drude-Lorentz theory. We solve this model using experimental information for the SiO{sub 2} optical fiber, and we determine the frequency-dependence of the non-linear absorption and the non-linear extinction of SiO{sub 2} optical fibers. Our results estimate that the average value over the solar spectrum for the non-linear extinction coefficient for SiO{sub 2} is k{sub 2}=10{sup -}29m{sup 2}V{sup -}2. With this result we conclude that the non-linear part of the absorption coefficient of SiO{sub 2} optical fibers during the transport of concentrated solar energy achieved by a circular concentrator is negligible, and therefore the use of optical fibers for solar applications is an actual option. [Spanish] Con el objeto de determinar la maxima energia solar que puede transportarse usando fibras opticas de SiO{sub 2} se requiere el analisis de absorcion no linear. En este trabajo modelamos la interaccion entre la radiacion solar y el nucleo de la fibra optica de SiO{sub 2} para determinar la dependencia de la absorcion de la intensidad radioactiva. Mediante el uso de las ecuaciones de Maxwell obtenemos la relacion entre el indice de refraccion y la susceptibilidad electrica hasta el segundo orden en terminos de intensidad del campo electrico. Esto no es

Serial quantitative MR assessment of optic neuritis in a case of neuromyelitis optica, using gadolinium-'enhanced' STIR imaging

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Barkhof, F.; Scheltens, P.; Valk, J. (Vrije Univ., Amsterdam (Netherlands). Dept. of Diagnostic Radiology); Waalewijn, C.; Uitdehaag, B.M.J.; Polman, C.H. (Vrije Univ., Amsterdam (Netherlands). Dept. of Neurology)

1991-02-01

A patient is presented with neuromyelitis optica. MR imaging, using a short inversion time inversion recovery (STIR) technique, clearly depicted the lesion in the left optic nerve. Subsequent serial STIR imaging, with and without Gadolinium-DTPA, allowed quantitative assessment of changes parallel to improved optic nerve function. STIR imaging is a sensitive technique to demonstrate optic nerve lesions, and enables quantitative assessment to be made of the effect of (steroid) medication. (orig.).

The expanded spectrum of neuromyelitis optica: evidences for a new definition

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Marco A Lana-Peixoto

2012-10-01

Full Text Available Neuromyelitis optica (NMO has been traditionally described as the association of recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM. Identification of aquaporin-4 antibody (AQP4-IgG has deeply changed the concept of NMO. A spectrum of NMO disorders (NMOSD has been formulated comprising conditions which include both AQP4-IgG seropositivity and one of the index events of the disease (recurrent or bilateral optic neuritis and LETM. Most NMO patients harbor asymptomatic brain MRI lesions, some of them considered as typical of NMO. Some patients with aquaporin-4 autoimmunity present brainstem, hypothalamic or encephalopathy symptoms either preceding an index event or occurring isolatedly with no evidence of optic nerve or spinal involvement. On the opposite way, other patients have optic neuritis or LETM in association with typical lesions of NMO on brain MRI and yet are AQP4-IgG seronegative. An expanded spectrum of NMO disorders is proposed to include these cases.

Neuromyelitis optica and multiple sclerosis: Seeing differences through optical coherence tomography

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Bennett, JL; de Seze, J; Lana-Peixoto, M; Palace, J; Waldman, A; Schippling, S; Tenembaum, S; Banwell, B; Greenberg, B; Levy, M; Fujihara, K; Chan, KH; Kim, HJ; Asgari, N; Sato, DK; Saiz, A; Wuerfel, J; Zimmermann, H; Green, A; Villoslada, P

2015-01-01

Neuromyelitis optica (NMO) is an inflammatory autoimmune disease of the central nervous system that preferentially targets the optic nerves and spinal cord. The clinical presentation may suggest multiple sclerosis (MS), but a highly specific serum autoantibody against the astrocytic water channel aquaporin-4 present in up to 80% of NMO patients enables distinction from MS. Optic neuritis may occur in either condition resulting in neuro-anatomical retinal changes. Optical coherence tomography (OCT) has become a useful tool for analyzing retinal damage both in MS and NMO. Numerous studies showed that optic neuritis in NMO typically results in more severe retinal nerve fiber layer (RNFL) and ganglion cell layer thinning and more frequent development of microcystic macular edema than in MS. Furthermore, while patients’ RNFL thinning also occurs in the absence of optic neuritis in MS, subclinical damage seems to be rare in NMO. Thus, OCT might be useful in differentiating NMO from MS and serve as an outcome parameter in clinical studies. PMID:25662342

Detection and clinical value of NMO-IgG in neuromyelitis optica

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De-hui HUANG

2014-09-01

Full Text Available Neuromyelitis optica (NMO is an inflammatory demyelinating disease of the central nervous system (CNS of autoimmune etiology which predominantly affects the optic nerves and spinal cord. In 2004, a highly specific serum antibody, NMO-IgG, was found in the sera of NMO patients. Subsequently, the target antigen of NMO-IgG was identified as aquaporin 4 (AQP4, a water channel densely expressed in optic nerves, spinal cord and area around cerebral ventricles. NMO-IgG/AQP4 antibody has demonstrated extreme importance for the diagnosis and differential diagnosis, the evaluation of disease activity, therapeutic effect and prognosis of NMO. In recent years, different techniques have been used to examine NMO-IgG in serum and cerebrospinal fluid, including tissue-based, cell-based and protein-based assays. In this review, the authors give an overview of the tests currently available for the detection of NMO-IgG and their clinical significance. doi: 10.3969/j.issn.1672-6731.2014.09.003

Neuromyelitis optica accompanied by nephrotic syndrome and autoimmune-related pancytopenia.

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ZhangBao, Jingzi; Zhou, Lei; Lu, Jiahong; Xi, Jianying; Zhao, Chongbo; Quan, Chao

2016-05-01

Neuromyelitis optica (NMO) associated with nephrotic syndrome and autoimmune-related pancytopenia has not been reported previously. We report herein a young woman who initially presented with bilateral blurring of vision and numbness in her hands. MRI disclosed multiple white matter lesions and a long cervical spinal cord lesion extending to the medulla oblongata. Serum aquaporin-4 antibody was positive and the patient was diagnosed with NMO. While in the hospital, she presented with hypoproteinemia and heavy proteinuria, meeting the diagnostic criteria of nephrotic syndrome. After high-dose methylprednisolone treatment, her vision improved significantly and urine protein quantity decreased. However, the patient subsequently developed severe pancytopenia with a positive Coombs' test. Thrombocytopenia finally led to uncontrollable gastrointestinal bleeding as the direct cause of the patient's death. This case illustrates the extremely rare condition of concurrence of NMO, nephrotic syndrome, and autoimmune pancytopenia in one patient, which suggests the involvement of organs beyond the central nervous system in NMO spectrum disorders. Copyright © 2016 Elsevier B.V. All rights reserved.

Posterior reversible encephalopathy syndrome masquerading as progressive multifocal leukoencephalopathy in rituximab treated neuromyelitis optica.

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Berger, Joseph R; Neltner, Janna; Smith, Charles; Cambi, Franca

2014-11-01

Both progressive multifocal leukoencephalopathy (PML) and posterior reversible encephalopathy syndrome (PRES) have been reported as complications of rituximab therapy. These disorders may appear indistinguishable on magnetic resonance imaging (MRI). We report on a 42 year old woman with neuromyelitis optica (NMO) of 10 years duration who developed extensive white matter disease affecting chiefly both parietal lobes 6 months after her first and only dose of rituximab. The MRI findings suggested the diagnosis of PML, but her history was more consistent with PRES. Ultimately, a brain biopsy was performed which was consistent with the diagnosis of PRES. PRES and PML may have overlapping symptomatology and be indistinguishable on MRI. An approach to distinguishing between these two disorders is addressed. Copyright © 2014. Published by Elsevier B.V.

Endocrinopathies in paediatric-onset neuromyelitis optica spectrum disorder with aquaporin 4 (AQP4) antibody.

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Hacohen, Yael; Messina, Silvia; Gan, Hoong-Wei; Wright, Sukhvir; Chandratre, Saleel; Leite, Maria Isabel; Fallon, Penny; Vincent, Angela; Ciccarelli, Olga; Wassmer, Evangeline; Lim, Ming; Palace, Jacqueline; Hemingway, Cheryl

2018-04-01

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. Morbid obesity was seen in 88% (7/8) of children of Caribbean origin. As endocrinopathies were prevalent in the majority of paediatric-onset AQP4-Ab NMOSD, endocrine surveillance and in particular early aggressive weight management is required for patients with AQP4-Ab NMOSD.

NMO-IgG: A Specific Biomarker for Neuromyelitis Optica

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Brian G. Weinshenker

2006-01-01

Full Text Available Neuromyelitis optica (NMO is an inflammatory demyelinating disease that principally targets the optic nerves and spinal cord and often leads to severe disability and occasionally life threatening respiratory failure. Although its clinical manifestations overlap with those of multiple sclerosis (MS, in established cases these two conditions can be distinguished on the basis of clinical, radiological, and routine spinal fluid studies. The diagnosis in early cases or limited forms of NMO is difficult. We recently discovered a unique IgG autoantibody (NMO-IgG that is highly specific to patients with NMO and thus a valuable diagnostic aid. Its antigen, aquaporin-4 (AQP4, is the central nervous system’s predominant water channel protein. This antibody has not yet been proven to be pathogenic, but several facts suggest that it might be, including the similarity of the immunohistochemical pattern of NMO-(AQP4 IgG binding to mouse CNS tissues to the pattern of immune complex deposition in autopsied patients’ spinal cord tissue. The spectrum of diseases identified by NMO-IgG is broader than has previously been recognized clinically and includes incomplete forms of NMO, such as recurrent transverse myelitis without optic neuritis and recurrent optic neuritis without myelitis.

Ovarian Reserve in Women With Neuromyelitis Optica Spectrum Disorder

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Jan Thöne

2018-06-01

Full Text Available Neuromyelitis optica spectrum disorder (NMOSD is a neuroinflammatory disease. The majority of NMOSD patients is seropositive for aquaporin-4 (AQP4 antibodies. AQP4 is the main water channel protein in the central nervous system, but has also been identified in the female reproductive system. Fertility issues and ovarian reserve has not yet been studied in females with NMOSD. The purpose of this study was to measure serum Anti-Müllerian hormone (AMH in females with NMOSD compared to healthy controls (HC, in combination with other lifestyle and reproduction parameters. AMH is independent from the menstrual cycle and a reliable indicator of both ovarian reserve and ovarian function. We included a total of 32 reproductive-age females, 18 HC and 14 with NMOSD. We used an enzymatically amplified two-site immunoassay to determine serum AMH level. In comparison to HC, mean AMH value was reduced in NMOSD. Apart from that significantly more women with NMOSD showed low AMH levels (< 0.8 ng/ml. Low AMH was associated with disease activity. In contrast, none of the immunotherapies for NMOSD, neither any reproductive life style parameter was associated with a decreased AMH. Our results contribute to understanding of hindered fertility in females with NMOSD and enables neurologists to better counsel female patients.

Neuromyelitis optica in pregnancy complicated by posterior reversible encephalopathy syndrome, eclampsia and fetal death.

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Igel, Catherine; Garretto, Diana; Robbins, Matthew S; Swerdlow, Michael; Judge, Nancy; Dayal, Ashlesha

2015-03-01

Neuromyelitis optica (NMO) is a demyelinating syndrome characterized by optic neuritis and acute myelitis with poor recovery and a progressive course. We report a poor outcome complicated by posterior reversible encephalopathy syndrome (PRES) and eclampsia and review available literature and current evidence for anticipation of adverse fetal and maternal effects. After a pregnancy complicated by multiple admissions for painful NMO exacerbations, a primiparous patient with seropositive NMO presented at 31 + 3/7 weeks with eclampsia, HELLP and subsequent fetal death. MRI confirmed PRES. NMO may be associated with eclampsia and leads to adverse maternal and fetal outcomes. Posited mechanisms include antibody-mediated placental damage and a heightened risk of eclampsia-associated PRES. Further characterization of the course of NMO and its relationship with pregnancy outcomes in larger series would be invaluable.

Default-mode network and deep gray-matter analysis in neuromyelitis optica patients.

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Rueda-Lopes, Fernanda C; Pessôa, Fernanda M C; Tukamoto, Gustavo; Malfetano, Fabíola Rachid; Scherpenhuijzen, Simone Batista; Alves-Leon, Soniza; Gasparetto, Emerson L

2018-02-20

The aim of our study was to detect functional changes in default-mode network of neuromyelitis optica (NMO) patients using resting-state functional magnetic resonance images and the evaluation of subcortical gray-matter structures volumes. NMO patients (n=28) and controls patients (n=19) were enrolled. We used the integrated registration and segmentation tool, part of FMRIB's Software Library (FSL) to segment subcortical structures including the thalamus, caudate nucleus, putamen, hippocampus and amygdalae. Resting-state functional magnetic resonance images were post-processed using the Multivariate Exploratory Linear Optimized Decomposition into Independent Components, also part of FSL. Average Z-values extracted from the default-mode network were compared between patients and controls using t-tests (P values default-mode network of patients compared to controls, notably in the precuneus and right hippocampus (corrected Pdefault-mode network. The hyperactivity of certain default-mode network areas may reflect cortical compensation for subtle structural damage in NMO patients. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

Neuromyelitis optica spectrum disorder in patient with systemic lupus erythematosus - our experience

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Božić Ksenija

2018-01-01

Full Text Available Introduction. Neuromyelitis optica spectrum disorder (NMOSD is a rare demyelinating immune-mediated central nervous system disease. It is extremely rare to occur in patients with systemic lupus erythematosus (SLE, and it represents a diagnostic and therapeutic challenge. Case report. A 38-year-old Caucasian woman with medical history of SLE and new onset of flaccid paraparesis, fecal and urinary incontinence, persistent nausea and vomiting was admitted to our hospital. Based on the clinical presentation, magnetic resonance imaging findings and positive aquaporin 4 (AQP4 antibodies, a NMOSD with coexisting SLE were diagnosed. Pulse-doses of cyclophosphamide and glucocorticoids were efficient in patient treatment. Conclusion. In a patient with SLE and symptoms of longitudinal extensive transverse myelitis and/or optic neuritis and area postrema syndrome, assessment of AQP4 antibodies is neccessary for diagnosing NMOSD. Accurate diagnosis, and timely and long-term administration of immunosuppressive therapy are crucial for favorable outcome of these two coexisting diseases.

The Contribution of Optical Coherence Tomography in Neuromyelitis Optica Spectrum Disorders

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Mateo, Javier; Esteban, Olivia; Martínez, Mireya; Grzybowski, Andrzej; Ascaso, Francisco Javier

2017-01-01

Neuromyelitis optica spectrum disorders (NMOSD) comprises a group of central nervous system disorders of inflammatory autoimmune origin that mainly affect the optic nerves and the spinal cord and can cause severe visual and general disability. The clinical signs are similar to those of multiple sclerosis (MS), with the result that it is often difficult to differentiate between the two, thus leading to misdiagnosis. As the treatment and prognosis of NMOSD and MS are different, it is important to make an accurate and early diagnosis of NMOSD. Optical coherence tomography (OCT) is a non-invasive technique that enables a quantitative study of the changes that the optic nerve and the macula undergo in several neurodegenerative diseases. Many studies have shown that some of these changes, such as retinal nerve fiber layer thinning or microcystic macular edema, can be related to alterations in the brain due to neurodegenerative disorders. The purpose of this mini-review is to show how OCT can be useful for the diagnosis of NMOSD and follow-up of affected patients, as well as for the differential diagnosis with MS. PMID:29085325

Neuromyelitis optica-IgG testing in an Indian cohort with neuromyelitis optica and related demyelinating disorders: Our experience

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Narayanan Unni

2013-01-01

Full Text Available Background: Neuromyelitis optica (NMO is an immune-mediated inflammatory demyelinating disorder of the central nervous system with a predilection for the optic nerves and the spinal cord. Immunopathological evidence suggests that the target antigen of the disease is aquaporin-4. An IgG antibody against this protein has been explored as a molecular marker for the disease and as a diagnostic tool due to its high sensitivity and specificity in various populations. Objective: To assess the value of NMO-IgG testing in Indian patients with clinical and magnetic resonance imaging features consistent with NMO and longitudinally extensive transverse myelitis (LETM. Materials and Methods: Forty-five patients with clinical and magnetic resonance imaging features consistent with NMO, LETM, and MS were tested for serum NMO-IgG. Of these patients, 22 patients satisfied revised (2006 Wingerchuk criteria for NMO (excluding NMO-IgG status and 11 patients had LETM. Twelve patients satisfied the revised (2010 McDonald criteria for multiple sclerosis (MS. Results: Of the 21 patients, satisfying the criteria for NMO and for whom the test results were available, 17 were positive for NMO-IgG (80.9%, and of the 11 patients having LETM, 6 (54.5% were positive for NMO-IgG. In one patient with NMO, the test result was not available. None of the 12 patients satisfying McDonald criteria for MS showed NMO-IgG seropositivity. Conclusion: Our study suggests that it is worthwhile to pursue NMO-IgG testing as a diagnostic tool for patients with clinical and Magnetic Resonance Imaging (MRI features consistent with NMO and LETM in the Indian population.

Neuromyelitis optica: a positive appraisal of seronegative cases.

Science.gov (United States)

Bernard-Valnet, R; Liblau, R S; Vukusic, S; Marignier, R

2015-12-01

Neuromyelitis optica (NMO) is a rare inflammatory disorder of the central nervous system. The hallmark of NMO is the presence of specific autoantibodies directed against aquaporin 4 (AQP4-IgG). AQP4-IgG, included in diagnostic criteria, has enlarged the clinical spectrum of NMO and serves to predict relapses. Moreover AQP4-IgG has provided unprecedented insight in the immunopathology of NMO, representing a rationale for therapeutic intervention with relevant novel treatment strategies specific for NMO. However, some patients remain seronegative for AQP4-IgG despite a definite diagnosis of NMO and the use of the finest methods for antibody detection. Interestingly, seronegative NMO (NMO(neg)) patients exhibit different demographic and disease-related characteristics in comparison to seropositive patients. The recent association with autoantibodies specific for myelin oligodendrocyte glycoprotein (MOG) is the main indication that disease mechanisms might differ in NMO(pos) and NMO(neg), challenging the position of NMO(neg) patients in the spectrum of demyelinating diseases and therapeutic strategies to be adopted. Thus, a reappraisal of the NMO(neg) population is needed to improve NMO care. Here the current knowledge regarding NMO(neg) is reviewed and hypotheses on its pathogenesis are made including a comprehensive description of detection methods and the prevalence of AQP4-IgG and a review of the epidemiological, clinical and paraclinical characteristics of NMO(neg); finally an integrated view of the general pathophysiological mechanisms underlying NMO(neg) is provided. © 2015 EAN.

Is pruritus an indicator of aquaporin-positive neuromyelitis optica?

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Netravathi, Manjunath; Saini, Jitender; Mahadevan, Anita; Hari-Krishna, Bollampalli; Yadav, Ravi; Pal, Pramod Kumar; Satishchandra, Parthasarathy

2017-05-01

Recently, pruritus has been recognised as an important association with neuromyelitis optica spectrum disorders (NMOSD). To determine the clinical and radiological characteristics of patients with NMOSD and pruritus. Among 57 consecutive patients with NMOSD, 15 (26.3% women) reported pruritus. All had aquaporin-4 (AQP4) antibodies. The mean age was 34.5 ± 9.1 years, age at onset was 31.3 ± 11.0 years and the duration of illness was 3.9 ± 3.1 years. Pruritus preceded the neurological disturbances in all the patients. Predominant patients experienced pruritus in the cervical dermatome (66.7%) followed by cervicothoracic region (13.3%), trigeminal nerve (13.3%) and lumbar region (6.7%). Lesions extending from cervicomedullary junction up to the thoracic segment was the most common site of affection (40%) followed by cervicothoracic (26.7%), cervicomedullary junction to cervical cord (13.3%), cervical cord (6.7%) and thoracic segment (6.7%). This report is one of the largest series reporting the close association of pruritus with onset of neurological symptoms in NMOSD. It highlights the importance of recognising this rare symptom which may help in making a correct diagnosis in a patient with suspected demyelinating disorder. In a patient with NMOSD, early treatment with immunomodulation during pruritus may prevent or minimise occurrence of neurological dysfunction.

Qualitative and quantitative autoradiographic investigations on DNA-repair in the pars optica retinae of the rabbit

International Nuclear Information System (INIS)

Kellner, G.; Reindl, E.; Pichler, L.; Hofer, H.

1974-01-01

In vitro and in vivo investigations into the incorporation of 3 H-thymidine into the nuclei of pars optica retinae of rabbits after β-irradiation with 60 krad were performed. The results of the in vitro and in vivo experiments are comparable with the in vitro data showing smaller statistical deviations. The rate comparable with the in vitro data showing smaller statistical deviations. The rate of incorporation of 3 H-thymidine into the nuclei of Ggl. opticum and Ggl. retinae is about the same, but it is significantly lower in the nuclei of photoreceptor cells by one order of magnitude. The in vitro experiment demonstrates that ganglion cells are capable of DNA repair even after circulation has been stopped for 15 or more minutes. (author)

Contribution of spinal cord biopsy to diagnosis of aquaporin-4 antibody positive neuromyelitis optica spectrum disorder.

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Ringelstein, M; Metz, I; Ruprecht, K; Koch, A; Rappold, J; Ingwersen, J; Mathys, C; Jarius, S; Brück, W; Hartung, H-P; Paul, F; Aktas, O

2014-06-01

Longitudinally extensive transverse myelitis is characteristic but not pathognomonic for neuromyelitis optica spectrum disorders (NMOSDs) and may mimic local tumors. In this retrospective study based on a cohort of 175 NMOSD patients we identified seven patients who initially presented with a longitudinally extensive spinal cord lesion and underwent spinal cord biopsy due to magnetic resonance imaging (MRI)-suspected malignancies. Remarkably, routine neuropathology was inconclusive and did not guide the diagnostic process to anti-aquaporin-4 (AQP4)-seropositive NMOSD. Serious postoperative complications occurred in 5/7 patients and persisted during follow-up in 2/7 patients (29%). Considering these sequelae, AQP4-antibody testing should be mandatory in patients with inconclusive longitudinally extensive spinal cord lesions prior to biopsy. © The Author(s) 2013.

Presentation of Neuromyelitis Optica with Recurrent Severe Myelitis and Acute Respiratory Failure in an Old Woman

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Saeed Razmeh

2017-06-01

Full Text Available Neuromyelitis Optica (NMO is a rare disease of the central nervous system that causes optic nerve and spinal cord involvement. The our patient first developed acute paraplegia that was treated with intravenous methylprednisolone with diagnosis of acute thoracic myelitis according to magnetic resonance imaging (MRI finding , concurrently with tapering of oral prednisolone, again affected by quadriplegia and respiratory failure. She was seropositivity for NMO-IgG that was negative in first admission and MRI of spine shows hyperintense lesion in whole cervical and upper thoracic MRI. With considering the findings, NMO was diagnosed and the plasmapheresis starts for her. We report a case of this syndrome because it can increase the physician’s awareness of the unusual manifestations of this syndrome.

Challenges and opportunities in designing clinical trials for neuromyelitis optica

Science.gov (United States)

Barron, Gerard; Behne, Jacinta M.; Bennett, Jeffery L.; Chin, Peter S.; Cree, Bruce A.C.; de Seze, Jerome; Flor, Armando; Fujihara, Kazuo; Greenberg, Benjamin; Higashi, Sayumi; Holt, William; Khan, Omar; Knappertz, Volker; Levy, Michael; Melia, Angela T.; Palace, Jacqueline; Smith, Terry J.; Sormani, Maria Pia; Van Herle, Katja; VanMeter, Susan; Villoslada, Pablo; Walton, Marc K.; Wasiewski, Warren; Wingerchuk, Dean M.; Yeaman, Michael R.

2015-01-01

Current management of neuromyelitis optica (NMO) is noncurative and only partially effective. Immunosuppressive or immunomodulatory agents are the mainstays of maintenance treatment. Safer, better-tolerated, and proven effective treatments are needed. The perceived rarity of NMO has impeded clinical trials for this disease. However, a diagnostic biomarker and recognition of a wider spectrum of NMO presentations has expanded the patient population from which study candidates might be recruited. Emerging insights into the pathogenesis of NMO have provided rationale for exploring new therapeutic targets. Academic, pharmaceutical, and regulatory communities are increasingly interested in meeting the unmet needs of patients with NMO. Clinical trials powered to yield unambiguous outcomes and designed to facilitate rapid evaluation of an expanding pipeline of experimental agents are needed. NMO-related disability occurs incrementally as a result of attacks; thus, limiting attack frequency and severity are critical treatment goals. Yet, the severity of NMO and perception that currently available agents are effective pose challenges to study design. We propose strategies for NMO clinical trials to evaluate agents targeting recovery from acute attacks and prevention of relapses, the 2 primary goals of NMO treatment. Aligning the interests of all stakeholders is an essential step to this end. PMID:25841026

Pruritus may be a common symptom related to neuromyelitis optica spectrum disorders.

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He, Zhiyong; Ren, Ming; Wang, Xiaofeng; Guo, Qifeng; Qi, Xiaokun

2017-04-01

To evaluate pruritus in patients with neuromyelitis optica spectrum disorders (NMOSD) and to characterize the relationship between pruritus and lesions of NMOSD. 61 patients with NMOSD were included in the study and their medical records were reviewed for pruritus, neurological symptoms and magnetic resonance imaging (MRI) images. We focused on the patients' history of pruritus, especially the severity, duration, region, and the relationship of pruritus with other symptoms of NMOSD. Of the 61 patients with NMOSD, 59 had longitudinally extensive transverse myelitis (LETM). 38 of these patients (64.4%) reported pruritus during the course of their illness, with 16 patients reporting pruritus as the initial symptoms followed by limb weakness. In 35 of 38 patients (92.1%), pruritus was located within the dermatomes innervated by the spinal nerves from the involved spinal cord. Our results show that pruritus is a common symptom of NMOSD and relates to the lesions in the spinal cord. Pruritus may indicate a new episode of myelitis in patients with NMOSD. Copyright © 2017 Elsevier B.V. All rights reserved.

Use of Advanced Magnetic Resonance Imaging Techniques in Neuromyelitis Optica Spectrum Disorder

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Kremer, Stephane; Renard, Felix; Achard, Sophie; Lana-Peixoto, Marco A.; Palace, Jacqueline; Asgari, Nasrin; Klawiter, Eric C.; Tenembaum, Silvia N.; Banwell, Brenda; Greenberg, Benjamin M.; Bennett, Jeffrey L.; Levy, Michael; Villoslada, Pablo; Saiz, Albert; Fujihara, Kazuo; Chan, Koon Ho; Schippling, Sven; Paul, Friedemann; Kim, Ho Jin; de Seze, Jerome; Wuerfel, Jens T.

2016-01-01

Brain parenchymal lesions are frequently observed on conventional magnetic resonance imaging (MRI) scans of patients with neuromyelitis optica (NMO) spectrum disorder, but the specific morphological and temporal patterns distinguishing them unequivocally from lesions caused by other disorders have not been identified. This literature review summarizes the literature on advanced quantitative imaging measures reported for patients with NMO spectrum disorder, including proton MR spectroscopy, diffusion tensor imaging, magnetization transfer imaging, quantitative MR volumetry, and ultrahigh-field strength MRI. It was undertaken to consider the advanced MRI techniques used for patients with NMO by different specialists in the field. Although quantitative measures such as proton MR spectroscopy or magnetization transfer imaging have not reproducibly revealed diffuse brain injury, preliminary data from diffusion-weighted imaging and brain tissue volumetry indicate greater white matter than gray matter degradation. These findings could be confirmed by ultrahigh-field MRI. The use of nonconventional MRI techniques may further our understanding of the pathogenic processes in NMO spectrum disorders and may help us identify the distinct radiographic features corresponding to specific phenotypic manifestations of this disease. PMID:26010909

Cognitive impairment differs between neuromyelitis optica spectrum disorder and multiple sclerosis.

Science.gov (United States)

Kim, Su-Hyun; Kwak, Kichang; Jeong, In Hye; Hyun, Jae-Won; Jo, Hyo-Jin; Joung, AeRan; Yu, Eun-Seung; Kim, Ji-Hee; Lee, Sang Hyun; Yun, Sooin; Joo, Jungnam; Lee, Dong-Kyun; Lee, Jong-Min; Kim, Ho Jin

2016-12-01

To compare the frequency and pattern of cognitive impairment (CI) between patients with neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS). A total of 82 NMOSD patients, 58 MS patients, and 45 healthy controls (HCs) underwent a neuropsychological assessment. CI was observed in 29% of NMOSD and 50% of MS patients (p < 0.001); CI was considered present if a patient scored lower than the fifth percentile compared with HCs in at least three domains. A lower frequency of CI was consistently found when CI was indicated by at least two failed tests (p < 0.001). MS patients performed worse than did NMOSD patients on verbal learning and verbal and visual memory tests. Levels of education and depression and the interval from disease onset to treatment were associated with a negative influence on cognition in patients with NMOSD. CI in patients with NMOSD may be not as common as in patients with MS. MS patients exhibited severe impairment, particularly on learning and memory tests, compared with NMOSD patients. Differential prevalence and patterns of CI between NMOSD and MS patients suggest that the two diseases have different mechanisms of brain injury. © The Author(s), 2016.

The Contribution of Optical Coherence Tomography in Neuromyelitis Optica Spectrum Disorders

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Javier Mateo

2017-09-01

Full Text Available Neuromyelitis optica spectrum disorders (NMOSD comprises a group of central nervous system disorders of inflammatory autoimmune origin that mainly affect the optic nerves and the spinal cord and can cause severe visual and general disability. The clinical signs are similar to those of multiple sclerosis (MS, with the result that it is often difficult to differentiate between the two, thus leading to misdiagnosis. As the treatment and prognosis of NMOSD and MS are different, it is important to make an accurate and early diagnosis of NMOSD. Optical coherence tomography (OCT is a non-invasive technique that enables a quantitative study of the changes that the optic nerve and the macula undergo in several neurodegenerative diseases. Many studies have shown that some of these changes, such as retinal nerve fiber layer thinning or microcystic macular edema, can be related to alterations in the brain due to neurodegenerative disorders. The purpose of this mini-review is to show how OCT can be useful for the diagnosis of NMOSD and follow-up of affected patients, as well as for the differential diagnosis with MS.

Autoimmune AQP4 channelopathies and neuromyelitis optica spectrum disorders.

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Hinson, Shannon R; Lennon, Vanda A; Pittock, Sean J

2016-01-01

Neuromyelitis optica (NMO) spectrum disorders (SD) represent an evolving group of central nervous system (CNS)-inflammatory autoimmune demyelinating diseases unified by a pathogenic autoantibody specific for the aquaporin-4 (AQP4) water channel. It was historically misdiagnosed as multiple sclerosis (MS), which lacks a distinguishing biomarker. The discovery of AQP4-IgG moved the focus of CNS demyelinating disease research from emphasis on the oligodendrocyte and myelin to the astrocyte. NMO is recognized today as a relapsing disease, extending beyond the optic nerves and spinal cord to include brain (especially in children) and skeletal muscle. Brain magnetic resonance imaging abnormalities, identifiable in 60% of patients at the second attack, are consistent with MS in 10% of cases. NMOSD-typical lesions (another 10%) occur in AQP4-enriched regions: circumventricular organs (causing intractable nausea and vomiting) and the diencephalon (causing sleep disorders, endocrinopathies, and syndrome of inappropriate antidiuresis). Advances in understanding the immunobiology of AQP4 autoimmunity have necessitated continuing revision of NMOSD clinical diagnostic criteria. Assays that selectively detect pathogenic AQP4-IgG targeting extracellular epitopes of AQP4 are promising prognostically. When referring to AQP4 autoimmunity, we suggest substituting the term "autoimmune aquaporin-4 channelopathy" for the term "NMO spectrum disorders." Randomized clinical trials are currently assessing the efficacy and safety of newer immunotherapies. Increasing therapeutic options based on understanding the molecular pathogenesis is anticipated to improve the outcome for patients with AQP4 channelopathy. © 2016 Elsevier B.V. All rights reserved.

Seronegative Neuromyelitis Optica: A Case Report of a Hispanic Male

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Nabeel Badri

2016-05-01

Full Text Available Neuromyelitis optica (NMO is a rare disease, common in white females and rarely reported in Hispanic males. It is usually associated with recurrent demyelinating spectrum that is autoimmune in nature. The diagnosis is usually confirmed by antibody biomarkers; however, they can be negative and lead to more dilemma in diagnosis. Furthermore, the course of disease and prognosis are different in seronegative as compared to seropositive NMO. Treatment is similar in both subgroups with new approaches under investigation for seronegative NMO patients. We present an interesting case of a 37-year-old Hispanic male who presented with sudden onset of lower extremity weakness, numbness, blurry vision, and urinary retention. Magnetic resonance imaging (MRI of the thoracic spine showed multiphasic demyelinating process involving the thoracic spinal cord. His brain MRI also revealed changes suggesting optic neuritis. The patient met the criteria for diagnosis of NMO by having optic neuritis and myelitis by imaging studies despite having negative aquaporin-4 antibodies (AQP4-Ab. His condition improved after plasma exchange. NMO can be difficult to distinguish from acute multiple sclerosis in the early stages of the disease. Having AQP4-Ab testing is important for diagnosis with imaging studies; however, negative antibody results cannot exclude the diagnosis, but rather group it in seronegative subtype. Ongoing studies and research suggest that seronegative NMO might have a different pathophysiology, manifestation, and prognosis.

Complexity and wide range of neuromyelitis optica spectrum disorders: more than typical manifestations

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Han, Jinming; Yang, Meng-ge; Zhu, Jie; Jin, Tao

2017-01-01

Neuromyelitis optica (NMO), considered to be mediated by autoantibodies, often cause severely disabling disorders of the central nervous system, and predominantly cause optic nerve damage and longitudinally extensive transverse myelitis. Remarkable progress has been made in deciphering NMO pathogenesis during the past decade. In 2015, the International Panel for NMO Diagnosis proposed the unifying term “NMO spectrum disorders” (NMOSD) and the updated NMOSD criteria reflects a wide range of disease and maintains reasonable specificity. Moreover, cumulative findings have indicated that NMOSD are frequently associated with multiple autoimmune diseases, thereby presenting complex clinical symptoms that make this disease more difficult to recognize. Notably, most neurologists do not heed these symptoms or comorbid conditions in patients with NMOSD. Whereas previous reviews have focused on pathogenesis, treatment, and prognosis in NMOSD, we summarize the present knowledge with particular emphasis on atypical manifestations and autoimmune comorbidities in patients with NMOSD. Furthermore, we emphasized the identification of these atypical characteristics to enable a broader and better understanding of NMOSD, and improve early accurate diagnosis and therapeutic decision making. PMID:29118581

Plasma complement biomarkers distinguish multiple sclerosis and neuromyelitis optica spectrum disorder.

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Hakobyan, Svetlana; Luppe, Sebastian; Evans, David Rs; Harding, Katharine; Loveless, Samantha; Robertson, Neil P; Morgan, B Paul

2017-06-01

Multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) are autoimmune inflammatory demyelinating diseases of the central nervous system. Although distinguished by clinicoradiological and demographic features, early manifestations can be similar complicating management. Antibodies against aquaporin-4 support the diagnosis of NMOSD but are negative in some patients. Therefore, there is unmet need for biomarkers that enable early diagnosis and disease-specific intervention. We investigated whether plasma complement proteins are altered in MS and NMOSD and provide biomarkers that distinguish these diseases. Plasma from 54 NMOSD, 40 MS and 69 control donors was tested in multiplex assays measuring complement activation products and proteins. Using logistic regression, we tested whether combinations of complement analytes distinguished NMOSD from controls and MS. All activation products were elevated in NMOSD compared to either control or MS. Four complement proteins (C1inh, C1s, C5 and FH) were higher in NMOSD compared to MS or controls. A model comprising C1inh and terminal complement complex (TCC) distinguished NMOSD from MS (area under the curve (AUC): 0.98), while C1inh and C5 distinguished NMOSD from controls (AUC: 0.94). NMOSD is distinguished from MS by plasma complement biomarkers. Selected complement analytes enable differential diagnosis. Findings support trials of anti-complement therapies in NMOSD.

Short segment myelitis as a first manifestation of neuromyelitis optica spectrum disorders.

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Huh, So-Young; Kim, Su-Hyun; Hyun, Jae-Won; Jeong, In Hye; Park, Min Su; Lee, Sang-Hyun; Kim, Ho Jin

2017-03-01

Some patients with neuromyelitis optica spectrum disorders (NMOSD) present with spinal cord lesions extending fewer than three vertebral segments (short transverse myelitis, STM), hindering an early diagnosis. We investigated the frequency and imaging characteristics of STM lesions in patients presenting with myelitis as an initial manifestation of NMOSD. Patients seen at three referral hospitals in Korea between June 2005 and March 2015 who met the following inclusion criteria were recruited for review: seropositivity for aquaporin-4 antibody, initial presentation with myelitis and spinal cord magnetic resonance imaging (MRI) performed within 1 month of initial myelitis onset. Of the 76 enrolled patients, 65 (85.5%) collectively had 69 longitudinally extensive transverse myelitis lesions, while the remaining 11 (14.5%) had a total of 15 STM lesions. Of the 15 STM lesions, 5 spanned 2.5 vertebral segments, 6 were continuous over two segments, 3 showed a length of 1.5 segments and 1 was confined to a single segment. On axial imaging, all of the STM lesions involved the central grey matter. These MRI findings suggested that STM does not preclude the possibility of an NMOSD diagnosis.

Systematic review of the published data on the worldwide prevalence of John Cunningham virus in patients with multiple sclerosis and neuromyelitis optica.

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Paz, Sonia Patricia Castedo; Branco, Luciana; Pereira, Marina Alves de Camargo; Spessotto, Caroline; Fragoso, Yara Dadalti

2018-01-01

John Cunningham virus (JCV) is a polyoma virus that infects humans, mainly in childhood or adolescence, and presents no symptomatic manifestations. JCV can cause progressive multifocal leukoencephalopathy (PML) in immunosuppressed individuals, including those undergoing treatment for multiple sclerosis (MS) and neuromyelitis optica (NMO). PML is a severe and potentially fatal disease of the brain. The prevalence of JCV antibodies in human serum has been reported to be between 50.0 and 90.0%. The aim of the present study was to review worldwide data on populations of patients with MS and NMO in order to establish the rates of JCV seropositivity in these individuals. The present review followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines and used the following search terms: "JCV" OR "JC virus" AND "multiple sclerosis" OR "MS" OR "NMO" OR "neuromyelitis optica" AND "prevalence." These terms were searched for both in smaller and in larger clusters of words. The databases searched included PubMed, MEDLINE, SciELO, LILACS, Google Scholar, and Embase. After the initial selection, 18 papers were included in the review. These articles reported the prevalence of JCV antibodies in the serum of patients with MS or NMO living in 26 countries. The systematic review identified data on 29,319 patients with MS/NMO and found that 57.1% of them (16,730 individuals) were seropositive for the anti-JCV antibody (range, 40.0 to 69.0%). The median worldwide prevalence of JCV among adults with MS or NMO was found to be 57.1%.

Asymmetric electrooptic response in a nematic liquid crystal

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Dascalu, Constanta [Politechnica University of Bucharest, Bucharest (Romania)

2001-06-01

An asymmetric electrooptic response in nematic liquid crystal (LC) has been obtained. The liquid crystal hybrid cell was made by using a standard configuration. One of the ITO (Indium Tin Oxide) electrodes was covered with a surfactant, which induces a homeotropic alignment. The second of the indium tin oxide electrodes was covered by a thin layer of photopolymer, which was previously mixed with an acid, which favours a process of release of protons. Such cations are responsible of electrochemical process in the LC leading to an asymmetric electrooptic response, which depend on the polarity of the applied electric field. This fact is due to an internal field, which change the effective voltage thresholds for the reorientation of the liquid crystal. During the anodic polarization, the optical switching is inhibited because the effective field decreases below the threshold value. On contrary for the opposite polarization the effective field is enough to determine a homeotropic alignment. [Spanish] Se ha obtenido una respuesta electro-optica asimetrica en cristales liquidos neumaticos. La celula hibrida de cristal liquido fue construida utilizando una configuracion estandar. Uno de los electrodos ITO fue cubierto con una pelicula delgada de material organico para inducir una alineacion homeotropa. El otro electrodo ITO fue cubierto con una pelicula delgada de fotopolimero anteriormente mezclada con un acido para favorecer la emision de protones. Estos cationes son responsables del proceso electroquimico en LC, conduciendo a una respuesta electro-optica asimetrica que depende de la polaridad del campo electrico aplicado. Este efecto es originado por un campo interno que cambia el umbral efectivo del voltaje para la reorientacion del cristal liquido. Durante la polarizacion anodica, la conmutacion optica se inhibe debido a que el campo efectivo disminuye abajo del valor del umbral. Por el contrario, para la polarizacion opuesta el campo efectivo es suficiente para

Anti-N-methyl-d-aspartate receptor encephalitis in a patient with neuromyelitis optica spectrum disorders.

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Luo, Jing-Jing; Lv, He; Sun, Wei; Zhao, Juan; Hao, Hong-Jun; Gao, Feng; Huang, Yi-Ning

2016-07-01

We described a female patient with anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis occurring sequentially with neuromyelitis optica spectrum disorders (NMOSD). The 19-year-old patient initially presented a diencephalic syndrome with aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) and brain lesions which involving bilateral medial temporal lobes and periependymal surfaces of the third ventricle on magnetic resonance imaging (MRI). Ten months later, the patient developed cognitive impairment, psychiatric symptoms and dyskinesia with left basal ganglia lesions on brain MRI. Meanwhile, the anti-NMDAR antibodies were positive in the patient's serum and cerebrospinal fluid, while the screening tests for an ovarian teratoma and other tumors were all negative. Hence, the patient was diagnosed NMOSD and anti-NMDAR encephalitis followed by low-dose rituximab treatment with a good response. This case was another evidence for demyelinating syndromes overlapping anti-NMDAR encephalitis in Chinese patients. Copyright © 2016 Elsevier B.V. All rights reserved.

Neuromyelitis optica: association with paroxysmal painful tonic spasms.

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Carnero Contentti, E; Leguizamón, F; Hryb, J P; Celso, J; Pace, J L Di; Ferrari, J; Knorre, E; Perassolo, M B

2016-10-01

Paroxysmal painful tonic spasms (PPTS) were initially described in multiple sclerosis (MS) but they are more frequent in neuromyelitis optica (NMO). The objective is to report their presence in a series of cases of NMO and NMO spectrum disorders (NMOSD), as well as to determine their frequency and clinical features. We conducted a retrospective assessment of medical histories of NMO/NMOSD patients treated in 2 hospitals in Buenos Aires (Hospital Durand and Hospital Álvarez) between 2009 and 2013. Out of 15 patients with NMOSD (7 with definite NMO and 8 with limited NMO), 4 presented PPTS (26.66%). PPTS frequency in the definite NMO group was 57.14% (4/7). Of the 9 patients with longitudinally extensive transverse myelitis (LETM), 44.44% (9/15) presented PPTS. Mean age was 35 years (range, 22-38 years) and all patients were women. Mean time between NMO diagnosis and PPTS onset was 7 months (range, 1-29 months) and mean time from last relapse of LETM was 30 days (range 23-40 days). LETM (75% cervicothoracic and 25% thoracic) was observed by magnetic resonance imaging (MRI) in all patients. Control over spasms and pain was achieved in all patients with carbamazepine (associated with gabapentin in one case). No favourable responses to pregabalin, gabapentin, or phenytoin were reported. PPTS are frequent in NMO. Mean time of PPTS onset is approximately one month after an LETM relapse, with extensive cervicothoracic lesions appearing on the MRI scan. They show an excellent response to carbamazepine but little or no response to pregabalin and gabapentin. Prospective studies with larger numbers of patients are necessary in order to confirm these results. Copyright © 2014 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

An unusual chiasmal visual defect in a patient with neuromyelitis optica: case report Comprometimento quiasmático incomum em um paciente com neuromielite óptica: relato de caso

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Roberta Martins da Silva Costa

2007-02-01

Full Text Available PURPOSE: To report the unusual visual field finding due to a chiasmal neuritis in a 33-year-old female with the diagnosis of optic neuromyelitis optica (Devic's syndrome. METHODS: We report a case of a 33 years old female with limb paraesthesias, weakness in the legs, bowel and bladder dysfunction that was referred to the "Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo" in October 1995. Six years and four months later she had an acute visual involvement. Ophthalmologic examination, laboratory studies, magnetic resonance imaging (MRI and a 24-2 threshold visual field in the Humphrey field analyzer were performed. RESULTS: The MRI scan showed enlargement and cavitation on the spinal cord and chiasmal involvement (thickening of the chiasm with contrast enhancement and no demyelinating lesions in the brain, brainstem, or cerebellum. The central 24-degree threshold field examination showed an inferior visual field defect bitemporally, disclosing a chiasmal involvement. CONCLUSION: Chiasmal involvement may occur in neuromyelitis optica, probably due to a plaque within the chiasm. The authors call attention to the importance of visual field examination with particular regard to quantifying the visual impairment and follow-up of these patients.OBJETIVO: Relatar o caso de uma mulher de 33 anos de idade, com o diagnóstico de neuromielite óptica (síndrome de Devic acometendo o quiasma óptico que apresentou um escotoma incomum no exame de campo visual. MÉTODOS: Uma paciente do sexo feminino, portadora de parestesias nos membros inferiores, fraqueza nas pernas, disfunção da defecação e disfunção urinária, foi encaminhada para o Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo em outubro do ano de 1995. Seis anos e quatro meses mais tarde ela sofreu acometimento visual agudo. Foram realizados exame oftalmológico completo, exame de líquor, resson

Widespread cortical thinning in patients with neuromyelitis optica spectrum disorder.

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Kim, S-H; Kwak, K; Hyun, J-W; Jeong, I H; Jo, H-J; Joung, A; Kim, J-H; Lee, S H; Yun, S; Joo, J; Lee, J-M; Kim, H J

2016-07-01

Studies on cortical involvement and its relationship with cognitive function in patients with neuromyelitis optica spectrum disorder (NMOSD) remain scarce. The objective of this study was to compare cortical thickness on magnetic resonance imaging (MRI) between patients with NMOSD and multiple sclerosis (MS) and to investigate its relationship with clinical features and cognitive function. This observational clinical imaging study of 91 patients with NMOSD, 52 patients with MS and 44 healthy controls was conducted from 1 December 2013 to 30 April 2015 at the institutional referral center. Three tesla MRI of the brain and neuropsychological tests were performed. Cortical thickness was measured using three-dimensional surface-based analysis. Both sets of patients exhibited cortical thinning throughout the entire brain cortex. Patients with MS showed a significantly greater reduction in cortical thickness over broad regions of the bilateral frontal and parieto-temporal cortices and the left precuneus compared to those with NMOSD. Memory functions in patients with MS were correlated with broad regional cortical thinning, whereas no significant associations were observed between cortical thickness and cognitive function in patients with NMOSD. Widespread cortical thinning was observed in patients with NMOSD and MS, but the extent of cortical thinning was greater in patients with MS. The more severe cortical atrophy may contribute to memory impairment in patients with MS but not in those with NMOSD. These results provide in vivo evidence that the severity and clinical relevance of cortical thinning differ between NMOSD and MS. © 2016 EAN.

Comparative clinical characteristics of neuromyelitis optica spectrum disorders with and without medulla oblongata lesions.

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Wang, Yanqiang; Zhang, Lei; Zhang, Bingjun; Dai, Yongqiang; Kang, Zhuang; Lu, Ciyong; Qiu, Wei; Hu, Xueqiang; Lu, Zhengqi

2014-05-01

Brainstem involvement, especially the medulla oblongata (MO), has been reported in neuromyelitis optica spectrum disorders (NMOSDs). The purpose of this study was to investigate retrospectively and compare clinical, laboratory, and imaging features of NMOSDs with and without MO lesions. A total of 170 patients with NMOSDs were enrolled, including 44 patients with MO lesions and 126 patients without MO lesions. Clinical features, laboratory tests, and magnetic resonance imaging findings among these patients were assessed. MO lesions were found in 25.9 % of the NMOSDs patients. The mean duration was 13 months. Patients with MO lesions had a higher Annualized relapse rate and Expanded Disability Status Score Scale. Headache, dizziness, nystagmus, dysarthria, intractable hiccup and nausea, choking cough or dysphagia, movement disorders, and neuropathic pain were more common in MO lesion patients. Patients with MO lesions were more frequently complicated with thyroid diseases. Multiple brain involvement, More importantly, Longitudinally extensive transverse myelitis were more frequently found in patients with MO lesions. MO lesions might be a symbol of more severe neurologic deficits and worse prognosis of NMOSDs.

Recurrent neuromyelitis optica with diffuse central nervous system involvement: case report Neuromielite óptica recorrente com envolvimento difuso do sistema nervoso central: relato de caso

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Renan B. Domingues

2004-06-01

Full Text Available Several demyelinating disorders can affect children. The differential diagnosis between these diseases is usually an arduous task. Diagnostic criteria have been proposed for some of these disorders, however most of them have not yet been clinically and prospectively validated. Here we present a case of a ten year-old boy with recurrent bilateral optic neuritis and spinal cord involvement. Clinical and cerebrospinal fluid data have fulfilled diagnostic criteria for Devic's neuromyelitis optica (NMO. The differential diagnosis with multiple sclerosis (MS has become troublesome since not only optic nerves and spinal cord were involved. In one of the relapses a left hemiparesis with facial involvement was registered. Magnetic resonance imaging was also compatible with MS. This case illustrates that CNS demyelinating disorders can fulfill diagnostic criteria for more than one demyelinating disease, making the clinical judgment an important tool in the management of these patients.Diversas doenças desmielinizantes podem ocorrer em crianças, sendo muitas vezes o diagnóstico diferencial entre elas difícil. Critérios diagnósticos têm sido propostos para algumas destas entidades, entretanto nenhum deles pode ser considerado definitivo. O objetivo deste trabalho é apresentar o caso de um paciente de 10 anos de idade, com quadro recorrente de neurite óptica bilateral e mielopatia. Os dados clínicos e liquóricos preencheram critérios para o diagnóstico de neuromielite óptica de Devic. O diagnóstico diferencial foi especialmente difícil em relação à esclerose múltipla, pois não apenas os nervos ópticos e medula foram acometidos, visto que em um dos surtos registrou-se hemiparesia, com acometimento facial. A ressonância magnética foi também compatível com esclerose múltipla. Este caso ilustra que pacientes com doenças desmielinizantes do SNC podem preencher critérios diagnósticos para mais de uma delas, o que torna o julgamento cl

Factors associated with the effectiveness of plasma exchange for the treatment of NMO-IgG-positive neuromyelitis optica spectrum disorders.

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Lim, Young-Min; Pyun, So Young; Kang, Bong-Hui; Kim, Jimin; Kim, Kwang-Kuk

2013-08-01

To identify factors associated with plasma exchange response in neuromyelitis optica (NMO) spectrum disorders, the clinical and magnetic resonance imaging (MRI) features of 31 NMO-IgG-positive patients receiving plasma exchange for steroid-resistant exacerbations were analyzed. Functional improvement was observed in 65% of the patients. A lower baseline Expanded Disability Status Scale score was associated with favorable response (p = 0.040). Patients without cord atrophy had a higher success rate than patients with atrophy (p = 0.016). Levels of NMO-IgG did not differ between responders and non-responders before and after plasma exchange. In conclusion, a minimal pre-existing disability is the primary determinant of the effectiveness of plasma exchange.

Treatment of neuromyelitis optica: state-of-the-art and emerging therapies

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Papadopoulos, Marios C.; Bennett, Jeffrey L.; Verkman, Alan S.

2014-01-01

Neuromyelitis optica (NMO) is an autoimmune disease of the CNS that is characterized by inflammatory demyelinating lesions in the spinal cord and optic nerve, potentially leading to paralysis and blindness. NMO can usually be distinguished from multiple sclerosis (MS) on the basis of seropositivity for IgG antibodies against the astrocytic water channel aquaporin-4 (AQP4). Differentiation from MS is crucial, because some MS treatments can exacerbate NMO. NMO pathogenesis involves AQP4-IgG antibody binding to astrocytic AQP4, which causes complement-dependent cytotoxicity and secondary inflammation with granulocyte and macrophage infiltration, blood–brain barrier disruption and oligodendrocyte injury. Current NMO treatments include general immunosuppressive agents, B-cell depletion, and plasma exchange. Therapeutic strategies targeting complement proteins, the IL-6 receptor, neutrophils, eosinophils and CD19—all initially developed for other indications—are under clinical evaluation for repurposing for NMO. Therapies in the preclinical phase include AQP4-blocking antibodies and AQP4-IgG enzymatic inactivation. Additional, albeit currently theoretical, treatment options include reduction of AQP4 expression, disruption of AQP4 orthogonal arrays, enhancement of complement inhibitor expression, restoration of the blood–brain barrier, and induction of immune tolerance. Despite the many therapeutic options in NMO, no controlled clinical trials in patients with this condition have been conducted to date. PMID:25112508

Risk of venous thromboembolism in neuromyelitis optica patients hospitalized for acute relapse.

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Farber, Rebecca Straus; Gross, Robert; Zakin, Elina; Fabian, Michelle

2017-06-01

Neuromyelitis optica spectrum disorder (NMOSD) patients may be at increased risk of venous thromboembolism (VTE) not only due to ambulatory disability but also due to systemic autoimmune and inflammatory mechanisms altering the hemostatic balance. To compare the risk of VTE in NMOSD versus multiple sclerosis (MS) patients hospitalized for acute relapses. Hospital admissions for MS or NMOSD exacerbations were retrospectively identified. Demographics and medical history were recorded. The relationship between visit diagnosis and presence of VTE within 6 weeks of relapse onset was assessed by univariate logistic regression. A multivariate model evaluated the relationship between diagnosis, age, race, gender, body mass index (BMI), disease modifying therapy use, oral corticosteroid use, oral contraceptive use, smoking, length of stay (LOS), and ambulatory status on VTE risk. A total of 30 NMOSD patients had 55 hospitalizations; 179 MS patients had 264 hospitalizations. Six NMOSD patients and one MS patient had VTE. NMOSD visits compared to MS visits had an odds ratio (OR) of VTE of 32.2 ( p = 0.002). NMOSD was more likely to be associated with VTE (OR = 17.4; p = 0.01) controlling for age, LOS, and ambulatory disability. NMOSD may be a risk factor for VTE. Larger prospective studies are required to confirm this risk and determine implications for prophylaxis.

Brain parenchymal damage in neuromyelitis optica spectrum disorder - A multimodal MRI study

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Pache, F.; Paul, F. [Max Delbrueck Center for Molecular Medicine and Charite Universitaetsmedizin Berlin, NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Berlin (Germany); Charite Universitaetsmedizin Berlin, Department of Neurology, Berlin (Germany); Zimmermann, H.; Lacheta, A.; Papazoglou, S.; Kuchling, J.; Wuerfel, J.; Brandt, A.U. [Max Delbrueck Center for Molecular Medicine and Charite Universitaetsmedizin Berlin, NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Berlin (Germany); Finke, C. [Charite Universitaetsmedizin Berlin, Department of Neurology, Berlin (Germany); Humboldt-Universitaet zu Berlin, Berlin School of Mind and Brain, Berlin (Germany); Hamm, B. [Charite Universitaetsmedizin Berlin, Department of Radiology, Berlin (Germany); Ruprecht, K. [Charite Universitaetsmedizin Berlin, Department of Neurology, Berlin (Germany); Scheel, M. [Max Delbrueck Center for Molecular Medicine and Charite Universitaetsmedizin Berlin, NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Berlin (Germany); Charite Universitaetsmedizin Berlin, Department of Radiology, Berlin (Germany)

2016-12-15

To investigate different brain regions for grey (GM) and white matter (WM) damage in a well-defined cohort of neuromyelitis optica spectrum disorder (NMOSD) patients and compare advanced MRI techniques (VBM, Subcortical and cortical analyses (Freesurfer), and DTI) for their ability to detect damage in NMOSD. We analyzed 21 NMOSD patients and 21 age and gender matched control subjects. VBM (GW/WM) and DTI whole brain (TBSS) analyses were performed at different statistical thresholds to reflect different statistical approaches in previous studies. In an automated atlas-based approach, Freesurfer and DTI results were compared between NMOSD and controls. DTI TBSS and DTI atlas based analysis demonstrated microstructural impairment only within the optic radiation or in regions associated with the optic radiation (posterior thalamic radiation p < 0.001, 6.9 % reduction of fractional anisotropy). VBM demonstrated widespread brain GM and WM reduction, but only at exploratory statistical thresholds, with no differences remaining after correction for multiple comparisons. Freesurfer analysis demonstrated no group differences. NMOSD specific parenchymal brain damage is predominantly located in the optic radiation, likely due to a secondary degeneration caused by ON. In comparison, DTI appears to be the most reliable and sensitive technique for brain damage detection in NMOSD. (orig.)

Gender effect on neuromyelitis optica spectrum disorder with aquaporin4-immunoglobulin G.

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Kim, Sung-Min; Waters, Patrick; Woodhall, Mark; Kim, Yoo-Jin; Kim, Jin-Ah; Cheon, So Young; Lee, Sehoon; Jo, Seong Rae; Kim, Dong Gun; Jung, Kyeong Cheon; Lee, Kwang-Woo; Sung, Jung-Joon; Park, Kyung Seok

2017-07-01

Neuromyelitis optica spectrum disorder with aquaporin4-immunoglobulin G (NMOSD-AQP4) is an inflammatory disease characterised by a high female predominance. However, the effect of gender in patients with NMOSD-AQP4 has not been fully evaluated. The aim of this study was to determine the effect of gender in clinical manifestations and prognosis of patients with NMOSD-AQP4. The demographics, clinical and radiological characteristics, pattern reversal visual evoked potential (VEP) test results, and prognosis of 102 patients (18 males) with NMOSD-AQP4 were assessed. Male patients had a higher age at onset (48.7 vs 41 years, p = 0.037) and less optic neuritis as the onset attack (17% vs 44%, p = 0.026), higher tendency to manifest as isolated myelitis over the follow-up period (67% vs 28%, p = 0.005), fewer optic neuritis attacks per year (0.08 vs 0.27, p gender was significantly associated with the absence of optic neuritis attacks over the follow-up period independent of their age of onset. In NMOSD-AQP4 patients, gender impacts on disease onset age and site of attack. This may be an important clue in identifying NMOSD-AQP4 patients with limited manifestations as well as in predicting their clinical courses.

Brain parenchymal damage in neuromyelitis optica spectrum disorder - A multimodal MRI study

International Nuclear Information System (INIS)

Pache, F.; Paul, F.; Zimmermann, H.; Lacheta, A.; Papazoglou, S.; Kuchling, J.; Wuerfel, J.; Brandt, A.U.; Finke, C.; Hamm, B.; Ruprecht, K.; Scheel, M.

2016-01-01

To investigate different brain regions for grey (GM) and white matter (WM) damage in a well-defined cohort of neuromyelitis optica spectrum disorder (NMOSD) patients and compare advanced MRI techniques (VBM, Subcortical and cortical analyses (Freesurfer), and DTI) for their ability to detect damage in NMOSD. We analyzed 21 NMOSD patients and 21 age and gender matched control subjects. VBM (GW/WM) and DTI whole brain (TBSS) analyses were performed at different statistical thresholds to reflect different statistical approaches in previous studies. In an automated atlas-based approach, Freesurfer and DTI results were compared between NMOSD and controls. DTI TBSS and DTI atlas based analysis demonstrated microstructural impairment only within the optic radiation or in regions associated with the optic radiation (posterior thalamic radiation p < 0.001, 6.9 % reduction of fractional anisotropy). VBM demonstrated widespread brain GM and WM reduction, but only at exploratory statistical thresholds, with no differences remaining after correction for multiple comparisons. Freesurfer analysis demonstrated no group differences. NMOSD specific parenchymal brain damage is predominantly located in the optic radiation, likely due to a secondary degeneration caused by ON. In comparison, DTI appears to be the most reliable and sensitive technique for brain damage detection in NMOSD. (orig.)

The photo refractive polymers, physics and photonic applications of these new materials; Los polimeros fotorrefractivos, fisica y aplicaciones fotonicas de estos nuevos materiales

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Maldonado, J. L. [Centro de Investigacion en Optica, Leon, Guanajuato (Mexico); Garcia M, J. [Universidad Nacional Autonoma de Mexico, Mexico, D.F. (Mexico)

2001-12-01

This paper is a review about the new photo refractive organic materials and their potential photonic applications, the photo refractive polymers. The physical properties required for observing photo refractivity, including charge generation, transport, and nonlinear optical properties are discussed for amorphous polymers. The nonlinear optical properties on a macroscopic level are related to the microscopic properties by using the Oriented Gas model. Four Wave Mixing (FWM) and Two Beam Coupling (TBC) experiments, to investigate the photo refractive properties of the material by measuring the diffraction efficiency {eta} of the polymeric gratings, and the optical gain coefficient {eta} are described. Kogelnik's Coupled Wave model to describe the photo refractive polymeric gratings is reviewed. Finally some applications of these new polymers are presented. [Spanish] Este articulo es una revision sobre los nuevos materiales organicos fotorrrefractivos y sus potenciales aplicaciones fotonicas: los polimeros fotorrefractivos. Se discuten las propiedades fisicas requeridas para observar fotorrefractividad, incluyendo la generacion de cargas, el transporte y las propiedades opticas no lineales en polimeros amorfos. Usando el modelo de gas orientado, se muestra como se relacionan las propiedades opticas no lineales a nivel marcoscopico con las propiedades microscopicas. Se describen los experimentos de mezcla de cuatro ondas (FWM) y dos ondas (TBC) para investigar las propiedades fotorrefractivas del material, midiendo la eficiencia de difraccion {eta} de las rejillas polimericas y el coeficiente de ganancia optica {eta}. Se revisa el modelo de ondas acopladas de Kogelnik para describir las rejillas polimericas fotorrefractivas. Finalmente se presentan algunas aplicaciones de estos nuevos polimeros.

Combination Treatment of C16 Peptide and Angiopoietin-1 Alleviates Neuromyelitis Optica in an Experimental Model

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Yuanyuan Zhang

2018-01-01

Full Text Available Neuromyelitis optica (NMO is an autoimmune inflammatory demyelinating disease that mainly affects the spinal cord and optic nerve, causing blindness and paralysis in some individuals. Moreover, NMO may cause secondary complement-dependent cytotoxicity (CDC, leading to oligodendrocyte and neuronal damage. In this study, a rodent NMO model, showing typical NMO pathogenesis, was induced with NMO-IgG from patient serum and human complement. We then tested whether the combination of C16, an αvβ3 integrin-binding peptide, and angiopoietin-1 (Ang1, a member of the endothelial growth factor family, could alleviate NMO in the model. Our results demonstrated that this combination therapy significantly decreased disease severity, inflammatory cell infiltration, secondary demyelination, and axonal loss, thus reducing neural death. In conclusion, our study suggests a possible treatment that can relieve progressive blindness and paralysis in an animal model of NMO through improvement of the inflammatory milieu.

MOG-IgG-Associated Optic Neuritis, Encephalitis, and Myelitis: Lessons Learned From Neuromyelitis Optica Spectrum Disorder

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dos Passos, Giordani Rodrigues; Oliveira, Luana Michelli; da Costa, Bruna Klein; Apostolos-Pereira, Samira Luisa; Callegaro, Dagoberto; Fujihara, Kazuo; Sato, Douglas Kazutoshi

2018-01-01

Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been found in some cases diagnosed as seronegative neuromyelitis optica spectrum disorder (NMOSD). MOG-IgG allowed the identification of a subgroup with a clinical course distinct from that of NMOSD patients who are seropositive for aquaporin-4-IgG antibodies. MOG-IgG is associated with a wider clinical phenotype, not limited to NMOSD, with the majority of cases presenting with optic neuritis (ON), encephalitis with brain demyelinating lesions, and/or myelitis. Therefore, we propose the term MOG-IgG-associated Optic Neuritis, Encephalitis, and Myelitis (MONEM). Depending on the clinical characteristics, these patients may currently be diagnosed with NMOSD, acute disseminated encephalomyelitis, pediatric multiple sclerosis, transverse myelitis, or ON. With specific cell-based assays, MOG-IgG is emerging as a potential biomarker of inflammatory disorders of the central nervous system. We review the growing body of evidence on MONEM, focusing on its clinical aspects. PMID:29670575

A case of seropositive Neuromyelitis Optica in a paediatric patient with co-existing acute nephrotic syndrome.

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Volkman, Thomas; Hemingway, Cheryl

2017-11-01

Neuromyelitis optica (NMO) and NMO spectrum disorder (NMOSD) is a rare relapsing autoimmune disease of the central nervous system constituting less than 1% of demyelinating diseases (Jeffery and Buncic, 1996). It preferentially affects the optic nerves and spinal cord, with the brain parenchyma generally spared. Demyelinating lesions are characterised by longitudinally extensive transverse myelitis (LETM) and often longitudinally extensive optic neuritis. Following the discovery of a novel pathogenic antibody, Aquaporin 4 in 2004 (Lennon et al., 2004) this disease has been seen as a separate entity from Multiple Sclerosis (MS). We report the case of a severe AQP4 IgG case of NMO in a 10 year old child. This case unusually had a coexisting diagnosis of acute nephrotic syndrome which has only been reported once previously in the literature 2 . This article will examine some of the treatment challenges and the spectrum of co-existing autoimmune disease in NMOSD. Copyright © 2017. Published by Elsevier B.V.

Cognitive impairment in neuromyelitis optica spectrum disorders: A comparison of the Wechsler Adult Intelligence Scale-III and the Wechsler Memory Scale Revised with the Rao Brief Repeatable Neuropsychological Battery

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Fujimori, Juichi; Nakashima, Ichiro; Baba, Toru; Meguro, Yuko; Ogawa, Ryo; Fujihara, Kazuo

2017-01-01

Background: Approximately 55% of patients with neuromyelitis optica spectrum disorder (NMOSD) show cognitive impairment as evaluated using the Rao Brief Repeatable Neuropsychological Battery (BRBN), but this frequency appears to be higher than the frequency of specific brain lesions in NMOSD. Objective: We studied whether cognitive impairment could be observed in NMOSD patients with no or minor non-specific brain lesions. Methods: We evaluated cognitive function in 12 NMOSD and 14 MS patients...

Serum 25-hydroxyvitamin D3 is associated with disease status in patients with neuromyelitis optica spectrum disorders in south China.

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Shan, Yilong; Tan, Sha; Zhang, Lei; Huang, Jianhua; Sun, Xiaobo; Wang, Yuge; Cai, Wei; Qiu, Wei; Hu, Xueqiang; Lu, Zhengqi

2016-10-15

Here, we investigated the relationship between serum 25-hydroxyvitamin D 3 (25[OH]D 3 ) levels and neuromyelitis optica spectrum disorder (NMOSD). Patients with NMOSD had lower 25(OH)D 3 levels than healthy people, with lower levels in patients in the acute phase than those in remission. An inverse correlation was found between 25(OH)D 3 and Expanded Disability Status Scale scores of patients during attacks. Higher serum 25(OH)D 3 levels were associated with greater amelioration of symptoms during corticosteroid therapy. These results indicate that decreased vitamin D may be involved in NMOSD pathogenesis, and that 25(OH)D 3 serum levels may reflect the severity of NMOSD in the acute phase. Copyright © 2016 Elsevier B.V. All rights reserved.

Comparative molecular dynamics study of neuromyelitis optica-immunoglobulin G binding to aquaporin-4 extracellular domains.

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Alberga, Domenico; Trisciuzzi, Daniela; Lattanzi, Gianluca; Bennett, Jeffrey L; Verkman, Alan S; Mangiatordi, Giuseppe Felice; Nicolotti, Orazio

2017-08-01

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system in which most patients have serum autoantibodies (called NMO-IgG) that bind to astrocyte water channel aquaporin-4 (AQP4). A potential therapeutic strategy in NMO is to block the interaction of NMO-IgG with AQP4. Building on recent observation that some single-point and compound mutations of the AQP4 extracellular loop C prevent NMO-IgG binding, we carried out comparative Molecular Dynamics (MD) investigations on three AQP4 mutants, TP 137-138 AA, N 153 Q and V 150 G, whose 295-ns long trajectories were compared to that of wild type human AQP4. A robust conclusion of our modeling is that loop C mutations affect the conformation of neighboring extracellular loop A, thereby interfering with NMO-IgG binding. Analysis of individual mutations suggested specific hydrogen bonding and other molecular interactions involved in AQP4-IgG binding to AQP4. Copyright © 2017 Elsevier B.V. All rights reserved.

Serological markers associated with neuromyelitis optica spectrum disorders in South India

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Lekha Pandit

2016-01-01

Full Text Available Background: Neuromyelitis optica spectrum disorders (NMOSDs represent 20% of all demyelinating disorders in South India. No studies have determined the seroprevalence to both antibodies against aquaporin-4FNx01 and antimyelin oligodendrocyte glycoprotein antibody (anti-MOG+ in this population. Objective: To identify and characterize seropositive patients for anti-aquaporin-4 antibody (anti-AQP4+ and anti-MOG+ in South India. Materials and Methods: We included 125 consecutive patients (15 children who were serologically characterized using live transfected cells to human M23-AQP4 or full-length MOG. Results: Among a total of 125 patients, 30.4% of patients were anti-AQP4+, 20% were anti-MOG+, and 49.6% were seronegative. No patient was positive for both. Anti-MOG+ patients represented 28.7% (25/87 of seronegative NMOSD. In comparison to anti-AQP4+ patients, anti-MOG+ patients were commonly male, had less frequent attacks and milder disability on expanded disability status score scale. Seronegative patients were also predominantly male, 36% (9/25 had monophasic longitudinally extensive transverse myelitis and disability was comparable with anti-AQP4+ patients. Lumbar cord involvement was common in anti-MOG+ and seronegatives, whereas anti-AQP4+ patients had more cervical lesions. Conclusion: Anti-AQP4+/anti-MOG + patients accounted for nearly half of the patients suspected of having NMOSD in South India, indicating that antibody testing may be useful on the management of subgroups with different prognosis.

White matter atrophy and cognitive dysfunctions in neuromyelitis optica.

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Frederic Blanc

Full Text Available Neuromyelitis optica (NMO is an inflammatory disease of central nervous system characterized by optic neuritis and longitudinally extensive acute transverse myelitis. NMO patients have cognitive dysfunctions but other clinical symptoms of brain origin are rare. In the present study, we aimed to investigate cognitive functions and brain volume in NMO. The study population consisted of 28 patients with NMO and 28 healthy control subjects matched for age, sex and educational level. We applied a French translation of the Brief Repeatable Battery (BRB-N to the NMO patients. Using SIENAx for global brain volume (Grey Matter, GM; White Matter, WM; and whole brain and VBM for focal brain volume (GM and WM, NMO patients and controls were compared. Voxel-level correlations between diminished brain concentration and cognitive performance for each tests were performed. Focal and global brain volume of NMO patients with and without cognitive impairment were also compared. Fifteen NMO patients (54% had cognitive impairment with memory, executive function, attention and speed of information processing deficits. Global and focal brain atrophy of WM but not Grey Matter (GM was found in the NMO patients group. The focal WM atrophy included the optic chiasm, pons, cerebellum, the corpus callosum and parts of the frontal, temporal and parietal lobes, including superior longitudinal fascicle. Visual memory, verbal memory, speed of information processing, short-term memory and executive functions were correlated to focal WM volumes. The comparison of patients with, to patients without cognitive impairment showed a clear decrease of global and focal WM, including brainstem, corticospinal tracts, corpus callosum but also superior and inferior longitudinal fascicles. Cognitive impairment in NMO patients is correlated to the decreased of global and focal WM volume of the brain. Further studies are needed to better understand the precise origin of cognitive impairment in

European ancestry predominates in neuromyelitis optica and multiple sclerosis patients from Brazil.

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Doralina Guimarães Brum

Full Text Available BACKGROUND: Neuromyelitis optica (NMO is considered relatively more common in non-Whites, whereas multiple sclerosis (MS presents a high prevalence rate, particularly in Whites from Western countries populations. However, no study has used ancestry informative markers (AIMs to estimate the genetic ancestry contribution to NMO patients. METHODS: Twelve AIMs were selected based on the large allele frequency differences among European, African, and Amerindian populations, in order to investigate the genetic contribution of each ancestral group in 236 patients with MS and NMO, diagnosed using the McDonald and Wingerchuck criteria, respectively. All 128 MS patients were recruited at the Faculty of Medicine of Ribeirão Preto (MS-RP, Southeastern Brazil, as well as 108 healthy bone marrow donors considered as healthy controls. A total of 108 NMO patients were recruited from five Neurology centers from different Brazilian regions, including Ribeirão Preto (NMO-RP. PRINCIPAL FINDINGS: European ancestry contribution was higher in MS-RP than in NMO-RP (78.5% vs. 68.7% patients. In contrast, African ancestry estimates were higher in NMO-RP than in MS-RP (20.5% vs. 12.5% patients. Moreover, principal component analyses showed that groups of NMO patients from different Brazilian regions were clustered close to the European ancestral population. CONCLUSIONS: Our findings demonstrate that European genetic contribution predominates in NMO and MS patients from Brazil.

The Immunology of Neuromyelitis Optica-Current Knowledge, Clinical Implications, Controversies and Future Perspectives.

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Jasiak-Zatonska, Michalina; Kalinowska-Lyszczarz, Alicja; Michalak, Slawomir; Kozubski, Wojciech

2016-03-02

Neuromyelitis optica (NMO) is an autoimmune, demyelinating disorder of the central nervous system (CNS) with typical clinical manifestations of optic neuritis and acute transverse myelitis attacks. Previously believed to be a variant of multiple sclerosis (MS), it is now considered an independent disorder which needs to be differentiated from MS. The discovery of autoantibodies against aquaporin-4 (AQP4-IgGs) changed our understanding of NMO immunopathogenesis and revolutionized the diagnostic process. AQP4-IgG is currently regarded as a specific biomarker of NMO and NMO spectrum disorders (NMOsd) and a key factor in its pathogenesis. Nevertheless, AQP4-IgG seronegativity in 10%-25% of NMO patients suggests that there are several other factors involved in NMO immunopathogenesis, i.e., autoantibodies against aquaporin-1 (AQP1-Abs) and antibodies against myelin oligodendrocyte glycoprotein (MOG-IgGs). This manuscript reviews current knowledge about NMO immunopathogenesis, pointing out the controversial issues and showing potential directions for future research. Further efforts should be made to broaden our knowledge of NMO immunology which could have important implications for clinical practice, including the use of potential novel biomarkers to facilitate an early and accurate diagnosis, and modern treatment strategies improving long-term outcome of NMO patients.

MR imaging findings of the corpus callosum region in the differentiation between multiple sclerosis and neuromyelitis optica

International Nuclear Information System (INIS)

Chen, Zhiye; Feng, Feng; Yang, Yang; Li, Jinfeng; Ma, Lin

2012-01-01

Purpose: To evaluate MR imaging findings in corpus callosum region for the discrimination between opticospinal multiple sclerosis (OSMS) and neuromyelitis optica (NMO). Materials and methods: Forty-two definite OSMS with seronegative NMO-IgG and 23 NMO with seropositive NMO-IgG, and 27 age-matched normal controls (NC) were recruited. Sagittal T2-FLAIR images with 2-mm slice thickness were obtained. Subcallosal dot-dash (SCDD) sign and subcallosal striations (SCS) sign were reviewed. Results: SCDD was more commonly detected in OSMS (28 of 42 patients) than in NMO (5 of 23 patients) (P 0.05). For comparing ROC analysis among SCDD, SCS, and SCDD + SCS for predicted probability through binary logistic regression analysis, SCDD + SCS had the largest area under ROC curve (0.777) than SCDD (0.725) and SCS (0.608). Conclusion: SCDD may be helpful in distinguishing OSMS from NMO. The regression equation may also be a simple and effective method of choice for the differentiation between OSMS and NMO.

CFHR1-Modified Neural Stem Cells Ameliorated Brain Injury in a Mouse Model of Neuromyelitis Optica Spectrum Disorders.

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Shi, Kaibin; Wang, Zhen; Liu, Yuanchu; Gong, Ye; Fu, Ying; Li, Shaowu; Wood, Kristofer; Hao, Junwei; Zhang, Guang-Xian; Shi, Fu-Dong; Yan, Yaping

2016-11-01

A major hurdle for effective stem cell therapy is ongoing inflammation in the target organ. Reconditioning the lesion microenvironment may be an effective way to promote stem cell therapy. In this study, we showed that engineered neural stem cells (NSCs) with complement factor H-related protein 1, a complement inhibitor protein, can attenuate inflammatory infiltration and immune-mediated damage of astrocytes, an important pathogenic progress in patients with neuromyelitis optica spectrum disorders. Furthermore, we demonstrated that transplantation of the complement factor H-related protein 1-modified NSCs effectively blocked the complement activation cascade and inhibited formation of the membrane attack complex, thus contributing to the protection of endogenous and transplanted NSC-differentiated astrocytes. Therefore, manipulation of the lesion microenvironment contributes to a more effective cell replacement therapeutic strategy for autoimmune diseases of the CNS. Copyright © 2016 by The American Association of Immunologists, Inc.

Diagnosis of neuromyelitis optica (NMO) spectrum disorders: is MRI obsolete?

International Nuclear Information System (INIS)

Downer, Jonathan James; Carter, Ranjana; Kueker, Wilhelm; Quaghebeur, Gerardine; Leite, Maria Isabel; Palace, Jacqueline

2012-01-01

Neuromyelitis optica (NMO) is a severe demyelinating disease that preferentially involves spinal cord and optic nerve. It is part of a spectrum of neurological conditions associated with antibodies to aquaporin-4 (AQP4). This study investigates the role of MRI where novel, more sensitive AQP4 antibody immunoassay techniques are being used. Retrospective review of neuroimaging in 69 patients (25 antibody positive, 44 antibody negative), investigated in the context of suspected NMO or NMO spectrum disorder, was performed independently by two consultant neuroradiologists. Longitudinally extensive, central spinal cord lesions were more frequent in AQP4 positive patients (95.2% vs 35.5%, p < 0.0001; 85.7% vs 45.2%, p = 0.015). Multiple sclerosis diagnostic criteria were less frequently fulfilled on brain MRI in antibody positive patients (5.6% vs 33.3%, p = 0.035). Juxtacortical and corpus callosal lesions were also less common in this group (16.7% vs 46.7%, p = 0.063; 5.6% vs 46.7%, p = 0.0034). Hypothalamic and periependymal disease related to the aqueduct was not seen in antibody negative patients. T1 hypointensity was more common in cord lesions of antibody positive patients (75.0% vs 35.3%, p = 0.037). However, this characteristic did not discriminate antibody positive and negative longitudinally extensive cord lesions (73.3% vs 62.5%, p = 0.66). The NMO spectrum of diseases are among an increasing number of neurological conditions defined by serological tests. However, despite improved immunoassay techniques, MRI of the brain and spinal cord continues to be among the first-line investigations in these patients, providing valuable diagnostic information that will help guide patient management. (orig.)

Gender differences among Chinese patients with neuromyelitis optica spectrum disorders.

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Sun, Hui; Sun, Xuan; Li, Jie; Huo, Yunyun; Wu, Lei; Huang, Dehui; Yu, Shengyuan; Wu, Weiping

2017-10-01

Neuromyelitis optica spectrum disorders (NMOSD) are autoimmune, inflammatory demyelinating diseases of the central nervous system, which have established variations in prevalence across different ethnicities and genders. The objective of this study was to investigate differences in clinical features among men and women with NMOSD, according to the 2015 diagnostic criteria. A total of 97 patients with NMOSD were recruited from inpatient neurology clinics in this retrospective study. Demographic and clinical data were extracted from the various databases. Data on epidemiology, clinical signs, initial symptoms, and laboratory indices of men and women with NMOSD were compared. The cohort of this study had a female/male ratio of 5.47:1, with annualized relapse rates of 0.72 in female and 0.56 in male patients. Among female patients, 29.2% and 53.6% initially experienced acute optic neuritis and acute myelitis, respectively, while the prevalence of these symptoms was 46.6% and 53.3% among male patients. A total of 14.6% and 2.4% of female patients had area postrema symptoms and other brainstem signs, respectively on study enrollment. The prevalence of anti-AQP4-autoantibodies and anti-thyroid peroxidase autoantibodies/anti-thyroglobulin autoantibodies (TPO/TG-Ab) was significantly higher among women (77% and 45.7%) than among men (46.1% and 13.3%) (P < 0.05 for both comparisons). A total of 11 women with NMOSD (11.3% of the cohort) also had autoimmune diseases. Women with NMOSD have higher morbidity levels than men with this disease and are more likely to have autoimmune diseases and brainstem lesions, especially in the area postrema. Copyright © 2017 Elsevier B.V. All rights reserved.

Diagnosis of neuromyelitis optica (NMO) spectrum disorders: is MRI obsolete?

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Downer, Jonathan James; Carter, Ranjana; Kueker, Wilhelm; Quaghebeur, Gerardine [John Radcliffe Hospital, Department of Neuroradiology, Oxford (United Kingdom); Leite, Maria Isabel; Palace, Jacqueline [Oxford University, Department of Clinical Neurology, Oxford (United Kingdom)

2012-04-15

Neuromyelitis optica (NMO) is a severe demyelinating disease that preferentially involves spinal cord and optic nerve. It is part of a spectrum of neurological conditions associated with antibodies to aquaporin-4 (AQP4). This study investigates the role of MRI where novel, more sensitive AQP4 antibody immunoassay techniques are being used. Retrospective review of neuroimaging in 69 patients (25 antibody positive, 44 antibody negative), investigated in the context of suspected NMO or NMO spectrum disorder, was performed independently by two consultant neuroradiologists. Longitudinally extensive, central spinal cord lesions were more frequent in AQP4 positive patients (95.2% vs 35.5%, p < 0.0001; 85.7% vs 45.2%, p = 0.015). Multiple sclerosis diagnostic criteria were less frequently fulfilled on brain MRI in antibody positive patients (5.6% vs 33.3%, p = 0.035). Juxtacortical and corpus callosal lesions were also less common in this group (16.7% vs 46.7%, p = 0.063; 5.6% vs 46.7%, p = 0.0034). Hypothalamic and periependymal disease related to the aqueduct was not seen in antibody negative patients. T1 hypointensity was more common in cord lesions of antibody positive patients (75.0% vs 35.3%, p = 0.037). However, this characteristic did not discriminate antibody positive and negative longitudinally extensive cord lesions (73.3% vs 62.5%, p = 0.66). The NMO spectrum of diseases are among an increasing number of neurological conditions defined by serological tests. However, despite improved immunoassay techniques, MRI of the brain and spinal cord continues to be among the first-line investigations in these patients, providing valuable diagnostic information that will help guide patient management. (orig.)

Sjögren syndrome and neuromyelitis optica spectrum disorder co-exist in a common autoimmune milieu

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Diogo C. Carvalho

2014-06-01

Full Text Available The relationship between Sjögren’s syndrome (SS and neuromyelitis optica spectrum disorder (NMOSD is not completely understood. We report two patients with both conditions and review 47 other previously reported cases meeting currently accepted diagnostic criteria, from 17 articles extracted from PubMed. Out of 44 patients whose gender was informed, 42 were females. Mean age at onset of neurological manifestation was 36.2 years (10-74. Serum anti-AQP4-IgG was positive in 32 patients, borderline in 1, and negative in 4. Our Case 1 was seronegative for AQP4-IgG and had no non-organ-specific autoantibodies other than anti-SSB antibodies. Our Case 2 had serum anti-AQP4, anti-SSA/SSB, anti-thyreoglobulin and anti-acethylcholine-receptor antibodies, as well as clinical hypothyreoidism, but no evidence of myasthenia gravis. Our Cases and others, as previously reported in literature, with similar heterogeneous autoimmune response to aquaporin-4, suggest that SS and NMO co-exist in a common autoimmune milieu which is not dependent on aquaporin-4 autoimmunity.

Long-term MRI findings in neuromyelitis optica: seropositive versus seronegative patients.

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Kıyat-Atamer, A; Ekizoğlu, E; Tüzün, E; Kürtüncü, M; Shugaiv, E; Akman-Demir, G; Eraksoy, M

2013-05-01

Neuromyelitis optica (NMO) is a severe demyelinating inflammatory disorder associated with serum antibodies against aquaporin 4 (AQP4-Ab). A significant number of patients with NMO remain seronegative over time. Long-term observational magnetic resonance imaging (MRI) studies of the CNS in patients with NMO are rare or of limited duration. The objective of this study is to determine long-term MRI characteristics of seropositive and seronegative patients, and assess possible overlap with multiple sclerosis (MS). Clinical and radiological characteristics of 28 patients with NMO at onset and of 17 patients after an average follow-up time of 9 years were recorded. Fifty percent of patients were seropositive for AQP4-Ab. Onset and final brain/spinal MRI scans were retrospectively analysed and compared. Significantly more patients in the seronegative group had brain lesions at onset. Spinal lesions of seropositive patients were longer and showed increased cord swelling at onset MRI scans. After the follow-up time the differences between both groups disappeared. Patients in the seropositive group tended to develop brain lesions over time. No patient fulfilled Barkhof's or McDonald's radiological criteria for MS at onset or over time. Brain MRI features show differences between seropositive and seronegative patients at time of onset in NMO, but differences between groups vanish over time. None of the AQP4-negative patients fulfill radiological MS criteria on a long-term basis, suggesting that seronegative NMO constitutes an independent entity. © 2012 The Author(s) European Journal of Neurology © 2012 EFNS.

Neuromyelitis optica IgG stimulates an immunological response in rat astrocyte cultures.

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Howe, Charles L; Kaptzan, Tatiana; Magaña, Setty M; Ayers-Ringler, Jennifer R; LaFrance-Corey, Reghann G; Lucchinetti, Claudia F

2014-05-01

Neuromyelitis optica (NMO) is a primary astrocyte disease associated with central nervous system inflammation, demyelination, and tissue injury. Brain lesions are frequently observed in regions enriched in expression of the aquaporin-4 (AQP4) water channel, an antigenic target of the NMO IgG serologic marker. Based on observations of disease reversibility and careful characterization of NMO lesion development, we propose that the NMO IgG may induce a dynamic immunological response in astrocytes. Using primary rat astrocyte-enriched cultures and treatment with NMO patient-derived serum or purified IgG, we observed a robust pattern of gene expression changes consistent with the induction of a reactive and inflammatory phenotype in astrocytes. The reactive astrocyte factor lipocalin-2 and a broad spectrum of chemokines, cytokines, and stress response factors were induced by either NMO patient serum or purified IgG. Treatment with IgG from healthy controls had no effect. The effect is disease-specific, as serum from patients with relapsing-remitting multiple sclerosis, Sjögren's, or systemic lupus erythematosus did not induce a response in the cultures. We hypothesize that binding of the NMO IgG to AQP4 induces a cellular response that results in transcriptional and translational events within the astrocyte that are consistent with a reactive and inflammatory phenotype. Strategies aimed at reducing the inflammatory response of astrocytes may short circuit an amplification loop associated with NMO lesion development. Copyright © 2014 Wiley Periodicals, Inc.

Neuromyelitis optica spectrum disorder: 2-deoxy-2-[18F]fluoro-D-glucose positron emission tomography findings--case report.

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Oyama, Hirofumi; Miwa, Shigeru; Noda, Tomoyuki; Sobajima, Atsuhiro; Kito, Akira; Maki, Hideki; Hattori, Kenichi; Wada, Kentaro

2012-01-01

A 51-year-old female with a history of rheumatoid arthritis rapidly developed anterior neck pain and paresis in the left upper and lower extremities and right lower extremity, sensory disturbance in the left upper and lower extremities, and bladder and rectal disorder. Adduction of the left eye and abduction of the right eye were also disturbed. Spinal magnetic resonance imaging demonstrated severe edema in the C1-T5 levels, which then deteriorated rapidly over 3 days, and lesions enhanced with gadolinium in the C1-C3 and C5-T3 levels. 2-Deoxy-2-[18F]fluoro-D-glucose positron emission tomography study demonstrated the inflammatory sites as segmental enhanced accumulation in the C1-C3, C5-C6, and T1 levels. The serum anti-aquaporin 4 antibody level was positive and she was diagnosed with neuromyelitis optica spectrum disorder. Marked improvement in the neurological conditions, concomitant with reduced spinal cord edema, was obtained by steroid pulse therapy.

Clinical characteristics of disabling attacks at onset in patients with neuromyelitis optica spectrum disorder.

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Seok, Jin Myoung; Cho, Eun Bin; Lee, Hye Lim; Cho, Hye-Jin; Min, Ju-Hong; Lee, Kwang Ho; Kim, Byoung Joon

2016-09-15

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. 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. Fifty-seven patients were enrolled (53 females; onset age, 41.9±14.8years). Ten patients (17.5%) had a DAO; four had become unable to walk without assistance following myelitis, and six had severe visual impairment following optic neuritis despite rescue treatments. Attack severity at nadir was the only clinical factor predicting a DAO (odds ratio, 2.120; 95% CI, 1.162-3.869; P=0.014). The use of immunosuppressants delayed the interval to the first relapse (P=0.003). Our study showed characteristics of NMO onset attacks that caused severe disability. However, no clinically modifiable factors predicted disabling attacks, except attack severity. Copyright © 2016 Elsevier B.V. All rights reserved.

Cognitive dysfunction in adult patients with neuromyelitis optica: a systematic review and meta-analysis.

Science.gov (United States)

Meng, Hao; Xu, Jun; Pan, Chenling; Cheng, Jiaxing; Hu, Yue; Hong, Yin; Shen, Yuehai; Dai, Hua

2017-08-01

The objective of this study was to investigate cognitive dysfunction in 24-60-year-old neuromyelitis optica (NMO) patients, demographically matched healthy subjects, and MS patients. We conducted a comprehensive literature review of the PubMed, Medline, EMBASE, CNKI, Wan Fang Date, Web of Science, and Cochrane Library databases from inception to May 2016 for case-control studies that reported cognitive test scores in NMO patients, healthy subjects, and MS patients. Outcome measures were cognitive function evaluations, including performance on attention, language, memory, information processing speed, and executive function tests. The meta-analysis included eight studies. NMO patients performed significantly worse on attention (P < 0.00001), language (P = 0.00008), memory (P = 0.00004), information processing speed (P < 0.00001), and executive function tests (P = 0.00009) than healthy subjects. There were no significant differences in performance between NMO patients and MS patients on these tests. This meta-analysis indicates that NMO patients aged 24-60 years have significantly worse cognitive performance than demographically matched healthy subjects. However, this was comparable to the performance of demographically matched MS patients. There is a need for further rigorous randomized controlled trials with focus on elucidating the underlying mechanism of cognitive dysfunction in NMO patients.

Altered topological organization of white matter structural networks in patients with neuromyelitis optica.

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Yaou Liu

Full Text Available OBJECTIVE: To investigate the topological alterations of the whole-brain white-matter (WM structural networks in patients with neuromyelitis optica (NMO. METHODS: The present study involved 26 NMO patients and 26 age- and sex-matched healthy controls. WM structural connectivity in each participant was imaged with diffusion-weighted MRI and represented in terms of a connectivity matrix using deterministic tractography method. Graph theory-based analyses were then performed for the characterization of brain network properties. A multiple linear regression analysis was performed on each network metric between the NMO and control groups. RESULTS: The NMO patients exhibited abnormal small-world network properties, as indicated by increased normalized characteristic path length, increased normalized clustering and increased small-worldness. Furthermore, largely similar hub distributions of the WM structural networks were observed between NMO patients and healthy controls. However, regional efficiency in several brain areas of NMO patients was significantly reduced, which were mainly distributed in the default-mode, sensorimotor and visual systems. Furthermore, we have observed increased regional efficiency in a few brain regions such as the orbital parts of the superior and middle frontal and fusiform gyri. CONCLUSION: Although the NMO patients in this study had no discernible white matter T2 lesions in the brain, we hypothesize that the disrupted topological organization of WM networks provides additional evidence for subtle, widespread cerebral WM pathology in NMO.

Evaluation of Clinical Interest of Anti-Aquaporin-4 Autoantibody Followup in Neuromyelitis Optica

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Jean-Baptiste Chanson

2013-01-01

Full Text Available Neuromyelitis optica (NMO is an autoimmune disease in which a specific biomarker named NMO-IgG and directed against aquaporin-4 (AQP4 has been found. A correlation between disease activity and anti-AQP4 antibody (Ab serum concentration or complement-mediated cytotoxicity has been reported, but the usefulness of longitudinal evaluation of these parameters remains to be evaluated in actual clinical practice. Thirty serum samples from 10 NMO patients positive for NMO-IgG were collected from 2006 to 2011. Anti-AQP4 Ab serum concentration and complement-mediated cytotoxicity were measured by flow cytometry using two quantitative cell-based assays (CBA and compared with clinical parameters. We found a strong correlation between serum anti-AQP4 Ab concentration and complement-mediated cytotoxicity (P<0.0001. Nevertheless, neither relapse nor worsening of impairment level was closely associated with a significant increase in serum Ab concentration or cytotoxicity. These results suggest that complement-mediated serum cytotoxicity assessment does not provide extra insight compared to anti-AQP4 Ab serum concentration. Furthermore, none of these parameters appears closely related to disease activity and/or severity. Therefore, in clinical practice, serum anti-AQP4 reactivity seems not helpful as a predictive biomarker in the followup of NMO patients as a means of predicting the onset of a relapse and adapting the treatment accordingly.

Complement-independent retinal pathology produced by intravitreal injection of neuromyelitis optica immunoglobulin G

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Christian M. Felix

2016-10-01

Full Text Available Abstract Background Neuromyelitis optica (NMO, an autoimmune inflammatory disease of the central nervous system, is often associated with retinal abnormalities including thinning of the retinal nerve fiber layer and microcystic changes. Here, we demonstrate that passive transfer of an anti-aquaporin-4 autoantibody (AQP4-IgG produces primary retinal pathology. Methods AQP4-IgG was delivered to adult rat retinas by intravitreal injection. Rat retinas and retinal explant cultures were assessed by immunofluorescence. Results Immunofluorescence showed AQP4-IgG deposition on retinal Müller cells, with greatly reduced AQP4 expression and increased glial fibrillary acidic protein by 5 days. There was mild retinal inflammation with microglial activation but little leukocyte infiltration and loss of retinal ganglion cells by 30 days with thinning of the ganglion cell complex. Interestingly, the loss of AQP4 was complement independent as seen in cobra venom factor-treated rats and in normal rats administered a mutated AQP4-IgG lacking complement effector function. Exposure of ex vivo retinal cultures to AQP4-IgG produced a marked reduction in AQP4 expression by 24 h, which was largely prevented by inhibitors of endocytosis or lysosomal acidification. Conclusions Passive transfer of AQP4-IgG results in primary, complement-independent retinal pathology, which might contribute to retinal abnormalities seen in NMO patients.

Abnormal brain function in neuromyelitis optica: A fMRI investigation of mPASAT.

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Wang, Fei; Liu, Yaou; Li, Jianjun; Sondag, Matthew; Law, Meng; Zee, Chi-Shing; Dong, Huiqing; Li, Kuncheng

2017-10-01

Cognitive impairment with the Neuromyelitis Optica (NMO) patients is debated. The present study is to study patterns of brain activation in NMO patients during a pair of task-related fMRI. We studied 20 patients with NMO and 20 control subjects matched for age, gender, education and handedness. All patients with NMO met the 2006 Wingerchuk diagnostic criteria. The fMRI paradigm included an auditory attention monitoring task and a modified version of the Paced Auditory Serial Addition Task (mPASAT). Both tasks were temporally and spatially balanced, with the exception of task difficulty. In mPASAT, Activation regions in control subjects included bilateral superior temporal gyri (BA22), left inferior frontal gyrus (BA45), bilateral inferior parietal lobule (BA7), left cingulate gyrus (BA32), left insula (BA13), and cerebellum. Activation regions in NMO patients included bilateral superior temporal gyri (BA22), left inferior frontal gyrus (BA9), right cingulate gyrus (BA32), right inferior parietal gyrus (BA40), left insula (BA13) and cerebellum. Some dispersed cognition related regions are greater in the patients. The present study showed altered cerebral activation during mPASAT in patients with NMO relative to healthy controls. These results are speculated to provide further evidence for brain plasticity in patients with NMO. Copyright © 2017 Elsevier B.V. All rights reserved.

STAT4 Polymorphisms are Associated with Neuromyelitis Optica Spectrum Disorders.

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Shi, Ziyan; Zhang, Qin; Chen, Hongxi; Lian, Zhiyun; Liu, Ju; Feng, Huiru; Miao, Xiaohui; Du, Qin; Zhou, Hongyu

2017-12-01

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. The results of multiple test comparisons were corrected using the Benjamini and Hochberg false discovery rate (FDR-BH). After correcting for multiple test comparisons, the minor alleles of four STAT4 SNPs exhibited significant association with increased risk of NMOSD (rs7574865 T, odds ratio [OR] = 1.66, 95% confidence interval [CI] 1.32-2.08, P corr  = 0.000; rs10181656 G, OR = 1.62, 95% CI 1.29-2.03, P corr  = 0.000; rs10168266 T, OR = 1.59, 95% CI 1.27-2.00, P corr  = 0.001; and rs13426947 A, OR = 1.51, 95% CI 1.21-1.90, P corr  = 0.004). Identical results were observed in the dominant, recessive, and additive models. In contrast, the G allele of rs7601754 displayed a protective effect against NMOSD (OR = 0.53, 95% CI 0.36-0.76, P corr  = 0.006). Our study indicates that STAT4 polymorphisms are associated with the risk of NMOSD, which provides novel insights into the underlying mechanisms of this disease.

Cognitive impairment in neuromyelitis optica spectrum disorders: A comparison of the Wechsler Adult Intelligence Scale-III and the Wechsler Memory Scale Revised with the Rao Brief Repeatable Neuropsychological Battery

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Juichi Fujimori

2017-12-01

Full Text Available Background: Approximately 55% of patients with neuromyelitis optica spectrum disorder (NMOSD show cognitive impairment as evaluated using the Rao Brief Repeatable Neuropsychological Battery (BRBN, but this frequency appears to be higher than the frequency of specific brain lesions in NMOSD. Objective: We studied whether cognitive impairment could be observed in NMOSD patients with no or minor non-specific brain lesions. Methods: We evaluated cognitive function in 12 NMOSD and 14 MS patients using the Wechsler Adult Intelligence Scale-III (WAIS-III, the Wechsler Memory Scale-Revised (WMS-R, and the BRBN. We judged as cognitively impaired patients whose scores were below the average by 2 standard deviations or greater in 2 or more cognitive domains. Results: Cognitive impairment was observed in 5 MS patients (35.7% and in the only NMOSD patient (8.3% with symptomatic brain lesions, but not in the other NMOSD patients who had no or minor non-specific brain lesions. Meanwhile, 5 NMOSD (41.7% and 4 MS (28.6% patients who had normal cognition according to the WAIS-III and WMS-R were assessed as cognitively impaired by the BRBN (which is not standardized for age. Conclusions: Cognitive function in NMOSD patients with no or mild non-specific brain lesions was preserved according to the WAIS-III and WMS-R. Keywords: Neuromyelitis Optica, Cognitive impairment, Wechsler Adult Intelligence Scale-III, Wechsler Memory Scale-Revised, Rao Brief Repeatable Neuropsychological Battery, Multiple sclerosis

MR imaging findings of the corpus callosum region in the differentiation between multiple sclerosis and neuromyelitis optica

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Chen, Zhiye, E-mail: yyqf@hotmail.com [Department of Radiology, PLA General Hospital, 28 Fuxing Road, Beijing 100853 (China); Feng, Feng, E-mail: cjr.fengfeng@vip.163.com [Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 1 Shuaifuyuan, Wangfujing, Beijing 100730 (China); Yang, Yang, E-mail: whitean0584@sina.com.cn [Department of Neurology, PLA General Hospital, 28 Fuxing Road, Beijing 100853 (China); Li, Jinfeng, E-mail: lijf_301@163.com [Department of Radiology, PLA General Hospital, 28 Fuxing Road, Beijing 100853 (China); Ma, Lin, E-mail: cjr.malin@vip.163.com [Department of Radiology, PLA General Hospital, 28 Fuxing Road, Beijing 100853 (China)

2012-11-15

Purpose: To evaluate MR imaging findings in corpus callosum region for the discrimination between opticospinal multiple sclerosis (OSMS) and neuromyelitis optica (NMO). Materials and methods: Forty-two definite OSMS with seronegative NMO-IgG and 23 NMO with seropositive NMO-IgG, and 27 age-matched normal controls (NC) were recruited. Sagittal T2-FLAIR images with 2-mm slice thickness were obtained. Subcallosal dot-dash (SCDD) sign and subcallosal striations (SCS) sign were reviewed. Results: SCDD was more commonly detected in OSMS (28 of 42 patients) than in NMO (5 of 23 patients) (P < 0.05). SCS showed no difference between OSMS (31 of 42 patients) and NMO (12 of 23 patients) (P > 0.05). For comparing ROC analysis among SCDD, SCS, and SCDD + SCS for predicted probability through binary logistic regression analysis, SCDD + SCS had the largest area under ROC curve (0.777) than SCDD (0.725) and SCS (0.608). Conclusion: SCDD may be helpful in distinguishing OSMS from NMO. The regression equation may also be a simple and effective method of choice for the differentiation between OSMS and NMO.

Soluble CD40 ligand contributes to blood-brain barrier breakdown and central nervous system inflammation in multiple sclerosis and neuromyelitis optica spectrum disorder.

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Masuda, Hiroki; Mori, Masahiro; Uchida, Tomohiko; Uzawa, Akiyuki; Ohtani, Ryohei; Kuwabara, Satoshi

2017-04-15

Soluble CD40 ligand (sCD40L) is reported to disrupt the blood-brain barrier (BBB). Cerebrospinal fluid (CSF) and serum sCD40L levels were measured in 29 multiple sclerosis (MS), 29 neuromyelitis optica spectrum disorder (NMOSD), and 27 disease control (DC) patients. In MS, serum sCD40L levels were higher than in DCs and positively correlated with the CSF/serum albumin ratio (Qalb). In NMOSD, CSF sCD40L levels were significantly increased compared to DCs, and were correlated to Qalb, CSF cell counts, protein concentrations, and interleukin-6 levels. sCD40L could be involved in BBB disruption in MS, whereas it may contribute to CNS inflammation in NMOSD. Copyright © 2017 Elsevier B.V. All rights reserved.

Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder

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Flanagan, Eoin P.; Fujihara, Kazuo; Kim, Ho Jin; Skejoe, Hanne P.; Wuerfel, Jens; Kuroda, Hiroshi; Kim, Su Hyun; Maillart, Elisabeth; Marignier, Romain; Pittock, Sean J.; Paul, Friedemann; Weinshenker, Brian G.

2017-01-01

Objective: To describe leptomeningeal blood-barrier impairment reflected by MRI gadolinium-enhanced lesions in patients with aquaporin-4 immunoglobulin G (AQP4-IgG)–positive neuromyelitis optica spectrum disorder (NMOSD). Methods: A retrospective case series of 11 AQP4-IgG–positive NMOSD patients with leptomeningeal enhancement (LME) were collected from 5 centers. External neuroradiologists, blinded to the clinical details, evaluated MRIs. Results: LME was demonstrated on postcontrast T1-weighted and fluid-attenuated inversion recovery images as a sign of leptomeningeal blood-barrier disruption and transient leakage of contrast agent into the subarachnoid space in 11 patients, 6 in the brain and 6 in the spinal cord. The patterns of LME were linear or extensive and were accompanied by periependymal enhancement in 5 cases and intraparenchymal enhancement in all cases. The location of LME in the spinal cord was adjacent to intraparenchymal contrast enhancement with involvement of a median number of 12 (range 5–17) vertebral segments. At the time of LME on MRI, all patients had a clinical attack such as encephalopathy (36%) and/or myelopathy (70%) with median interval between symptom onset and LME of 12 days (range 2–30). LME occurred in association with an initial area postrema attack (44%), signs of systemic infection (33%), or AQP4-IgG in CSF (22%) followed by clinical progression. LME was found at initial clinical presentation in 5 cases and at clinical relapses leading to a diagnosis of NMOSD in 6 cases. Conclusion: This study suggests that altered leptomeningeal blood barrier may be accompanied by intraparenchymal blood-brain barrier breakdown in patients with AQP4-IgG–positive NMOSD during relapses. PMID:28451627

Frequency of autoimmune disorders and autoantibodies in patients with neuromyelitis optica.

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Pereira, Wildéa Lice de Carvalho Jennings; Reiche, Edna Maria Vissoci; Kallaur, Ana Paula; Oliveira, Sayonara Rangel; Simão, Andréa Name Colado; Lozovoy, Marcell Alysson Batisti; Schiavão, Lucas José Vaz; Rodrigues, Paula Raquel do Vale Pascoal; Alfieri, Daniela Frizon; Flauzino, Tamires; Kaimen-Maciel, Damacio Ramón

2017-06-01

The aim of this study was to report the frequency of autoimmune disorders and autoantibodies in 22 patients with neuromyelitis optica (NMO), as well as whether the seropositivity for autoantibodies differs between anti-aquaporin 4 (AQP4) positive and AQP4 negative NMO patients. Demographic, medical records, and a profile of autoantibodies were evaluated in 22 NMO patients, including AQP4, anti-thyroid-stimulating hormone receptor, antinuclear antibodies (ANA), anti-thyroperoxidase (anti-TPO), anti-thyroglobulin (anti-Tg), anti-double-stranded DNA, anti-neutrophil cytoplasmic, anti-cyclic citrullinate peptide, rheumatoid factor, anti-SSA/Ro, anti-SSB/La, anti-Smith antibodies (anti-Sm), anti-ribonucleoprotein, anti-nucleosome, and anti-Scl70. Thyroid-stimulating hormone and free thyroxin were measured. The frequency of women was higher than men (95.5% vs. 4.5%) and 68.2% were Afro-Brazilians. Six (27.3%) patients presented other autoimmune disorders, such as Hashimoto thyroiditis (n=2), Graves' disease (n=1), juvenile idiopathic arthritis (n=1), systemic lupus erythematosus and systemic sclerosis (n=1), and Raynaud's phenomenon (n=1). The most frequent autoantibodies were anti-AQP4 (54.5%), anti-nucleosome (31.8%), ANA (27.3%), anti-TPO (22.7%), and anti-Tg (22.7%). Difference was not observed in the frequency of autoimmune disorders when the patients were compared according to their anti-AQP4 status. The results of the present study underscored that the NMO patients present high frequency of autoantibodies against cellular antigens and the presence of autoimmune disorders. Further studies with large number of NMO patients may contribute to advances in the understanding of NMO disease mechanisms.

Nuevas aplicaciones de las lentes de contacto

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Blanco Menéndez, Patricia

2017-01-01

Revisión bibliográfica de los estudios para el seguimiento y monitorización de patologías de elevada prevalencia (glaucoma y diabetes mellitus) de una forma no invasiva y administración de fármacos mediante el uso de lentes de contacto. Grado en Optica y Optometría

Clinical and imaging characteristics and autoantibody analysis of neuromyelitis optica spectrum disorders

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Nan LIU

2014-09-01

Full Text Available Objective To observe the clinical and imaging characteristics and the changes of autoimmune antibodies in the serum and cerebrospinal fluid (CSF of patients with neuromyelitis optica spectrum disorders (NMOSDs.  Methods The data of 10 patients with NMOSDs in Aviation General Hospital of China Medical University from January 2011 to June 2014 were collected. The clinical and imaging features were retrospectively reviewed, and NMO-IgG in serum and CSF, anti-nuclear antibody (ANA, homocysteine (Hcy and thyroid function were analyzed.  Results Cranial and spinal MRI of these patients showed that brain stem was involved in 3 cases, cervical cord in 3 cases, thoracic cord in 6 cases, and cervical-thoracic cord in one case. Serum NMO-IgG were tested in 8 cases, among whom 3 patients were positive (3/8 and 5 were negative (5/8. ANA was positive in one case (1/3, and thyroglobulin (TG antibody and thyroid peroxidase (TPO antibody were positive in 2 cases (2/3. Hypothyroidism occured in 2 cases, hyperthyroidism occured in one case, and Hcy rised in 2 cases.  Conclusions NMOSDs frequently occur in young and middle-aged women. Patients who were highly suspected with NMOSDs should receive tests of autoimmune antibodies in the serum and CSF, and cranial and spinal MRI examination, in order to make a definite diagnosis and receive appropriate treatment. Retesting the autoimmune antibodies should be done in catabasis, in order to identify the relationship between autoimmune antibodies and NMOSDs. doi: 10.3969/j.issn.1672-6731.2014.09.010

Imbalance in multiple sclerosis and neuromyelitis optica: association with deep sensation disturbance.

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Demura, Yutaka; Kinoshita, Masako; Fukuda, Osamu; Nose, Shouzou; Nakano, Hitoshi; Juzu, Akira; Murase, Nagako; Yamamoto, Kenji

2016-12-01

Abnormality in balance is one of the most important causes of gait disturbance which has a direct impact to disability and medical cost in multiple sclerosis (MS) and neuromyelitis optica (NMO). However, characteristics of imbalance in these two diseases have not been fully elucidated. The aim of this study was to evaluate the degree and features of imbalance using stabilography, the degree of deep sensation disturbance using tibial nerve somatosensory evoked potentials (SEP), and their association with clinical impairment, in patients with MS and NMO. Seven NMO patients and seven MS patients with balance disturbance were examined. The relationship among stabilography measurements representing the degree and features of imbalance, height-adjusted P38 peak latency of SEP, and neurological functional disability, were analyzed. Stabilography evaluation showed a significantly severer degree of imbalance in NMO than in MS. Romberg quotient of the patients with brainstem lesions was significantly larger than those without them. In all patients, length of excursion per second significantly correlated positively with anterio-posterior-axis power spectra at intermediate frequency band. In all patients and in NMO, P38 peak latency adjusted by height significantly correlated positively with anterio-posterior-axis power spectra at intermediate frequency band. These findings suggest that the degree of imbalance of MS and NMO possibly correlate with deep sensation disturbance, which could be evaluated by anterio-posterior-axis power spectra at intermediate frequency band by stabilography. Severer imbalance in NMO than MS may be associated with the severe longitudinally extensive spinal cord lesions.

Circulating regulatory B cell subsets in patients with neuromyelitis optica spectrum disorders.

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Han, Jinming; Sun, Li; Wang, Zhongkun; Fan, Xueli; Wang, Lifang; Song, Yang-Yang; Zhu, Jie; Jin, Tao

2017-07-01

This study analyzed the populations of three different subsets of regulatory B cells (Bregs) in the peripheral blood mononuclear cells (PBMCs) of patients with neuromyelitis optica spectrum disorders (NMOSDs) and explored the relationship between the changes in these subsets of Bregs and the severity of NMOSD. A total of 22 patients with relapsed NMOSDs before treatment were recruited in our study, along with 20 age and gender-matched healthy controls, from May 2015 to March 2016. The percentages and numbers for three different subsets of Bregs including the CD19 + CD24 hi CD38 hi , CD19 + CD24 hi CD27 + , and CD19 + CD5 + CD1d hi populations were evaluated in parallel by flow cytometry. Afterwards, correlations between the change of three different subsets of Bregs and disease severity were analyzed. We found significantly lower percentages of CD19 + CD24 hi CD38 hi and CD19 + CD5 + CD1d hi Bregs in NMOSDs patients than in healthy individuals. In contrast, the CD19 + CD24 hi CD27 + Bregs population was significantly higher in NMOSDs patients than in healthy individuals. However, the three different Bregs subsets showed no significant correlation with expanded disability status scale (EDSS) or annualized relapse rate (ARR). Our findings suggest that the subsets of Bregs may play complex roles in the pathogenesis of NMOSDs and are not correlated with clinical disease severity. Further insights into the potential role of subsets of Bregs could increase our basic knowledge of NMOSDs pathogenesis.

Assessment of Optic Nerve Impairment in Patients with Neuromyelitis Optica by MR Diffusion Tensor Imaging.

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Zhiye Chen

Full Text Available Diffusion tensor imaging (DTI has been used for the evaluation of the white matter integrity. In this study, we evaluated optic nerve impairment in patients with neuromyelitis optica (NMO using DTI.Optic nerve DTI were performed on 28 NMO patients and 38 normal controls. Fractional anisotropy (FA values were measured in the anterior, middle, and posterior parts of the intraorbital optic nerve segment. For the posterior intraorbital optic nerve, FA values of BI (0.20±0.07, MI (0.24±0.16, and NA (0.25±0.14 decreased significantly compared with that of NC (0.43±0.07 (P<0.05, and ROC analysis demonstrated that the area under the curve (AUC measurements for BI vs. NC, MI vs. NC, NA vs. NC, and NMO (including BI, MI, and NA vs. NC were 0.99, 0.93, 0.88, and 0.96, respectively. The corresponding diagnostic sensitivities of ROC analysis were 100%, 80%, 80%, and 91%; and the specificities were 93%, 97%, 91%, and 93%.Decreased FA value in the intraorbital optic nerve, especially in the posterior part of the nerve, was demonstrated as a characteristic MR feature for NMO-related optic nerve impairment.

Multifrequency magnetic resonance elastography of the brain reveals tissue degeneration in neuromyelitis optica spectrum disorder

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Streitberger, Kaspar-Josche; Fehlner, Andreas; Sack, Ingolf; Pache, Florence; Lacheta, Anna; Papazoglou, Sebastian; Brandt, Alexander; Bellmann-Strobl, Judith; Ruprecht, Klemens; Braun, Juergen; Paul, Friedemann; Wuerfel, Jens

2017-01-01

Application of multifrequency magnetic resonance elastography (MMRE) of the brain parenchyma in patients with neuromyelitis optica spectrum disorder (NMOSD) compared to age matched healthy controls (HC). 15 NMOSD patients and 17 age- and gender-matched HC were examined using MMRE. Two three-dimensional viscoelastic parameter maps, the magnitude G* and phase angle φ of the complex shear modulus were reconstructed by simultaneous inversion of full wave-field data in 1.9-mm isotropic resolution at 7 harmonic drive frequencies from 30 to 60 Hz. In NMOSD patients, a significant reduction of G* was observed within the white matter fraction (p = 0.017), predominantly within the thalamic regions (p = 0.003), compared to HC. These parameters exceeded the reduction in brain volume measured in patients versus HC (p = 0.02 whole-brain volume reduction). Volumetric differences in white matter fraction and the thalami were not detectable between patients and HC. However, phase angle φ was decreased in patients within the white matter (p = 0.03) and both thalamic regions (p = 0.044). MMRE reveals global tissue degeneration with accelerated softening of the brain parenchyma in patients with NMOSD. The predominant reduction of stiffness is found within the thalamic region and related white matter tracts, presumably reflecting Wallerian degeneration. (orig.)

Multifrequency magnetic resonance elastography of the brain reveals tissue degeneration in neuromyelitis optica spectrum disorder

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Streitberger, Kaspar-Josche [Charite - Universitaetsmedizin Berlin, Department of Radiology, Berlin (Germany); Charite - Universitaetsmedizin Berlin, Department of Neurology with Experimental Neurology, Berlin (Germany); Fehlner, Andreas; Sack, Ingolf [Charite - Universitaetsmedizin Berlin, Department of Radiology, Berlin (Germany); Pache, Florence [Charite - Universitaetsmedizin Berlin, Department of Neurology with Experimental Neurology, Berlin (Germany); Charite - Universitaetsmedizin Berlin, NeuroCure Clinical Research Center, Berlin (Germany); Lacheta, Anna; Papazoglou, Sebastian; Brandt, Alexander [Charite - Universitaetsmedizin Berlin, NeuroCure Clinical Research Center, Berlin (Germany); Bellmann-Strobl, Judith [Max Delbrueck Center for Molecular Medicine and Charite - Universitaetsmedizin Berlin, Experimental and Clinical Research Center, Berlin (Germany); Ruprecht, Klemens [Charite - Universitaetsmedizin Berlin, Department of Neurology with Experimental Neurology, Berlin (Germany); Braun, Juergen [Charite - Universitaetsmedizin Berlin, Institute of Medical Informatics, Berlin (Germany); Paul, Friedemann [Charite - Universitaetsmedizin Berlin, Department of Neurology with Experimental Neurology, Berlin (Germany); Charite - Universitaetsmedizin Berlin, NeuroCure Clinical Research Center, Berlin (Germany); Max Delbrueck Center for Molecular Medicine and Charite - Universitaetsmedizin Berlin, Experimental and Clinical Research Center, Berlin (Germany); Wuerfel, Jens [Charite - Universitaetsmedizin Berlin, NeuroCure Clinical Research Center, Berlin (Germany); Max Delbrueck Center for Molecular Medicine and Charite - Universitaetsmedizin Berlin, Experimental and Clinical Research Center, Berlin (Germany); Medical Image Analysis Center (MIAC AG), Basel (Switzerland)

2017-05-15

Application of multifrequency magnetic resonance elastography (MMRE) of the brain parenchyma in patients with neuromyelitis optica spectrum disorder (NMOSD) compared to age matched healthy controls (HC). 15 NMOSD patients and 17 age- and gender-matched HC were examined using MMRE. Two three-dimensional viscoelastic parameter maps, the magnitude G* and phase angle φ of the complex shear modulus were reconstructed by simultaneous inversion of full wave-field data in 1.9-mm isotropic resolution at 7 harmonic drive frequencies from 30 to 60 Hz. In NMOSD patients, a significant reduction of G* was observed within the white matter fraction (p = 0.017), predominantly within the thalamic regions (p = 0.003), compared to HC. These parameters exceeded the reduction in brain volume measured in patients versus HC (p = 0.02 whole-brain volume reduction). Volumetric differences in white matter fraction and the thalami were not detectable between patients and HC. However, phase angle φ was decreased in patients within the white matter (p = 0.03) and both thalamic regions (p = 0.044). MMRE reveals global tissue degeneration with accelerated softening of the brain parenchyma in patients with NMOSD. The predominant reduction of stiffness is found within the thalamic region and related white matter tracts, presumably reflecting Wallerian degeneration. (orig.)

Neuromyelitis optica and the evolving spectrum of autoimmune aquaporin-4 channelopathies: a decade later

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Pittock, Sean J.; Lucchinetti, Claudia F.

2015-01-01

The discovery of AQP4-IgG (a pathogenic antibody that targets the astrocytic water channel aquaporin-4) as the first sensitive and specific biomarker for any inflammatory central nervous system demyelinating disease, has shifted emphasis from the oligodendrocyte and myelin to the astrocyte as a central immunopathogenic player. Neuromyelitis optica (NMO) spectrum disorders (SD) represent an evolving spectrum of IDDs extending beyond the optic nerves and spinal cord to include the brain (especially in children) and, rarely, muscle. NMOSD typical brain lesions are located in areas that highly express the target antigen, AQP4, including the circumventricular organs (accounting for intractable nausea and vomiting) and the diencephalon (accounting for sleep disorders, endocrinopathies, and syndrome of inappropriate antidiuresis). Magnetic resonance imaging (MRI) brain abnormalities fulfill Barkoff criteria for multiple sclerosis in up to 10% of patients. As the spectrum broadens, the importance of highly specific assays that detect pathogenic AQP4-IgG targeting extracellular epitopes of AQP4 cannot be overemphasized. The rapid evolution of our understanding of the immunobiology of AQP4 autoimmunity necessitates continuing revision of NMOSD diagnostic criteria. Here, we describe scientific advances that have occurred since the discovery of NMO-IgG in 2004 and review novel targeted immunotherapies. We also suggest that NMOSDs should now be considered under the umbrella term autoimmune aquaporin-4 channelopathy. PMID:26096370

Lesions of the posterior limb of the internal capsule in neuromyelitis optica spectrum disorder.

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Long, Youming; Wu, Linzhan; Zhong, Rong; Ouyang, Xiaoming; Liang, Junyan; Gao, Cong; Chen, Xiaohui; Qiu, Wei; Chang, Yanyu; Wang, Zhanhang; Ye, Jinlong

2017-05-01

Posterior limb of the internal capsule lesions (PLICL) are one of the MRI features of neuromyelitis optica spectrum disorder (NMOSD). However, there is no evidence that such lesions are pathogenically related to NMOSD. We retrospectively analyzed features of PLICL in NMOSD, and other central nervous system inflammatory disorders, in 561 patients. We also examined the pathological samples of six patients. Of the 561 patients investigated, PLICL were found in 65 patients (11.6%). Lesions were bilateral in 26 cases (40%) and unilateral in 39 cases (60%). Unilateral lesions were mainly located on the left side (74.3%, 29/39). Of the 65 patients with PLICL, 46 patients had NMOSD (70.8%) and were positive for anti-aquaporin (AQP4-IgG), four had NMOSD (6.2%) and were AQP4-IgG negative, 10 patients had multiple sclerosis (MS), three patients had NMDAR encephalitis, and two had autoimmune meningoencephalitis. Of the six patients whose pathological samples were evaluated, all had PLICL and were negative for AQP4-IgG, and none had pathological NMOSD lesion features. These cases included three patients with multiple sclerosis, one with anti-N-methyl-D-aspartate receptor encephalitis, and two with autoimmune meningoencephalitis. In conclusion, PLICL are found not only in patients with NMOSD, but also in MS and other disorders.

Neuromyelitis optica spectrum disorders: long-term safety and efficacy of rituximab in Caucasian patients.

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Radaelli, M; Moiola, L; Sangalli, F; Esposito, F; Barcella, V; Ferrè, L; Rodegher, M; Colombo, B; Fazio, R; Martinelli, V; Comi, G

2016-04-01

To assess the long-term benefit-risk profile of repeated courses of rituximab in Caucasian patients affected by neuromyelitis optica (NMO) and related disorders, in everyday clinical practice. This is a prospective observational study performed at San Raffaele Hospital, Milan, Italy. From February 2006, we recruited 21 patients affected by NMO and NMO spectrum of disorders (NMOSD) whom underwent at least one cycle of intravenous (i.v.) rituximab and then were followed for at least 2 years. At a mean follow-up time of 48 months, we observed a significant reduction of the annualized relapse rate (ARR), from 2.0 to 0.16 (p < 0.01); and of the median Expanded Disability Status Scale (EDSS), from 5.5 to 4.0 (p < 0.013). There were 12 patients (57%) who remained disease free during the follow-up period. Five patients (24%) reported mild hematological adverse events. Serious infectious adverse events were reported by another four patients: These were all wheelchair bound at the beginning of their rituximab treatment. A fixed treatment scheme of rituximab, with re-treatment every 6 months, was efficacious for NMO and NMOSD, with a good safety profile; however, to obtain an even better benefit-risk ratio, close monitoring of CD19(+) B cells should be performed before the re-treatment of patients with high-level disability, concomitant leukopenia and hypogammaglobulinemia. © The Author(s), 2015.

MRI abnormalities and related risk factors of the brain in patients with neuromyelitis optica

International Nuclear Information System (INIS)

Xiao Hui; Ma Lin; Lou Xin; Cai Youquan; Wang Yulin; Wang Yan; Wu Lei; Wu Weiping

2011-01-01

Objective: To investigate the MRI features of the brain in patients with neuromyelitis optica (NMO), and to evaluate the correlation between the brain abnormalities and related risk factors. Methods: Fifty-four patients with definite NMO according to 2006 Wingerchuk diagnosis criteria were enrolled in this study. MRI scanning of the brain was performed in these patients. Distribution and signal features of all the lesions were analyzed. A Logistic regression analysis was used to evaluate the risk factors of brain abnormalities. Results: Twenty-four NMO patients (44.4%) showed unremarkable findings and thirty (55.6%) showed abnormalities on brain MRI. Multiple and non-specific small lesions in the subcortical white matter and grey-white matter junction were the most frequent abnormalities on brain MRI (13/30, 43.3%). Typical lesion locations included corpus callosum, subependyma of ventricles, hypothalamus and brain stem. The lesions showed punctate, patchy and linear abnormal signals. Post-contrast MRI showed no abnormal enhancement in 16 cases. Logistic regression analysis showed that coexisting autoimmune disease or infection. history had correlations with abnormalities of the brain on MRI (OR=3.519, P<0.05). Conclusions: There was a high incidence of brain abnormalities in NMO. Subependymal white matter, corpus callosum, hypothalamus and brain stem were often involved in NMO. NMO patients with coexisting autoimmune disease and infection history had higher risk of brain abnormalities. (authors)

Linear lesions may assist early diagnosis of neuromyelitis optica and longitudinally extensive transverse myelitis, two subtypes of NMOSD.

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Cai, Wei; Tan, Sha; Zhang, Lei; Shan, Yilong; Wang, Yanqiang; Lin, Yinyao; Zhou, Fangjing; Zhang, Bingjun; Chen, Xiaoyu; Zhou, Li; Wang, Yuge; Huang, Xuehong; Men, Xuejiao; Li, Haiyan; Qiu, Wei; Hu, Xueqiang; Lu, Zhengqi

2016-01-15

To investigate the relationship between linear lesions (LL) and the development of longitudinally extensive spinal cord lesions (LESCL) in Chinese patients with neuromyelitis optica or longitudinally extensive transverse myelitis. The clinical records of 143 patients with these conditions were reviewed. Forty-one patients with LL were divided into three groups according to the order of appearance of LL and LESCL (simultaneously [n=10], LL first [n=26], or LESCL first [n=5]). The remaining 102 patients without LL were used as a control group. Patients who developed LL first demonstrated a lower annualized relapse rate than those in the simultaneous group (1.00 [0.23-10.00] vs. 4.38 [0.60-6.67], p=0.017) and the control group (1.00 [0.23-10.00] vs. 2.00 [0.24-10.00], p=0.007). Among all patients with LL, there were significantly more who developed them before LESCL than those who developed them after LESCL (poptica and longitudinally extensive transverse myelitis. Copyright © 2015 Elsevier B.V. All rights reserved.

The Pathology of an Autoimmune Astrocytopathy: Lessons Learned from Neuromyelitis Optica

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Lucchinetti, Claudia F.; Guo, Yong; Popescu, Bogdan F. Gh.; Fujihara, Kazuo; Itoyama, Yasuto; Misu, Tatsuro

2014-01-01

Neuromyelitis optica (NMO) is a disabling autoimmune astrocytopathy characterized by typically severe and recurrent attacks of optic neuritis and longitudinally-extensive myelitis. Until recently, NMO was considered an acute aggressive variant of multiple sclerosis (MS), despite the fact that early studies postulated that NMO and MS may be two distinct diseases with a common clinical picture. With the discovery of a highly specific serum autoantibody (NMO-IgG), Lennon and colleagues provided the first unequivocal evidence distinguishing NMO from MS and other CNS inflammatory demyelinating disorders. The target antigen of NMO-IgG was confirmed to be aquaporin-4 (AQP4), the most abundant water channel protein in the central nervous system (CNS), mainly expressed on astrocytic foot processes at the blood brain barrier, subpial and subependymal regions. Pathological studies demonstrated that astrocytes were selectively targeted in NMO as evidenced by the extensive loss of immunoreactivities for the astrocytic proteins, AQP4 and glial fibrillary acidic protein (GFAP), as well as perivascular deposition of immunoglobulins and activation of complement even within lesions with a relative preservation of myelin. In support of these pathological findings, GFAP levels in the cerebrospinal fluid (CSF) during acute NMO exacerbations were found to be remarkably elevated in contrast to MS where CSF-GFAP levels did not substantially differ from controls. Additionally, recent experimental studies showed that AQP4 antibody is pathogenic, resulting in selective astrocyte destruction and dysfunction in vitro, ex vivo, and in vivo. These findings strongly suggest that NMO is an autoimmune astrocytopathy where damage to astrocytes exceeds both myelin and neuronal damage. This chapter will review recent neuropathological studies that have provided novel insights into the pathogenic mechanisms, cellular targets, as well as the spectrum of tissue damage in NMO. PMID:24345222

Anti-thyroid antibodies and thyroid function in neuromyelitis optica spectrum disorders.

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Wang, Xuan; Yi, Huan; Liu, Jia; Li, Min; Mao, Zhi-Feng; Xu, Li; Peng, Fu-Hua

2016-07-15

Neuromyelitis optica spectrum disorders (NMOSD) are complicated neuroautoimmune disorders which can coexist with other organ-specific autoimmune disorders. The most frequently specific organ is the thyroid. The aim of this study is to evaluate the thyroid function of NMOSD patients and detect the difference between anti-thyroid antibodies (ATAbs) seropositive and seronegative NMOSD patients. 88 patients diagnosed with NMOSD were enrolled and their thyroid functions were evaluated. They were divided into two groups by ATAbs abnormalities. In addition, demographic characteristics, clinical symptoms and MRI scan results of brain and spinal cord were assessed. Anti-thyroid peroxidase antibodies (TPOAbs) and anti-thyroglobulin antibodies (TGAbs) seropositivities were detected more frequently in patients with NMOSDs when compared with healthy controls (37.5% vs 14.9%, P=0.01, Diff22.6%, 95CI[9.0%, 34.9%]; 31.8% vs 16.2%, P=0.022, Diff15.6%, 95CI[2.27%, 27.9%]). In NMOSD patients, the Expanded disability status scale score (EDSS) score was significantly higher in ATAbs seropositive group than that in ATAbs seronegative group (median 6.5 vs 3.75, P=0.012). However, there is no significant difference for demographic characteristics and other clinical symptoms. Moreover, NMOSD patients with ATAbs abnormalities had more brain and cervical cord lesions when compared with ATAbs negative NMOSD patients (83.8% vs 61.4%, P=0.029, Diff22.4%, 95CI[0.9%, 40.9%]; 93.9% vs 59.6%, P=0.001, Diff34.3%, 95CI[13.6%, 50.4%]). NMOSD patients have a higher frequency of ATAbs abnormalities. ATAbs may be associated with disability status, brain abnormalities and cervical cord lesions. Copyright © 2016 Elsevier B.V. All rights reserved.

Efficacy and safety of rituximab in neuromyelitis optica: Review of evidence

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Masoud Etemadifar

2017-01-01

Full Text Available Neuromyelitis optica (NMO is an autoimmune inflammatory disease of the central nervous system with preferential involvement in the optic nerve and spinal cord with a widespread spectrum of clinical features; multiple therapeutic agents have been used with different results. Recent evidence points to B-cell-mediated humoral immunity in the pathogenesis of NMO. Rituximab targets the CD20 antigen on B-cells. Treatment leads to profound B-cell depletion, principally over an antibody-dependent cell cytotoxicity mechanism. The aim of our study was to review clinical trials to elucidate the impact of rituximab on the relapse rate, Expanded Disability Status Scale (EDSS, and progression of disability in NMO. We performed a comprehensive review of all studies that evaluated clinical and paraclinical effects of rituximab on NMO. MEDLINE-PubMed, Web of Sciences, EMBASE, and Cochrane databases up to June 2016 included in our searches. In addition, reference lists from articles identified by search as well as a key review article to identify additional articles included in the study. Rituximab targets the CD20 antigen on B-cells and decreases attack frequency and severity in patients with NMO; however, it does not remove attacks, even when modifying treatment to achieve B-cell depletion. Most of the investigations revealed that EDSS significantly in all patients with rituximab treatment will be decreased after treatment with rituximab. No new or enlarged lesions or pathological gadolinium enhancement was observed in serial brain and spinal cord magnetic resonance imaging, except for those observed concomitantly with clinical relapses and the median length of spinal cord lesions was significantly reduced after therapy. Rituximab targets the CD20 antigen and decreases attack frequency and severity in patients with NMO.

Differentiating neuromyelitis optica from other causes of longitudinally extensive transverse myelitis on spinal magnetic resonance imaging

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Pekcevik, Yeliz; Mitchell, Charles H; Mealy, Maureen A; Orman, Gunes; Lee, In H; Newsome, Scott D; Thompson, Carol B; Pardo, Carlos A; Calabresi, Peter A; Levy, Michael; Izbudak, Izlem

2016-01-01

Background Although spinal magnetic resonance imaging (MRI) findings of neuromyelitis optica (NMO) have been described, there is limited data available that help differentiate NMO from other causes of longitudinally extensive transverse myelitis (LETM). Objective To investigate the spinal MRI findings of LETM that help differentiate NMO at the acute stage from multiple sclerosis (MS) and other causes of LETM. Methods We enrolled 94 patients with LETM into our study. Bright spotty lesions (BSL), the lesion distribution and location were evaluated on axial T2-weighted images. Brainstem extension, cord expansion, T1 darkness and lesion enhancement were noted. We also reviewed the brain MRI of the patients during LETM. Results Patients with NMO had a greater amount of BSL and T1 dark lesions (p < 0.001 and 0.003, respectively). The lesions in NMO patients were more likely to involve greater than one-half of the spinal cord’s cross-sectional area; to enhance and be centrally-located, or both centrally- and peripherally-located in the cord. Of the 62 available brain MRIs, 14 of the 27 whom were NMO patients had findings that may be specific to NMO. Conclusions Certain spinal cord MRI features are more commonly seen in NMO patients and so obtaining brain MRI during LETM may support diagnosis. PMID:26209588

Spinal cord lesions in patients with neuromyelitis optica: a retrospective long-term MRI follow-up study

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Krampla, Wolfgang; Hruby, Walter [SMZ-Ost Donauspital, Department of Radiology, Vienna (Austria); Aboul-Enein, Fahmy; Jecel, Julia; Kristoferitsch, Wolfgang [SMZ-Ost Donauspital, Department of Neurology, Vienna (Austria); Lang, Wilfried [Hospital of Barmherzige Brueder, Department of Neurology, Vienna (Austria); Fertl, Elisabeth [Krankenanstalt Rudolfstiftung, Department of Neurology, Vienna (Austria)

2009-10-15

Neuromyelitis optica (NMO) is characterised by a particular pattern of the optic nerves and the spinal cord. Long-term MRI follow-up studies of spinal NMO lesions are rare, or limited by short observation periods. In nine patients with definite NMO or recurrent longitudinally extensive transverse myelitis (LETM) with NMO-IgG serum antibodies, repeated MRI examinations of the spine were carried out over a period of up to 11 years and evaluated regarding the changes over time in this retrospective study. In eight patients spinal cord lesions were located centrally, involving the grey and white matter. In the first examination after clinical onset changes resembled a stroke of the anterior spinal artery in two patients. Symmetrical signal alterations within the grey matter were observed. In one patient this pattern was transient, but it remained in the other. During the chronic stage, either a variable degree of spinal cord atrophy and high signal alterations, or almost complete remission of the lesions, was observed. Spinal MRI of patients with NMO myelitis can resemble a stroke. MRI of acute NMO stages did not allow a prediction of the clinical outcome. To a variable degree, NMO left behind typical defects which correlated with the clinical outcome. (orig.)

Spinal cord lesions in patients with neuromyelitis optica: a retrospective long-term MRI follow-up study

International Nuclear Information System (INIS)

Krampla, Wolfgang; Hruby, Walter; Aboul-Enein, Fahmy; Jecel, Julia; Kristoferitsch, Wolfgang; Lang, Wilfried; Fertl, Elisabeth

2009-01-01

Neuromyelitis optica (NMO) is characterised by a particular pattern of the optic nerves and the spinal cord. Long-term MRI follow-up studies of spinal NMO lesions are rare, or limited by short observation periods. In nine patients with definite NMO or recurrent longitudinally extensive transverse myelitis (LETM) with NMO-IgG serum antibodies, repeated MRI examinations of the spine were carried out over a period of up to 11 years and evaluated regarding the changes over time in this retrospective study. In eight patients spinal cord lesions were located centrally, involving the grey and white matter. In the first examination after clinical onset changes resembled a stroke of the anterior spinal artery in two patients. Symmetrical signal alterations within the grey matter were observed. In one patient this pattern was transient, but it remained in the other. During the chronic stage, either a variable degree of spinal cord atrophy and high signal alterations, or almost complete remission of the lesions, was observed. Spinal MRI of patients with NMO myelitis can resemble a stroke. MRI of acute NMO stages did not allow a prediction of the clinical outcome. To a variable degree, NMO left behind typical defects which correlated with the clinical outcome. (orig.)

Anti-N-methyl-D-aspartate receptor(NMDAR) antibody encephalitis presents in atypical types and coexists with neuromyelitis optica spectrum disorder or neurosyphilis.

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Qin, Kaiyu; Wu, Wenqing; Huang, Yuming; Xu, Dongmei; Zhang, Lei; Zheng, Bowen; Jiang, Meijuan; Kou, Cheng; Gao, Junhua; Li, Wurong; Zhang, Jinglin; Wang, Sumei; Luan, Yanfei; Yan, Chaoling; Xu, Dan; Zheng, Xinmei

2017-01-05

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. A 33-year-old man presented with dysarthria, movement disorder and occasional seizures. He had 6 relapses in 28 years. When suffered from upper respiratory tract syndrome, he developed behavioral and consciousness impairment. Cranial MRI was normal. Viral PCR studies and oncologic work-up were negative. Anti-NMDAR antibody was detected in CSF and serum. A 21-year-old female manifested dizziness and diplopia ten months and six months before, respectively. Both responded to steroid therapy and improved completely. This time she presented with progressive left limb and facial anesthesia, walking and holding unsteadily. Spinal cord MRI follow-up showed abnormality of medulla oblongata and cervical cord(C1). Anti-AQP4 and anti-NMDAR were positive in CSF. Steroid-pulse therapy ameliorated her symptoms. A 37-year-old male experienced worsening vision. He was confirmed neurosyphilis since the CSF tests for syphilis were positive. Protein was elevated and the oligoclonal IgG bands(OB) and anti-NMDAR was positive in CSF. Anti-aquaporin 4(AQP4) antibodies and NMO-IgG were negative. Cranial MRI showed high FLAIR signal on frontal lobe and low T2 signal adjacent to the right cornu posterious ventriculi lateralis. Treatment for neurosyphlis was commenced with gradual improvement. A 39-year-old male, developed serious behavioral and psychiatric symptoms. Examination showed abnormal pupils and unsteady gait. He was confirmed neurosyphilis according to the CSF tests for syphilis. Anti-NMDAR was positive in CSF and serum

Anti-Aquaporin-4 Antibody-Positive Neuromyelitis Optica Presenting with Syndrome of Inappropriate Antidiuretic Hormone Secretion as an Initial Manifestation

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H. Nakajima

2011-10-01

Full Text Available The distribution of neuromyelitis optica (NMO-characteristic brain lesions corresponds to sites of high aquaporin-4 (AQP4 expression, and the brainstem and hypothalamus lesions that express high levels of AQP4 protein are relatively characteristic of NMO. The syndrome of inappropriate antidiuretic hormone secretion (SIADH is one of the important causes of hyponatremia and results from an abnormal production or sustained secretion of antidiuretic hormone (ADH. SIADH has been associated with many clinical states or syndromes, and the hypothalamic-neurohypophyseal system regulates the feedback control system for ADH secretion. We report the case of a 63-year-old man with NMO, whose initial manifestation was hyponatremia caused by SIADH. Retrospective analysis revealed that the serum anti-AQP4 antibody was positive, and an MRI scan showed a unilateral lesion in the hypothalamus. SIADH recovered completely with regression of the hypothalamic lesion. As such, NMO should even be considered in patients who develop SIADH and have no optic nerve or spinal cord lesions but have MRI-documented hypothalamic lesions.

A voxel-based morphometry study of brain volume changes in patients with neuromyelitis optica

International Nuclear Information System (INIS)

Duan Yunyun; Liu Yaou; Liang Peipeng; Huang Jing; Ren Zhuoqiong; Ye Jing; Dong Huiqing; Chen Hai; Li Kuncheng

2012-01-01

Objective: To detect changes of regional grey matter and white matter volume in patients of neuromyelitis optica (NMO) by voxel-based morphometry (VBM), and investigate its relationship with clinical variables. Methods: Conventional magnetic resonance imaging (MRI) and structural three-dimensional MRI were obtained from 20 NMO and 20 sex-and age-matched healthy volunteers. The comparison of grey matter and white matter volume between the two groups was analyzed by VBM tools of statistical parametric mapping (SPM) 5. Pearson correlation analysis was used to assess correlations between regional volume decrease and disease duration and expanded disability status scale (EDSS) scores in NMO patients. Results: Compared with normal controls, NMO patients had grey matter atrophy in several cortical regions, such as right inferior frontal gyrus (cluster size 514), left superior temporal gyrus (282), right middle temporal gyrus (229) and right insula (211) (t=3.58-5.11, AlphaSim corrected, P<0.05). White matter atrophy was found in several subcortical regions in NMO patients, such as right precentral and postcentral gyrus (cluster size 457, 110), left middle frontal gyrus (285), and right inferior parietal lobule (231) (t=2.90-4.25, AlphaSim corrected, P<0.05). Grey matter and white matter volume loss were not significantly correlated with clinical duration or EDSS score in NMO. Conclusion: By means of VBM, regional atrophy of grey matter and white matter is found in NMO patients, which may provide evidence for brain structural abnormality in NMO. (authors)

Variants of Interferon Regulatory Factor 5 are Associated with Neither Neuromyelitis Optica Nor Multiple Sclerosis in the Southeastern Han Chinese Population

Institute of Scientific and Technical Information of China (English)

Qi-Bing Liu; Lei Wu; Gui-Xian Zhao; Ping-Ping Cai; Zhen-Xin Li; Zhi-Ying Wu

2015-01-01

Background:Neuromyelitis optica (NMO) and multiple sclerosis (MS) are demyelinating disorders of the central nervous system.Interferon regulatory factor 5 (IRF5) is a common susceptibility gene to different autoimmune disorders.However,the association of IRF5 variants with NMO and MS patients has not been well studied.Therefore,we aimed to evaluate whether IRF5 variants were associated with NMO and MS in the Southeastern Han Chinese population.Methods:Four single nucleotide polymorphisms (SNPs) were selected and genotyped by matrix-assisted laser desorption/ionization time of flight mass spectrometry in 111 NMO patients,145 MS patients and 300 controls from Southeastern China.Results:None of these 4 SNPs was associated with NMO or MS patients.Conclusions:Our preliminary study indicates that genetic variants in IRF5 may affect neither NMO nor MS in the Southeastern Han Chinese population.Further studies with a large sample size and diverse ancestry populations are needed to clarify this issue.

Frequency of brain MRI abnormalities in neuromyelitis optica spectrum disorder at presentation: A cohort of Latin American patients.

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Carnero Contentti, Edgar; Daccach Marques, Vanessa; Soto de Castillo, Ibis; Tkachuk, Veronica; Antunes Barreira, Amilton; Armas, Elizabeth; Chiganer, Edson; de Aquino Cruz, Camila; Di Pace, José Luis; Hryb, Javier Pablo; Lavigne Moreira, Carolina; Lessa, Carmen; Molina, Omaira; Perassolo, Monica; Soto, Arnoldo; Caride, Alejandro

2018-01-01

Brain magnetic resonance imaging (BMRI) lesions were classically not reported in neuromyelitis optica (NMO). However, BMRI lesions are not uncommon in NMO spectrum disorder (NMOSD) patients. To report BMRI characteristic abnormalities (location and configuration) in NMOSD patients at presentation. Medical records and BMRI characteristics of 79 patients with NMOSD (during the first documented attack) in Argentina, Brazil and Venezuela were reviewed retrospectively. BMRI abnormalities were observed in 81.02% of NMOSD patients at presentation. Forty-two patients (53.1%) showed typical-NMOSD abnormalities. We found BMRI abnormalities at presentation in the brainstem/cerebellum (n = 26; 32.9%), optic chiasm (n = 16; 20.2%), area postrema (n = 13; 16.4%), thalamus/hypothalamus (n = 11; 13.9%), corpus callosum (n = 11; 13.9%), periependymal-third ventricle (n = 9; 11.3%), corticospinal tract (n = 7; 8.8%), hemispheric white matter (n = 1; 1.2%) and nonspecific areas (n = 49; 62.03%). Asymptomatic BMRI lesions were more common. The frequency of brain MRI abnormalities did not differ between patients who were positive and negative for aquaporin 4 antibodies at presentation. Typical brain MRI abnormalities are frequent in NMOSD at disease onset. Copyright © 2017 Elsevier B.V. All rights reserved.

Early and extensive spinal white matter involvement in neuromyelitis optica.

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Hayashida, Shotaro; Masaki, Katsuhisa; Yonekawa, Tomomi; Suzuki, Satoshi O; Hiwatashi, Akio; Matsushita, Takuya; Watanabe, Mitsuru; Yamasaki, Ryo; Suenaga, Toshihiko; Iwaki, Toru; Murai, Hiroyuki; Kira, Jun-Ichi

2017-05-01

Studies of longitudinally extensive spinal cord lesions (LESCLs) in neuromyelitis optica (NMO) have focused on gray matter, where the relevant antigen, aquaporin-4 (AQP4), is abundant. Because spinal white matter pathology in NMO is not well characterized, we aimed to clarify spinal white matter pathology of LESCLs in NMO. We analyzed 50 spinal cord lesions from eleven autopsied NMO/NMO spectrum disorder (NMOSD) cases. We also evaluated LESCLs with three or fewer spinal cord attacks by 3-tesla MRI in 15 AQP4 antibody-positive NMO/NMOSD patients and in 15 AQP4 antibody-negative multiple sclerosis (MS) patients. Pathological analysis revealed seven cases of AQP4 loss and four predominantly demyelinating cases. Forty-four lesions from AQP4 loss cases involved significantly more frequently posterior columns (PC) and lateral columns (LC) than anterior columns (AC) (59.1%, 63.6%, and 34.1%, respectively). The posterior horn (PH), central portion (CP), and anterior horn (AH) were similarly affected (38.6%, 36.4% and 31.8%, respectively). Isolated perivascular inflammatory lesions with selective loss of astrocyte endfoot proteins, AQP4 and connexin 43, were present only in white matter and were more frequent in PC and LC than in AC (22.7%, 29.5% and 2.3%, P corr  = 0.020, and P corr  = 0.004, respectively). MRI indicated LESCLs more frequently affected PC and LC than AC in anti-AQP4 antibody-seropositive NMO/NMOSD (86.7%, 60.0% and 20.0%, P corr  = 0.005, and P corr  = 0.043, respectively) and AQP4 antibody-seronegative MS patients (86.7%, 73.3% and 33.3%, P corr  = 0.063, and P corr  = 0.043, respectively). PH, CP and AH were involved in 93.3%, 86.7% and 73.3% of seropositive patients, respectively, and in 53.3%, 60.0% and 40.0% of seronegative patients, respectively. NMO frequently and extensively affects spinal white matter in addition to central gray matter, especially in PC and LC, where isolated perivascular lesions with astrocyte endfoot

Ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorders.

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Zalewski, Nicholas L; Morris, Padraig P; Weinshenker, Brian G; Lucchinetti, Claudia F; Guo, Yong; Pittock, Sean J; Krecke, Karl N; Kaufmann, Timothy J; Wingerchuk, Dean M; Kumar, Neeraj; Flanagan, Eoin P

2017-03-01

We assessed the frequency and characteristics of ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorder (NMOSD) myelitis and myelitis of other cause. We reviewed spinal cord MRIs for ring-enhancing lesions from 284 aquaporin-4 (AQP4)-IgG seropositive patients at Mayo Clinic from 1996 to 2014. Inclusion criteria were as follows: (1) AQP4-IgG seropositivity, (2) myelitis attack and (3) MRI spinal cord demonstrating ring-enhancement. We identified two groups of control patients with: (1) longitudinally extensive myelopathy of other cause (n=66) and (2) myelitis in the context of a concurrent or subsequent diagnosis of multiple sclerosis (MS) from a population-based cohort (n=30). Ring-enhancement was detected in 50 of 156 (32%) myelitis episodes in 41 patients (83% single; 17% multiple attacks). Ring-enhancement was noted on sagittal and axial images in 36 of 43 (84%) ring enhancing myelitis episodes and extended a median of two vertebral segments (range, 1-12); in 21 of 48 (44%) ring enhancing myelitis episodes, the ring extended greater than or equal to three vertebrae. Ring-enhancement was accompanied by longitudinally extensive (greater than or equal to three vertebral segments) T2-hyperintensity in 44 of 50 (88%) ring enhancing myelitis episodes. One case of a spinal cord biopsy during ring-enhancing myelitis revealed tissue vacuolation and loss of AQP4 immunoreactivity with preserved axons. The clinical characteristics of ring-enhancing myelitis episodes did not differ from non-ring-enhancing episodes. Ring-enhancing spinal cord lesions were more common in NMOSD than other causes of longitudinally extensive myelopathy (50/156 (32%) vs 0/66 (0%); p≤0.001) but did not differ between NMOSD and MS (50/156 (32%) vs 6/30 (20%); p=0.20). Spinal cord ring-enhancement accompanies one-third of NMOSD myelitis episodes and distinguishes NMOSD from other causes of longitudinally extensive myelopathies but not from MS. Published by the BMJ Publishing

Application of diffusional kurtosis imaging to detect occult brain damage in multiple sclerosis and neuromyelitis optica.

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Qian, Wenshu; Chan, Koon Ho; Hui, Edward S; Lee, Chi Yan; Hu, Yong; Mak, Henry Ka-Fung

2016-11-01

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. Diffusional kurtosis imaging was employed to discriminate NMO and MS patients at the early or stable stage from each other, and from healthy volunteers. The presence of alterations in diffusion and diffusional kurtosis metrics in normal-appearing white matter (NAWM) and diffusely increased mean diffusivity (MD) in the cortical normal-appearing gray matter (NAGM) favors the diagnosis of MS rather than NMO. Meanwhile, normal diffusivities and kurtosis metrics in all NAWM as well as increases in MD in the frontal and temporal NAGM suggest NMO. Our results suggest that diffusion and diffusional kurtosis metrics may well aid in discriminating the two diseases. Copyright © 2016 John Wiley & Sons, Ltd.

Painful tonic spasm in neuromyelitis optica spectrum disorders: Prevalence, clinical implications and treatment options.

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Liu, Ju; Zhang, Qin; Lian, Zhiyun; Chen, Hongxi; Shi, Ziyan; Feng, Huiru; Miao, Xiaohui; Du, Qin; Zhou, Hongyu

2017-10-01

Painful tonic spasm (PTS) is a common symptom in patients with neuromyelitis optica spectrum disorders (NMOSD). This study aimed to obtain further insights into the prevalence, characteristics, and treatment of PTS in patients with NMOSD, and to systematically investigate and compare the clinical features and prognosis of NMOSD with and without PTS. We reviewed the medical records and prospectively interviewed patients with NMOSD who attended the West China Hospital of Sichuan University in Chengdu, China between September 2014 and December 2016. In total, 52 of the 230 patients with NMOSD experienced PTS (22.61%). Patients with NMOSD and PTS were characterized by a higher age at onset (P = 0.017), higher annual relapse rate (ARR) (P = 0.003), higher ARR of myelitis (P = 0.011), and a tendency to experience pruritus (P = 0.025). Sodium channel blocking antiepileptic drugs (carbamazepine or oxcarbazepine) had higher efficacy than gabapentin in the treatment of PTS (P = 0.001). Although the progression index was higher in patients with PTS, this difference did not reach statistical significance (P = 0.05). Our study suggested that immunosuppressors for the prevention of relapse should be administered without delay in patients with NMOSD and PTS. Owing to the side effects of carbamazepine, we recommend oxcarbazepine as the first-line of treatment for PTS in patients with NMOSD. Whether PTS is a marker of disease severity in NMOSD remains to be determined, requiring a long-term prospective observational study. Copyright © 2017 Elsevier B.V. All rights reserved.

Comparison of spontaneous brain activity revealed by regional homogeneity in AQP4-IgG neuromyelitis optica-optic neuritis versus MOG-IgG optic neuritis patients: a resting-state functional MRI study

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Wang J

2017-10-01

Full Text Available Junqing Wang,1,* Yuan Tian,2,* Yi Shao,3,* Hui Feng,1 Limin Qin,1 Weiwei Xu,1 Hongjuan Liu,1 Quangang Xu,1 Shihui Wei,1 Lin Ma2 1Department of Ophthalmology, 2Department of Radiology, Chinese PLA General Hospital, Beijing, 3Department of Ophthalmology, the First Affiliated Hospital of Nanchang University, Nanchang, Jiangxi, People’s Republic of China *These authors contributed equally to this work Objective: Many previous studies have demonstrated that neuromyelitis optica (NMO patients have abnormalities of brain anatomy and function. However, differences in spontaneous brain activity between myelin oligodendrocyte glycoprotein (MOG-IgG ON and aquaporin 4(AQP4-neuromyelitis optica-optic neuritis (ON remain unknown. In the current study, we investigated the brain neural homogeneity in MOG-IgG ON versus AQP4-IgG NMO-ON subjects by regional homogeneity (ReHo method using magnetic resonance imaging (MRI. Patients and methods: A total of 32 NMO-ON and ON subjects (21 with AQP4-IgG+NMO-ON and 11 with MOG-IgG+ON and 34 healthy controls (HCs closely matched for age were recruited, and scans were performed for all subjects. A one-way analysis of variance (ANOVA was performed to determine the regions in which the ReHo was different across the three groups. NMO-ON and ON subjects were distinguished from HCs by a receiver operating characteristic (ROC curve. The relationship between the mean ReHo in many brain regions and clinical features in NMO subjects was calculated by Pearson correlation analysis. Results: Compared with HCs, MOG-IgG+ON subjects had significantly decreased ReHo values in the posterior lobe of the left cerebellum and increased ReHo values in the left inferior frontal gyrus, right prefrontal gyrus, and left precentral/postcentral gyrus. AQP4-IgG+NMO-ON subjects showed higher ReHo values in the left inferior frontal gyrus and right middle temporal/occipital gyrus. Compared with MOG-IgG+ON subjects, AQP4-IgG+NMO-ON subjects had lower Re

The cervical spinal cord in neuromyelitis optica patients: A comparative study with multiple sclerosis using diffusion tensor imaging

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Pessôa, Fernanda Miraldi Clemente, E-mail: fernandamiraldi@hotmail.com [Federal University of Rio de Janeiro, Medical Student, Rua Rodolpho Paulo Rocco, 255, Cidade Universitária, Ilha do Fundão, Rio de Janeiro, RJ (Brazil); Lopes, Fernanda Cristina Rueda, E-mail: frueda81@hotmail.com [Department of Radiology, Federal University of Rio de Janeiro, Avenida das Américas, 4666 sl 325, Barra da Tijuca, Rio de Janeiro, RJ (Brazil); Costa, João Victor Altamiro, E-mail: victoraltamiro@gmail.com [Department of Radiology, Federal University of Rio de Janeiro, Rua Rodolpho Paulo Rocco, 255, Cidade Universitária, Ilha do Fundão, Rio de Janeiro, RJ (Brazil); Leon, Soniza Vieira Alves, E-mail: sonizavleon@globo.com [Department of Neurology, Federal University of Rio de Janeiro, Rua Rodolpho Paulo Rocco, 255, Cidade Universitária, Ilha do Fundão, Rio de Janeiro, RJ (Brazil); Domingues, Romeu Côrtes, E-mail: romeu@CDPi.com.br [CDPI – Clínica de Diagnóstico Por Imagem, Avenida das Américas, 4666 sl 325, Barra da Tijuca, Rio de Janeiro, RJ (Brazil); Gasparetto, Emerson Leandro, E-mail: egasparetto@gmail.com [Department of Radiology, Federal University of Rio de Janeiro, Avenida das Américas, 4666 sl 325, Barra da Tijuca, Rio de Janeiro, RJ (Brazil); CDPI – Clínica de Diagnóstico Por Imagem, Avenida das Américas, 4666 sl 325, Barra da Tijuca, Rio de Janeiro, RJ (Brazil)

2012-10-15

Introduction: This study aims to evaluate “in vivo” the integrity of the normal-appearing spinal cord in patients with neuromyelitis optica (NMO), using diffusion tensor MR imaging, comparing to controls and patients with multiple sclerosis (MS). Materials and methods: We studied 8 patients with NMO and 17 without any neurologic disorder. Also, 32 MS patients were selected. Fractional anisotropy (FA), axial diffusivity (AD), radial diffusivity (RD) and mean diffusivity (MD) were calculated within regions of interest at C2 and C7 levels in the four columns of the spinal cord. Results: At C2, the FA value was decreased in NMO patients compared to MS and controls in the anterior column. Also in this column, RD value showed increase in NMO compared to MS and to controls. The FA value of the posterior column was decreased in NMO in comparison to controls. At C7, AD value was higher in NMO than in MS in the right column. At the same column, MD values were increased in NMO compared to MS and to controls. Conclusions: There is extensive NASC damage in NMO patients, including peripheral areas of the cervical spinal cord, affecting the white matter, mainly caused by demyelination. This suggests a new spinal cord lesion pattern in NMO in comparison to MS.

The cervical spinal cord in neuromyelitis optica patients: A comparative study with multiple sclerosis using diffusion tensor imaging

International Nuclear Information System (INIS)

Pessôa, Fernanda Miraldi Clemente; Lopes, Fernanda Cristina Rueda; Costa, João Victor Altamiro; Leon, Soniza Vieira Alves; Domingues, Romeu Côrtes; Gasparetto, Emerson Leandro

2012-01-01

Introduction: This study aims to evaluate “in vivo” the integrity of the normal-appearing spinal cord in patients with neuromyelitis optica (NMO), using diffusion tensor MR imaging, comparing to controls and patients with multiple sclerosis (MS). Materials and methods: We studied 8 patients with NMO and 17 without any neurologic disorder. Also, 32 MS patients were selected. Fractional anisotropy (FA), axial diffusivity (AD), radial diffusivity (RD) and mean diffusivity (MD) were calculated within regions of interest at C2 and C7 levels in the four columns of the spinal cord. Results: At C2, the FA value was decreased in NMO patients compared to MS and controls in the anterior column. Also in this column, RD value showed increase in NMO compared to MS and to controls. The FA value of the posterior column was decreased in NMO in comparison to controls. At C7, AD value was higher in NMO than in MS in the right column. At the same column, MD values were increased in NMO compared to MS and to controls. Conclusions: There is extensive NASC damage in NMO patients, including peripheral areas of the cervical spinal cord, affecting the white matter, mainly caused by demyelination. This suggests a new spinal cord lesion pattern in NMO in comparison to MS

Optical Coherence Tomography and Magnetic Resonance Imaging in Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorder.

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Manogaran, Praveena; Hanson, James V M; Olbert, Elisabeth D; Egger, Christine; Wicki, Carla; Gerth-Kahlert, Christina; Landau, Klara; Schippling, Sven

2016-11-15

Irreversible disability in multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) is largely attributed to neuronal and axonal degeneration, which, along with inflammation, is one of the major pathological hallmarks of these diseases. Optical coherence tomography (OCT) is a non-invasive imaging tool that has been used in MS, NMOSD, and other diseases to quantify damage to the retina, including the ganglion cells and their axons. The fact that these are the only unmyelinated axons within the central nervous system (CNS) renders the afferent visual pathway an ideal model for studying axonal and neuronal degeneration in neurodegenerative diseases. Structural magnetic resonance imaging (MRI) can be used to obtain anatomical information about the CNS and to quantify evolving pathology in MS and NMOSD, both globally and in specific regions of the visual pathway including the optic nerve, optic radiations and visual cortex. Therefore, correlations between brain or optic nerve abnormalities on MRI, and retinal pathology using OCT, may shed light on how damage to one part of the CNS can affect others. In addition, these imaging techniques can help identify important differences between MS and NMOSD such as disease-specific damage to the visual pathway, trans-synaptic degeneration, or pathological changes independent of the underlying disease process. This review focuses on the current knowledge of the role of the visual pathway using OCT and MRI in patients with MS and NMOSD. Emphasis is placed on studies that employ both MRI and OCT to investigate damage to the visual system in these diseases.

Fatigue in patients with neuromyelitis optica spectrum disorder and its impact on quality of life.

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Jin Myoung Seok

Full Text Available Fatigue is a prevalent symptom and major burden in neuroimmunological diseases. In neuromyelitis optica spectrum disorder (NMOSD, a severe autoimmune central nervous system (CNS inflammatory disease with autoantibodies reactive to aquaporin-4, there are few reports about fatigue and quality of life (QOL. We aimed to evaluate the severity of fatigue and its relationship with QOL in patients with NMOSD. We prospectively studied patients with NMOSD who were in remission and seropositive for anti-aquaporin-4 antibody, and they were divided into 2 groups based on the presence of fatigue assessed using the Functional Assessment of Chronic Illness Therapy-fatigue score. Sleep quality, depression, pain, and QOL were also evaluated. A total of 35 patients were enrolled (mean age, 46.5 ± 14.1 years; female: male = 29:6, and the median Expanded Disability Status Scale (EDSS score was 2.0 (range, 0 to 8.0. The patients with fatigue (N = 25, 71.4% had poorer sleep quality and more severe depression than those without fatigue (p = 0.009 and p = 0.001. Both the physical and mental QOL scores were lower in patients with fatigue than in those without fatigue (p = 0.033 and p = 0.004. Multiple linear regression analyses showed that the degree of fatigue with EDSS score and pain were independent predictors of physical aspects of QOL (B = 0.382, p = 0.001, whereas depression was the only predictor of the mental components of QOL (B = -0.845, p = <0.001. Fatigue is a common symptom and an important predictor of QOL in patients with NMOSD.

Abnormal baseline brain activity in patients with neuromyelitis optica: A resting-state fMRI study

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Liu Yaou; Liang Peipeng; Duan Yunyun; Jia Xiuqin; Wang Fei; Yu Chunshui; Qin Wen; Dong Huiqing; Ye Jing; Li Kuncheng

2011-01-01

Purpose: Recent immunopathologic and MRI findings suggest that tissue damage in neuromyelitis optica (NMO) is not limited to spinal cord and optic nerve, but also in brain. Baseline brain activity can reveal the brain functional changes to the tissue damages and give clues to the pathophysiology of NMO, however, it has never been explored by resting-state functional MRI (fMRI). We used regional amplitude of low frequency fluctuation (ALFF) as an index in resting-state fMRI to investigate how baseline brain activity changes in patients with NMO. Methods: Resting-state fMRIs collected from seventeen NMO patients and seventeen age- and sex-matched normal controls were compared to investigate the ALFF difference between the two groups. The relationships between ALFF in regions with significant group differences and the EDSS (Expanded Disability Status Scale), disease duration were further explored. Results: Our results showed that NMO patients had significantly decreased ALFF in precuneus, posterior cingulate cortex (PCC) and lingual gyrus; and increased ALFF in middle frontal gyrus, caudate nucleus and thalamus, compared to normal controls. Moderate negative correlations were found between the EDSS and ALFF in the left middle frontal gyrus (r = -0.436, p = 0.040) and the left caudate (r = -0.542, p = 0.012). Conclusion: The abnormal baseline brain activity shown by resting-state fMRI in NMO is relevant to cognition, visual and motor systems. It implicates a complex baseline brain status of both functional impairments and adaptations caused by tissue damages in these systems, which gives clues to the pathophysiology of NMO.

Abnormal baseline brain activity in patients with neuromyelitis optica: A resting-state fMRI study

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Liu Yaou [Department of Radiology, Xuanwu Hospital, Capital Medical University, Beijing 100053 (China); Liang Peipeng [Department of Radiology, Xuanwu Hospital, Capital Medical University, Beijing 100053 (China); International WIC institute, Beijing University of Technology, Beijing 100024 (China); Duan Yunyun; Jia Xiuqin; Wang Fei; Yu Chunshui; Qin Wen [Department of Radiology, Xuanwu Hospital, Capital Medical University, Beijing 100053 (China); Dong Huiqing; Ye Jing [Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing 100053 (China); Li Kuncheng, E-mail: likuncheng1955@yahoo.com.cn [Department of Radiology, Xuanwu Hospital, Capital Medical University, Beijing 100053 (China)

2011-11-15

Purpose: Recent immunopathologic and MRI findings suggest that tissue damage in neuromyelitis optica (NMO) is not limited to spinal cord and optic nerve, but also in brain. Baseline brain activity can reveal the brain functional changes to the tissue damages and give clues to the pathophysiology of NMO, however, it has never been explored by resting-state functional MRI (fMRI). We used regional amplitude of low frequency fluctuation (ALFF) as an index in resting-state fMRI to investigate how baseline brain activity changes in patients with NMO. Methods: Resting-state fMRIs collected from seventeen NMO patients and seventeen age- and sex-matched normal controls were compared to investigate the ALFF difference between the two groups. The relationships between ALFF in regions with significant group differences and the EDSS (Expanded Disability Status Scale), disease duration were further explored. Results: Our results showed that NMO patients had significantly decreased ALFF in precuneus, posterior cingulate cortex (PCC) and lingual gyrus; and increased ALFF in middle frontal gyrus, caudate nucleus and thalamus, compared to normal controls. Moderate negative correlations were found between the EDSS and ALFF in the left middle frontal gyrus (r = -0.436, p = 0.040) and the left caudate (r = -0.542, p = 0.012). Conclusion: The abnormal baseline brain activity shown by resting-state fMRI in NMO is relevant to cognition, visual and motor systems. It implicates a complex baseline brain status of both functional impairments and adaptations caused by tissue damages in these systems, which gives clues to the pathophysiology of NMO.

Connexin 43 astrocytopathy linked to rapidly progressive multiple sclerosis and neuromyelitis optica.

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Katsuhisa Masaki

Full Text Available BACKGROUND: Multiple sclerosis (MS and neuromyelitis optica (NMO occasionally have an extremely aggressive and debilitating disease course; however, its molecular basis is unknown. This study aimed to determine a relationship between connexin (Cx pathology and disease aggressiveness in Asian patients with MS and NMO. METHODS/PRINCIPAL FINDINGS: Samples included 11 autopsied cases with NMO and NMO spectrum disorder (NMOSD, six with MS, and 20 with other neurological diseases (OND. Methods of analysis included immunohistochemical expression of astrocytic Cx43/Cx30, oligodendrocytic Cx47/Cx32 relative to AQP4 and other astrocytic and oligodendrocytic proteins, extent of demyelination, the vasculocentric deposition of complement and immunoglobulin, and lesion staging by CD68 staining for macrophages. Lesions were classified as actively demyelinating (n=59, chronic active (n=58 and chronic inactive (n=23. Sera from 120 subjects including 30 MS, 30 NMO, 40 OND and 20 healthy controls were examined for anti-Cx43 antibody by cell-based assay. Six NMO/NMOSD and three MS cases showed preferential loss of astrocytic Cx43 beyond the demyelinated areas in actively demyelinating and chronic active lesions, where heterotypic Cx43/Cx47 astrocyte oligodendrocyte gap junctions were extensively lost. Cx43 loss was significantly associated with a rapidly progressive disease course as six of nine cases with Cx43 loss, but none of eight cases without Cx43 loss regardless of disease phenotype, died within two years after disease onset (66.7% vs. 0%, P=0.0090. Overall, five of nine cases with Cx43 loss and none of eight cases without Cx43 loss had distal oligodendrogliopathy characterized by selective myelin associated glycoprotein loss (55.6% vs. 0.0%, P=0.0296. Loss of oligodendrocytic Cx32 and Cx47 expression was observed in most active and chronic lesions from all MS and NMO/NMOSD cases. Cx43-specific antibodies were absent in NMO/NMOSD and MS patients. CONCLUSIONS

Retinal ganglion cell-inner plexiform and nerve fiber layers in neuromyelitis optica.

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Hu, Sai-Jing; Lu, Pei-Rong

2018-01-01

To determine the thickness of the retinal ganglion cell-inner plexiform layer (GCIPL) and the retinal nerve fiber layer (RNFL) in patients with neuromyelitis optica (NMO). We conducted a cross-sectional study that included 30 NMO patients with a total of 60 eyes. Based on the presence or absence of optic neuritis (ON), subjects were divided into either the NMO-ON group (30 eyes) or the NMO-ON contra group (10 eyes). A detailed ophthalmologic examination was performed for each group; subsequently, the GCIPL and the RNFL were measured using high-definition optical coherence tomography (OCT). In the NMO-ON group, the mean GCIPL thickness was 69.28±21.12 µm, the minimum GCIPL thickness was 66.02±10.02 µm, and the RNFL thickness were 109.33±11.23, 110.47±3.10, 64.92±12.71 and 71.21±50.22 µm in the superior, inferior, temporal and nasal quadrants, respectively. In the NMO-ON contra group, the mean GCIPL thickness was 85.12±17.09 µm, the minimum GCIPL thickness was 25.39±25.1 µm, and the RNFL thicknesses were 148.33±23.22, 126.36±23.45, 82.21±22.30 and 83.36±31.28 µm in the superior, inferior, temporal and nasal quadrants, respectively. In the control group, the mean GCIPL thickness was 86.98±22.37 µm, the minimum GCIPL thickness was 85.28±10.75 µm, and the RNFL thicknesses were 150.22±22.73, 154.79±60.23, 82.33±7.01 and 85.62±13.81 µm in the superior, inferior, temporal and nasal quadrants, respectively. The GCIPL and RNFL were thinner in the NMO-ON contra group than in the control group ( P deviation (MD) and corrected pattern standard deviation (PSD) in the NMO-ON group ( P <0.05). The thickness of the GCIPL and RNFL, as measured using OCT, may indicate optic nerve damage in patients with NMO.

Pontes e rupturas no fabular de Wilson Bueno

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Furquim, Fulvia Maria Giaretta de Almeida

2008-01-01

O trabalho tem por objetivo analisar tres obras contemporaneas do escritor paranaense Wilson Bueno (1949- ), que fazem parte de um projeto literario, em que as personagens sao apresentadas de uma visao inicialmente plural, como se apenas uma delas pudesse ser a representacao do todo, para uma optica individualista, caracteristica de um sistema capitalista. Sao elas: Manual de Zoofilia (1991), Jardim Zoologico (1999) e Cachorrros do Ceu (2005). O escritor contemporaneo segue a tradicao dos bes...

Adult methylmalonic acidemia presented as neuromyelitis optica: one case report

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Sheng-de LI

2015-10-01

Full Text Available A 26-year-old male was admitted to our department, complaining of cognitive impairment, urine incontinence for 3 months, blurred vision for one month and numbness of bilateral lower limbs for 20 days. Presumed as “depression” and “viral encephalitis”, antidepressant and dexamethasone had been given but had no response. Neurological examination demonstrated impaired orientation to time and place; hearing impairment of right ear; normal muscle force in upper limbs, proximal lower muscle force was 2 and distal was 0; normal tendon reflex in both upper limbs; diminished tendon reflex in both lower limbs; left palmomental reflex (+; bilateral Babinski sign (+. Below T10: diminished superficial, deep sensation and cortical sensory. Cranial MRI on admission revealed widened sulci in bilateral cleft and frontal, temporal and insular lobes, indicating brain atrophy. Spinal MRI revealed high-intensity signals of C3-7 level and T1-12 level. The patient was diagnosed as “neuromyelitis optica (NMO” at first, but cognitive impairment is really rare in NMO. It finally turned out to be “inherited metabolic diseases” with the negative results of aquaporin 4 (AQP4, NMO-IgG, GM1, voltage-gated potassium channel (VGKC from serum and cerebrospinal fluid (CSF. The elevated level of plasm homocysteine [30.79 mmol/L (5-20 mmol/L] and urine methylmalonic acid [0.40 mmol/L (0.001 mmol/L] ascertained the diagnosis of methylmalonic acidemia. The patient was given oral treatment of folate 5 mg (3 times a day, 13 days and levocarnitine 1 g (3 times a day, 8 days and intramuscular injection of mecobalamine 1mg (once a day, 4 days or 0.50 mg (once a day, 8 days and adenosylcobalamine 0.50 mg (once a day, 8 days. Sixteen days on discharge, the patient’s neurological examination revealed no obvious recovery of vision; lower muscle force: about Ⅳ, right sensory level: T12-L1, and left sensory level lowered to L3. Reexamination of MRI revealed brain atrophy

Cognitive performance of neuromyelitis optica patients: comparison with multiple sclerosis

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Sandra Vanotti

2013-06-01

Full Text Available The aim of the present research was to investigate cognitive pattern of patients with neuromyelitis optica (NMO and to compare it with multiple sclerosis (MS patients' performance. Methods: Fourteen NMO, 14 relapsing remitting multiple sclerosis (RRMS, and 14 healthy control patients participated in the investigation. Neuropsychological functions were evaluated with the Brief Repeatable Neuropsychological Battery for MS; Symbol Digit Modalities Test; Digit Span; and Semantic Fluency. Results: Fifty-seven percent of NMO patients and 42.85% of the MS ones had abnormal performance in at least two cognitive tests. The NMO Group showed abnormal performance in verbal fluency, verbal and visual memories, with greater attention deficits. NMO patients outperformed healthy control in the paced auditory serial addition test (PASAT. However, no difference was found between NMO and RRMS patients. Conclusions: The NMO Group showed more dysfunction in attention and verbal fluencies than in verbal and visual memories. When compared with the MS patients, a similar dysfunction pattern was found. O objetivo da presente pesquisa foi investigar o padrão cognitivo de pacientes com neuromielite óptica (NMO e compará-lo com o desempenho de pacientes com esclerose múltipla (EM. Métodos: Quatorze pacientes com NMO, 14 com esclerose múltipla recorrente remitente (EMRR e 14 participantes do Controle saudáveis participaram da presente investigação. As funções neuropsicológicas foram avaliadas com a Bateria Breve de Testes Neuropsicológicos de Rao, Teste Símbolo Digit e a Fluência Semântica. Resultados: Cinquenta e sete por cento dos pacientes com NMO e 42,85% daqueles com EM apresentaram desempenho anormal em pelo menos dois testes cognitivos. O Grupo NMO apresentarou desempenho anormal na fluência verbal e nas memórias visual e verbal, com maiores déficits de atenção. Pacientes com NMO superaram os controles saudáveis em PASAT. No entanto, não foi

Immunotherapies in neuromyelitis optica spectrum disorder: efficacy and predictors of response.

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Stellmann, Jan-Patrick; Krumbholz, Markus; Friede, Tim; Gahlen, Anna; Borisow, Nadja; Fischer, Katrin; Hellwig, Kerstin; Pache, Florence; Ruprecht, Klemens; Havla, Joachim; Kümpfel, Tania; Aktas, Orhan; Hartung, Hans-Peter; Ringelstein, Marius; Geis, Christian; Kleinschnitz, Christoph; Berthele, Achim; Hemmer, Bernhard; Angstwurm, Klemens; Young, Kim Lea; Schuster, Simon; Stangel, Martin; Lauda, Florian; Tumani, Hayrettin; Mayer, Christoph; Zeltner, Lena; Ziemann, Ulf; Linker, Ralf Andreas; Schwab, Matthias; Marziniak, Martin; Then Bergh, Florian; Hofstadt-van Oy, Ulrich; Neuhaus, Oliver; Zettl, Uwe; Faiss, Jürgen; Wildemann, Brigitte; Paul, Friedemann; Jarius, Sven; Trebst, Corinna; Kleiter, Ingo

2017-08-01

To analyse predictors for relapses and number of attacks under different immunotherapies in patients with neuromyelitis optica spectrum disorder (NMOSD). This is a retrospective cohort study conducted in neurology departments at 21 regional and university hospitals in Germany. Eligible participants were patients with aquaporin-4-antibody-positive or aquaporin-4-antibody-negative NMOSD. Main outcome measures were HRs from Cox proportional hazard regression models adjusted for centre effects, important prognostic factors and repeated treatment episodes. 265 treatment episodes with a mean duration of 442 days (total of 321 treatment years) in 144 patients (mean age at first attack: 40.9 years, 82.6% female, 86.1% aquaporin-4-antibody-positive) were analysed. 191 attacks occurred during any of the treatments (annual relapse rate=0.60). The most common treatments were rituximab (n=77, 111 patient-years), azathioprine (n=52, 68 patient-years), interferon-β (n=32, 61 patient-years), mitoxantrone (n=34, 32.1 patient-years) and glatiramer acetate (n=17, 10 patient-years). Azathioprine (HR=0.4, 95% CI 0.3 to 0.7, p=0.001) and rituximab (HR=0.6, 95% CI 0.4 to 1.0, p=0.034) reduced the attack risk compared with interferon-β, whereas mitoxantrone and glatiramer acetate did not. Patients who were aquaporin-4-antibody-positive had a higher risk of attacks (HR=2.5, 95% CI 1.3 to 5.1, p=0.009). Every decade of age was associated with a lower risk for attacks (HR=0.8, 95% CI 0.7 to 1.0, p=0.039). A previous attack under the same treatment tended to be predictive for further attacks (HR=1.5, 95% CI 1.0 to 2.4, p=0.065). Age, antibody status and possibly previous attacks predict further attacks in patients treated for NMOSD. Azathioprine and rituximab are superior to interferon-β. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Diffusion-weighted imaging helps differentiate multiple sclerosis and neuromyelitis optica-related acute optic neuritis.

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Wan, Hailin; He, Huijin; Zhang, Fang; Sha, Yan; Tian, Guohong

2017-06-01

To evaluate the apparent diffusion coefficient (ADC) values between multiple sclerosis (MS) and neuromyelitis optica (NMO)-related acute optic neuritis (ON) patients and predict their optic nerve atrophy of optic coherence tomography (OCT) parameters. Nineteen MS and 15 NMO-related acute ON patients who underwent a diffusion-weighted imaging sequence in 3.0 Tesla MR scanner and a follow-up OCT examination after 6 months were included. The ADC values, thickness of the retinal nerve fiber layer (RNFL) and the macular ganglion cell complex (GCC) between MS and NMO related ON were assessed. The mean ADC value of the NMO-ON, (0.691 ± 0.195[SD]) × 10 -3 mm 2 /s, was significantly smaller (P = 0.0133) than that of MS-ON. The mean ADC value of MS-ON, (0.879 ± 0.144) × 10 -3 mm 2 /s, was significantly smaller (P < 0.0001) than that of control group, (1.025 ± 0.067) × 10 -3 mm 2 /s. Using an ADC value smaller than 0.830 × 10 -3 mm 2 /s as the threshold value for differentiating MS-ON from NMO-ON patients, the highest accuracy of 76.7%, with 75.0% sensitivity and 78.3% specificity, was obtained. The ADC value measured at the acute stage of ON was correlated with the thickness of the RNFL (r = 0.441; P = 0.006) and the GCC (r = 0.526; P < 0.0001) after 6 months. The ADC value might be helpful for differentiating MS-ON from NMO-ON patients. The decreased ADC value was correlated with optic nerve atrophy on OCT. 3 Technical Efficacy: Stage 2 J. MAGN. RESON. IMAGING 2017;45:1780-1785. © 2016 International Society for Magnetic Resonance in Medicine.

Quantitative Susceptibility Mapping Indicates a Disturbed Brain Iron Homeostasis in Neuromyelitis Optica - A Pilot Study.

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Thomas Martin Doring

Full Text Available Dysregulation of brain iron homeostasis is a hallmark of many neurodegenerative diseases and can be associated with oxidative stress. The objective of this study was to investigate brain iron in patients with Neuromyelitis Optica (NMO using quantitative susceptibility mapping (QSM, a quantitative iron-sensitive MRI technique. 12 clinically confirmed NMO patients (6 female and 6 male; age 35.4y±14.2y and 12 age- and sex-matched healthy controls (7 female and 5 male; age 33.9±11.3y underwent MRI of the brain at 3 Tesla. Quantitative maps of the effective transverse relaxation rate (R2* and magnetic susceptibility were calculated and a blinded ROI-based group comparison analysis was performed. Normality of the data and differences between patients and controls were tested by Kolmogorov-Smirnov and t-test, respectively. Correlation with age was studied using Spearman's rank correlation and an ANCOVA-like analysis. Magnetic susceptibility values were decreased in the red nucleus (p0.95; between -15 and -22 ppb depending on reference region with a trend toward increasing differences with age. R2* revealed significantly decreased relaxation in the optic radiations of five of the 12 patients (p<0.0001; -3.136±0.567 s-1. Decreased relaxation in the optic radiation is indicative for demyelination, which is in line with previous findings. Decreased magnetic susceptibility in the red nucleus is indicative for a lower brain iron concentration, a chemical redistribution of iron into less magnetic forms, or both. Further investigations are necessary to elucidate the pathological cause or consequence of this finding.

Diffusion tensor imaging of brain in relapsing neuromyelitis optica

International Nuclear Information System (INIS)

Yu Chunshui; Li Kuncheng; Qin Wen; Lin Fuchun; Jiang Tianzi

2007-01-01

Objective: To investigate the presence of occult brain tissue damage in patients with relapsing neuromyelitis optica (RNMO) and its possible mechanism by using diffusion tensor imaging (DTI). Methods: DTI scans were performed in 16 patients with RNMO and 16 sex- and age-matched healthy controls. Histogram analysis of mean diffusivity (MD) and fractional anisotropy (FA) was performed in brain tissue (BT), white matter (WM) and gray matter (GM) to detect the presence of occult brain tissue damage in RNMO patients. Region of interest (ROI) analysis of MD and FA was also performed in 6 dedicated regions with or without direct connection with spinal cord or optic nerve to determine the relationship between occult brain tissue damage and the damage of spinal cord and optic nerve. Results Patients with RNMO had a significantly higher average MD of the BT [RNMO (0.95 ± 0.02) x 10 -3 mm 2 /s, controls (0.91 ± 0.03) x 10 -3 mm 2 /s, t=3.940, P -3 mm 2 /s, controls(0.80 ± 0.02) x 10 -3 mm 2 /s, t=3.117, P=0.004] an.d GM [RNMO (1.06 ± 0.04) x 10 -3 mm 2 /s, controls (0.88 ± 0.05) x 10 -3 mm 2 /s, t=4.031, P -3 mm 2 /s, controls (0.81 ± 0.02) x 10 -3 mm 2 /s, t=4.373, P -3 mm 2 /s, controls (1.11 ± 0.10) x 10 -3 mm 2 /s, t=4.260, P -3 mm 2 /s, controls (0.87 ± 0.05) x 10 -3 mm 2 /s, t4.391, P -3 mm 2 /s, controls (0.72 ± O.01) x 10 -3 mm 2 /s, t=4.683, P -3 mm 2 /s, controls (0.82+0.03) x 10-3 mm2/s, t = 4. 619, P -3 mm 2 /s, controls (0.73±0.03) x 10 -3 mm 2 /s, t =2.804, P=0.009 and splenium of corpus callosum: RNMO(0.77 ± 0.05) x 10 -3 mm 2 /s, controls (0.73 ± 0.04) x 10 -3 mm 2 /s, t=2.234, P=0.033] and FA [genu of corpus callosum: RNMO 0.82± 0.03 ,controls 0.82 ± 0.03, t=0.196, P=0.846 and splenium of corpus caltosum: RNMO 0.83±0.03, controls 0.83 ± 0.02, t=0.333, P=0.741] between RNMO patients and controls. Conclusion: RNMO patients have occult brain tissue damage, which might be related to the antegrade and retrograde degeneration secondary to lesions in

Study of optic nerve in patients with neuromyelitis optica using diffusion tensor imaging

International Nuclear Information System (INIS)

Chen Zhiye; Zhu Lijun; Lou Xin; Li Jinfeng; Yang Yang; Ma Lin

2012-01-01

Objective: To explore the diagnostic value of optic diffusion tensor imaging (DTI) in detecting the impairment of optic nerve in neuromyelitis optica (NMO) patients. Methods: Conventional MRI and optic DTI were performed in 28 NMO patients and 38 normal controls (NC). Fractional anisotropy (FA) values were measured in the anterior part, middle part and posterior part of intraorbital segment of optic nerve. The patients were classified into 3 groups based on the impairment of vision and visual evoked potential (VEP): monocular impairment (MI) group, 10 eyes; biocular impairment (BI) group, 36 eyes; and normal-appearing (NA) group, 10 eyes. All patients were performed with the evaluation of expanded disability status scale (EDSS). One-way analysis of variance (ANOVA), receiver operating characteristic (ROC) curve, and Spearman correlation analysis were performed among the subgroups of NMO and normal controls. Results: There was significantly statistical difference between the four groups (F=43.54, P<0.01). Decreased FA values were demonstrated in the MI group (0.29 ±0.08), BI group (0.27 ±0.08), and NA group (0.35 ±0.13) compared with NC (0.45 ±0.07) (P<0.01). FA value in BI group was significantly lower than that of NA group (P<0.01). Area under curve by ROC analysis in NC vs MI, NC vs BI, NC vs NA, and NC vs NMO was 0.92, 0.95, 0.74, and 0.91, respectively. The diagnostic sensitivity of ROC was 80%, 86%, 50%, and 79%, respectively. The diagnostic specificity of ROC was 95% for the each compared groups. FA value showed no correlation with EDSS for each NMO groups, and showed negative correlation with disease duration for BI group (r=-0.371, P<0.05). Conclusions: Various degrees of optic nerve injuries, indicated by decreased FA value,are present in NMO patients, and optic DTI may be a simple and effective tool for the quantitative evaluation of optic nerve in NMO patients. (authors)

Development of a patient-centred conceptual framework of health-related quality of life in neuromyelitis optica: a qualitative study.

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Methley, Abigail M; Mutch, Kerry; Moore, Perry; Jacob, Anu

2017-02-01

Neuromyelitis optica (NMO) is an auto-immune disease that can cause severe visual and mobility impairments. Research on health-related quality of life (HRQoL) in NMO is scarce, limiting knowledge on factors influencing HRQoL and support needs. This study provides the first qualitative exploration of HRQoL in NMO, conducted to provide a conceptual framework for the development of an NMO patient-reported outcome measure. Fifteen people with NMO (aged 18-74; 11 women, 4 men) participated in semi-structured interviews; data were analysed using constant comparative analysis. HRQoL in NMO is a multifaceted concept incorporating highly subjective perceptions of normality and meaning. Four major themes were identified: impact of physical symptoms on daily living, utilizing support to achieve independence, expectations for life and meaningful roles in life and purpose. Themes highlighted the importance of perceived normality, and its relationship to attaining life goals comparable to peers, as underpinning evaluations of HRQoL. Many people with severe disability reported a high HRQoL, suggesting the inappropriateness of assuming a negative HRQoL on the basis of an individual's neurological impairment. These findings further the conceptual understanding of HRQoL in NMO, informing patient-care approaches and the development of an NMO-specific patient-reported outcome measure. © 2015 The Authors. Health Expectation published by John Wiley & Sons Ltd.

Aquaporin-4 antibody in neuromyelitis optica: re-testing study in a large population from China.

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Long, Youming; Liang, Junyan; Zhong, Rong; Wu, Linzhan; Qiu, Wei; Lin, Shaopeng; Gao, Cong; Chen, Xiaohui; Zheng, Xueping; Yang, Ning; Gao, Min; Wang, Zhanhang

2017-09-01

Aquaporin-4 (AQP4) antibody sero-positivity is critically important in neuromyelitis optica (NMO). However, the sensitivity of different assays is highly variable. Repeating detection with a highly sensitive assay in a large population is necessary in the case of so-called negative NMO. Retrospective analysis where AQP4 antibodies were detected by commercial cell-based assay (CBA), in-house M23-CBA and in-house M1-CBA. Of the 1011 serum samples, 206 (20.4%) were sero-positive by primary commercial CBA. In the retest, all 206 participants positive by primary commercial CBA also yielded positive results by in-house M23-CBA and the second commercial CBA again, but only 124 positive in in-house M1-CBA. Among the 805 participants negative by primary commercial CBA, 71 participants were positive for in-house M23-CBA, of which 20 participants were positive for the second commercial CBA, and none were positive by in-house M1-CBA. Of the 171 cerebral spinal fluid samples, 75 (43.9%) were positive by primary commercial CBA. All 75 participants positive by primary commercial CBA also yielded positive results by in-house M23-CBA and the second commercial CBA. Forty-nine (65.3%) of these 75 participants were positive by in-house M1-CBA. Among the 96 participants negative by primary commercial CBA, 15 participants were positive for in-house M23-CBA and none were positive by in-house M1-CBA and the second commercial CBA. Different AQP4 isoforms in CBA result in different detection effects, and in-house M23-CBA is the most sensitive method. Some AQP4 antibody-negative NMO may be subject to diagnostic uncertainty due to limitations of the assays.

Low T3 syndrome in neuromyelitis optica spectrum disorder: Associations with disease activity and disability.

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Cho, Eun Bin; Min, Ju-Hong; Cho, Hye-Jin; Seok, Jin Myoung; Lee, Hye Lim; Shin, Hee Young; Lee, Kwang-Ho; Kim, Byoung Joon

2016-11-15

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. Forty-nine NMOSD patients with anti-aquaporin-4 antibody and 392 age- and sex-matched healthy controls were included. We measured the levels of thyroid hormones and anti-thyroid antibodies. The prevalence of clinical hypothyroidism, subclinical hyperthyroidism, and low T3 syndrome were higher in patients with NMOSD (4.1%, 12.2%, and 20.4%, respectively) compared with healthy controls (0.3%, 2.8%, and 0.5%, respectively; p=0.034, p=0.001, and p<0.001, respectively). However, anti-thyroperoxidase antibody (anti-TPO)-positivity did not significantly differ between NMOSD patients (20.4%) and controls (11.5%). Low T3 syndrome was more prevalent among patients during an attack (N=10/19, 52.6%) than those in remission (N=1/30, 3.3%). In addition, patients with low T 3 syndrome had significantly higher EDSS scores at the last visits as well as at sampling compared to those without low T3 syndrome. T3 levels were inversely correlated with EDSS score at the last visit after adjustment for age, sex, disease duration, clinical status (attack vs. remission), oral prednisolone use, iv methylprednisolone use, other immunosuppressive agents use, and the location of lesion (ρ=-0.416, p=0.010). Our study suggests that thyroid dysfunction is frequent in patients with NMOSD; particularly, serum T3 levels may be a useful indicator of disease activity and disability in NMOSD. Copyright © 2016 Elsevier B.V. All rights reserved.

Experimental mouse model of optic neuritis with inflammatory demyelination produced by passive transfer of neuromyelitis optica-immunoglobulin G

Science.gov (United States)

2014-01-01

Background Although optic neuritis (ON) is a defining feature of neuromyelitis optica (NMO), appropriate animal models of NMO ON are lacking. Most NMO patients are seropositive for immunoglobulin G autoantibodies (NMO-IgG) against the astrocyte water channel aquaporin-4 (AQP4). Methods Several approaches were tested to develop a robust, passive-transfer mouse model of NMO ON, including NMO-IgG and complement delivery by: (i) retrobulbar infusion; (ii) intravitreal injection; (iii) a single intracranial injection near the optic chiasm; and (iv) 3-days continuous intracranial infusion near the optic chiasm. Results Little ON or retinal pathology was seen using approaches (i) to (iii). Using approach (iv), however, optic nerves showed characteristic NMO pathology, with loss of AQP4 and glial fibrillary acidic protein immunoreactivity, granulocyte and macrophage infiltration, deposition of activated complement, demyelination and axonal injury. Even more extensive pathology was created in mice lacking complement inhibitor protein CD59, or using a genetically modified NMO-IgG with enhanced complement effector function, including significant loss of retinal ganglion cells. In control studies, optic nerve pathology was absent in treated AQP4-deficient mice, or in wild-type mice receiving control (non-NMO) IgG and complement. Conclusion Passive transfer of NMO-IgG and complement by continuous infusion near the optic chiasm in mice is sufficient to produce ON with characteristic NMO pathology. The mouse model of NMO ON should be useful in further studies of NMO pathogenesis mechanisms and therapeutics. PMID:24468108

Development of an Aquaporin-4 Orthogonal Array of Particle-Based ELISA for Neuromyelitis Optica Autoantibodies Detection.

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Francesco Pisani

Full Text Available Serological markers of Nuromyelitis Optica (NMO, an autoimmune disorder of the central nervous system, are autoantibodies targeting the astrocytic water channel aquaporin-4 (AQP4. We have previously demonstrated that the main epitopes for these autoantibodies (AQP4-IgG are generated by the supramolecular arrangement of AQP4 tetramers into an Orthogonal Array of Particles (OAPs. Many tests have been developed to detect AQP4-IgG in patient sera but several procedural issues affect OAP assembly and consequently test sensitivity. To date, the protein based ELISA test shows the lowest sensitivity while representing a valid alternative to the more sensitive cell based assay (CBA, which, however, shows economic, technical and interpretation problems. Here we have developed a high perfomance ELISA in which native OAPs are used as the molecular target. To this aim a native size exclusion chromatography method has been developed to isolate integral, highly pure and AQP4-IgG-recognized OAPs from rat brain. These OAPs were immobilized and oriented on a plastic plate by a sandwich approach and 139 human sera were tested, including 67 sera from NMO patients. The OAP-ELISA showed a 99% specificity and a higher sensitivity (91% compared to the CBA test. A comparative analysis revealed an end-point titer three orders of magnitude higher than the commercial ELISA and six times higher than our in-house CBA test. We show that CNS-extracted OAPs are crucial elements in order to perform an efficient AQP4-IgG test and the OAP-ELISA developed represents a valid alternative to the CBA currently used.

A treatable cause of myelopathy and vision loss mimicking neuromyelitis optica spectrum disorder: late-onset biotinidase deficiency.

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Yilmaz, Sanem; Serin, Mine; Canda, Ebru; Eraslan, Cenk; Tekin, Hande; Ucar, Sema Kalkan; Gokben, Sarenur; Tekgul, Hasan; Serdaroglu, Gul

2017-06-01

Biotinidase deficiency is characterized by severe neurological manifestations as hypotonia, lethargy, ataxia, hearing loss, seizures and developmental retardation in its classical form. Late-onset biotinidase deficiency presents distinctly from the classical form such as limb weakness and vision problems. A 14-year-old boy presented with progressive vision loss and upper limb weakness. The patient was initiated steroid therapy with a preliminary diagnosis of neuromyelitis optica spectrum disorder due to the craniospinal imaging findings demonstrating optic nerve, brainstem and longitudinally extensive spinal cord involvement. Although the patient exhibited partial clinical improvement after pulse steroid therapy, craniocervical imaging performed one month after the initiation of steroid therapy did not show any regression. The CSF IgG index was <0.8 (normal: <0.8), oligoclonal band and aquaporin-4 antibodies were negative. Metabolic investigations revealed a low biotinidase enzyme activity 8% (0.58 nmoL/min/mL; normal range: 4.4 to 12). Genetic testing showed c.98-104delinsTCC and p.V457 M mutations in biotinidase (BTD) gene. At the third month of biotin replacement therapy, control craniospinal MRI demonstrated a complete regression of the lesions. The muscle strength of the case returned to normal. His visual acuity was 7/10 in the left eye and 9/10 in the right. The late-onset form of the biotinidase deficiency should be kept in mind in all patients with myelopathy with or without vision loss, particularly in those with inadequate response to steroid therapy. The family screening is important to identify asymptomatic individuals and timely treatment.

Longitudinally extensive transverse myelitis in neuromyelitis optica: a prospective study of 13 Caucasian patients and literature review.

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Bălaşa, Rodica; Maier, Smaranda; Bajko, Zoltan; Motataianu, Anca; Crişan, Alexandra; Bălaşa, Adrian

2015-12-01

Neuromyelitis optica (NMO) is a homogenous disease that can be diagnosed by an association of clinical, neuroimaging and serological aspects. We analysed our 4 years NMO series with longitudinally extensive transverse myelitis (LETM) during the disease course. We included consecutive adult Caucasian patients who were diagnosed with definite NMO, or cases of NMO-IgG seropositive LETM considered as limited forms of NMO. Patients included were negative for other diseases (autoimmune, infectious, etc.). We report the Expanded Disability Status Scale (EDSS), brain and spine MRI, CSF, NMO-IgG, treatment, motor and visual outcome. Thirteen cases fulfilled the inclusion criteria, and the mean follow-up period was 3.74 ± 1.8 years. The initial motor deficit was severe with the mean value of motor functional parameter of 4.46 ± 1 and improved at discharge to 2.53 ± 1.4 (p < 0.001). With treatment, the outcome after LETM attack was good in 10 patients, with a significant improvement of the EDSS mainly upon motor deficit, while visual function had a very slight amelioration. The CSF analysis was normal in 8 cases; spinal MRI showed evidence of LETM in all patients while brain MRI was normal in 7. NMO-IgG is a biomarker for NMO that is of diagnostic value in cases of isolated LETM. LETM has a better outcome than ON in NMO Caucasians. Spinal MRI is essential for NMO diagnosis in the presence of LETM and the absence of multiple brain MRI lesions. Maintenance immunosuppressive therapy reduces the frequency of attacks.

Eleven episodes of recurrent optic neuritis of the same eye for 22 years eventually diagnosed as neuromyelitis optica spectrum disorder.

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Yew, Yih Chian; Hor, Jyh Yung; Lim, Thien Thien; Kanesalingam, Ruban; Ching, Yee Ming; Arip, Masita; Easaw, P E Samuel; Eow, Gaik Bee

2016-11-01

It is difficult to predict whether a particular attack of neuromyelitis optica spectrum disorder (NMOSD) will affect the optic nerve [optic neuritis (ON): unilateral or bilateral], spinal cord (myelitis), brain or brainstem, or a combination of the above. We report an interesting case of recurrent ON of the same eye for a total of 11 episodes in a Chinese woman. Over a period of 22 years, the attacks only involved the left eye, and never the right eye and also no myelitis. For a prolonged duration, she was diagnosed as recurrent idiopathic ON. Only until she was tested positive for aquaporin 4 antibody that her diagnosis was revised to NMOSD. Optical coherence tomography revealed thinning of the retinal nerve fibre layer (RNFL) for the affected left eye, while the RNFL thickness was within normal range for the unaffected right eye. The disability accrual in NMOSD is generally considered to be attack-related - without a clinical attack of ON, there shall be no visual impairment, and no significant subclinical thinning of RNFL. Our case is in agreement with this notion. This is in contrast to multiple sclerosis where subclinical RNFL thinning does occur. This case highlights the importance of revisiting and questioning a diagnosis of recurrent idiopathic ON particularly when new diagnostic tools are available. Copyright © 2016 Elsevier B.V. All rights reserved.

Efficacy of Polyvalent Human Immunoglobulins in an Animal Model of Neuromyelitis Optica Evoked by Intrathecal Anti-Aquaporin 4 Antibodies

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Benedikt Grünewald

2016-08-01

Full Text Available 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. Utilizing this animal model, we here tested the effects of systemically and intrathecally applied pooled human immunoglobulins (IVIg using a preventive and a therapeutic paradigm. In NMO-IgG animals, prophylactic application of systemic IVIg led to a reduced median disease score of 2.4 on a 0–10 scale, in comparison to 4.1 with sham treatment. Therapeutic IVIg, applied systemically after the 10th intrathecal NMO-IgG injection, significantly reduced the disease score by 0.8. Intrathecal IVIg application induced a beneficial effect in animals with NMO-IgG (median score IVIg 1.6 vs. sham 3.7 or with rAB-AQP4 (median score IVIg 2.0 vs. sham 3.7. We here provide evidence that treatment with IVIg ameliorates disease symptoms in this passive-transfer model, in analogy to former studies investigating passive-transfer animal models of other antibody-mediated disorders.

Projeto de um amplificador de transimpedancia monolitico implementado em GaAs com CAG para recepção em comunicações opticas

OpenAIRE

Valentino Corso

1998-01-01

Resumo: Este trabalho descreve o projeto e a implementação de um amplificador monolítico de transimpedância, para ser utilizado no estágio de pré-amplificação de receptores ópticos, operando em 51,84Mb/s. Protótipos do circuito projetado foram fabricados pela TriQuint Semiconductor, utilizando a tecnologia de Arseneto de Gálio (GaAs) com transistores MESFET de depleção de 0,5/mm. O amplificador desenvolvido tem características de baixo ruído, ganho de tensão de aproximadamente 30dB e baixo c...

Comparison study of imaging features of multiple sclerosis and neuromyelitis optica

International Nuclear Information System (INIS)

Liu Jianguo; Zhang Hailing; Zheng Kuihong; Zhang Wenluo; Dong Qinwen; Qi Xiaokun

2012-01-01

Objective: To compare the imaging characteristics of multiple sclerosis (MS) and neuromyelitis optica (NMO) for better diagnosis and differential diagnosis. Methods: The brain and spinal MRI images of 60 MS and 48 NMO cases were retrospectively reviewed. The imaging characteristics including the predilection site, morphological features, enhancement manifestations were summarized. All data was analyzed by using t test and Chi square test with SPSS 13.0. Results: (1) The three top predilection sites of brain in head MRI of MS patients were periventricular white matter (34 cases in 60), subcortical white matter (27 cases in 60), brain stem (23 cases in 60). MS lesions also were found in basal ganglia, cerebellum, corpus callosum and thalamus,as well as cortex (9 cases in 60). By contrast, brain lesions were observed in 59.4% (19/32) of NMO patients, and the three top predilection sites of NMO by turns were brain stem (13 cases in 19), periventricular white matter (12 cases in 19), subcortical white matter (7 cases in 19). Furthermore, the lesions surrounding third ventricle (6 cases in 19) and the tegmentum of brain stem near peri-aqueduct (8 cases in 19) in NMO were not found in patients of MS. The involvement of brain stem and thalamus was more frequent in NMO than in MS (χ 2 =5.267, 6.004, P<0.05, respectively). (2) The lesions of spinal cord in MS patients were typically oval, peripheral, and asymmetric, but in NMO patients they were longitudinally extensive and centrally located. The mean number of involved vertebral segments in NMO patients was significantly more than that in MS patients (7.3 vs 2.2, t=-9.288, P<0.01). Furthermore, the number of spinal cord lesions in MS patients was remarkably more than that in NMO (2.0 vs 1.3, t=4.565, P<0.01). The ratios of occurrence of spinal cord swelling and distension of NMO patients was 58.3% (28/48), which was significantly higher than 21.9% in MS (7/32, χ 2 =10.370, P<0.01). (3) The enhancement pattern in MS was

Comparisons of presentations and outcomes of neuromyelitis optica patients with and without Sjögren's syndrome.

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Zhong, Yu Hua; Zhong, Zhi Gang; Zhou, Zhou; Ma, Zhen Yu; Qiu, Meng Yao; Peng, Fu Hua; Zhang, Wei Xi

2017-02-01

Patients with neuromyelitis optica (NMO) often have an accompanying autoimmune disease, most commonly, but not limited to Sjögren's syndrome (SS). The aim of this study was to compare clinical and laboratory features between NMO patients with and without SS and to investigate the prognosis of NMO in patients with and without SS. Twenty-three NMO patients with SS and 42 NMO patients without SS were included. Clinical and laboratory profiles were compared, including annual relapse rate and time from onset of NMO to Expanded Disability Status Scale (EDSS) scores of 4.0 and 6.0. More NMO patients with SS than those without SS had anti-nuclear antibody, anti-SS-A/Ro and anti-SS-B/La antibodies (91.3 vs. 35.7%, p < 0.001, 87.0 vs. 2.3%, p < 0.001, and 34.8 vs. 0.0%, p < 0.001, respectively). Serum immunoglobulins (IgA, IgM and IgG) were markedly increased in NMO patients with SS in comparison with those without SS. Annual relapse rate and the time from disease onset to an EDSS score of 4.0 and 6.0 were not significantly different between the two groups. No differences between the two groups were found for the other parameters, including AQP-4 antibody status, length of spinal cord lesion and brain lesions. These results imply that NMO in SS more likely represents coexistence with SS rather than representing the result of direct central nervous system involvement in SS. Autoimmune response appears to be more intense in the NMO group with SS, but did not cause a more severe prognosis in comparison with the group without SS, indicating that we should pay attention to the potential benefit of the antinuclear antibodies in NMO.

Elevated Plasma Chemokines for Eosinophils in Neuromyelitis Optica Spectrum Disorders during Remission

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Yanping Tong

2018-02-01

Full Text Available BackgroundA prominent pathological feature of neuromyelitis optica spectrum disorders (NMOSD is markedly greater eosinophilic infiltration than that seen in other demyelinating diseases, like multiple sclerosis (MS. Eosinophils express the chemokine receptor CCR3, which is activated by eotaxins (CCL11/eotaxin-1, CCL24/eotaxin-2, CCL26/eotaxin-3 and CCL13 [monocyte chemoattractant protein (MCP-4]. Moreover, CCL13 is part of the chemokine set that activates CCR2. The present study aimed to evaluate plasma levels of eotaxins (CCL11, CCL24, and CCL26 and MCPs (CCL13, CCL2, CCL8, and CCL7 in patients with NMOSD during remission.MethodsHealthy controls (HC; n = 30 and patients with MS (n = 47 and NMOSD (n = 58 in remission were consecutively enrolled in this study between January 2016 and August 2017. Plasma CCL11, CCL24, CCL26, CCL2, CCL8, CCL7, CCL13, tumor necrosis factor (TNF-α, and interleukin (IL-1β levels were detected using the human cytokine multiplex assay.ResultsPlasma CCL13, CCL11, and CCL26 levels were all significantly higher in patients with NMOSD than in HC and patients with MS. No significant differences were found in the CCL13, CCL11, or CCL26 levels between patients with NMOSD receiving and not receiving immunosuppressive therapy. The plasma levels of TNF-α and IL-1β, which stimulate the above chemokines, were higher in patients with NMOSD than in HC. There was no difference in CCL24 levels among the three groups. In most cases, the CCL7 levels were below the threshold value of the human cytokine multiplex assay, which is in line with other studies. Adjusted multiple regression analyses showed a positive association of CCL13 levels with the number of relapses after controlling gender, age, body mass index, and disease duration in patients with NMOSD.ConclusionThe study indicates that in NMOSD, the overproduction of cytokines such as IL-1β and TNF-α during remission stimulates eosinophilic chemoattractants such as

Retinal ganglion cell-inner plexiform and nerve fiber layers in neuromyelitis optica

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Sai-Jing Hu

2018-01-01

Full Text Available AIM: To determine the thickness of the retinal ganglion cell-inner plexiform layer (GCIPL and the retinal nerve fiber layer (RNFL in patients with neuromyelitis optica (NMO. METHODS: We conducted a cross-sectional study that included 30 NMO patients with a total of 60 eyes. Based on the presence or absence of optic neuritis (ON, subjects were divided into either the NMO-ON group (30 eyes or the NMO-ON contra group (10 eyes. A detailed ophthalmologic examination was performed for each group; subsequently, the GCIPL and the RNFL were measured using high-definition optical coherence tomography (OCT. RESULTS: In the NMO-ON group, the mean GCIPL thickness was 69.28±21.12 μm, the minimum GCIPL thickness was 66.02±10.02 μm, and the RNFL thickness were 109.33±11.23, 110.47±3.10, 64.92±12.71 and 71.21±50.22 μm in the superior, inferior, temporal and nasal quadrants, respectively. In the NMO-ON contra group, the mean GCIPL thickness was 85.12±17.09 μm, the minimum GCIPL thickness was 25.39±25.1 μm, and the RNFL thicknesses were 148.33±23.22, 126.36±23.45, 82.21±22.30 and 83.36±31.28 μm in the superior, inferior, temporal and nasal quadrants, respectively. In the control group, the mean GCIPL thickness was 86.98±22.37 μm, the minimum GCIPL thickness was 85.28±10.75 μm, and the RNFL thicknesses were 150.22±22.73, 154.79±60.23, 82.33±7.01 and 85.62±13.81 μm in the superior, inferior, temporal and nasal quadrants, respectively. The GCIPL and RNFL were thinner in the NMO-ON contra group than in the control group (P<0.05; additionally, the RNFL was thinner in the inferior quadrant in the NMO-ON group than in the control group (P<0.05. Significant correlations were observed between the GCIPL and RNFL thickness measurements as well as between thickness measurements and the two visual field parameters of mean deviation (MD and corrected pattern standard deviation (PSD in the NMO-ON group (P<0.05. CONCLUSION: The thickness of the GCIPL

Comparison of grey matter atrophy between patients with neuromyelitis optica and multiple sclerosis: A voxel-based morphometry study

International Nuclear Information System (INIS)

Duan Yunyun; Liu Yaou; Liang Peipeng; Jia Xiuqin; Yu Chunshui; Qin Wen; Sun Hui; Liao Zhangyuan; Ye Jing; Li Kuncheng

2012-01-01

Purpose: Previous studies have established regional grey matter (GM) loss in multiple sclerosis (MS). However, whether there is any regional GM atrophy in neuromyelitis optica (NMO) and the difference between NMO and MS is unclear. The present study addresses this issue by voxel-based morphometry (VBM). Methods: Conventional magnetic resonance imaging (MRI) and T1-weighted three-dimensional MRI were obtained from 26 NMO patients, 26 relapsing–remitting MS (RRMS) patients, and 26 normal controls. An analysis of covariance model assessed with cluster size inference was used to compare GM volume among three groups. The correlations of GM volume changes with disease duration, expanded disability status scale (EDSS) and brain T2 lesion volume (LV) were analyzed. Results: GM atrophy was found in NMO patients in several regions of frontal, temporal, parietal lobes and insula (uncorrected, p < 0.001). While extensive GM atrophy was found in RRMS patients, including most cortical regions and the deep grey matter (corrected for multiple comparisons, p < 0.01). Compared with NMO, those with RRMS had significant GM loss in bilateral thalami, caudate, left parahippocampal gyrus, right hippocampus and insula (corrected, p < 0.01). In RRMS group, regional GM loss in right caudate and bilateral thalami were strongly correlated with brain T2LV. Conclusions: Our study found the difference of GM atrophy between NMO and RRMS patients mainly in deep grey matter. The correlational results suggested axonal degeneration from lesions on T2WI may be a key pathogenesis of atrophy in deep grey matter in RRMS.

Patients with neuromyelitis optica have a more severe disease than patients with relapsingremitting multiple sclerosis, including higher risk of dying of a demyelinating disease

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Denis Bernardi Bichuetti

2013-05-01

Full Text Available Although neuromyelitis optica (NMO is known to be a more severe disease than relapsing-remitting multiple sclerosis (RRMS, few studies comparing both conditions in a single center have been done. Methods: Comparison of our previously published cohort of 41 NMO patients with 177 RRMS patients followed in the same center, from 1994 to 2007. Results: Mean age of onset was 32.6 for NMO and 30.2 for RRMS (p=0.2062 with mean disease duration of 7.4 years for NMO and 10.3 years for RRMS. Patients with NMO had a higher annualized relapse rate (1.0 versus 0.8, p=0.0013 and progression index (0.9 versus 0.6, p≪0.0001, with more patients reaching expanded disability status scale (EDSS 6.0 (39 versus 17%, p=0.0036. The odds ratio for reaching EDSS 6.0 and being deceased due to NMO in comparison to RRMS were, respectively, 3.14 and 12.15. Conclusion: Patients with NMO have a more severe disease than patients with RRMS, including higher risk of dying of a demyelinating disease.

Estudio espectroscópico de la radiación electromagnética ultravioleta, visible e infrarroja cercana sobre la obra pictórica.

OpenAIRE

D'ANTONI, PABLO LUIS

2013-01-01

La Tesis "Estudio Espectroscópico de la Radiación Electromagnética, Ultravioleta Visible e Infrarroja cercana sobre la Obra Pictórica" de Pablo D'Antoni desarrolla un método no invasivo para caracterizar, medir y predecir daños a los pigmentos y aglutinantes en obras pictóricas, probablemente por la radiación electromagnética (REM). Usando 3 formas de Espectroscopia: 1) Irradiancia, 2) FORS(Espectroscopía de Reflectancia (y Absorbancia) con Fibra Optica, se analizan los efectos producidos por...

Comparative brain stem lesions on MRI of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis.

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Zhengqi Lu

Full Text Available BACKGROUND: Brain stem lesions are common in patients with acute disseminated encephalomyelitis (ADEM, neuromyelitis optica (NMO, and multiple sclerosis (MS. OBJECTIVES: To investigate comparative brain stem lesions on magnetic resonance imaging (MRI among adult patients with ADEM, NMO, and MS. METHODS: Sixty-five adult patients with ADEM (n = 17, NMO (n = 23, and MS (n = 25 who had brain stem lesions on MRI were enrolled. Morphological features of brain stem lesions among these diseases were assessed. RESULTS: Patients with ADEM had a higher frequency of midbrain lesions than did patients with NMO (94.1% vs. 17.4%, P<0.001 and MS (94.1% vs. 40.0%, P<0.001; patients with NMO had a lower frequency of pons lesions than did patients with MS (34.8% vs. 84.0%, P<0.001 and ADEM (34.8% vs. 70.6%, P = 0.025; and patients with NMO had a higher frequency of medulla oblongata lesions than did patients with ADEM (91.3% vs. 35.3%, P<0.001 and MS (91.3% vs. 36.0%, P<0.001. On the axial section of the brain stem, the majority (82.4% of patients with ADEM showed lesions on the ventral part; the brain stem lesions in patients with NMO were typically located in the dorsal part (91.3%; and lesions in patients with MS were found in both the ventral (44.0% and dorsal (56.0% parts. The lesions in patients with ADEM (100% and NMO (91.3% had poorly defined margins, while lesions of patients with MS (76.0% had well defined margins. Brain stem lesions in patients with ADEM were usually bilateral and symmetrical (82.4%, while lesions in patients with NMO (87.0% and MS (92.0% were asymmetrical or unilateral. CONCLUSIONS: Brain stem lesions showed various morphological features among adult patients with ADEM, NMO, and MS. The different lesion locations may be helpful in distinguishing these diseases.

Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report

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Pavlisa Goran

2009-10-01

Full Text Available Abstract Background Neuromyelitis optica (NMO is an idiopathic, severe, inflammatory demyelinating disease of the central nervous system, that causes severe optic neuritis and myelitis attacks. Early discrimination between multiple sclerosis (MS and NMO is important, as optimum treatment for both diseases may differ considerably. Case Presentation We report a case of a patient who initially presented as longitudinally extensive transverse myelitis (LETM, having spastic upper extremities diparesis and spastic paraplegia, C2/C3 sensory level and urinary incontinence, as well as extensive inflammatory spinal cord lesions from C2 level to conus. After 5 months the patient had another attack of transverse myelitis, had electrophysiological findings consistent with optic neuritis, was seropositive for NMO-IgG (aquaporin-4 IgG and thus fulfilled NMO diagnostic criteria. Following treatment of disease attacks with pulse corticosteroid therapy and intravenous immunoglobulins, we included oral azathioprine in a combination with oral prednisone in the therapy. Since there was no significant clinical improvement, we decided to use cyclophosphamide therapy, which resulted in good clinical improvement and gradual decrease of cord swelling. Conclusion In this NMO case report we wanted to emphasize the extensiveness of inflammatory spinal cord changes in our patient, from C2 level to conus. In the conclusion it is important to say that accurate, early diagnosis and distinction from MS is critical to facilitate initiation of immunosuppressive therapy for attack prevention.

Prevalence of neuromyelitis optica spectrum disorder in the multi-ethnic Penang Island, Malaysia, and a review of worldwide prevalence.

Science.gov (United States)

Hor, Jyh Yung; Lim, Thien Thien; Chia, Yuen Kang; Ching, Yee Ming; Cheah, Chun Fai; Tan, Kenny; Chow, Han Bing; Arip, Masita; Eow, Gaik Bee; Easaw, P E Samuel; Leite, M Isabel

2018-01-01

Neuromyelitis optica spectrum disorder (NMOSD) occurs worldwide in all ethnicities. Recently, population-based studies have shown that NMOSD is more common among non-White populations. There is scarce data about NMOSD prevalence in South East Asian populations. (1) A population-based study was undertaken to estimate NMOSD prevalence in the multi-ethnic Penang Island, Malaysia, comprising Chinese, Malays, and Indians. Medical records of NMOSD patients followed up at the Penang General Hospital (the neurology referral centre in Penang Island) were reviewed. The 2015 diagnostic criteria of the International Panel for NMO Diagnosis were used for case ascertainment. (2) A review of population-based prevalence studies of NMOSD worldwide was carried out. PubMed and conference proceedings were searched for such studies. Of the 28 NMOSD patients, 14 were residents of Penang Island on prevalence day [13 (93%) Chinese and one (7%) Malay]. All 14 patients were females and aquaporin 4 seropositive. The prevalence of NMOSD in Penang Island was 1.99/100,000 population; according to ethnicities, the prevalence in Chinese was significantly higher than in Malays (3.31/100,000 vs 0.43/100,000, respectively, p = 0.0195). Based on our and other population-based studies, among Asians, East Asian origin populations (Chinese and Japanese) appear to have higher NMOSD prevalence than other Asian ethnic groups. Worldwide, Blacks seem to have the highest NMOSD prevalence. More studies in different geographical regions and ethnic groups will be useful to further inform about potential factors in NMOSD pathogenesis. Copyright © 2017 Elsevier B.V. All rights reserved.

Para-Hermitian and para-quaternionic manifolds

International Nuclear Information System (INIS)

Ivanov, S.; Zamkovoy, S.

2003-10-01

A set of canonical para-Hermitian connections on an almost para-Hermitian manifold is defined. A Para-hermitian version of the Apostolov-Gauduchon generalization of the Goldberg-Sachs theorem in General Relativity is given. It is proved that the Nijenhuis tensor of a Nearly para-Kaehler manifolds is parallel with respect to the canonical connection. Salamon's twistor construction on quaternionic manifold is adapted to the para-quaternionic case. A locally conformally hyper-para-Kaehler (hypersymplectic) flat structure with parallel Lee form on the Kodaira-Thurston complex surfaces modeled on S 1 x SL (2, R)-tilde is constructed. Anti-self-dual locally conformally hyper-para-Kaehler (hypersymplectic) neutral metrics with non vanishing Weyl tensor are obtained on the Inoe surfaces. An example of anti-self-dual neutral metric which is not locally conformally hyper-para-Kaehler (hypersymplectic) is constructed. (author)

Para-Hermitian and para-quaternionic manifolds

Energy Technology Data Exchange (ETDEWEB)

Ivanov, S [University of Sofia ' St. Kl. Ohridski' , Faculty of Mathematics and Informatics, Sofia (Bulgaria) and Abdus Salam International Centre for Theoretical Physics, Trieste (Italy); Zamkovoy, S [University of Sofia ' St. Kl. Ohridski' , Faculty of Mathematics and Informatics, Sofia (Bulgaria)

2003-10-01

A set of canonical para-Hermitian connections on an almost para-Hermitian manifold is defined. A Para-hermitian version of the Apostolov-Gauduchon generalization of the Goldberg-Sachs theorem in General Relativity is given. It is proved that the Nijenhuis tensor of a Nearly para-Kaehler manifolds is parallel with respect to the canonical connection. Salamon's twistor construction on quaternionic manifold is adapted to the para-quaternionic case. A locally conformally hyper-para-Kaehler (hypersymplectic) flat structure with parallel Lee form on the Kodaira-Thurston complex surfaces modeled on S{sup 1} x SL (2, R)-tilde is constructed. Anti-self-dual locally conformally hyper-para-Kaehler (hypersymplectic) neutral metrics with non vanishing Weyl tensor are obtained on the Inoe surfaces. An example of anti-self-dual neutral metric which is not locally conformally hyper-para-Kaehler (hypersymplectic) is constructed. (author)

The major brain endocannabinoid 2-AG controls neuropathic pain and mechanical hyperalgesia in patients with neuromyelitis optica.

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Hannah L Pellkofer

Full Text Available Recurrent myelitis is one of the predominant characteristics in patients with neuromyelitis optica (NMO. While paresis, visual loss, sensory deficits, and bladder dysfunction are well known symptoms in NMO patients, pain has been recognized only recently as another key symptom of the disease. Although spinal cord inflammation is a defining aspect of neuromyelitis, there is an almost complete lack of data on altered somatosensory function, including pain. Therefore, eleven consecutive patients with NMO were investigated regarding the presence and clinical characteristics of pain. All patients were examined clinically as well as by Quantitative Sensory Testing (QST following the protocol of the German Research Network on Neuropathic Pain (DFNS. Additionally, plasma endocannabinoid levels and signs of chronic stress and depression were determined. Almost all patients (10/11 suffered from NMO-associated neuropathic pain for the last three months, and 8 out of 11 patients indicated relevant pain at the time of examination. Symptoms of neuropathic pain were reported in the vast majority of patients with NMO. Psychological testing revealed signs of marked depression. Compared to age and gender-matched healthy controls, QST revealed pronounced mechanical and thermal sensory loss, strongly correlated to ongoing pain suggesting the presence of deafferentation-induced neuropathic pain. Thermal hyperalgesia correlated to MRI-verified signs of spinal cord lesion. Heat hyperalgesia was highly correlated to the time since last relapse of NMO. Patients with NMO exhibited significant mechanical and thermal dysesthesia, namely dynamic mechanical allodynia and paradoxical heat sensation. Moreover, they presented frequently with either abnormal mechanical hypoalgesia or hyperalgesia, which depended significantly on plasma levels of the endogenous cannabinoid 2-arachidonoylglycerole (2-AG. These data emphasize the high prevalence of neuropathic pain and hyperalgesia

The Urine Proteome Profile Is Different in Neuromyelitis Optica Compared to Multiple Sclerosis: A Clinical Proteome Study.

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Helle H Nielsen

Full Text Available Inflammatory demyelinating diseases of the CNS comprise a broad spectrum of diseases like neuromyelitis optica (NMO, NMO spectrum disorders (NMO-SD and multiple sclerosis (MS. Despite clear classification criteria, differentiation can be difficult. We hypothesized that the urine proteome may differentiate NMO from MS.The proteins in urine samples from anti-aquaporin 4 (AQP4 seropositive NMO/NMO-SD patients (n = 32, patients with MS (n = 46 and healthy subjects (HS, n = 31 were examined by quantitative liquid chromatography-tandem mass spectrometry (LC-MS/MS after trypsin digestion and iTRAQ labelling. Immunoglobulins (Ig in the urine were validated by nephelometry in an independent cohort (n = 9-10 pr. groups.The analysis identified a total of 1112 different proteins of which 333 were shared by all 109 subjects. Cluster analysis revealed differences in the urine proteome of NMO/NMO-SD compared to HS and MS. Principal component analysis also suggested that the NMO/NMO-SD proteome profile was useful for classification. Multivariate regression analysis revealed a 3-protein profile for the NMO/NMO-SD versus HS discrimination, a 6-protein profile for NMO/NMO-SD versus MS discrimination and an 11-protein profile for MS versus HS discrimination. All protein panels yielded highly significant ROC curves (AUC in all cases >0.85, p≤0.0002. Nephelometry confirmed the presence of increased Ig-light chains in the urine of patients with NMO/NMO-SD.The urine proteome profile of patients with NMO/NMO-SD is different from MS and HS. This may reflect differences in the pathogenesis of NMO/NMO-SD versus MS and suggests that urine may be a potential source of biomarkers differentiating NMO/NMO-SD from MS.

Severe structural and functional visual system damage leads to profound loss of vision-related quality of life in patients with neuromyelitis optica spectrum disorders.

Science.gov (United States)

Schmidt, Felix; Zimmermann, Hanna; Mikolajczak, Janine; Oertel, Frederike C; Pache, Florence; Weinhold, Maria; Schinzel, Johann; Bellmann-Strobl, Judith; Ruprecht, Klemens; Paul, Friedemann; Brandt, Alexander U

2017-01-01

Neuromyelitis optica spectrum disorders (NMOSD) are characterized by devastating optic neuritis attacks causing more structural damage and visual impairment than in multiple sclerosis (MS). The objective of this study was to compare vision-related quality of life in NMOSD and MS patients and correlate it to structural retinal damage and visual function. Thirty-one NMOSD and 31 matched MS patients were included. Vision-related quality of life was assessed with the 39-item National Eye Institute Visual Function Questionnaire (NEI-VFQ). All patients underwent retinal optical coherence tomography and visual acuity and contrast sensitivity measurements. Vision-related quality of life was reduced in NMOSD compared to MS patients. This difference was driven by a higher incidence of bilateral and more severe optic neuritis in the NMOSD group. Retinal thinning and visual impairment were significantly greater in the NMOSD cohort. Lower vision-related quality of life was associated with more retinal damage and reduced visual function as assessed by visual acuity and contrast sensitivity. NMOSD-related bilateral ON-attacks cause severe structural damage and visual impairment that lead to severe loss of vision-related quality of life. The NEI-VFQ is a helpful tool to monitor vision-related quality of life in NMOSD patients. Copyright © 2016 Elsevier B.V. All rights reserved.

Relapsing-Remitting MS (RRMS)

Medline Plus

Full Text Available ... Associated Myelopathy (HAM) Neuromyelitis Optica (NMO) Schilder's Disease Transverse Myelitis d Symptoms & Diagnosis d Diagnosing MS d ... Overview (.pdf) Download Document Pediatric MS Learn More Transverse Myelitis Learn More Neuromyelitis Optica (NMO) Learn More ...

Characteristic cerebrospinal fluid cytokine/chemokine profiles in neuromyelitis optica, relapsing remitting or primary progressive multiple sclerosis.

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Takuya Matsushita

Full Text Available BACKGROUND: Differences in cytokine/chemokine profiles among patients with neuromyelitis optica (NMO, relapsing remitting multiple sclerosis (RRMS, and primary progressive MS (PPMS, and the relationships of these profiles with clinical and neuroimaging features are unclear. A greater understanding of these profiles may help in differential diagnosis. METHODS/PRINCIPAL FINDINGS: We measured 27 cytokines/chemokines and growth factors in CSF collected from 20 patients with NMO, 26 with RRMS, nine with PPMS, and 18 with other non-inflammatory neurological diseases (OND by multiplexed fluorescent bead-based immunoassay. Interleukin (IL-17A, IL-6, CXCL8 and CXCL10 levels were significantly higher in NMO patients than in OND and RRMS patients at relapse, while granulocyte-colony stimulating factor (G-CSF and CCL4 levels were significantly higher in NMO patients than in OND patients. In NMO patients, IL-6 and CXCL8 levels were positively correlated with disability and CSF protein concentration while IL-6, CXCL8, G-CSF, granulocyte-macrophage colony-stimulating factor (GM-CSF and IFN-γ were positively correlated with CSF neutrophil counts at the time of sample collection. In RRMS patients, IL-6 levels were significantly higher than in OND patients at the relapse phase while CSF cell counts were negatively correlated with the levels of CCL2. Correlation coefficients of cytokines/chemokines in the relapse phase were significantly different in three combinations, IL-6 and GM-CSF, G-CSF and GM-CSF, and GM-CSF and IFN-γ, between RRMS and NMO/NMOSD patients. In PPMS patients, CCL4 and CXCL10 levels were significantly higher than in OND patients. CONCLUSIONS: Our findings suggest distinct cytokine/chemokine alterations in CSF exist among NMO, RRMS and PPMS. In NMO, over-expression of a cluster of Th17- and Th1-related proinflammatory cytokines/chemokines is characteristic, while in PPMS, increased CCL4 and CXCL10 levels may reflect on-going low grade T cell

Relapsing-Remitting MS (RRMS)

Medline Plus

Full Text Available ... ADEM) Balo’s Disease HTLV-I Associated Myelopathy (HAM) Neuromyelitis Optica (NMO) Schilder's Disease Transverse Myelitis d Symptoms & Diagnosis ... Other Conditions to Rule Out Lyme Disease Lupus Neuromyelitis Optica Acute Disseminated Encephalomyelitis (ADEM) d For Clinicians d ...

Disease: H01717 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available . Many cases of optic neuritis are associated with multiple sclerosis or neuromyelitis optica or can occur i...feron beta [DR:D04554 D00746] Glatiramer acetate [DR:D04318] See also H01490 Multiple sclerosis and H01491 Neuromyelitis optica

Comparison of brain and spinal cord magnetic resonance imaging features in neuromyelitis optica spectrum disorders patients with or without aquaporin-4 antibody.

Science.gov (United States)

Fan, Moli; Fu, Ying; Su, Lei; Shen, Yi; Wood, Kristofer; Yang, Li; Liu, Yaou; Shi, Fu-Dong

2017-04-01

The spinal cord and brain measurements are rarely investigated in neuromyelitis optica (NMO) patients with and without antibodies to aquaporin-4 (AQP4), directly compared to multiple sclerosis (MS) patients. To investigate magnetic resonance imaging (MRI) features of both brain and spinal cord in NMO patients with and without antibodies to AQP4, compared with MS patients and healthy controls (HC). We recruited 55 NMO including 30 AQP4 (+) and 25 AQP4 (-), 25 MS and 25 HC. Brain and spinal cord MRIs were obtained for each participant. Brain lesions (BL), whole brain and deep grey matter volumes (DGMV), white matter diffusion metrics and spinal cord lesions were measured and compared among groups. The incidence of BL was lower in the AQP4 (+) group than in the AQP4 (-) and MS groups (p<0.05). In the AQP4 (+) group, there was a lower incidence of infratentorial lesions (ITL) and higher spinal cord lesions length than in the MS group (p<0.05). The thalamic and hippocampal volumes were smaller in the AQP4 (-) group and MS group than in the HC group (p<0.05). The NMO patients with AQP4 (-) showed higher prevalence of BL, ITL, and similar spinal cord lesion length, compared to AQP4 (+), and demonstrated deep grey matter atrophy, suggesting an intermediate phenotype between that of typical MS and NMO. Copyright © 2017 Elsevier B.V. All rights reserved.

Recombinant IgG1 Fc hexamers block cytotoxicity and pathological changes in experimental in vitro and rat models of neuromyelitis optica.

Science.gov (United States)

Tradtrantip, Lukmanee; Felix, Christian M; Spirig, Rolf; Morelli, Adriana Baz; Verkman, A S

2018-05-01

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. Fc hexamers at low concentration also prevented antibody-dependent cellular cytotoxicity produced by AQP4-IgG and natural killer cells. Serum from rats administered a single intravenous dose of Fc hexamers at 50 mg/kg taken at 8 h did not produce complement-dependent cytotoxicity when added to AQP4-IgG-treated AQP4-expressing cell cultures. In an experimental rat model of NMO produced by intracerebral injection of AQP4-IgG, Fc hexamers at 50 mg/kg administered before and at 12 h after AQP4-IgG fully prevented astrocyte injury, complement activation, inflammation and demyelination. These results support the potential therapeutic utility of recombinant IgG1 Fc hexamers in AQP4-IgG seropositive NMO. Copyright © 2018 Elsevier Ltd. All rights reserved.

Neuromyelitis optica study model based on chronic infusion of autoantibodies in rat cerebrospinal fluid.

Science.gov (United States)

Marignier, R; Ruiz, A; Cavagna, S; Nicole, A; Watrin, C; Touret, M; Parrot, S; Malleret, G; Peyron, C; Benetollo, C; Auvergnon, N; Vukusic, S; Giraudon, P

2016-05-18

Devic's neuromyelitis optica (NMO) is an autoimmune astrocytopathy, associated with central nervous system inflammation, demyelination, and neuronal injury. Several studies confirmed that autoantibodies directed against aquaporin-4 (AQP4-IgG) are relevant in the pathogenesis of NMO, mainly through complement-dependent toxicity leading to astrocyte death. However, the effect of the autoantibody per se and the exact role of intrathecal AQP4-IgG are still controversial. To explore the intrinsic effect of intrathecal AQP4-IgG, independent from additional inflammatory effector mechanisms, and to evaluate its clinical impact, we developed a new animal model, based on a prolonged infusion of purified immunoglobulins from NMO patient (IgG(AQP4+), NMO-rat) and healthy individual as control (Control-rat) in the cerebrospinal fluid (CSF) of live rats. We showed that CSF infusion of purified immunoglobulins led to diffusion in the brain, spinal cord, and optic nerves, the targeted structures in NMO. This was associated with astrocyte alteration in NMO-rats characterized by loss of aquaporin-4 expression in the spinal cord and the optic nerves compared to the Control-rats (p = 0.001 and p = 0.02, respectively). In addition, glutamate uptake tested on vigil rats was dramatically reduced in NMO-rats (p = 0.001) suggesting that astrocytopathy occurred in response to AQP4-IgG diffusion. In parallel, myelin was altered, as shown by the decrease of myelin basic protein staining by up to 46 and 22 % in the gray and white matter of the NMO-rats spinal cord, respectively (p = 0.03). Loss of neurofilament positive axons in NMO-rats (p = 0.003) revealed alteration of axonal integrity. Then, we investigated the clinical consequences of such alterations on the motor behavior of the NMO-rats. In a rotarod test, NMO-rats performance was lower compared to the controls (p = 0.0182). AQP4 expression, and myelin and axonal integrity were preserved in AQP4-Ig

Autologous mesenchymal stem cells applied on the pressure ulcers had produced a surprising outcome in a severe case of neuromyelitis optica

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Adriana Octaviana Dulamea

2015-01-01

Full Text Available Recent studies provided evidence that mesenchymal stem cells (MSCs have regenerative potential in cutaneous repair and profound immunomodulatory properties making them a candidate for therapy of neuroimmunologic diseases. Neuromyelitis optica (NMO is an autoimmune, demyelinating central nervous system disorder characterized by a longitudinally extensive spinal cord lesion. A 46-year-old male diagnosed with NMO had relapses with paraplegia despite treatment and developed two stage IV pressure ulcers (PUs on his legs. The patient consented for local application of autologous MSCs on PUs. MSCs isolated from the patient′s bone marrow aspirate were multiplied in vitro during three passages and embedded in a tridimensional collagen-rich matrix which was applied on the PUs. Eight days after MSCs application the patient showed a progressive healing of PUs and improvement of disability. Two months later the patient was able to walk 20 m with bilateral assistance and one year later he started to walk without assistance. For 76 months the patient had no relapse and no adverse event was reported. The original method of local application of autologous BM-MSCs contributed to healing of PUs. For 6 years the patient was free of relapses and showed an improvement of disability. The association of cutaneous repair, sustained remission of NMO and improvement of disability might be explained by a promotion/optimization of recovery mechanisms in the central nervous system even if alternative hypothesis should be considered. Further studies are needed to assess the safety and efficacy of mesenchymal stem cells in NMO treatment.

Multicentre comparison of a diagnostic assay

DEFF Research Database (Denmark)

Waters, Patrick; Reindl, Markus; Saiz, Albert

2016-01-01

) assays in neuromyelitis optica spectrum disorders (NMOSD). METHODS: Coded samples from patients with neuromyelitis optica (NMO) or NMOSD (101) and controls (92) were tested at 15 European diagnostic centres using 21 assays including live (n=3) or fixed cell-based assays (n=10), flow cytometry (n=4...

Astrocytic autoantibody of neuromyelitis optica (NMO-IgG) binds to aquaporin-4 extracellular loops, monomers, tetramers and high order arrays

Science.gov (United States)

Iorio, Raffaele; Fryer, James P.; Hinson, Shannon R.; Fallier-Becker, Petra; Wolburg, Hartwig; Pittock, Sean J.; Lennon, Vanda A.

2012-01-01

The principal central nervous system (CNS) water channel, aquaporin-4 (AQP4), is confined to astrocytic and ependymal membranes and is the target of a pathogenic autoantibody, neuromyelitis optica (NMO)-IgG. This disease-specific autoantibody unifies a spectrum of relapsing CNS autoimmune inflammatory disorders of which NMO exemplifies the classic phenotype. Multiple sclerosis and other immune-mediated demyelinating disorders of the CNS lack a distinctive biomarker. Two AQP4 isoforms, M1 and M23, exist as homotetrameric and heterotetrameric intramembranous particles (IMPs). Orthogonal arrays of predominantly M23 particles (OAPs) are an ultrastructural characteristic of astrocytic membranes. We used high-titered serum from 32 AQP4-IgG-seropositive patients and 85 controls to investigate the nature and molecular location of AQP4 epitopes that bind NMO-IgG, and the influence of supramolecular structure. NMO-IgG bound to denatured AQP4 monomers (68% of cases), to native tetramers and high order arrays (90% of cases), and to AQP4 in live cell membranes (100% of cases). Disease-specific epitopes reside in extracellular loop C more than in loops A or E. IgG binding to intracellular epitopes lacks disease specificity. These observations predict greater disease specificity and sensitivity for tissue-based and cell-based serological assays employing “native” AQP4 than assays employing denatured AQP4 and fragments. NMO-IgG binds most avidly to plasma membrane surface AQP4 epitopes formed by loop interactions within tetramers and by intermolecular interactions within high order structures. The relative abundance and localization of AQP4 high order arrays in distinct CNS regions may explain the variability in clinical phenotype of NMO spectrum disorders. PMID:22906356

Tolerogenic Dendritic Cells as a Promising Antigen-Specific Therapy in the Treatment of Multiple Sclerosis and Neuromyelitis Optica From Preclinical to Clinical Trials

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Georgina Flórez-Grau

2018-05-01

Full Text Available The identification of activated T-lymphocytes restricted to myelin-derived immunogenic peptides in multiple sclerosis (MS and aquaporin-4 water channel in neuromyelitis optica (NMO in the blood of patients opened the possibility for developing highly selective and disease-specific therapeutic approaches. Antigen presenting cells and in particular dendritic cells (DCs represent a strategy to inhibit pro-inflammatory T helper cells. DCs are located in peripheral and lymphoid tissues and are essential for homeostasis of T cell-dependent immune responses. The expression of a particular set of receptors involved in pathogen recognition confers to DCs the property to initiate immune responses. However, in the absence of danger signals different DC subsets have been revealed to induce active tolerance by inducing regulatory T cells, inhibiting pro-inflammatory T helper cells responses or both. Interestingly, several protocols to generate clinical-grade tolerogenic DC (Tol-DC in vitro have been described, offering the possibility to restore the homeostasis to central nervous system-related antigens. In this review, we discuss about different DC subsets and their role in tolerance induction, the different protocols to generate Tol-DCs and preclinical studies in animal models as well as describe recent characterization of Tol-DCs for clinical application in autoimmune diseases and in particular in MS and NMO patients. In addition, we discuss the clinical trials ongoing based on Tol-DCs to treat different autoimmune diseases.

Remote-actuator used in environments for high electromagnetic interference; Actuador telecontrolado para ambientes de alta interferencia electromagnetica

Energy Technology Data Exchange (ETDEWEB)

Perez Abad, Carlos Alberto; Velazquez Hernandez, Jose Conrado; Montero Cervantes, Julio Cesar [Instituto de Investigaciones Electricas, Cuernavaca, Morelos (Mexico)]. E-mail: caperez@iie.org.mx; jconrado@iie.org.mx; jcmc@iie.org.mx

2010-11-15

This article presents the design of an actuator telecontrolled used in environments of high electromagnetic interference, especially designed for high-power laboratories Comision Federal de Electricidad, LAPEM (Laboratorio de pruebas de equipos y materiales) basically the actuator is an electronic component in a series of hardware and software involved in short circuit tests performed LAPEM but because of its design can be used in any industry involving fire remotely due to the electromagnetic pollution caused by high currents and voltages involved in the tests, the means of communication between the actuator and programmable controller sequences (CPS) is optical fiber that ensures the integrity of the control signal that activates the device. We present the electronic modules that comprise it and the evidence and findings. [Spanish] En este articulo se presenta el diseno de un actuador telecontrolado utilizado en ambientes de alta interferencia electromagnetica, en especial fue disenado para los laboratorios de alta potencia del LAPEM (Laboratorio de pruebas de equipos y materiales), Comision Federal de Electricidad, basicamente el actuador es un componente electronico de una serie de hardware y software involucrados en las pruebas de corto circuito que realiza el LAPEM pero debido a su diseno puede ser utilizado en cualquier otra industria que involucre disparos en forma remota, debido a la contaminacion electromagnetica causada por las altas corrientes y voltajes involucradas en las pruebas, el medio de comunicacion entre el actuador y el Controlador programable de Secuencias (CPS) es fibra optica con esto se asegura la integridad de la senal de control que activa el dispositivo. Se presenta los modulos electronicos que lo componen asi como las pruebas y resultados obtenidos.

Iron deposition of the deep grey matter in patients with multiple sclerosis and neuromyelitis optica: A control quantitative study by 3D-enhanced susceptibility-weighted angiography (ESWAN)

International Nuclear Information System (INIS)

Chen Xuan; Zeng Chun; Luo Tianyou; Ouyang Yu; Lv Fajin; Rumzan, Reshiana; Wang Zhongping; Li Qi; Wang Jingjie; Hou Huanxin

2012-01-01

Purpose: Previous studies have detected abnormal iron deposition in the deep grey matter (DGM) of multiple sclerosis (MS). The regional specificity of the DGM iron deposition in neuromyelitis optica (NMO) is still unclear. We compared the differences in the DGM iron concentration between MS and NMO patients. Methods: We enrolled 42 relapsing–remitting MS (RRMS) patients, 42 NMO patients and 42 healthy controls undergoing brain conventional MRI and three-dimensional (3D)-enhanced T 2 *-weighted angiography (ESWAN) sequences. We obtained the mean phase values (MPVs) for ESWAN-filtered phase images. An analysis of covariance (ANCOVA) was used to compare MPVs among three groups. The correlations of MPVs changes with disease duration and expanded disability status scale (EDSS) were analyzed. Results: The RRMS patients had higher DGM iron concentration than did the NMO and control groups, but only the bilateral substantia nigra (SN) showed a significant statistical difference among three groups (p 0.05). Furthermore, no correlations were found between the DGM iron concentration and EDSS (p > 0.05). Conclusions: We confirm the iron concentration in the DGM iron content of MS patients is more than NMO patients and healthy controls in the same age range. Furthermore, the disease duration was found to be a significant contributor to patients with MS.

Role of AQP4 Antibody Serostatus and its Prediction of Visual Outcome in Neuromyelitis Optica: A Systematic Review and Meta-Analysis.

Science.gov (United States)

Lin, Nan; Liu, Qing; Wang, Xiaoyu; Ma, Jianmei; Li, Yuyuan

2017-01-01

Backgroud: Neuromyelitis optica (NMO) is an autoimmune inflammatory disorder, which is characterized by severe attacks of optic neuritis and myelitis. Antibodies (Ab) to aquaporin-4 (AQP4) (or NMO-IgG) as a serological biomarker of NMO have been widespread used. Nevertheless, some NMO patients remain seronegative for AQP4-Ab and/or have no detected optic nerve involvement. In addition, no consensus exists on the association between AQP4-Ab serostatus and visual outcome in NMO. To drive a more precise estimate of this postulated relationship, a metaanalysis was performed based on existing relevant studies. Studies were searched by PubMed and MEDLINE up to March 2016. Study quality was assessed, and meta-analysis was conducted using the RevMan 5.1. Odds ratios with 95% confidence interval were calculated and funnel plot was applied to assess the potential publication bias. In a total of 1288 relevant studies, 18 studies satisfied the eligibility criteria and were included in the systemic review. Only 9 studies appeared eligible for the meta-analysis, together including 624 AQP4-Ab-positive and 119 AQP4-Ab-negative NMO patients. The results revealed associations between AQP4-Ab seropositivity and visual impairment in NMO (OR, 3.16; 95% CI, 1.09, 9.19; P = 0.03). The results of subgroup analyses based on different methods of AQP-4 detection also showed significantly differences between AQP4-Ab seropositivity and visual impairment in NMO, especially in CBA subgroup. This meta-analysis indicates that AQP4-Ab serostatus has the positive with poor visual outcome in NMO. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Aquaporin-4 autoantibodies in neuromyelitis optica spectrum disorders: comparison between tissue-based and cell-based indirect immunofluorescence assays

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Chan Koon H

2010-09-01

Full Text Available Abstract Background Neuromyelitis optica spectrum disorders (NMOSD are severe central nervous system inflammatory demyelinating disorders (CNS IDD characterized by monophasic or relapsing, longitudinally extensive transverse myelitis (LETM and/or optic neuritis (ON. A significant proportion of NMOSD patients are seropositive for aquaporin-4 (AQP4 autoantibodies. We compared the AQP4 autoantibody detection rates of tissue-based indirect immunofluorescence assay (IIFA and cell-based IIFA. Methods Serum of Chinese CNS IDD patients were assayed for AQP4 autoantibodies by tissue-based IIFA using monkey cerebellum and cell-based IIFA using transfected HEK293 cells which express human AQP4 on their cell membranes. Results In total, 128 CNS IDD patients were studied. We found that 78% of NMO patients were seropositive for AQP4 autoantibodies by cell-based IIFA versus 61% by tissue-based IFA (p = 0.250, 75% of patients having relapsing myelitis (RM with LETM were seropositive by cell-based IIFA versus 50% by tissue-based IIFA (p = 0.250, and 33% of relapsing ON patients were seropositive by cell-based IIFA versus 22% by tissue-based IIFA (p = 1.000; however the differences were not statistically significant. All patients seropositive by tissue-based IIFA were also seropositive for AQP4 autoantibodies by cell-based IIFA. Among 29 NMOSD patients seropositive for AQP4 autoantibodies by cell-based IIFA, 20 (69% were seropositive by tissue-based IIFA. The 9 patients seropositive by cell-based IIFA while seronegative by tissue-based IIFA had NMO (3, RM with LETM (3, a single attack of LETM (1, relapsing ON (1 and a single ON attack (1. Among 23 NMO or RM patients seropositive for AQP4 autoantibodies by cell-based IIFA, comparison between those seropositive (n = 17 and seronegative (n = 6 by tissue-based IIFA revealed no differences in clinical and neuroradiological characteristics between the two groups. Conclusion Cell-based IIFA is slightly more sensitive

Optimizing the management of neuromyelitis optica and spectrum disorders in resource poor settings: Experience from the Mangalore demyelinating disease registry

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Lekha Pandit

2013-01-01

Full Text Available Background: In resource-poor settings, the management of neuromyelitis optica (NMO and NMO spectrum (NMOS disorders is limited because of delayed diagnosis and financial constraints. Aim: To device a cost-effective strategy for the management of NMO and related disorders in India. Materials and Methods: A cost-effective and disease-specific protocol was used for evaluating the course and treatment outcome of 70 consecutive patients. Results: Forty-five patients (65% had a relapse from the onset and included NMO (n = 20, recurrent transverse myelitis (RTM; n = 10, and recurrent optic neuritis (ROPN; n = 15. In 38 (84.4% patients presenting after multiple attacks, the diagnosis was made clinically. Only 7 patients with a relapsing course were seen at the onset and included ROPN (n = 5, NMO (n = 1, and RTM (n = 1. They had a second attack after a median interval of 1 ± 0.9 years, which was captured through our dedicated review process. Twenty-five patients had isolated longitudinally extensive transverse myelitis (LETM, of which 20 (80% remained ambulant at follow-up of 3 ± 1.9 years. Twelve patients (17% with median expanded disability status scale (EDSS of 8.5 at entry had a fatal outcome. Serum NMO-IgG testing was done in selected patients, and it was positive in 7 of 18 patients (39%. Irrespective of the NMO-IgG status, the treatment compliant patients (44.4% showed significant improvement in EDSS (P ≤ 0.001. Conclusions : Early clinical diagnosis and treatment compliance were important for good outcome. Isolated LETM was most likely a post-infectious demyelinating disorder in our set-up. NMO and NMOS disorders contributed to 14.9% (45/303 of all demyelinating disorders in our registry.

Brain Tissue Volumes and Perfusion Change with the Number of Optic Neuritis Attacks in Relapsing Neuromyelitis Optica: A Voxel-Based Correlation Study.

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Carlos A Sánchez-Catasús

Full Text Available Recent neuroimaging studies show that brain abnormalities in neuromyelitis optica (NMO are more frequent than earlier described. Yet, more research considering multiple aspects of NMO is necessary to better understand these abnormalities. A clinical feature of relapsing NMO (RNMO is that the incremental disability is attack-related. Therefore, association between the attack-related process and neuroimaging might be expected. On the other hand, the immunopathological analysis of NMO lesions has suggested that CNS microvasculature could be an early disease target, which could alter brain perfusion. Brain tissue volume changes accompanying perfusion alteration could also be expected throughout the attack-related process. The aim of this study was to investigate in RNMO patients, by voxel-based correlation analysis, the assumed associations between regional brain white (WMV and grey matter volumes (GMV and/or perfusion on one side, and the number of optic neuritis (ON attacks, myelitis attacks and/or total attacks on the other side. For this purpose, high resolution T1-weighted MRI and perfusion SPECT imaging were obtained in 15 RNMO patients. The results showed negative regional correlations of WMV, GMV and perfusion with the number of ON attacks, involving important components of the visual system, which could be relevant for the comprehension of incremental visual disability in RNMO. We also found positive regional correlation of perfusion with the number of ON attacks, mostly overlapping the brain area where the WMV showed negative correlation. This provides evidence that brain microvasculature is an early disease target and suggests that perfusion alteration could be important in the development of brain structural abnormalities in RNMO.

Systematic review of the published data on the worldwide prevalence of John Cunningham virus in patients with multiple sclerosis and neuromyelitis optica

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Sonia Patricia Castedo Paz

2018-01-01

Full Text Available OBJECTIVES John Cunningham virus (JCV is a polyoma virus that infects humans, mainly in childhood or adolescence, and presents no symptomatic manifestations. JCV can cause progressive multifocal leukoencephalopathy (PML in immunosuppressed individuals, including those undergoing treatment for multiple sclerosis (MS and neuromyelitis optica (NMO. PML is a severe and potentially fatal disease of the brain. The prevalence of JCV antibodies in human serum has been reported to be between 50.0 and 90.0%. The aim of the present study was to review worldwide data on populations of patients with MS and NMO in order to establish the rates of JCV seropositivity in these individuals. METHODS The present review followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and used the following search terms: “JCV” OR “JC virus” AND “multiple sclerosis” OR “MS” OR “NMO” OR “neuromyelitis optica” AND “prevalence.” These terms were searched for both in smaller and in larger clusters of words. The databases searched included PubMed, MEDLINE, SciELO, LILACS, Google Scholar, and Embase. RESULTS After the initial selection, 18 papers were included in the review. These articles reported the prevalence of JCV antibodies in the serum of patients with MS or NMO living in 26 countries. The systematic review identified data on 29,319 patients with MS/NMO and found that 57.1% of them (16,730 individuals were seropositive for the anti-JCV antibody (range, 40.0 to 69.0%. CONCLUSIONS The median worldwide prevalence of JCV among adults with MS or NMO was found to be 57.1%.

Measurement of chromatic dispersion of microstructure optical fibers using interferometric method

Czech Academy of Sciences Publication Activity Database

Peterka, Pavel; Kaňka, Jiří; Honzátko, Pavel; Káčik, D.

2008-01-01

Roč. 38, č. 2 (2008), s. 295-303 ISSN 0078-5466 R&D Projects: GA AV ČR 1ET300670502; GA MŠk 1P05OC002 Institutional research plan: CEZ:AV0Z20670512 Keywords : chromatic dispersion * optica l fiber measurement * microstructure optica l fibers Subject RIV: JA - Electronics ; Optoelectronics, Electrical Engineering Impact factor: 0.204, year: 2008

Cognitive impairment in neuromyelitis optica spectrum disorders: A comparison of the Wechsler Adult Intelligence Scale-III and the Wechsler Memory Scale Revised with the Rao Brief Repeatable Neuropsychological Battery.

Science.gov (United States)

Fujimori, Juichi; Nakashima, Ichiro; Baba, Toru; Meguro, Yuko; Ogawa, Ryo; Fujihara, Kazuo

2017-12-01

Approximately 55% of patients with neuromyelitis optica spectrum disorder (NMOSD) show cognitive impairment as evaluated using the Rao Brief Repeatable Neuropsychological Battery (BRBN), but this frequency appears to be higher than the frequency of specific brain lesions in NMOSD. We studied whether cognitive impairment could be observed in NMOSD patients with no or minor non-specific brain lesions. We evaluated cognitive function in 12 NMOSD and 14 MS patients using the Wechsler Adult Intelligence Scale-III (WAIS-III), the Wechsler Memory Scale-Revised (WMS-R), and the BRBN. We judged as cognitively impaired patients whose scores were below the average by 2 standard deviations or greater in 2 or more cognitive domains. Cognitive impairment was observed in 5 MS patients (35.7%) and in the only NMOSD patient (8.3%) with symptomatic brain lesions, but not in the other NMOSD patients who had no or minor non-specific brain lesions. Meanwhile, 5 NMOSD (41.7%) and 4 MS (28.6%) patients who had normal cognition according to the WAIS-III and WMS-R were assessed as cognitively impaired by the BRBN (which is not standardized for age). Cognitive function in NMOSD patients with no or mild non-specific brain lesions was preserved according to the WAIS-III and WMS-R.

Serie: Madera para construcción Acabados para madera

OpenAIRE

Sáenz-Muñoz, Marta; Instituto Tecnológico de Costa Rica

2012-01-01

Durante mucho tiempo, la madera ha sido utilizada como material de construcción para resolver principalmente objetivos estructurales; sin embargo, actualmente, el uso de la madera ha tomado mucho auge en la estética de las construcciones, para brindar apariencia y calidez. En la actualidad, el mercado ofrece una amplia gama productos para dar diversos “acabados” a la madera, los cuales sirven para resaltar la belleza de algunas de ellas, realzar características como el color y la veta, o bien...

Study of the Generation of Intense Pulsed Electron Beams Using Glow Discharges

Science.gov (United States)

1988-02-01

de Investigaciones Opticas, Rep. Argentina. (2) On leave from PROFET (UNCPBA) Programa de Fisica Experimental Tandil, Rep. Argentina. (3) Edwards... de Investigaciones Opticas, (CIC-BA) Rep. Argentina. Section III-D. Measurements of the plasma density andB. T. Szapiro is on leave from Programa de ...discussed. The radial profiles Szapiro was supported by a fellowship from the Universidad Nacional de show the presence of a high-current-density

Absorbing materials of thin film for photovoltaic structures prepared by the process of pyrolytic spraying; Materiales absorbedores en pelicula delgada para estructuras fotovoltaicas preparados por el proceso de rocio pirolitico

Energy Technology Data Exchange (ETDEWEB)

Calixto Rodriguez, Manuela

2006-11-15

InS{sub 2}, SnS{sub 2} y SnS por la tecnica de rocio pirolitico. Se analizaron las propuestas estructurales, opticas y electricas de estas peliculas para determinar el conjunto de parametros de deposito con los cuales se obtienen las mejores propiedades fisicas para la aplicacion de las mismas en estructuras fotovoltaicas. Se encontro que la relacion molar en solucion de los compuestos de partida y la temperatura de deposito son los parametros dominantes en el proceso de crecimiento de las peliculas. Para el deposito de peliculas delgadas se sulfuro de indio se usaron acetato de indio y N, N-dimetil tiourea como compuestos precursores de In y S, respectivamente. Se obtuvieron peliculas policristalinas del compuesto In{sub 2}S{sub 3} empleando temperaturas de substrato, Ts, entre 400 y 450 grados centigrados, y soluciones de acetato de indio y N, N-dimetil tiourea con relaciones molares (In:S) de 1:8 y 1:1. La brecha de energia, Eg, de estas peliculas varia de 2.04 a 2.67 eV, y el valor de la conductividad en obscuridad, D, varia de 10-7 a 1 (cm)-1 en funcion de la relacion molar en solucion. Se encontro que las peliculas depositadas empleando una relacion molar de In:S 1:8 son de naturaleza intrinseca independientemente de Ts, mientras que las depositadas empleando una relacion molar de In:S =1:1 y Ts 400 grados Celsius poseen conductividad tipo-n. Empleando una relacion molar de In:S = 1:1 y Ts = 500 grados centigrados se obtiene el compuesto In{sub 2}O{sub 3} policristalino cuyas propiedades opticas y electricas son: Eg 3 eV y D=11 (cm)-1, con conductividad electrica tipo-n. Las peliculas depositadas empleando una relacion molar de In:S = 1:1 y Ts 450 grados centigrados son las que tienen las caracteristicas apropiadas para su uso como material tipo-n en estructuras fotovoltaicas (Eg=2 eV y D=1-1 com-1).

Treatment of Outliers to Study Railway Vibrations Transmission Tratamiento de outliers para el estudio de transmisión de vibraciones del ferrocarril

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Nicoletta González–Cancelas

2012-12-01

Full Text Available This paper presents the data analysis of the vibration induced by the propagation of waves caused by passing trains, metro and tram. These dataare taken with an optical fiber accelerometer within the framework of the research project TRAVIESA (Transmisión de Vibraciones del ferrocarril urbanoal entorno: Estrategias, Tecnologías y materiales para su atenuación fundedby CDTI and the Ministry of Science and Innovation of the Government ofSpain. The methodology was developed to characterize the level of vibrationsthat affect different elements of railway infrastructure and nearby buildings.Optical fiber accelerometers were used and the measurements were made bythe Technological Institute of Optics, Colour and Imaging (AIDO.En este trabajo se presenta el resulado del análisis de datos de vibracionesinducidas por la propagación de ondas originadas por el paso de trenes, metroy tranvía. Dichos datos han sido tomados con aelerómetros de fibra optica,dentro del marco del proyecto de investigación TRAVIESA (Transmisión de Vibraciones del ferrocarril urbano al entorno: Estrategias, Tecnologías y materialespara su atenuación financiado por el CDTI y el Ministerio De Cienciae Innovación del Gobierno de España. La metodología desarrollada permite caracterizar el nivel de las vibraciones que afectan a los diferentes elementos dela infraestructura ferroviaria y los edificios cercanos. La tecnología de los acelerómetroses la fibra óptica y las mediciones fueron realizadas por el InstitutoTecnológico de Óptica, Color e Imagen (AIDO.

Para-bosons and Para-fermions in Quantum Mechanics

International Nuclear Information System (INIS)

Cattani, M.S.D.; Fernandes, N.C.

1982-01-01

Within the framework of the ordinary quantum mechanics, a detailed study of the energy eigenfunctions of N identical particles using the irreducible representations of the permutation group in the Hilbert space is performed. It is shown that the para-states, as occurs with the boson and fermion states, are compatible with the postulates of quantum mechanics and with the principle of indistinguishability. A mathematical support for the existence of para-bosons and para-fermions is given. Gentile's quantum statistics is, in a certain sense, justified. (Author) [pt

[Proportion and significance of CD1d(hi)CD5⁺CD19⁺ regulatory B cell in peripheral blood of patients with neuromyelitis optica].

Science.gov (United States)

Yang, Fen; Huang, Dehui; Cheng, Chen; Wu, Weiping

2015-03-01

To detect the proportion of CD1d(hi)CD5⁺CD19⁺ regulatory B cells (Bregs) in peripheral blood of the patients with neuromyelitis optica (NMO), and explore whether CD1d(hi)CD5⁺CD19⁺ Bregs can play a role as a biomarker in the diagnosis of NMO versus multiple sclerosis (MS). Flow cytometry was performed to detect the proportion of CD1d(hi)CD5⁺CD19⁺ Bregs in peripheral blood from 44 cases of NMO, 38 cases of MS, and 30 healthy controls. The serum level of aquaporin-4 antibody (AQP4-Ab) of patients with NMO was detected by indirect immunofluorescence assay. The proportion of CD1d(hi)CD5⁺CD19⁺ Bregs in CD19⁺ B cells and lymphocytes was significantly lower in NMO group than in MS and control groups; however, there was no significant difference between MS group and control group. The proportion of CD1d(hi)CD5⁺CD19⁺ Bregs in CD19⁺ B cells and lymphocytes was lower in AQP4-Ab-positive NMO patients than in AQP4-Ab-negative NMO patients, and the difference was statistically significant. CD1d(hi)CD5⁺CD19⁺ Bregs may be a biomarker in the differential diagnosis of NMO versus MS.

Frequency and prognostic impact of antibodies to aquaporin-4 in patients with optic neuritis

DEFF Research Database (Denmark)

Jarius, Sven; Frederiksen, Jette Lautrup Battistini; Waters, Patrick

2010-01-01

Antibodies to aquaporin-4 (AQP4-Ab) are found in 60-80% of patients with neuromyelitis optica (NMO), a severely disabling inflammatory CNS disorder of putative autoimmune aetiology, which predominantly affects the optic nerves and spinal cord.......Antibodies to aquaporin-4 (AQP4-Ab) are found in 60-80% of patients with neuromyelitis optica (NMO), a severely disabling inflammatory CNS disorder of putative autoimmune aetiology, which predominantly affects the optic nerves and spinal cord....

Marcadores para la Realidad Aumentada para fines educativos

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María Reina Zarate Nava

2013-12-01

Full Text Available La Realidad Aumentada (AR es considerada por algunos expertos como la tecnología en crecimiento para el 2014, en diferentes sectores: ingeniería, educación, juegos y negocios, siendo este último el más usado en él 2013. Sin embargo en el sector educativo la AR se presenta en algunos proyectos que se han desarrollado para diferentes áreas de las ciencias, como Química, Física y Matemáticas, lo anterior con el objetivo de facilitar el proceso de Enseñanza-Aprendizaje. En el presente artículo se presenta el modelo de patrones utilizado para un Proyecto AR enfocado en Química, el cual presenta un conjunto de patrones que fueron valorados para facilitar el rastreo de los objetos y a su vez el entendimiento de algunos materiales de los laboratorios de Química I.

Generating para-water from para-hydrogen: A Gedankenexperiment.

Science.gov (United States)

Ivanov, Konstantin L; Bodenhausen, Geoffrey

2018-07-01

A novel conceptual approach is described that is based on the transfer of hyperpolarization from para-hydrogen in view of generating a population imbalance between the two spin isomers of H 2 O. The approach is analogous to SABRE (Signal Amplification By Reversible Exchange) and makes use of the transfer of spin order from para-hydrogen to H 2 O in a hypothetical organometallic complex. The spin order transfer is expected to be most efficient at avoided level crossings. The highest achievable enrichment levels of para- and ortho-water are discussed. Copyright © 2018 Elsevier Inc. All rights reserved.

EVALUACION DE LA RESISTENCIA A LA CORROSION DEL SISTEMA PRIMER EPOXICO RICO EN ZINC/ACABADO POLISILOXANO POR MEDIO DE ESPECTROSCOPIA DE IMPEDANCIA ELECTROQUIMICA

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ANDRES ALBERTO GUZMAN ROSAS

2011-01-01

Full Text Available En este trabajo fue estudiado el sistema primer epoxico rico en zinc / acabado polisiloxano aplicado sobre laminas de acero al carbono laminado en caliente y sometido a camaras de corrosion acelerada (inmersion total, niebla salina, ciclo de corrosion y prohesion por un periodo de hasta 5000 horas. Las probetas bajo estudio se evaluaron cada 20 dias por medio de espectroscopia de impedancia electroquimica y al final de la prueba se realizaron chequeos visuales y microscopia optica para ver la morfologia de la interface metal-recubrimiento y microespectroscopia Raman para identificar la presencia de posibles productos de corrosion. Los resultados de impedancia fueron simulados por medio de circuitos electricos equivalentes. Para el sistema se encontraron modulos de impedancia del orden de 1011 ¿.cm2 en prohesion, 1010 ¿.cm2 en ciclo de corrosion, 109 ¿.cm2 tanto niebla salina como en las inmersiones totales (NaCl 10-2M y Na2SO4 10-2M. Este sistema tuvo durante el tiempo de pruebas un comportamiento excelente con altas resistencias de pelicula y de transferencia de carga y bajas capacitancias de pelicula y de doble capa.

The neuromyelitis optica presentation and the aquaporin-4 antibody in HIV-seropositive and seronegative patients in KwaZulu-Natal, South Africa

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Ahmed I. Bhigjee

2017-01-01

Full Text Available Background: The association of the anti-aquaporin-4 (AQP-4 water channel antibody with neuromyelitis optica (NMO syndrome has been described from various parts of the world. There has been no large study describing this association from southern Africa, an HIV endemic area. HIV patients often present with visual disturbance or features of a myelopathy but seldom both either simultaneously or consecutively. We report our experience of NMO in the era of AQP-4 testing in HIV-positive and HIV-negative patients seen in KwaZulu-Natal, South Africa. Methods: A retrospective chart review was undertaken of NMO cases seen from January 2005 to April 2016 in two neurology units serving a population of 7.1 million adults. The clinical, radiological and relevant laboratory data were extracted from the files and analysed. Results: There were 12 HIV-positive patients (mean age 33 years, 9 (75% were women and all 12 were black patients. Of the 17 HIV-negative patients (mean age 32 years, 15 (88% were women and 10 (59% were black people. The clinical features in the two groups ranged from isolated optic neuritis, isolated longitudinally extensive myelitis or combinations. Recurrent attacks were noted in six HIV-positive patients and six HIV-negative patients. The AQP-4 antibody was positive in 4/10 (40% HIV-positive patients and 11/13 (85% HIV-negative patients. The radiological changes ranged from longitudinal hyperintense spinal cord lesions and long segment enhancing lesions of the optic nerves. Three patients, all HIV-positive, had tumefactive lesions with incomplete ring enhancement. Conclusion: This study confirms the presence of AQP-4-positive NMO in southern Africa in both HIV-positive and HIV-negative patients. The simultaneous or consecutive occurrence of optic neuritis and myelitis in an HIV-positive patient should alert the clinician to test for the AQP-4 antibody. It is important to recognise this clinical syndrome as specific therapy is available

Detection of Cherenkov radiation: the inverse problem to generation of nondiffracting beams

Czech Academy of Sciences Publication Activity Database

Miler, Miroslav; Pala, Jan

2003-01-01

Roč. 33, 2-3 (2003), s. 307-313 ISSN 0078-5466. [Polish-Czech-Slovak Optica l Conference on Wave and Quantum Aspects of Contemporary Optics PCS /13./. Krzyzowa, 09.09.2002-13.09.2002] R&D Projects: GA ČR GA102/01/0429 Institutional research plan: CEZ:AV0Z2067918 Keywords : Cherenkov radiation * holographic optica l elements * laser beams * diffraction Subject RIV: JA - Electronics ; Optoelectronics, Electrical Engineering Impact factor: 0.221, year: 2003

Uma ferramenta para planejamento de estudos para concursos

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Leonardo Pimentel

2015-11-01

Full Text Available De acordo com a constituição brasileira, a única forma de se tornar um empregado do governo é passar em um concurso público. O exame de conhecimento do cargo é um dos componentes mais importantes de um concurso. Estudar para esse exame é uma tarefa que requer planejamento. Um meio de obter um bom planejamento é construir uma grade de horários que relacione as matérias a serem estudadas com os respectivos horários disponíveis para o estudo. Esse tipo de problema se assemelha bastante à gerência de projetos, em que se procura estabelecer a distribuição das atividades ao longo do tempo disponível. Assim, torna-se interessante explorar a dinâmica de estudos para concursos como um projeto. O presente trabalho apresenta a ferramenta de apoio ao estudos - Passe-me, baseada no ciclo PDCA (do inglês, Plan-Do-Check-Act. Por se tratar de uma ferramenta web, o tempo de resposta para produzir uma grade de estudos é fundamental. A ferramenta faz uso de algoritmo genético para calcular a grade de horários priorizando a velocidade de resposta. A ferramenta foi avaliada em diferentes cenários de uso e obteve desempenho satisfatório durante a produção da grade de estudos.

Docencia para el logro y educación para emprender

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Luis Gerardo Rodríguez Gómez

1994-05-01

Full Text Available RESUMEN En el primer artículo, el autor plasmó los elementos relevantes para una propuesta de educación para emprender. En este describe el desarrollo docente como expresión de una intensión, saber y un quehacer, encaminados a la formación del hombre emprendedor. El artículo se divide en tres partes. En la primera, se reseña los factores que orientan la intensión docente para emprender, a partir de un discurso y formas metodológicas establecidas con esa intención. En segundo lugar, se asume el espacio y tiempo la clase como unidad de análisis, para lograr objetivos de tipo institucional, programático y temático y en la parte final, se examina la evaluación docente y se hacen consideraciones generales para una propuesta de evaluación,  que surge al investigar la actuación del maestro.

A comparative performance study of a photovoltaic concentrator system with discrete mirror and continuos profile for two different absorber shapes

Energy Technology Data Exchange (ETDEWEB)

H, Saiful; Rezau, K.M [University of Dhaka, Dhaka (Bangladesh)

2000-07-01

Profiles of parabolic concentrators of discrete mirror and continuos surface mirror have been designed for combined electrical thermal photovoltaic systems. In the design the changes of concentration ratio, effect of reflection, angle of incidence over the absorber have been taken into account for maximum energy collection. The performances of the system are studied for solar cells of modified grid finger for illuminations from 1-10 sun. The local concentration ratio (LCR) distribution over the absorbers for both the concentrator, the optical efficiency, thermal efficiency, electrical and thermal power output and overall efficiency have been evaluated for different values of beam radiation concentration ratio and focal distance. [Spanish] Se han disenado perfiles de concentradores parabolicos de espejo discreto y de superficie continua para sistemas fotovoltaicos combinados electricos y termicos. En el diseno los cambios de la proporcion de concentracion, del efecto de la reflexion, del angulo de incidencia sobre el observador se han tenido en cuenta para una maxima recoleccion de energia. Los rendimientos del sistema se han estudiado para celdas solares de parrilla modificada para iluminaciones solares de 1-10. Han sido evaluados para diferentes valores de la proporcion de la concentracion de la radiacion en el rayo y la distancia focal la proporcion de concentracion local (LCR) de la distribucion en los absorbedores, para el concentrador la eficiencia optica, la eficiencia termica, electrica, la produccion de energia termica y electrica y la eficiencia total.

Changes in B and T-cell subsets and NMO-IgG levels after immunoglobulins and rituximab treatment for an acute attack of neuromyelitis optica.

Science.gov (United States)

de Andrés, C; Teijeiro, R; Saiz, A; Fernández, P; Sánchez-Ramón, S

2015-06-01

There is increasing evidence supporting that neuromyelitis optica (NMO) is an inflammatory humoral mediated disorder associated with NMO-IgG/AQP-4 antibodies. However, little is known about the subsets of B cells and T cells that contribute to the pathogenesis or therapy response. To describe the clinical and immunological changes associated with intravenous immunoglobulins (IV-Igs) plus rituximab (RTX) in a patient with a severe acute attack of NMO and intrathecal synthesis of NMO-IgG/AQP-4, who previously did not respond to intravenous methylprednisolone and plasma exchange. We sequentially analysed the levels of NMO-IgG/AQP-4 by immunohistochemistry, and B and T cells subsets by multiparametric flow-cytometry, in the CSF and peripheral blood (PB), before and alter IV-Igs plus RTX therapy. In the CSF before treatment, and compared with PB, there was a higher percentage of CD4(+) T cells and a lower percentage of CD8(+) T cells and CD19(+) B cells. After therapy, the percentage of CD4(+) T cells remained high, and that of CD8(+) T cells increased. The observed decrease in the percentage of CD19(+) B cells was lower than in the PB. When the CSF was compared, it was found that the percentage of effector-memory and effector CD8(+) T cells had increased after therapy, and that of IgM memory B cells and switched-memory B cells decreased. The observed changes paralleled the decrease of NMO-IgG/AQP-4 results to negative and the clinical improvement. Our findings confirm that, besides intrathecal humoral immune response against AQP4, B and T cell subsets are involved in the modulation of inflammation within and outside the central nervous system. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.

Guided Optical Structures in the Military Environment

Science.gov (United States)

1986-05-01

gyroscopes using integrated optica " (ref.9A), H.C.Lefevre, J.P. Bettini, S.Vatoux and M.Papuohon showed that considerable progress had meanwhile been made...Rotating Optical Fiber Ring Intorferometer", Applied Optica , 16, p. 2605, 1977. 7. V. Vali, R. W. Shorthill, A. Goldstein, and R. S. Krogstad, "Laser...Trmometro, a fibra ottica- Italian Patent No. 04155 A/82 (1982) under extension. 7-5 10 A.M. Sohoggi, M. Dranci, G. Conforti, R. Falcioi, G.P. Preti

Modelos para estimar consumo e exigências nutricionais para poedeiras comerciais

OpenAIRE

Palma Bendezu, Hilda Cristina [UNESP

2016-01-01

O objetivo deste trabalho foi elaborar um modelo para calcular as ingestões de aminoácidos e energia para a fase de maturação sexual e produção de ovos. As exigências de energia, aminoácidos e consumo de ração foram calculados pelo método fatorial, considerando na fase de maturação sexual a quantidade de nutrientes requerida para mantença e a quantidade de nutrientes necessários para crescimento e deposição de nutrientes corporais em cada componente do corpo (ovário, oviduto, corpo livre de ...

Modelos para estimar as exigências de energia metabolizável para poedeiras

OpenAIRE

Sakomura,Nilva Kazue; Basaglia,Roberta; Sá-Fortes,Cristina M. L.; Fernandes,João Batista K.

2005-01-01

Objetivou-se, com este trabalho, elaborar um modelo para estimar as exigências de energia metabolizável (EM) para poedeiras leves da linhagem Lohmann LSL, utilizando-se o método fatorial. Para determinar o efeito da temperatura sobre as exigências de EM para mantença, foram conduzidos experimentos em câmaras climáticas com temperaturas constantes de 12, 22 e 31ºC, utilizando a técnica do abate comparativo. A exigência de energia líquida para o ganho de peso foi determinada por meio da regress...

Neuromyelitis Optica

Science.gov (United States)

... may help prevent with accumulation of disability. Rarely, muscle weakness can be severe enough to cause breathing difficulties and may require the use of artificial ventilation. x Prognosis Most individuals with NMO have ...

Significados da gravidez e da maternidade: discursos de primíparas e multíparas

OpenAIRE

Silva, Flavia Baroni; Souza, Laura Vilela e; Scorsolini-Comin, Fabio

2013-01-01

Resumo: O objetivo foi compreender os sentidos sobre maternidade e as vivências da gravidez em gestantes primíparas e multíparas. Foram entrevistadas seis gestantes, sendo três primíparas e três multíparas. Nas falas, foram trazidos temas como dificuldades físicas, alterações corporais, repercussão da gravidez no relacionamento conjugal, planejamento da gravidez e envolvimento paterno. Observou-se, nas primíparas, que a inexperiência trouxe insegurança, ao passo que, em algumas multíparas, ho...

Estrategias para negociaciones exitosas.

OpenAIRE

José B. Parra V; Evelinda Santiago J.; Misael Murillo M.; Candy Atonal N.

2010-01-01

Las estrategias de negociación son herramientas para disminuir conflictos o para intercambiar bienes y servicios. Una negociación exitosa crea relaciones de solidaridad y propicia reciprocidad, cooperación y confianza para quienes tendrán relaciones permanentes de negociación con beneficios para ambas partes. Una estrategia de negociación exitosa requiere manejar el desarrollo del ambiente social del grupo de personas, con la finalidad de tener presente las fronteras que no pueden irrumpirse....

Neuromyelitis optica spectrum disorders: comparison of clinical and magnetic resonance imaging characteristics of AQP4-IgG versus MOG-IgG seropositive cases in the Netherlands.

Science.gov (United States)

van Pelt, E D; Wong, Y Y M; Ketelslegers, I A; Hamann, D; Hintzen, R Q

2016-03-01

Neuromyelitis optica spectrum disorders (NMOSDs) are a group of rare inflammatory demyelinating disorders of the central nervous system. The identification of specific antibodies directed to aquaporin 4 (AQP4-IgG) led to the distinction from multiple sclerosis. However, up to 25% of the clinically diagnosed NMO patients are seronegative for AQP4-IgG. A subgroup of these patients might be identified by antibodies directed to myelin oligodendrocyte glycoprotein (MOG-IgG). Our objective was to investigate whether the clinical characteristics of these patients differ. Using a cell-based assay, samples of 61 AQP4-IgG seronegative patients and 41 AQP4-IgG seropositive patients with clinically NMOSD were analysed for the presence of MOG-IgG. Clinical characteristics of the AQP4-IgG, MOG-IgG seropositive and double seronegative NMOSD patients were compared. Twenty of the 61 AQP4-IgG seronegative patients tested MOG-IgG seropositive (33%). MOG-IgG seropositive patients were more frequently males in contrast to AQP4-IgG seropositive patients (55% vs. 15%, P < 0.01) and Caucasians (90% vs. 63%, P = 0.03). They more frequently presented with coincident optic neuritis and transverse myelitis (40% vs. 12%, P = 0.02) and had a monophasic disease course (70% vs. 29%, P < 0.01). AQP4-IgG seropositive patients were 2.4 times more likely to suffer from relapses compared with MOG-IgG seropositive patients (relative risk 2.4, 95% confidence interval 1.2-4.7). AQP4-IgG seropositive patients had higher Expanded Disability Status Scale levels at last follow-up (P < 0.01). Antibodies directed to MOG identify a subgroup of AQP4-IgG seronegative NMO patients with generally a favourable monophasic disease course. © 2015 EAN.

Para-equilibrium phase diagrams

International Nuclear Information System (INIS)

Pelton, Arthur D.; Koukkari, Pertti; Pajarre, Risto; Eriksson, Gunnar

2014-01-01

Highlights: • A rapidly cooled system may attain a state of para-equilibrium. • In this state rapidly diffusing elements reach equilibrium but others are immobile. • Application of the Phase Rule to para-equilibrium phase diagrams is discussed. • A general algorithm to calculate para-equilibrium phase diagrams is described. - Abstract: If an initially homogeneous system at high temperature is rapidly cooled, a temporary para-equilibrium state may result in which rapidly diffusing elements have reached equilibrium but more slowly diffusing elements have remained essentially immobile. The best known example occurs when homogeneous austenite is quenched. A para-equilibrium phase assemblage may be calculated thermodynamically by Gibbs free energy minimization under the constraint that the ratios of the slowly diffusing elements are the same in all phases. Several examples of calculated para-equilibrium phase diagram sections are presented and the application of the Phase Rule is discussed. Although the rules governing the geometry of these diagrams may appear at first to be somewhat different from those for full equilibrium phase diagrams, it is shown that in fact they obey exactly the same rules with the following provision. Since the molar ratios of non-diffusing elements are the same in all phases at para-equilibrium, these ratios act, as far as the geometry of the diagram is concerned, like “potential” variables (such as T, pressure or chemical potentials) rather than like “normal” composition variables which need not be the same in all phases. A general algorithm to calculate para-equilibrium phase diagrams is presented. In the limit, if a para-equilibrium calculation is performed under the constraint that no elements diffuse, then the resultant phase diagram shows the single phase with the minimum Gibbs free energy at any point on the diagram; such calculations are of interest in physical vapor deposition when deposition is so rapid that phase

"Estudio tribologico de aceros para moldes. Aplicacion al moldeo por inyeccion de polibutilentereftalato reforzado con fibra de vidrio"

Science.gov (United States)

Martinez Mateo, Isidoro Jose

fabricacion del molde, tienen una gran influencia sobre su comportamiento en servicio a lo largo de la vida util del molde. En la primera parte del presente estudio, a partir de ensayos punzon sobre disco, se ha determinado la relacion entre la resistencia al desgaste y la dureza de aceros para moldes obtenidos a partir de bloques de gran espesor, estudiando los principales mecanismos de desgaste que tienen lugar. A continuacion, con el fin de determinar el dano superficial que sufren los aceros para moldes en condiciones reales de inyeccion, se han estudiado distintos tipos de aceros utilizados comercialmente en moldes de inyeccion de polimeros y materiales compuestos, seleccionando las condiciones de operacion para determinar la variacion de la rugosidad superficial del acero en funcion del material inyectado, del numero de operaciones sucesivas de inyeccion y de la orientacion del flujo de inyeccion, mediante tecnicas de perfilometria optica y microscopia electronica de barrido. Ademas del dano superficial sufrido por el acero con el numero de piezas inyectadas, tambien se ha determinado la evolucion de la rugosidad superficial de los materiales inyectados, polibutilentereftalato (PBT) puro y materiales compuestos derivados de PBT por adicion de un 20 o un 50% en peso de fibra de vidrio. En el caso de las piezas inyectadas, se ha caracterizado su microestructura en funcion del flujo de inyeccion y de la densidad de fibra, se han determinado sus propiedades termicas y dinamico-mecanicas, asi como la variacion de la rugosidad superficial de las piezas inyectadas con el numero de operaciones de inyeccion y con la geometria de las distintas secciones de las piezas. Finalmente, se ha evaluado la resistencia a la abrasion de PBT reforzado con un 50% de fibra, en funcion del numero de piezas inyectadas y de la direccion de rayado con respecto a la orientacion del flujo de inyeccion.

Estrategia para elevar la motivación laboral; factor imprescindible para mejorar nuestra productividad.

OpenAIRE

Reina de los Ángeles Carballé Piñón

2015-01-01

Este trabajo "Estrategia para elevar la motivación laboral; factor imprescindible para mejorar nuestra productividad" resulta de la investigación realizada para tesis de maestría; se realizó el estudio de 2 entidades, nos permitió conocer las principales posiciones que sobre motivación existen, factores que la influencian, cómo incide en el desempeño laboral y que acciones diseñar para elevarla. Las 2 entidades seleccionadas, claves en el desempeño empresarial son un centro de interfase y un...

Estrategias para negociaciones exitosas.

Directory of Open Access Journals (Sweden)

José B. Parra V

2010-01-01

Full Text Available Las estrategias de negociación son herramientas para disminuir conflictos o para intercambiar bienes y servicios. Una negociación exitosa crea relaciones de solidaridad y propicia reciprocidad, cooperación y confianza para quienes tendrán relaciones permanentes de negociación con beneficios para ambas partes. Una estrategia de negociación exitosa requiere manejar el desarrollo del ambiente social del grupo de personas, con la finalidad de tener presente las fronteras que no pueden irrumpirse. Otro aspecto importante, es el lenguaje corporal utilizado por las personas y las tácticas que se practican. El manejo de esta información permite diseñar diferentes modelos de negociaciones para cada caso específico. Este documento propone la utilización de la herramienta MONACO, diseñada por los autores, que es un software útil para encontrar los mejores tácticas posibles dentro de una negociación; basándose en el lenguaje corporal que muestra el negociante oponente durante cada oferta monetaria. El uso de este software da al negociador la oportunidad de manejar las negociaciones con mayor control, el negociador que usa el software puede elegir una estrategia de acuerdo a las relaciones que espera mantener con el negociador oponente.

Neuromyelitis optica in Austria in 2011: to bridge the gap between neuroepidemiological research and practice in a study population of 8.4 million people.

Directory of Open Access Journals (Sweden)

Fahmy Aboul-Enein

Full Text Available BACKGROUND: In 2008 the Austrian Task Force for Neuromyelitis Optica (NMO started a nation-wide network for information exchange and multi-centre collaboration. Their aim was to detect all patients with NMO or NMO spectrum disorders (NMO-SD in Austria and to analyse their disease courses and response to treatment. METHODS: (1 As of March 2008, 1957 serum samples (of 1557 patients have been tested with an established cell based immunofluorescence aquaporin-4 antibody (AQP4-ab assay with a high sensitivity and specificity (both >95%. All tests were performed in a single reference laboratory (Clinical Dept. of Neurology of the Innsbruck Medical University. (2 A nation-wide survey with several calls for participation (via email newsletters, articles in the official journal of the Austrian Society of Neurology, and workshops was initiated in 2008. All collected data will be presented in a way that allows that every individual patient can be traced back in order to ensure transparency and to avoid any data distortion in future meta-analyses. The careful and detailed presentation allows the visualization and comparison of the different disease courses in real time span. Failure and response to treatment are made visible at one glance. Database closure was 31 December 2011. All co-operators were offered co-authorship. RESULTS: All 71 NMO- or NMO-SD patients with AQP4-ab positivity (age range 12.3 to 79.6 years were analysed in detail. Sex ratio (m:f = 1:7 and the proportion of patients without oligoclonal bands in cerebrospinal fluid (86.6% were in line with previously published results. All identified patients were Caucasians. CONCLUSIONS: A nationwide collaboration amongst Austrian neurologists with good network communications made it possible to establish a database of 71 AQP4-ab positive patients with NMO/NMO-SD. This database is presented in detail and provides the basis for further studies and international cooperation in order to investigate this

Increased occurrence of anti-AQP4 seropositivity and unique HLA Class II associations with neuromyelitis optica (NMO), among Muslim Arabs in Israel.

Science.gov (United States)

Brill, Livnat; Mandel, Micha; Karussis, Dimitrios; Petrou, Panayiota; Miller, Keren; Ben-Hur, Tamir; Karni, Arnon; Paltiel, Ora; Israel, Shoshana; Vaknin-Dembinsky, Adi

2016-04-15

Previous studies have revealed different human leukocyte antigen (HLA) associations in multiple sclerosis (MS) and neuromyelitis optica (NMO), further discriminating these two demyelinating pathological conditions. In worldwide analyses, NMO and opticospinal MS are represented at higher proportions among demyelinating conditions in African, East-Asian and Latin American populations. There are currently no data regarding the prevalence of NMO in Middle East Muslims. The population in Israel is diverse in many ways, and includes subpopulations, based on religion and ethnicity; some exhibit genetic homogeneity. In Israel, the incidence of MS is lower in the Muslim population than the Jewish population and Muslims carry different allele frequency distribution of HLA haplotypes. To evaluate the occurrence of anti-AQP4 seropositivity in the Israeli Muslim population among patients with central nervous system (CNS) demyelinating conditions; and to identify the HLA DR and DQ profiles of Muslim Arab Israeli patients with NMO spectrum of diseases (NMOSD). The prevalence of anti-AQP4 seropositivity was analyzed in 342 samples, obtained from patients with various CNS demyelinating conditions and in a validation set of 310 samples. HLA class II alleles (HLA-DRB1 and DQB1) were examined in DNA samples from 35 Israeli Muslim Arabs NMO patients and compared to available data from 74 Israeli Muslim controls. Our data reveal a significantly increased prevalence of anti-AQP4 seropositivity, indicative of NMOSD, in Muslim Arab Israeli patients with initial diagnosis of a CNS demyelinating syndrome. In this population, there was a positive association with the HLA-DRB1*04:04 and HLA-DRB1*10:01 alleles (p=0.03), and a strong negative association with the HLA-DRB1*07 and HLA-DQB1*02:02 alleles (p=0.003, p=0.002). Our findings indicate a possibly increased prevalence of NMOSD in Muslim Arabs in Israel with distinct (positive and negative) HLA associations. Further studies in patients with

¿Argumentar para definir o definir para argumentar?

OpenAIRE

Silva, Luz; Samper, Carmen

2013-01-01

Se presenta y se ilustra un marco de referencia de un estudio en curso para obtener el título de Maestría en Docencia de la Matemática de la Universidad Pedagógica Nacional (Colombia); estudio sobre la conexión entre las acciones de definir y argumentar, que puede contribuir a la práctica de profesores en ejercicio y en formación. La habilidad para construir una definición es un posible indicio de comprensión, mientras que saberla de memoria no garantiza la comprensión del concepto (Vinner, 1...

Guias ópticos para sensores

OpenAIRE

Marcos de Castro Pacitti

1993-01-01

Neste trabalho foram estudados guias ópticos para atuarem como sensores ou em sistemas sensores. Ênfase especial foi dada em guias ópticos retangulares, para dispositivos ópticos integrados, e fibras ópticas elípticas. A analise de fibras ópticas elípticas resultou em um modelamento pratico para projeto das mesmas em aplicações onde se deseja operar com dois modos guiados, situação esta que se mostra muito atraente para implementação de diversos tipos de sensores a fibra óptica. A seguir es...

Labbtex: Toolbox para generación de informes en LATEX para Matlab

Directory of Open Access Journals (Sweden)

José Luis Almazán Gárate

2012-10-01

Full Text Available En este artículo se presenta el software desarrollado por el Equipo H3lite dentro del Departamento de Ingeneniería Civil. Transportes de la Escuela de Ingenieros de Caminos, Canales y Puertos de la Universidad Politécnica de Madrid para la generación de informes enLATEX mediante el software Matlab® y la integración en sus rutinas, Labbtex.La librería Labbtex proporciona un marco flexible para mezclar texto y código Matlab® para la generación automática de documentos. Un rchivo fuente simple contiene el texto de documentación y el código Matlab, al correr la aplicación se genera un documento final LATEX que contiene el texto, gráficos y tablas indicados con el formato de un documento LATEX. El código Matlab genera un documento LATEX usando la sintaxis. Así, LATEX (para composición de texto de alta calidad y Matlab® (para cálculo matemático pueden usarse simultáneamente. Esto permite la generación de informes en tiempo real con un uso de recursos mínimo.

Surface albedo measurements in Mexico City metropolitan area

Energy Technology Data Exchange (ETDEWEB)

Castro, T; Mar, B; Longoria, R; Ruiz Suarez, L. G [Centro de Ciencias de la Atmosfera, UNAM, Mexico, D.F. (Mexico); Morales, L [Instituto de Geografia, UNAM, Mexico, D.F. (Mexico)

2001-04-01

Optical and thermal properties of soils are important input data for the meteorological and photochemical modules of air quality models. As development of these models increase on spatial resolution good albedo data become more important. In this paper measurements of surface albedo of UV (295-385 nm) and visible (450-550 nm) radiation are reported for different urban and rural surfaces in the vicinity of Mexico City. It was found for the downtown zone and average albedo value of 0.05 which is in very good agreement with reported values for urban surfaces. Our albedo values measured in UV region for grey cement and green grass are of 0.10 and 0.009, respectively, and quite similar to those found at the literature of 0.11 and 0.008 for those type of surfaces. [Spanish] Las propiedades opticas y termicas de suelos son datos importantes para los modulos meteorologicos y fotoquimicos de los modelos de calidad del aire. Conforme aumenta la resolucion espacial del modelo se vuelve mas importante contar con buenos datos de albedo. En este articulo se presentan mediciones de albedo superficial de radiacion Ultravioleta (295-385 nm) y visible (450-550 nm) para diferentes superficies urbanas. Los valores medidos de albedo en la region UV para cemento gris y pasto verde son de 0.10 y 0.009, respectivamente, y son muy similares a los reportados en la literatura, 0.11 y 0.008 para este tipo de superficies.

Desenvolvimento de um sensor de campo eletrico alternado com transmissão por fibra-optica

OpenAIRE

Swinka Filho, Vitoldo

1996-01-01

Orientador: Rene Robert Dissertação (mestrado) - Universidade Federal do Parana Resumo: Neste trabalho e mostrado o desenvolvimento de um sensor de campo elétrico que utiliza o principio da carga induzida por campo elétrico alternado em superfícies condutoras. O sensor e composto por duas semi-esferas de alumínio isoladas eletricamente e possui um circuito eletrônico incorporado alimentado por baterias, e um sistema de transmissão por fibraoptica ligado a uma unidade remota para leitura...

Internet como instrumento para la Yihad

Directory of Open Access Journals (Sweden)

Alfonso Merlos García

2006-01-01

Full Text Available Las extraordinarias ventajas que ofrece Internet lo han convertido en un instrumento clave y dinámico en la estrategia de los yihadistas. Los medios de comunicación, la clase política y las agencias de seguridad e información han tendido a concentrar sus esfuerzos en la neutralización del ciberterrorismo sin prestar suficiente atención a los múltiples usos de Internet que están haciendo los neosalafistas: para la propaganda y para la guerra psicológica, para el reclutamiento y para la financiación, para la documentación y para la planificación de atentados. Las democracias deberían considerar el coste que tendrá la derrota frente al movimiento yihadista globalizado en la batalla por el ciberespacio

Cross-elicitation responses to 2-methoxymethyl-para-phenylenediamine in para-phenylenediamine-allergic individuals

NARCIS (Netherlands)

Schuttelaar, M.-L.; Coenraads, P.-J.; Blömeke, B.; Goebel, C.

2016-01-01

The introduction of a methoxymethyl side chain into para-phenylenediamine (PPD) resulted in the development of a new hair dye molecule: 2-methoxymethyl-para-phenylenediamine (Me-PPD). Besides having excellent hair colouring properties, this PPD derivate has significantly lower skin-sensitizing

Oro para Colombia

OpenAIRE

Rubio Forero, María Claudia

2016-01-01

Las mujeres han debido atravesar un largo camino partiendo de la discriminación hasta empezar un proceso de equidad en la sociedad y en el deporte, de esta manera llegar a ser parte de un evento como los Juegos Olímpicos. Esta tesis toma el caso de tres atletas colombianas y describe su proceso de formación para lograr dos medallas de oro para el país.

Infraestructura para laboratorios de acceso remoto

Directory of Open Access Journals (Sweden)

López Luro, Francisco

2009-01-01

Full Text Available Las tecnologías de Internet permiten el uso de sistemas de software distribuido para el acceso en forma remota a laboratorios físicos y virtuales, para llevar a cabo actividades de investigación experimental a distancia. A partir de los marcos teóricos que definen los estructurantes de los laboratorios, los implementadores de laboratorios de acceso remoto deben diseñar y desarrollar arquitecturas de servicios que permitan un acceso flexible y controlado. Este trabajo define una infraestructura para la implementación de aplicaciones para el acceso remoto a laboratorios físicos y virtuales y para la gestión de los mismos. Se presentan conceptos que permiten extender la modalidad tradicional de conexión con un dispositivo, para llevar adelante actividades prácticas de laboratorio colaborativas y concurrentes.

Diseño de imágenes para ciegos, material didáctico para niños con discapacidad visual.

OpenAIRE

FUENTES NIEVES, FABIOLA MIREYA

2014-01-01

El objetivo de este proyecto es contribuir investigación para la creación, la aplicación, la transformación del quehacer profesional del diseño gráfico en el conocimiento y las habilidades, en la formación de profesionales en el área del diseño para material didáctico para esta disciplina, por lo cual es necesario cambiar respuestas para lo local y/o para la globalización. En la historia del diseño, se plantea, proyectar para la globalización, para la humanidad. Ahora el planteamiento de r...

The para-petroleum industry

International Nuclear Information System (INIS)

2002-07-01

The para-petroleum industry includes societies that realize studies and installations for the deposits exploitation. This document presents the situation of the para-petroleum industry in 2001, the world investment growth of the activity, the french societies financial results and an inventory of the main operations in the amalgamation domain. (A.L.B.)

Adesao ao guia alimentar para populacao brasileira

Directory of Open Access Journals (Sweden)

Eliseu Verly Junior

2013-12-01

Full Text Available OBJETIVO : Analisar a adesão ao Guia Alimentar para População Brasileira. MÉTODOS : Amostra composta por participantes do Inquérito de Saúde de São Paulo (n = 1.661 que preencheram dois recordatórios de 24 horas. Foi utilizado modelo bivariado de efeito misto para a razão entre o consumo de energia do grupo de alimentos e o consumo calórico total. A razão estimada foi utilizada para calcular o percentual de indivíduos com consumo abaixo ou acima da recomendação. RESULTADOS : Pelo menos 80,0% da população consome abaixo do recomendado para: leite e derivados; frutas e sucos de frutas; e cereais, tubérculos e raízes; aproximadamente 60,0% para legumes e verduras; 30,0% para feijões; e 8,0% para carnes e ovos. Adolescentes apresentaram a maior inadequação para legumes e verduras (90,0%, e o estrato de maior renda foi associado à menor inadequação para óleos, gorduras e sementes oleaginosas (57,0%. CONCLUSÕES : Foi observado consumo inadequado dos grupos de alimentos relacionados com aumento do risco de doenças crônicas.

Algoritmos para problemas de empacotamento

OpenAIRE

Eduardo Candido Xavier

2006-01-01

Resumo: Neste trabalho estudamos diversos problemas de empacotamento considerados NP-difíceis. Assumindo a hipótese de que P ? NP, sabemos que não existem algoritmos eficientes (complexidade de tempo polinomial) exatos para resolver tais problemas. Uma das abordagens consideradas para tratar tais problemas é a de algoritmos de aproximação, que são algoritmos eficientes e que geram soluções com garantia de qualidade. Neste trabalho apresentamos alguns algoritmos aproximados para problemas de e...

CONFERENCE NOTE: CETO—Centro de Ciências e Tecnologias Opticas, Trends in Optical Fibre Metrology and Standards

Science.gov (United States)

1994-01-01

requirements of the new generation of analogue and digital fibre optical systems, which require sophisticated measurement techniques employing complex instruments unique to optical measurements. The school will foster and enhance the interaction between material, devices, systems, and standards-oriented R&D communities, as well as between engineers concerned with design and manufacturers of systems and instrumentation. Topics Review of optical fibre communication technology and systems Measurement techniques for fibre characterization: Reliability and traceability Reference fibres and calibration artefacts Ribbon fibres Mechanical and environmental testing Fibre reliability Polarimetric measurements Passive components characterization: Splices and connectors Couplers, splitters, taps and WDMs Optical fibres and isolators WDM technologies and applications: WDM technologies Tunable optical filters Fibre amplifiers and sources: Performances and characterization Design and standards Nonlinear effects Subsystem design and standards: Design and fabrication techniques Performance degradation and reliability Evaluation of costs/performance/technology Sensors IR - optical fibres Plastic fibres Instrumentation Registration Participation free of charge for postgraduate students, with some grants available for travel and lodging expenses. All correspondence should be addressed to: Secretariat, Trends in Optical Fibre Metrology and Standards, a/c Prof. Olivério D D Soares, Centro de Ciências e Tecnologias Opticas, Lab. Fisica - Faculdade de Ciências, Praça Gomes Teixeira, P-4000 Porto, Portugal. Tel: 351-2-310290, 351-2-2001648; Fax: 351-2-319267.

Variants of Interleukin-7/Interleukin-7 Receptor Alpha are Associated with Both Neuromyelitis Optica and Multiple Sclerosis Among Chinese Han Population in Southeastern China

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Jing-Cong Zhuang

2015-01-01

Full Text Available Background: Neuromyelitis optica (NMO and multiple sclerosis (MS are autoimmune demyelinating diseases of the central nerve system. Interleukin-7 (IL-7 and interleukin-7 receptor alpha (IL-7Rα were proved to be important in the pathogenesis of both diseases because of the roles they played in the differentiations of autoimmune lymphocytes. The variants of both genes had been identified to be associated with MS susceptibility in Caucasian, Japanese and Korean populations. However, the association of these variants with NMO and MS has not been well studied in Chinese Southeastern Han population. Here, we aimed to evaluate the association of six IL-7 variants (rs1520333, rs1545298, rs4739140, rs6993386, rs7816065, and rs2887502 and one variant of IL-7RA (rs6897932 with NMO and MS among Chinese Han population in southeastern China. Methods: Matrix-assisted laser desorption/ionization time of flight mass spectrometry (MassARRAY system and Sanger sequencing were used to determine the variants of IL-7 and IL-7RA in 167 NMO patients, 159 MS patients and 479 healthy controls among Chinese Han population in southeastern China. Samples were excluded if the genotyping success rate <90%. Results: Statistical differences were observed in the genotypes of IL-7 rs1520333 in MS patients and IL-7RA rs6897932 in NMO patients, compared with healthy controls (P = 0.035 and 0.034, respectively. There was a statistically significant difference in the genotypes of IL-7 rs2887502 between MS and NMO patients (P = 0.014. And there were statistically significant differences in the rs6897932 genotypes (P = 0.004 and alleles (P = 0.042 between NMO-IgG positive patients and healthy controls. Conclusions: The study suggested that among Chinese Han population in southeastern China, the variant of IL-7RA (rs6897932 was associated with NMO especially NMO-IgG positive patients while the variant of IL-7 (rs1520333 with MS patients. And the genotypic differences of IL-7 rs2887502

Variants of Interleukin-7/Interleukin-7 Receptor Alpha are Associated with Both Neuromyelitis Optica and Multiple Sclerosis Among Chinese Han Population in Southeastern China

Institute of Scientific and Technical Information of China (English)

Jing-Cong Zhuang; Lei Wu; Mei-Zhen Qian; Ping-Ping Cai; Qi-Bing Liu; Gui-Xian Zhao; Zhen-Xin Li

2015-01-01

Background: Neuromyelitis optica (NMO) and multiple sclerosis (MS) are autoimmune demyelinating diseases of the central nerve system.Interleukin-7 (IL-7) and interleukin-7 receptor alpha (IL-7Rα) were proved to be important in the pathogenesis of both diseases because of the roles they played in the differentiations of autoimmune lymphocytes.The variants of both genes had been identified to be associated with MS susceptibility in Caucasian, Japanese and Korean populations.However, the association of these variants with NMO and MS has not been well studied in Chinese Southeastern Han population.Here, we aimed to evaluate the association of six IL-7 variants (rs 1520333, rs1545298, rs4739140, rs6993386, rs7816065, and rs2887502) and one variant of IL-7RA (rs6897932) with NMO and MS among Chinese Han population in southeastem China.Methods: Matrix-assisted laser desorption/ionization time of flight mass spectrometry (MassARRAY system) and Sanger sequencing were used to determine the variants ofIL-7 and IL-7RA in 167 NMO patients, 159 MS patients and 479 healthy controls among Chinese Han population in southeastern China.Samples were excluded if the genotyping success rate ＜90％.Results: Statistical differences were observed in the genotypes ofIL-7 rs 1520333 in MS patients and IL-7RA rs6897932 in NMO patients,compared with healthy controls (P =0.035 and 0.034, respectively).There was a statistically significant difference in the genotypes of IL-7 rs2887502 between MS and NMO patients (P =0.014).And there were statistically significant differences in the rs6897932 genotypes (P =0.004) and alleles (P =0.042) between NMO-IgG positive patients and healthy controls.Conclusions: The study suggested that among Chinese Han population in southeastern China, the variant of IL-7RA (rs6897932) was associated with NMO especially NMO-IgG positive patients while the variant of IL-7 (rs1520333) with MS patients.And the genotypic differences ofIL-7 rs2887502 between MS and NMO

Construcción de un indicador para medir competencias básicas para la vida

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Andrea Collado Chaves

2006-01-01

Full Text Available La necesidad de construir un indicador para cuantificar el nivel de Competencias Básicas para la Vida (CBV, nace con la evaluación los resultados de un proyecto dirigido a población joven. El propósito de ese proyecto es construir capital humano y empleabilidad en hombres y mujeres con edades entre 13 y 18 años, desertores o en riesgo de desertar del sistema educativo, sin empleo formal y que viven en ambientes de pobreza y exclusión. Una de las dimensiones de empleabilidad son las Competencias Básicas para la Vida. La dimensión de competencias básicas para la vida se refiere a aspectos de personalidad asociados con el desempeño del individuo en sus relaciones consigo mismo y con los demás. Los datos provienen de una investigación cuyo propósito consiste en evaluar los resultados de una intervención en población joven de comunidades pobres y excluidas. La construcción del indicador uso técnicas psicométricas para la validación de las distintas subdimensiones que pertenecen al concepto de competencias básicas para la vida. Según el marco conceptual este indicador está compuesto por seis dimensiones, la validación empírica hecha en este artículo incluye solamente cuatro de ellas, todas de carácter actitudinal. El indicador posee algunas ventajas en términos de la capacidad de discriminación debido a que su distribución tiende a ser normal. Sin embargo se ve afectaba por valores extremos a la izquierda del promedio y valores modales a una desviación estándar (positiva del promedio. Estos resultados serán útiles para discusiones futuras sobre los puntos de corte (baremos, el comportamiento del indicador cuando se correlaciona con deseabilidad social y la integración de la dimensión de Competencias Básicas para la Vida en el indicador de empleabilidad.

Propuesta metodológica para hacer un diagnóstico de la Educación para el Desarrollo

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Francisco Javier Gómez González

2012-01-01

Full Text Available El presente artículo quiere dar a conocer el diseño metodológico para la elaboración de un diagnóstico de situación de la educación para el desarrollo en Castilla y León, que se ha ideado desde el Observatorio de Cooperación Internacional de la Universidad de Valladolid con el fin de que el conocimiento sobre la situación actual de la educación para el desarrollo en Castilla y León sirva de base para la formulación de una futura estrategia de acción en educación para el desarrollo en la Comunidad.

Requisitos de clientes para o desenvolvimento de um implemento a fim de fechar taipas abertas para drenagem

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Ulisses Giacomini Frantz

2015-04-01

Full Text Available O arroz irrigado é cultivado na maior parte do RS com aplainamento da superfície do solo em desnível e sistema de cultivo mínimo. Com a semeadura em solo seco, há necessidade de conformar drenos, que cortam transversalmente as taipas, na área de cultivo. Para o estabelecimento da lâmina de água definitiva, essas taipas abertas para drenagem devem ser fechadas e, para essa finalidade, não existe um equipamento específico que execute tal atividade com devida agilidade e qualidade, sendo necessário o desenvolvimento de um implemento com este fim. Ainda, para o processo de desenvolvimento de máquinas agrícolas, faz-se necessário o levantamento de uma série de informações. Na fase de projetação, essas informações são necessárias para o estabelecimento das necessidades dos clientes/usuários e dos requisitos dos clientes, para posteriormente serem transformadas em requisitos de projeto e estabelecidas as especificações de projeto. Desse modo, este trabalho objetivou determinar os requisitos dos clientes, a partir das suas necessidades, para o projeto de um implemento com a função de fechamento de taipas abertas para drenagem no arroz irrigado. As informações foram levantadas a partir de pesquisas bibliográficas e aplicação de questionários a futuros clientes/usuários. Como resultados, foram identificadas as necessidades dos clientes, assim como 25 requisitos de clientes

Hiperacumulación de metales : ¿una ventaja para la planta y para el hombre?

OpenAIRE

Llugany i Ollé, Mercè; Tolrà Pérez, Roser; Poschenrieder, Charlotte; Barceló Coll, Juan

2007-01-01

La hiperacumulación es la capacidad que tienen algunas plantas para concentrar metales en sus tejidos a niveles muy por encima de los normales sin presentar síntomas de toxicidad. Estas plantas hiperacumuladoras han desarrollado mecanismos internos de tolerancia a la toxicidad por metales. Esta peculiaridad las hace útiles para el hombre como herramienta en las nuevas tecnologías de fitoremediación. Sin embargo, ¿cual es el beneficio de esta hiperacumulación de metales para la propia planta? ...

Hiperacumulación de metales: ¿una ventaja para la planta y para el hombre?

OpenAIRE

M. Llugany; R. Tolrà; C. Poschnrieder; J. Barceló

2007-01-01

La hiperacumulación es la capacidad que tienen algunasplantas para concentrar metales en sus tejidos a niveles muy por encima de los normales sin presentar síntomas de toxicidad. Estas plantas hiperacumuladoras han desarrollado mecanismos internos de tolerancia a la toxicidad por metales. Esta peculiaridad las hace útiles para el hombre como herramienta en las nuevas tecnologías de fitoremediación. Sin embargo, ¿cual es el beneficio de esta hiperacumulación de metales para la propia planta? S...

¿Para que la historia?

OpenAIRE

Jaime Jaramillo Uribe

1998-01-01

Para qué sirve la historia?. ¿Puede el conocimiento del pasado darnos pautas para comprender el presente? Sobre este, como sobre tantos problemas teóricos de la historia, se han realizado largos e inacabados debates entre historiadores y filósofos. En este ensayo se acepta como eficaz la hipótesis de que conocer el origen y el desarrollo de un proceso es buen fundamento para explicarse una situación final. En el caso de la historia colombiana, se parte de la base de que al producirse la indep...

Demyelinating syndrome in SLE: review of different disease subtypes and report of a case series

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E. Chessa

2017-12-01

Full Text Available Demyelinating syndrome (DS is a rare manifestation of systemic lupus erythematosus (SLE (1% with high clinical heterogeneity and potentially severe prognosis. It can represent a diagnostic and therapeutic challenge for clinicians. A recent study described 5 different patterns of demyelinating disease presentation, characterised by specific clinical, laboratory and brain and spine magnetic resonance imaging abnormalities: 1 neuromyelitis optica; 2 neuromyelitis optica spectrum disorders; 3 DS prevalently involving the brain; 4 DS prevalently involving the brainstem; 5 clinically isolated syndrome. In this review we briefly discuss typical characteristics of each DS presentation in SLE and we describe 5 illustrative clinical cases, one for each subset of DS, considering both diagnostic and therapeutic options.

Subvenciones para instituciones Directrices para gastos de ...

International Development Research Centre (IDRC) Digital Library (Canada)

IDRC CRDI

División de Administración de Subvenciones ... En la Parte 2 del Acuerdo de Subvención estándar de IDRC se incluyen los límites ... pagar los costos de sustitución (tiempo libre para personal académico) de los investigadores principales.

Zoneamento agroclimático da palma forrageira (Opuntia sp. para o estado da Paraíba

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Bergson G. Bezerra

2014-07-01

Full Text Available A palma forrageira tornou-se, ao longo dos anos, uma importante alternativa forrageira no semiárido brasileiro (SAB principalmente em longos períodos de estiagens. Apesar de sua magnitude para a agropecuária, seu cultivo tem dispensando tratos culturais básicos e embasamento técnico-científico quanto às suas exigências climáticas. Assim, o principal objetivo deste estudo foi elaborar o zoneamento agroclimático da cultura da palma forrageira (Opuntia sp. para o estado da Paraíba. O zoneamento agroclimático da palma forrageira foi elaborado com base nos indicadores climáticos delineados na literatura e nos dados climatológicos de precipitação e temperatura (média, máxima e mínima de 97 localidades do estado da Paraíba. De acordo com os resultados obtidos, a mesorregião da Borborema é a que apresenta as condições climáticas mais favoráveis para o cultivo da palma forrageira. As mesorregiões do Agreste, Sertão e a parte do Litoral, são aptas porém com restrições. No entanto, recomenda-se o cultivo da palma forrageira em todo o território do estado da Paraíba, exceto para a parte costeira da mesorregião do Litoral e região em torno de Areia. Em ambos os casos a inaptidão ocorre em função do excesso de precipitação.

Plan estratégico para la empresa Global Logistics (GL) para el periodo 2017-2019

OpenAIRE

Castello Manky, Luis Fernando; Grados Iturrizaga, Miguel Augusto; Hudson Okumura, Ciro

2017-01-01

El presente trabajo de investigación tiene como finalidad el desarrollo de un plan estratégico para la empresa Global Logistics (GL) para los años 2017 a 2019. Inicia con el análisis y diagnóstico situacional, donde se analiza el macroentorno en base al análisis PESTEL y luego se procede a analizar la industria considerando las cinco fuerzas de Porter, para, finalmente, determinar las oportunidades y amenazas que enfrenta la empresa. Una vez concluido el análisis y diagnóstico situacional, se...

Procedimiento para el cierre de canteras de materiales para construcción en Cuba

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Julio Montero-Matos

2016-03-01

Full Text Available Con el propósito de facilitar a los concesionarios de canteras de materiales para la construcción una herramienta metodológica para efectuar el cierre de estas y garantizar una minería sostenible se propuso un procedimiento en seis etapas a partir del diagnóstico realizado en 22 canteras, ubicadas en tres regiones mineras cubanas. El procedimiento busca disminuir los impactos ambientales provocados por la explotación de las canteras y ofrecer a la industria un estándar para lograr uniformidad en la planificación y ejecución del cierre con un costo mínimo.

Desenvolvimento de filmes biodegradáveis para cobertura de solo, ensacamento de frutos e sacos para mudas

OpenAIRE

Ana Paula Bilck

2010-01-01

Filmes plásticos de polietileno e polipropileno derivados do petróleo são amplamente utilizados na agricultura para a confecção de estufas, sacos para produção de mudas e filmes para cobertura do solo. Estes filmes constituem um sério problema ambiental, pois devido ao seu elevado peso molecular e propriedades hidrofóbicas, possuem alta estabilidade química, levando um longo tempo para se decompor totalmente e também são de difícil reciclagem. A utilização de polímeros biodegradáveis é uma al...

Taller para desarrollar la creatividad

OpenAIRE

MARTÍNEZ GUEROLA, BEATRIZ

2015-01-01

[ES] Partimos de la base de que la creatividad es una capacidad realmente valiosa, no solo para el ámbito artístico, si no para cualquier aspecto de la vida. Este proyecto está enfocado a un taller diseñado para que a partir de técnicas de expresión gráfico-plásticas podamos estimular y desarrollar la creatividad, principalmente en niños de tercer ciclo de primaria. Con este proyecto relacionamos el arte y la creatividad que se asocia a esté con la educación. Martínez Guerola, B. (2015). T...

Sistema solar para aquecimento ambiente e aquecimento de piscinas: uma solução promissora para climas do Sul da Europa

OpenAIRE

Carvalho, M. J.; Neves, Ana

2006-01-01

A avaliação do sistema apresentado neste trabalho foi focada em sistemas que podem fornecer água quente, aquecimento ambiente e aquecimento de piscinas, e que são projectados para climas do Sul da Europa, especialmente para habitações uni-familiares. Pelas características climáticas do Sul da Europa, o aquecimento ambiente é apenas necessário para um curto período do ano, sendo para esta avaliação considerado um período de seis meses para aquecimento ambiente e um período de seis meses para a...

MODELO PARA DETERMINAR FUNÇÕES TRANSFERÊNCIA PARA ANÁLISE DE ESTABILIDADE DE VANTS

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Igor Mayer Soares

2017-01-01

Full Text Available Neste trabalho foi apresentado um modelo para determinar as funções de transferência para análise da estabilidade longitudinal de Veículos Aéreos Não Tripulados (VANT através do desenvolvimento simplificado das equações do movimento de aeronaves. O sistema foi simplificado considerando que o motor e o arrasto na superfície de comando não influenciam na dinâmica do veículo, pois o mesmo é significativamente menor que um avião. Analisamos também a resposta em função do tempo para as deflexões das superfícies de comando. Nos resultados apresentados é possível perceber que mesmo simples, o modelo oferece uma boa aproximação para análise dinâmica de VANTs e predizer sua estabilidade e qualidade de voo.

MODELO PARA LA CREACIÓN DEL CONOCIMIENTO PARA PYMES

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Marino Valencia Rodríguez

2009-01-01

Full Text Available La creciente importancia del conocimiento, como nuevo factor de producción, hace que la creación y transferencia se convierta en una de las principales prioridades de las organizaciones. El presente artículo describe tanto los fundamentos de la teoría de los recursos y capacidades de la empresa como el enfoque de la gestión del conocimiento, con sus procesos de generación y transferencia del conocimiento. Estos sirven de guía básica teórica para alcanzar el siguiente objetivo: Diseñar un modelo de generación y transferencia de conocimiento para los procesos de dirección, gestión humana y del conocimiento para PyMES, con el fin de que éstas alcancen mayores niveles de competitividad. El modelo se diseña, a partir del direccionamiento estratégico de la organización. Está conformado por tres etapas, articuladas con elementos y actividades. Las acciones se enfocan en dos factores clave de éxito: la cultura organizacional y la formación. La utilización del modelo permitirá a las PyMES optimizar los recursos y capacidades disponibles, lo cual se refleja en el desempeño del trabajador y en el fortalecimiento de la cultura organizacional.

Flora da Paraíba, Brasil: Loganiaceae Flora of Paraíba, Brazil: Loganiaceae

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Kiriaki Nurit

2005-06-01

Full Text Available Neste trabalho apresenta-se o tratamento taxonômico da família Loganiaceae, como parte do projeto "Flora da Paraíba", que vem sendo realizado com o objetivo de identificar e catalogar as espécies da flora local. Realizouse coletas e observações de campo para as identificações, descrições e ilustrações botânicas que foram efetuadas após estudos morfológicos, com o auxílio da bibliografia especializada, complementados pela análise de fotos de tipos, espécimes dos herbários EAN, JPB e IPA, e comparação com material identificado por especialistas. Registrou-se para a Paraíba quatro espécies: Spigelia anthelmia L., com ampla distribuição, e três espécies de Strychnos, somente coletadas em remanescentes de Mata Atlântica, S. atlantica Krukoff & Barneby, S. parvifolia A. DC. e S. trinervis (Vell. Mart.This work constitutes a taxonomic treatment of the Loganiaceae family as part of the project "Flora da Paraíba", which have been carried out with the objective to identify and catalogue the species of the local flora. The botanical identifications and illustrations were made by morphological studies supported by bibliography, analysis of the types, specimens from herbaria EAN, JPB and IPA, and comparison with samples previously identified by specialists, complemented by field observations. Four species of Loganiaceae belonging two genera were found in State of Paraíba: Spigelia anthelmia L. that has wide distribution and three species of Strychnos, found on remains of Atlantic forest, which are S. atlantica Krukoff & Barneby, S. parvifolia A. DC. and S. trinervis (Vell. Mart.

Educación para emprender una alternativa para el siglo XXI

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Luis Gerardo Rodríguez Gómez

2015-05-01

Full Text Available RESUMEN El artículo describe la experiencia educativa del programa Físico- Deportivo orientado a estimular la actitud para emprender dentro de una concepción de forma integral, mediante Asignaturas de contenido Sico- físico, como parte del desarrollo curricular en Facultades de Administración de Empresas e ingeniería de Sistemas en la Escuela de Administración de Negocios (EAN. El desarrollo temático hace énfasis en un proceso orientado  a superar formas de educación encaminadas al logro de habilidades técnicas para el ejercicio de un empleo y propone desde  la educación delas conductas  motrices, modos de encausar la energía sicosomática con el fin de asimilar la incertidumbre del momento histórico que se vive en los países de lento desarrollo, para corresponder a un mundo que empuña la bandera de la diversidad, el cambio acelerado y la competencia tecnológica.

La carrera profesional para el profesorado

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José Gimeno Sacristán

2010-01-01

Full Text Available La carrera del profesorado es un instrumento para el reconocimiento del mérito de los profesores en el desarrollo de su profesionalidad -lo cual supone atender a la mejora de sus capacidades cognitivas como a su competencia reflexivo-práctica- cuyos fines son la motivación para el ejercicio de sus funciones, reforzar su compromiso con la educación en general y como forma de reconocer con justicia el mérito de los mejores profesionales. Desde un punto de vista estrictamente educativo, la carrera debe servir para la mejora de las prácticas de enseñanza que incidan en la mejora de los aprendizajes. Esta pretensión nos exige explicitar los criterios para determinar lo que entendemos como buenas prácticas y precisar los criterios para su evaluación. La complejidad de la enseñanza requiere metodologías adecuadas para abordar procesos de índole cualitativa. La regulación de la carrera docente tiene que superar tanto el cuantitativismo fácil en los métodos y la regulación burocrática de niveles de calidad, así como contemplar un conjunto de incentivos no solamente salariales.

Tiempo para un cambio

Science.gov (United States)

Woltjer, L.

1987-06-01

En la reunion celebrada en diciembre dei ano pasado informe al Consejo de mi deseo de terminar mi contrato como Director General de la ESO una vez que fuera aprobado el proyecto dei VLT, que se espera sucedera hacia fines de este aAo. Cuando fue renovada mi designacion hace tres aAos, el Consejo conocia mi intencion de no completar los cinco aAos dei contrato debido a mi deseo de disponer de mas tiempo para otras actividades. Ahora, una vez terminada la fase preparatoria para el VLT, Y habiendose presentado el proyecto formalmente al Consejo el dia 31 de marzo, y esperando su muy probable aprobacion antes dei termino de este ano, me parece que el 10 de enero de 1988 presenta una excelente fecha para que se produzca un cambio en la administracion de la ESO.

Leasing: una alternativa de financiamiento para PYMES

OpenAIRE

Bedjan, María Belén

2008-01-01

En la actualidad las pequeñas y medianas empresas encuentran dificultades para acceder a las líneas tradicionales de créditos bancarios. Por otro lado las empresas necesitan actualizarse constantemente para poder ser competitivas en el mercado. Es aquí donde el leasing nace como una alternativa de financiación para las PyMEs. Este trabajo pretendió demostrar la conveniencia financiera del leasing con respecto a los préstamos bancarios para la compra de maquinarias agrícolas ...

Estrategia para elevar la motivación laboral; factor imprescindible para mejorar nuestra productividad.

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Reina de los Ángeles Carballé Piñón

2015-12-01

Full Text Available Este trabajo "Estrategia para elevar la motivación laboral; factor imprescindible para mejorar nuestra productividad" resulta de la investigación realizada para tesis de maestría; se realizó el estudio de 2 entidades, nos permitió conocer las principales posiciones que sobre motivación existen, factores que la influencian, cómo incide en el desempeño laboral y que acciones diseñar para elevarla. Las 2 entidades seleccionadas, claves en el desempeño empresarial son un centro de interfase y un centro generador de conocimientos, nuestro objetivo principal es sugerir propuestas que eleven la motivación de los trabajadores del CIGET(centro de interfase y la Universidad de Sancti Spíritus(centro generador de conocimientos por desempeñar una mejor labor y lograr así aumento de la eficacia, eficiencia y productividad; este se concreta específicamente en determinar qué factores ocasionan desmotivación en los trabajadores del CIGET y la Universidad, identificar aquellos factores que realmente motivan a estas personas, de manera individual como colectivamente y trazar una estrategia con el objetivo de elevar la motivación de los trabajadores hacia la actividad que realizan y dotar a estos de herramientas para la identificación de actitudes positivas o negativas en el ámbito laboral y cómo potenciarlas o minimizarlas según sea el caso.

Competencia para desarrollar la alfabetización digital

OpenAIRE

González Rodríguez, José Santiago; Navarrete Aguirre, Mariana; Molina Zacate, Ma. Gabriela

2011-01-01

Introducción, Objetivos Generales, Temario: (1) ¿Qué significa la competencia para desarrollar la alfabetización digital? (2) ¿Por qué es relevante que adquiera la competencia para desarrollar la alfabetización digital en mi formación? (3) ¿Cómo aprendo la competencia para desarrollar la alfabetización digital? (4) ¿Cómo aplico la competencia para desarrollar la alfabetización digital? (5) ¿Cómo puedo autoevaluar mi competencia para desarrollar la alfabetización digital (...

Motivacao para o tratamento. Dados psicometricos da escala SOCRATES 8D e estrategias psicoterapeuticas para motivar para o tratamento

OpenAIRE

Janeiro, Luís; Faísca, Luís

2008-01-01

A motivação para o tratamento é considerada um factor fundamental para o processo de mudança e tratamento do toxicodependente. Prochaska, Diclemente, e Norcross (1992) consideram que a motivação que o sujeito possui no início do tratamento determina directamente os resultados alcançados no mesmo. Diclemente, Bellino, e Neavins (1999) acrescentam, ainda, que a estreita relação entre os resultados obtidos no tratamento e a motivação aquando do seu início pode ser medida pela formação precoce de...

La estrategia educativa 2020 o las limitaciones del Banco Mundial para promover el "aprendizaje para todos"

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Antoni Verger

2011-12-01

Full Text Available LA NUEVA Estrategia Educativa 2020 del Banco Mundial establece las prioridades de reforma educativa en países en vías de desarrollo para la década siguiente. El título explícito de la estrategia, Aprendizaje para Todos, es un claro reconocimiento de que, más allá de políticas centradas en el acceso, se debe hacer algo más para asegurar que la educación derive en experiencias positivas de aprendizaje. Sin embargo, como este artículo sostiene, las opciones de políticas explícitas y latentes en la Estrategia 2020 no son las más adecuadas para lograr el Aprendizaje para Todos. El artículo desarrolla tres tipos de argumentos al respecto. El primero se refiere al fuerte apego del Banco a un conocimiento disciplinario y un enfoque metodológico que es insuficiente para entender lo que aprenden los niños en la escuela y por qué. El segundo argumento se refiere al sesgo pro-mercado de la Estrategia por lo que respecta a la reforma del sector público y a nuevas formas de oferta educativa. En tercer lugar, el artículo señala las principales ausencias de la Estrategia, con especial atención a las omisiones relacionadas con la compleja relación entre educación y pobreza.

Inteligencia Emocional para tiempos de crisis

OpenAIRE

Fernández-Berrocal, Pablo

2014-01-01

La Inteligencia Emocional se concibe como un conjunto de habilidades, tanto básicas como complejas, dirigidas a “unificar las emociones y el razonamiento”, esto es, usar las emociones para facilitar el razonamiento y los procesos de pensamiento, y usar nuestro razonamiento para pensar de forma inteligente acerca de nuestras emociones (Mayer y Salovey, 1997). En concreto, la IE se define como una habilidad mental que incluye “la capacidad para percibir, valorar y expresar las emociones con...

GRASP para o PQA: um limite de aceitação para soluções iniciais

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Range Maria Cristina

2000-01-01

Full Text Available O Problema Quadrático de Alocação (PQA pertence à classe dos problemas NP-Hard e desafia os pesquisadores tanto em sua teoria quanto em sua parte computacional. Pela sua alta complexidade muitos métodos heurísticos têm sido desenvolvidos para tentar resolvê-lo aproximadamente. A metaheurística GRASP (greedy randomized adaptive search procedures se mostrou bastante eficiente. Neste trabalho, uma proposta para descartar soluções iniciais supostamente ruins é apresentada com base na normalização de custos calculadas num intervalo entre limites de solução. Para este GRASP restrito, foi observada uma redução do tempo computacional para encontrar as soluções ótimas ou soluções viáveis de boa qualidade quando comparado ao GRASP original.

Paisajes para un desarrollo sustentable y participativo

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Rafael Mata Olmo

2014-11-01

Para terminar, el texto recoge una experiencia de proyecto territorial de paisaje, la del Plan Insular de Menorca (Islas Baleares, España, aprobado en 2003. Se sintetizan sus determinaciones sobre la protección de determinados terrenos por sus altos valores paisajísticos, sus directrices para que las políticas sectoriales (turismo, agricultura, infraestructuras, etc. incorporen criterios paisajísticos, y sus iniciativas para la gestión y mejora del paisaje y para el fomento del acceso público a su contemplación y disfrute.

Sistema de realidad aumentada para aplicaciones Android

OpenAIRE

Fernández Sánchez, Natalia Mercedes

2012-01-01

El objetivo principal de este proyecto es la creación de un sistema de realidad aumentada para el sistema operativo Android con idea de que sea utilizado para la creación de un videojuego, pero que a su vez sea lo suficientemente versátil como para poder utilizarse en otro tipo de aplicaciones. De este objetivo principal podemos obtener los objetivos secundarios: - Conocer las características que ofrecen los teléfonos móviles y como éstas pueden ser aprovechadas para la crea...

Marco de referencia para el desarrollo de un sistema de apoyo para la toma de decisiones para la gestión de inventarios

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Carlos Alberto Castro Zuluaga

2014-01-01

Full Text Available El principal objetivo de este artículo es presentar un marco de referencia para desarrollar un sistema de soporte a la toma de decisiones para la gestión de inventarios que permita definir técnicamente los parámetros de una política de control de inventario específica. El modelo propuesto puede ser utilizado de forma genérica en casi cualquier tipo de industria, el cual incluye las entradas fundamentales, una descripción de los procesos internos requeridos y las salidas esperadas. En la investigación se utilizaron métodos inductivos-deductivos para definir el marco de referencia propuesto, el cual se fundamenta en dos indicadores clave en la gestión de inventario: niveles de servicio y costos. Finalmente, mediante una simulación es posible concluir que utilizando los parámetros teóricos para el modelo de inventario punto de reorden-cantidad fija, que son encontrados por el marco de referencia propuesto, se obtienen aproximadamente los valores esperados tanto de los niveles de servicio como de los costos.

Meios e fins do desenvolvimento para Celso Furtado

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Fabio Antonio de Campos

2014-10-01

Full Text Available O objetivo desse artigo é expor o conceito de desenvolvimento para Celso Furtado. Para tanto, nos interessa apresentar o desenvolvimento nacional como um fim em sua abordagem sobre a formação social brasileira Para atingir tal estágio, os meios para o autor seriam assegurados por um típico desenvolvimento capitalista. Dentro dessa problemática, mostraremos também o alcance e os limites dessa concepção para o caráter dependente e subdesenvolvido do capitalismo brasileiro.

Metodología para un proceso de planeación estratégica para las PYMES

OpenAIRE

Bonilla Zhindón, Daniela

2014-01-01

Todas las organizaciones, grandes, medianas, pequeñas, públicas y privadas, requieren de un plan estratégico estructurado para determinar las posiciones futuras en las que desean estar, por este motivo el propósito del presente estudio es construir una metodología transversal para el levantamiento de la planeación estratégica para el sector de calzado en la ciudad de Cuenca, mediante un análisis del estado en la que se encuentran las empresas en cuanto al proceso de planificación estratégica,...

Coccocypselum pulchellum (Rubiaceae, nuevo registro para Argentina

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Elsa L. Cabral

2007-01-01

Full Text Available Se cita Coccocypselum pulchellum por primera vez para Argentina, en Predio Guaraní, Misiones. Esta es la tercera especie de Coccocypselum registrada para la flora de Argentina junto con C. hasslerianum y C. lanceolatum. Se incluyen descripciones, ilustraciones y una clave para reconocer las tres especies argentinas de Coccocypselum.

Modelo para determinar as exigências de proteína para poedeiras Modelling protein utilization in laying hens

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Nilva Kazue Sakomura

2002-11-01

Full Text Available O objetivo do trabalho foi elaborar um modelo para estimar as exigências de proteína bruta (PB para poedeiras leves, usando o método fatorial. Para determinar as exigências de proteína bruta (PB para manutenção foi utilizada a técnica do balanço de nitrogênio. A exigência de proteína bruta para o ganho de peso foi determinada em função do conteúdo de nitrogênio na carcaça e a eficiência de utilização do nitrogênio da dieta. A exigência de PB, para produção de ovos, foi determinada considerando o teor de PB determinado nos ovos e a eficiência de deposição do nitrogênio no ovo. A partir dos valores das exigências para manutenção, para o ganho e produção foi elaborada uma equação para predizer as exigências diárias de PB (g/ ave/ dia para poedeiras: PB = 1,94. P0,75 + 0,48.G + 0,301.O, em que P = peso corporal (kg, G = ganho de peso diário (g/dia e O = massa de ovos produzida (g/ave/dia.The objective of this study was to determine a model for crude protein requirements (CP for laying hen by the factorial method. The protein maintenance requirement was determine by the nitrogen balance technique . The crude protein requirement for weight gain was determined based on body nitrogen content and nitrogen efficiency for body deposition. The crude protein requirement for egg production was determined based on the nitrogen content of eggs and nitrogen efficiency for egg deposition. Considering the requirements for maintenance, egg production and weight gain, it was elaborated a protein requirement model for laying hen: PB = 1.94xW.75 + 0.480xG + 0,301x E, where PB = requirement (g/bird/day, W = body weight (kg, G = daily weight gain (g/day and E = egg mass (g/bird/day.

Tuberculose em Salvador: custos para o sistema de saúde e para as famílias

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Costa João G

2005-01-01

Full Text Available OBJETIVO: A tuberculose é uma das maiores causas de mortalidade no mundo, porém seus efeitos econômicos são pouco conhecidos. O objetivo do estudo foi o de estimar os custos do tratamento e prevenção da tuberculose para o sistema de saúde (público e privado e para as famílias. MÉTODOS: O estudo foi realizado no município de Salvador, BA, em 1999. Os dados para estimação dos custos para o sistema de saúde foram coletados nas secretarias de saúde, centros de saúde e em uma entidade filantrópica. Os custos públicos e privados foram estimados pela metodologia da contabilidade de custos. Os dados de custos para as famílias foram coletados por meio de questionários e incluem despesas com transporte, alimentação e outros, bem como as perdas de renda associadas à doença. RESULTADOS: O custo médio para tratamento de um caso novo de tuberculose foi de aproximadamente R$186,00 (US$103; para o tratamento de um paciente multiresistente o custo foi 27 vezes mais alto. Os custos para o serviço público corresponderam a 65% em internações, 32% em tratamento e apenas 3% em prevenção. As famílias comprometeram cerca de 33% da sua renda com despesas relacionadas a tuberculose. CONCLUSÕES: Apesar do fato das famílias não terem que pagar por medicamentos e tratamento, dado que este serviço é oferecido pelo Estado, os custos familiares ligados a perda de rendimentos devido a doença foram muito elevados. A proporção utilizada em prevenção pelo serviço público é pequena. Um maior investimento em campanhas de prevenção poderia não somente diminuir o número de casos, mas também, levar a um diagnósticos precoce, diminuindo os custos associados à hospitalização. A falta de um sistema integrado de custos não permite a visualização dos custos nos diversos setores.

UNA PROPUESTA EVALUATIVA PARA ACTIVIDADES DE EDUCACIÓN AMBIENTAL PARA LA SOSTENIBILIDAD

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Rosa M. Medir Huerta

2016-01-01

Full Text Available El propósito de este artículo es presentar una propuesta evaluativa para ac - tividades de educación ambiental para la sostenibilidad en el ámbito escolar ofrecidas desde entidades externas a la escuela. Para ello, se realiza una eva - luación real de un programa educativo de una administración pública que se orienta al refuerzo del currículo de educación infantil, primaria y secundaria. El objetivo de la evaluación es diagnosticar la presencia, en las actividades estudiadas, de principios clave de la educación ambiental para la sosteni - bilidad identificados por el equipo de investigación a partir de referencias internacionales. La metodología es la investigación evaluativa de programas dentro del paradigma crítico de la investigación educativa y con herramientas de tipo cuantitativo. Se utiliza, básicamente, un cuestionario expresamente diseñado que valora la presencia y la ausencia de los principios establecidos de la educación ambiental para la sostenibilidad. Los resultados nos aportan nuevas informaciones acerca de las fortalezas y las debilidades del desarrollo de actividades de educación ambiental. Se llega a una valoración cuantificada de los principios definidos en tres grupos, según su necesidad de mejora, y se determina que los principios mejor tratados en las actividades son los que se relacionan con los contenidos disciplinares más clásicos, tratados con buenos y distintos métodos didácticos. En cambio, las debilidades detectadas se refieren a aspectos cruciales de la educación ambiental tales como experimentar nuevas sensaciones, aprender a buscar información, pensamiento crítico, sentimiento de pertenencia a un lugar, trabajo en colaboración, adquirir una visión global de la realidad, y reflexionar sobre la vida cotidiana y sobre el futuro deseado.

TRABALHAR PARA ESTUDAR / ESTUDAR PARA TRABALHAR: REALIDADE E POSSIBILIDADES

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Janes Siqueira

2011-07-01

Full Text Available O trabalho que apresentamos é parte da pesquisa com os estudantes das licenciaturas e da pedagogia da Universidade de Santa Cruz do Sul-UNISC-RS-Brasil. Este está inserido na linha de pesquisa: educação, trabalho e emancipação. Queremos compreender, à luz do materialismo histórico e dialético e das categorias da crítica da economia política, os significados atribuídos pelos estudantes universitários ao fenômeno trabalho e estudo. É uma pesquisa preferentemente qualitativa de natureza dialética. Para Marx, a essência da realidade humana reside no trabalho, mas a fonte de toda a riqueza está na natureza. Logo, trabalho, natureza e sociedade estão em relação dialética. Trabalho, portanto, é a ideia central articuladora, e natureza e sociedade devem ser estudadas em conexão com o trabalho. Marx trata o trabalho, no modo de produção capitalista, como impedimento ao desenvolvimento humano. Propõe o mesmo como um ato de criação e auto-expressão humana que não deve ter um valor. A compreensão dessa premissa é necessária para que os trabalhadores possam significar e ressignificar o trabalho para além da ideologia dominante. Algumas categorias aparecem como relevantes para a análise: condições de trabalho e de estudo, necessidade de formação, predominância do trabalho sobre o estudo, dificuldade de conciliar tempo de estudo e de trabalho. Na relação com o objeto de estudo, ressaltaremos a crise estrutural do capital, a desregulamentação das leis do trabalho e sua flexibilização e as contradições entre trabalho e estudo bem como quais possibilidades emancipatórias são visualizadas ou sonhadas pelos trabalhadores-estudantes. PALAVRAS-CHAVE: contradições entre trabalho e educação, condições e significados, realidade e possibilidades.

Educación para la sostenibilidad

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Educadores por la sostenibilidad

2006-04-01

Full Text Available La importancia dada por los expertos en sostenibilidad al papel de la educación queda reflejada en el lanzamiento mismo de la Década de la Educación para el Desarrollo Sostenible o, mejor, para un futuro sostenible (2005-2014 a cuyo impulso y desarrollo esta destinado este artículo, así como la página web que la Organización de Estados Iberoamericanos ha habilitado dentro de su portal digital, para la celebración de la Década.

Obtención de ferromanganeso alto carbono y escoria para el desarrollo de materiales para soldar

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Lorenzo Perdomo-González

2000-03-01

Full Text Available En el presente trabajo se realiza un análisis de la posibilidad de empleo de pirolusita en el desarrollo de aleaciones al manganeso y escorias con un sistema de óxidos MnO-SiO2-CaO con características propias para el desarrollo de fundentes para la soldadura automática para arco sumergido (SAAS. A partir de la composición química del mineral de manganeso se realizan los cálculos del proceso de reducción con el empleo de la tecnología sin fundente en presencia de coque como reductor, obteniéndose las adecuadas composiciones y las propiedades de los productos del proceso. Se realiza una corrida experimental de obtención de ferromanganeso con adiciones de dolomita, caolín y fluorita como correctores de la escoria. Se conforma una matriz de fundente para arco sumergido con la escoria y se le evalúan las propiedades tecnológicas de soldadura.

Avaliação das competências de professores de enfermagem para desenvolver programas educativos para adultos

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Patricia Bover Draganov

2013-08-01

Full Text Available Avaliar o desempenho que o enfermeiro docente de enfermagem atribui a si, nas competências para desenvolver programas educativos para adultos, foi objetivo desse estudo descritivo, comparativo, transversal e quantitativo, desenvolvido com 226 docentes enfermeiros de cursos de graduação em enfermagem paulistanos. Os dados foram colhidos por escala de Likert, lançados em planilha Excel® e analisados por estatística descritiva e testes não paramétricos de Wilcoxon e Friedman. A população tinha predominantemente entre 46-55 anos (87/38,49%, eram mulheres (198/87, 61% e mestres (180/79, 65%. Os professores consideraram-se distantes das competências que almejavam alcançar. A menor habilidade esteve em construir desenhos de programas para atender às situações de aprendizagem e a maior, em avaliar programas de ensino. O maior desejo esteve em desenhar programas com formatos criativos e o menor desejo para usar conselhos, comitês e força-tarefa. Concluiuse que desenhar programas educativos para adultos ainda é algo pouco explorado e pouco experenciado por esses docentes.

Caminhos para a gestão integrada do território no Vale do Paraíba de 1950 a 2004

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José Luiz Ronca Caruso

2004-01-01

Full Text Available The Paraíba Valley region and its political-administrative frame. The region and its two phases of Planning: the economic development and the sustainable one. The Paraíba Valley Road System as a policy of the economic planning. The Paraíba Valley Region and its physical conformation: the Paraíba do Sul river basin. The water resources management in the Paraíba do Sul river basin. Two levels of management: the federal and the one of the State. The water resources Planning and its possibilities for a sustainable development of the region, through a comprehensive management

Universidades accesibles para todos

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Roxana Stupp Kupiec

2001-01-01

Full Text Available Este trabajo pretende hacer conciencia sobrelos factores que inciden en la construcción colectiva deuniversidades dispuestas a innovar y transformar suideología, sus políticas, el currículum, los servicios estu-diantiles, la infraestructura física y administrativa y lasestrategias de comunicación e información, bajo el para-digma de la accesibilidad en el entorno. Se hace referen-cia al marco legal internacional y nacional que orientalas acciones que deben planificarse e implementarse enlas instituciones de Educación Superior.En la construcción de universidades accesibles para to-dos se requiere de un cambio en la concepción curricu-lar. Se analizan conceptos como la flexibilidad en la for-mación y el acceso a los planes de estudio para respondera las necesidades educativas de todos los estudiantes. Para promover estas transformaciones se proponen prin-cipios y prácticas tales como la interdisciplinareidad y lainclusión del tema de la discapacidad en todas las carre-ras. Además, se presentan ejemplos de aplicación en dife-rentes áreas del perfil profesional, en la formación deldocente universitario y en actividades de proyección so-cial, vida estudiantil e investigación. En el ámbito estu-diantil, se describen algunos servicios y procesos que sehan desarrollado en la Universidad de Costa Rica

Propuesta para el cálculo del Índice Dm para la validez de los ítemes

OpenAIRE

Dominguez Lara, Sergio

2013-01-01

Se presenta un programa en formato MS Excel para calcular el índice Dm para la validez de los ítemes. La validez es un proceso de acumulación de pruebas para apoyar la interpretación y el uso de las puntuaciones de los tests psicológicos, siendo las relaciones de los puntajes con otras variables externas una importante fuente de validación. Una variante es la llamada validez relacionada al criterio, en la cual se espera una correlación significativa con aquellos criterios externos que son teó...

Educación para el Ocio: Ha llegado la hora de un nuevo objetivo para una vieja idea.

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Susan Hutchinson

2012-01-01

Full Text Available La Educación para el Ocio nos sitúa ante una vieja idea, que está siendo examinada con renovado vigor en Canadá. Profesionales e investigadores de diversas disciplinas (por ejemplo, en el ámbito de Salud, la Justicia, la Educación o la Recreación reconocen, cada vez más, que son muchas las personas que pueden beneficiarse del ocio y de las oportunidades de recreación en sus comunidades, aún cuando son bastantes las no pueden hacerlo porque carecen de alfabetización en ocio. Esto es, de los conocimientos, habilidades y confianza que se necesitan para darle un significado personal, que incida positivamente en su bienestar y calidad de vida. La Educación para el Ocio es un medio clave para mejorar la alfabetización en ocio. El texto presenta un modelo de educación para el ocio desarrollado por la Dra. Brenda Robertson, argumentando cómo y por qué la Educación para el Ocio es necesaria para abordar la salud mental y el bienestar de las personas que sufren marginación en sus comunidades. Complementariamente se presentan algunas recomendaciones para avanzar esta Educación, como un componente fundamental de la agenda nacional para la recreación en Canadá.--------------------------------------------------------------------Leisure education is an old idea that is being examined with renewed vigour in Canada. Practitioners and researchers from various disciplines (e.g., health, justice, education, recreation are recognising anew that many people who could benefit from leisure or recreation opportunities in their communities are unable to do so because they lack leisure literacy. Leisure literacy refers to the knowledge, skills and confidence to engage in personally meaningful, health-enhancing leisure. Leisure education is a key means to enhance leisure literacy. The paper presents a model of leisure education developed by Dr. Brenda Robertson and argues for how and why leisure education is needed to address the mental health and well

O efeito trabalhador adicional para filhos no Brasil

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Elzira Lúcia de Oliveira

2014-06-01

Full Text Available O objetivo deste trabalho é testar a hipótese da existência do efeito trabalhador adicional para filhos no Brasil, procurando identificar se a situação de desemprego do chefe de família fará com que algum membro da família, cuja condição seja filho, transite para a População Economicamente Ativa - PEA. A base de dados utilizada foi a Pesquisa Mensal de Emprego - PME realizada pelo IBGE, que permite a construção de painéis para análise longitudinal de dados. A hipótese foi testada para pelo menos um filho com idade de 10 a 18 anos, entre 2002 e 2013, para as regiões metropolitanas de Belo Horizonte, Salvador, Porto Alegre, São Paulo, Rio de Janeiro e Recife, que compõem a área de abrangência da PME. Este trabalho admite a existência de diferencial por sexo do chefe para o efeito trabalhador adicional de filhos de 10 a 18 anos. Os resultados mostraram haver um efeito positivo maior para chefes homens do que para chefes mulheres, sendo que a variável de transição do filho para a atividade não apresentou significância estatística que permitisse assumir a existência do efeito. Corroborou-se a hipótese da existência de diferencial por sexo, contudo no sentido oposto ao da hipótese assumida.

Biocomercio: una alternativa para el desarrollo sostenible

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Carlos Murillo R.

2008-01-01

Full Text Available El desarrollo sostenible en países ricos en biodiversidad dependerá de la capacidad de los gobiernos de implementar los arreglos institucionales necesarios para conciliar la preservación de los recursos naturales con el uso de los mismos para la producción de bienes y servicios que puedan potenciar el crecimiento económico y un mayor bienestar para las comunidades locales donde estos recursos se encuentren. Este es el objetivo de la Iniciativa de Biocomercio que ha venido impulsando la Conferencia de Naciones Unidas para el Comercio y el Desarrollo (UNCTAD en distintos países de Latinoamérica. En el tanto estos principios y los establecidos en el CDB se puedan viabilizar e inspiren los distintos Programas Nacionales de Biocomercio en los países de la región, parecería no haber dudas de que la Iniciativa abre nuevas puertas para el uso, aprovechamiento y desarrollo sostenible de los mismos.

ANÁLISE, MODELAGEM E IMPRESSÃO 3D DE UM FRAME PARA VANTs DO TIPO QUADRÍCOPTEROS PARA VOOS EM AMBIENTE INDOOR

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Rafael Rocha Gomes

2017-01-01

Full Text Available O foco do projeto de pesquisa foi analisar, modelar e construir utilizando uma impressora 3D, um frame de um Veículo Aéreo Não Tripulado (VANT para voos em ambiente indoor. Esta aeronave será usada para experimentos laboratoriais, que exijam características especiais para ambientes indoor e de pesquisa, tais como, ser compacto, leve e uma estrutura que minimize os riscos aos operadores, pois dividirá espaço com pessoas. A motivação desta pesquisa se deu pelo fato dos frames disponíveis no mercado serem voltados para voos outdoor, como por exemplo, os utilizados para monitoramento. Com o intuito de proporcionar uma solução de aeronave para espaço laboratorial indoor, a proposta deste trabalho foi de modelar e imprimir em 3D um protótipo de frame que atenda todos os requisitos necessários para este tipo de voo.

Para una Mejor Comprensión del Desarrollo: Análisis conceptual, lógica para su abordaje e instrumentos para su medición

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Héctor Cárcamo

2006-01-01

cross nacional para la realización de estudios vinculados al desarrollo. Se plantea una revisión de conceptos fundamentales como el de desarrollo y la necesidad de la equivalencia funcional. El documento continúa con una reflexión guiada desde la noción de desarrollo social; asumiendo críticamente que el desarrollo se ha plasmado como un intento por lograr una radiografía del todo que ha olvidado la integración de sus partes constituyentes, negando de esta manera la diversidad como condición esencial para dar sentido a la idea de una visión integradora. Finalmente se realiza una revisión de CASEN como instrumento para medir el desarrollo social en Chile.

GOOGLE DRIVE: POTENCIALIDADES PARA O DESIGN DE MATERIAL EDUCACIONAL DIGITAL (MED PARA ENSINO DE LÍNGUAS

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Rosinda de Castro Guerra RAMOS

2017-07-01

Full Text Available Este trabalho tem como objetivo apresentar uma experiência sobre como o Google Drive pode ser usado para o desenho de MED (Material Educacional Digital para o ensino-aprendizagem de línguas, devido ao crescente uso das TDICs (Tecnologias Digitais de Informação e Comunicação na área de educação e aos desafios que o design de materiais educacionais para o ambiente digital nos lançam.  Para apresentar nossa experiência, faremos uma discussão teórica sobre os conceitos de desenvolvimento de material didático (TOMLINSON, 2003; RAMOS, 2009, de material educacional digital –MED – (BEHAR, 2009, multimodalidade (ROJO e BARBOSA, 2015, a lacuna existente em relação ao  letramento digital na formação de professores (LEFFA, 2013, os níveis/tipos  de interação em ambiente digital (HIRUMI, 2013 e sua relação na coconstrução de novos conhecimentos (VIGOTSKY, 1989, 1998; OLIVEIRA, 1997; BRASIL, 1998. Em seguida, descreveremos o Google Drive e as funcionalidades dos aplicativos de produtividade Google Documentos, Planilhas, Apresentações e Formulários. Finalizaremos com considerações sobre essa experiência e suas contribuições para a área de formação de professores de línguas.

Factores de riesgo para el neonato pequeño para la edad gestacional en un hospital de Lima, Perú

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J. Eduardo Tejeda-Mariaca

Full Text Available Objetivos. Identificar factores de riesgo para neonatos a término pequeños para la edad gestacional. Materiales y métodos. Cohorte retrospectiva que utilizó datos del Sistema Informático Materno Perinatal del Hospital María Auxiliadora de Lima, del período 2000 a 2010. Se evaluó la edad materna, paridad, nivel educativo, estado civil, índice de masa corporal pregestacional, número de controles prenatales, presencia de patologías como preeclampsia, eclampsia, infección urinaria y diabetes gestacional como factores de riesgo en pequeños para edad gestacional. El peso para la edad gestacional fue calculado sobre la base de percentiles peruanos. Se calcularon los riesgos relativos crudos (RR y ajustados (RRa con sus intervalos de confianza al 95% usando modelos lineales generalizados log binomial. Resultados. Se incluyeron 64 670 gestantes. La incidencia de pequeños para la edad gestacional fue 7,2%. La preeclampsia (RRa 2,0; IC 95%: 1,86-2,15, eclampsia (RRa 3,22; IC 95%: 2,38-4,35, bajo peso materno (RRa 1,38; IC 95%: 1,23-1,54, nuliparidad (RRa 1,32; IC 95%: 1,23-1,42, edad ≥35 años (RRa 1,16; IC 95%: 1,04-1,29, tener controles prenatales de 0 a 2 (RRa 1,43; IC 95%: 1,32-1,55 y 3 a 5 (RRa 1,22; IC 95%: 1,14-1,32 fueron factores de riesgo para ser pequeños para la edad gestacional. Conclusiones. Es necesario identificar a las gestantes con factores de riesgo como los encontrados para disminuir la condición de pequeños para la edad gestacional. Se debe actuar poniendo énfasis en factores modificables, tales como la frecuencia de sus controles prenatales

Ciclo Hápticos: Para Quinteto de Sopros

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Jorge Luiz de Lima Santos

2016-06-01

Full Text Available Memorial e partitura de Ciclo Hápticos, escrita em 2014 para quinteto de sopros. O memorial descreve brevemente as ferramentas metodológicas empregadas para o planejamento e criação da obra. De um lado, para produção do material melódico-harmônico foi empregado o Sistema-Gr de composição musical, desenvolvido por Carlos Almada (2014. Do outro lado, para organização formal, utilizamos o software Partitions - que faz parte do conjunto de programas do Parsemat, elaborado por Pauxy Gentil-Nunes (2009 – o qual informa o conjunto total de possibilidades de partições de um dado número.

Palladium-mediated conversion of para-aminoarylboronic esters into para-aminoaryl- 11C-methanes

DEFF Research Database (Denmark)

Andersen, Valdemar Lykke; Herth, Matthias Manfred; Lehel, Szabolcs

2013-01-01

of free amines. Aryl boronic esters containing primary, secondary, and tertiary amines were successfully converted into corresponding labeled methyl derivatives in suf¿cient radiochemical yield to apply this method for tracer development. This procedure was applied to the labeling of CIMBI-712......Cross-couplings are an alternative to conventional 11C-methylations which are generally employed in PET tracer synthesis. Therefore, we set out to develop a general procedure for the synthesis of para-11CH3 labeled aromatic amines from the corresponding para-aminoarylboronic esters in the presence...

Sistema de autenticidad para aplicaciones de análisis de eventos para seguridad.

OpenAIRE

ESCAMILLA PARDO, TONI

2017-01-01

[ES] En este proyecto se afronta la creación de un sistema distribuido capaz de garantizar, en aplicaciones empleadas para el análisis de eventos de seguridad, la autenticidad de los datos transmitidos entre el host cliente y servidor con el fin de asegurar la integridad de los mismos en la transmisión entre ambas partes, así como evitar la posible alteración en el destino. Para ello, se utilizará la tecnología blockchain a modo de base de datos en la que se almacenen los hashe...

Para uma abordagem virtuosa do Jornalismo

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Rogério Christofoletti

2012-06-01

Full Text Available As últimas décadas têm favorecido o crescimento do interesse e dos estudos sobre a ética jornalística. No Brasil e em outras praças, as abordagens deste tema têm sido na maioria das vezes por um caráter deontológico ou utilitarista. Neste artigo, chamamos a atenção para uma perspectiva que privilegie as virtudes como pontos de partida para os debates acerca das condutas dos profissionais. Uma ética das virtudes remete a Aristóteles e aos estoicos, mas vem sendo retomada por autores mais contemporâneos, como Macintyre e Comte-Sponville, entre outros. No jornalismo, esta abordagem não é definitiva ou totalizante, mas contribui para uma compreensão mais ampla da discussão, chamando inclusive a atenção para recomendações éticas úteis, práticas e atuais.

Educación para la Salud: espacio curricular optativo para los estudiantes de la carrera de Medicina

OpenAIRE

Susacasa, Sandra; Candreva, Anna; Cerasa, Silvana; Azanza, Marisa

2016-01-01

La educación para la salud adquiere relevancia en los programas institucionales internacionales. Estos se orientan a promover un mayor nivel de salud en la población. Sin embargo, las problemáticas vinculadas al campo de la educación para la salud, de acuerdo con los informes epidemiológicos, no están mejorando. Aún más, la situación se vuelve más crítica. En nuestro país las demandas sociales expresan la urgente necesidad de instalar la Educación para la Salud en espacios educativos y de sal...

Modelo para la implementación de controles internos para empresas constructoras.

OpenAIRE

Choez Chiquito, Wilton Antonio

2015-01-01

La presente investigación se refiere al tema Metodología para la implementación de nuevos métodos de Control para las empresas de construcción en el Ecuador. Donde las características principales de este sector ha sido su rápido crecimiento en las últimas dos décadas, con un alto número de trabajadores de obras con bajo o ningún nivel de educación, falta de compromiso por parte de los empleados de obra por el alto nivel de rotación que puede existir debido a que las construcciones son por per...

ELEMENTOS PARA LA CONSTRUCION DE UN PERFIL PARA EL GERENTE SOCIAL EN UN ENTORNO GLOBALIZADO

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Antonio Bastidas Unigarro

2009-07-01

Full Text Available El perfil del gerente social adecuado para enfrentar los retos del mundo globalizado se ha estructurado a partir de cuatro requerimientos fundamentales: los conocimientos, la experiencia, las habilidades y las actitudes, los cuales se analizan como competencias indispensables para administrar la problemática compleja, llena de incertidumbres y desafíos del nuevo milenio. La propuesta para la construcción de un perfil de este gerente parte de considerar tres tipos de competencias: las funcionales, las comportamentales y los requisitos de estudio y experiencia. Su construcción parte del análisis de la Gerencia Social en el estado neoliberal, escenario, que reclama la formación de gerentes sociales comprometidos en la construcción de una sociedad mas justa, incluyente, participativa, democrática y solidaria.

Nuevo lenguaje para un nuevo medio

Directory of Open Access Journals (Sweden)

Inma Martín Herrera

2014-12-01

Full Text Available El concepto tradicional de comunicación unidereccional se ha roto para convertirse en comunicación pluridireccional que permitirá la retroalimentación informativa. Las nuevas formas de redactar, contar diseñar y presentar la información en Internet exige nuevos hábitos de lectura por parte de la audiencia. El lector deberá tomar conciencia de que está frente a un medio diferente con el que debe familiarizarse para hacer uso correcto del soporte digital, se informará adecuadamente para disfrutar de las ventajas del innovador medio, conocido como Periodismo de servicios o Nuevo Periodismo.

“Libros para niñas y libros para niños”: Presencia de estereotipos de género en una colección de libros para dibujar

Directory of Open Access Journals (Sweden)

Yolanda Cabrera García-Ochoa

2014-06-01

Full Text Available Los aprendizajes que se producen a partir de las propuestas imaginarias y simbólicas en los materiales didácticos pueden contener elementos sexistas. El objetivo del estudio consiste en determinar, a través de un análisis de contenido, si la colección de libros para dibujar “Doodle para niñas” y “Doodle para niños” representa roles estereotipados de género en sus imágenes y en el texto escrito. Tanto a nivel de representación gráfica como escrita, ambos libros utilizan roles estereotipados sobre todo en detrimento de la mujer. Aunque el sistema educativo español señala hace años un modelo igualitario para eliminar la discriminación, los avances conseguidos desde un prisma constitucional y legal, van muy por delante de unos sistemas culturales y sociales que apenas han evolucionado.

Plan de mercadeo para una promotora de eventos corporativos

OpenAIRE

Burbano Zhindon, Andrea Cristina; Chavez Lopez, Maria Cecilia; Orlando Haro, Sofia Pierina; Miranda, Jorge Luis

2009-01-01

Este proyecto presenta una alternativa para todas aquellas empresas que realizan eventos frecuentemente, ofreciéndoles un servicio personalizado para la coordinación de cada uno de los detalles que conlleva la organización de un evento. El objetivo fundamental de este estudio es desarrollar un plan de mercadeo para el servicio antes mencionado, aplicando los principios del marketing para su correcta implementación.

Agricultura familiar para el desarrollo rural incluyente

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Laksmi Reddiar Krishnamurthy

2017-04-01

Full Text Available Las comunidades indígenas Mayas de la península de Yucatán han practicado la antigua tradición de agricultura familiar, en particular los huertos caseros, para garantizar su seguridad alimentaria. Con el objetivo de mejorar la práctica tradicional considerando paradigmas de la ciencia moderna, por una parte, se colectaron datos para definir la complejidad estructural y diversidad funcional a partir de 20 huertos familiares en cinco comunidades: X - Maben, X - Pichil, X - Yatil, San José II y Melchor Ocampo; y por otra, se organizaron grupos de discusión para dilucidar la estrategia de gestión practicada por las comunidades nativas. Los resultados mostraron que los huertos son manejados principalmente por las mujeres. También mostraron que el propósito principal del crecimiento y mantenimiento de los huertos familiares es garantizar la producción de alimentos nutritivos durante todo el año. Y, por último que los huertos caseros también sirven para propósitos secundarios tales como la provisión de productos y servicios para la medicina tradicional. El estudio sugiere que se debe de promover e invertir en huertos caseros para mejorar las estrategias de desarrollo incluyente en ambientes socio-culturales y biofísicos similares.

Patrones de diseño para mejorar la accesibilidad y uso de aplicaciones sociales para adultos mayores

Directory of Open Access Journals (Sweden)

Huizilopoztli Luna García

2015-07-01

Full Text Available El objetivo de este artículo fue proponer una colección no exhaustiva de 36 patrones para el diseño de interacciones en aplicaciones sociales para adultos mayores. La propuesta representa un punto de partida confiable para facilitar, a diseñadores/desarrolladores, la integración de usabilidad en las interfaces de estas tecnologías con el fin de fomentar su aceptación, uso y adopción entre los adultos mayores. Esta propuesta se basa en esfuerzos previos comúnmente expresados como criterios y guías de diseño para definir los posibles problemas de usabilidad en este tipo de interfaces, y se concentra en reforzar estos enfoques mediante la integración de una mayor descripción de tales anomalías y alternativas de solución bajo una estructura de patrones útil para los diseñadores/desarrolladores. Se realizó un estudio de usabilidad con la técnica «evaluación heurística» (comúnmente utilizada en interacción hombre-máquina para obtener la percepción de los usuarios sobre un diseño particular. El trabajo se llevó a cabo desde dos perspectivas: el punto de vista de expertos técnicos y la percepción de un grupo social de adultos mayores. Los resultados reflejaron que los patrones propuestos propician la creación de interfaces bien diseñadas capaces de ofrecer una mejor experiencia de uso y promueven un impacto positivo en la calidad de vida de los adultos mayores.

Alimentos balanceados para perros en Costa Rica

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Ruth Vargas

2001-01-01

Full Text Available Diagnóstico de la comercialización de alimentos balanceados para perros en Costa Rica. Para ampliar los reportes oficiales de la comercialización de alimentos para perros se creó una base de datos que incluyó cantidad, costo, empaque y formulación durante el período 1998 a agosto del 2000. De 1995 a 1998 y de 1996 a 1999 la producción nacional incrementó un 90,4% y la importación un 42,56% respectivamente, ocupando los alimentos nacionales un 72% del tonelaje y un 70% del valor en dólares americanos. No fue posible determinar cuál es el empaque más comercializado, pero si que los alimentos extrusados son los que ocupan el primer lugar y que los alimentos recomendados para cachorros y adultos son los que más se comercializan. La ausencia de datos en las declaraciones sugiere la necesidad de incrementar el control en las mismas en caso de requerirse un estudio de mercado, ya que contrariamente a esta situación el mayor número de garantías inscritas corresponden separadamente para las fases de cachorros y adultos. Merece también atención que las garantías indican mayores porcentajes de proteína cruda que los recomendados por la AAFCO y que al menos 36 fórmulas son recomendadas para estados sanitarios específicos (p.e. para perros con problemas de alergias, cálculos renales, pérdidas de pelo, etc. sin que oficialmente se encuentre registrada ninguna fórmula medicada

Quo vadis optica quantorum?

International Nuclear Information System (INIS)

Lewenstein, M.

2005-01-01

In my talk I will present the recent developments of quantum optics, and in particular physics of ultra-cold gases that occur at the interplay between quantum optics, atomic physics, quantum information, statistical mechanics, condensed matter physics and even high energy physics, and touch the same frontiers and challenges of modern physics. In particular I will discuss the possibility of studying and discovering new phenomena in physics of frustrated anti-ferromagnets, and about possibility of studying some aspects of abelian and non-abelian gauge field theories. (author)

Actitud Positiva: la base para la competitividad

Directory of Open Access Journals (Sweden)

Marco Anderson Espinoza

2008-04-01

Full Text Available Una estrategia empresarial enfocado en el mejoramiento continuo de la productividad para la competitividad, depende significativamente del manejo inteligente de tres variables visibles:Calidad, Entrega oportuna y Costo. No obstante, estas tres variables no suelen sersuficientes. La estrategia debe ser complementada con el manejo adecuado de variables intangibles como la Motivación y las Condiciones de Trabajo, con la aplicación de Tecnología Ambientalmente Amigable, en un marco de Actitud Positiva.A final de cuentas, la Actitud Positiva es fundamental para el recurso humano de cualquier organización, a fin de construir una plataforma sólida y estable que sirva de base para soportar un proceso sostenible de mejoramiento de la productividad, para aumentar la competitividad como estrategia empresarial exitosa.

Technological properties of kaolin from Para; Propriedades tecnologicas do caulin do Para

Energy Technology Data Exchange (ETDEWEB)

Hildebrando, E.; Martin-Cortes, G.R.; Esper, F.J.; Wiebeck, H.; Alves Junior, P.; Valenzuela-Diaz, F.R., E-mail: germac@usp.br [Universidade de Sao Paulo (PMT/EP/USP), Sao Paulo, SP (Brazil). Escola Politecnica. Departamento de Engenharia Metalurgica e de Materiais

2011-07-01

The NE of the State of Para, is notable for its large reserves of kaolin for paper. Kaolin is a mineral composed of hydrated aluminum silicates such as kaolinite and halloysite. The exploitation of these reserves to generate products with higher commercial value, especially in the industries of adsorbents and catalysts, is what motivates the continuous study of technological properties of the Amazon kaolin's. Thus, this paper presents a technological characterization of a sample of kaolin from Para State by X-ray diffraction, SEM - scanning electron microscopy and infrared spectroscopy. The results of preliminary tests indicate that the sample consists for the most part by kaolinite present in low concentrations of quartz and anatase. (author)

Desarrollo de manuales para la inspección y revisión de instalaciones petrolíferas para uso propio, para suministro a vehículos y transporte de mercancías peligrosas por carretera

OpenAIRE

Cardell Bilbao, Pablo Juan

2010-01-01

El objetivo de este proyecto es la realización de un manual que aúne y clarifique toda la documentación, la normativa y los procedimientos necesarios para cumplir las auditorías (revisiones e inspecciones) en las instalaciones existentes, los trámites para la apertura de nuevas instalaciones para suministro a vehículos (especialmente las Estaciones de Servicio (EESS), y para Usos Propios (UUPP) en Canarias, y el transporte de mercancías peligrosas por carretera (ADR), mostrando el procedimien...

Oxitocina: nuevas perspectivas para una droga antigua

OpenAIRE

Steven L. CLARK; Kathleen Rice SIMPSON; G. Eric KNOX; Thomas J. GARITE

2010-01-01

La oxitocina es el medicamento más comúnmente asociada con la prevención de complicaciones perinatales y recientemente fue incluida por el Instituto para la Administración Medicamentosa Segura (Institute for Safe Medication Practices - ISMP) en una pequeña lista de medicamentos “con alto grado de riesgos”, lo que puede “necesitar salvaguardas especiales para reducir el riesgo de error”. Las recomendaciones actuales para administrar este medicamento son vagas en relación a la indicación, tiemp...

INICIATIVAS PARA EL CAMBIO

Directory of Open Access Journals (Sweden)

César Alfonso Defrancisco-Larrañaga

2013-07-01

Full Text Available El planeta no atraviesa por su mejor momento en materia de conservación y renovación de sus recursos naturales. Claramente en los últimos tiempos los recursos hídricos han venido haciéndose cada vez menores; el hombre ha notado con preocupación y vivido en carne propia este fenómeno, que de no ser tratado a tiempo, podría atentar contra la existencia misma. Esto lo ha alentado a buscar métodos que sirvan como solución para contrarrestar éste mal y crear conciencia entre sus iguales, para así poder garantizar su permanencia y la de su entorno durante muchas generaciones. En países desarrollados del continente europeo y en algunos lugares de América del norte, el uso de sistemas de reutilización y tratamiento de aguas grises y aprovechamiento de las aguas lluvias se ha convertido en una constante y nos están dando las pautas para poder seguir el ejemplo y así de esta manera poder aportar a la causa ambiental y a su vez crear un ahorro en nuestros gastos mensuales de consumo.

AUTONOMÍA PARA APRENDER Y AUTONOMÍA PARA VIVIR

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Miryam Narváez Rivero

2005-04-01

Full Text Available RESUMEN: El sistema educativo tiene el reto actual de formar individuos autónomos. Esta autonomía no implica solo el que las personas tengan los recursos necesarios para aprender a aprender, sino que puedan convivir armónicamente en comunidad, escuchándose a sí mismos para tomar sus propias decisiones, sin estar determinados por deseos instintivos ni modelos coercitivos impuestos por la sociedad. Así, para desarrollar autonomía, le corresponde a la educación trabajar tres elementos: la voluntad, la libertad y la autoestima. De esta forma, una educación promotora de una verdadera autonomía deberá tener un carácter dialógico, haciendo hincapié en la integración solidaria de la persona con su comunidad. Nos referimos a una persona que no ha renunciado a sus metas ni aspiraciones, sino que sabe cómo guiarlas hacia un proyecto común de bienestar general. ABSTRACT: The educative system’s latest challenge is to educate independent individuals. This autonomy does not just imply that people have the necessary resources to learn to learn, but the ones they need to live together in harmony with others, listening to themselves to make their own decisions without being determined by instinctive desires nor coercive models imposed by the society. It is so, that to develop autonomy, the educational system must work with these three elements: will, freedom and self-esteem. Also, an educational system which promotes a true autonomy must have a dialogic character, insisting on the individual solidarity integration with the community; with this, we mean a person who has not resigned to his goals or aspirations, but that knows how to guide them towards a common project for the community well-being.

Significado de la calidad de vida del adulto mayor para sí mismo y para su familia

Directory of Open Access Journals (Sweden)

Martha Vera

2007-09-01

Full Text Available Objetivo: Comprender el significado que la expresión lingüística ‘calidad de vida del adulto mayor’ tiene para el adulto mayor y para su familia. Diseño: Estudio cualitativo, descriptivo, interpretativo - comprensivo, fundamentado en la Teoría de las Representaciones Sociales. Lugar: Asentamiento Humano Laura Caller, en Los Olivos. Participantes: Adultos mayores y sus respectivos grupos familiares. Métodos: Las técnicas e instrumentos utilizados fueron la observación, entrevista a profundidad y grupos focales; guía de observación y dos cuestionarios con ocho preguntas norteadoras, previamente validados. Principales medidas de resultados: Significado de calidad de vida para el adulto mayor y para la familia. Resultados: Participaron dieciséis adultos mayores con sus respectivos grupos familiares, entre 2004 y 2006. Los discursos a través de la ‘objetivación’ y el ‘anclaje’ y los análisis general, ideográfico-axial y nomotético-selectivo permitieron la emergencia de cuatro representaciones sociales: a conocimiento sobre la vejez y ser un adulto mayor; b elementos principales y secundarios del significado de calidad de vida del adulto mayor; c valores y actitudes del adulto mayor y familia sobre la calidad de vida; y, d necesidades del adulto mayor como ser social y la calidad de vida. Conclusiones: Para el adulto mayor, calidad de vida significa tener paz y tranquilidad, ser cuidado y protegido por la familia con dignidad, amor y respeto, y tener satisfechas como ser social sus necesidades de libre expresión, decisión, comunicación e información. Para la familia, que el adulto mayor satisfaga sus necesidades de alimentación, vestido, higiene y vivienda.

Relapsing-Remitting MS (RRMS)

Medline Plus

Full Text Available ... CIS) Newly Diagnosed Diagnosing Tools Other Conditions to Rule Out For Clinicians Treating MS Comprehensive Care Find ... CSF) Evoked Potentials (EP) d Other Conditions to Rule Out Lyme Disease Lupus Neuromyelitis Optica Acute Disseminated ...

Demyeliniserende sygdom hos børn med akutte neurologiske symptomer

DEFF Research Database (Denmark)

Olofsson, Isa Amalie; Skov, Liselotte; Miranda, Maria Jose

2015-01-01

Demyelinating diseases in children is a broad group of illnesses, which affect the central nervous system. Demyelinating diseases can be monophasic or chronic and comprise acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, multiple sclerosis and neuromyelitis optica...

Frequency and prognostic impact of antibodies to aquaporin-4 in patients with optic neuritis

DEFF Research Database (Denmark)

Jarius, Sven; Frederiksen, Jette Lautrup Battistini; Waters, Patrick

2010-01-01

Antibodies to aquaporin-4 (AQP4-Ab) are found in 60-80% of patients with neuromyelitis optica (NMO), a severely disabling inflammatory CNS disorder of putative autoimmune aetiology, which predominantly affects the optic nerves and spinal cord....

ÉTICA PARA O DESENVOLVIMENTO SUSTENTÁVEL – UMA RESPONSABILIDADE PARA COM AS GERAÇÕES FUTURAS

Directory of Open Access Journals (Sweden)

Patrícia Melo Silva

2011-02-01

Full Text Available O trabalho apresenta uma discussão sobre como a ética pode contribuir para a ciência se desenvolver de maneira sustentável com o intuito de garantir a biodiversidade em uma sociedade que cresce de acordo com a moral utilitarista. Os principais aspectos analisados foram questões da ética como proposta oposta à moral utilitarista, além de aspectos relacionados à economia global, ao sistema capitalista e à interdisciplinaridade. O principal objetivo do trabalho é analisar como a ética pode contribuir para o desenvolvimento sustentável e para o respeito à biodiversidade. A metodologia utilizada foi a de análise bibliográfica.

Nueva Sede para la Academia

Directory of Open Access Journals (Sweden)

Mario Camacho Pinto

1994-06-01

Full Text Available

Una nueva sede adecuadamente dotada siempre estuvo en la mente de nuestros expresidentes Académicos César Augusto Pantoja, Hernando Groot, Pablo Gómez Martínez, Jorge Cavelier Gaviria y Efraim Otero-Ruiz, con quienes me tocó en suerte trabajar 12 años consecutivos, cerca de ellos a través de mi cargo de Coordinardor de la Comisión Permanente de Biblioteca y Publicaciones y de la Revista MEDICINA que se ha venido publicando sin interrupciones dentro de un compañerismo ejemplar; además de otras distinciones operativas que me inducen a expresar gratitud.

Ahora, además, como Secretario de la Coporación por elección unánime de la Asamblea hace dos años, he sido testigo ático de una intensa, inteligente e inagotable actividad que nuestro Presidente Académico Juan Jacobo Muñoz Delgado quien siempre con el visto bueno de la Junta Directiva, mediante un acelerado ritmo de trabajo se impuso la tarea de convertir en realidad inmediata tan anhelada y necesaria idea.

En efecto, la nueva sede fue inaugurada oficialmente el día 2 de septiembre de 1993.

Este Editorial es la constancia escrita de tal febril actividad que para proporcionar evidencia a los lectores de “MEDICINA” resumo en breves incisos que muestran el tránsito a un estado con superávit de excepción para esta clase de instituciones:

1. Terminación de la obra ya iniciada de la remodelación arquitectónica, tanto exterior de seguridad y aspecto como interior con suficientes y modernas instalaciones.

2. Cerramiento total con altas y elegantes rejas metálicas y doble portería por la calle 69 y la carrera 7a.

3. Aparcadero lujosamente adoquinado, amplio y seguro para 30 automóviles.

4. Decoración interior estéticamente consultada, bien lograda en cortinas y clásicas lámparas de cristal obsequiadas por conocidos académicos. Un par de originales urnas precolombianas en el hall de entrada.

5. Zona social doble, amplia

Hacia un nuevo diseño para el aprendizaje: escenarios educativos para la Web 2.0

Directory of Open Access Journals (Sweden)

Alejandro Canales Cruz

2014-10-01

Full Text Available En este artículo se retoma la caracterización de pedagogía en la virtualidad, la cual se fundamenta en la apropiación tecnológica en función de la educación y voluntad de los participantes. Se propone la convergencia del diseño tecnopedagógico, unido a la apropiación creativa y a los usos pedagógicos efectivos para maximizar el proceso educativo. Se plantea el empleo de la especificación IMS Learning Design (LD para la construcción de escenarios educativos como un proceso sistémico con actividades interrelacionadas que permiten crear ambientes que en verdad faciliten, de forma mediada, el proceso educativo. Finalmente, se presenta una herramienta para la autoría de escenarios educativos.

Modelo de negocio para bebidas funcionales Ceiba

OpenAIRE

Gil Giraldo, Jhonatan; Restrepo Mesa, Juan Eduardo

2017-01-01

El presente documento, modelo de negocio para bebidas naturales Ceiba, es el trabajo de grado realizado por los autores con el fin de optar al título de magíster en Administración (MBA) de la Universidad EAFIT -- El cual esta soportado en los diferentes conceptos y metodologías de modelo de negocio y planes de negocio -- Por tratarse de un prototipo que busca definir los elementos estratégicos necesarios para su desarrollo, arrojó como resultado que la metodología más adecuada para ser aplica...

Middleware Cerberus usando RFID para rastreabilidade bovina

OpenAIRE

Silva, Márcio Roberto

2009-01-01

A gerência na zootecnia de precisão é cada vez mais necessária para garantia de lucros e a conquista de novos mercados os quais exigem qualidade e certificação. Nesta dissertação é abordado um sistema de rastreabilidade para o controle na bovinocultura utilizando chips RFID. Sendo que a identificação segura dos animais é a base para a rastreabilidade bovina e bubalina. Em vista as exigências do mercado consumidor o governo brasileiro instituiu o Sistema Brasileiro de Identificação e Certifica...

Test para la profilaxis de la prematuridad

OpenAIRE

Cruz Laguna, Gladys María

2008-01-01

La prematuridad constituye un problema mundial de salud, la comunidad científica busca predictores para disminuir su incidencia. La investigación persiguió obtener una escala de puntuación que modele las características cervicales y permita diagnosticar la condición de riesgo de las gestantes para extender el período de gestación y lograr nacimientos al término. Se realizó un cuasiexperimento en gestantes con factores de riesgo para la prematuridad del municipio Holguín. La muestra fue confor...

para mujeres y niños vulnerables

International Development Research Centre (IDRC) Digital Library (Canada)

... a nivel global . 73. Generar confianza y crear espacio para el compromiso . 75 .... para construir sistemas de salud sólidos y han colaborado inmensamente con ..... instituciones sanitarias se ve obligado a recolectar información y a registrar ...

Modelo para la Gestión de la Innovación por Proyectos para Empresas que aplican el Perfeccionamiento Empresarial en Cuba

OpenAIRE

Antonio M. Navarro López

2008-01-01

Las Empresas que aplican el Perfeccionamiento Empresarial, no cuentan con un Modelo para la Gestión de la Innovación para el desarrollo Empresarial. Por lo que se propone éste Modelo para las mismas y generalizándose de esta forma el conocimiento de Gestión de la Innovación de la Empresa de Proyectos de Arquitectura e Ingeniería de Matanzas, Cuba, lo que permite el desarrollo Empresarial y la Implantación del Sub Sistema de Innovación para estas Empresas en Perfeccionamiento Empresarial. Se p...

Retos para la agricultura en Costa Rica

Directory of Open Access Journals (Sweden)

Oscar Arias M.

2005-01-01

Full Text Available Retos para la agricultura en Costa Rica es un análisis crítico del desarrollo agrícola de Costa Rica de los últimos 25 años. La diversificación agrícola que promovió Costa Rica en la década de los ochenta, permitió ampliar la oferta exportable y reducir la vulnerabilidad de la dependencia económica de productos tradicionales, como café y banano. Los retos de la economía global y el establecimiento de tratados comerciales con muchos países, hacen necesario que el país promueva en el sector agrícola la exportación con mayor valor agregado, para lo cual es necesario una modernización y reconversión productiva, ya que el modelo actual está agotado. Debemos mejorar sustancialmente los bienes y servicios que ofrecemos; para este propósito, algunos aspectos como la imagen de marca del país con tradición democrática, respeto a los derechos laborales, así como las buenas prácticas de manejo ambiental, deben publicitarse. Como una herramienta clave para la incorporación de mayor valor agregado a nuestra producción agrícola, debemos promover la inversión en investigación y desarrollo, que históricamente ha sido escasa (0,4% del PIB. En vista de que el Estado Costarricense ha demostrado una incapacidad crónica para impulsar la ciencia y la tecnología como una herramienta para nuestro desarrollo, se propone un estímulo a la inversión privada y el fortalecimiento de una alianza con el Estado y las universidades. Se analiza la conveniencia del fortalecimiento de la autosuficiencia alimentaria y de que las empresas pequeñas y medianas jueguen un papel más activo en la agro-exportación. Respecto a los tratados comerciales que se han venido negociando, se considera la conveniencia para el país, ya que son instrumentos para integrar nuestro quehacer económico a nivel mundial, y nuestro deber es el de luchar para que el sector agropecuario tenga oportunidad de subsistir competitivamente según esas nuevas reglas y

Matemática para la familia

OpenAIRE

Cittadino, José

1994-01-01

Para muchos profesores de matemáticas ha sido de primordial interés encontrar estrategias didácticas apropiadas para orientar el aprendizaje de las matemáticas escolares de los niños (as) y jóvenes de manera que las clases se conviertan en encuentros agradables y la relación matemática-aprendizaje- profesor sea la mejor.

Educación para emprender, una opción para el mundo en desarrollo

Directory of Open Access Journals (Sweden)

Luis Gerardo Rodríguez

2015-05-01

Full Text Available RESUMEN La búsqueda de las formas educativas para corresponder con el momento histórico que vive el mundo de hoy, ha sacudido de reformas, adecuaciones y reconversiones, la geografía de nuestros países aspirantes al desarrollo. En Latinoamérica la reforma educativa es una constante y este fenómeno implica, que como educadores estemos obligados a asumir en mayor o menor proporción, el impacto de tal explosión reformista. Construir desde el quehacer cotidiano, formas de educación para orientar de manera coherente la formación de hombres para dirigir organizaciones en el mundo  de hoy, implica general un proceso educativo acorde con la reforma de la educación superior en el macro de la apertura y globalización  de la economía, y  la cual problemática social en la que se vislumbran la inestabilidad del futuro, plasmada en la crisis de la certeza y el reto de la complejidad

Physical properties of PbO-ZnO-P2O5 glasses II. refractive index and optical properties

Czech Academy of Sciences Publication Activity Database

Tichá, H.; Schwarz, J.; Tichý, Ladislav; Mertens, R.

2004-01-01

Roč. 6, č. 3 (2004), s. 747-753 ISSN 1454-4164 R&D Projects: GA AV ČR KSK2050602 Keywords : optica l properties * refractive index Subject RIV: CA - Inorganic Chemistry Impact factor: 1.003, year: 2004

Demyeliniserende sygdom hos børn med akutte neurologiske symptomer

DEFF Research Database (Denmark)

Olofsson, Isa Amalie; Skov, Liselotte; Miranda, Maria Jose

2015-01-01

Demyelinating diseases in children is a broad group of illnesses, which affect the central nervous system. Demyelinating diseases can be monophasic or chronic and comprise acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, multiple sclerosis and neuromyelitis optica. Demye...

Um Termômetro para as Macro-Prudenciais

Directory of Open Access Journals (Sweden)

Fabio Kanczuk

2013-12-01

Full Text Available Desenvolvemos um modelo com fricções no crédito tanto para firmas como famílias. Crédito às firmas é tratado como nos modelos de acelerador financeiro (e.g. Bernanke e Gilchrist (1999. Os juros sobre os recursos emprestados às famílias dependem de seu endividamento, como em Curdia e Woodford (2010. O modelo é estimado para o Brasil, utilizado para estudar os episódios de desaceleração, e para a extração dos prêmios de financiamento (destilados a partir de dados não financeiros, os quais são comparados com informações sobre crédito às pessoas físicas e jurídicas. Dessa forma, o obtém-se um termômetro para mensurar como medidas prudenciais sobre o crédito afetam atividade e inflação.

Metodologia de la Administración Federal de Aviación para el Diseño de estructuras de Pavimento flexible para aeropuertos

Directory of Open Access Journals (Sweden)

Julián Rodrigo Quintero González

2013-08-01

Full Text Available Se representan los aspectos técnicos fundamentales y principalesconsideraciones de la metodologia propuesta por la Administracion Federal de Aviación FAA para el diseño de estructuras de pavimento para aeropuertos. Se exponen los aspectos sobre las variables consideradas en el diseño (estructurales tránsito, factores ambientales y factores intrinsecos, se establecen las caracteristicas de las variables consideradas por el metodo (subrasante y materiales para la estructura, geometría del tren de aterrizaje, número de decolajes equivalentes y peso bruto maximo del decolaje y se explica el procedimiento utilizado para el tratamiento de la información requerida en diseño estructural de pavimentos flexibles para aeropuertos.

Construyendo campos para el aprendizaje creativo. Método del juego

Directory of Open Access Journals (Sweden)

Paco Cerezo Sánchez

2008-01-01

Full Text Available Este documento presenta el marco teórico, el camino recorrido y los resultados de una experiencia piloto para la formación de formadores y de trabajadores del sector de transportes, experiencia hecha para desarrollar competencias interpersonales.Avanzamos desde el constructivismo y proponemos un método de aprendizaje en grupo, denominado Método del juego porque se basa en juegos sociales experienciados por los participantes (alumnos durante el taller.Aquí, hablo de los contenidos formativos - dirigidos especialmente a la formación de formadores- y también de la forma de aprender y de la estructura oculta en los procesos de aprendizaje. Describo un marco teórico que permite aplicar métodos de aprendizaje grupal y creativo para la formación continua, y aporto herramientas para que los formadores las adapten y construyan sus propios juegos didácticos. El marco creativo de aprendizaje y este método del juego nacen de experiencias entrenando a actores de teatro de calle (desde 1983, formadores y directivos (desde 1991 y aplicaciones para eLearning (1996-2007 en diferentes sectores y países europeos; experiencia consolidada con el equipo de www.formador.org (2005-2008. Al principio usaba juegos extraídos del circo, ahora son juegos sociales o de mesa.El marco creativo y el método del juego aquí propuestos sirven para desarrollar: el potencial humano, la autonomía personal, la creatividad y la innovación, las competencias interpersonales, de trabajo en equipo, para comprender y solucionar conflictos; la habilidad para comprender y solucionar problemas complejos; la habilidad para aprender a aprender.Así, esta propuesta para aprender jugando en grupo recoge las experiencias acumuladas durante 20 años formando formadores y complementa a otras investigaciones y productos1, muchos de ellos específicos para la formación de formadores. Para Aprender jugando en grupo hemos creado un paquete integrado por el marco teórico creador de

Confianza y capital social: evidencia para México

Directory of Open Access Journals (Sweden)

Rubén Martínez-Cárdenas

2015-01-01

Full Text Available Desde la perspectiva económica la confianza entre los miembros de una sociedad está asociada al funcionamiento eficiente de los mercados, al reforzamiento de contratos y, en general, a la capacidad para generar desarrollo y bienestar. En este artículo exploramos empíricamente los determinantes de la confianza en México, en particular, aislamos la relación entre dos medidas de capital social, una para el individuo y otra para su entorno. Nuestros resultados muestran que la confianza aumenta con estas medidas de capital social y con ausencia de se - gregación es mayor en comunidades pequeñas, a mayor edad y para habitantes del centro-occidente, entre otras.

ESPIRITUALIDADE PARA OUTRO MUNDO POSSÍVEL

Directory of Open Access Journals (Sweden)

Agenor Brighent

2007-01-01

Full Text Available Teve lugar, em Nairobi, o II Fórum Mundial de Teologia e Libertação, entre os dias 16e 19de janeiro de 2007.O primeiro havia sido realizado em Porto Alegre, em janeiro de 2005, cujos resultados estão publicados em L.C. SUSIN (org., Teologia para outro mundo possível, São Paulo: Paulinas, 2006 (485 pp.. O segundo teve como tema - "espiritualidade para outro mundo possível". Fizeram-se presentes 300participantes, oriundos dos cinco Continentes, em sua maioria da África, especialmente de Nairobi, seguida da Europa, América, Ásia e Oceania, respectivamente. Para além da reflexão e dos conteúdos, o simples encontro e cruzamento de buscas e realizações em esfera planetária, já justifica por si só o evento. Em tempos de crise de paradigmas e dos metarrelatos, a experiência se erige como lugar privilegiado para repensar a razão e as razões do existir, do mundo e da fé. Os trabalhos tiveram lugar no Carmelite Center, nos arredores da contrastante capital do Quênia.

Tithonia diversifolia: especie para ramoneo en sistemas silvopastoriles y métodos para estimar su consumo.

Directory of Open Access Journals (Sweden)

Estefanía Mejía-Díaz

2016-12-01

Full Text Available El objetivo del presente trabajo fue recopilar avances investigativos sobre aspectos agronómicos, nutricionales y productivos de T. diversifolia Hemsl. A Gray en sistemas silvopastorils (SSP como estrategia de producción ganadera y conocer metodologías para estimar el consumo de forraje de vacas bajo SSP. La introducción de sistemas silvopastoriles proporciona una vegetación estrati cada, además de generar diversos bienes, servicios ambientales y bienestar animal, constituyéndose, desde el punto de vista productivo, ecológico, económico y social, en una de las modalidades más prometedoras de los sistemas agroforestales. En la presente revisión se realizó una descripción de la arbustiva T. diversifolia Hemsl. A Gray, como especie estratégica para ser introducida en los sistemas silvopastoriles sean estos de baja o alta densidad, la cual por su calidad composicional ha sido reportada como una arbustiva con potencial para ser utilizada en la nutrición de rumiantes, teniendo un posible efecto positivo sobre índices productivos y económicos. Se discutieron tres técnicas utilizadas para estimar el consumo de forraje por bovinos en pastoreo, las cuales pueden ser aplicadas tanto en sistemas tradicionales como en sistemas silvopastoriles.

ParaChoice Model.

Energy Technology Data Exchange (ETDEWEB)

Heimer, Brandon Walter [Sandia National Lab. (SNL-CA), Livermore, CA (United States); Levinson, Rebecca Sobel [Sandia National Lab. (SNL-CA), Livermore, CA (United States); West, Todd H. [Sandia National Lab. (SNL-CA), Livermore, CA (United States)

2017-12-01

Analysis with the ParaChoice model addresses three barriers from the VTO Multi-Year Program Plan: availability of alternative fuels and electric charging station infrastructure, availability of AFVs and electric drive vehicles, and consumer reluctance to purchase new technologies. In this fiscal year, we first examined the relationship between the availability of alternative fuels and station infrastructure. Specifically, we studied how electric vehicle charging infrastructure affects the ability of EVs to compete with vehicles that rely on mature, conventional petroleum-based fuels. Second, we studied how the availability of less costly AFVs promotes their representation in the LDV fleet. Third, we used ParaChoice trade space analyses to help inform which consumers are reluctant to purchase new technologies. Last, we began analysis of impacts of alternative energy technologies on Class 8 trucks to isolate those that may most efficaciously advance HDV efficiency and petroleum use reduction goals.

Quantitative study of the cervical spinal cord damage in patients with multiple sclerosis and neuromyelitis optica using diffusion tensor imaging

International Nuclear Information System (INIS)

Hou Huanxin; Li Yongmei; Lu Fajin; Luo Tianyou; Ouyang Yu; Zeng Chun; Zhang Zhiwei

2012-01-01

Objective: To investigate the changes of the cervical spinal cord in patients with relapsing-remitting multiple sclerosis (RRMS) and relapsing neuromyelitis optica (RNMO) using diffusion tensor imaging (DTI) and to analyze its correlations with clinical disability scores. Methods: Thirty patients with MS (MS group),28 patients with NMO (NMO group) and 20 healthy volunteers were imaged using DTI on a 3.0 Tesla scanner. DTI indices of cervical spinal cord from all participants were measured, including mean diffusivity (MD) and fractional anisotropy (FA), and the correlations between the DTI metrics and the expanded disability status scale (EDSS) scores were assessed. One-way ANOVA, Dunnett-t test and Spearman correlation analysis were used for statistics. Results: (1) The values of MD among three groups were different at C3 level for left lateral and dorsal columns, C4 level for the central gray substance and dorsal columns, and C5-C6 level for all structures. There were significant differences among them (F=4.006-36.814, P<0.05). The values of FA were significantly different at all levels (F=5.561-98.128, P<0.05). (2) Compared with the control group, the values of MD were increased and FA were decreased for both MS and NMO groups, there were significant differences among them (t=-0.320-3.138, P<0.05). In MS and NMO groups, there were no significant differences of MD (t=-1.183-0.069, P>0.05), while the FA at C4-C6 levels (including the central gray substance, dorsal columns,right lateral columns and left lateral columns) for NMO group were 0.57 ± 0.09, 0.56 ± 0.11, 0.54 ±0.10, 0.57±0.09, 0.55 ±0.11, 0.52 ±0.13, 0.55 ±0.11, 0.54 ±0.13, 0.54±0.10, 0.54±0.11, 0.53 ±0.13, 0.52 ±0.11; and for MS group were 0.67 ±0.10, 0.68 ±0.10, 0.68 ±0.10, 0.70 ±0.12, 0.68 ±0.11, 0.69±0.10, 0.68 ±0.11, 0.69 ±0.12, 0.67 ±0.14, 0.68 ±0.15, 0.69 ±0.14, 0.69 ±0.16, and there were significant differences between two groups (t=-0.011-0.169, P<0.05). (3) Univariate

Modificación del caldo extracto de levadura manitol para la producción a mediana escala de inoculantes para leguminosas

Directory of Open Access Journals (Sweden)

Ernesto Ormeño-Orrillo

2014-06-01

Full Text Available El caldo extracto de levadura manitol (LM, un medio ampliamente utilizado para el cultivo de rizobios, fue modificado para reducir su costo y utilizarlo en la producción a mediana escala de inoculantes para leguminosas. Los dos ingredientes más costosos, el extracto de levadura y el manitol, fueron reducidos o reemplazados con substratos más económicos. Se pudo reducir la concentración de extracto de levadura a 0,05 g/L sin afectar el crecimiento cuando se agregó 1,1 g/L de ácido glutámico o glutamato de sodio grado alimento. El manitol pudo ser substituido por 12,5 g/L de glicerina grado farmacéutico para las cepas de Bradyrhizobium o por 10 g/L de azúcar grado alimento para las cepas de Rhizobium. No se alteraron las propiedades simbióticas de las cepas cultivados en los medios modificados.

Cognitive performance of neuromyelitis optica patients: comparison with multiple sclerosis Desempenho cognitivo de pacientes com neuromielite óptica: comparação com esclerose múltipla

Directory of Open Access Journals (Sweden)

Sandra Vanotti

2013-06-01

Full Text Available The aim of the present research was to investigate cognitive pattern of patients with neuromyelitis optica (NMO and to compare it with multiple sclerosis (MS patients' performance. Methods: Fourteen NMO, 14 relapsing remitting multiple sclerosis (RRMS, and 14 healthy control patients participated in the investigation. Neuropsychological functions were evaluated with the Brief Repeatable Neuropsychological Battery for MS; Symbol Digit Modalities Test; Digit Span; and Semantic Fluency. Results: Fifty-seven percent of NMO patients and 42.85% of the MS ones had abnormal performance in at least two cognitive tests. The NMO Group showed abnormal performance in verbal fluency, verbal and visual memories, with greater attention deficits. NMO patients outperformed healthy control in the paced auditory serial addition test (PASAT. However, no difference was found between NMO and RRMS patients. Conclusions: The NMO Group showed more dysfunction in attention and verbal fluencies than in verbal and visual memories. When compared with the MS patients, a similar dysfunction pattern was found. O objetivo da presente pesquisa foi investigar o padrão cognitivo de pacientes com neuromielite óptica (NMO e compará-lo com o desempenho de pacientes com esclerose múltipla (EM. Métodos: Quatorze pacientes com NMO, 14 com esclerose múltipla recorrente remitente (EMRR e 14 participantes do Controle saudáveis participaram da presente investigação. As funções neuropsicológicas foram avaliadas com a Bateria Breve de Testes Neuropsicológicos de Rao, Teste Símbolo Digit e a Fluência Semântica. Resultados: Cinquenta e sete por cento dos pacientes com NMO e 42,85% daqueles com EM apresentaram desempenho anormal em pelo menos dois testes cognitivos. O Grupo NMO apresentarou desempenho anormal na fluência verbal e nas memórias visual e verbal, com maiores déficits de atenção. Pacientes com NMO superaram os controles saudáveis em PASAT. No entanto, não foi

Plan de negocio para una empresa que alquila bombas sumergibles para el sector minero

OpenAIRE

Pérez León Corzo, Stepahnie Pamela

2016-01-01

El presente trabajo de investigación evalúa la viabilidad operativa y financiera para operar una pequeña empresa dedicada al alquiler de bombas sumergibles para la actividad de desagüe de medianas y pequeñas empresas del sector minero. Estructuralmente, la empresa estará constituida por la alta dirección conformada por tres socios quienes aportarán el 30% de la inversión inicial, el 70% restante se obtendrá mediante un financiamiento a mediano plazo con un banco local. La línea media estará c...

African Journal of Neurological Sciences 2012 - Vol. 31, No 2 http ...

African Journals Online (AJOL)

AJNS WEBMASTERS

évolution, sans remettre en cause le diagnostic de NMO [7]. ... alors que 65 % des patients à forme monophasique marchaient sans aide [16] (Encadré VI). .... of different techniques to detect anti-aquaporin 4 antibodies in neuromyelitis optica patients.

Experimental study of para- and ortho-H3+ recombination

International Nuclear Information System (INIS)

Plasil, R; Varju, J; Hejduk, M; Dohnal, P; KotrIk, T; Glosik, J

2011-01-01

Recombination of H 3 + with electrons is a key process for many plasmatic environments. Recent experiments on storage ring devices used ion sources producing H 3 + with enhanced populations of H 3 + ions in the para nuclear spin configuration to shed light on the theoretically predicted faster recombination of para states. Although increased recombination rates were observed, no in situ characterization of recombining ions was performed. We present a state selective recombination study of para- and ortho-H 3 + ions with electrons at 77 K in afterglow plasma in a He/Ar/H 2 gas-mixture. Both spin configurations of H 3 + have been observed in situ with a near infrared cavity ring down spectrometer (NIR-CRDS) using the two lowest energy levels of H 3 + . Using hydrogen with an enhanced population of H 2 molecules in para states allowed us to influence the [para-H 3 + ]/[ortho-H 3 + ] ratio in the discharge and in the afterglow. We observed an increase in the measured effective recombination rate coefficients with the increase of the fraction of para-H 3 + . Measurements with different fractions of para-H 3 + at otherwise identical conditions allowed us to determine the binary recombination rate coefficients for pure para-H 3 + p α bin (77 K) = (2.0±0.4)x10 -7 cm 3 s -1 and pure ortho-H 3 + o α bin (77 K) = (4±3)x10 -8 cm 3 s -1 .

Experimental study of para- and ortho-H3+ recombination

Science.gov (United States)

Plašil, R.; Varju, J.; Hejduk, M.; Dohnal, P.; Kotrík, T.; Glosík, J.

2011-07-01

Recombination of H3+ with electrons is a key process for many plasmatic environments. Recent experiments on storage ring devices used ion sources producing H3+ with enhanced populations of H3+ ions in the para nuclear spin configuration to shed light on the theoretically predicted faster recombination of para states. Although increased recombination rates were observed, no in situ characterization of recombining ions was performed. We present a state selective recombination study of para- and ortho-H3+ ions with electrons at 77 K in afterglow plasma in a He/Ar/H2 gas-mixture. Both spin configurations of H3+ have been observed in situ with a near infrared cavity ring down spectrometer (NIR-CRDS) using the two lowest energy levels of H3+. Using hydrogen with an enhanced population of H2 molecules in para states allowed us to influence the [para-H3+]/[ortho-H3+] ratio in the discharge and in the afterglow. We observed an increase in the measured effective recombination rate coefficients with the increase of the fraction of para-H3+. Measurements with different fractions of para-H3+ at otherwise identical conditions allowed us to determine the binary recombination rate coefficients for pure para-H3+ pαbin(77 K) = (2.0±0.4)×10-7 cm3s-1 and pure ortho-H3+ oαbin(77 K) = (4±3)×10-8 cm3s-1.

Exhibidor de cinturones para Montagne

OpenAIRE

Galletti, Julia

2017-01-01

En el exhibidor de cinturones diseñado para la marca de ropa deportiva Montagne se produjo un trabajo estético-formal en relación a la simbología característica de la marca. Su principal característica es ser un exhibidor 360°, es decir que desde cualquier punto de vista se puede obtener un buen panorama del producto expuesto. Está confeccionado en materiales livianos para facilitar su movilidad dentro del local estipulado (exhibición en vidriera o mostrador). Asimismo, es totalmente desarmab...

Educación para un nuevo humanismo

Directory of Open Access Journals (Sweden)

GUILLERMO HOYOS-VÁSQUEZ

2009-01-01

Full Text Available Se parte del tema de la responsabilidad social universitaria, evaluada muchas veces con base en indicadores, especialmente de pertinencia. Se advierten los peligros que esto conlleva para la educación en general, si no se toma como punto de partida el sentido mismo de universidad y de educación, es decir, si no se parte de la identidad propia de las instituciones educativas. Se sugiere, por tanto, volver a retomar la discusión en torno a la formación humanista en los procesos educativos, para lo cual se analizan muy brevemente la Carta sobre el humanismo, de Martin Heidegger; las Normas para el parque humano, de Peter Sloterdijk; la Universidad sin condición, de Jacques Derrida y El cultivo de la humanidad, de Martha Nussbaum. Se cierra el ensayo con la propuesta de Jürgen Habermas de una teoría comunicativa de la educación para la ciudadanía.

Actividad Física: Pasaporte Para La Salud

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Sandra M.M. MATSUDO

2012-05-01

Full Text Available Las nuevas recomendaciones de actividad física para la salud, que cuentan con la aprobación de la oms, el cdc, el Colegio Americano de Medicina Deportiva (acsm y la American Heart Association, enfatizan que al menos 30 minutos diarios, por lo menos cinco días de la semana (de preferencia todos los días de la semana, de forma continua o en sesiones acumuladas de 10 o 15 minutos, de una actividad de intensidad moderada pueden ser suficientes para traer beneficios para la salud y la prevención, tratamiento, control y rehabilitación de las enfermedades crónicas no transmisibles. Por lo tanto, los especialistas en ciencias del deporte y las autoridades sanitarias recomiendan que toda persona incluya en su vida cotidiana, ya sea en el hogar, en el trabajo o en la comunidad, una actividad física regular para recuperar o mantener la salud.

Proposta de um instrumento para avaliar eventos adversos em odontologia

Directory of Open Access Journals (Sweden)

Claudia Dolores Trierweiler Sampaio de Oliveira Corrêa

2017-11-01

Full Text Available Resumo: O objetivo deste estudo foi propor um conjunto de rastreadores para a pesquisa de eventos adversos em odontologia ambulatorial no Brasil. O instrumento foi elaborado em duas etapas: (i para construir um conjunto preliminar de rastreadores foi realizada uma revisão da literatura, a fim de identificar a composição das ferramentas de rastreamento utilizadas nas demais áreas da saúde e os principais eventos adversos encontrados em odontologia; (ii para validar os rastreadores preliminarmente construídos foi organizado um painel de especialistas empregando o método Delphi modificado. Foram elaborados quatorze rastreadores para compor um instrumento com critérios explícitos para identificar potenciais eventos adversos no cuidado odontológico, essenciais para os estudos de revisão retrospectiva de prontuários. Pesquisas relacionadas à segurança do paciente em odontologia ainda são bastante incipientes em relação às demais áreas da saúde. Este trabalho pretendeu contribuir para a investigação nesse campo. O aporte da literatura e a ajuda da expertise do painel de especialistas permitiram a elaboração de um conjunto de rastreadores para a detecção de eventos adversos odontológicos, no entanto, são necessários estudos adicionais para testar a validade do instrumento.

Sistemática para avaliação da conformidade: uma proposta para uso de cão-guia

OpenAIRE

Brito, Joana D'Arc de

2009-01-01

O objetivo do presente estudo é identificar e propor quais os principais requisitos para a Avaliação dos Centros de Treinamento, de Treinadores e dos Instrutores Autônomos de cães-guia, de forma a atender o que prescreve a Lei n.º 11.126, de 27 de junho de 2005 no concernente às necessidades de adaptação dos segmentos envolvidos. Esta pesquisa foi estruturada em 3 (três) etapas. No primeiro momento utilizou-se de fontes bibliográficas para o levantamento de obras de referência para o desenvol...

Creación de materiales didácticos orales para la clase de lengua para la traducción

OpenAIRE

González Royo, Carmen; Chiapello, Stefania; Martín Sánchez, Teresa; Pascual Escagedo, Consuelo; Puigdevall Balafuy, Núria; Mura, G. Angela; Regagliolo, Alberto

2016-01-01

“Creación de materiales didácticos orales para la clase de lengua para la traducción” (3546) es una red que, desde una perspectiva de investigación-acción, investiga la implementación de materiales docentes para los currículos de lengua y traducción en las asignaturas de E/LE e I/LE en los grados de Lenguas Extranjeras y Traducción e Interpretación de la Universidad de Alicante, Università Suor Orsola Benincasa de Nápoles y Università degli Studi di Salerno. A partir de los materiales produci...

TV Móvel: onde estamos e para onde vamos

Directory of Open Access Journals (Sweden)

Clara-Luz Álvarez

2010-05-01

Full Text Available O artigo apresenta (1 as características técnicas da TV Móvel [Mobile TV] e sua diferença para o IPTV e a TV pela Internet [Internet TV], (2 as questões relativas a rede, espectro e equipamento que influenciarão o arcabouço normativo, (3 as oportunidades geradas para a TV Móvel quando da migração para a TV digital, e (4 um estudo de caso do México pertinente ao marco regulatório dos serviços de telecomunicações e de radiodifusão, aos atores do mercado, à disponibilidade de espectro, à migração para a TV digital e outras questões proeminentes para a TV Móvel.

Preparar para el futuro: el rol del educador

Directory of Open Access Journals (Sweden)

Myrna A. Cuevas Ruiz

2000-12-01

Full Text Available El artículo aborda el problema de la educación a la luz de las esperanzas y exigencias que se le plantean y de sus posibilidades reales para responder a los retos. En este marco, ubica la figura del educador, las cualidades que deben caracterizarlo para responder a las demandas de los retos educativos y los requerimientos que, en el contexto de la profesión docente, deben satisfacerse para impulsar los cambios.

QRLabel y Blackbird: 2 aplicaciones accesibles para Android

OpenAIRE

Mercade Aguila, Gisela

2012-01-01

El objetivo de este proyecto es el de realizar dos aplicaciones para dispositivos móviles con sistema operativo Android. Estas aplicaciones están ideadas para ser usadas por una persona invidente o con deficiencias visuales. El transcurso del desarrollo de cada aplicacion sirve para conocer y experimentar con diferentes herramientas que dispone este sistema operativo, y de este modo estudiar sus ventajas y limitaciones. Se presentan dispositivos y prototipos de dispositivos diseñados exclusiv...

Análise do EIA/RIMA do Terminal de Combustíveis da Paraíba (TECOP para fins de licenciamento ambiental

Directory of Open Access Journals (Sweden)

Valdeniza Delmondes Pereira

2016-09-01

Full Text Available O estudo de impacto ambiental precede ao licenciamento ambiental, sendo obrigatório para atividades efetiva ou potencialmente poluidoras ou capazes de degradar o meio ambiente. Este trabalho teve como objetivo fazer a análise do EIA/RIMA do Terminal de Combustíveis da Paraíba Ltda-TECOP, observando se foram cumpridas as exigências legais para a caracterização dos impactos ambientais, e verificando também a existência de medidas mitigadoras para os impactos adversos e indicação de monitoramento para os referidos impactos identificados. Trata-se de uma pesquisa descritiva e documental, que teve como objetos de estudo, o EIA/RIMA e o Processo de Licenciamento Ambiental do TECOP, cujos dados foram coletados junto ao Setor de Documentação da SUDEMA. O EIA/RIMA se mostrou extremamente superficial no que diz respeito à caracterização dos impactos ambientais, estes não sendo devidamente caracterizados conforme determina a Resolução Conama 01/86, artigo 6, inciso II. Os atributos para a correta caracterização dos impactos não foram levados em consideração sequer para os principais impactos adversos passíveis de ocorrerem na fase de operação do empreendimento. O estudo também não mostrou clareza quanto à proposição das medidas mitigadoras e monitoramento para os impactos identificados, pois nem todos os impactos adversos continham a indicação de mitigação e como seriam monitorados.

Modelos para gestão de riscos em cadeias de suprimentos: revisão, análise e diretrizes para futuras pesquisas

Directory of Open Access Journals (Sweden)

Robson Nogueira Tomas

2013-01-01

Full Text Available O objetivo deste artigo é apresentar uma revisão bibliográfica, que possa ser considerada completa e inédita na literatura científica nacional, tendo como foco modelos para gestão de riscos em cadeias de suprimentos. Para tanto, foram revisados 103 artigos científicos publicados entre 2000 e 2012. Inicialmente, buscou-se categorizar os tipos de riscos abordados nos artigos científicos segundo a descrição proposta por Christopher e Peck (2004, a saber: internos à empresa focal (operações, processo e controle; externos para a empresa focal, mas internos para a cadeia (fornecimento e demanda; e externos à cadeia (macroambiente. Buscou-se, também, identificar as etapas elementares na composição de um modelo para gestão de riscos em cadeias de suprimentos. Uma vez categorizados os tipos de riscos e identificadas as quatro etapas elementares, efetuou-se uma análise a partir de três perspectivas: i as opções de modelos disponíveis na literatura para a prática de tal gestão, considerando-se os tipos de riscos e etapas que os compõem; ii um modelo final a ser proposto; e iii elementos que podem ser acrescentados ao modelo final. As principais contribuições deste trabalho dizem respeito a possibilitar melhor entendimento dos tipos de riscos que podem incidir sobre uma cadeia de suprimentos, discutir os modelos mais adequados para geri-los, além de oferecer diretrizes para futuras pesquisas na temática em questão.

Relapsing-Remitting MS (RRMS)

Medline Plus

Full Text Available ... Multiple Sclerosis and Your Emotions (.pdf) Download Brochure Disease Modifying Therapies Overview (.pdf) Download Document Pediatric MS Learn More Transverse Myelitis Learn More Neuromyelitis Optica (NMO) Learn More HTLV-I ... Learn More Acute Disseminated Encephalomyelitis (ADEM) Learn More ...

Aquaporin-4-autoimmunity in patients with systemic lupus erythematosus

DEFF Research Database (Denmark)

Asgari, Nasrin; Jarius, Sven; Laustrup, Helle

2018-01-01

BACKGROUND: Serum immunoglobulin G targeting the astrocyte water channel aquaporin-4 (AQP4) in the central nervous system (CNS) is a biomarker for neuromyelitis optica spectrum disease (NMOSD). Co-existence of NMOSD with systemic lupus erythematosus (SLE) putatively suggests susceptibility...

Construcción de un indicador para medir competencias básicas para la vida

OpenAIRE

Andrea Collado Chaves

2006-01-01

La necesidad de construir un indicador para cuantificar el nivel de Competencias Básicas para la Vida (CBV), nace con la evaluación los resultados de un proyecto dirigido a población joven. El propósito de ese proyecto es construir capital humano y empleabilidad en hombres y mujeres con edades entre 13 y 18 años, desertores o en riesgo de desertar del sistema educativo, sin empleo formal y que viven en ambientes de pobreza y exclusión. Una de las dimensiones de empleabilidad son las Competenc...

MODELO PARA LA GESTIÓN DEL CONOCIMIENTO ORGANIZACIONAL EN LA SUITE PARA LA DIRECCIÓN INTEGRADA POR PROYECTOS GESPRO

Directory of Open Access Journals (Sweden)

Anisleiby Fernández Hernández

2014-06-01

Full Text Available La economía mundial se dirige hacia un nuevo modelo basado en el conocimiento, aportando ventajas competitivas gracias a su gestión. La Dirección Integrada por Proyectos requiere de la aplicación de un modelo para la gestión del conocimiento, con el objetivo de garantizar la elevación de la calidad de los nuevos proyectos. En el trabajo se presenta un modelo que combina y complementa diferentes estrategias para identificar, adquirir, retener, desarrollar y utilizar el conocimiento necesario en la realización de los proyectos, apoyadas por el uso de las Tecnologías de la Informática y las Comunicaciones. Este modelo responde y está en correspondencia con la versión 13.05 de la Suite para la Dirección Integrada por Proyectos Gespro de Software libre perteneciente a la Universidad de las Ciencias Informáticas, que se presenta como proceso para la gestión del conocimiento.

"Colaborar para Competir"

Directory of Open Access Journals (Sweden)

Martha Liliana Hijuelos-Cárdenas

2015-06-01

Full Text Available De acuerdo con Michael Porter, los Clusters se definen como “Una agrupación de empresas e instituciones relacionadas entre sí, pertenecientes a un mismo sector o segmento de mercado, que se encuentran próximas geográficamente y que colaboran para ser más competitivas”. Los Clusters son en última instancia una herramienta de competitividad basada en la cooperación de sus miembros, y es una de las estrategias recientes, en el país, para incrementar la productividad y competitividad y las apuestas productivas desde el nivel local. Y es precisamente esta cercanía geográfica de las empresas de un mismo sector, lo que a la vez que genera la competencia entre ellas, también les facilita el acceso a insumos y a conocimientos específicos y en última instancia les permite promover la innovación.

ParaTrough v1.0: Librería en Modelica para Simulación de Plantas Termosolares

Directory of Open Access Journals (Sweden)

Juan A. Romera Cabrerizo

2017-10-01

Full Text Available Resumen: El presente trabajo describe una librería desarrollada en Modelica que utiliza el entorno Dymola 6.1 para modelar y simular plantas termosolares de tecnología de colector cilindro-parabólico. El actual software de modelado y simulación es cada vez más potente gracias a los avances en computación y programación, pudiendo conseguir estimaciones muy precisas del comportamiento de estas plantas térmicas. Como mejora a otras propuestas actuales, la librería ParaTrough se ofrece como una herramienta pública, gratuita bajo licencia Modelica License 2, de código libre, flexible, modular, y por lo tanto fácilmente ampliable y modificable para los requerimientos específicos de cada planta y proceso en particular. En la versión 1.0 contemplada en este artículo, esta librería se puede usar para el modelado y simulación del recurso solar y del sistema de fluido de transferencia calorífica sin cambio de fase. Los modelos han sido validados con datos reales de una planta en operación, Andasol 3, en los términos municipales de Aldeire y La Calahorra (Granada. El objetivo de ParaTrough es poder ser utilizada gratuitamente y de forma amigable por analistas de procesos para uno o varios de los siguientes casos: evaluación del rendimiento, detección de fallos, exploración de nuevos modos de operación y optimización de la planta. Aunque en futuras versiones se puedan añadir otros elementos, esta aportación cubre una nueva área de aplicación específica para el software de Modelica y en su estado actual facilita la operación y mantenimiento de estas plantas. Abstract: This paper describes a Modelica-based library developed to the modeling and simulation of solar thermal plants with parabolic trough collectors. The Dymola 6.1 environment has been used. Unlike other commercial tools, the ParaTrough library is offered as a free open source tool, under Modelica License 2. Its modular code makes it easily extensible and modifiable to

Framework Multi-LIBRAS para integração e colaboração de soluções para deficientes auditivos

Directory of Open Access Journals (Sweden)

Luciana Pereira Oliveira

2017-08-01

Full Text Available Vem crescendo o número de soluções para pessoas com deficiência auditiva. Por exemplo, vêm surgindo diversas soluções de tradutores de Português para LIBRAS para facilitar a comunicação entre ouvintes e surdos. Por outro lado, muitos projetos de pesquisa estão descontinuados, não sendo possível executar os aplicativos desenvolvidos e muito menos repetir experimentos descritos nos artigos. Então, como os pesquisadores poderiam trabalhar de maneira colaborativa a construção de novos aplicativos e melhorias dos softwares existentes? Seria possível distintos grupos de pesquisa criarem um dicionário de Português e LIBRAS aberto? Objetivando-se esse ambiente colaborativo, definiu-se o framework Multi-LIBRAS, que oferece flexibilidade e extensibilidade para integrar qualquer solução para LIBRAS e algoritmos de avaliação. O artigo irá apresentar uma visão geral, as camadas externas e internas do framework bem como exemplificar o uso do Multi-LIBRAS, que dá suporte a soluções descritas em qualquer linguagem de programação.

Pequenos para idade gestacional: fator de risco para mortalidade neonatal

Directory of Open Access Journals (Sweden)

Almeida Márcia Furquim de

1998-01-01

Full Text Available OBJETIVO: Estudar as variáveis contempladas na Declaração de Nascimento (DN como possíveis fatores de risco para nascimentos pequenos para a idade gestacional (PIG e o retardo de crescimento intra-uterino como fator de risco para a mortalidade neonatal. MATERIAL E MÉTODO: As variáveis existentes na DN foram obtidas diretamente de prontuários hospitalares. Os dados referem-se a uma coorte de nascimentos obtida por meio da vinculação das declarações de nascimento e óbito, correspondendo a 2.251 nascimentos vivos hospitalares, de mães residentes, ocorridos no Município de Santo André, Região Metropolitana de São Paulo, no período de l/1 a 30/6/1992, e aos óbitos neonatais verificados nessa coorte. RESULTADOS: Obteve-se a proporção de 4,3% de nascimentos PIG, significativamente maior entre os recém-nascidos de pré-termo e pós-termo, entre os nascimentos cujas mães tinham mais de 35 anos de idade e grau de instrução inferior ao primeiro grau completo. Os recém-nascidos PIG apresentam maior risco de morte neonatal que aqueles que não apresentavam sinais de retardo de crescimento intra-uterino. CONCLUSÕES: Em áreas com menor freqüência de baixo peso ao nascer, é importante investigar a presença de retardo de crescimento intra-uterino entre os nascimentos prematuros e não apenas nos nascimentos de termo. O registro da data da última menstruação (ou da idade gestacional em semanas não agregadas na DN facilitaria a detecção de PIGs na população de recém-nascidos.

Propuesta de implementación de un modelo de calidad para la selección de software base para servidores.

OpenAIRE

Espinel Mena, Gonzalo Patricio; Montaluisa Yugla, Franklin Javier

2012-01-01

La propuesta de implementación plantea la utilización de un modelo de calidad estructurado para ayudar a resolver el problema de adquirir paquetes de software de base que encajen normalmente dentro de un entorno organizacional. El modelo de calidad estructurado para un dominio de un paquete dado proporcionará una taxonomía de características de software de calidad, y métricas para calcular su valor. El modelo de calidad se basa la norma ISO/IEC 9126 de la ISO (Organización Internacional de No...

Desenvolvimento de uma abordagem computacional para a descoberta de compostos-líderes para fármacos anticancerígenos

OpenAIRE

Cruz, Sara Miguel Dinis Mamede da

2016-01-01

Atualmente o cancro é uma das principais causas de morte, causando uma crescente demanda para a descoberta de fármacos para o seu tratamento. O objetivo desta dissertação foi a construção de modelos computacionais para auxiliar a descoberta de novos compostos líder anticancerígenos contra o cancro do cólon. Foram realizados dois diferentes estudos da Relação Quantitativa Estrutura Atividade (QSAR), o primeiro intitulado Modelo A, que utilizou conjuntos de descritores estruturais e fingerpr...

Cubierta de madera para una nave industrial

Directory of Open Access Journals (Sweden)

Hossdorf, Heinz

1958-05-01

Full Text Available Para la ampliación de los talleres de Hans Schmidlin, de Basilea, Suiza, dedicados a la prefabricación de elementos de construcción de madera y metales ligeros para fachadas, se ha construido una nueva nave, rectangular, de 49x55,4 m en planta.

Secadores solares - térmicos para granos y frutas para utilizarlo en zonas remotas

OpenAIRE

Delgado, E.; Peralta, J.; Barriga, A.

2009-01-01

Los objetivos principales de esta investigación son: • Dar a conocer la ventaja que tiene el uso de la utilización de la energía solar térmica para sistemas de secadores solares en la disminución de la humedad de los producto. • Valorar la importancia de la conservación energética y ambiental a través del uso de energía limpia. • Dar a conocer diseños de sistema de secado solar para alimentos, principalmente granos y frutas acorde a las características del producto, geográficas, técnicas y so...

Novo teste para diagnóstico das afasias

Directory of Open Access Journals (Sweden)

José G. Albernaz

1953-06-01

Full Text Available Um novo teste para diagnóstico e contrôle do tratamento das diferentes formas de afasia, especialmente adaptado para a língua portuguêsa, é apresentado e descrito em detalhe. O teste, que será de valor para clínicos e psicólogos interessados no tratamento e reabilitação de pacientes afásicos, se executa em cêrca de 30 minutos, interpreta-se fàcilmente e fornece os elementos necessários para o planejamento da reeducação da linguagem. Além disso, a repetição do teste de tempos em tempos indicará os progressos do paciente e sugerirá modificações do plano de tratamento no momento oportuno.

Os motivos das relações entre orientação para mercado, orientação para aprendizagem e sucesso de novos produtos

Directory of Open Access Journals (Sweden)

Mauren do Couto Soares

2016-04-01

Full Text Available O objetivo deste trabalho é identificar os motivos das relações entre os construtos de orientação para mercado, orientação para aprendizagem e sucesso de novos produtos. Neste sentido, foram coletadas 32 entrevistas com executivos de empresas de setores representativos da economia brasileira. Os principais resultados indicam que: (1 grande parte dos motivos encontrados nas entrevistas corrobora as evidências levantadas na literatura; (2 algumas variáveis podem moderar as relações entre orientação para mercado, orientação para aprendizagem e sucesso de novos produtos, entre elas a motivação, a burocracia e os recursos humanos; e, (3 empresas que integram os construtos de orientação para mercado, orientação para aprendizagem e sucesso de novos produtos podem potencializar sua performance, facilitando a obtenção de uma vantagem competitiva sustentável.

Entrenamiento de fuerza para la salud

OpenAIRE

Ortiz Cervera, Vicente

1996-01-01

Este artículo describe de manera breve y clara los efectos del entrenamiento de fuerza sobre la salud, al mismo tiempo da pautas para la realización y programación de los entrenamientos de fuerza para la salud en distintas edades. Se muestran las diferentes variables del entrenamiento de la fuerza junto con ejemplos de entrenamientos, basándose en las pautas marcadas en la revisión bibliográfica descrita.

Páginas web para el aprendizaje

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Isabel ÁLVAREZ

2016-04-01

Full Text Available Este artículo plantea la construcción de paginas web como vehículo para facilitar la integracion de las tecnologias de la informacion y la comunicacion en el aula para futuros maestros. El término Página Web Coherente enfatiza la utilización de páginas cuyo objetivo principal es promover el aprendizaje siguiendo un proceso colaborativo, una estructura integradora y un contenido educativo.

Movement and equipositioning of plasmids by ParA filament disassembly

DEFF Research Database (Denmark)

Ringgaard, Simon; van Zon, Jeroen; Howard, Martin

2009-01-01

, plasmids consistently migrate behind disassembling ParA cytoskeletal structures, suggesting that ParA filaments pull plasmids by depolymerization. The perpetual cycles of ParA assembly and disassembly result in continuous relocation of plasmids, which, on time averaging, results in equidistribution...

Contribuição para o estudo de sistemas de tratamento de água para abastecimento associados à remoção de compostos farmacêuticos

OpenAIRE

Cid, Joana Ferraz Leal de Sobral

2011-01-01

Dissertação apresentada na Faculdade de Ciências e Tecnologia da Universidade Nova de Lisboa para obtenção do grau de Mestre em Engenharia do Ambiente, perfil Engenharia Sanitária Este trabalho pretende contribuir para o estudo de sistemas de tratamento de água para abastecimento associados à remoção de compostos farmacêuticos. Assim, para melhor compreender a importância da remoção dos compostos farmacêuticos da água para abastecimento surge uma abordagem sobre a presença dos compostos...