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Sample records for ions absorcao optica

  1. Estudo de propriedades estruturais e opticas de multicamadas epitaxiais emissoras de luz baseadas em InGaN/GaN

    Science.gov (United States)

    Pereira, Sergio Manuel de Sousa

    Esta tese apresenta os resultados de uma investigacao experimental em filmes epitaxiais emissores de luz baseados em InxGa1-xN. O InxGa1-xN e uma liga semicondutora ternaria do grupo III-N muito utilizada como camada activa numa gama de dispositivos optoelectronicos em desenvolvimento, incluindo diodos emissores de luz (LEDs) e diodos laser (LDs), para operacao na regiao do visivel e ultravioleta do espectro electromagnetico. Neste estudo, caracterizam-se as propriedade opticas e estruturais de camadas simples e pocos quânticos multiplos (Multiple Quantum Wells, MQWs) de InxGa1-xN/GaN, com enfase nas suas propriedades fisicas fundamentais. O objectivo central do trabalho prende-se com a compreensao mais profunda dos processos fisicos que estao por tras das suas propriedades opticas, preenchendo o fosso existente entre aplicacoes tecnologicas e o conhecimento cientifico. Nomeadamente, a tese aborda os problemas da medicao da fraccao de InN (x) em multicamadas ultrafinas sujeitas a tensoes, a influencia da composicao e das tensoes microscopicas nas propriedades opticas e estruturais. A questao relativa a segregacao de fases em multicamadas de InxGa1-xN/GaN e tambem discutida a luz dos resultados obtidos. A metodologia seguida assenta na integracao de resultados obtidos por tecnicas complementares atraves de uma analise sistematica e multidisciplinar. Esta abordagem passa pela combinacao de: 1) Crescimento de amostras por deposicao epitaxial em fase de vapor organometalico (MOVPE) com caracteristicas especificas de forma a tentar isolar parâmetros estruturais, tais como espessura e composicao; 2) Caracterizacao nanoestrutural por microscopia de forca atomica (AFM), microscopica electronica de varrimento (SEM), difraccao de raios-X e retro-dispersao de Rutherford (RBS); 3) Caracterizacao optica a escalas complementares por: espectroscopia de absorcao optica (OA), fotoluminescencia (PL), catodoluminescencia (CL) e microscopia confocal (CM) com analise espectral. Com

  2. 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.

  3. Spectrum of pediatric neuromyelitis optica.

    Science.gov (United States)

    Lotze, Timothy E; Northrop, Jennifer L; Hutton, George J; Ross, Benjamin; Schiffman, Jade S; Hunter, Jill V

    2008-11-01

    Our goal was to describe the spectrum of clinical phenotypes, laboratory and imaging features, and treatment in pediatric patients with neuromyelitis optica. The study consisted of a retrospective chart review of patients followed in a pediatric multiple sclerosis center with a diagnosis of neuromyelitis optica spectrum disorder. Nine patients with neuromyelitis optica spectrum disorders were included, all of whom were female. There were 4 black children, 2 Latin American children, 2 white children, and 1 child of mixed Latin American/white heritage. Median age at initial attack was 14 years (range: 1.9-16 years). Median disease duration was 4 years (range: 0.6-9 years). Tests for neuromyelitis optica immunoglobulin G were positive for 7 patients. Eight patients had transverse myelitis and optic neuritis, and 1 patient had longitudinally extensive transverse myelitis without optic neuritis but had a positive neuromyelitis optica immunoglobulin G antibody titer. Cerebral involvement on MRI was found in all subjects, 5 of whom were symptomatic with encephalopathy, seizures, hemiparesis, aphasia, vomiting, or hiccups. Immunosuppressive therapy reduced attack frequency and progression of disability. Pediatric neuromyelitis optica has a diverse clinical presentation and may be difficult to distinguish from multiple sclerosis in the early stages of the disease. The recognition of the broad spectrum of this disease to include signs and symptoms of brain involvement is aided by the availability of a serum biomarker: neuromyelitis optica immunoglobulin G. Early diagnosis and immunosuppresive treatment may help to slow the accumulation of severe disability.

  4. Immunotherapy of Neuromyelitis Optica

    Science.gov (United States)

    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

  5. INTRODUCTION TO ACTA OPTICA SINICA

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    Acta Optica Sinica is a scientific periodical on optics,sponsored by Chinese Optical Society, jointly undertaken by Shanghai Institute of Optics and Fine Mechanics,Changchun Institute of Optics, Fine Mechanics and Physics,the Chinese Academy of Sciences, monthly,first published in 1981. The chief editor is Professor Xu Zhizhan, Academician of the Chinese Academy of Sciences. Now Acta Optica Sinica is a source periodical of the following international index periodicals: SA,CA,EI and PЖ.

  6. [Topics of neuromyelitis optica].

    Science.gov (United States)

    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.

  7. Neuromyelitis Optica Spectrum Disorders.

    Science.gov (United States)

    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.

  8. Aquaporin 4 and neuromyelitis optica

    Science.gov (United States)

    Papadopoulos, Marios C; Verkman, A S

    2013-01-01

    Neuromyelitis optica is an inflammatory demyelinating disorder of the CNS. The discovery of circulating IgG1 antibodies against the astrocyte water channel protein aquaporin 4 (AQP4) and the evidence that AQP4-IgG is involved in the development of neuromyelitis optica revolutionised our understanding of the disease. However, important unanswered questions remain—for example, we do not know the cause of AQP4-IgG-negative disease, how astrocyte damage causes demyelination, the role of T cells, why peripheral AQP4-expressing organs are undamaged, and how circulating AQP4-IgG enters neuromyelitis optica lesions. New drug candidates have emerged, such as aquaporumab (non-pathogenic antibody blocker of AQP4-IgG binding), sivelestat (neutrophil elastase inhibitor), and eculizumab (complement inhibitor). Despite rapid progress, randomised clinical trials to test new drugs will be challenging because of the small number of individuals with the disorder. PMID:22608667

  9. 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...

  10. Neuromyelitis Optica Immunoglobulin G in a Child

    OpenAIRE

    Hudson, Lynsee A.; Bernard, Timothy J.; Tseng, Brian S.; Miller, Bradford R.; Corboy, John R.

    2006-01-01

    Neuromyelitis optica or Devic’s syndrome is an uncommon demyelinating disorder that preferentially attacks the spinal cord and optic nerves. Although it is well described in adults, childhood neuromyelitis optica has rarely been reported in the literature and is frequently misdiagnosed as severe multiple sclerosis. Recently, a serum immunoglobulin G test for neuromyelitis optica has become available which may clarify and accelerate the diagnosis. This report describes a child with recurrent m...

  11. Progressive cerebral atrophy in neuromyelitis optica.

    Science.gov (United States)

    Warabi, Yoko; Takahashi, Toshiyuki; Isozaki, Eiji

    2015-12-01

    We report two cases of neuromyelitis optica patients with progressive cerebral atrophy. The patients exhibited characteristic clinical features, including elderly onset, secondary progressive tetraparesis and cognitive impairment, abnormally elevated CSF protein and myelin basic protein levels, and extremely highly elevated serum anti-AQP-4 antibody titer. Because neuromyelitis optica pathology cannot switch from an inflammatory phase to the degenerative phase until the terminal phase, neuromyelitis optica rarely appears as a secondary progressive clinical course caused by axonal degeneration. However, severe intrathecal inflammation and massive destruction of neuroglia could cause a secondary progressive clinical course associated with cerebral atrophy in neuromyelitis optica patients. © The Author(s), 2015.

  12. Neuromyelitis Optica Lesion Mimicking Brainstem Glioma

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2007-12-01

    Full Text Available A 12-year-old girl who presented with weakness of the left extremities and right sided sixth cranial nerve palsy had neuromyelitis optica (NMO mistaken for brainstem glioma on MRI, in a report from Brain Research Institute, Yonsei University College of Medicine,Seoul, Republic of KoreaNeuromyelitis Optica, Optic-Spinal Syndrome, Spectroscopy.

  13. Neuromyelitis optica: a diffusional kurtosis imaging study.

    Science.gov (United States)

    Doring, T M; Lopes, F C R; Kubo, T T A; Tukamoto, G; Kimura, M C; Strecker, R M; Domingues, R C; Gasparetto, E L

    2014-12-01

    Conventional MR imaging typically yields normal images of the brain or indicates lesions in areas of high aquaporin expression in patients with neuromyelitis optica. Diffusional kurtosis imaging was applied in patients with neuromyelitis optica to determine whether this technique could detect alterations in diffusion and diffusional kurtosis parameters in normal-appearing white matter and to explore the relationship between diffusional kurtosis imaging and DTI parameters. Thirteen patients with neuromyelitis optica and 13 healthy controls underwent MR imaging of the brain with conventional and diffusional kurtosis imaging sequences. Tract-based spatial statistics and region-of-interest-based analyses were conducted to identify differences between patients with neuromyelitis optica and controls through conventional DTI and diffusional kurtosis imaging parameters. The parameters were correlated to determine the potential relationship between them. Compared with healthy controls, several diffusional kurtosis imaging and DTI parameters were altered in various fiber tracts of patients with neuromyelitis optica (P optica. We found a negative correlation between diffusional kurtosis imaging (radial kurtosis, axial kurtosis, mean kurtosis) and the corresponding DTI parameters (radial diffusivity, axial diffusivity, mean diffusivity). Positive correlations were found for radial kurtosis and mean kurtosis with fractional anisotropy. This study demonstrated differences in conventional diffusion and diffusional kurtosis parameters, especially radial kurtosis, in the normal-appearing white matter of patients with neuromyelitis optica compared with healthy controls. Larger studies of patients with neuromyelitis optica should be performed to assess the potential clinical impact of these findings. © 2014 by American Journal of Neuroradiology.

  14. Neuromyelitis optica in a young child with positive serum autoantibody

    OpenAIRE

    Loma, Ingrid P.; Asato, Miya R.; Filipink, Robyn A.; Alper, Gulay

    2008-01-01

    Relapsing neuromyelitis optica is rare in children. The identification of highly specific serum autoantibody marker (neuromyelitis optica –immunoglobulin G) differentiates neuromyelitis optica from other demyelinating disorders particularly in clinically challenging cases. We present a child with multiple episodes of transverse myelitis and optic neuritis with positive neuromyelitis optica-immunoglobulin G titers consistent with a diagnosis of relapsing neuromyelitis optica. Serial titers of ...

  15. Multisystem involvement in neuromyelitis optica

    Directory of Open Access Journals (Sweden)

    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.

  16. 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.

  17. 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

  18. 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

  19. 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).

  20. 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

  1. 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

  2. Neuromyelitis Optica: A Case Report

    Directory of Open Access Journals (Sweden)

    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.

  3. OPTIC NEURITIS IN NEUROMYELITIS OPTICA

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    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

  4. 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

  5. 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...

  6. 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...

  7. 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

  8. Relapse of Neuromyelitis Optica Spectrum Disorder Associated with Intravenous Lidocaine

    Directory of Open Access Journals (Sweden)

    Akiyuki Uzawa

    2011-01-01

    Full Text Available Lidocaine unmasks silent symptoms and eases neuropathic pain in multiple sclerosis patients; however, the effects of lidocaine in neuromyelitis optica have never been reported. We describe the case of a 59-year-old Japanese woman with neuromyelitis optica spectrum disorder who developed optic neuritis 1 day after intravenous lidocaine injection for treating allodynia. Her symptom seemed to result from a relapse of neuromyelitis optica induced by lidocaine administration, and not because of the transient effects of intravenous lidocaine administration. The possibility that lidocaine administration results in relapse of neuromyelitis optica due to its immunomodulating effects cannot be ruled out.

  9. 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.

  10. 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....

  11. Prolonged Remission in Neuromyelitis Optica Following Cessation of Rituximab Treatment.

    Science.gov (United States)

    Weinfurtner, Kelley; Graves, Jennifer; Ness, Jayne; Krupp, Lauren; Milazzo, Maria; Waubant, Emmanuelle

    2015-09-01

    Neuromyelitis optica is an autoimmune disease characterized by acute episodes of transverse myelitis and optic neuritis. Several small, open-label studies suggest rituximab, a monoclonal antibody against CD20, prevents relapses in neuromyelitis optica; however, there is little consensus on timing or duration of treatment. Here we report four patients with severe relapsing neuromyelitis optica who were stabilized on rituximab and, after discontinuing treatment, continued to experience prolonged remission of their disease. Remission ranged from 4.5 to 10.5 years total, including 3 to 9 years off all therapies. The patients had sustained clinical responses despite normal B-lymphocyte levels and, in at least 2 patients, continued seropositivity for aquaporin-4 antibodies. These cases suggest that rituximab may induce prolonged remission in certain neuromyelitis optica patients, and they highlight the need for further elucidation of rituximab's mechanism in neuromyelitis optica. © The Author(s) 2014.

  12. Membranous nephropathy with Neuromyelitis optica spectrum disorder.

    Science.gov (United States)

    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.

  13. EFNS guidelines on diagnosis and management of neuromyelitis optica

    DEFF Research Database (Denmark)

    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...

  14. 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

  15. 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.

  16. Longitudinally extensive optic neuritis in neuromyelitis optica spectrum disorder.

    Science.gov (United States)

    Pula, John H; Kattah, Jorge C; Keung, Bonnie; Wang, Huaping; Daily, Jennifer

    2014-10-15

    Neuomyelitis optica, sarcoid, and multiple sclerosis can all cause optic neuritis. Further means of distinguishing the causes of optic neuritis among these etiologies would be valuable for the clinician. This is a retrospective, cohort study from a single university based hospital and neuro-ophthalmology clinic. Blinded interpretation of orbit MRIs was performed on patients with acute optic neuritis from multiple sclerosis (n=25), sarcoid (n=5) and neuromyelitis optica spectrum disorder (n=6). A length of >40 mm anterior visual pathway enhancement distinguished neuromyelitis optica spectrum disorder from multiple sclerosis (p=0.0376). No statistically significant differences were found for presence of pain or papillitis, however there was a trend for bilateral involvement and chiasmal involvement in neuromyelitis optica spectrum disorder compared to multiple sclerosis. In acute optic neuritis, enhancing anterior visual pathway lesion length >40 mm helps differentiate neuromyelitis optica spectrum disorder from multiple sclerosis. This degree of involvement can be considered longitudinally extensive optic neuritis. Further characterization is necessary as this degree of enhancement occurs in other clinical syndromes besides neuromyelitis optica. Copyright © 2014 Elsevier B.V. All rights reserved.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    Khorooshi, Reza; Wlodarczyk, Agnieszka; Asgari, Nasrin; Owens, Trevor

    2013-09-01

    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 the formation of neuromyelitis optica lesions. Immunoglobulin G from a neuromyelitis optica patient was injected intracerebrally with human complement to type I interferon receptor deficient and wildtype mice. Loss of aquaporin-4 and glial fibrillary acidic protein was reduced in type I interferon receptor deficient mice brain. Our findings suggest that type I interferon signaling contributes to neuromyelitis optica pathogenesis. Copyright © 2013 Elsevier Inc. All rights reserved.

  20. Neuromyelitis optica with onset in childhood and adolescence.

    Science.gov (United States)

    Fragoso, Yara Dadalti; Ferreira, Maria L B; Oliveira, Enedina M L; Domingues, Renan B; Ribeiro, Taysa A G J; Brooks, Joseph B B; Claudino, Rinaldo; Netto, Jussara M K; Gomes, Sidney; Adoni, Tarso; Carneiro, Denise S D; Fonseca, Daiana R P; Fragomeni, Manuela O; Oliveira, Francisco T M; Oliveira, Celso L S; Saldanha, Patricia C O; Souza, Jorge M B

    2014-01-01

    Neuromyelitis optica with onset before the age of 18 years is a relatively rare, yet potentially devastating condition. The objective of the present study was to contribute to the study of early-onset neuromyelitis optica with a case series. Data were collected from medical records of Brazilian neurologists caring for patients with neuromyelitis optica occurring in childhood and adolescence. Twenty-nine patients with neuromyelitis optica occurring before the age of 18 years and fulfilling the diagnostic criteria were identified. The average age at disease onset was 13 years and the patients had had an average disease duration of 6 years. The expanded disability scale score at the latest consultation was, on average, 4.7, and one patient had died from the disease. The 29 patients had had an average 4.5 relapses during the disease, accounting for 0.75 relapses per year, irrespective of the medication used. All patients were using one or more of the following medications: azathioprine, prednisone, immunoglobulin, and glatiramer acetate. Neuromyelitis optica with onset in childhood and adolescence is a poorly understood condition that is often disabling and difficult to manage. Copyright © 2014 Elsevier Inc. All rights reserved.

  1. Detection of anti-aquaporin-4 autoantibodies in the sera of Chinese neuromyelitis optica patients

    Institute of Scientific and Technical Information of China (English)

    Miao Li; Weiheng Su; Jie Wang; Francesco Pisani; Antonio Frigeri; Tonghui Ma

    2013-01-01

    In this study, we recruited 10 neuromyelitis optica patients, two multiple sclerosis patients and two myelitis patients. Chinese hamster lung fibroblast (V79) cells transfected with a human aquaporin-4-mCherry fusion protein gene were used to detect anti-aquaporin-4 antibody in neuromyelitis optica patient sera by immunofluorescence. Anti-aquaporin-4 autoantibody was stably detected by immunofluorescence in neuromyelitis optica patient sera exclusively. The sensitivity of the assay for neuromyelitis optica was 90% and the specificity for neuromyelitis optica was 100%. The anti-aquaporin-4 antibody titers in sera were tested with serial dilutions until the signal disappeared. A positive correlation was detected between Expanded Disability Status Scale scores and serum anti-aquaporin-4 antibody titers. The anti-aquaporin-4 antibody assay is highly sensitive and specific in the sera of Chinese neuromyelitis optica patients. Detection of aquaporin-4 autoantibody is important for the diagnosis and treatment of neuromyelitis optica.

  2. Detection of anti-aquaporin-4 autoantibodies in the sera of Chinese neuromyelitis optica patients.

    Science.gov (United States)

    Li, Miao; Su, Weiheng; Wang, Jie; Pisani, Francesco; Frigeri, Antonio; Ma, Tonghui

    2013-03-15

    In this study, we recruited 10 neuromyelitis optica patients, two multiple sclerosis patients and two myelitis patients. Chinese hamster lung fibroblast (V79) cells transfected with a human aquaporin-4-mCherry fusion protein gene were used to detect anti-aquaporin-4 antibody in neuromyelitis optica patient sera by immunofluorescence. Anti-aquaporin-4 autoantibody was stably detected by immunofluorescence in neuromyelitis optica patient sera exclusively. The sensitivity of the assay for neuromyelitis optica was 90% and the specificity for neuromyelitis optica was 100%. The anti-aquaporin-4 antibody titers in sera were tested with serial dilutions until the signal disappeared. A positive correlation was detected between Expanded Disability Status Scale scores and serum anti-aquaporin-4 antibody titers. The anti-aquaporin-4 antibody assay is highly sensitive and specific in the sera of Chinese neuromyelitis optica patients. Detection of aquaporin-4 autoantibody is important for the diagnosis and treatment of neuromyelitis optica.

  3. Detection of anti-aquaporin-4 autoantibodies in the sera of Chinese neuromyelitis optica patients★

    Science.gov (United States)

    Li, Miao; Su, Weiheng; Wang, Jie; Pisani, Francesco; Frigeri, Antonio; Ma, Tonghui

    2013-01-01

    In this study, we recruited 10 neuromyelitis optica patients, two multiple sclerosis patients and two myelitis patients. Chinese hamster lung fibroblast (V79) cells transfected with a human aquaporin-4-mCherry fusion protein gene were used to detect anti-aquaporin-4 antibody in neuromyelitis optica patient sera by immunofluorescence. Anti-aquaporin-4 autoantibody was stably detected by immunofluorescence in neuromyelitis optica patient sera exclusively. The sensitivity of the assay for neuromyelitis optica was 90% and the specificity for neuromyelitis optica was 100%. The anti-aquaporin-4 antibody titers in sera were tested with serial dilutions until the signal disappeared. A positive correlation was detected between Expanded Disability Status Scale scores and serum anti-aquaporin-4 antibody titers. The anti-aquaporin-4 antibody assay is highly sensitive and specific in the sera of Chinese neuromyelitis optica patients. Detection of aquaporin-4 autoantibody is important for the diagnosis and treatment of neuromyelitis optica. PMID:25206717

  4. 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

  5. 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.

  6. 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.

  7. Brain Abnormalities in Neuromyelitis Optica Spectrum Disorder

    Directory of Open Access Journals (Sweden)

    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.

  8. 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.

  9. 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

  10. MRI characteristics of neuromyelitis optica spectrum disorder

    Science.gov (United States)

    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

  11. Autoantibodies in patients with neuromyelitis optica

    Directory of Open Access Journals (Sweden)

    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

  12. Brain MRI abnormalities in neuromyelitis optica

    Energy Technology Data Exchange (ETDEWEB)

    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.

  13. Immunopathogenesis in Myasthenia Gravis and Neuromyelitis Optica

    Science.gov (United States)

    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

  14. Interferon Alpha Association with Neuromyelitis Optica

    Directory of Open Access Journals (Sweden)

    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.

  15. Autoimmune thyroiditis associated with neuromyelitis optica (NMO

    Directory of Open Access Journals (Sweden)

    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.

  16. 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.

  17. 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.

  18. Brain MRI lesions in neuromyelitis optica: clinical case; Lesiones cerebrales de resonancia magnetica en neuromielitis optica: caso clinico

    Energy Technology Data Exchange (ETDEWEB)

    Rosales Bravo, Luis Guillermo; Heyden Cordero, Marvin; Chinchilla Weinstok, Dennis; Mendelewicz Goldwaig, Isaias, E-mail: neurologia.cima@gmail.com [Caja Costarricense del Seguro Social, Hospital Mexico, Div. de Neurologia, San Jose (Costa Rica)

    2011-10-15

    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) [Spanish] Muchos casos de pacientes con neuromielitis optica se han presentado sin lesiones desmielinizantes en la sustancia blanca cerebral, se ha documentado que esta entidad puede causarlas desde el inicio de la enfermedad o a traves de su evolucion natural. Los metodos de diagnostico en la actualidad como la Imagen por Resonancia Magnetica (IRM) y la determinacion de anticuerpos especificos en plasma (como la antiaquaporina-4) han logrado diagnosticar algunos casos de neuromielitis optica que inicialmente fueron confundidos con esclerosis multiple. La enfermedad en Costa Rica ha sido poco prevalente y no se ha conocido con exactitud cual es la prevalencia e incidencia. El grado de afeccion ha sido ilustrado a traves de un caso clinico, tanto en la sustancia blanca cerebral como espinal, en un paciente con neuromielitis optica durante un periodo de seguimiento de 4 anos. Este es el primer caso que ha sido reportado en la literatura cientifica de Costa Rica. (autor)

  19. Neuromyelitis optica-- en vigtig differentialdiagnose til multipel sklerose

    DEFF Research Database (Denmark)

    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...

  20. Update on neuromyelitis optica: natural history and management

    Science.gov (United States)

    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

  1. [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.

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

    Science.gov (United States)

    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…

  3. 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

  4. 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

  5. Clinical evaluation of rituximab treatment for neuromyelitis optica.

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  7. Trigeminal root entry zone involvement in neuromyelitis optica and multiple sclerosis.

    Science.gov (United States)

    Sugiyama, Atsuhiko; Mori, Masahiro; Masuda, Hiroki; Uchida, Tomohiko; Muto, Mayumi; Uzawa, Akiyuki; Ito, Shoichi; Kuwabara, Satoshi

    2015-08-15

    Trigeminal root entry zone abnormality on brain magnetic resonance imaging has been frequently reported in multiple sclerosis patients, but it has not been investigated in neuromyelitis optica patients. Brain magnetic resonance imaging of 128 consecutive multiple sclerosis patients and 46 neuromyelitis optica patients was evaluated. Trigeminal root entry zone abnormality was present in 11 (8.6%) of the multiple sclerosis patients and two (4.3%) of the neuromyelitis optica patients. The pontine trigeminal root entry zone may be involved in both multiple sclerosis and neuromyelitis optica. Copyright © 2015 Elsevier B.V. All rights reserved.

  8. Gray Matter Volume Reduction Is Associated with Cognitive Impairment in Neuromyelitis Optica.

    Science.gov (United States)

    Wang, Q; Zhang, N; Qin, W; Li, Y; Fu, Y; Li, T; Shao, J; Yang, L; Shi, F-D; Yu, C

    2015-10-01

    Whether gray matter impairment occurs in neuromyelitis optica is a matter of ongoing debate, and the association of gray matter impairment with cognitive deficits remains largely unknown. The purpose of this study was to investigate gray matter volume reductions and their association with cognitive decline in patients with neuromyelitis optica. This study included 50 patients with neuromyelitis optica and 50 sex-, age-, handedness-, and education-matched healthy subjects who underwent high-resolution structural MR imaging examinations and a battery of cognitive assessments. Gray matter volume and cognitive differences were compared between the 2 groups. The correlations of the regional gray matter volume with cognitive scores and clinical variables were explored in the patients with neuromyelitis optica. Compared with healthy controls (635.9 ± 51.18 mL), patients with neuromyelitis optica (602.8 ± 51.03 mL) had a 5.21% decrease in the mean gray matter volume of the whole brain (P optica affected the frontal and temporal cortices and the right thalamus (false discovery rate correction, P optica (Alphasim correction, P optica had impairments in memory, information processing speed, and verbal fluency (P optica and is associated with cognitive impairment and disease severity in this group. © 2015 by American Journal of Neuroradiology.

  9. 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

  10. 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 ...

  11. 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.

  12. 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.

  13. 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.

  14. Systemic manifestations and pathogenesis exploration of neuromyelitis optica spectrum disorders

    Directory of Open Access Journals (Sweden)

    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

  15. Brainstem and limbic encephalitis with paraneoplastic neuromyelitis optica.

    Science.gov (United States)

    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.

  16. A Case of Neuromyelitis Optica Masquerading as Miller Fisher Syndrome

    Directory of Open Access Journals (Sweden)

    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.

  17. 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.

  18. 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

  19. 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.

  20. 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

  1. 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.

  2. 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...

  3. 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

  4. 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.

  5. Finding NMO: The Evolving Diagnostic Criteria of Neuromyelitis Optica

    Science.gov (United States)

    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

  6. Visual evoked potentials in neuromyelitis optica and its spectrum disorders.

    Science.gov (United States)

    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.

  7. Seronegative neuromyelitis optica presenting with life-threatening respiratory failure.

    Science.gov (United States)

    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.

  8. 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...

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

    Directory of Open Access Journals (Sweden)

    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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  12. 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.

  13. Synthesis and optical characterization of ZrO{sub 2} pure and doped with Er{sup 3+} ions for use as thermoluminescent dosimeter; Sintesis y caracterizacion optica de ZrO{sub 2} puro y dopado con iones Er{sup 3+} para su aplicacion como dosimetro termoluminiscente

    Energy Technology Data Exchange (ETDEWEB)

    Gutierrez E, R.; Sosa F, R.; Arrieta C, A. M., E-mail: ralf_gutierrez@yahoo.com [Universidad Autonoma Metropolitana, Unidad Iztapalapa, Departamento de Fisica, Av. San Rafael Atlixco 186, Col. Vicentina, 09340 Mexico D. F. (Mexico)

    2015-10-15

    Full text: There are different natural minerals and synthetic inorganic compounds exhibiting the phenomenon of thermoluminescence (Tl); however only part of them meet the requirements for use as Tl dosimeters. In this research the synthesis and characterization of ZrO{sub 2} pure and doped with Er{sup 3+} ions are presented, at a concentration of 0.5 % mol prepared by the sol-gel method in monolithic way and transparent for its possible application as thermoluminescent dosimeter. The materials are characterized by absorption spectroscopy, emission and excitation in the region of UV-Vis-Nir, in which spectra corresponding to ZrO{sub 2} and characteristic bands of Er{sup 3+} doped are observed, identifying the mechanisms of energy transfer for the emitted radiation as one of the most important points in the Tl materials design. It was observed that the threshold dose detection and accuracy of measurements depend on the efficiency of energy conversion. In addition an analysis by scanning electron microscopy, EDS and X-ray diffraction on the morphologic and spectroscopic changes caused by the reduction of organic material is presented. These were carried out in two different drying processes: at room temperature and annealing at 500 degrees C. All these physical and chemical properties are crucial in deciding its application in dosimetry of ionizing radiation. (Author)

  14. 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.

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

    Science.gov (United States)

    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

  16. 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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

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

    DEFF Research Database (Denmark)

    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....

  20. 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

  1. 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

  2. Widespread cortical thinning in patients with neuromyelitis optica spectrum disorder.

    Science.gov (United States)

    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.

  3. Is pruritus an indicator of aquaporin-positive neuromyelitis optica?

    Science.gov (United States)

    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.

  4. 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.

  5. NMO-IgG: A Specific Biomarker for Neuromyelitis Optica

    Directory of Open Access Journals (Sweden)

    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.

  6. Seronegative Neuromyelitis Optica: A Case Report of a Hispanic Male

    Directory of Open Access Journals (Sweden)

    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.

  7. 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.

  8. Autoimmune AQP4 channelopathies and neuromyelitis optica spectrum disorders.

    Science.gov (United States)

    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.

  9. Ovarian Reserve in Women With Neuromyelitis Optica Spectrum Disorder

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

  11. 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...

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

    Science.gov (United States)

    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…

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  15. 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.

  16. 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

  17. 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

  18. 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

  19. Neuromyelitis optica: association with paroxysmal painful tonic spasms.

    Science.gov (United States)

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  1. 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.)

  2. 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.

  3. Gender differences among Chinese patients with neuromyelitis optica spectrum disorders.

    Science.gov (United States)

    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.

  4. White matter atrophy and cognitive dysfunctions in neuromyelitis optica.

    Directory of Open Access Journals (Sweden)

    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

  5. STAT4 Polymorphisms are Associated with Neuromyelitis Optica Spectrum Disorders.

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    DEFF Research Database (Denmark)

    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...

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

    Directory of Open Access Journals (Sweden)

    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.

  9. Cognitive performance of neuromyelitis optica patients: comparison with multiple sclerosis

    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

  10. Early and extensive spinal white matter involvement in neuromyelitis optica.

    Science.gov (United States)

    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

  11. Adult methylmalonic acidemia presented as neuromyelitis optica: one case report

    Directory of Open Access Journals (Sweden)

    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

  12. 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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  16. Acute Respiratory Failure due to Neuromyelitis Optica Treated Successfully with Plasmapheresis

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  1. 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

  2. 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

  3. 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...

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

    Science.gov (United States)

    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

  5. 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...

  6. 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)

  7. Convergence spasm due to aquaporin-positive neuromyelitis optica spectrum disorder

    Directory of Open Access Journals (Sweden)

    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.

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

    DEFF Research Database (Denmark)

    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...

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

    DEFF Research Database (Denmark)

    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...

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    NARCIS (Netherlands)

    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

  14. 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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  2. 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.

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

    Science.gov (United States)

    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.

  4. 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...

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

  9. Catálogo de Radio-Fuentes Opticas con Astrolabio Fotoeléctrico PAII

    Science.gov (United States)

    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.

  10. Seronegative Neuromyelitis Optica Spectrum Disorder following Exposure to Hepatitis B Vaccination

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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

  12. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  18. 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.)

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

    Science.gov (United States)

    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

  20. 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 ...

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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

  3. The Contribution of Optical Coherence Tomography in Neuromyelitis Optica Spectrum Disorders

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    International Nuclear Information System (INIS)

    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.)

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

    Science.gov (United States)

    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.

  8. 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)

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  2. 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.

  3. Detection and clinical value of NMO-IgG in neuromyelitis optica

    Directory of Open Access Journals (Sweden)

    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

  4. The Contribution of Optical Coherence Tomography in Neuromyelitis Optica Spectrum Disorders

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  13. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  20. 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)

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

    Directory of Open Access Journals (Sweden)

    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.

  2. 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.)

  3. 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...

  4. 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.

    Science.gov (United States)

    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

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

    Directory of Open Access Journals (Sweden)

    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

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

    Science.gov (United States)

    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.

  7. 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.

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  11. Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  15. 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.

  16. 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.

  17. 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.

  18. 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.

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

    Science.gov (United States)

    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.

  20. 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)

  1. 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.

  2. 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

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

    Science.gov (United States)

    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.

  4. 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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

  7. 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

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

    Science.gov (United States)

    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.

  9. 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)

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

  14. 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.).

  15. 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

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

    Directory of Open Access Journals (Sweden)

    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

  17. 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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  20. 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.

  1. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

  5. 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

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

  8. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  10. 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

  11. 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.)

  12. 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.

  13. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  18. 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.

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

    International Nuclear Information System (INIS)

    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.

  20. 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

  1. 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.

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

    Science.gov (United States)

    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.

  3. 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

  4. 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

  5. Ion-ion collisions

    International Nuclear Information System (INIS)

    Salzborn, Erhard; Melchert, Frank

    2000-01-01

    Collisions between ions belong to the elementary processes occurring in all types of plasmas. In this article we give a short overview about collisions involving one-electron systems. For collisions involving multiply-charged ions we limit the discussion to one specific quasi-one-electron system. (author)

  6. Hydroxycholesterol Levels in the Serum and Cerebrospinal Fluid of Patients with Neuromyelitis Optica Revealed by LC-Ag+CIS/MS/MS and LC-ESI/MS/MS with Picolinic Derivatization: Increased Levels and Association with Disability during Acute Attack.

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    Eunju Cha

    Full Text Available Neuromyelitis optica (NMO is an inflammatory demyelinating disease of the central nervous system (CNS. Hydroxycholesterols (OHCs, metabolites of CNS cholesterol, are involved in diverse cellular responses to inflammation and demyelination, and may also be involved in the pathogenesis of NMO. We aimed to develop a sensitive and reliable method for the quantitative analysis of three major OHCs (24S-, 25-, and 27-OHCs, and to evaluate their concentration in the cerebrospinal fluid (CSF and serum of patients with NMO. The levels of the three OHCs in the serum and CSF were measured using liquid chromatography-silver ion coordination ionspray tandem mass spectrometry and liquid chromatography-electrospray ionization tandem mass spectrometry with picolinyl ester derivatization, respectively. The linear range was 5-250 ng/mL for 24S- and 27-OHC, and 0.5-25 ng/mL for 25-OHC in serum, and was 0.1-5 ng/mL for 24S- and 27-OHC, and 0.03-1 ng/mL for 25-OHC in CSF. Precision and accuracy were 0.5%-14.7% and 92.5%-109.7%, respectively, in serum, and were 0.8%-7.7% and 94.5%-119.2%, respectively, in CSF. Extraction recovery was 82.7%-90.7% in serum and 68.4%-105.0% in CSF. When analyzed in 26 NMO patients and 23 control patients, the 25-OHC (0.54 ± 0.96 ng/mL vs. 0.09 ± 0.04 ng/mL, p = 0.032 and 27-OHC (2.68 ± 3.18 ng/mL vs. 0.68 ± 0.25 ng/mL, p = 0.005 were increased in the CSF from NMO patients. When we measured the OHCCSF index that controls the effects of blood-brain barrier disruption on the level of OHC in the CSF, the 27-OHCCSF index was associated with disability (0.723; 95% confidence interval (CI- 0.181, 0.620; p = 0.002, while the 24-OHCCSF index (0.518; 95% CI- 1.070, 38.121; p = 0.040 and 25-OHCCSF index (0.677; 95% CI- 4.313, 18.532; p = 0.004 were associated with the number of white blood cells in the CSF of NMO patients. Our results imply that OHCs in the CNS could play a role in the pathogenesis of NMO.

  7. Hydroxycholesterol Levels in the Serum and Cerebrospinal Fluid of Patients with Neuromyelitis Optica Revealed by LC-Ag+CIS/MS/MS and LC-ESI/MS/MS with Picolinic Derivatization: Increased Levels and Association with Disability during Acute Attack

    Science.gov (United States)

    Park, Ki Duk; Park, Kyung Seok; Lee, Kwang-Woo; Kim, Sung-Min; Lee, Jaeick

    2016-01-01

    Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system (CNS). Hydroxycholesterols (OHCs), metabolites of CNS cholesterol, are involved in diverse cellular responses to inflammation and demyelination, and may also be involved in the pathogenesis of NMO. We aimed to develop a sensitive and reliable method for the quantitative analysis of three major OHCs (24S-, 25-, and 27-OHCs), and to evaluate their concentration in the cerebrospinal fluid (CSF) and serum of patients with NMO. The levels of the three OHCs in the serum and CSF were measured using liquid chromatography-silver ion coordination ionspray tandem mass spectrometry and liquid chromatography-electrospray ionization tandem mass spectrometry with picolinyl ester derivatization, respectively. The linear range was 5–250 ng/mL for 24S- and 27-OHC, and 0.5–25 ng/mL for 25-OHC in serum, and was 0.1–5 ng/mL for 24S- and 27-OHC, and 0.03–1 ng/mL for 25-OHC in CSF. Precision and accuracy were 0.5%–14.7% and 92.5%–109.7%, respectively, in serum, and were 0.8%–7.7% and 94.5%–119.2%, respectively, in CSF. Extraction recovery was 82.7%–90.7% in serum and 68.4%–105.0% in CSF. When analyzed in 26 NMO patients and 23 control patients, the 25-OHC (0.54 ± 0.96 ng/mL vs. 0.09 ± 0.04 ng/mL, p = 0.032) and 27-OHC (2.68 ± 3.18 ng/mL vs. 0.68 ± 0.25 ng/mL, p = 0.005) were increased in the CSF from NMO patients. When we measured the OHCCSF index that controls the effects of blood–brain barrier disruption on the level of OHC in the CSF, the 27-OHCCSF index was associated with disability (0.723; 95% confidence interval (CI)– 0.181, 0.620; p = 0.002), while the 24-OHCCSF index (0.518; 95% CI– 1.070, 38.121; p = 0.040) and 25-OHCCSF index (0.677; 95% CI– 4.313, 18.532; p = 0.004) were associated with the number of white blood cells in the CSF of NMO patients. Our results imply that OHCs in the CNS could play a role in the pathogenesis of NMO. PMID:27942009

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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%.

  11. Ion implantation

    International Nuclear Information System (INIS)

    Dearnaley, Geoffrey

    1975-01-01

    First, ion implantation in semiconductors is discussed: ion penetration, annealing of damage, gettering, ion implanted semiconductor devices, equipement requirements for ion implantation. The importance of channeling for ion implantation is studied. Then, some applications of ion implantation in metals are presented: study of the corrosion of metals and alloys; influence or ion implantation on the surface-friction and wear properties of metals; hyperfine interactions in implanted metals

  12. 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

    OpenAIRE

    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...

  13. 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.

  14. 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

  15. 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)

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  19. 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.

  20. 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.

  1. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  3. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

  6. Ion Colliders

    CERN Document Server

    Fischer, W

    2014-01-01

    High-energy ion colliders are large research tools in nuclear physics to study the Quark-Gluon-Plasma (QGP). The range of collision energy and high luminosity are important design and operational considerations. The experiments also expect flexibility with frequent changes in the collision energy, detector fields, and ion species. Ion species range from protons, including polarized protons in RHIC, to heavy nuclei like gold, lead and uranium. Asymmetric collision combinations (e.g. protons against heavy ions) are also essential. For the creation, acceleration, and storage of bright intense ion beams, limits are set by space charge, charge change, and intrabeam scattering effects, as well as beam losses due to a variety of other phenomena. Currently, there are two operating ion colliders, the Relativistic Heavy Ion Collider (RHIC) at BNL, and the Large Hadron Collider (LHC) at CERN.

  7. Recoil ions

    International Nuclear Information System (INIS)

    Cocke, C.L.; Olson, R.E.

    1991-01-01

    The collision of a fast moving heavy ion with a neutral atomic target can produce very highly charged but slowly moving target ions. This article reviews experimental and theoretical work on the production and use of recoil ions beyond the second ionization state by beams with specific energies above 0.5 MeV/amu. A brief historical survey is followed by a discussion of theoretical approaches to the problem of the removal of many electrons from a neutral target by a rapid, multiply charged projectile. A discussion of experimental techniques and results for total and differential cross sections for multiple ionization of atomic and molecular targets is given. Measurements of recoil energy are discussed. The uses of recoil ions for in situ spectroscopy of multiply charged ions, for external beams of slow, highly charged ions and in ion traps are reviewed. Some possible future opportunities are discussed. (orig.)

  8. Ion colliders

    International Nuclear Information System (INIS)

    Fischer, W.

    2010-01-01

    Ion colliders are research tools for high-energy nuclear physics, and are used to test the theory of Quantum Chromo Dynamics (QCD). The collisions of fully stripped high-energy ions create matter of a temperature and density that existed only microseconds after the Big Bang. Ion colliders can reach higher densities and temperatures than fixed target experiments although at a much lower luminosity. The first ion collider was the CERN Intersecting Storage Ring (ISR), which collided light ions (77Asb1, 81Bou1). The BNL Relativistic Heavy Ion Collider (RHIC) is in operation since 2000 and has collided a number of species at numerous energies. The CERN Large Hadron Collider (LHC) started the heavy ion program in 2010. Table 1 shows all previous and the currently planned running modes for ISR, RHIC, and LHC. All three machines also collide protons, which are spin-polarized in RHIC. Ion colliders differ from proton or antiproton colliders in a number of ways: the preparation of the ions in the source and the pre-injector chain is limited by other effects than for protons; frequent changes in the collision energy and particle species, including asymmetric species, are typical; and the interaction of ions with each other and accelerator components is different from protons, which has implications for collision products, collimation, the beam dump, and intercepting instrumentation devices such a profile monitors. In the preparation for the collider use the charge state Z of the ions is successively increased to minimize the effects of space charge, intrabeam scattering (IBS), charge change effects (electron capture and stripping), and ion-impact desorption after beam loss. Low charge states reduce space charge, intrabeam scattering, and electron capture effects. High charge states reduce electron stripping, and make bending and acceleration more effective. Electron stripping at higher energies is generally more efficient. Table 2 shows the charge states and energies in the

  9. Ion colliders

    Energy Technology Data Exchange (ETDEWEB)

    Fischer, W.

    2011-12-01

    Ion colliders are research tools for high-energy nuclear physics, and are used to test the theory of Quantum Chromo Dynamics (QCD). The collisions of fully stripped high-energy ions create matter of a temperature and density that existed only microseconds after the Big Bang. Ion colliders can reach higher densities and temperatures than fixed target experiments although at a much lower luminosity. The first ion collider was the CERN Intersecting Storage Ring (ISR), which collided light ions [77Asb1, 81Bou1]. The BNL Relativistic Heavy Ion Collider (RHIC) is in operation since 2000 and has collided a number of species at numerous energies. The CERN Large Hadron Collider (LHC) started the heavy ion program in 2010. Table 1 shows all previous and the currently planned running modes for ISR, RHIC, and LHC. All three machines also collide protons, which are spin-polarized in RHIC. Ion colliders differ from proton or antiproton colliders in a number of ways: the preparation of the ions in the source and the pre-injector chain is limited by other effects than for protons; frequent changes in the collision energy and particle species, including asymmetric species, are typical; and the interaction of ions with each other and accelerator components is different from protons, which has implications for collision products, collimation, the beam dump, and intercepting instrumentation devices such a profile monitors. In the preparation for the collider use the charge state Z of the ions is successively increased to minimize the effects of space charge, intrabeam scattering (IBS), charge change effects (electron capture and stripping), and ion-impact desorption after beam loss. Low charge states reduce space charge, intrabeam scattering, and electron capture effects. High charge states reduce electron stripping, and make bending and acceleration more effective. Electron stripping at higher energies is generally more efficient. Table 2 shows the charge states and energies in the

  10. 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.

  11. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  13. 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.

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

    Directory of Open Access Journals (Sweden)

    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%.

  15. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  17. 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

    Directory of Open Access Journals (Sweden)

    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

  18. ion irradiation

    Indian Academy of Sciences (India)

    Swift heavy ions interact predominantly through inelastic scattering while traversing any polymer medium and produce excited/ionized atoms. Here samples of the polycarbonate Makrofol of approximate thickness 20 m, spin coated on GaAs substrate were irradiated with 50 MeV Li ion (+3 charge state). Build-in ...

  19. Ion microprobes

    International Nuclear Information System (INIS)

    Coles, J.N.; Long, J.V.P.

    1977-01-01

    An ion microprobe is described that has an ion extraction arrangement comprising two separate paths for ions and electrons diverging from a common point. A cone shaped or pyramidal guard electrode surrounds each path the apex angles being equal and coinciding with the said point. The guard electrodes are positioned to lie tangentially to each other and to a planar surface including the said point. An aperture is provided for the two paths at the apexes of both guard electrodes, and electrical connections between the guard electrodes enable the same potential to be applied to both guard electrodes. Means are provided for generating oppositely polarised electric fields within the guard electrodes, together with means for causing a focused ion beam to strike the common point without suffering astigmatism. The means for causing a focused ion beam to strike the said point includes an ion gun for directing an ion beam along one of the paths and means to provide an axial accelerating field there along. Optical viewing means are also provided. Existing designs enable only ions or electrons, but not both, to be extracted at any one time. (U.K.)

  20. Ion implantation

    International Nuclear Information System (INIS)

    Johnson, E.

    1986-01-01

    It is the purpose of the present paper to give a review of surface alloy processing by ion implantation. However, rather than covering this vast subject as a whole, the survey is confined to a presentation of the microstructures that can be found in metal surfaces after ion implantation. The presentation is limited to alloys processed by ion implantation proper, that is to processes in which the alloy compositions are altered significantly by direct injection of the implanted ions. The review is introduced by a presentation of the processes taking place during development of the fundamental event in ion implantation - the collision cascade, followed by a summary of the various microstructures which can be formed after ion implantation into metals. This is compared with the variability of microstructures that can be achieved by rapid solidification processing. The microstructures are subsequently discussed in the light of the processes which, as the implantations proceed, take place during and immediately after formation of the individual collision cascades. These collision cascades define the volumes inside which individual ions are slowed down in the implanted targets. They are not only centres for vigorous agitation but also the sources for formation of excess concentrations of point defects, which will influence development of particular microstructures. A final section presents a selection of specific structures which have been observed in different alloy systems. (orig./GSCH)

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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

  3. 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

  4. 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

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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

  7. 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

  8. Ion channeling

    International Nuclear Information System (INIS)

    Erramli, H.; Blondiaux, G.

    1994-01-01

    Channeling phenomenon was predicted, many years ago, by stark. The first channeling experiments were performed in 1963 by Davies and his coworkers. Parallely Robinson and Oen have investigated this process by simulating trajectories of ions in monocrystals. This technique has been combined with many methods like Rutherford Backscattering Spectrometry (R.B.S.), Particles Induced X-rays Emission (P.I.X.E) and online Nuclear Reaction (N.R.A.) to localize trace elements in the crystal or to determine crystalline quality. To use channeling for material characterization we need data about the stopping power of the incident particle in the channeled direction. The ratios of channeled to random stopping powers of silicon for irradiation in the direction have been investigated and compared to the available theoretical results. We describe few applications of ion channeling in the field of materials characterization. Special attention is given to ion channeling combined with Charged Particle Activation Analysis (C.P.A.A.) for studying the behaviour of oxygen atoms in Czochralski silicon lattices under the influence of internal gettering and in different gaseous atmospheres. Association between ion channeling and C.P.A.A was also utilised for studying the influence of the growing conditions on concentration and position of carbon atoms at trace levels in the MOVPE Ga sub (1-x) Al sub x lattice. 6 figs., 1 tab., 32 refs. (author)

  9. Ion source

    International Nuclear Information System (INIS)

    1977-01-01

    The specifications of a set of point-shape electrodes of non-corrodable material that can hold a film of liquid material of equal thickness is described. Contained in a jacket, this set forms an ion source. The electrode is made of tungsten with a glassy carbon layer for insulation and an outer layer of aluminium-oxide ceramic material

  10. Ion-ion collisions and ion storage rings

    International Nuclear Information System (INIS)

    Mowat, J.R.

    1988-01-01

    Improved understanding of fundamental ion-ion interactions is expected to emerge from research carried out with ion storage rings. In this short survey the significant advantages and unique features that make stored ions useful targets for collision experiments are reviewed and discussed. It is pointed out that improvements to existing ion-ion experiments, as well as qualitatively new experiments, should occur over the next few years as ion storage rings become available for atomic physics. Some new experiments are suggested which are difficult if not impossible with present-day technology, but which seem feasible at storage rings facilities. (orig.)

  11. Ion Beam Extraction by Discrete Ion Focusing

    DEFF Research Database (Denmark)

    2010-01-01

    An apparatus (900) and methods are disclosed for ion beam extraction. In an implementation, the apparatus includes a plasma source (or plasma) (802) and an ion extractor (804). The plasma source is adapted to generate ions and the ion extractor is immersed in the plasma source to extract a fracti...

  12. 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

    Directory of Open Access Journals (Sweden)

    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

  13. ION GUN

    Science.gov (United States)

    Dandl, R.A.

    1961-10-24

    An ion gun is described for the production of an electrically neutral ionized plasma. The ion gun comprises an anode and a cathode mounted in concentric relationship with a narrow annulus between. The facing surfaces of the rear portions of the anode and cathode are recessed to form an annular manifold. Positioned within this manifold is an annular intermediate electrode aligned with the an nulus between the anode and cathode. Gas is fed to the manifold and an arc discharge is established between the anode and cathode. The gas is then withdrawn from the manifold through the annulus between the anode and cathode by a pressure differential. The gas is then ionized by the arc discharge across the annulus. The ionized gas is withdrawn from the annulus by the combined effects of the pressure differential and a collimating magnetic field. In a 3000 gauss magnetic field, an arc voltage of 1800 volts, and an arc current of 0.2 amp, a plasma of about 3 x 10/sup 11/ particles/cc is obtained. (AEC)

  14. Heavy ions

    CERN Multimedia

    CERN. Geneva; Antinori, Federico

    2001-01-01

    Colliding two heavy nuclei at ultrarelativistic energies allows to create in the laboratory a bulk system with huge density, pressure and temperature and to study its properties. It is estimated that in Pb-Pb collisions at CERN-SPS we reach over an appreciable volume an energy density which exceeds by more than a factor 20 that of normal nuclear matter. At such densities, the hadrons are so closely packed that they interpenetrate; novel physics phenomena are expected to appear. QCD predicts that under such conditions a phase transition from a system composed of colourless hadrons to a Quark-Gluon Plasma (QGP) should occur. A rich ultrarelativistic heavy-ion physics programme is under way both at BNL-AGS and at CERN-SPS since 1986. The results obtained so far have led CERN to officially announce evidence for a new state of matter last year. A long-range programme of heavy-ion physics at higher energies is under way (BNL-RHIC) and in preparation (CERN-LHC). These lectures are meant as an introduction to the phy...

  15. Heavy ions

    CERN Multimedia

    CERN. Geneva. Audiovisual Unit

    2002-01-01

    Colliding two heavy nuclei at ultrarelativistic energies allows to create in the laboratory a bulk system with huge density, pressure and temperature and to study its properties. It is estimated that in Pb-Pb collisions at CERN-SPS we reach over an appreciable volume an energy density which exceeds by more than a factor 20 that of normal nuclear matter. At such densities, the hadrons are so closely packed that they interpenetrate; novel physics phenomena are expected to appear. QCD predicts that under such conditions a phase transition from a system composed of colourless hadrons to a Quark-Gluon Plasma (QGP) should occur. A rich ultrarelativistic heavy-ion physics programme is under way both at BNL-AGS and at CERN-SPS since 1986. The results obtained so far have led CERN to officially announce evidence for a new state of matter last year. A long-range programme of heavy-ion physics at higher energies is under way (BNL-RHIC) and in preparation (CERN-LHC). These lectures are meant as an introduction to the phy...

  16. 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.

  17. 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.

  18. Ion beam monitoring

    International Nuclear Information System (INIS)

    McKinney, C.R.

    1980-01-01

    An ion beam analyzer is specified, having an ion source for generating ions of a sample to be analyzed, means for extracting the sample ions, means for focusing the sample ions into a beam, separation means positioned along the ion beam for selectively deflecting species of ions, and means for detecting the selected species of ions. According to the specification, the analyzer further comprises (a) means for disabling at least a portion of the separation means, such that the ion beam from the source remains undeflected; (b) means located along the path of the undeflected ion beam for sensing the sample ions; and (c) enabling means responsive to the sensing means for automatically re-enabling the separation means when the sample ions reach a predetermined intensity level. (author)

  19. Recoil ion spectroscopy with heavy ions

    International Nuclear Information System (INIS)

    Beyer, H.F.; Mann, R.

    1984-01-01

    This chapter examines the production of very high charge state ions in single ion-atom collisions. Topics considered include some aspects of highly ionized atoms, experimental approaches, the production of highly charged target ions (monoatomic targets, recoil energy distribution, molecular fragmentation, outer-shell rearrangement, lifetime measurements, a comparison of projectile-, target-, and plasma-ion stripping), and secondary collision experiments (selective electron capture, potential applications). The heavy-ion beams for the described experiments were provided by accelerators such as tandem Van de Graaff facility and the UNILAC

  20. Surface negative ion production in ion sources

    International Nuclear Information System (INIS)

    Belchenko, Y.

    1993-01-01

    Negative ion sources and the mechanisms for negative ion production are reviewed. Several classes of sources with surface origin of negative ions are examined in detail: surface-plasma sources where ion production occurs on the electrode in contact with the plasma, and ''pure surface'' sources where ion production occurs due to conversion or desorption processes. Negative ion production by backscattering, impact desorption, and electron- and photo-stimulated desorption are discussed. The experimental efficiencies of intense surface negative ion production realized on electrodes contacted with hydrogen-cesium or pure hydrogen gas-discharge plasma are compared. Recent modifications of surface-plasma sources developed for accelerator and fusion applications are reviewed in detail

  1. Gas phase ion chemistry

    CERN Document Server

    Bowers, Michael T

    1979-01-01

    Gas Phase Ion Chemistry, Volume 1 covers papers on the advances of gas phase ion chemistry. The book discusses the advances in flow tubes and the measurement of ion-molecule rate coefficients and product distributions; the ion chemistry of the earth's atmosphere; and the classical ion-molecule collision theory. The text also describes statistical methods in reaction dynamics; the state selection by photoion-photoelectron coincidence; and the effects of temperature and pressure in the kinetics of ion-molecule reactions. The energy distribution in the unimolecular decomposition of ions, as well

  2. Laser ion sources

    Energy Technology Data Exchange (ETDEWEB)

    Bykovskij, Yu

    1979-02-01

    The characteristics a laser source of multiply-ionized ions are described with regard to the interaction of laser radiation and matter, ion energy spectrum, angular ion distribution. The amount of multiple-ionization ions is evaluated. Out of laser source applications a laser injector of multiple-ionization ions and nuclei, laser mass spectrometry, laser X-ray microradiography, and a laser neutron generators are described.

  3. Ion Beam Propulsion Study

    Science.gov (United States)

    2008-01-01

    The Ion Beam Propulsion Study was a joint high-level study between the Applied Physics Laboratory operated by NASA and ASRC Aerospace at Kennedy Space Center, Florida, and Berkeley Scientific, Berkeley, California. The results were promising and suggested that work should continue if future funding becomes available. The application of ion thrusters for spacecraft propulsion is limited to quite modest ion sources with similarly modest ion beam parameters because of the mass penalty associated with the ion source and its power supply system. Also, the ion source technology has not been able to provide very high-power ion beams. Small ion beam propulsion systems were used with considerable success. Ion propulsion systems brought into practice use an onboard ion source to form an energetic ion beam, typically Xe+ ions, as the propellant. Such systems were used for steering and correction of telecommunication satellites and as the main thruster for the Deep Space 1 demonstration mission. In recent years, "giant" ion sources were developed for the controlled-fusion research effort worldwide, with beam parameters many orders of magnitude greater than the tiny ones of conventional space thruster application. The advent of such huge ion beam sources and the need for advanced propulsion systems for exploration of the solar system suggest a fresh look at ion beam propulsion, now with the giant fusion sources in mind.

  4. Negative ion sources

    International Nuclear Information System (INIS)

    Ishikawa, Junzo; Takagi, Toshinori

    1983-01-01

    Negative ion sources have been originally developed at the request of tandem electrostatic accelerators, and hundreds of nA to several μA negative ion current has been obtained so far for various elements. Recently, the development of large current hydrogen negative ion sources has been demanded from the standpoint of the heating by neutral particle beam injection in nuclear fusion reactors. On the other hand, the physical properties of negative ions are interesting in the thin film formation using ions. Anyway, it is the present status that the mechanism of negative ion action has not been so fully investigated as positive ions because the history of negative ion sources is short. In this report, the many mechanisms about the generation of negative ions proposed so far are described about negative ion generating mechanism, negative ion source plasma, and negative ion generation on metal surfaces. As a result, negative ion sources are roughly divided into two schemes, plasma extraction and secondary ion extraction, and the former is further classified into the PIG ion source and its variation and Duoplasmatron and its variation; while the latter into reflecting and sputtering types. In the second half of the report, the practical negative ion sources of each scheme are described. If the mechanism of negative ion generation will be investigated more in detail and the development will be continued under the unified know-how as negative ion sources in future, the development of negative ion sources with which large current can be obtained for any element is expected. (Wakatsuki, Y.)

  5. 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

    Directory of Open Access Journals (Sweden)

    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

  6. Ion sources for heavy ion fusion

    International Nuclear Information System (INIS)

    Yu, S.S.; Eylon, S.; Chupp, W.

    1995-09-01

    The development of ion sources for heavy ion fusion will be reported with particular emphasis on a recently built 2 MV injector. The new injector is based on an electrostatic quadrupole configuration, and has produced pulsed K + ions of 950 mA peak from a 6.7 inch curved alumino silicate source. The ion beam has reached 2.3 MV with an energy flatness of ±0.2% over 1 micros. The measured normalized edge emittance of less than 1 π mm-mr is close to the source temperature limit. The design, construction, performance, and comparisons with three-dimensional particle-in-cell simulations will be described

  7. [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.

  8. Electron Beam Ion Sources

    CERN Document Server

    Zschornacka, G.; Thorn, A.

    2013-12-16

    Electron beam ion sources (EBISs) are ion sources that work based on the principle of electron impact ionization, allowing the production of very highly charged ions. The ions produced can be extracted as a DC ion beam as well as ion pulses of different time structures. In comparison to most of the other known ion sources, EBISs feature ion beams with very good beam emittances and a low energy spread. Furthermore, EBISs are excellent sources of photons (X-rays, ultraviolet, extreme ultraviolet, visible light) from highly charged ions. This chapter gives an overview of EBIS physics, the principle of operation, and the known technical solutions. Using examples, the performance of EBISs as well as their applications in various fields of basic research, technology and medicine are discussed.

  9. Ions and light

    CERN Document Server

    Bowers, Michael T

    2013-01-01

    Gas Phase Ion Chemistry, Volume 3: Ions and Light discusses how ions are formed by electron impact, ion-molecule reactions, or electrical discharge. This book discusses the use of light emitted by excited molecules to characterize either the chemistry that formed the excited ion, the structure of the excited ion, or both.Organized into 10 chapters, this volume begins with an overview of the extension of the classical flowing afterglow technique to include infrared and chemiluminescence and laser-induced fluorescence detection. This text then examines the experiments involving molecules that ar

  10. Negative-ion states

    International Nuclear Information System (INIS)

    Compton, R.N.

    1982-01-01

    In this brief review, we discuss some of the properties of atomic and molecular negative ions and their excited states. Experiments involving photon reactions with negative ions and polar dissociation are summarized. 116 references, 14 figures

  11. Quadrupole Ion Traps

    Indian Academy of Sciences (India)

    to do precision spectroscopic measurements on these ions. ... Bonn, investigated the non-magnetic quadrupole mass filter, .... the details of which will be discussed in the subse- ... the radial plane the ion undergoes a circular motion with the.

  12. Negative ion detachment processes

    International Nuclear Information System (INIS)

    Champion, R.L.; Doverspike, L.D.

    1990-10-01

    This paper discusses the following topics: H - and D - collisions with atomic hydrogen; collisional decomposition of SF 6 - ; two-electron loss processes in negative ion collisions; associative electron detachment; and negative ion desorption from surfaces

  13. Ion sources in AMS

    International Nuclear Information System (INIS)

    Iyer, Indira S.

    1997-01-01

    Accelerator Mass Spectrometry (AMS) entails the sputtering of various samples in an ion source followed by high precision mass analysis of the sputtered ion species in a Tandem Electrostatic Accelerator. A brief account is given

  14. Single Cathode Ion Thruster

    Data.gov (United States)

    National Aeronautics and Space Administration — Objective is to design an electrostatic ion thruster that is more efficient, simpler, and lower cost than the current gridded ion thruster. Initial objective is to...

  15. Applications of decelerated ions

    International Nuclear Information System (INIS)

    Johnson, B.M.

    1985-03-01

    Many facilities whose sole purpose had been to accelerate ion beams are now becoming decelerators as well. The development and current status of accel-decel operations is reviewed here. Applications of decelerated ions in atomic physics experiments are discussed

  16. Fundamentals of ion exchange

    International Nuclear Information System (INIS)

    Townsend, R.P.

    1993-01-01

    In this paper the fundamentals of ion exchange mechanisms and their thermodynamics are described. A range of ion exchange materials is considered and problems of communication and technology transfer between scientists working in the field are discussed. (UK)

  17. Gas phase ion chemistry

    CERN Document Server

    Bowers, Michael T

    1979-01-01

    Gas Phase Ion Chemistry, Volume 2 covers the advances in gas phase ion chemistry. The book discusses the stabilities of positive ions from equilibrium gas-phase basicity measurements; the experimental methods used to determine molecular electron affinities, specifically photoelectron spectroscopy, photodetachment spectroscopy, charge transfer, and collisional ionization; and the gas-phase acidity scale. The text also describes the basis of the technique of chemical ionization mass spectrometry; the energetics and mechanisms of unimolecular reactions of positive ions; and the photodissociation

  18. Ion mobility spectrometry

    CERN Document Server

    Eiceman, GA

    2005-01-01

    Key Developments for Faster, More Precise Detection Capabilities Driven by the demand for the rapid and advanced detection of explosives, chemical and biological warfare agents, and narcotics, ion mobility spectrometry (IMS) undergone significant refinements in technology, computational capabilities, and understanding of the principles of gas phase ion chemistry and mobility. Beginning with a thorough discussion of the fundamental theories and physics of ion mobility, Ion Mobility Spectrometry, Second Edition describes the recent advances in instrumentation and newly

  19. Intense ion beam generator

    International Nuclear Information System (INIS)

    Humphries, S. Jr.; Sudan, R.N.

    1977-01-01

    Methods and apparatus for producing intense megavolt ion beams are disclosed. In one embodiment, a reflex triode-type pulsed ion accelerator is described which produces ion pulses of more than 5 kiloamperes current with a peak energy of 3 MeV. In other embodiments, the device is constructed so as to focus the beam of ions for high concentration and ease of extraction, and magnetic insulation is provided to increase the efficiency of operation

  20. Ion sources for accelerators

    International Nuclear Information System (INIS)

    Alton, G.D.

    1974-01-01

    A limited review of low charge sate positive and negative ion sources suitable for accelerator use is given. A brief discussion is also given of the concepts underlying the formation and extraction of ion beams. Particular emphasis is placed on the technology of ion sources which use solid elemental or molecular compounds to produce vapor for the ionization process

  1. Negative Ion Density Fronts

    International Nuclear Information System (INIS)

    Igor Kaganovich

    2000-01-01

    Negative ions tend to stratify in electronegative plasmas with hot electrons (electron temperature Te much larger than ion temperature Ti, Te > Ti ). The boundary separating a plasma containing negative ions, and a plasma, without negative ions, is usually thin, so that the negative ion density falls rapidly to zero-forming a negative ion density front. We review theoretical, experimental and numerical results giving the spatio-temporal evolution of negative ion density fronts during plasma ignition, the steady state, and extinction (afterglow). During plasma ignition, negative ion fronts are the result of the break of smooth plasma density profiles during nonlinear convection. In a steady-state plasma, the fronts are boundary layers with steepening of ion density profiles due to nonlinear convection also. But during plasma extinction, the ion fronts are of a completely different nature. Negative ions diffuse freely in the plasma core (no convection), whereas the negative ion front propagates towards the chamber walls with a nearly constant velocity. The concept of fronts turns out to be very effective in analysis of plasma density profile evolution in strongly non-isothermal plasmas

  2. Nanostructures by ion beams

    Science.gov (United States)

    Schmidt, B.

    Ion beam techniques, including conventional broad beam ion implantation, ion beam synthesis and ion irradiation of thin layers, as well as local ion implantation with fine-focused ion beams have been applied in different fields of micro- and nanotechnology. The ion beam synthesis of nanoparticles in high-dose ion-implanted solids is explained as phase separation of nanostructures from a super-saturated solid state through precipitation and Ostwald ripening during subsequent thermal treatment of the ion-implanted samples. A special topic will be addressed to self-organization processes of nanoparticles during ion irradiation of flat and curved solid-state interfaces. As an example of silicon nanocrystal application, the fabrication of silicon nanocrystal non-volatile memories will be described. Finally, the fabrication possibilities of nanostructures, such as nanowires and chains of nanoparticles (e.g. CoSi2), by ion beam synthesis using a focused Co+ ion beam will be demonstrated and possible applications will be mentioned.

  3. Enhanced ion acoustic fluctuations and ion outflows

    Directory of Open Access Journals (Sweden)

    F. R. E. Forme

    1999-02-01

    Full Text Available A number of observations showing enhanced ion acoustic echoes observed by means of incoherent scatter radars have been reported in the literature. The received power is extremely enhanced by up to 1 or 2 orders of magnitude above usual values, and it is mostly contained in one of the two ion acoustic lines. This spectral asymmetry and the intensity of the received signal cannot be resolved by the standard analysis procedure and often causes its failure. As a result, and in spite of a very clear spectral signature, the analysis is unable to fit the plasma parameters inside the regions of ion acoustic turbulence. We present European Incoherent Scatter radar (EISCAT observations of large ion outflows associated with the simultaneous occurrence of enhanced ion acoustic echoes. The ion fluxes can reach 1014 m-2 s-1 at 800 km altitude. From the very clear spectral signatures of these echoes, a method is presented to extract estimates of the electron temperature and the ion drift within the turbulent regions. It is shown that the electron gas is strongly heated up to 11 000 K. Also electron temperature gradients of about 0.02 K/m exist. Finally, the estimates of the electron temperature and of the ion drift are used to study the possible implications for the plasma transport inside turbulent regions. It is shown that strong electron temperature gradients cause enhancement of the ambipolar electric field and can account for the observed ion outflows.Key words. Ionosphere (auroral ionosphere; ionosphere · magnetosphere interactions; plasma waves and instabilities.

  4. Vacuum Arc Ion Sources

    CERN Document Server

    Brown, I.

    2013-12-16

    The vacuum arc ion source has evolved into a more or less standard laboratory tool for the production of high-current beams of metal ions, and is now used in a number of different embodiments at many laboratories around the world. Applications include primarily ion implantation for material surface modification research, and good performance has been obtained for the injection of high-current beams of heavy-metal ions, in particular uranium, into particle accelerators. As the use of the source has grown, so also have the operational characteristics been improved in a variety of different ways. Here we review the principles, design, and performance of vacuum arc ion sources.

  5. Heavy-ion radiography

    International Nuclear Information System (INIS)

    Fabrikant, J.I.; Tobias, C.A.; Holley, W.R.; Benton, E.V.; Woodruff, K.H.; MacFarland, E.W.

    1983-01-01

    High energy, heavy-ion beams offer superior discrimination of tissue electron densities at very low radiation doses. This characteristic has potential for diagnostic medical imaging of neoplasms arising in the soft tissues and organs because it can detect smaller inhomogeneities than x rays. Heavy-ion imaging may also increase the accuracy of cancer radiotherapy planning involving use of accelerated charged particles. In the current physics research program of passive heavy-ion imaging, critical modulation transfer function tests are being carried out in heavy-ion projection radiography and heavy-ion computerized tomography. The research goal is to improve the heavy-ion imaging method until it reaches the limits of its theoretical resolution defined by range straggling, multiple scattering, and other factors involved in the beam quality characteristics. Clinical uses of the imaging method include the application of heavy-ion computerized tomography to heavy-ion radiotherapy planning, to the study of brain tumors and other structures of the head, and to low-dose heavy-ion projection mammography, particularly for women with dense breasts where other methods of diagnosis fail. The ions used are primarily 300 to 570 MeV/amu carbon and neon ions accelerated at the Lawrence Berkeley Laboratory Bevalac

  6. Ion implantation into iron

    International Nuclear Information System (INIS)

    Iwaki, Masaya

    1978-01-01

    The distribution of implanted ions in iron, the friction characteristics and the corrosion of iron were studied. The distribution of Ni or Cr ions implanted into mild steel was measured. The accelerated voltage was 150 keV, and the beam current density was about 2 microampere/cm 2 . The measurement was made with an ion microanalyzer. The measured distribution was compared with that of LSS theory. Deep invasion of Ni was seen in the measured distribution. The distribution of Cr ions was different from the distribution calculated by the LSS theory. The relative friction coefficient of mild steel varied according to the dose of implanted Cu or N ions, and to the accelerating voltage. Formation of compound metals on the surfaces of metals by ion-implantation was investigated for the purpose to prevent the corrosion of metals. The resistance of mild steel in which Ni ions were implanted was larger than that of mild steel without any treatment. (Kato, T.)

  7. Crater formation by single ions, cluster ions and ion "showers"

    CERN Document Server

    Djurabekova, Flyura; Timko, Helga; Nordlund, Kai; Calatroni, Sergio; Taborelli, Mauro; Wuensch, Walter

    2011-01-01

    The various craters formed by giant objects, macroscopic collisions and nanoscale impacts exhibit an intriguing resemblance in shapes. At the same time, the arc plasma built up in the presence of sufficiently high electric fields at close look causes very similar damage on the surfaces. Although the plasma–wall interaction is far from a single heavy ion impact over dense metal surfaces or the one of a cluster ion, the craters seen on metal surfaces after a plasma discharge make it possible to link this event to the known mechanisms of the crater formations. During the plasma discharge in a high electric field the surface is subject to high fluxes (~1025 cm-2s-1) of ions with roughly equal energies typically of the order of a few keV. To simulate such a process it is possible to use a cloud of ions of the same energy. In the present work we follow the effect of such a flux of ions impinging the surface in the ‘‘shower’’ manner, to find the transition between the different mechanisms of crater formati...

  8. Optical effects of ion implantation

    International Nuclear Information System (INIS)

    Townsend, P.D.

    1987-01-01

    The review concerns the effects of ion implantation that specifically relate to the optical properties of insulators. Topics which are reviewed include: ion implantation, ion range and damage distributions, colour centre production by ion implantation, high dose ion implantation, and applications for integrated optics. Numerous examples are presented of both diagnostic and industrial examples of ion implantation effects in insulators. (U.K.)

  9. Ion beam stabilization in ion implantation equipment

    International Nuclear Information System (INIS)

    Pina, L.

    1973-01-01

    The results are presented of experimental efforts aimed at ion beam current stabilization in an equipment for ion implantation in solids. The related problems of power supplies are discussed. Measured characteristics of laboratory equipment served the determination of the parameters to be required of the supplies as well as the design and the construction of the supplies. The respective wiring diagram is presented. (J.K.)

  10. Calculation of ion storage in electron beams with account of ion-ion interactions

    International Nuclear Information System (INIS)

    Perel'shtejn, Eh.A.; Shirkov, G.D.

    1979-01-01

    Ion storage in relativistic electron beams was calculated taking account of ion-ion charge exchange and ionization. The calculations were made for nitrogen ion storage from residual gas during the compression of electron rings in the adhezator of the JINR heavy ion accelerator. The calculations were made for rings of various parameters and for various pressures of the residual gas. The results are compared with analogous calculations made without account of ion-ion processes. It is shown that at heavy loading of a ring by ions ion-ion collisions play a significant part, and they should be taken into account while calculating ion storage

  11. 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

  12. Duopigatron ion source studies

    International Nuclear Information System (INIS)

    Bacon, F.M.; Bickes, R.W. Jr.; O'Hagan, J.B.

    1978-07-01

    Ion source performance characteristics consisting of total ion current, ion energy distribution, mass distribution, and ion current density distribution were measured for several models of a duopigatron. Variations on the duopigatron design involved plasma expansion cup material and dimensions, secondary cathode material, and interelectrode spacings. Of the designs tested, the one with a copper and molybdenum secondary cathode and a mild steel plasma expansion cup proved to give the best results. The ion current density distribution was peaked at the center of the plasma expansion cup and fell off to 80 percent of the peak value at the cup wall for a cup 15.2 mm deep. A total ion current of 180 mA consisting of 60 to 70 percent atomic ions was produced with an arc current of 20 A and source pressure of 9.3 Pa. More shallow cups produced a larger beam current and a more sharply peaked ion current density distribution. Typical ion energy distributions were bell-shaped curves with a peak 10 to 20 V below anode potential and with ion energies extending 30 to 40 V on either side of the peak

  13. Quantitative ion implantation

    International Nuclear Information System (INIS)

    Gries, W.H.

    1976-06-01

    This is a report of the study of the implantation of heavy ions at medium keV-energies into electrically conducting mono-elemental solids, at ion doses too small to cause significant loss of the implanted ions by resputtering. The study has been undertaken to investigate the possibility of accurate portioning of matter in submicrogram quantities, with some specific applications in mind. The problem is extensively investigated both on a theoretical level and in practice. A mathematical model is developed for calculating the loss of implanted ions by resputtering as a function of the implanted ion dose and the sputtering yield. Numerical data are produced therefrom which permit a good order-of-magnitude estimate of the loss for any ion/solid combination in which the ions are heavier than the solid atoms, and for any ion energy from 10 to 300 keV. The implanted ion dose is measured by integration of the ion beam current, and equipment and techniques are described which make possible the accurate integration of an ion current in an electromagnetic isotope separator. The methods are applied to two sample cases, one being a stable isotope, the other a radioisotope. In both cases independent methods are used to show that the implantation is indeed quantitative, as predicted. At the same time the sample cases are used to demonstrate two possible applications for quantitative ion implantation, viz. firstly for the manufacture of calibration standards for instrumental micromethods of elemental trace analysis in metals, and secondly for the determination of the half-lives of long-lived radioisotopes by a specific activity method. It is concluded that the present study has advanced quantitative ion implantation to the state where it can be successfully applied to the solution of problems in other fields

  14. Heating tokamaks via the ion-cyclotron and ion-ion hybrid resonances

    International Nuclear Information System (INIS)

    Perkins, F.W.

    1977-04-01

    For the ion-ion hybrid resonance it is shown that: (1) the energy absorption occurs via a sequence of mode conversions; (2) a poloidal field component normal to the ion-ion hybrid mode conversion surface strongly influences the mode conversion process so that roughly equal electron and ion heating occurs in the present proton-deuterium experiments, while solely electron heating is predicted to prevail in deuterium-tritium reactors; (3) the ion-ion hybrid resonance suppresses toroidal eigenmodes; and (4) wave absorption in minority fundamental ion-cyclotron heating experiments will be dominated by ion-ion hybrid mode conversion absorption for minority concentrations exceeding roughly 1 percent. For the ion-cyclotron resonance, it is shown that: (1) ion-cyclotron mode conversion leads to surface electron heating; and (2) ion-cyclotron mode conversion absorption dominates fundamental ion-cyclotron absorption thereby preventing efficient ion heating

  15. Optical and electrical properties of thin films of bismuth ferric oxide; Propiedades opticas y electricas de peliculas delgadas de oxido de bismuto ferrico

    Energy Technology Data Exchange (ETDEWEB)

    Cardona R, D.

    2014-07-01

    The bismuth ferric oxide (BFO) has caused great attention in recent years because of their multi ferric properties, making it very attractive for different technological applications. In this paper simultaneous ablation of two white (Bi and Fe{sub 2}O{sub 3}) was used in a reactive atmosphere (containing oxygen) to deposit thin films of BFO. The composition of the films is changed by controlling the plasma parameters such as the average kinetic energy of the ions (E p) and the plasma density (Np). The effects caused by excess of Bi and Fe in atomic structure and the optical and electrical properties of the films BiFeO{sub 3} in terms of plasma parameters were studied. The X-ray diffraction patterns of BFO samples with excess of bismuth above 2% at. They exhibited small changes in structure leading to improved levels of leakage currents compared to levels of the film with a stoichiometry close to BiFeO{sub 3} composition. These samples showed a secondary phase (Bi{sub 2}5FeO{sub 4}0 selenite type) that led to the increase in the values of band gap and resistivity as well as the improvement of the piezoelectric properties. On the other hand, the films with iron excess showed as secondary phase compounds of iron oxide (α - γ-Fe{sub 2}O{sub 3}) that caused increments in the conductivity and decrease in the values of band gap. The results are discussed in terms of the excesses of Bi and Fe which were correlated with the plasma parameters. (Author)

  16. Atmospheric ions and pollution

    International Nuclear Information System (INIS)

    Renoux, A.

    1977-01-01

    The various types of atmospheric ions are defined, the main sources of natural atmospheric radioactivity inducing the formation of radioactive ions in the air are then recalled. The basic equations governing the formation of these ions are indicated and the most current experimental methods used for detecting them are described (Zeleny tubes, Erikson tubes). The special properties of these ions are examined, they are particularly emphasized for the smaller ones. The existence of a discret spectrum of mobilities is shown and the presence of big negative radioactive ions is investigated. Indicative information are given on the granulometric distribution of the atmospheric radioactivity in the air, from small positive Ra A ion fixation on aerosols [fr

  17. Materials analysis fast ions

    CERN Document Server

    Denker, A; Rauschenberg, J; Röhrich, J; Strub, E

    2006-01-01

    Materials analysis with ion beams exploits the interaction of ions with the electrons and nuclei in the sample. Among the vast variety of possible analytical techniques available with ion beams we will restrain to ion beam analysis with ion beams in the energy range from one to several MeV per mass unit. It is possible to use either the back-scattered projectiles (RBS – Rutherford Back Scattering) or the recoiled atoms itself (ERDA – Elastic Recoil Detection Analysis) from the elastic scattering processes. These techniques allow the simultaneous and absolute determination of stoichiometry and depth profiles of the detected elements. The interaction of the ions with the electrons in the sample produces holes in the inner electronic shells of the sample atoms, which recombine and emit X-rays characteristic for the element in question. Particle Induced X-ray Emission (PIXE) has shown to be a fast technique for the analysis of elements with an atomic number above 11.

  18. Ion thruster performance model

    International Nuclear Information System (INIS)

    Brophy, J.R.

    1984-01-01

    A model of ion thruster performance is developed for high flux density cusped magnetic field thruster designs. This model is formulated in terms of the average energy required to produce an ion in the discharge chamber plasma and the fraction of these ions that are extracted to form the beam. The direct loss of high energy (primary) electrons from the plasma to the anode is shown to have a major effect on thruster performance. The model provides simple algebraic equations enabling one to calculate the beam ion energy cost, the average discharge chamber plasma ion energy cost, the primary electron density, the primary-to-Maxwellian electron density ratio and the Maxwellian electron temperature. Experiments indicate that the model correctly predicts the variation in plasma ion energy cost for changes in propellant gas (Ar, Kr, and Xe), grid transparency to neutral atoms, beam extraction area, discharge voltage, and discharge chamber wall temperature

  19. Ion emission microscope microanalyzer

    International Nuclear Information System (INIS)

    Cherepin, V.T.; Olckovsky, V.L.

    1977-01-01

    In the ion microanalyzer (microprobe) the object is exposed to the bombardment of a highly focused ion beam, the secondary ions emitted from the object being analyzed by means of a mass filter. In order to be able to control the position of an analysis synchronous to the local analysis of an object an ion-optical converter (electron image with a fluorescent screen) is placed behind the aperture diaphragm in the direction of the secondary ion beam. The converter allows to make visible in front of the mass filter a non-split ion image characterizing the surface of the surface investigated. Then a certain section may be selected for performing chemical and isotope analyses. (DG) [de

  20. Outlook for ion exchange

    International Nuclear Information System (INIS)

    Kunin, R.

    1977-01-01

    This paper presents the history and theory of ion exchange technology and discusses the usefulness of ion exchange resins which found broad applications in chemical operations. It is demonstrated that the theory of ion exchange technology seems to be moving away from the physical chemist back to the polymer chemist where it started originally. This but confronted the polymer chemists with some knotty problems. It is pointed out that one has still to learn how to use ion exchange materials as efficiently as possible in terms of the waste load that is being pumped into the environment. It is interesting to note that, whereas ion exchange is used for abating pollution, it is also a polluter. One must learn how to use ion exchange as an antipollution device, and at the same time minimize its polluting properties

  1. HEAVY ION LINEAR ACCELERATOR

    Science.gov (United States)

    Van Atta, C.M.; Beringer, R.; Smith, L.

    1959-01-01

    A linear accelerator of heavy ions is described. The basic contributions of the invention consist of a method and apparatus for obtaining high energy particles of an element with an increased charge-to-mass ratio. The method comprises the steps of ionizing the atoms of an element, accelerating the resultant ions to an energy substantially equal to one Mev per nucleon, stripping orbital electrons from the accelerated ions by passing the ions through a curtain of elemental vapor disposed transversely of the path of the ions to provide a second charge-to-mass ratio, and finally accelerating the resultant stripped ions to a final energy of at least ten Mev per nucleon.

  2. Electron-ion collisions

    International Nuclear Information System (INIS)

    Crandall, D.H.

    1982-01-01

    This discussion concentrates on basic physics aspects of inelastic processes of excitation, ionization, and recombination that occur during electron-ion collisions. Except for cases of illustration along isoelectronic sequences, only multicharged (at least +2) ions will be specifically discussed with some emphasis of unique physics aspects associated with ionic charge. The material presented will be discussed from a primarily experimental viewpoint with most attention to electron-ion interacting beams experiments

  3. Ion implantation in semiconductors

    International Nuclear Information System (INIS)

    Gusev, V.; Gusevova, M.

    1980-01-01

    The historical development is described of the method of ion implantation, the physical research of the method, its technological solution and practical uses. The method is universally applicable, allows the implantation of arbitrary atoms to an arbitrary material, ensures high purity of the doping element. It is linked with sample processing at low temperatures. In implantation it is possible to independently change the dose and energy of the ions thereby affecting the spatial distribution of the ions. (M.S.)

  4. Ion implantation in semiconductors

    Energy Technology Data Exchange (ETDEWEB)

    Gusev, V; Gusevova, M

    1980-06-01

    The historical development of the method of ion implantation, the physical research of the method, its technological solution and practical uses is described. The method is universally applicable, allows the implantation of arbitrary atoms to an arbitrary material and ensures high purity of the doping element. It is linked with sample processing at low temperatures. In implantation it is possible to independently change the dose and energy of the ions thereby affecting the spatial distribution of the ions.

  5. Ion implantation in metals

    International Nuclear Information System (INIS)

    Vook, F.L.

    1977-02-01

    The application of ion beams to metals is rapidly emerging as a promising area of research and technology. This report briefly describes some of the recent advances in the modification and study of the basic properties of metals by ion implantation techniques. Most of the research discussed illustrates some of the new and exciting applications of ion beams to metals which are under active investigation at Sandia Laboratories, Albuquerque

  6. Heavy-ion targets

    International Nuclear Information System (INIS)

    Adair, H.L.; Kobisk, E.H.

    1985-01-01

    This chapter examines the characteristics of targets required in heavy-ion accelerator physics experiments. The effects of target parameters on heavy-ion experimental results are reviewed. The target fabrication and characterization techniques used to minimize experimental problems during heavy-ion bombardment are described. Topics considered include target thickness and uniformity, target lifetime, target purity, substrate materials, Doppler shift effects, metal preparations, and target preparation methods

  7. Cluster ion beam facilities

    International Nuclear Information System (INIS)

    Popok, V.N.; Prasalovich, S.V.; Odzhaev, V.B.; Campbell, E.E.B.

    2001-01-01

    A brief state-of-the-art review in the field of cluster-surface interactions is presented. Ionised cluster beams could become a powerful and versatile tool for the modification and processing of surfaces as an alternative to ion implantation and ion assisted deposition. The main effects of cluster-surface collisions and possible applications of cluster ion beams are discussed. The outlooks of the Cluster Implantation and Deposition Apparatus (CIDA) being developed in Guteborg University are shown

  8. Neutron ion temperature measurement

    International Nuclear Information System (INIS)

    Strachan, J.D.; Hendel, H.W.; Lovberg, J.; Nieschmidt, E.B.

    1986-11-01

    One important use of fusion product diagnostics is in the determination of the deuterium ion temperature from the magnitude of the 2.5 MeV d(d,n) 3 He neutron emission. The detectors, calibration methods, and limitations of this technique are reviewed here with emphasis on procedures used at PPPL. In most tokamaks, the ion temperature deduced from neutrons is in reasonable agreement with the ion temperature deduced by other techniques

  9. Ion transport in stellarators

    International Nuclear Information System (INIS)

    Ho, D.D.M.; Kulsrud, R.M.

    1985-09-01

    Stellarator ion transport in the low-collisionality regime with a radial electric field is calculated by a systematic expansion of the drift-Boltzmann equation. The shape of the helical well is taken into account in this calculation. It is found that the barely trapped ions with three to four times the thermal energy give the dominant contribution to the diffusion. Expressions for the ion particle and energy fluxes are derived

  10. Ion optics in an ion source system

    Energy Technology Data Exchange (ETDEWEB)

    Abdel-Salam, F W; Moustafa, O A; El-Khabeary, H [Accelerators Dept, Nuclear Research Center, Atomic Energy Authority, Cairo (Egypt)

    1997-12-31

    An analysis of ion beams from an ion source which consisted of a hemispherical anode, a plane earthed cathode, and a focusing electrode has been carried out. The focal properties of such electrode arrangement were studied using axially symmetric fields. Axial and radial electric fields were obtained as functions of the axial distance. It was found that the radial component of the gradient of potential pushes the ions towards the axis, which indicates the convergent action of the system. The effect of voltage variation between the boundary and the focusing electrode on the position of the plasma boundary are given using the experimental data of the ion source characteristics and its geometrical parameters. The advantages of plasma diffusing outside the source through a small aperture were used by applying a potential to the focusing electrode. It was possible to extract a large ion current from the expanded plasma. The system constituted a lens with a focal length of 29.4 mm. 7 figs.

  11. Ion trap device

    Science.gov (United States)

    Ibrahim, Yehia M.; Smith, Richard D.

    2016-01-26

    An ion trap device is disclosed. The device includes a series of electrodes that define an ion flow path. A radio frequency (RF) field is applied to the series of electrodes such that each electrode is phase shifted approximately 180 degrees from an adjacent electrode. A DC voltage is superimposed with the RF field to create a DC gradient to drive ions in the direction of the gradient. A second RF field or DC voltage is applied to selectively trap and release the ions from the device. Further, the device may be gridless and utilized at high pressure.

  12. Doppler ion program description

    International Nuclear Information System (INIS)

    Henline, P.

    1980-12-01

    The Doppler spectrometer is a conventional Czerny-Turner grating spectrometer with a 1024 channel multiple detector. Light is dispersed across the detector, and its output yields a spectrum covering approximately 200 A. The width of the spectral peak is directly proportional to the temperature of the emitting ions, and determination of the impurity ion temperature allows one to infer the plasma ion temperature. The Doppler ion software system developed at General Atomic uses a TRACOR Northern 1710-31 and an LSI-11/2. The exact configuration of Doublet III is different from TRACOR Northern systems at other facilities

  13. Lithium-ion batteries

    CERN Document Server

    Yoshio, Masaki; Kozawa, Akiya

    2010-01-01

    This book is a compilation of up-to-date information relative to Li-Ion technology. It provides the reader with a single source covering all important aspects of Li-Ion battery operations. It fills the gap between the old original Li-Ion technology and present state of the technology that has developed into a high state of practice. The book is designed to provide a single source for an up-to-date description of the technology associated with the Li-Ion battery industry. It will be useful to researchers interested in energy conversion for the direct conversion of chemical energy into electrica

  14. Correlation ion mobility spectroscopy

    Science.gov (United States)

    Pfeifer, Kent B [Los Lunas, NM; Rohde, Steven B [Corrales, NM

    2008-08-26

    Correlation ion mobility spectrometry (CIMS) uses gating modulation and correlation signal processing to improve IMS instrument performance. Closely spaced ion peaks can be resolved by adding discriminating codes to the gate and matched filtering for the received ion current signal, thereby improving sensitivity and resolution of an ion mobility spectrometer. CIMS can be used to improve the signal-to-noise ratio even for transient chemical samples. CIMS is especially advantageous for small geometry IMS drift tubes that can otherwise have poor resolution due to their small size.

  15. Heavy ion storage rings

    International Nuclear Information System (INIS)

    Schuch, R.

    1987-01-01

    A brief overview of synchrotron storage rings for heavy ions, which are presently under construction in different accelerator laboratories is given. Ions ranging from protons up to uranium ions at MeV/nucleon energies will be injected into these rings using multiturn injection from the accelerators available or being built in these laboratories. After injection, it is planned to cool the phase space distribution of the ions by merging them with cold electron beams or laser beams, or by using stochastic cooling. Some atomic physics experiments planned for these rings are presented. 35 refs

  16. High current ion sources

    International Nuclear Information System (INIS)

    Brown, I.G.

    1989-06-01

    The concept of high current ion source is both relative and evolutionary. Within the domain of one particular kind of ion source technology a current of microamperers might be 'high', while in another area a current of 10 Amperes could 'low'. Even within the domain of a single ion source type, what is considered high current performance today is routinely eclipsed by better performance and higher current output within a short period of time. Within their fields of application, there is a large number of kinds of ion sources that can justifiably be called high current. Thus, as a very limited example only, PIGs, Freemen sources, ECR sources, duoplasmatrons, field emission sources, and a great many more all have their high current variants. High current ion beams of gaseous and metallic species can be generated in a number of different ways. Ion sources of the kind developed at various laboratories around the world for the production of intense neutral beams for controlled fusion experiments are used to form large area proton deuteron beams of may tens of Amperes, and this technology can be used for other applications also. There has been significant progress in recent years in the use of microwave ion sources for high current ion beam generation, and this method is likely to find wide application in various different field application. Finally, high current beams of metal ions can be produced using metal vapor vacuum arc ion source technology. After a brief consideration of high current ion source design concepts, these three particular methods are reviewed in this paper

  17. Absorption refrigeration cycle applied to offshore platforms; Refrigeracao por absorcao aplicada a plataformas de petroleo

    Energy Technology Data Exchange (ETDEWEB)

    Ferreira, Maximino Joaquim Pina [KROMAV Engenharia, Rio de Janeiro, RJ (Brazil); Pinto, Luiz Antonio Vaz; Belchior, Carlos Rodrigues Pereira [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Coordenacao dos Programas de Pos-graduacao de Engenharia (COPPE)

    2004-07-01

    To produce cold from the heat seems a task unlikely or even impossible. However, absorption systems produce cooling from heat sources and it exist since the century XIX. In industrial places is very important to improve the energy use, even more in places where the activities involve great costs and incomes. Traditionally the alternatives conflict in the aspects of initial and operational costs. This paper describes the absorption systems operation and its main advantages and disadvantages, when compared to the traditional systems with compressor. The known fact that a vapor compressor system presents larger efficiency is not enough to validate it for all of the applications. In this sense, the initial and operational analysis of the costs of the absorption systems becomes interesting. In spite of, double effect absorption systems are demonstrating the evolution of the absorption cycle in order to obtain better performance. Turbo-generators and Turbo-compressors of the offshore platforms are thermal machines that reject great amount of heat in the exhaust gases. This heat is used for heating of water used in the Process Plant. The processes of separation of the mixture water-oil-gas from the well, for instance, use that heat. Even after the passage of the water in the Plant of Process, the residual heat is still enough for the use in absorption systems. A simulation is done using real data of an offshore platform. Two possible alternatives are compared under technical and economical aspects. Sensibility analysis is also performed in order to verify possible impacts of variations of electric power cost. (author)

  18. Simulator for design absorption refrigeration system; Simulador para projeto de ciclos de refrigeracao por absorcao

    Energy Technology Data Exchange (ETDEWEB)

    Morejon, C.F.M.; Brum, N. de C. [Universidade Federal, Rio de Janeiro, RJ (Brazil). Coordenacao dos Programas de Pos-graduacao de Engenharia. Programa de Engenharia Mecanica]. E-mails: Camilo_freddy@hotmail.com; Nisio@serv.com.ufrj.br

    2000-07-01

    This work presents a development of a thermal fluid dynamics model and a solution of a steady state absorption refrigeration cycle, with ammonia water as the working fluid. Analytical thermodynamics models expressing the enthalpy in function of pressure, temperature and composition (h=f(P,T,x)), are used with the aim to design all of the cycle devices, moved by any type of energy such as solar, natural gas, steam or electrical energy (Morejon and Hackenberg, 1978). The development of the analysis is carried out by the application of thermal fluid dynamics concepts together with a detailed study of the heat and mass transfer in the different cycle stages. The thermodynamic cycle model, obtained from equation of state for ammonia - water mixtures (Ziegker and Trepp, 1984), is represented by the relation h - x (enthalpy-composition) for different pressures and temperatures. The obtained models are used to implement computational codes in MAPLE-V facilitating the design and simulation of refrigeration system. This study can be applied in the systems of air conditioning and refrigeration chambers design. (author)

  19. Ion mobilities and ion-atom interaction potentials

    International Nuclear Information System (INIS)

    Gatland, I.R.

    1982-01-01

    The techniques for measuring the mobilities of ions in gases, relating interaction potentials to mobilities, and determining potentials from experimental mobilities are reviewed. Applications are presented for positive alkali ions and negative halogen ions in inert gases. (Auth.)

  20. Ion-beam technologies

    Energy Technology Data Exchange (ETDEWEB)

    Fenske, G.R. [Argonne National Lab., IL (United States)

    1993-01-01

    This compilation of figures and diagrams reviews processes for depositing diamond/diamond-like carbon films. Processes addressed are chemical vapor deposition (HFCVD, PACVD, etc.), plasma vapor deposition (plasma sputtering, ion beam sputtering, evaporation, etc.), low-energy ion implantation, and hybrid processes (biased sputtering, IBAD, biased HFCVD, etc.). The tribological performance of coatings produced by different means is discussed.

  1. Ion beam studies

    International Nuclear Information System (INIS)

    Freeman, J.H.; Chivers, D.J.; Gard, G.A.; Temple, W.

    1977-04-01

    A description of techniques for the production of intense beams of heavy ions is given. A table of recommended operational procedures for most elements is included. The ionisation of boron is considered in some detail because of its particular importance as a dopant for ion implantation. (author)

  2. Where do ions solvate?

    Indian Academy of Sciences (India)

    We study a simple model of ionic solvation inside a water cluster. The cluster is modeled as a spherical dielectric continuum. It is found that unpolarizable ions always prefer the bulk solvation. On the other hand, for polarizable ions, there exists a critical value of polarization above which surface solvation becomes ...

  3. Ion implantation of metals

    International Nuclear Information System (INIS)

    Dearnaley, G.

    1976-01-01

    In this part of the paper descriptions are given of the effects of ion implantation on (a) friction and wear in metals; and (b) corrosion of metals. In the study of corrosion, ion implantation can be used either to introduce a constituent that is known to convey corrosion resistance, or more generally to examine the parameters which control corrosion. (U.K.)

  4. Heavy ion fusion

    International Nuclear Information System (INIS)

    Bangerter, R.O.

    1986-01-01

    This report on the International Symposium on Heavy Ion Fusion held May 27-29, 1986 summarizes the problems and achievements in the areas of targets, accelerators, focussing, reactor studies, and system studies. The symposium participants recognize that there are large uncertainties in Heavy Ion Fusion but many of them are also optimistic that HIF may ultimately be the best approach to fusion

  5. High brightness ion source

    International Nuclear Information System (INIS)

    Dreyfus, R.W.; Hodgson, R.T.

    1975-01-01

    A high brightness ion beam is obtainable by using lasers to excite atoms or molecules from the ground state to an ionized state in increments, rather than in one step. The spectroscopic resonances of the atom or molecule are used so that relatively long wavelength, low power lasers can be used to obtain such ion beam

  6. Heavy ion inertial fusion

    International Nuclear Information System (INIS)

    Fessenden, T.J.; Friedman, A.

    1991-01-01

    This report describes the research status in the following areas of research in the field of heavy ion inertial fusion: (1) RF accelerators, storage rings, and synchrotrons; (2) induction linacs; (3) recirculation induction accelerator approach; (4) a new accelerator concept, the ''Mirrortron''; (5) general issues of transport, including beam merging, production of short, fat quadrupoles with nearly linear focusing, calculations of beam behaviour in image fields; 3-D electrostatic codes on drift compression with misalignments and transport around bends; (6) injectors, ion sources and RFQs, a.o., on the development of a 27 MHz RFQ to be used for the low energy portion of a new injector for all ions up to Uranium, and the development of a 2 MV carbon ion injector to provide 16 C + beams of 0.5 A each for ILSE; (7) beam transport from accelerator to target, reporting, a.o., the feasibility to suppress third-order aberrations; while Particle-in-Cell simulations on the propagation of a non-neutral ion beam in a low density gas identified photo-ionization by thermal X-rays from the target as an important source of defocusing; (9) heavy ion target studies; (10) reviewing experience with laser drivers; (11) ion cluster stopping and muon catalyzed fusion; (12) heavy ion systems, including the option of a fusion-fission burner. 1 tab

  7. Ion mobility sensor system

    Science.gov (United States)

    Xu, Jun; Watson, David B.; Whitten, William B.

    2013-01-22

    An ion mobility sensor system including an ion mobility spectrometer and a differential mobility spectrometer coupled to the ion mobility spectrometer. The ion mobility spectrometer has a first chamber having first end and a second end extending along a first direction, and a first electrode system that generates a constant electric field parallel to the first direction. The differential mobility spectrometer includes a second chamber having a third end and a fourth end configured such that a fluid may flow in a second direction from the third end to the fourth end, and a second electrode system that generates an asymmetric electric field within an interior of the second chamber. Additionally, the ion mobility spectrometer and the differential mobility spectrometer form an interface region. Also, the first end and the third end are positioned facing one another so that the constant electric field enters the third end and overlaps the fluid flowing in the second direction.

  8. Cryogenic surface ion traps

    International Nuclear Information System (INIS)

    Niedermayr, M.

    2015-01-01

    Microfabricated surface traps are a promising architecture to realize a scalable quantum computer based on trapped ions. In principle, hundreds or thousands of surface traps can be located on a single substrate in order to provide large arrays of interacting ions. To this end, trap designs and fabrication methods are required that provide scalable, stable and reproducible ion traps. This work presents a novel surface-trap design developed for cryogenic applications. Intrinsic silicon is used as the substrate material of the traps. The well-developed microfabrication and structuring methods of silicon are utilized to create simple and reproducible traps. The traps were tested and characterized in a cryogenic setup. Ions could be trapped and their life time and motional heating were investigated. Long ion lifetimes of several hours were observed and the measured heating rates were reproducibly low at around 1 phonon per second at a trap frequency of 1 MHz. (author) [de

  9. Molecular ion photofragment spectroscopy

    International Nuclear Information System (INIS)

    Bustamente, S.W.

    1983-11-01

    A new molecular ion photofragment spectrometer is described which features a supersonic molecular beam ion source and a radio frequency octapole ion trap interaction region. This unique combination allows several techniques to be applied to the problem of detecting a photon absorption event of a molecular ion. In particular, it may be possible to obtain low resolution survey spectra of exotic molecular ions by using a direct vibrational predissociation process, or by using other more indirect detection methods. The use of the spectrometer is demonstrated by measuring the lifetime of the O 2 + ( 4 π/sub u/) metastable state which is found to consist of two main components: the 4 π/sub 5/2/ and 4 π/sub -1/2/ spin components having a long lifetime (approx. 129 ms) and the 4 π/sub 3/2/ and 4 π/sub 1/2/ spin components having a short lifetime (approx. 6 ms)

  10. Ion Implantation of Polymers

    DEFF Research Database (Denmark)

    Popok, Vladimir

    2012-01-01

    The current paper presents a state-of-the-art review in the field of ion implantation of polymers. Numerous published studies of polymers modified by ion beams are analysed. General aspects of ion stopping, latent track formation and changes of structure and composition of organic materials...... are discussed. Related to that, the effects of radiothermolysis, degassing and carbonisation are considered. Specificity of depth distributions of implanted into polymers impurities is analysed and the case of high-fluence implantation is emphasised. Within rather broad topic of ion bombardment, the focus...... is put on the low-energy implantation of metal ions causing the nucleation and growth of nanoparticles in the shallow polymer layers. Electrical, optical and magnetic properties of metal/polymer composites are under the discussion and the approaches towards practical applications are overviewed....

  11. A novel ion imager for secondary ion mass spectrometry

    International Nuclear Information System (INIS)

    Matsumoto, Kazuya; Miyata, Kenji; Nakamura, Tsutomu

    1993-01-01

    This paper describes a new area detector for secondary ion mass spectrometry (SIMS) ion microscope, and its performance. The operational principle is based on detecting the change in potential of a floating photodiode caused by the ion-induced secondary-electron emission and the incoming ion itself. The experiments demonstrated that 10 1 -10 5 aluminum ions per pixel can be detected with good linear response. Moreover, relative ion sensitivities from hydrogen to lead were constant within a factor of 2. The performance of this area detector provides the potential for detection of kiloelectronvolt ion images with current ion microscopy

  12. Ion thermometers - nuclear reactor applications

    International Nuclear Information System (INIS)

    Rosenkranz, J.; Jakes, D.

    The principle is briefly described of ion thermometers and the effects are reported of radiation on the ion crystal properties. The results show that ion thermometers are applicable for in-core measurements. (J.P.)

  13. Plasma source ion implantation

    International Nuclear Information System (INIS)

    Conrad, J.R.; Forest, C.

    1986-01-01

    The authors' technique allows the ion implantation to be performed directly within the ion source at higher currents without ion beam extraction and transport. The potential benefits include greatly increased production rates (factors of 10-1000) and the ability to implant non-planar targets without rastering or shadowing. The technique eliminates the ion extractor grid set, beam raster equipment, drift space and target manipulator equipment. The target to be implanted is placed directly within the plasma source and is biased to a large negative potential so that plasma ions gain energy as they accelerate through the potential drop across the sheath that forms at the plasma boundary. Because the sheath surrounds the target on all sides, all surfaces of the target are implanted without the necessity to raster the beam or to rotate the target. The authors have succeeded in implanting nitrogen ions in a silicon target to the depths and concentrations required for surface treatment of materials like stainless steel and titanium alloys. They have performed ESCA measurements of the penetration depth profile of a silicon target that was biased to 30 kV in a nitrogen discharge plasma. Nitrogen ions were implanted to a depth of 700A at a peak concentration of 30% atomic. The measured profile is quite similar to a previously obtained profile in titanium targets with conventional techniques

  14. Laser-cooled atomic ions as probes of molecular ions

    Energy Technology Data Exchange (ETDEWEB)

    Brown, Kenneth R.; Viteri, C. Ricardo; Clark, Craig R.; Goeders, James E.; Khanyile, Ncamiso B.; Vittorini, Grahame D. [Schools of Chemistry and Biochemistry, Computational Science and Engineering and Physics, Georgia Institute of Technology, Atlanta, GA 30332 (United States)

    2015-01-22

    Trapped laser-cooled atomic ions are a new tool for understanding cold molecular ions. The atomic ions not only sympathetically cool the molecular ions to millikelvin temperatures, but the bright atomic ion fluorescence can also serve as a detector of both molecular reactions and molecular spectra. We are working towards the detection of single molecular ion spectra by sympathetic heating spectroscopy. Sympathetic heating spectroscopy uses the coupled motion of two trapped ions to measure the spectra of one ion by observing changes in the fluorescence of the other ion. Sympathetic heating spectroscopy is a generalization of quantum logic spectroscopy, but does not require ions in the motional ground state or coherent control of the ion internal states. We have recently demonstrated this technique using two isotopes of Ca{sup +} [Phys. Rev. A, 81, 043428 (2010)]. Limits of the method and potential applications for molecular spectroscopy are discussed.

  15. Ion-Ion Plasmas Produced by Electron Beams

    Science.gov (United States)

    Fernsler, R. F.; Leonhardt, D.; Walton, S. G.; Meger, R. A.

    2001-10-01

    The ability of plasmas to etch deep, small-scale features in materials is limited by localized charging of the features. The features charge because of the difference in electron and ion anisotropy, and thus one solution now being explored is to use ion-ion plasmas in place of electron-ion plasmas. Ion-ion plasmas are effectively electron-free and consist mainly of positive and negative ions. Since the two ion species behave similarly, localized charging is largely eliminated. However, the only way to produce ion-ion plasmas at low gas pressure is to convert electrons into negative ions through two-body attachment to neutrals. While the electron attachment rate is large at low electron temperatures (Te < 1 eV) in many of the halogen gases used for processing, these temperatures occur in most reactors only during the afterglow when the heating fields are turned off and the plasma is decaying. By contrast, Te is low nearly all the time in plasmas produced by electron beams, and therefore electron beams can potentially produce ion-ion plasmas continuously. The theory of ion-ion plasmas formed by pulsed electron beams is examined in this talk and compared with experimental results presented elsewhere [1]. Some general limitations of ion-ion plasmas, including relatively low flux levels, are discussed as well. [1] See the presentation by D. Leonhardt et al. at this conference.

  16. Heavy-ion dosimetry

    International Nuclear Information System (INIS)

    Schimmerling, W.

    1980-03-01

    This lecture deals with some of the more important physical characteristics of relativistic heavy ions and their measurement, with beam delivery and beam monitoring, and with conventional radiation dosimetry as used in the operation of the BEVALAC biomedical facility for high energy heavy ions (Lyman and Howard, 1977; BEVALAC, 1977). Even so, many fundamental aspects of the interaction of relativistic heavy ions with matter, including important atomic physics and radiation chemical considerations, are not discussed beyond the reminder that such additional understanding is required before an adequte perspective of the problem can be attained

  17. Ion implantation technology

    CERN Document Server

    Downey, DF; Jones, KS; Ryding, G

    1993-01-01

    Ion implantation technology has made a major contribution to the dramatic advances in integrated circuit technology since the early 1970's. The ever-present need for accurate models in ion implanted species will become absolutely vital in the future due to shrinking feature sizes. Successful wide application of ion implantation, as well as exploitation of newly identified opportunities, will require the development of comprehensive implant models. The 141 papers (including 24 invited papers) in this volume address the most recent developments in this field. New structures and possible approach

  18. IN MEMORIAM ION VATAMANU

    Directory of Open Access Journals (Sweden)

    S.P. Palii

    2012-12-01

    Full Text Available A dreamer in his creative solitude, an objective and lucid analyst of history and contemporaneity, an energetic and decisive leader with an uncanny ability for crisis management – all these describe Ion Vatamanu. His wife Elena and daughters Mihaela, Mariana, Leontina treasure a personal universe in which the magical spark of Ion Vatamanu’s love and joy of life meld the everyday in and out of poetry. Ion Vatamanu’s instantaneous connection to the audiences and deeply felt words still touch the hearts of his many colleagues and friends. Downloads: 2

  19. Ion implantation for microelectronics

    International Nuclear Information System (INIS)

    Dearnaley, G.

    1977-01-01

    Ion implantation has proved to be a versatile and efficient means of producing microelectronic devices. This review summarizes the relevant physics and technology and assesses the advantages of the method. Examples are then given of widely different device structures which have been made by ion implantation. While most of the industrial application has been in silicon, good progress continues to be made in the more difficult field of compound semiconductors. Equipment designed for the industrial ion implantation of microelectronic devices is discussed briefly. (Auth.)

  20. Multivalent ion conducting solids

    Energy Technology Data Exchange (ETDEWEB)

    Imanaka, N. [Osaka Univ., Suita, Osaka (Japan). Dept. of Applied Chemistry

    2008-07-01

    Solid electrolytes possess important characteristics for industrial applications. Only a single ionic species can macroscopically migrate in these solids. This paper described a the new NASICON (M-Zr-Nb-P-O) type system, exhibiting an exceptionally high level of trivalent M3+ ion conductivity on polycrystalline solids. The partial substitution of the smaller higher valent Nb5+ ion for Zr4+ stabilized the NASICON phase and realized the M3+ ion conduction in the NASICON structure. It was concluded that the conductivities of the series are comparable to those of the practically applied solid electrolytes of oxide anion conductors of YSZ and CSZ. 3 refs., 2 figs.

  1. Heavy ion physics

    International Nuclear Information System (INIS)

    Kalpakchieva, R.; Cherepanov, E.A.

    1993-01-01

    The international school-seminar on heavy ion physics had been organized in Dubna in may of 1993. The scientific program of reports covers the following main topics: synthesis and properties of heavy nuclei; synthesis and investigation of properties of exotic nuclei; experiments with radioactive nuclear beams; interaction between complex nuclei at low and intermediate energies. It also includes reports on laser spectroscopy and exotic nuclear beams, on some application of heavy ion beams for the problems of solid state physics, on construction of multidetector facilities and on developing of heavy ion accelerator complexes. Short communication

  2. Compact microwave ion source

    International Nuclear Information System (INIS)

    Leung, K.N.; Walther, S.; Owren, H.W.

    1985-05-01

    A small microwave ion source has been fabricated from a quartz tube with one end enclosed by a two grid accelerator. The source is also enclosed by a cavity operated at a frequency of 2.45 GHz. Microwave power as high as 500 W can be coupled to the source plasma. The source has been operated with and without multicusp fields for different gases. In the case of hydrogen, ion current density of 200 mA/cm -2 with atomic ion species concentration as high as 80% has been extracted from the source

  3. Jet laser ion source

    International Nuclear Information System (INIS)

    Dem'yanov, A.V.; Sidorov, S.V.

    1994-01-01

    External laser injector of multicharged ions (MCI) is developed in which wide-aperture aberration-free wire gauze spherical shape electrodes are applied for effective MCI extraction from laser plasma and beam focusing. Axial plasma compression by solenoid magnetic field is used to reduce ion losses due to transverse movement of the scattering laser plasma. Transverse magnetic field created by another solenoid facilitates the effective laser plasma braking and consequently, leads to the narrowing of energy spectrum of plasma ions and its shift towards lower energies. 2 refs.; 3 figs

  4. Carbon Ion Therapy

    DEFF Research Database (Denmark)

    Bassler, Niels; Hansen, David Christoffer; Herrmann, Rochus

    On the importance of choice of target size for selective boosting of hypoxic tumor subvolumina in carbon ion therapy Purpose: Functional imaging methods in radiotherapy are maturing and can to some extent uncover radio resistant structures found within a tumour entity. Selective boost of identified...... effect. All cell lines investigated here did not reach an OER of 1, even for the smaller structures, which may indicate that the achievable dose average LET of carbon ions is too low, and heavier ions than carbon may be considered for functional LET-painting....

  5. Atomic negative ions

    International Nuclear Information System (INIS)

    Brage, T.

    1991-01-01

    We review some of the recent progress in the studies of alkaline-earth, negative ions. Computations of autodetachment rates, electron affinities and transition wavelengths are discussed and some new and improved results are given

  6. Ion exchange phenomena

    Energy Technology Data Exchange (ETDEWEB)

    Bourg, I.C.; Sposito, G.

    2011-05-01

    Ion exchange phenomena involve the population of readily exchangeable ions, the subset of adsorbed solutes that balance the intrinsic surface charge and can be readily replaced by major background electrolyte ions (Sposito, 2008). These phenomena have occupied a central place in soil chemistry research since Way (1850) first showed that potassium uptake by soils resulted in the release of an equal quantity of moles of charge of calcium and magnesium. Ion exchange phenomena are now routinely modeled in studies of soil formation (White et al., 2005), soil reclamation (Kopittke et al., 2006), soil fertilitization (Agbenin and Yakubu, 2006), colloidal dispersion/flocculation (Charlet and Tournassat, 2005), the mechanics of argillaceous media (Gajo and Loret, 2007), aquitard pore water chemistry (Tournassat et al., 2008), and groundwater (Timms and Hendry, 2007; McNab et al., 2009) and contaminant hydrology (Chatterjee et al., 2008; van Oploo et al., 2008; Serrano et al., 2009).

  7. Ion implantation control system

    International Nuclear Information System (INIS)

    Gault, R. B.; Keutzer, L. L.

    1985-01-01

    A control system is disclosed for an ion implantation system of the type in which the wafers to be implanted are mounted around the periphery of a disk which rotates and also moves in a radial direction relative to an ion beam to expose successive sections of each wafer to the radiation. The control system senses beam current which passes through one or more apertures in the disk and is collected by a Faraday cup. This current is integrated to obtain a measure of charge which is compared with a calculated value based upon the desired ion dosage and other parameters. The resultant controls the number of incremental steps the rotating disk moves radially to expose the adjacent sections of each wafer. This process is continued usually with two or more traverses until the entire surface of each wafer has been implanted with the proper ion dosage

  8. Sensing with Ion Channels

    CERN Document Server

    Martinac, Boris

    2008-01-01

    All living cells are able to detect and translate environmental stimuli into biologically meaningful signals. Sensations of touch, hearing, sight, taste, smell or pain are essential to the survival of all living organisms. The importance of sensory input for the existence of life thus justifies the effort made to understand its molecular origins. Sensing with Ion Channels focuses on ion channels as key molecules enabling biological systems to sense and process the physical and chemical stimuli that act upon cells in their living environment. Its aim is to serve as a reference to ion channel specialists and as a source of new information to non specialists who want to learn about the structural and functional diversity of ion channels and their role in sensory physiology.

  9. Ion implantation apparatus

    International Nuclear Information System (INIS)

    Forneris, J.L.; Hicks, W.W.; Keller, J.H.; McKenna, C.M.; Siermarco, J.A.; Mueller, W.F.

    1981-01-01

    The invention relates to ion bombardment or implantation apparatus. It comprises an apparatus for bombarding a target with a beam of ions, including an arrangement for measuring the ion beam current and controlling the surface potential of the target. This comprises a Faraday cage formed, at least in part, by the target and by walls adjacent to, and electrically insulated from, the target and surrounding the beam. There is at least one electron source for supplying electrons to the interior of the Faraday cage and means within the cage for blocking direct rectilinear radiation from the source to the target. The target current is measured and combined with the wall currents to provide a measurement of the ion beam current. The quantity of electrons supplied to the interior of the cage can be varied to control the target current and thereby the target surface potential. (U.K.)

  10. Electrostatic ion acoustic waves

    International Nuclear Information System (INIS)

    Hasegawa, A.

    1983-01-01

    In this paper, certain aspects of plasma physics are illustrated through a study of electrostatic ion acoustic waves. The paper consists of three Sections. Section II deals with linear properties of the ion acoustic wave including derivation of the dispersions relation with the effect of Landau damping and of an ambient magnetic field. The section also introduces the excitation processes of the ion acoustic wave due to an electron drift or to a stimulated Brillouin scattering. The nonlinear properties are introduced in Section III and IV. In Section III, incoherent nonlinear effects such as quasilinear and mode-coupling saturations of the instability are discussed. The coherent nonlinear effects such as the generation of ion acoustic solitons, shocks and weak double layers are presented in Section IV. (Auth.)

  11. Trapping radioactive ions

    CERN Document Server

    Kluge, Heinz-Jürgen

    2004-01-01

    Trapping devices for atomic and nuclear physics experiments with radioactive ions are becoming more and more important at accelerator facilities. While about ten years ago only one online Penning trap experiment existed, namely ISOLTRAP at ISOLDE/CERN, meanwhile almost every radioactive beam facility has installed or plans an ion trap setup. This article gives an overview on ion traps in the operation, construction or planing phase which will be used for fundamental studies with short-lived radioactive nuclides such as mass spectrometry, laser spectroscopy and nuclear decay spectroscopy. In addition, this article summarizes the use of gas cells and radiofrequency quadrupole (Paul) traps at different facilities as a versatile tool for ion beam manipulation like retardation, cooling, bunching, and cleaning.

  12. Ion plasma electron gun

    International Nuclear Information System (INIS)

    Wakalopulos, G.

    1976-01-01

    In the disclosed electron gun positive ions generated by a hollow cathode plasma discharge in a first chamber are accelerated through control and shield grids into a second chamber containing a high voltage cold cathode. These positive ions bombard a surface of the cathode causing the cathode to emit secondary electrons which form an electron beam having a distribution adjacent to the cathode emissive surface substantially the same as the distribution of the ion beam impinging upon the cathode. After passing through the grids and the plasma discharge chamber, the electron beam exits from the electron gun via a foil window. Control of the generated electron beam is achieved by applying a relatively low control voltage between the control grid and the electron gun housing (which resides at ground potential) to control the density of the positive ions bombarding the cathode

  13. Ion sensing method

    Science.gov (United States)

    Smith, Richard Harding; Martin, Glenn Brian

    2004-05-18

    The present invention allows the determination of trace levels of ionic substances in a sample solution (ions, metal ions, and other electrically charged molecules) by coupling a separation method, such as liquid chromatography, with ion selective electrodes (ISE) prepared so as to allow detection at activities below 10.sup.-6 M. The separation method distributes constituent molecules into fractions due to unique chemical and physical properties, such as charge, hydrophobicity, specific binding interactions, or movement in an electrical field. The separated fractions are detected by means of the ISE(s). These ISEs can be used singly or in an array. Accordingly, modifications in the ISEs are used to permit detection of low activities, specifically, below 10.sup.-6 M, by using low activities of the primary analyte (the molecular species which is specifically detected) in the inner filling solution of the ISE. Arrays constructed in various ways allow flow-through sensing for multiple ions.

  14. EPR of uranium ions

    International Nuclear Information System (INIS)

    Ursu, I.; Lupei, V.

    1984-02-01

    A review of the electron paramagnetic resonance data on the uranium ions is given. After a general account of the electronic structure of the uranium free atoms and ions, the influence of the external fields (magnetic field, crystal fields) is discussed. The main information obtained from EPR studies on the uranium ions in crystals are emphasized: identification of the valence and of the ground electronic state, determination of the structure of the centers, crystal field effects, role of the intermediate coupling and of the J-mixing, role of the covalency, determination of the nuclear spin, maqnetic dipole moment and electric quadrupole moment of the odd isotopes of uranium. These data emphasize the fact that the actinide group has its own identity and this is accutely manifested at the beginning of the 5fsup(n) series encompassed by the uranium ions. (authors)

  15. Trapping radioactive ions

    International Nuclear Information System (INIS)

    Kluge, H.-J.; Blaum, K.

    2004-01-01

    Trapping devices for atomic and nuclear physics experiments with radioactive ions are becoming more and more important at accelerator facilities. While about ten years ago only one online Penning trap experiment existed, namely ISOLTRAP at ISOLDE/CERN, meanwhile almost every radioactive beam facility has installed or plans an ion trap setup. This article gives an overview on ion traps in the operation, construction or planing phase which will be used for fundamental studies with short-lived radioactive nuclides such as mass spectrometry, laser spectroscopy and nuclear decay spectroscopy. In addition, this article summarizes the use of gas cells and radiofrequency quadrupole (Paul) traps at different facilities as a versatile tool for ion beam manipulation like retardation, cooling, bunching, and cleaning

  16. Ion exchange equilibrium constants

    CERN Document Server

    Marcus, Y

    2013-01-01

    Ion Exchange Equilibrium Constants focuses on the test-compilation of equilibrium constants for ion exchange reactions. The book first underscores the scope of the compilation, equilibrium constants, symbols used, and arrangement of the table. The manuscript then presents the table of equilibrium constants, including polystyrene sulfonate cation exchanger, polyacrylate cation exchanger, polymethacrylate cation exchanger, polysterene phosphate cation exchanger, and zirconium phosphate cation exchanger. The text highlights zirconium oxide anion exchanger, zeolite type 13Y cation exchanger, and

  17. Heavy ion accelerators

    International Nuclear Information System (INIS)

    Schmelzer, C.

    1974-01-01

    This review of the present state of work on heavy-ion accelerators pays particular attention to the requirements for nuclear research. It is divided into the following sections: single-particle versus collective acceleration, heavy-ion accelerators, beam quality, and a status report on the UNILAC facility. Among the topics considered are the recycling cyclotron, linacs with superconducting resonators, and acceleration to the GeV/nucleon range. (8 figures, 2 tables) (U.S.)

  18. Ion Mass Determination

    DEFF Research Database (Denmark)

    2010-01-01

    An apparatus (100) is described for determining the mass of ions, the apparatus configured to hold a plasma (101 ) having a plasma potential. The apparatus (100) comprises an electrode (102) having a surface extending in a surface plane and an insulator (104) interfacing with the electrode (102......, and a processing unit (108) configured to interpret the detected impact locations in terms of the mass of the impacting ions....

  19. Ion beam diagnosis

    International Nuclear Information System (INIS)

    Strehl, P.

    1994-04-01

    This report is an introduction to ion beam diagnosis. After a short description of the most important ion beam parameters measurements of the beam current by means of Faraday cups, calorimetry, and beam current transformers and measurements of the beam profile by means of viewing screens, profile grids and scanning devices, and residual gas ionization monitors are described. Finally measurements in the transverse and longitudinal phase space are considered. (HSI)

  20. Ion implantation - an introduction

    International Nuclear Information System (INIS)

    Townsend, P.D.

    1986-01-01

    Ion implantation is a widely used technique with a literature that covers semiconductor production, surface treatments of steels, corrosion resistance, catalysis and integrated optics. This brief introduction outlines advantages of the technique, some aspects of the underlying physics and examples of current applications. Ion implantation is already an essential part of semiconductor technology while in many other areas it is still in an early stage of development. The future scope of the subject is discussed. (author)

  1. 2010 ion run: completed!

    CERN Multimedia

    CERN Bulletin

    2010-01-01

    After a very fast switchover from protons to lead ions, the LHC has achieved performances that allowed the machine to exceed both peak and integrated luminosity by a factor of three. Thanks to this, experiments have been able to produce high-profile results on ion physics almost immediately, confirming that the LHC was able to keep its promises for ions as well as for protons.   First direct observation of jet quenching. A seminar on 2 December was the opportunity for the ALICE, ATLAS and CMS collaborations to present their first results on ion physics in front of a packed auditorium. These results are important and are already having a major impact on the understanding of the physics processes that involve the basic constituents of matter at high energies. In the ion-ion collisions, the temperature is so high that partons (quarks and gluons), which are usually constrained inside the nucleons, are deconfined to form a highly dense and hot soup known as quark-gluon plasma (QGP). The existence of ...

  2. Collective focusing ion accelerator

    International Nuclear Information System (INIS)

    Goldin, F.J.

    1986-01-01

    The principal subject of this dissertation is the trapping confinement of pure electron plasmas in bumpy toroidal magnetic fields, with particular attention given to the trapping procedure and the behavior of the plasma during the final equilibrium. The most important aspects of the equilibrium studied were the qualitative nature of the plasma configuration and motion and its density, distribution and stability. The motivation for this study was that an unneutralized cloud of electrons contained in a toroidal system, sufficiently dense and stable, may serve to electrostatically focus ions (against centrifugal and self space charge forces) in a cyclic ion accelerator. Such an accelerator, known as a Collective Focusing Ion Accelerator (CFIA) could be far smaller than conventional designs (which use external magnetic fields directly to focus the ions) due to the smaller gyro-radium of an electron in a magnetic field of given strength. The electron cloud generally drifted poloidally at a finite radius from the toroidal minor axis. As this would preclude focusing ions with such clouds, damping this motion was investigated. Finite resistance in the normally perfectly conductive vessel wall did this. In further preparation for a working CFIA, additional experiments studied the effect of ions on the stability of the electron cloud

  3. Ion mixing and numerical simulation of different ions produced in the ECR ion source

    International Nuclear Information System (INIS)

    Shirkov, G.D.

    1996-01-01

    This paper is to continue theoretical investigations and numerical simulations in the physics of ECR ion sources within the CERN program on heavy ion acceleration. The gas (ion) mixing effect in ECR sources is considered here. It is shown that the addition of light ions to the ECR plasma has three different mechanisms to improve highly charged ion production: the increase of confinement time and charge state of highly ions as the result of ion cooling; the concentration of highly charged ions in the central region of the source with high energy and density of electrons; the increase of electron production rate and density of plasma. The numerical simulations of lead ion production in the mixture with different light ions and different heavy and intermediate ions in the mixture with oxygen, are carried out to predict the principal ECR source possibilities for LHC applications. 18 refs., 23 refs

  4. Biomaterials modification by ion beam

    International Nuclear Information System (INIS)

    Zhang Tonghe; Yi Zhongzhen; Zhang Xu; Wu Yuguang

    2001-01-01

    Ion beam technology is one of best ways for the modification of biomaterials. The results of ion beam modification of biomaterials are given. The method and results of improved biocompatibility are indicated by ion beam technology. The future development of ion beam modification of biomaterials is discussed

  5. Ion-selective electrode reviews

    CERN Document Server

    Thomas, J D R

    1983-01-01

    Ion-Selective Electrode Reviews, Volume 5 is a collection of articles that covers ion-speciation. The book aims to present the advancements of the range and capabilities of selective ion-sensors. The topics covered in the selection are neutral carrier based ion-selective electrodes; reference electrodes and liquid junction effects in ion-selective electrode potentiometry; ion transfer across water/organic phase boundaries and analytical; and carbon substrate ion-selective electrodes. The text will be of great use to chemists and chemical engineers.

  6. Ion optics of RHIC EBIS

    Energy Technology Data Exchange (ETDEWEB)

    Pikin, A.; Alessi, J.; Beebe, E.; Kponou, A.; Okamura, M.; Raparia, D.; Ritter, J.; Tan, Y.; Kuznetsov, G.

    2011-09-10

    RHIC EBIS has been commissioned to operate as a versatile ion source on RHIC injection facility supplying ion species from He to Au for Booster. Except for light gaseous elements RHIC EBIS employs ion injection from several external primary ion sources. With electrostatic optics fast switching from one ion species to another can be done on a pulse to pulse mode. The design of an ion optical structure and the results of simulations for different ion species are presented. In the choice of optical elements special attention was paid to spherical aberrations for high-current space charge dominated ion beams. The combination of a gridded lens and a magnet lens in LEBT provides flexibility of optical control for a wide range of ion species to satisfy acceptance parameters of RFQ. The results of ion transmission measurements are presented.

  7. Ion beam inertial fusion

    International Nuclear Information System (INIS)

    Bangerter, R.O.

    1995-01-01

    About twenty years ago, A. W. Maschke of Brookhaven National Laboratory and R. L. Martin of Argonne National Laboratory recognized that the accelerators that have been developed for high energy and nuclear physics are, in many ways, ideally suited to the requirements of inertial fusion power production. These accelerators are reliable, they have a long operating life, and they can be efficient. Maschke and Martin noted that they can focus ion beams to small focal spots over distances of many meters and that they can readily operate at the high pulse repetition rates needed for commercial power production. Fusion, however, does impose some important new constraints that are not important for high energy or nuclear physics applications. The most challenging new constraint from a scientific standpoint is the requirement that the accelerator deliver more than 10 14 W of beam power to a small quantity (less than 100 mg) of matter. The most challenging constraint from an engineering standpoint is accelerator cost. Maschke showed theoretically that accelerators could produce adequate work. Heavy-ion fusion is widely recognized to be a promising approach to inertial fusion power production. It provides an excellent opportunity to apply methods and technology developed for basic science to an important societal need. The pulsed-power community has developed a complementary, parallel approach to ion beam fusion known as light-ion fusion. The talk will discuss both heavy-ion and light-ion fusion. It will explain target physics requirements and show how they lead to constraints on the usual accelerator parameters such as kinetic energy, current, and emittance. The talk will discuss experiments that are presently underway, specifically experiments on high-current ion sources and injectors, pulsed-power machines recirculating induction accelerators, and transverse beam combining. The talk will give a brief description of a proposed new accelerator called Elise

  8. Ion-selective electrode reviews

    CERN Document Server

    Thomas, J D R

    1985-01-01

    Ion-Selective Electrode Reviews, Volume 7 is a collection of papers that covers the applications of electrochemical sensors, along with the versatility of ion-selective electrodes. The coverage of the text includes solid contact in membrane ion-selective electrodes; immobilized enzyme probes for determining inhibitors; potentiometric titrations based on ion-pair formation; and application of ion-selective electrodes in soil science, kinetics, and kinetic analysis. The text will be of great use to chemists and chemical engineers.

  9. Clues From Pluto's Ions

    Science.gov (United States)

    Kohler, Susanna

    2016-05-01

    Nearly a year ago, in July 2015, the New Horizons spacecraft passed by the Pluto system. The wealth of data amassed from that flyby is still being analyzed including data from the Solar Wind Around Pluto (SWAP) instrument. Recent examination of this data has revealedinteresting new information about Plutos atmosphere and how the solar wind interacts with it.A Heavy Ion TailThe solar wind is a constant stream of charged particles released by the Sun at speeds of around 400 km/s (thats 1 million mph!). This wind travels out to the far reaches of the solar system, interacting with the bodies it encounters along the way.By modeling the SWAP detections, the authors determine the directions of the IMF that could produce the heavy ions detected. Red pixels represent IMF directions permitted. No possible IMF could reproduce the detections if the ions are nitrogen (bottom panels), and only retrograde IMF directions can produce the detections if the ions are methane. [Adapted from Zirnstein et al. 2016]New Horizons data has revealed that Plutos atmosphere leaks neutral nitrogen, methane, and carbon monoxide molecules that sometimes escape its weak gravitational pull. These molecules become ionized and are subsequently picked up by the passing solar wind, forming a tail of heavy ions behind Pluto. The details of the geometry and composition of this tail, however, had not yet been determined.Escaping MethaneIn a recent study led by Eric Zirnstein (Southwest Research Institute), the latest analysis of data from the SWAP instrument on board New Horizons is reported. The team used SWAPs ion detections from just after New Horizons closest approach to Pluto to better understand how the heavy ions around Pluto behave, and how the solar wind interacts with Plutos atmosphere.In the process of analyzing the SWAP data, Zirnstein and collaborators first establish what the majority of the heavy ions picked up by the solar wind are. Models of the SWAP detections indicate they are unlikely

  10. Study of ion exchange equilibrium and determination of heat of ion exchange by ion chromatography

    International Nuclear Information System (INIS)

    Liu Kailu; Yang Wenying

    1996-01-01

    Ion chromatography using pellicularia ion exchange resins and dilute solution can be devoted to the study of ion exchange thermodynamics and kinetics. Ion exchange equilibrium equation was obtained, and examined by the experiments. Based on ion exchange equilibrium, the influence of eluent concentration and resin capacity on adjusted retention volumes was examined. The effect of temperature on adjusted retention volumes was investigated and heats of ion exchange of seven anions were determined by ion chromatography. The interaction between anions and skeleton structure of resins were observed

  11. Ion nitriding of aluminium

    International Nuclear Information System (INIS)

    Fitz, T.

    2002-09-01

    The present study is devoted to the investigation of the mechanism of aluminium nitriding by a technique that employs implantation of low-energy nitrogen ions and diffusional transport of atoms. The nitriding of aluminium is investigated, because this is a method for surface modification of aluminium and has a potential for application in a broad spectrum of fields such as automobile, marine, aviation, space technologies, etc. However, at present nitriding of aluminium does not find any large scale industrial application, due to problems in the formation of stoichiometric aluminium nitride layers with a sufficient thickness and good quality. For the purposes of this study, ion nitriding is chosen, as an ion beam method with the advantage of good and independent control over the process parameters, which thus can be related uniquely to the physical properties of the resulting layers. Moreover, ion nitriding has a close similarity to plasma nitriding and plasma immersion ion implantation, which are methods with a potential for industrial application. (orig.)

  12. The ion circus

    Energy Technology Data Exchange (ETDEWEB)

    Minaya Ramirez, Enrique [GSI Helmholtzzentrum, Darmstadt (Germany); Lunney, David [CSNSM- IN2P3/CNRS, Universite de Paris-Sud, Orsay (France)

    2010-07-01

    The ability to prepare radioactive beams for experiments in nuclear structure has seen important developments in recent years. The use of ion traps and buffer-gas cooling now enables the accumulation and purification of even short-lived nuclides. This is a key point for future installations since higher intensity also brings increased isobaric contamination which can be disastrous for background. Until now, the development of beam cooler/bunchers has relied on linear (radiofrequency quadrupole) Paul traps. In this contribution we describe the progress in developing a novel circular Paul trap. The ion circus, so named for its ability to trap ions at different positions along the ring circumference and to eject them in either perpendicular or tangential direction, has also been designed to cool and mass separate the ions over a longer flight path. The resolving power is increased as the ions orbit in the ring and are cooled with buffer gas at a much lower pressure. The first prototype is now under test in Orsay. We report results of the first tests and the future program.

  13. Inverted magnetron ion source

    International Nuclear Information System (INIS)

    Singh, B.; Boyarsky, D.

    1985-01-01

    The present invention provides, in a preferred embodiment, a cylindrical stainless steel cathode with end pieces thereon to form a cathode chamber within. In addition, in a preferred embodiment, there is a stainless steel rod which passes axially through the cathode chamber and which is electrically insulated therefrom at the end pieces. The stainless steel cathode has first and second apertures formed therein with the first to be connected to a source of ionizable gas and the second to act as the opening through which there passes a stream of ions to an ion beam target. A magnetic flux source is coupled to the cathode chamber to pass magnetic flux therethrough and a voltage source is connected between the anode and the cathode to provide an electrostatic field therebetween whereby when ionizable gas is fed into the cathode chamber, it is ionized and a stream of ions emanates from the second aperture. In a preferred embodiment there is further provided an electrostatic ion focusing means to focus the ion stream emanating from the second aperture

  14. Neuromyelitis optica: clinical features, immunopathogenesis and treatment

    Science.gov (United States)

    Jarius, S; Wildemann, B; Paul, F

    2014-01-01

    The term ‘neuromyelitis optica’ (‘Devic's syndrome’, NMO) refers to a syndrome characterized by optic neuritis and myelitis. In recent years, the condition has raised enormous interest among scientists and clinical neurologists, fuelled by the detection of a specific serum immunoglobulin (Ig)G reactivity (NMO-IgG) in up to 80% of patients with NMO. These autoantibodies were later shown to target aquaporin-4 (AQP4), the most abundant water channel in the central nervous system (CNS). Here we give an up-to-date overview of the clinical and paraclinical features, immunopathogenesis and treatment of NMO. We discuss the widening clinical spectrum of AQP4-related autoimmunity, the role of magnetic resonance imaging (MRI) and new diagnostic means such as optical coherence tomography in the diagnosis of NMO, the role of NMO-IgG, T cells and granulocytes in the pathophysiology of NMO, and outline prospects for new and emerging therapies for this rare, but often devastating condition. Other Articles published in this series Paraneoplastic neurological syndromes. Clinical and Experimental Immunology 2014, 175: 336–48. Disease-modifying therapy in multiple sclerosis and chronic inflammatory demyelinating polyradiculoneuropathy: common and divergent current and future strategies. Clinical and Experimental Immunology 2014, 175: 359–72. Monoclonal antibodies in treatment of multiple sclerosis. Clinical and Experimental Immunology 2014, 175: 373–84. CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders. Clinical and Experimental Immunology 2014, 175: 385–96. Requirement for safety monitoring for approved multiple sclerosis therapies: an overview. Clinical and Experimental Immunology 2014, 175: 397–407. Myasthenia gravis: an update for the clinician. Clinical and Experimental Immunology 2014, 175: 408–18. Cerebral vasculitis in adults: what are the steps in order to establish the diagnosis? Red flags and pitfalls. Clinical and Experimental Immunology 2014, 175: 419–24. Multiple sclerosis treatment and infectious issues: update 2013. Clinical and Experimental Immunology 2014, 175: 425–38. Diagnosis, pathogenesis and treatment of myositis: recent advances 2014, 175: 349–58. Management of disease-modifying treatments in neurological autoimmune diseases of the central nervous system 2014, 176: 135–48. PMID:24666204

  15. Demographic and clinical features of neuromyelitis optica

    DEFF Research Database (Denmark)

    Pandit, L.; Asgari, Nasrin; Apiwattanakul, M.

    2015-01-01

    Asia, the Caribbean, and Cuba suggest that the incidence and prevalence of NMO ranges from 0.05-0.4 and 0.52-4.4 per 100,000, respectively. Mean age at onset (32.6-45.7) and median time to first relapse (8-12 months) was similar. Most studies reported an excess of disease in women and a relapsing...

  16. Majority ion heating near the ion-ion hybrid layer in tokamaks

    International Nuclear Information System (INIS)

    Phillips, C.K.; Hosea, J.C.; Ignat, D.; Majeski, R.; Rogers, J.H.; Schilling, G.; Wilson, J.R.

    1995-08-01

    Efficient direct majority ion heating in a deuterium-tritium (D-T) reactor-grade plasma via absorption of fast magnetosonic waves in the ion cyclotron range of frequencies (ICRF) is discussed. Majority ion heating results from resonance overlap between the cyclotron layers and the D-T ion-ion hybrid layer in hot, dense plasmas for fast waves launched with high parallel wavenumbers. Analytic and numerical models are used to explore the regime in ITER plasmas

  17. Ion channels versus ion pumps: the principal difference, in principle.

    Science.gov (United States)

    Gadsby, David C

    2009-05-01

    The incessant traffic of ions across cell membranes is controlled by two kinds of border guards: ion channels and ion pumps. Open channels let selected ions diffuse rapidly down electrical and concentration gradients, whereas ion pumps labour tirelessly to maintain the gradients by consuming energy to slowly move ions thermodynamically uphill. Because of the diametrically opposed tasks and the divergent speeds of channels and pumps, they have traditionally been viewed as completely different entities, as alike as chalk and cheese. But new structural and mechanistic information about both of these classes of molecular machines challenges this comfortable separation and forces its re-evaluation.

  18. Heavy-ion radiography

    International Nuclear Information System (INIS)

    Fabrikant, J.I.; Tobias, C.A.; Holley, W.R.; Benton, E.V.

    1981-01-01

    Heavy-particle radiography has clinical potential as a newly developed noninvasive low-dose imaging procedure that provides increased resolution of minute density differences in soft tissues of the body. The method utilizes accelerated high-energy ions, primarily carbon and neon, at the Bevalac accelerator at the Lawrence Berkeley Laboratory. The research program for medicine utilizes heavy-ion radiography for low-dose mammography, for treatment planning for cancer patients, and for imaging and accurate densitometry of skeletal structures, brain and spinal neoplasms, and the heart. The potential of heavy-ion imaging, and particularly reconstruction tomography, is now proving to be an adjunct to existing diagnostic imaging procedures in medicine, both for applications to the diagnosis, management and treatment of clinical cancer in man, and for the early detection of small soft-tissue tumors at low radiation dose

  19. Heavy ion inertial fusion

    International Nuclear Information System (INIS)

    Keefe, D.; Sessler, A.M.

    1980-01-01

    Inertial fusion has not yet been as well explored as magnetic fusion but can offer certain advantages as an alternative source of electric energy for the future. Present experiments use high-power beams from lasers and light-ion diodes to compress the deuterium-tritium (D-T) pellets but these will probably be unsuitable for a power plant. A more promising method is to use intense heavy-ion beams from accelerator systems similar to those used for nuclear and high-energy physics; the present paper addresses itself to this alternative. As will be demonstrated the very high beam power needed poses new design questions, from the ion-source through the accelerating system, the beam transport system, to the final focus. These problems will require extensive study, both theoretically and experimentally, over the next several years before an optimum design for an inertial fusion driver can be arrived at. (Auth.)

  20. Acceleration of radioactive ions

    International Nuclear Information System (INIS)

    Laxdal, R.E.

    2003-01-01

    There is an intense interest world-wide in the use of radioactive ion beams (RIBs) for experiment. In many existing or proposed facilities ions are produced or collected at source potential, ionized and re-accelerated. Within the past year three new ISOL based facilities have added dedicated post-accelerators to deliver accelerated RIBs to experiment. The paper gives an overview of RIB accelerators present and future, and explores the inherent features in the various acceleration methods with an emphasis on heavy ion linacs. The ISAC-I and ISAC-II post-accelerators are discussed as examples. Commissioning results and initial operating experience with ISAC-I will be presented

  1. Ion accelerators for space

    International Nuclear Information System (INIS)

    Slobodrian, R.J.; Potvin, L.

    1991-01-01

    The main purpose of the accelerators is to allow ion implantation in space stations and their neighborhoods. There are several applications of interest immediately useful in such environment: as ion engines and thrusters, as implanters for material science and for hardening of surfaces (relevant to improve resistance to micrometeorite bombardment of exposed external components), production of man made alloys, etc. The microgravity environment of space stations allows the production of substances (crystalline and amorphous) under conditions unknown on earth, leading to special materials. Ion implantation in situ of those materials would thus lead uninterruptedly to new substances. Accelerators for space require special design. On the one hand it is possible to forego vacuum systems simplifying the design and operation but, on the other hand, it is necessary to pay special attention to heat dissipation. Hence it is necessary to construct a simulator in vacuum to properly test prototypes under conditions prevailing in space

  2. Heavy ion inertial fusion

    International Nuclear Information System (INIS)

    Keefe, D.; Sessler, A.M.

    1980-07-01

    Inertial fusion has not yet been as well explored as magnetic fusion but can offer certain advantages as an alternative source of electric energy for the future. Present experiments use high-power beams from lasers and light-ion diodes to compress the deuterium-tritium (D-T) pellets but these will probably be unsuitable for a power plant. A more promising method is to use intense heavy-ion beams from accelerator systems similar to those used for nuclear and high-energy physics; the present paper addresses itself to this alternative. As will be demonstrated the very high beam power needed poses new design questions, from the ion source through the accelerating system, the beam transport system, to the final focus. These problems will require extensive study, both theoretically and experimentally, over the next several years before an optimum design for an inertial fusion driver can be arrived at

  3. Ion cyclotron resonance heating

    International Nuclear Information System (INIS)

    Tajima, T.

    1982-01-01

    Ion cyclotron resonance heating of plasmas in tokamak and EBT configurations has been studied using 1-2/2 and 2-1/2 dimensional fully self-consistent electromagnetic particle codes. We have tested two major antenna configurations; we have also compared heating efficiencies for one and two ion species plasmas. We model a tokamak plasma with a uniform poloidal field and 1/R toroidal field on a particular q surface. Ion cyclotron waves are excited on the low field side by antennas parallel either to the poloidal direction or to the toroidal direction with different phase velocities. In 2D, minority ion heating (vsub(perpendicular)) and electron heating (vsub(parallel),vsub(perpendicular)) are observed. The exponential electron heating seems due to the decay instability. The minority heating is consistent with mode conversion of fast Alfven waves and heating by electrostatic ion cyclotron modes. Minority heating is stronger with a poloidal antenna. The strong electron heating is accompanied by toroidal current generation. In 1D, no thermal instability was observed and only strong minority heating resulted. For an EBT plasma we model it by a multiple mirror. We have tested heating efficiency with various minority concentrations, temperatures, mirror ratios, and phase velocities. In this geometry we have beach or inverse beach heating associated with the mode conversion layer perpendicular to the toroidal field. No appreciable electron heating is observed. Heating of ions is linear in time. For both tokamak and EBT slight majority heating above the collisional rate is observed due to the second harmonic heating. (author)

  4. Ion-selective electrodes

    Energy Technology Data Exchange (ETDEWEB)

    Mikhelson, Konstantin N. [St. Petersburg State Univ. (Russian Federation). Ion-Selective Electrode Laboratory

    2013-06-01

    Ion-selective electrodes (ISEs) have a wide range of applications in clinical, environmental, food and pharmaceutical analysis as well as further uses in chemistry and life sciences. Based on his profound experience as a researcher in ISEs and a course instructor, the author summarizes current knowledge for advanced teaching and training purposes with a particular focus on ionophore-based ISEs. Coverage includes the basics of measuring with ISEs, essential membrane potential theory and a comprehensive overview of the various classes of ion-selective electrodes. The principles of constructing ISEs are outlined, and the transfer of methods into routine analysis is considered.

  5. Ion beam texturing

    Science.gov (United States)

    Hudson, W. R.

    1977-01-01

    A microscopic surface texture was created by sputter-etching a surface while simultaneously sputter-depositing a lower sputter yield material onto the surface. A xenon ion-beam source was used to perform the texturing process on samples as large as 3-cm diameter. Textured surfaces have been characterized with SEM photomicrographs for a large number of materials including Cu, Al, Si, Ti, Ni, Fe, stainless steel, Au, and Ag. A number of texturing parameters are studied including the variation of texture with ion-beam powder, surface temperature, and the rate of texture growth with sputter etching time.

  6. Magnetic ions in crystals

    CERN Document Server

    Stevens, K W

    2014-01-01

    There have been many demonstrations, particularly for magnetic impurity ions in crystals, that spin-Hamiltonians are able to account for a wide range of experimental results in terms of much smaller numbers of parameters. Yet they were originally derived from crystal field theory, which contains a logical flaw; electrons on the magnetic ions are distinguished from those on the ligands. Thus there is a challenge: to replace crystal field theory with one of equal or greater predictive power that is based on a surer footing. The theory developed in this book begins with a generic Hamiltonian, on

  7. [Relativistic heavy ion research

    International Nuclear Information System (INIS)

    1990-01-01

    At Brookhaven National Laboratory, participation in the E802 Experiment, which is the first major heavy-ion experiment at the BNL-AGS, was the main focus of the group during the past four years. The emphases of the E802 experiment were on (a) accurate particle identification and measurements of spectra over a wide kinematical domain (5 degree LAB < 55 degree, p < 20 GeV/c); and (b) measurements of small-angle two-particle correlations, with event characterization tools: multiplicity array, forward and large-angle calorimeters. This experiment and other heavy ion collision experiments are discussed in this report

  8. Secondary ion emission

    International Nuclear Information System (INIS)

    Krohn, V.E.

    1976-01-01

    The Saha-Langmuir equation that describes the equilibrium emission process, surface ionization, has also been used to describe ion yields observed in the non-equilibrium emission process, sputtering. In describing sputtering, it is probably best to include the potential due to an image charge in the expression for negative as well as positive ion yield and to treat the work function and the temperature as parameters having limited physical significance. Arguments are presented to suggest that sputtered material is not emitted from a plasma. (Auth.)

  9. Heavy ion collisions

    International Nuclear Information System (INIS)

    Jacak, B.V.

    1994-01-01

    Heavy ion collisions at very high energies provide an opportunity to recreate in the laboratory the conditions which existed very early in the universe, just after the big bang. We prepare matter at very high energy density and search for evidence that the quarks and gluons are deconfined. I describe the kinds of observables that are experimentally accessible to characterize the system and to search for evidence of new physics. A wealth of information is now available from CERN and BNL heavy ion experiments. I discuss recent results on two particle correlations, strangeness production, and dilepton and direct photon distributions

  10. Ion-selective electrodes

    CERN Document Server

    Mikhelson, Konstantin N

    2013-01-01

    Ion-selective electrodes (ISEs) have a wide range of applications in clinical, environmental, food and pharmaceutical analysis as well as further uses in chemistry and life sciences. Based on his profound experience as a researcher in ISEs and a course instructor, the author summarizes current knowledge for advanced teaching and training purposes with a particular focus on ionophore-based ISEs. Coverage includes the basics of measuring with ISEs, essential membrane potential theory and a comprehensive overview of the various classes of ion-selective electrodes. The principles of constructing I

  11. Double chamber ion source

    International Nuclear Information System (INIS)

    Uman, M.F.; Winnard, J.R.; Winters, H.F.

    1978-01-01

    The ion source is comprised of two discharge chambers one of which is provided with a filament and an aperture leading into the other chamber which in turn has an extraction orifice. A low voltage arc discharge is operated in an inert gas atmosphere in the filament chamber while an arc of higher voltage is operated in the second ionization chamber which contains a vapor which will give the desired dopant ion species. The entire source is immersed in an axial magnetic field parallel to a line connecting the filament, the aperture between the two chambers and the extraction orifice. (author)

  12. Asymmetric ion trap

    Science.gov (United States)

    Barlow, Stephan E.; Alexander, Michael L.; Follansbee, James C.

    1997-01-01

    An ion trap having two end cap electrodes disposed asymmetrically about a center of a ring electrode. The inner surface of the end cap electrodes are conformed to an asymmetric pair of equipotential lines of the harmonic formed by the application of voltages to the electrodes. The asymmetry of the end cap electrodes allows ejection of charged species through the closer of the two electrodes which in turn allows for simultaneously detecting anions and cations expelled from the ion trap through the use of two detectors charged with opposite polarity.

  13. [Relativistic heavy ion research

    International Nuclear Information System (INIS)

    1991-01-01

    The present document describes our second-year application for a continuation grant on relativistic heavy-ion research at Nevis Laboratories, Columbia University, over the two-year period starting from November 15, 1990. The progress during the current budget year is presented. This year, construction of RHIC officially began. As a result, the entire Nevis nuclear physics group has made a coherent effort to create new proposal for an Open Axially Symmetric Ion Spectrometer (OASIS) proposal. Future perspectives and our plans for this proposal are described

  14. Small Column Ion Exchange

    International Nuclear Information System (INIS)

    Huff, Thomas

    2010-01-01

    Small Column Ion Exchange (SCIX) leverages a suite of technologies developed by DOE across the complex to achieve lifecycle savings. Technologies are applicable to multiple sites. Early testing supported multiple sites. Balance of SRS SCIX testing supports SRS deployment. A forma Systems Engineering Evaluation (SEE) was performed and selected Small Column Ion Exchange columns containing Crystalline Silicotitanate (CST) in a 2-column lead/lag configuration. SEE considered use of Spherical Resorcinol-Formaldehyde (sRF). Advantages of approach at SRS include: (1) no new buildings, (2) low volume of Cs waste in solid form compared to aqueous strip effluent; and availability of downstream processing facilities for immediate processing of spent resin.

  15. Electron capture in ion atom and ion-ion collisions

    International Nuclear Information System (INIS)

    Barat, M.

    1986-01-01

    Electron capture (EC) by positive ions in collision with various targets has remained one of the most important subjects of research since the early 30's. From a theoretical point of view, EC is obviously a coupled 3-body problem: at least two cores and an active electron that jumps between them. Practical interest in EC arose in a variety of fields. Recently a renewed interest arose from the physics of thermonuclear fusion, where capture by highly charged ionic impurities were found to be an important process in tokamak devices. For that reasons, a number of reviews were devoted to this subject during the past years, including lectures given in various NATO advanced science institutes. The aim of this lecture is not at all to add a new review to this list, but (i) to summarize the very basis of the present theoretical approaches at low and moderate collision energy, (ii) to pinpoint some crucial difficulties in the theoretical treatment, (iii) to select specific examples which, to the taste of the author, reflect some present practical interest, or some significant advances. 48 references, 38 figures, 1 table

  16. Ion-selective electrode reviews

    CERN Document Server

    Thomas, J D R

    1982-01-01

    Ion-Selective Electrode Reviews, Volume 3, provides a review of articles on ion-selective electrodes (ISEs). The volume begins with an article on methods based on titration procedures for surfactant analysis, which have been developed for discrete batch operation and for continuous AutoAnalyser use. Separate chapters deal with detection limits of ion-selective electrodes; the possibility of using inorganic ion-exchange materials as ion-sensors; and the effect of solvent on potentials of cells with ion-selective electrodes. Also included is a chapter on advances in calibration procedures, the d

  17. Cooling of molecular ion beams

    International Nuclear Information System (INIS)

    Wolf, A.; Krohn, S.; Kreckel, H.; Lammich, L.; Lange, M.; Strasser, D.; Grieser, M.; Schwalm, D.; Zajfman, D.

    2004-01-01

    An overview of the use of stored ion beams and phase space cooling (electron cooling) is given for the field of molecular physics. Emphasis is given to interactions between molecular ions and electrons studied in the electron cooler: dissociative recombination and, for internally excited molecular ions, electron-induced ro-vibrational cooling. Diagnostic methods for the transverse ion beam properties and for the internal excitation of the molecular ions are discussed, and results for phase space cooling and internal (vibrational) cooling are presented for hydrogen molecular ions

  18. Analytical applications of ion exchangers

    CERN Document Server

    Inczédy, J

    1966-01-01

    Analytical Applications of Ion Exchangers presents the laboratory use of ion-exchange resins. This book discusses the development in the analytical application of ion exchangers. Organized into 10 chapters, this book begins with an overview of the history and significance of ion exchangers for technical purposes. This text then describes the properties of ion exchangers, which are large molecular water-insoluble polyelectrolytes having a cross-linked structure that contains ionic groups. Other chapters consider the theories concerning the operation of ion-exchange resins and investigate th

  19. Development of focused ion beam systems with various ion species

    International Nuclear Information System (INIS)

    Ji Qing; Leung, K.-N.; King, Tsu-Jae; Jiang Ximan; Appleton, Bill R.

    2005-01-01

    Conventional focused ion beam systems employ a liquid-metal ion source (LMIS) to generate high-brightness beams, such as Ga + beams. Recently there has been an increased need for focused ion beams in areas like biological studies, advanced magnetic-film manufacturing and secondary-ion mass spectroscopy (SIMS). In this article, status of development on focused ion beam systems with ion species such as O 2 + , P + , and B + will be reviewed. Compact columns for forming focused ion beams from low energy (∼3keV), to intermediate energy (∼35keV) are discussed. By using focused ion beams, a SOI MOSFET is fabricated entirely without any masks or resist

  20. Energy landscapes for mobile ions in ion conducting solids

    Indian Academy of Sciences (India)

    molecular dynamics (MD) simulations yields quantitative predictions of the ion transport characteristics. As ... Solid electrolytes; bond valence analysis; ion transport in glasses. 1. .... clusters are considered to contribute only to a.c. conduc-.

  1. Neuromielitis óptica con alta expresión de acuaporina-4 y anticuerpos anti-acuaporina-4 positivos en suero Neuromyelitis optica with high aquaporin-4 expression and positive serum aquaporin-4 autoantibodies

    Directory of Open Access Journals (Sweden)

    Alejandra Báez

    2012-04-01

    Full Text Available La presencia de anticuerpos IgG en suero, con blanco en los canales de acuaporina-4, es específica de la neuromielitis óptica (NMO. El 60% de los pacientes con NMO presentan lesiones cerebrales en la resonancia magnética (RM; en un 8% (mayoría niños estas lesiones se consideraron "atípicas". Presentamos dos pacientes con NMO y lesiones en el SNC de alta expresión de acuaporina-4. Caso 1: varón de 50 años, que comenzó con pérdida de visión en ojo derecho (OD. Recibió tratamiento empírico con metilprednisolona 1 g/d x 3 días. Al mes presentó dolor generalizado y hemiparesia derecha; nuevamente recibió metilprednisolona 1 g/d x 5 días e IgG IV 400 mg/kg/d × 5 días. Recuperó la deambulación persistiendo el dolor y fenómenos paroxísticos en los 4 miembros. Potenciales evocados visuales: P100, ojo izquierdo (OI 123 mseg. OD sin respuesta. La RM de cerebro (FLAIR mostró hiperintensidad en nervio óptico derecho, hipotálamo y comisura blanca anterior. RM cervical: lesión medular extensa (5 cuerpos vertebrales. Caso 2: mujer de 53 años, con disminución de la agudeza visual en ambos ojos y parestesias en miembros inferiores que remitieron espontáneamente. Evolucionó al mes con cuadriparesia e incontinencia esfinteriana. Recibió metilprednisolona 1 g/d x 5 días, sin mejoría. Potenciales evocados visuales: P100 OI 124 mseg. OD 128 mseg. RM cerebro: (FLAIR hiperintensidad hipotalámica y periacueductal. RM cervical: lesión medular extensa (7 cuerpos vertebrales. Anticuerpos anti-acuaporina-4 positivos en ambos pacientes (inmunofluorescencia indirecta. Las lesiones consideradas "atípicas", como aquí, en sitios con alta densidad de proteínas canales de agua AQP4 deberán considerarse para el diagnóstico diferencial.Disease-specific aquaporin-4 antibodies (NMO-IgG are the main effector of lesions in neuromyelitis optica (NMO patients. Brain MRI lesions are detected in 60% of them, with 8% (almost infants at sites of high

  2. An RF ion source based primary ion gun for secondary ion mass spectroscopy

    International Nuclear Information System (INIS)

    Menon, Ranjini; Nabhiraj, P.Y.; Bhandari, R.K.

    2011-01-01

    In this article we present the design, development and characterization of an RF plasma based ion gun as a primary ion gun for SIMS application. RF ion sources, in particular Inductively Coupled Plasma (ICP) ion sources are superior compared to LMIS and duoplasmtron ion sources since they are filamentless, can produce ions of gaseous elements. At the same time, ICP ion sources offer high angular current density which is an important factor in producing high current in small spot size on the target. These high current microprobes improve the signal to noise ratio by three orders as compared to low current ion sources such as LMIS. In addition, the high current microprobes have higher surface and depth profiling speeds. In this article we describe a simple ion source in its very basic form, two lens optical column and characteristics of microprobe

  3. Polyatomic ions from a high current ion implanter driven by a liquid metal ion source

    Science.gov (United States)

    Pilz, W.; Laufer, P.; Tajmar, M.; Böttger, R.; Bischoff, L.

    2017-12-01

    High current liquid metal ion sources are well known and found their first application as field emission electric propulsion thrusters in space technology. The aim of this work is the adaption of such kind of sources in broad ion beam technology. Surface patterning based on self-organized nano-structures on, e.g., semiconductor materials formed by heavy mono- or polyatomic ion irradiation from liquid metal (alloy) ion sources (LMAISs) is a very promising technique. LMAISs are nearly the only type of sources delivering polyatomic ions from about half of the periodic table elements. To overcome the lack of only very small treated areas by applying a focused ion beam equipped with such sources, the technology taken from space propulsion systems was transferred into a large single-end ion implanter. The main component is an ion beam injector based on high current LMAISs combined with suited ion optics allocating ion currents in the μA range in a nearly parallel beam of a few mm in diameter. Different types of LMAIS (needle, porous emitter, and capillary) are presented and characterized. The ion beam injector design is specified as well as the implementation of this module into a 200 kV high current ion implanter operating at the HZDR Ion Beam Center. Finally, the obtained results of large area surface modification of Ge using polyatomic Bi2+ ions at room temperature from a GaBi capillary LMAIS will be presented and discussed.

  4. Heavy ion transfer reactions

    International Nuclear Information System (INIS)

    Weisser, D.C.

    1977-06-01

    To complement discussions on the role of γ rays in heavy ion induced reactions, the author discusses the role played by particle detection. Transfer reactions are part of this subject and are among those in which one infers the properties of the residual nucleus in a reaction by observing the emerging light nucleus. Inelastic scattering ought not be excluded from this subject, although no particles are transferred, because of the role it plays in multistep reactions and in fixing O.M. parameters describing the entrance channel of the reaction. Heavy ion transfer reaction studies have been under study for some years and yet this research is still in its infancy. The experimental techniques are difficult and the demands on theory rigorous. One of the main products of heavy ion research has been the thrust to re-examine the assumptions of reaction theory and now include many effects neglected for light ion analysis. This research has spurred the addition of multistep processes to simple direct processes and coupled channel calculations. (J.R.)

  5. Heavy ion accelerator GANIL

    International Nuclear Information System (INIS)

    1975-04-01

    This article presents GANIL, a large national heavy ion accelerator. The broad problems of nuclear physics, atomic physics, astrophysics and physics of condensed media which can be approached and studied with this machine are discussed first, after which the final construction project is described. The project comprises a circular injector, a separated sector cyclotron up beam stripper, and a second separated cyclotron downstream [fr

  6. Requirements for Ion Sources

    International Nuclear Information System (INIS)

    Scrivens, R

    2013-01-01

    Ion sources produce beams for a large variety of different physical experiments, industrial processes and medical applications. In order to characterize the beam delivered by them, a list of requirements is necessary. In this chapter the list of principal requirements is specified and definitions for them are given. (author)

  7. Heavy ion fusion

    International Nuclear Information System (INIS)

    Hofmann, Ingo

    1993-01-01

    With controlled thermonuclear fusion holding out the possibility of a prolific and clean new source of energy, the goal remains elusive after many years of continual effort. While the conventional Tokamak route with magnetic confinement continues to hit the headlines, other alternatives are now becoming competitive. One possible solution is to confine the thermonuclear fuel pellet by high power beams. Current research and perspectives for future work in such inertial confinement was the subject of the 'Prospects for Heavy Ion Fusion' European Research Conference held in Aghia Pelaghia, Crete, last year. Its main focus was on the potential of heavy ion accelerators as well as recent advances in target physics with high power lasers and light ion beams. Carlo Rubbia declared that high energy accelerators, with their high efficiency, are the most promising approach to economical fusion energy production. However the need for cost saving in the driver accelerator requires new ideas in target design tailored to the particularities of heavy ion beams, which need to be pushed to the limits of high current and phase space density at the same time

  8. Industrial ion source technology

    Science.gov (United States)

    Kaufman, H. R.; Robinson, R. S.

    1978-01-01

    An analytical model was developed to describe the development of a coned surface texture with ion bombardment and simultaneous deposition of an impurity. A mathematical model of sputter deposition rate from a beveled target was developed in conjuction with the texturing models to provide an important input to that model. The establishment of a general procedure that will allow the treatment of manay different sputtering configurations is outlined. Calculation of cross sections for energetic binary collisions was extened to Ar, Kr.. and Xe with total cross sections for viscosity and diffusion calculated for the interaction energy range from leV to 1000eV. Physical sputtering and reactive ion etching experiments provided experimental data on the operating limits of a broad beam ion source using CF4 as a working gas to produce reactive species in a sputtering beam. Magnetic clustering effects are observed when Al is seeded with Fe and sputtered with Ar(?) ions. Silicon was textured at a micron scale by using a substrate temperature of 600 C.

  9. Ion implantation into diamond

    International Nuclear Information System (INIS)

    Sato, Susumu

    1994-01-01

    The graphitization and the change to amorphous state of diamond surface layer by ion implantation and its characteristics are reported. In the diamond surface, into which more than 10 16 ions/cm 2 was implanted, the diamond crystals are broken, and the structure changes to other carbon structure such as amorphous state or graphite. Accompanying this change of structure, the electric conductivity of the implanted layer shows two discontinuous values due to high resistance and low resistance. This control of structure can be done by the temperature of the base during the ion implantation into diamond. Also it is referred to that by the base temperature during implantation, the mutual change of the structure between amorphous state and graphite can be controlled. The change of the electric resistance and the optical characteristics by the ion implantation into diamond surface, the structural analysis by Raman spectroscopy, and the control of the structure of the implanted layer by the base temperature during implantation are reported. (K.I.)

  10. Application of ion exchangers

    International Nuclear Information System (INIS)

    Markhol, M.

    1985-01-01

    Existing methods of multi-element separation for radiochemical analysis are considered. The majority of existing methods is noted to be based on application of organic and inorganic ion exchangers. Distillation, coprecipitation, extraction as well as combination of the above methods are also used. Concrete flowsheets of multi-element separation are presented

  11. Heavy-ion microscopy

    International Nuclear Information System (INIS)

    Kraft, G.; Yang, T.C.H.; Richards, T.; Tobias, C.A.

    1980-01-01

    This chapter briefly describes the techniques of optical microscopy, scanning and transmission electron microscopy, soft x-ray microscopy and compares these latter techniques with heavy-ion microscopy. The resolution obtained with these various types of microscopy are compared and the influence of the etching procedure on total resolution is discussed. Several micrographs of mammalian cells are included

  12. Field ion microscopy

    International Nuclear Information System (INIS)

    Ramanathan, D.

    1975-01-01

    The basic features of the Field-Ion Microscope (FIM) and the theory of image formation are explained. Design parameters of the FIM, factors limiting its resolution, interpretation of the image, etc are briefly outlined. Relative merits of the various imaging gases and the applications of the FIM are also covered. (K.B.)

  13. Relativisitic heavy ion collisions

    International Nuclear Information System (INIS)

    Tannenbaum, M.J.

    1987-01-01

    Some of the objectives and observables of Relativistic Heavy Ion Physics are presented. The first experimental results from oxygen interactions at CERN, 200 GeV/c per nucleon, and BNL, 14.5 GeV/c per nucleon are shown. The data indicate more energy emission than was originally predicted. 25 refs., 19 figs

  14. Ion-Beam-Excited Electrostatic Ion Cyclotron Waves

    DEFF Research Database (Denmark)

    Michelsen, Poul; Pécseli, Hans; Juul Rasmussen, Jens

    1976-01-01

    Self-excited electrostatic ion cyclotron waves were observed in an ion-beam-plasma system produced in a DP-operated Q-machine. The frequency of the waves showed the theoretically predicted variation with the magnetic field.......Self-excited electrostatic ion cyclotron waves were observed in an ion-beam-plasma system produced in a DP-operated Q-machine. The frequency of the waves showed the theoretically predicted variation with the magnetic field....

  15. Compact RF ion source for industrial electrostatic ion accelerator

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, Hyeok-Jung, E-mail: hjkwon@kaeri.re.kr; Park, Sae-Hoon; Kim, Dae-Il; Cho, Yong-Sub [Korea Multi-purpose Accelerator Complex, Korea Atomic Energy Research Institute, Gyeongsangbukdo 38180 (Korea, Republic of)

    2016-02-15

    Korea Multi-purpose Accelerator Complex is developing a single-ended electrostatic ion accelerator to irradiate gaseous ions, such as hydrogen and nitrogen, on materials for industrial applications. ELV type high voltage power supply has been selected. Because of the limited space, electrical power, and robust operation, a 200 MHz RF ion source has been developed. In this paper, the accelerator system, test stand of the ion source, and its test results are described.

  16. Compact RF ion source for industrial electrostatic ion accelerator

    Science.gov (United States)

    Kwon, Hyeok-Jung; Park, Sae-Hoon; Kim, Dae-Il; Cho, Yong-Sub

    2016-02-01

    Korea Multi-purpose Accelerator Complex is developing a single-ended electrostatic ion accelerator to irradiate gaseous ions, such as hydrogen and nitrogen, on materials for industrial applications. ELV type high voltage power supply has been selected. Because of the limited space, electrical power, and robust operation, a 200 MHz RF ion source has been developed. In this paper, the accelerator system, test stand of the ion source, and its test results are described.

  17. Negative ion sources for tandem accelerator

    International Nuclear Information System (INIS)

    Minehara, Eisuke

    1980-08-01

    Four kinds of negative ion sources (direct extraction Duoplasmatron ion source, radial extraction Penniing ion source, lithium charge exchange ion source and Middleton-type sputter ion source) have been installed in the JAERI tandem accelerator. The ion sources can generate many negative ions ranging from Hydrogen to Uranium with the exception of Ne, Ar, Kr, Xe and Rn. Discussions presented in this report include mechanisms of negative ion formation, electron affinity and stability of negative ions, performance of the ion sources and materials used for negative ion production. Finally, the author will discuss difficult problems to be overcome in order to get any negative ion sufficiently. (author)

  18. Heavy-ion radiography and heavy-ion computed tomography

    International Nuclear Information System (INIS)

    Fabrikant, J.I.; Holley, W.R.; McFarland, E.W.; Tobias, C.a.

    1982-02-01

    Heavy-ion projection and CT radiography is being developed into a safe, low-dose, noninvasive radiological procedure that can quantitate and image small density differences in human tissues. The applications to heavy-ion mammography and heavy-ion CT imaging of the brain in clinical patients suggest their potential value in cancer diagnosis

  19. Using Ion Exchange Chromatography to Separate and Quantify Complex Ions

    Science.gov (United States)

    Johnson, Brian J.

    2014-01-01

    Ion exchange chromatography is an important technique in the separation of charged species, particularly in biological, inorganic, and environmental samples. In this experiment, students are supplied with a mixture of two substitution-inert complex ions. They separate the complexes by ion exchange chromatography using a "flash"…

  20. Recharging of a screened ion on the molecular ion

    International Nuclear Information System (INIS)

    Karbovanets, M.I.; Lazur, V.Yu.; Yudin, G.L.; Gosudarstvennyj Komitet po Ispol'zovaniyu Atomnoj Ehnergii SSSR, Obninsk. Fiziko-Ehnergeticheskij Inst.)

    1987-01-01

    Charge exchange of a screened ion on a molecular ion is studied in the Oppenheimer-Brinkman-Cramers approximation. To calculate ion exchange probabilities and cross sections summed over the final degenerated electron states method of Green functions analogous to that applied earlier in the direct Coulomb excitation theory and atomic ionization is developed