AustralieFrV1, Main, Exploration, bibRecord, 002803

Status of diagnostic approaches to AQP4-IgG seronegative NMO and NMO/MS overlap syndromes

Identifieur interne : 002803 ( Main/Exploration ); précédent : 002802; suivant : 002804

Status of diagnostic approaches to AQP4-IgG seronegative NMO and NMO/MS overlap syndromes

Auteurs : Maciej Jury Czyk [Royaume-Uni, Pologne] ; Brian Weinshenker [États-Unis] ; Gulsen Akman-Demir [Turquie] ; Nasrin Asgari [Danemark] ; David Barnes [Royaume-Uni] ; Mike Boggild [Australie] ; Abhijit Chaudhuri [Royaume-Uni] ; Marie D Ooghe [Belgique] ; Nikos Evangelou [Royaume-Uni] ; Ruth Geraldes [Portugal] ; Zsolt Illes [Danemark] ; Anu Jacob [Royaume-Uni] ; Ho Jin Kim [Corée du Sud] ; Ingo Kleiter [Allemagne] ; Michael Levy [États-Unis] ; Romain Marignier [France] ; Christopher Mcguigan [Irlande (pays)] ; Katy Murray [Royaume-Uni] ; Ichiro Nakashima [Japon] ; Lekha Pandit [Inde] ; Friedemann Paul [Allemagne] ; Sean Pittock [États-Unis] ; Krzysztof Selmaj [Pologne] ; Jérôme De Sèze [France] ; Aksel Siva [Turquie] ; Radu Tanasescu [Royaume-Uni, Roumanie] ; Sandra Vukusic [France] ; Dean Wingerchuk [États-Unis] ; Damian Wren [Royaume-Uni] ; Isabel Leite [Royaume-Uni] ; Jacqueline Palace [Royaume-Uni]

Source :

Journal of neurology [ 0340-5354 ] ; 2015.

RBID : PMC:4816597

Descripteurs français

KwdFr :
- Aquaporine-4 (immunologie), Autoanticorps (sang), Encéphalomyélite aigüe disséminée (anatomopathologie), Encéphalomyélite aigüe disséminée (diagnostic), Encéphalomyélite aigüe disséminée (traitement médicamenteux), Humains, Immunoglobuline G (immunologie), Neuromyélite optique (anatomopathologie), Neuromyélite optique (diagnostic), Neuromyélite optique (traitement médicamenteux), Sclérose en plaques (anatomopathologie), Sclérose en plaques (diagnostic), Sclérose en plaques (traitement médicamenteux), Syndrome.
MESH :
- anatomopathologie : Encéphalomyélite aigüe disséminée, Neuromyélite optique, Sclérose en plaques.
- diagnostic : Encéphalomyélite aigüe disséminée, Neuromyélite optique, Sclérose en plaques.
- immunologie : Aquaporine-4, Immunoglobuline G.
- sang : Autoanticorps.
- traitement médicamenteux : Encéphalomyélite aigüe disséminée, Neuromyélite optique, Sclérose en plaques.
- Humains, Syndrome.

English descriptors

KwdEn :
- Aquaporin 4 (immunology), Autoantibodies (blood), Encephalomyelitis, Acute Disseminated (diagnosis), Encephalomyelitis, Acute Disseminated (drug therapy), Encephalomyelitis, Acute Disseminated (pathology), Humans, Immunoglobulin G (immunology), Multiple Sclerosis (diagnosis), Multiple Sclerosis (drug therapy), Multiple Sclerosis (pathology), Neuromyelitis Optica (diagnosis), Neuromyelitis Optica (drug therapy), Neuromyelitis Optica (pathology), Syndrome.
MESH :
- chemical , blood : Autoantibodies.
- chemical , immunology : Aquaporin 4, Immunoglobulin G.
- diagnosis : Encephalomyelitis, Acute Disseminated, Multiple Sclerosis, Neuromyelitis Optica.
- drug therapy : Encephalomyelitis, Acute Disseminated, Multiple Sclerosis, Neuromyelitis Optica.
- pathology : Encephalomyelitis, Acute Disseminated, Multiple Sclerosis, Neuromyelitis Optica.
- Humans, Syndrome.

Abstract

Distinguishing aquaporin-4 IgG(AQP4-IgG)-negative neuromyelitis optica spectrum disorders (NMOSD) from opticospinal predominant multiple sclerosis (MS) is a clinical challenge with important treatment implications. The objective of the study was to examine whether expert clinicians diagnose and treat NMO/MS overlapping patients in a similar way. 12 AQP4-IgG-negative patients were selected to cover the range of clinical scenarios encountered in an NMO clinic. 27 NMO and MS experts reviewed their clinical vignettes, including relevant imaging and laboratory tests. Diagnoses were categorized into four groups (NMO, MS, indeterminate, other) and management into three groups (MS drugs, immunosuppression, no treatment). The mean proportion of agreement for the diagnosis was low (p_o = 0.51) and ranged from 0.25 to 0.73 for individual patients. The majority opinion was divided between NMOSD versus: MS (nine cases), monophasic longitudinally extensive transverse myelitis (LETM) (1), acute disseminated encephalomyelitis (ADEM) (1) and recurrent isolated optic neuritis (RION) (1). Typical NMO features (e.g., LETM) influenced the diagnosis more than features more consistent with MS (e.g., short TM). Agreement on the treatment of patients was higher (p_o = 0.64) than that on the diagnosis with immunosuppression being the most common choice not only in patients with the diagnosis of NMO (98 %) but also in those indeterminate between NMO and MS (74 %). The diagnosis in AQP4-IgG-negative NMO/MS overlap syndromes is challenging and diverse. The classification of such patients currently requires new diagnostic categories, which incorporate lesser degrees of diagnostic confidence. Long-term follow-up may identify early features or biomarkers, which can more accurately distinguish the underlying disorder.

Url:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4816597

DOI: 10.1007/s00415-015-7952-8
PubMed: 26530512
PubMed Central: 4816597

Affiliations:

Le document en format XML

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<author><name sortKey="Jacob, Anu" sort="Jacob, Anu" uniqKey="Jacob A" first="Anu" last="Jacob">Anu Jacob</name>
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<author><name sortKey="Kim, Ho Jin" sort="Kim, Ho Jin" uniqKey="Kim H" first="Ho Jin" last="Kim">Ho Jin Kim</name>
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<author><name sortKey="Mcguigan, Christopher" sort="Mcguigan, Christopher" uniqKey="Mcguigan C" first="Christopher" last="Mcguigan">Christopher Mcguigan</name>
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<author><name sortKey="Murray, Katy" sort="Murray, Katy" uniqKey="Murray K" first="Katy" last="Murray">Katy Murray</name>
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<author><name sortKey="Nakashima, Ichiro" sort="Nakashima, Ichiro" uniqKey="Nakashima I" first="Ichiro" last="Nakashima">Ichiro Nakashima</name>
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<country xml:lang="fr">Japon</country>
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<author><name sortKey="Pandit, Lekha" sort="Pandit, Lekha" uniqKey="Pandit L" first="Lekha" last="Pandit">Lekha Pandit</name>
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<author><name sortKey="Paul, Friedemann" sort="Paul, Friedemann" uniqKey="Paul F" first="Friedemann" last="Paul">Friedemann Paul</name>
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<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Neurology, NeuroCure Clinical Research Center and Clinical and Experimental Multiple Sclerosis Research Center, Charité-Universitätsmedizin Berlin, Berlin</wicri:regionArea>
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<settlement type="city">Berlin</settlement>
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<author><name sortKey="Pittock, Sean" sort="Pittock, Sean" uniqKey="Pittock S" first="Sean" last="Pittock">Sean Pittock</name>
<affiliation wicri:level="2"><nlm:aff id="A22">Department of Neurology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Neurology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN</wicri:regionArea>
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</placeName>
</affiliation>
</author>
<author><name sortKey="Selmaj, Krzysztof" sort="Selmaj, Krzysztof" uniqKey="Selmaj K" first="Krzysztof" last="Selmaj">Krzysztof Selmaj</name>
<affiliation wicri:level="1"><nlm:aff id="A23">Department of Neurology, Medical University of Lodz, Lodz, Poland</nlm:aff>
<country xml:lang="fr">Pologne</country>
<wicri:regionArea>Department of Neurology, Medical University of Lodz, Lodz</wicri:regionArea>
<wicri:noRegion>Lodz</wicri:noRegion>
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</author>
<author><name sortKey="De Seze, Jerome" sort="De Seze, Jerome" uniqKey="De Seze J" first="Jérôme" last="De Sèze">Jérôme De Sèze</name>
<affiliation wicri:level="3"><nlm:aff id="A24">Neurology Service, University Hospital of Strasbourg, Strasbourg, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Neurology Service, University Hospital of Strasbourg, Strasbourg</wicri:regionArea>
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</affiliation>
</author>
<author><name sortKey="Siva, Aksel" sort="Siva, Aksel" uniqKey="Siva A" first="Aksel" last="Siva">Aksel Siva</name>
<affiliation wicri:level="1"><nlm:aff id="A25">Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey</nlm:aff>
<country xml:lang="fr">Turquie</country>
<wicri:regionArea>Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul</wicri:regionArea>
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</affiliation>
</author>
<author><name sortKey="Tanasescu, Radu" sort="Tanasescu, Radu" uniqKey="Tanasescu R" first="Radu" last="Tanasescu">Radu Tanasescu</name>
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<country xml:lang="fr">Royaume-Uni</country>
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</affiliation>
<affiliation wicri:level="1"><nlm:aff id="A26">Department of Neurology, Neurosurgery and Psychiatry, University of Medicine and Pharmacy Carol Davila, Bucharest, Romania</nlm:aff>
<country xml:lang="fr">Roumanie</country>
<wicri:regionArea>Department of Neurology, Neurosurgery and Psychiatry, University of Medicine and Pharmacy Carol Davila, Bucharest</wicri:regionArea>
<wicri:noRegion>Bucharest</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Vukusic, Sandra" sort="Vukusic, Sandra" uniqKey="Vukusic S" first="Sandra" last="Vukusic">Sandra Vukusic</name>
<affiliation wicri:level="1"><nlm:aff id="A16">Service de Neurologie A, Hopital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, 69677 Lyon, France</nlm:aff>
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<author><name sortKey="Wingerchuk, Dean" sort="Wingerchuk, Dean" uniqKey="Wingerchuk D" first="Dean" last="Wingerchuk">Dean Wingerchuk</name>
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<wicri:regionArea>Mayo Clinic Division of Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, 13400 E Shea BLVD, Scottsdale 85259, AZ</wicri:regionArea>
<placeName><region type="state">Arizona</region>
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<author><name sortKey="Wren, Damian" sort="Wren, Damian" uniqKey="Wren D" first="Damian" last="Wren">Damian Wren</name>
<affiliation wicri:level="3"><nlm:aff id="A5">Department of Neurology, Atkinson Morley’s Wing, St George’s Hospital, London, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Department of Neurology, Atkinson Morley’s Wing, St George’s Hospital, London</wicri:regionArea>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
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<author><name sortKey="Leite, Isabel" sort="Leite, Isabel" uniqKey="Leite I" first="Isabel" last="Leite">Isabel Leite</name>
<affiliation wicri:level="4"><nlm:aff id="A1">Nuffield Department of Clinical Neurosciences, Level 3, West Wing, John Radcliffe Hospital, University of Oxford, Headley Way, Headington, Oxford OX3 9DU, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Nuffield Department of Clinical Neurosciences, Level 3, West Wing, John Radcliffe Hospital, University of Oxford, Headley Way, Headington, Oxford OX3 9DU</wicri:regionArea>
<orgName type="university">Université d'Oxford</orgName>
<placeName><settlement type="city">Oxford</settlement>
<region type="nation">Angleterre</region>
<region type="région" nuts="1">Oxfordshire</region>
</placeName>
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<author><name sortKey="Palace, Jacqueline" sort="Palace, Jacqueline" uniqKey="Palace J" first="Jacqueline" last="Palace">Jacqueline Palace</name>
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<author><name sortKey="Jury Czyk, Maciej" sort="Jury Czyk, Maciej" uniqKey="Jury Czyk M" first="Maciej" last="Jury Czyk">Maciej Jury Czyk</name>
<affiliation wicri:level="4"><nlm:aff id="A1">Nuffield Department of Clinical Neurosciences, Level 3, West Wing, John Radcliffe Hospital, University of Oxford, Headley Way, Headington, Oxford OX3 9DU, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
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<affiliation wicri:level="1"><nlm:aff id="A23">Department of Neurology, Medical University of Lodz, Lodz, Poland</nlm:aff>
<country xml:lang="fr">Pologne</country>
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<author><name sortKey="Weinshenker, Brian" sort="Weinshenker, Brian" uniqKey="Weinshenker B" first="Brian" last="Weinshenker">Brian Weinshenker</name>
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<country xml:lang="fr">États-Unis</country>
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<placeName><region type="state">Minnesota</region>
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<author><name sortKey="Akman Demir, Gulsen" sort="Akman Demir, Gulsen" uniqKey="Akman Demir G" first="Gulsen" last="Akman-Demir">Gulsen Akman-Demir</name>
<affiliation wicri:level="1"><nlm:aff id="A3">Department of Neurology, School of Medicine, Istanbul Bilim University, Istanbul, Turkey</nlm:aff>
<country xml:lang="fr">Turquie</country>
<wicri:regionArea>Department of Neurology, School of Medicine, Istanbul Bilim University, Istanbul</wicri:regionArea>
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</affiliation>
</author>
<author><name sortKey="Asgari, Nasrin" sort="Asgari, Nasrin" uniqKey="Asgari N" first="Nasrin" last="Asgari">Nasrin Asgari</name>
<affiliation wicri:level="1"><nlm:aff id="A4">Department of Neurology, Vejle Hospital and Neurobiology, Institute of Molecular Medicine, University of Southern Denmark, Odense, Denmark</nlm:aff>
<country xml:lang="fr">Danemark</country>
<wicri:regionArea>Department of Neurology, Vejle Hospital and Neurobiology, Institute of Molecular Medicine, University of Southern Denmark, Odense</wicri:regionArea>
<wicri:noRegion>Odense</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Barnes, David" sort="Barnes, David" uniqKey="Barnes D" first="David" last="Barnes">David Barnes</name>
<affiliation wicri:level="3"><nlm:aff id="A5">Department of Neurology, Atkinson Morley’s Wing, St George’s Hospital, London, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Department of Neurology, Atkinson Morley’s Wing, St George’s Hospital, London</wicri:regionArea>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Boggild, Mike" sort="Boggild, Mike" uniqKey="Boggild M" first="Mike" last="Boggild">Mike Boggild</name>
<affiliation wicri:level="1"><nlm:aff id="A6">The Townsville Hospital, 100 Angus Smith Drive, Douglas Qld 4814, Douglas, Townsville, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>The Townsville Hospital, 100 Angus Smith Drive, Douglas Qld 4814, Douglas, Townsville</wicri:regionArea>
<wicri:noRegion>Townsville</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Chaudhuri, Abhijit" sort="Chaudhuri, Abhijit" uniqKey="Chaudhuri A" first="Abhijit" last="Chaudhuri">Abhijit Chaudhuri</name>
<affiliation wicri:level="1"><nlm:aff id="A7">Department of Neurology, Queens Hospital Rom Valley Way, Romford RM7 0AG, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Department of Neurology, Queens Hospital Rom Valley Way, Romford RM7 0AG</wicri:regionArea>
<wicri:noRegion>Romford RM7 0AG</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="D Ooghe, Marie" sort="D Ooghe, Marie" uniqKey="D Ooghe M" first="Marie" last="D Ooghe">Marie D Ooghe</name>
<affiliation wicri:level="3"><nlm:aff id="A8">Department of Neurology, University Hospital Brussel, Vrije Universiteit Brussel, Brussels, Belgium</nlm:aff>
<country xml:lang="fr">Belgique</country>
<wicri:regionArea>Department of Neurology, University Hospital Brussel, Vrije Universiteit Brussel, Brussels</wicri:regionArea>
<placeName><settlement type="city">Bruxelles</settlement>
<region nuts="2">Région de Bruxelles-Capitale</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Evangelou, Nikos" sort="Evangelou, Nikos" uniqKey="Evangelou N" first="Nikos" last="Evangelou">Nikos Evangelou</name>
<affiliation wicri:level="4"><nlm:aff id="A9">Division of Clinical Neuroscience, Queens Medical Center, University of Nottingham, Derby Road, Nottingham NG7 2UH, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Division of Clinical Neuroscience, Queens Medical Center, University of Nottingham, Derby Road, Nottingham NG7 2UH</wicri:regionArea>
<orgName type="university">Université de Nottingham</orgName>
<placeName><settlement type="city">Nottingham</settlement>
<region type="nation">Angleterre</region>
<region type="région" nuts="1">Nottinghamshire</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Geraldes, Ruth" sort="Geraldes, Ruth" uniqKey="Geraldes R" first="Ruth" last="Geraldes">Ruth Geraldes</name>
<affiliation wicri:level="1"><nlm:aff id="A10">Neuroscience Department, Santa Maria Hospital, University of Lisbon, Lisbon, Portugal</nlm:aff>
<country xml:lang="fr">Portugal</country>
<wicri:regionArea>Neuroscience Department, Santa Maria Hospital, University of Lisbon, Lisbon</wicri:regionArea>
<wicri:noRegion>Lisbon</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Illes, Zsolt" sort="Illes, Zsolt" uniqKey="Illes Z" first="Zsolt" last="Illes">Zsolt Illes</name>
<affiliation wicri:level="1"><nlm:aff id="A11">Department of Neurology, Institute of Clinical Research, Odense, Odense University Hospital, University of Southern Denmark, Odense, Denmark</nlm:aff>
<country xml:lang="fr">Danemark</country>
<wicri:regionArea>Department of Neurology, Institute of Clinical Research, Odense, Odense University Hospital, University of Southern Denmark, Odense</wicri:regionArea>
<wicri:noRegion>Odense</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Jacob, Anu" sort="Jacob, Anu" uniqKey="Jacob A" first="Anu" last="Jacob">Anu Jacob</name>
<affiliation wicri:level="1"><nlm:aff id="A12">NMO Clinical Service, The Walton Centre, Liverpool, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>NMO Clinical Service, The Walton Centre, Liverpool</wicri:regionArea>
<wicri:noRegion>Liverpool</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Kim, Ho Jin" sort="Kim, Ho Jin" uniqKey="Kim H" first="Ho Jin" last="Kim">Ho Jin Kim</name>
<affiliation wicri:level="3"><nlm:aff id="A13">Department of Neurology, National Cancer Center, Seoul, South Korea</nlm:aff>
<country xml:lang="fr">Corée du Sud</country>
<wicri:regionArea>Department of Neurology, National Cancer Center, Seoul</wicri:regionArea>
<placeName><settlement type="city">Séoul</settlement>
<region type="capital">Région capitale de Séoul</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Kleiter, Ingo" sort="Kleiter, Ingo" uniqKey="Kleiter I" first="Ingo" last="Kleiter">Ingo Kleiter</name>
<affiliation wicri:level="1"><nlm:aff id="A14">Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany</nlm:aff>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum</wicri:regionArea>
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<wicri:noRegion>Bochum</wicri:noRegion>
<wicri:noRegion>Bochum</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Levy, Michael" sort="Levy, Michael" uniqKey="Levy M" first="Michael" last="Levy">Michael Levy</name>
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<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Neuromyelitis Optica Clinic, John Hopkins University, 1800 E. Orleans St., Baltimore, MD 21287</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Marignier, Romain" sort="Marignier, Romain" uniqKey="Marignier R" first="Romain" last="Marignier">Romain Marignier</name>
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<country xml:lang="fr">France</country>
<wicri:regionArea>Service de Neurologie A, Hopital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, 69677 Lyon</wicri:regionArea>
<placeName><region type="region" nuts="2">Auvergne-Rhône-Alpes</region>
<region type="old region" nuts="2">Rhône-Alpes</region>
<settlement type="city">Lyon</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Mcguigan, Christopher" sort="Mcguigan, Christopher" uniqKey="Mcguigan C" first="Christopher" last="Mcguigan">Christopher Mcguigan</name>
<affiliation wicri:level="1"><nlm:aff id="A17">University College Dublin, St. Vincent’s University Hospital, Elm Park, Dublin 4, Ireland</nlm:aff>
<country xml:lang="fr">Irlande (pays)</country>
<wicri:regionArea>University College Dublin, St. Vincent’s University Hospital, Elm Park, Dublin 4</wicri:regionArea>
<wicri:noRegion>Dublin 4</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Murray, Katy" sort="Murray, Katy" uniqKey="Murray K" first="Katy" last="Murray">Katy Murray</name>
<affiliation wicri:level="4"><nlm:aff id="A18">Anne Rowling Regenerative Neurology Clinic, University of Edinburgh, Chancellor’s Building, Edinburgh EH16 4SB, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Anne Rowling Regenerative Neurology Clinic, University of Edinburgh, Chancellor’s Building, Edinburgh EH16 4SB</wicri:regionArea>
<orgName type="university">Université d'Édimbourg</orgName>
<placeName><settlement type="city">Édimbourg</settlement>
<region type="country">Écosse</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Nakashima, Ichiro" sort="Nakashima, Ichiro" uniqKey="Nakashima I" first="Ichiro" last="Nakashima">Ichiro Nakashima</name>
<affiliation wicri:level="1"><nlm:aff id="A19">Department of Neurology, Tohoku University School of Medicine, Sendai 980-8574, Japan</nlm:aff>
<country xml:lang="fr">Japon</country>
<wicri:regionArea>Department of Neurology, Tohoku University School of Medicine, Sendai 980-8574</wicri:regionArea>
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</affiliation>
</author>
<author><name sortKey="Pandit, Lekha" sort="Pandit, Lekha" uniqKey="Pandit L" first="Lekha" last="Pandit">Lekha Pandit</name>
<affiliation wicri:level="1"><nlm:aff id="A20">Nitte University, Mangalore 575018, Karnataka, India</nlm:aff>
<country xml:lang="fr">Inde</country>
<wicri:regionArea>Nitte University, Mangalore 575018, Karnataka</wicri:regionArea>
<wicri:noRegion>Karnataka</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Paul, Friedemann" sort="Paul, Friedemann" uniqKey="Paul F" first="Friedemann" last="Paul">Friedemann Paul</name>
<affiliation wicri:level="3"><nlm:aff id="A21">Department of Neurology, NeuroCure Clinical Research Center and Clinical and Experimental Multiple Sclerosis Research Center, Charité-Universitätsmedizin Berlin, Berlin, Germany</nlm:aff>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Neurology, NeuroCure Clinical Research Center and Clinical and Experimental Multiple Sclerosis Research Center, Charité-Universitätsmedizin Berlin, Berlin</wicri:regionArea>
<placeName><region type="land" nuts="3">Berlin</region>
<settlement type="city">Berlin</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Pittock, Sean" sort="Pittock, Sean" uniqKey="Pittock S" first="Sean" last="Pittock">Sean Pittock</name>
<affiliation wicri:level="2"><nlm:aff id="A22">Department of Neurology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Neurology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN</wicri:regionArea>
<placeName><region type="state">Minnesota</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Selmaj, Krzysztof" sort="Selmaj, Krzysztof" uniqKey="Selmaj K" first="Krzysztof" last="Selmaj">Krzysztof Selmaj</name>
<affiliation wicri:level="1"><nlm:aff id="A23">Department of Neurology, Medical University of Lodz, Lodz, Poland</nlm:aff>
<country xml:lang="fr">Pologne</country>
<wicri:regionArea>Department of Neurology, Medical University of Lodz, Lodz</wicri:regionArea>
<wicri:noRegion>Lodz</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="De Seze, Jerome" sort="De Seze, Jerome" uniqKey="De Seze J" first="Jérôme" last="De Sèze">Jérôme De Sèze</name>
<affiliation wicri:level="3"><nlm:aff id="A24">Neurology Service, University Hospital of Strasbourg, Strasbourg, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Neurology Service, University Hospital of Strasbourg, Strasbourg</wicri:regionArea>
<placeName><region type="region">Grand Est</region>
<region type="old region">Alsace (région administrative)</region>
<settlement type="city">Strasbourg</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Siva, Aksel" sort="Siva, Aksel" uniqKey="Siva A" first="Aksel" last="Siva">Aksel Siva</name>
<affiliation wicri:level="1"><nlm:aff id="A25">Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey</nlm:aff>
<country xml:lang="fr">Turquie</country>
<wicri:regionArea>Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul</wicri:regionArea>
<wicri:noRegion>Istanbul</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Tanasescu, Radu" sort="Tanasescu, Radu" uniqKey="Tanasescu R" first="Radu" last="Tanasescu">Radu Tanasescu</name>
<affiliation wicri:level="4"><nlm:aff id="A9">Division of Clinical Neuroscience, Queens Medical Center, University of Nottingham, Derby Road, Nottingham NG7 2UH, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Division of Clinical Neuroscience, Queens Medical Center, University of Nottingham, Derby Road, Nottingham NG7 2UH</wicri:regionArea>
<orgName type="university">Université de Nottingham</orgName>
<placeName><settlement type="city">Nottingham</settlement>
<region type="nation">Angleterre</region>
<region type="région" nuts="1">Nottinghamshire</region>
</placeName>
</affiliation>
<affiliation wicri:level="1"><nlm:aff id="A26">Department of Neurology, Neurosurgery and Psychiatry, University of Medicine and Pharmacy Carol Davila, Bucharest, Romania</nlm:aff>
<country xml:lang="fr">Roumanie</country>
<wicri:regionArea>Department of Neurology, Neurosurgery and Psychiatry, University of Medicine and Pharmacy Carol Davila, Bucharest</wicri:regionArea>
<wicri:noRegion>Bucharest</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Vukusic, Sandra" sort="Vukusic, Sandra" uniqKey="Vukusic S" first="Sandra" last="Vukusic">Sandra Vukusic</name>
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<country xml:lang="fr">France</country>
<wicri:regionArea>Service de Neurologie A, Hopital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, 69677 Lyon</wicri:regionArea>
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<placeName><settlement type="city">Lyon</settlement>
<region type="region" nuts="2">Auvergne-Rhône-Alpes</region>
<region type="old region" nuts="2">Rhône-Alpes</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Wingerchuk, Dean" sort="Wingerchuk, Dean" uniqKey="Wingerchuk D" first="Dean" last="Wingerchuk">Dean Wingerchuk</name>
<affiliation wicri:level="2"><nlm:aff id="A27">Mayo Clinic Division of Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, 13400 E Shea BLVD, Scottsdale 85259, AZ, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Mayo Clinic Division of Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, 13400 E Shea BLVD, Scottsdale 85259, AZ</wicri:regionArea>
<placeName><region type="state">Arizona</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Wren, Damian" sort="Wren, Damian" uniqKey="Wren D" first="Damian" last="Wren">Damian Wren</name>
<affiliation wicri:level="3"><nlm:aff id="A5">Department of Neurology, Atkinson Morley’s Wing, St George’s Hospital, London, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Department of Neurology, Atkinson Morley’s Wing, St George’s Hospital, London</wicri:regionArea>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Leite, Isabel" sort="Leite, Isabel" uniqKey="Leite I" first="Isabel" last="Leite">Isabel Leite</name>
<affiliation wicri:level="4"><nlm:aff id="A1">Nuffield Department of Clinical Neurosciences, Level 3, West Wing, John Radcliffe Hospital, University of Oxford, Headley Way, Headington, Oxford OX3 9DU, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Nuffield Department of Clinical Neurosciences, Level 3, West Wing, John Radcliffe Hospital, University of Oxford, Headley Way, Headington, Oxford OX3 9DU</wicri:regionArea>
<orgName type="university">Université d'Oxford</orgName>
<placeName><settlement type="city">Oxford</settlement>
<region type="nation">Angleterre</region>
<region type="région" nuts="1">Oxfordshire</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Palace, Jacqueline" sort="Palace, Jacqueline" uniqKey="Palace J" first="Jacqueline" last="Palace">Jacqueline Palace</name>
<affiliation wicri:level="4"><nlm:aff id="A1">Nuffield Department of Clinical Neurosciences, Level 3, West Wing, John Radcliffe Hospital, University of Oxford, Headley Way, Headington, Oxford OX3 9DU, UK</nlm:aff>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Nuffield Department of Clinical Neurosciences, Level 3, West Wing, John Radcliffe Hospital, University of Oxford, Headley Way, Headington, Oxford OX3 9DU</wicri:regionArea>
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<placeName><settlement type="city">Oxford</settlement>
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<region type="région" nuts="1">Oxfordshire</region>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j">Journal of neurology</title>
<idno type="ISSN">0340-5354</idno>
<idno type="eISSN">1432-1459</idno>
<imprint><date when="2015">2015</date>
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<term>Autoantibodies (blood)</term>
<term>Encephalomyelitis, Acute Disseminated (diagnosis)</term>
<term>Encephalomyelitis, Acute Disseminated (drug therapy)</term>
<term>Encephalomyelitis, Acute Disseminated (pathology)</term>
<term>Humans</term>
<term>Immunoglobulin G (immunology)</term>
<term>Multiple Sclerosis (diagnosis)</term>
<term>Multiple Sclerosis (drug therapy)</term>
<term>Multiple Sclerosis (pathology)</term>
<term>Neuromyelitis Optica (diagnosis)</term>
<term>Neuromyelitis Optica (drug therapy)</term>
<term>Neuromyelitis Optica (pathology)</term>
<term>Syndrome</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Aquaporine-4 (immunologie)</term>
<term>Autoanticorps (sang)</term>
<term>Encéphalomyélite aigüe disséminée (anatomopathologie)</term>
<term>Encéphalomyélite aigüe disséminée (diagnostic)</term>
<term>Encéphalomyélite aigüe disséminée (traitement médicamenteux)</term>
<term>Humains</term>
<term>Immunoglobuline G (immunologie)</term>
<term>Neuromyélite optique (anatomopathologie)</term>
<term>Neuromyélite optique (diagnostic)</term>
<term>Neuromyélite optique (traitement médicamenteux)</term>
<term>Sclérose en plaques (anatomopathologie)</term>
<term>Sclérose en plaques (diagnostic)</term>
<term>Sclérose en plaques (traitement médicamenteux)</term>
<term>Syndrome</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="blood" xml:lang="en"><term>Autoantibodies</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="immunology" xml:lang="en"><term>Aquaporin 4</term>
<term>Immunoglobulin G</term>
</keywords>
<keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Encéphalomyélite aigüe disséminée</term>
<term>Neuromyélite optique</term>
<term>Sclérose en plaques</term>
</keywords>
<keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Encephalomyelitis, Acute Disseminated</term>
<term>Multiple Sclerosis</term>
<term>Neuromyelitis Optica</term>
</keywords>
<keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Encéphalomyélite aigüe disséminée</term>
<term>Neuromyélite optique</term>
<term>Sclérose en plaques</term>
</keywords>
<keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Encephalomyelitis, Acute Disseminated</term>
<term>Multiple Sclerosis</term>
<term>Neuromyelitis Optica</term>
</keywords>
<keywords scheme="MESH" qualifier="immunologie" xml:lang="fr"><term>Aquaporine-4</term>
<term>Immunoglobuline G</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Encephalomyelitis, Acute Disseminated</term>
<term>Multiple Sclerosis</term>
<term>Neuromyelitis Optica</term>
</keywords>
<keywords scheme="MESH" qualifier="sang" xml:lang="fr"><term>Autoanticorps</term>
</keywords>
<keywords scheme="MESH" qualifier="traitement médicamenteux" xml:lang="fr"><term>Encéphalomyélite aigüe disséminée</term>
<term>Neuromyélite optique</term>
<term>Sclérose en plaques</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Humans</term>
<term>Syndrome</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Humains</term>
<term>Syndrome</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en"><p id="P1">Distinguishing aquaporin-4 IgG(AQP4-IgG)-negative neuromyelitis optica spectrum disorders (NMOSD) from opticospinal predominant multiple sclerosis (MS) is a clinical challenge with important treatment implications. The objective of the study was to examine whether expert clinicians diagnose and treat NMO/MS overlapping patients in a similar way. 12 AQP4-IgG-negative patients were selected to cover the range of clinical scenarios encountered in an NMO clinic. 27 NMO and MS experts reviewed their clinical vignettes, including relevant imaging and laboratory tests. Diagnoses were categorized into four groups (NMO, MS, indeterminate, other) and management into three groups (MS drugs, immunosuppression, no treatment). The mean proportion of agreement for the diagnosis was low (<italic>p</italic>
<sub>o</sub>
 = 0.51) and ranged from 0.25 to 0.73 for individual patients. The majority opinion was divided between NMOSD versus: MS (nine cases), monophasic longitudinally extensive transverse myelitis (LETM) (1), acute disseminated encephalomyelitis (ADEM) (1) and recurrent isolated optic neuritis (RION) (1). Typical NMO features (e.g., LETM) influenced the diagnosis more than features more consistent with MS (e.g., short TM). Agreement on the treatment of patients was higher (<italic>p</italic>
<sub>o</sub>
 = 0.64) than that on the diagnosis with immunosuppression being the most common choice not only in patients with the diagnosis of NMO (98 %) but also in those indeterminate between NMO and MS (74 %). The diagnosis in AQP4-IgG-negative NMO/MS overlap syndromes is challenging and diverse. The classification of such patients currently requires new diagnostic categories, which incorporate lesser degrees of diagnostic confidence. Long-term follow-up may identify early features or biomarkers, which can more accurately distinguish the underlying disorder.</p>
</div>
</front>
</TEI>
<affiliations><list><country><li>Allemagne</li>
<li>Australie</li>
<li>Belgique</li>
<li>Corée du Sud</li>
<li>Danemark</li>
<li>France</li>
<li>Inde</li>
<li>Irlande (pays)</li>
<li>Japon</li>
<li>Pologne</li>
<li>Portugal</li>
<li>Roumanie</li>
<li>Royaume-Uni</li>
<li>Turquie</li>
<li>États-Unis</li>
</country>
<region><li>Alsace (région administrative)</li>
<li>Angleterre</li>
<li>Arizona</li>
<li>Auvergne-Rhône-Alpes</li>
<li>Berlin</li>
<li>Grand Est</li>
<li>Grand Londres</li>
<li>Maryland</li>
<li>Minnesota</li>
<li>Nottinghamshire</li>
<li>Oxfordshire</li>
<li>Rhône-Alpes</li>
<li>Région capitale de Séoul</li>
<li>Région de Bruxelles-Capitale</li>
<li>Écosse</li>
</region>
<settlement><li>Berlin</li>
<li>Bruxelles</li>
<li>Londres</li>
<li>Lyon</li>
<li>Nottingham</li>
<li>Oxford</li>
<li>Strasbourg</li>
<li>Séoul</li>
<li>Édimbourg</li>
</settlement>
<orgName><li>Université d'Oxford</li>
<li>Université d'Édimbourg</li>
<li>Université de Nottingham</li>
</orgName>
</list>
<tree><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Jury Czyk, Maciej" sort="Jury Czyk, Maciej" uniqKey="Jury Czyk M" first="Maciej" last="Jury Czyk">Maciej Jury Czyk</name>
</region>
<name sortKey="Barnes, David" sort="Barnes, David" uniqKey="Barnes D" first="David" last="Barnes">David Barnes</name>
<name sortKey="Chaudhuri, Abhijit" sort="Chaudhuri, Abhijit" uniqKey="Chaudhuri A" first="Abhijit" last="Chaudhuri">Abhijit Chaudhuri</name>
<name sortKey="Evangelou, Nikos" sort="Evangelou, Nikos" uniqKey="Evangelou N" first="Nikos" last="Evangelou">Nikos Evangelou</name>
<name sortKey="Jacob, Anu" sort="Jacob, Anu" uniqKey="Jacob A" first="Anu" last="Jacob">Anu Jacob</name>
<name sortKey="Leite, Isabel" sort="Leite, Isabel" uniqKey="Leite I" first="Isabel" last="Leite">Isabel Leite</name>
<name sortKey="Murray, Katy" sort="Murray, Katy" uniqKey="Murray K" first="Katy" last="Murray">Katy Murray</name>
<name sortKey="Palace, Jacqueline" sort="Palace, Jacqueline" uniqKey="Palace J" first="Jacqueline" last="Palace">Jacqueline Palace</name>
<name sortKey="Tanasescu, Radu" sort="Tanasescu, Radu" uniqKey="Tanasescu R" first="Radu" last="Tanasescu">Radu Tanasescu</name>
<name sortKey="Wren, Damian" sort="Wren, Damian" uniqKey="Wren D" first="Damian" last="Wren">Damian Wren</name>
</country>
<country name="Pologne"><noRegion><name sortKey="Jury Czyk, Maciej" sort="Jury Czyk, Maciej" uniqKey="Jury Czyk M" first="Maciej" last="Jury Czyk">Maciej Jury Czyk</name>
</noRegion>
<name sortKey="Selmaj, Krzysztof" sort="Selmaj, Krzysztof" uniqKey="Selmaj K" first="Krzysztof" last="Selmaj">Krzysztof Selmaj</name>
</country>
<country name="États-Unis"><region name="Minnesota"><name sortKey="Weinshenker, Brian" sort="Weinshenker, Brian" uniqKey="Weinshenker B" first="Brian" last="Weinshenker">Brian Weinshenker</name>
</region>
<name sortKey="Levy, Michael" sort="Levy, Michael" uniqKey="Levy M" first="Michael" last="Levy">Michael Levy</name>
<name sortKey="Pittock, Sean" sort="Pittock, Sean" uniqKey="Pittock S" first="Sean" last="Pittock">Sean Pittock</name>
<name sortKey="Wingerchuk, Dean" sort="Wingerchuk, Dean" uniqKey="Wingerchuk D" first="Dean" last="Wingerchuk">Dean Wingerchuk</name>
</country>
<country name="Turquie"><noRegion><name sortKey="Akman Demir, Gulsen" sort="Akman Demir, Gulsen" uniqKey="Akman Demir G" first="Gulsen" last="Akman-Demir">Gulsen Akman-Demir</name>
</noRegion>
<name sortKey="Siva, Aksel" sort="Siva, Aksel" uniqKey="Siva A" first="Aksel" last="Siva">Aksel Siva</name>
</country>
<country name="Danemark"><noRegion><name sortKey="Asgari, Nasrin" sort="Asgari, Nasrin" uniqKey="Asgari N" first="Nasrin" last="Asgari">Nasrin Asgari</name>
</noRegion>
<name sortKey="Illes, Zsolt" sort="Illes, Zsolt" uniqKey="Illes Z" first="Zsolt" last="Illes">Zsolt Illes</name>
</country>
<country name="Australie"><noRegion><name sortKey="Boggild, Mike" sort="Boggild, Mike" uniqKey="Boggild M" first="Mike" last="Boggild">Mike Boggild</name>
</noRegion>
</country>
<country name="Belgique"><region name="Région de Bruxelles-Capitale"><name sortKey="D Ooghe, Marie" sort="D Ooghe, Marie" uniqKey="D Ooghe M" first="Marie" last="D Ooghe">Marie D Ooghe</name>
</region>
</country>
<country name="Portugal"><noRegion><name sortKey="Geraldes, Ruth" sort="Geraldes, Ruth" uniqKey="Geraldes R" first="Ruth" last="Geraldes">Ruth Geraldes</name>
</noRegion>
</country>
<country name="Corée du Sud"><region name="Région capitale de Séoul"><name sortKey="Kim, Ho Jin" sort="Kim, Ho Jin" uniqKey="Kim H" first="Ho Jin" last="Kim">Ho Jin Kim</name>
</region>
</country>
<country name="Allemagne"><noRegion><name sortKey="Kleiter, Ingo" sort="Kleiter, Ingo" uniqKey="Kleiter I" first="Ingo" last="Kleiter">Ingo Kleiter</name>
</noRegion>
<name sortKey="Paul, Friedemann" sort="Paul, Friedemann" uniqKey="Paul F" first="Friedemann" last="Paul">Friedemann Paul</name>
</country>
<country name="France"><region name="Auvergne-Rhône-Alpes"><name sortKey="Marignier, Romain" sort="Marignier, Romain" uniqKey="Marignier R" first="Romain" last="Marignier">Romain Marignier</name>
</region>
<name sortKey="De Seze, Jerome" sort="De Seze, Jerome" uniqKey="De Seze J" first="Jérôme" last="De Sèze">Jérôme De Sèze</name>
<name sortKey="Vukusic, Sandra" sort="Vukusic, Sandra" uniqKey="Vukusic S" first="Sandra" last="Vukusic">Sandra Vukusic</name>
</country>
<country name="Irlande (pays)"><noRegion><name sortKey="Mcguigan, Christopher" sort="Mcguigan, Christopher" uniqKey="Mcguigan C" first="Christopher" last="Mcguigan">Christopher Mcguigan</name>
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</country>
<country name="Japon"><noRegion><name sortKey="Nakashima, Ichiro" sort="Nakashima, Ichiro" uniqKey="Nakashima I" first="Ichiro" last="Nakashima">Ichiro Nakashima</name>
</noRegion>
</country>
<country name="Inde"><noRegion><name sortKey="Pandit, Lekha" sort="Pandit, Lekha" uniqKey="Pandit L" first="Lekha" last="Pandit">Lekha Pandit</name>
</noRegion>
</country>
<country name="Roumanie"><noRegion><name sortKey="Tanasescu, Radu" sort="Tanasescu, Radu" uniqKey="Tanasescu R" first="Radu" last="Tanasescu">Radu Tanasescu</name>
</noRegion>
</country>
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</affiliations>
</record>

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Status of diagnostic approaches to AQP4-IgG seronegative NMO and NMO/MS overlap syndromes

Status of diagnostic approaches to AQP4-IgG seronegative NMO and NMO/MS overlap syndromes

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