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Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies

BACKGROUND: Longitudinally extensive transverse myelitis (LETM) is classically related to aquaporin (AQP4)-antibodies (Ab) neuromyelitis optica spectrum disorders (NMOSD) or more recently to myelin oligodendrocyte glycoprotein (MOG)-Ab associated disease. However, some patients remain negative for a...

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Autores principales: Maillart, Elisabeth, Durand-Dubief, Françoise, Louapre, Céline, Audoin, Bertrand, Bourre, Bertrand, Derache, Nathalie, Ciron, Jonathan, Collongues, Nicolas, de Sèze, Jérome, Cohen, Mikael, Lebrun-Frenay, Christine, Hadhoum, Nawel, Zéphir, Hélène, Deschamps, Romain, Carra-Dallière, Clarisse, Labauge, Pierre, Kerschen, Philippe, Montcuquet, Alexis, Wiertlewski, Sandrine, Laplaud, David, Runavot, Gwenaëlle, Vukusic, Sandra, Papeix, Caroline, Marignier, Romain
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7178729/
https://www.ncbi.nlm.nih.gov/pubmed/32326965
http://dx.doi.org/10.1186/s12974-020-01773-w
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author Maillart, Elisabeth
Durand-Dubief, Françoise
Louapre, Céline
Audoin, Bertrand
Bourre, Bertrand
Derache, Nathalie
Ciron, Jonathan
Collongues, Nicolas
de Sèze, Jérome
Cohen, Mikael
Lebrun-Frenay, Christine
Hadhoum, Nawel
Zéphir, Hélène
Deschamps, Romain
Carra-Dallière, Clarisse
Labauge, Pierre
Kerschen, Philippe
Montcuquet, Alexis
Wiertlewski, Sandrine
Laplaud, David
Runavot, Gwenaëlle
Vukusic, Sandra
Papeix, Caroline
Marignier, Romain
author_facet Maillart, Elisabeth
Durand-Dubief, Françoise
Louapre, Céline
Audoin, Bertrand
Bourre, Bertrand
Derache, Nathalie
Ciron, Jonathan
Collongues, Nicolas
de Sèze, Jérome
Cohen, Mikael
Lebrun-Frenay, Christine
Hadhoum, Nawel
Zéphir, Hélène
Deschamps, Romain
Carra-Dallière, Clarisse
Labauge, Pierre
Kerschen, Philippe
Montcuquet, Alexis
Wiertlewski, Sandrine
Laplaud, David
Runavot, Gwenaëlle
Vukusic, Sandra
Papeix, Caroline
Marignier, Romain
author_sort Maillart, Elisabeth
collection PubMed
description BACKGROUND: Longitudinally extensive transverse myelitis (LETM) is classically related to aquaporin (AQP4)-antibodies (Ab) neuromyelitis optica spectrum disorders (NMOSD) or more recently to myelin oligodendrocyte glycoprotein (MOG)-Ab associated disease. However, some patients remain negative for any diagnosis, despite a large work-up including AQP4-Ab and MOG-Ab. Data about natural history, disability outcome, and treatment are limited in this group of patients. We aimed to (1) describe clinical, biological, and radiological features of double seronegative LETM patients; (2) assess the clinical course and identify prognostic factors; and (3) assess the risk of recurrence, according to maintenance immunosuppressive therapy. METHODS: Retrospective evaluation of patients with a first episode of LETM, tested negative for AQP-Ab and MOG-Ab, from the French nationwide observatory study NOMADMUS. RESULTS: Fifty-three patients (median age 38 years (range 16–80)) with double seronegative LETM were included. Median nadir EDSS at onset was 6.0 (1–8.5), associated to a median EDSS at last follow-up of 4.0 (0–8). Recurrence was observed in 24.5% of patients in the 18 following months, with a median time to first relapse of 5.7 months. The risk of recurrence was lower in the group of patients treated early with an immunosuppressive drug (2/22, 9%), in comparison with untreated patients (10/31, 32%). CONCLUSIONS: A first episode of a double seronegative LETM is associated to a severe outcome and a high rate of relapse in the following 18 months, suggesting that an early immunosuppressive treatment may be beneficial in that condition.
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spelling pubmed-71787292020-04-26 Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies Maillart, Elisabeth Durand-Dubief, Françoise Louapre, Céline Audoin, Bertrand Bourre, Bertrand Derache, Nathalie Ciron, Jonathan Collongues, Nicolas de Sèze, Jérome Cohen, Mikael Lebrun-Frenay, Christine Hadhoum, Nawel Zéphir, Hélène Deschamps, Romain Carra-Dallière, Clarisse Labauge, Pierre Kerschen, Philippe Montcuquet, Alexis Wiertlewski, Sandrine Laplaud, David Runavot, Gwenaëlle Vukusic, Sandra Papeix, Caroline Marignier, Romain J Neuroinflammation Research BACKGROUND: Longitudinally extensive transverse myelitis (LETM) is classically related to aquaporin (AQP4)-antibodies (Ab) neuromyelitis optica spectrum disorders (NMOSD) or more recently to myelin oligodendrocyte glycoprotein (MOG)-Ab associated disease. However, some patients remain negative for any diagnosis, despite a large work-up including AQP4-Ab and MOG-Ab. Data about natural history, disability outcome, and treatment are limited in this group of patients. We aimed to (1) describe clinical, biological, and radiological features of double seronegative LETM patients; (2) assess the clinical course and identify prognostic factors; and (3) assess the risk of recurrence, according to maintenance immunosuppressive therapy. METHODS: Retrospective evaluation of patients with a first episode of LETM, tested negative for AQP-Ab and MOG-Ab, from the French nationwide observatory study NOMADMUS. RESULTS: Fifty-three patients (median age 38 years (range 16–80)) with double seronegative LETM were included. Median nadir EDSS at onset was 6.0 (1–8.5), associated to a median EDSS at last follow-up of 4.0 (0–8). Recurrence was observed in 24.5% of patients in the 18 following months, with a median time to first relapse of 5.7 months. The risk of recurrence was lower in the group of patients treated early with an immunosuppressive drug (2/22, 9%), in comparison with untreated patients (10/31, 32%). CONCLUSIONS: A first episode of a double seronegative LETM is associated to a severe outcome and a high rate of relapse in the following 18 months, suggesting that an early immunosuppressive treatment may be beneficial in that condition. BioMed Central 2020-04-23 /pmc/articles/PMC7178729/ /pubmed/32326965 http://dx.doi.org/10.1186/s12974-020-01773-w Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Research
Maillart, Elisabeth
Durand-Dubief, Françoise
Louapre, Céline
Audoin, Bertrand
Bourre, Bertrand
Derache, Nathalie
Ciron, Jonathan
Collongues, Nicolas
de Sèze, Jérome
Cohen, Mikael
Lebrun-Frenay, Christine
Hadhoum, Nawel
Zéphir, Hélène
Deschamps, Romain
Carra-Dallière, Clarisse
Labauge, Pierre
Kerschen, Philippe
Montcuquet, Alexis
Wiertlewski, Sandrine
Laplaud, David
Runavot, Gwenaëlle
Vukusic, Sandra
Papeix, Caroline
Marignier, Romain
Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies
title Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies
title_full Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies
title_fullStr Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies
title_full_unstemmed Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies
title_short Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies
title_sort outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without aqp4/mog antibodies
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7178729/
https://www.ncbi.nlm.nih.gov/pubmed/32326965
http://dx.doi.org/10.1186/s12974-020-01773-w
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