Outcome and risk of recurrence in a large ...
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Article dans une revue scientifique: Article original
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Title :
Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without aqp4/mog antibodies
Author(s) :
Maillart, Elisabeth [Auteur]
Durand-Dubief, Francoise [Auteur]
Louapre, Celine [Auteur]
Audoin, Bertrand [Auteur]
Bourre, Bertrand [Auteur]
Derache, Nathalie [Auteur]
Ciron, Jonathan [Auteur]
Collongues, Nicolas [Auteur]
De Seze, Jerome [Auteur]
Cohen, Mikael [Auteur]
Lebrun-Frenay, Christine [Auteur]
Hadhoum, Nawal [Auteur]
ZEPHIR, Helene [Auteur]
Lille Neurosciences & Cognition (LilNCog) - U 1172
Deschamps, Romain [Auteur]
Carra-Dalliere, Clarisse [Auteur]
Labauge, Pierre [Auteur]
Kerschen, Philippe [Auteur]
Montcuquet, Alexis [Auteur]
Wiertlewski, Sandrine [Auteur]
Laplaud, David [Auteur]
Runavot, Gwenaelle [Auteur]
Vukusic, Sandra [Auteur]
Papeix, Caroline [Auteur]
Marignier, Romain [Auteur]
Durand-Dubief, Francoise [Auteur]
Louapre, Celine [Auteur]
Audoin, Bertrand [Auteur]
Bourre, Bertrand [Auteur]
Derache, Nathalie [Auteur]
Ciron, Jonathan [Auteur]
Collongues, Nicolas [Auteur]
De Seze, Jerome [Auteur]
Cohen, Mikael [Auteur]
Lebrun-Frenay, Christine [Auteur]
Hadhoum, Nawal [Auteur]
ZEPHIR, Helene [Auteur]
Lille Neurosciences & Cognition (LilNCog) - U 1172
Deschamps, Romain [Auteur]
Carra-Dalliere, Clarisse [Auteur]
Labauge, Pierre [Auteur]
Kerschen, Philippe [Auteur]
Montcuquet, Alexis [Auteur]
Wiertlewski, Sandrine [Auteur]
Laplaud, David [Auteur]
Runavot, Gwenaelle [Auteur]
Vukusic, Sandra [Auteur]
Papeix, Caroline [Auteur]
Marignier, Romain [Auteur]
Journal title :
Journal of neuroinflammation
Abbreviated title :
J Neuroinflammation
Volume number :
17
Pages :
128
Publication date :
2020-04-23
ISSN :
1742-2094
Keyword(s) :
Seronegative
Treatment
Outcome
Neuromyelitis optica
Longitudinally extensive transverse myelitis
Treatment
Outcome
Neuromyelitis optica
Longitudinally extensive transverse myelitis
HAL domain(s) :
Sciences du Vivant [q-bio]
English abstract : [en]
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 ...
Show more >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.Show less >
Show more >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.Show less >
Language :
Anglais
Audience :
Internationale
Popular science :
Non
Administrative institution(s) :
CHU Lille
Inserm
Université de Lille
Inserm
Université de Lille
Collections :
Research team(s) :
Neuroinflammation & Multiple Sclerosis (NEMESIS)
Submission date :
2021-06-23T13:45:57Z