Author: "Romain Marignier" / Topic: neurology (clinical) - Searchworks@Jio Institute Digital Library Search Results

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1. Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease

Author: Brenda Banwell, Jeffrey L Bennett, Romain Marignier, Ho Jin Kim, Fabienne Brilot, Eoin P Flanagan, Sudarshini Ramanathan, Patrick Waters, Silvia Tenembaum, Jennifer S Graves, Tanuja Chitnis, Alexander U Brandt, Cheryl Hemingway, Rinze Neuteboom, Lekha Pandit, Markus Reindl, Albert Saiz, Douglas Kazutoshi Sato, Kevin Rostasy, Friedemann Paul, Sean J Pittock, Kazuo Fujihara, and Jacqueline Palace
Subjects: Neurology (clinical)
Abstract: Serum antibodies directed against myelin oligodendrocyte glycoprotein (MOG) are found in patients with acquired CNS demyelinating syndromes that are distinct from multiple sclerosis and aquaporin-4-seropositive neuromyelitis optica spectrum disorder. Based on an extensive literature review and a structured consensus process, we propose diagnostic criteria for MOG antibody-associated disease (MOGAD) in which the presence of MOG-IgG is a core criterion. According to our proposed criteria, MOGAD is typically associated with acute disseminated encephalomyelitis, optic neuritis, or transverse myelitis, and is less commonly associated with cerebral cortical encephalitis, brainstem presentations, or cerebellar presentations. MOGAD can present as either a monophasic or relapsing disease course, and MOG-IgG cell-based assays are important for diagnostic accuracy. Diagnoses such as multiple sclerosis need to be excluded, but not all patients with multiple sclerosis should undergo screening for MOG-IgG. These proposed diagnostic criteria require validation but have the potential to improve identification of individuals with MOGAD, which is essential to define long-term clinical outcomes, refine inclusion criteria for clinical trials, and identify predictors of a relapsing versus a monophasic disease course.
Published: 2023

2. Pregnancy and post-partum in patients with myelin-oligodendrocyte glycoprotein antibody-associated disease

Author: Clarisse Carra-Dallière, Fabien Rollot, Romain Deschamps, Jonathan Ciron, Sandra Vukusic, Bertrand Audoin, Aurélie Ruet, Elisabeth Maillart, Caroline Papeix, Hélène Zephir, David Laplaud, Mikael Cohen, Bertrand Bourre, Illiasse El-Bahi, Pierre Labauge, Romain Casey, Xavier Ayrignac, and Romain Marignier
Subjects: Neurology, Neurology (clinical)
Abstract: Background and objective: Myelin-oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) frequently initiates during childbearing years. This study investigated the impact of pregnancy and post-partum on MOGAD activity. Methods: Retrospective analysis of clinical and demographic data from a multicenter French cohort of adult patients with MOGAD. All adult female patients who had a pregnancy after disease onset or in the year before disease onset were included. The annualized relapse rate was evaluated in patients who had a pregnancy after disease onset, to evaluate the impact of pregnancy and post-partum on MOGAD course. Results: Twenty-five informative pregnancies after disease onset were identified. No relapse was recorded during these pregnancies and only three relapses occurred during the first 3 months post-partum. The annualized relapse rate decreased from 0.67 (95% confidence interval: 0.40–1.10) during the pre-pregnancy period to 0 (95% confidence interval: 0–0.21) during pregnancy and to 0.22 (95% confidence interval: 0.09–0.53) during the first year post-partum. Among 144 female patients in their childbearing age recorded in the database, 18 (12.5%) reported their first symptoms during pregnancy or in the 12 months post-partum. Discussion: Our study suggests a marked reduction of MOGAD relapse rate during pregnancy and the post-partum period. Prospective studies on the role of pregnancy and delivery in MOGAD course are needed.
Published: 2022

3. Diagnostic value of intereye difference metrics for optic neuritis in aquaporin-4 antibody seropositive neuromyelitis optica spectrum disorders

Author: Frederike Cosima Oertel, Hanna G Zimmermann, Seyedamirhosein Motamedi, Claudia Chien, Orhan Aktas, Philipp Albrecht, Marius Ringelstein, Anitha Dcunha, Lekha Pandit, Elena H Martinez-Lapiscina, Bernardo Sanchez-Dalmau, Pablo Villoslada, Jacqueline Palace, Adriana Roca-Fernández, Maria Isabel Leite, Srilakshmi M Sharma, Letizia Leocani, Marco Pisa, Marta Radaelli, Marco Aurélio Lana-Peixoto, Mariana Andrade Fontenelle, Joachim Havla, Fereshteh Ashtari, Rahele Kafieh, Alireza Dehghani, Mohsen Pourazizi, Romain Marignier, Alvaro Cobo-Calvo, Nasrin Asgari, Anu Jacob, Saif Huda, Yang Mao-Draayer, Ari J Green, Rachel Kenney, Michael R Yeaman, Terry J Smith, Lawrence Cook, Alexander U Brandt, Friedemann Paul, Axel Petzold, Neurology, Ophthalmology, APH - Mental Health, APH - Methodology, and Amsterdam Neuroscience - Neuroinfection & -inflammation
Subjects: Psychiatry and Mental health, Surgery, Neurology (clinical), Function and Dysfunction of the Nervous System
Abstract: BackgroundThe novel optic neuritis (ON) diagnostic criteria include intereye differences (IED) of optical coherence tomography (OCT) parameters. IED has proven valuable for ON diagnosis in multiple sclerosis but has not been evaluated in aquaporin-4 antibody seropositive neuromyelitis optica spectrum disorders (AQP4+NMOSD). We evaluated the diagnostic accuracy of intereye absolute (IEAD) and percentage difference (IEPD) in AQP4+NMOSD after unilateral ON >6 months before OCT as compared with healthy controls (HC).MethodsTwenty-eight AQP4+NMOSD after unilateral ON (NMOSD-ON), 62 HC and 45 AQP4+NMOSD without ON history (NMOSD-NON) were recruited by 13 centres as part of the international Collaborative Retrospective Study on retinal OCT in Neuromyelitis Optica study. Mean thickness of peripapillary retinal nerve fibre layer (pRNFL) and macular ganglion cell and inner plexiform layer (GCIPL) were quantified by Spectralis spectral domain OCT. Threshold values of the ON diagnostic criteria (pRNFL: IEAD 5 µm, IEPD 5%; GCIPL: IEAD: 4 µm, IEPD: 4%) were evaluated using receiver operating characteristics and area under the curve (AUC) metrics.ResultsThe discriminative power was high for NMOSD-ON versus HC for IEAD (pRNFL: AUC 0.95, specificity 82%, sensitivity 86%; GCIPL: AUC 0.93, specificity 98%, sensitivity 75%) and IEPD (pRNFL: AUC 0.96, specificity 87%, sensitivity 89%; GCIPL: AUC 0.94, specificity 96%, sensitivity 82%). The discriminative power was high/moderate for NMOSD-ON versus NMOSD-NON for IEAD (pRNFL: AUC 0.92, specificity 77%, sensitivity 86%; GCIP: AUC 0.87, specificity 85%, sensitivity 75%) and for IEPD (pRNFL: AUC 0.94, specificity 82%, sensitivity 89%; GCIP: AUC 0.88, specificity 82%, sensitivity 82%).ConclusionsResults support the validation of the IED metrics as OCT parameters of the novel diagnostic ON criteria in AQP4+NMOSD.
Published: 2023

4. Clinical and MRI measures to identify non-acute MOG-antibody disease in adults

Author: Cortese, Rosa, Battaglini, Marco, Ferran, Prados, Alessia, Bianchi, Lukas, Haider, Anu, Jacob, Jacqueline, Palace, Silvia, Messina, Friedemann, Paul, Jens, Wuerfel, Romain, Marignier, Françoise, Durand-Dubief, Carolina de Medeiros Rimkus, Dagoberto, Callegaro, Douglas Kazutoshi Sato, Massimo, Filippi, Maria Assunta Rocca, Laura, Cacciaguerra, Alex, Rovira, Jaume, Sastre-Garriga, Georgina, Arrambide, Yaou, Liu, Yunyun, Duan, Claudio, Gasperini, Carla, Tortorella, Serena, Ruggieri, Maria Pia Amato, Ulivelli, Monica, Sergiu, Groppa, Matthias, Grothe, Sara, Llufriu, Maria, Sepulveda, Carsten, Lukas, Barbara, Bellenberg, Ruth, Schneider, Piotr, Sowa, Elisabeth, G Celius, Anne-Katrin, Proebstel, Özgür, Yaldizli, Jannis, Müller, Bruno, Stankoff, Benedetta, Bodini, Luca, Carmisciano, Maria Pia Sormani, Frederik, Barkhof, DE STEFANO, Nicola, Olga, Ciccarelli, Cortese, Rosa, Battaglini, Marco, Prados, Ferran, Bianchi, Alessia, Haider, Luka, Jacob, Anu, Palace, Jacqueline, Messina, Silvia, Paul, Friedemann, Wuerfel, Jen, Marignier, Romain, Durand-Dubief, Françoise, de Medeiros Rimkus, Carolina, Callegaro, Dagoberto, Sato, Douglas Kazutoshi, Filippi, Massimo, Rocca, Maria Assunta, Cacciaguerra, Laura, Rovira, Alex, Sastre-Garriga, Jaume, Arrambide, Georgina, Liu, Yaou, Duan, Yunyun, Gasperini, Claudio, Tortorella, Carla, Ruggieri, Serena, Amato, Maria Pia, Ulivelli, Monica, Groppa, Sergiu, Grothe, Matthia, Llufriu, Sara, Sepulveda, Maria, Lukas, Carsten, Bellenberg, Barbara, Schneider, Ruth, Sowa, Piotr, Celius, Elisabeth G, Proebstel, Anne-Katrin, Yaldizli, Özgür, Müller, Janni, Stankoff, Bruno, Bodini, Benedetta, Carmisciano, Luca, Sormani, Maria Pia, Barkhof, Frederik, De Stefano, Nicola, and Ciccarelli, Olga
Subjects: aquaporin 4-antibody positive neuromyelitis optica spectrum disorder, differential diagnosis, imaging, multiple sclerosis, myelin oligodendrocyte glycoprotein antibody-associated disease, Neurology (clinical)
Abstract: MRI and clinical features of myelin oligodendrocyte glycoprotein (MOG)-antibody disease may overlap with those of other inflammatory demyelinating conditions posing diagnostic challenges, especially in non-acute phases and when serologic testing for MOG antibodies is unavailable or shows uncertain results. We aimed to identify MRI and clinical markers that differentiate non-acute MOG-antibody disease from aquaporin 4 (AQP4)-antibody neuromyelitis optica spectrum disorder and relapsing remitting multiple sclerosis, guiding in the identification of patients with MOG-antibody disease in clinical practice. In this cross-sectional retrospective study, data from 16 MAGNIMS centres were included. Data collection and analyses were conducted from 2019 to 2021. Inclusion criteria were: diagnosis of MOG-antibody disease; AQP4-neuromyelitis optica spectrum disorder and multiple sclerosis; brain and cord MRI at least 6 months from relapse; and Expanded Disability Status Scale (EDSS) score on the day of MRI. Brain white matter T2 lesions, T1-hypointense lesions, cortical and cord lesions were identified. Random forest models were constructed to classify patients as MOG-antibody disease/AQP4-neuromyelitis optica spectrum disorder/multiple sclerosis; a leave one out cross-validation procedure assessed the performance of the models. Based on the best discriminators between diseases, we proposed a guide to target investigations for MOG-antibody disease. One hundred and sixty-two patients with MOG-antibody disease [99 females, mean age: 41 (±14) years, median EDSS: 2 (0–7.5)], 162 with AQP4-neuromyelitis optica spectrum disorder [132 females, mean age: 51 (±14) years, median EDSS: 3.5 (0–8)], 189 with multiple sclerosis (132 females, mean age: 40 (±10) years, median EDSS: 2 (0–8)] and 152 healthy controls (91 females) were studied. In young patients (
Published: 2023

5. Prospective longitudinal study on prognostic factors of visual recovery and structural change after a first episode of optic neuritis

Author: Romain Deschamps, Natalia Shor, Catherine Vignal, Jessica Guillaume, Marine Boudot de la Motte, Flore Salviat, Augustin Lecler, Romain Marignier, Rabih Hage, Sarah Coulette, Samuel Bidot, Antoine Gueguen, Martine Mauget‐Faysse, Caroline Bensa, Vivien Vasseur, Olivier Gout, and Cedric Lamirel
Subjects: Multiple Sclerosis, Optic Neuritis, Neurology, Vision Disorders, Humans, Longitudinal Studies, Prospective Studies, Neurology (clinical), Prognosis, Tomography, Optical Coherence
Abstract: This study was undertaken to determine the role of optical coherence tomography (OCT) in predicting the final visual and structural outcome, and to evaluate the correlation between functional eye outcome and retinal changes, in patients with a first episode of optic neuritis (ON).In this prospective study, consecutive adult patients with acute ON underwent ophthalmological evaluation at baseline and at 1 and 12 months, including OCT measurements of peripapillary retinal nerve fiber layer (pRNFL), macular ganglion cell and inner plexiform layer, and inner nuclear layer thicknesses; high- and low-contrast visual acuity; visual field assessment; and baseline brain magnetic resonance imaging. Univariate and multivariate linear regressions were used to assess predictive factors of outcome. Correlations between 12-month visual function and retinal structure were estimated by Spearman coefficients. Two groups of patients were analyzed, with or without multiple sclerosis (MS).Among 116 patients, 79 (68.1%) had MS, and 37 (31.9%) had ON not related to MS (including 19 idiopathic [i.e., isolated] ON, and 13 and five with myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies, respectively). We found no independent predictive factor of visual and retinal outcome. Analysis of the relationship between the visual field test (mean deviation) and pRNFL thickness demonstrated a threshold of 75.4 μm and 66.4 μm, below which the mean deviation was worse, for patients with MS (p = 0.007) and without MS (p lt; 0.001), respectively.We found that inner retinal layer measurements during the first month are not predictive of final outcome. The critical threshold of axonal integrity, below which visual function is damaged, is different between patients with and without MS.
Published: 2022

6. Relevance of Kappa free light chains index in patients with aquaporin4 or myelin‐oligodendrocyte‐glycoprotein antibodies

Author: Romain Deschamps, Natalia Shor, Caroline Papeix, Marine Boudot de la Motte, Caroline Bensa, Romain Marignier, Augustin Lecler, Catherine Vignal‐Clermont, Pascale Ghillani, Marianne Gazzano, Elisabeth Maillart, and Delphine Sterlin
Subjects: Neurology, Neurology (clinical)
Published: 2023

7. Minor salivary gland biopsy for the diagnosis of neurosarcoidosis

Author: Nicolas Fournier, Yvan Jamilloux, Mathieu Gerfaud-Valentin, Nathalie Streichenberger, Sandra Vukusic, Romain Marignier, Géraldine Androdias, and Pascal Seve
Subjects: Neurology, Neurology (clinical)
Abstract: Introduction: The definite diagnosis of neurosarcoidosis is challenging since it requires a compatible histology of the nervous system. When neurosarcoidosis is suspected, other systemic manifestations are investigated to confirm the diagnosis. A minor salivary gland biopsy (MSGB) is often performed since it is minimally invasive. The objective of the present study was to assess its performance for the diagnosis of neurosarcoidosis. Methods: A retrospective single-center study included patients who underwent a MSGB in a tertiary neurological university hospital (Lyon, France) between 2015 and 2018. Clinical presentations unlikely to be compatible with neurosarcoidosis were excluded. Positive cases of neurosarcoidosis were defined as definite, probable, and possible cases, according to the latest international neurosarcoidosis diagnostic criteria from the Neurosarcoidosis Consortium Consensus Group. Results: A total of 529 patients underwent a MSGB for clinical manifestations compatible with neurosarcoidosis. Among the 13 who fulfilled the criteria for neurosarcoidosis, only one had a positive MSGB. The sensitivity of MSGB was 7.7% (95% CI [0.2–36.0%]) and the specificity was 100.0% (95% CI [99.3–100%]). Discussion/Conclusion: Considering the low sensitivity of MSGB for the diagnosis of NS, MSGB should be performed in selected indications, including a suspicion of spinal cord sarcoidosis, or when there is a strong clinical, laboratory and radiological suspicion of NS. MSGB should rather not be performed when the chest CT-scan does not show signs of pulmonary or lymph node sarcoidosis.
Published: 2023

8. Is there a correlation between MOG‐associated disorder and SARS‐CoV‐2 infection?

Author: Sara Mariotto, Sara Carta, Alessandro Dinoto, Giuseppe Lippi, Gian Luca Salvagno, Laura Masin, Daniela Alberti, Romain Marignier, and Sergio Ferrari
Subjects: Neurology, SARS-CoV-2, Immunoglobulin G, COVID-19, Humans, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), Pandemics, Autoantibodies
Abstract: Anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) distinguish a group of inflammatory disorders which can be preceded by specific or non-specific infections. A few single cases have been reported in association with SARS-CoV-2 infection, but a specific study on the correlation between COVID-19 and myelin oligodendrocyte glycoprotein (MOG)-associated disorder (MOGAD) has not yet been performed. The aim of this study was to determine the impact of the pandemic on this condition.We analysed SARS-CoV-2 serology in patients newly diagnosed with MOGAD (1 August 2020 to 31 May 2021). MOG-Ab-seronegative age- and time-matched subjects were used as controls. SARS-CoV-2 immunoglobulin G (IgG) levels were analysed using an anti-SARS-CoV-2 US Food and Drug Administration-approved ELISA assay and confirmed with a trimeric anti-SARS-CoV-2 S1/S2 IgG immunochemiluminescent test, concomitantly assaying the anti-receptor binding domain (RBD) of spike protein IgG and anti-RBD total Ig. We actually compared the number of cases referred in each of the last 3 years.Presence of SARS-CoV-2 IgG antibodies was more common (12/30, 40%) in MOGAD patients than in controls (6/30, 20%), although the difference was not significant (p = 0.16; odds ratio 2.67, 95% confidence interval 0.85-9.17). The most common clinical presentations of MOGAD SARS-CoV-2-seropositive patients included optic neuritis (n = 6) and myelitis (n = 3). The number of diagnosed cases increased over the last 3 years, in particular, when including cases referred to us before the COVID-19 pandemic, in the initial phase of the first wave and in the late phase of the second wave (n = 9, rate 10.6% in 2019; n = 13, rate 12.3% in 2020; n = 15, rate 14.7% in 2021).Our findings provide preliminary data on SARS-CoV-2 as a potential trigger of MOGAD.
Published: 2022

9. SAkuraBONSAI: Protocol design of a novel, prospective study to explore clinical, imaging, and biomarker outcomes in patients with AQP4-IgG-seropositive neuromyelitis optica spectrum disorder receiving open-label satralizumab

Author: Jeffrey L. Bennett, Kazuo Fujihara, Ho Jin Kim, Romain Marignier, Kevin C. O'Connor, Robert C. Sergott, Anthony Traboulsee, Heinz Wiendl, Jens Wuerfel, Scott S. Zamvil, Veronica G. Anania, Regine Buffels, Thomas Künzel, Annemarie N. Lekkerkerker, Siân Lennon-Chrimes, and Sean J. Pittock
Subjects: Neurology, Neurology (clinical)
Abstract: BackgroundNeuromyelitis optica spectrum disorder (NMOSD) is a rare, autoimmune disease of the central nervous system that produces acute, unpredictable relapses causing cumulative neurological disability. Satralizumab, a humanized, monoclonal recycling antibody that targets the interleukin-6 receptor, reduced NMOSD relapse risk vs. placebo in two Phase 3 trials: SAkuraSky (satralizumab ± immunosuppressive therapy; NCT02028884) and SAkuraStar (satralizumab monotherapy; NCT02073279). Satralizumab is approved to treat aquaporin-4 IgG-seropositive (AQP4-IgG+) NMOSD. SAkuraBONSAI (NCT05269667) will explore fluid and imaging biomarkers to better understand the mechanism of action of satralizumab and the neuronal and immunological changes following treatment in AQP4-IgG+ NMOSD.ObjectivesSAkuraBONSAI will evaluate clinical disease activity measures, patient-reported outcomes (PROs), pharmacokinetics, and safety of satralizumab in AQP4-IgG+ NMOSD. Correlations between imaging markers (magnetic resonance imaging [MRI] and optical coherence tomography [OCT]) and blood and cerebrospinal fluid (CSF) biomarkers will be investigated.Study designSAkuraBONSAI is a prospective, open-label, multicenter, international, Phase 4 study that will enroll approximately 100 adults (18–74 years) with AQP4-IgG+ NMOSD. This study includes two patient cohorts: newly diagnosed, treatment-naïve patients (Cohort 1; n = 60); and inadequate responders to recent (n = 40). Satralizumab monotherapy (120 mg) will be administered subcutaneously at Weeks 0, 2, 4, and Q4W thereafter for a total of 92 weeks.EndpointsDisease activity related to relapses (proportion relapse-free, annualized relapse rate, time to relapse, and relapse severity), disability progression (Expanded Disability Status Scale), cognition (Symbol Digit Modalities Test), and ophthalmological changes (visual acuity; National Eye Institute Visual Function Questionnaire-25) will all be assessed. Peri-papillary retinal nerve fiber layer and ganglion cell complex thickness will be monitored using advanced OCT (retinal nerve fiber layer and ganglion cell plus inner plexiform layer thickness). Lesion activity and atrophy will be monitored by MRI. Pharmacokinetics, PROs, and blood and CSF mechanistic biomarkers will be assessed regularly. Safety outcomes include the incidence and severity of adverse events.ConclusionsSAkuraBONSAI will incorporate comprehensive imaging, fluid biomarker, and clinical assessments in patients with AQP4-IgG+ NMOSD. SAkuraBONSAI will provide new insights into the mechanism of action of satralizumab in NMOSD, while offering the opportunity to identify clinically relevant neurological, immunological, and imaging markers.
Published: 2023
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10. Pregnancy and neuromyelitis optica spectrum disorders: 2022 recommendations from the French Multiple Sclerosis Society

Author: Sandra Vukusic, Romain Marignier, Jonathan Ciron, Bertrand Bourre, Mikael Cohen, Romain Deschamps, Maxime Guillaume, Laurent Kremer, Julie Pique, Clarisse Carra-Dalliere, Laure Michel, Emmanuelle Leray, Anne-Marie Guennoc, David Laplaud, Géraldine Androdias, Caroline Bensa, Kevin Bigaut, Damien Biotti, Pierre Branger, Olivier Casez, Elodie Daval, Cécile Donze, Anne-Laure Dubessy, Cécile Dulau, Françoise Durand-Dubief, Benjamin Hebant, Arnaud Kwiatkowski, Julien Lannoy, Adil Maarouf, Eric Manchon, Guillaume Mathey, Xavier Moisset, Alexis Montcuquet, Thomas Roux, Elisabeth Maillart, Christine Lebrun-Frenay, Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Hospices Civils de Lyon (HCL), Institut Toulousain des Maladies Infectieuses et Inflammatoires (Infinity), Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), CHU Rouen, Normandie Université (NU), Fondation Ophtalmologique Adolphe de Rothschild [Paris], Les Hôpitaux Universitaires de Strasbourg (HUS), Centre de Référence des Maladies Inflammatoires Rares du Cerveau et de la Moelle (MIRCEM), CHU Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre d'Investigation Clinique [Rennes] (CIC), Université de Rennes (UR)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], École des Hautes Études en Santé Publique [EHESP] (EHESP), Département Méthodes quantitatives en santé publique (METIS), Centre de Recherches sur l'Action Politique en Europe (ARENES), Université de Rennes (UR)-Institut d'Études Politiques [IEP] - Rennes-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Centre National de la Recherche Scientifique (CNRS), Recherche sur les services et le management en santé (RSMS), Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Collectif de recherche handicap, autonomie et société inclusive (CoRHASI), Centre d’Investigation Clinique de Nantes (CIC Nantes), Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre hospitalier universitaire de Nantes (CHU Nantes), Techniques de l'Ingénierie Médicale et de la Complexité - Informatique, Mathématiques et Applications Grenoble - UMR 5525 (TIMC-IMAG), VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes (UGA)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP ), Université Grenoble Alpes (UGA), Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), and Hôpital Pasteur [Nice] (CHU)
Subjects: disease modifying treatments, Neurology, breastfeeding, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Neurology (clinical), pregnancy, Neuromyelitis optica, reproductive assistance
Abstract: Background: In 2020, the French Multiple Sclerosis (MS) Society (SFSEP) decided to develop a national evidence-based consensus on pregnancy in MS. As neuromyelitis optica spectrum disorders (NMOSD) shares a series of commonalities with MS, but also some significant differences, specific recommendations had to be developed. Objectives: To establish recommendations on pregnancy in women with NMOSD. Methods: The French Group for Recommendations in Multiple Sclerosis (France4MS) reviewed PubMed and universities databases (January 1975 through June 2021). The RAND/UCLA appropriateness method, which was developed to synthesise the scientific literature and expert opinions on health care topics, was used to reach a formal agreement. Fifty-six MS experts worked on the full-text review and initial wording of recommendations. A sub-group of nine NMOSD experts was dedicated to analysing available data on NMOSD. A group of 62 multidisciplinary healthcare specialists validated the final proposal of summarised evidence. Results: A strong agreement was reached for all 66 proposed recommendations. They cover diverse topics, such as pregnancy planning, follow-up during pregnancy and postpartum, delivery routes, loco-regional analgesia or anaesthesia, prevention of postpartum relapses, breastfeeding, vaccinations, reproductive assistance, management of relapses, and disease-modifying treatments. Conclusion: Physicians and patients should be aware of the new and specific evidence-based recommendations of the French MS Society for pregnancy in women with NMOSD. They should help harmonise counselling and treatment practise, allowing for better individualised choices.
Published: 2023

11. Pregnancy and multiple sclerosis: 2022 recommendations from the French multiple sclerosis society

Author: Sandra Vukusic, Clarisse Carra-Dalliere, Jonathan Ciron, Elisabeth Maillart, Laure Michel, Emmanuelle Leray, Anne-Marie Guennoc, Bertrand Bourre, David Laplaud, Géraldine Androdias, Caroline Bensa, Kevin Bigaut, Damien Biotti, Pierre Branger, Olivier Casez, Mikael Cohen, Elodie Daval, Romain Deschamps, Cécile Donze, Anne-Laure Dubessy, Cécile Dulau, Françoise Durand-Dubief, Maxime Guillaume, Benjamin Hebant, Laurent Kremer, Arnaud Kwiatkowski, Julien Lannoy, Adil Maarouf, Eric Manchon, Guillaume Mathey, Xavier Moisset, Alexis Montcuquet, Julie Pique, Thomas Roux, Romain Marignier, Christine Lebrun-Frenay, Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Observatoire Français de la Sclérose En Plaques [Lyon] (OFSEP), Université de Lyon, Fondation Eugène Devic EDMUS, Hôpital Gui de Chauliac, Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Institut Toulousain des Maladies Infectieuses et Inflammatoires (Infinity), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre Ressources et Compétences sclérose en plaques (CRC-SEP) [CHU Toulouse] (CRC-SEP ), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de Ressources et de Compétences sur la Sclérose en Plaques (CRC-SEP) [CHU Pitié-Salpêtrière] (CRC-SEP Paris APHP), Centre d'Investigation Clinique [Rennes] (CIC), Université de Rennes (UR)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], École des Hautes Études en Santé Publique [EHESP] (EHESP), Centre de Recherches sur l'Action Politique en Europe (ARENES), Université de Rennes (UR)-Institut d'Études Politiques [IEP] - Rennes-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Centre National de la Recherche Scientifique (CNRS), Recherche sur les services et le management en santé (RSMS), Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Service de neurologie [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU), Centre d’Investigation Clinique de Nantes (CIC Nantes), Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre hospitalier universitaire de Nantes (CHU Nantes), Hospices Civils de Lyon, Departement de Neurologie (HCL), Hôpital de la Fondation Ophtalmologique Adolphe de Rothschild [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Ressources et de Compétences sur la Sclérose en Plaques (CRC-SEP) [Strasbourg] (CRC-SEP Alsace), Les Hôpitaux Universitaires de Strasbourg (HUS), Service de Neurologie [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Université Grenoble Alpes (UGA), Translational Research in Autoimmunity and Inflammation Group (TIMC-T-RAIG), Translational Innovation in Medicine and Complexity / Recherche Translationnelle et Innovation en Médecine et Complexité - UMR 5525 (TIMC ), VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes (UGA)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP ), Université Grenoble Alpes (UGA)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes (UGA)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP ), Techniques de l'Ingénierie Médicale et de la Complexité - Informatique, Mathématiques et Applications Grenoble - UMR 5525 (TIMC-IMAG), Centre Ressources et Compétences sclérose en plaques (CRC-SEP) [CHU de Nice Pasteur 2] (CRC-SEP Côte d'Azur), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Fondation Ophtalmologique Adolphe de Rothschild [Paris], Groupe Hospitalier de l'Institut Catholique de Lille (GHICL), Université catholique de Lille (UCL), Hôpital Saint Philibert [Lomme], Groupement des Hôpitaux de l'Institut Catholique de Lille (GHICL), Université catholique de Lille (UCL)-Université catholique de Lille (UCL), Faculté de Médecine, Maïeutique, Sciences de la Santé - Campus VAUBAN [Lille] (F2M2S), CHU Saint-Antoine [AP-HP], Centre Ressources et Compétences sclérose en plaques (CRC-SEP) [CHU de Bordeaux] (CRC-SEP - Hôpital Pellegrin), Hôpital neurologique et neurochirurgical Pierre Wertheimer [CHU - HCL], Hospices Civils de Lyon (HCL), Normandie Université (NU), Centre Hospitalier de Lens, Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier de Gonesse, Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Neuro-Dol (Neuro-Dol), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Clermont Auvergne (UCA), Unité de Recherche Clinique de la Côte d’Azur [Nice] (URRIS UR2CA), Université Côte d'Azur (UCA), Jonchère, Laurent, Hôpital Gui de Chauliac [CHU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Ressources et Compétences sclérose en plaques (CRC-SEP) [CHU Toulouse] (CRC-SEP Toulouse), Département Neurologie [CHU Toulouse], Pôle Neurosciences [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Pôle Neurosciences [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Unité de Recherche Clinique de la Côte d’Azur (URRIS UR2CA), and Centre Hospitalier Universitaire de Nice (CHU Nice)-Université Côte d'Azur (UCA)
Subjects: Multiple sclerosis, [SDV] Life Sciences [q-bio], Neurology, breastfeeding, [SDV]Life Sciences [q-bio], Neurology (clinical), disease-modifying treatments, pregnancy, reproductive assistance
Abstract: Objective: The objective of this study was to develop evidence-based recommendations on pregnancy management for persons with multiple sclerosis (MS). Background: MS typically affects young women in their childbearing years. Increasing evidence is available to inform questions raised by MS patients and health professionals about pregnancy issues. Methods: The French Group for Recommendations in Multiple Sclerosis (France4MS) reviewed PubMed and university databases (January 1975 through June 2021). The RAND/UCLA appropriateness method was developed to synthesise the scientific literature and expert opinions on healthcare topics; it was used to reach a formal agreement. Fifty-six MS experts worked on the full-text review and initial wording of recommendations. A group of 62 multidisciplinary healthcare specialists validated the final proposal of summarised evidence. Results: A strong agreement was reached for all 104 proposed recommendations. They cover diverse topics, such as pregnancy planning, follow-up during pregnancy and postpartum, delivery routes, locoregional analgesia or anaesthesia, prevention of postpartum relapses, breastfeeding, vaccinations, reproductive assistance, management of relapses and disease-modifying treatments. Conclusion: The 2022 recommendations of the French MS society should be helpful to harmonise counselling and treatment practice for pregnancy in persons with MS, allowing for better and individualised choices.
Published: 2023

12. Anti-Argonaute antibodies as a potential biomarker in NMOSD

Author: Sara Carta, Do Le Duy, Veronique Rogemond, Nathalie Derache, Hugo Chaumont, Agnès Fromont, Sebastien Cabasson, Marine Boudot de la Motte, Jerome Honnorat, and Romain Marignier
Subjects: immunology, Psychiatry and Mental health, myelin, myelopathy, Surgery, Neurology (clinical), neuroimmunology
Abstract: Background and objectivesNeuromyelitis optica spectrum disorders (NMOSDs) are a group of diseases mainly characterised by recurrent optic neuritis and/or myelitis. Most cases are associated with a pathogenic antibody against aquaporin-4 (AQP4-Ab), while some patients display autoantibodies targeting the myelin oligodendrocyte glycoprotein (myelin oligodendrocyte glycoprotein antibodies (MOG-Abs)). Anti-Argonaute antibodies (Ago-Abs) were first described in patients with rheumatological conditions and were recently reported as a potential biomarker in patients with neurological disorders. The aims of the study were to investigate if Ago-Abs can be detected in NMOSD and to evaluate its clinical usefulness.MethodsSera from patients prospectively referred to our centre with suspected NMOSD were tested for AQP4-Abs, MOG-Abs and Ago-Abs with cell-based assays.ResultsThe cohort included 104 prospective patients: 43 AQP4-Abs-positive cases, 34 MOG-Abs positive cases and 27 double-negative patients. Ago-Abs were detected in 7 of 104 patients (6.7%). Clinical data were available for six of seven patients. The median age at onset of patients with Ago-Abs was 37.5 [IQR 28.8–50.8]; five of six patients tested positive also for AQP4-Abs. Clinical presentation at onset was transverse myelitis in five patients, while one presented with diencephalic syndrome and experienced a transverse myelitis during follow-up. One case presented a concomitant polyradiculopathy. Median EDSS score at onset was 7.5 [IQR 4.8–8.4]; median follow-up was 40.3 months [IQR 8.3–64.7], and median EDSS score at last evaluation was 4.25 [IQR 1.9–5.5].ConclusionAgo-Abs are present in a subset of patients with NMOSD and, in some cases, represent the only biomarker of an autoimmune process. Their presence is associated with a myelitis phenotype and a severe disease course.
Published: 2023

13. Application of diagnostic criteria for optic neuritis – Authors' reply

Author: Axel Petzold, Yaou Liu, Clare Fraser, Mathias Abegg, Raed Alroughani, Daniah Alshowaeir, Regina Alvarenga, Cécile Andris, Nasrin Asgari, Yael Barnett, Roberto Battistella, Raed Behbehani, Thomas Berger, Mukharram M. Bikbov, Damien Biotti, Valerie Biousse, Antonella Boschi, Milan Brazdil, Andrei Brezhnev, Peter Calabresi, Monique Cordonnier, Fiona Costello, Franz Marie Cruz, Leonardo Provetti Cunha, Smail Daoudi, Romain Deschamps, Jerome DeSeze, Ricarda Diem, Masoud Etemadifar, Jose Flores-Rivera, Pedro Fonseca, Jette Frederiksen, Elliot Frohman, Teresa Frohman, Caroline FromentTilikete, Kazuo Fujihara, Alberto Gálvez, Riadh Gouider, Fernando Gracia, Nikolaos Grigoriadis, José Manuel Guajardo, Mario Habek, Marko Hawlina, Elena Hernández-Martínez de Lapiscina, Juzar Hooker, Jyh Yung Hor, William Howlett, Yumin Huang-Link, Zhannat Idrissova, Zsolt Illes, Jasna Jancic, Panitha Jindahra, Dimitrios Karussis, Emilia Kerty, Ho Jin Kim, Wolf Lagrèze, Letizia Leocani, Netta Levin, Petra Liskova, Youssoufa Maiga, Romain Marignier, Chris McGuigan, Dália Meira, Harold Merle, Mário L.R. Monteiro, Anand Moodley, Frederico Moura, Silvia Muñoz, Sharik Mustafa, Ichiro Nakashima, Susana Noval, Carlos Oehninger, Olufunmilola Ogun, Afekhide Omoti, Lekha Pandit, Friedemann Paul, Gema Rebolleda, Stephen Reddel, Konrad Rejdak, Robert Rejdak, Alfonso Rodriguez-Morales, Marie-Bénédicte Rougier, Maria Jose Sa, Bernardo Sanchez-Dalmau, Deanna Saylor, Ismail Shatriah, Aksel Siva, Hadas Stiebel-Kalish, Gabriella Szatmary, Linh Ta, Sylvia Tenembaum, Huy Tran, Yevgen Trufanov, Vincent VanPesch, An-Guor Wang, Mike P. Wattjes, Ernie Willoughby, Magd Zakaria, Jasmin Zvornicanin, Laura Balcer, Gordon T. Plant, Neurology, Ophthalmology, APH - Mental Health, APH - Methodology, and Amsterdam Neuroscience - Neuroinfection & -inflammation
Subjects: Neurology (clinical)
Published: 2023

14. Encéphalite à anticorps anti-NMDAr et atteinte radiologique extra-limbique : une population particulière au pronostic plus sévère ?

Author: Laura Khatib, Nicolas Lundahl Ciano-Petersen Lundahl, Julie Pique, Romain Marignier, François Cotton, Jérôme Honnorat, and Bastien Joubert
Subjects: Neurology, Neurology (clinical)
Published: 2023

15. Glial Fibrillary Acidic Protein Autoimmunity

Author: Daniela Molinier-Tiganas, Jonathan Biberon, Quentin Bodard, Véronique Rogemond, Anne Ruiz, Bastien Bouldoires, Celine Derollez, Philippe Diraison, Daniela Andriuta, Alberto Vogrig, Amelie Leblanc, Thomas De Broucker, Aditya Ambati, Philippe Kerschen, Géraldine Picard, Thierry Tchoumi, Eve Chanson, Anne-Laure Kaminsky, Irina Grigorashvili-Coin, Clementine Fort, Fanny Duval, Roxana Genet, Jérôme Honnorat, Kumaran Deiva, Sergio Muñiz-Castrillo, Mathilde Goudot, Bastien Joubert, François Sellal, Gabriel Mirebeau, Anais Dutray, Marion Philbert, Guillaume Rieul, Véronique Bourg, Erwan Morvan, Jonathan Ciron, Adrien Bigot, Romain Marignier, Roxana Ameli, Lucie Hopes, Jean Louis Devoize, Nahema Issa, Mickael Bonnan, Elena-Camelia Rusu, Laurent Kremer, Florence Rulquin, Alice Gravier Dumonceau, Marie Benaiteau, Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Département de Neuroradiologie [Centre Hospitalier Lyon Sud - HCL], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Hospices Civils de Lyon (HCL), Institut NeuroMyoGène (INMG), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Centre de Référence Maladie Rare 'Syndromes neurologiques Paranéoplasiques', Hospices Civils de Lyon (HCL)-Hopital Neurologique, CHU Toulouse [Toulouse], Institut Toulousain des Maladies Infectieuses et Inflammatoires (Infinity), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de neurologie [Le Kremlin Bicêtre], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Institut de Recherche en Infectiologie de Montpellier (IRIM), Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Institut National de la Santé et de la Recherche Médicale (INSERM), Excitabilité nerveuse et thérapeutique (ENT), Hôpital Henri Mondor-EA 4391, Service de Physiologie Explorations Fonctionnelles-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Hôpitaux Civils de Colmar, Centre Psychothérapique de Nancy [Laxou] (CPN), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Groupe hospitalier Pellegrin, CHU Bordeaux [Bordeaux], Service de neurologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier Saint Jean de Perpignan, Centre Hospitalier d'Angoulême (CH Angoulême), Université des Antilles - UFR des sciences médicales Hyacinthe Bastaraud (UA UFR SM), Université des Antilles (UA), CHU Clermont-Ferrand, Service de Neurologie [Brest], Hôpital d'Instruction des Armées 'Clermont-Tonnerre' (HIA), Commissariat à l'énergie atomique et aux énergies alternatives - Laboratoire d'Electronique et de Technologie de l'Information (CEA-LETI), Direction de Recherche Technologique (CEA) (DRT (CEA)), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA), Service de neurologie [Amiens], CHU Amiens-Picardie, Laboratoire de Neurosciences Fonctionnelles et Pathologies - UR UPJV 4559 (LNFP), Université de Picardie Jules Verne (UPJV), Université Grenoble Alpes - UFR Médecine (UGA UFRM), Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Hôpital Pasteur [Nice] (CHU), CHU de Saint-Brieuc, CHU Pau, Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut du Cerveau = Paris Brain Institute (ICM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), CHU Strasbourg, Université Grenoble Alpes (UGA), and ANR-18-RHUS-0012,BETPSY,Biomarkers in autoimmune EncephaliTis and Paraneoplastic neurological SYndromes(2018)
Subjects: Adult, Male, Pathology, medicine.medical_specialty, [SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology, Myelitis, Autoimmunity, Autoimmune Diseases, Cohort Studies, Autoimmune Diseases of the Nervous System, Cerebrospinal fluid, Glial Fibrillary Acidic Protein, medicine, Humans, Child, Autoantibodies, Retrospective Studies, biology, Glial fibrillary acidic protein, business.industry, Autoantibody, Meningoencephalitis, medicine.disease, Hyperintensity, biology.protein, [SDV.IMM]Life Sciences [q-bio]/Immunology, Immunohistochemistry, Neurology (clinical), Antibody, business
Abstract: Background and ObjectivesTo report the clinical, biological, and imaging features and clinical course of a French cohort of patients with glial fibrillary acidic protein (GFAP) autoantibodies.MethodsWe retrospectively included all patients who tested positive for GFAP antibodies in the CSF by immunohistochemistry and confirmed by cell-based assay using cells expressing human GFAPα since 2017 from 2 French referral centers.ResultsWe identified 46 patients with GFAP antibodies. Median age at onset was 43 years, and 65% were men. Infectious prodromal symptoms were found in 82%. Other autoimmune diseases were found in 22% of patients, and coexisting neural autoantibodies in 11%. Tumors were present in 24%, and T-cell dysfunction in 23%. The most frequent presentation was subacute meningoencephalitis (85%), with cerebellar dysfunction in 57% of cases. Other clinical presentations included myelitis (30%) and visual (35%) and peripheral nervous system involvement (24%). MRI showed perivascular radial enhancement in 32%, periventricular T2 hyperintensity in 41%, brainstem involvement in 31%, leptomeningeal enhancement in 26%, and reversible splenial lesions in 4 cases. A total of 33 of 40 patients had a monophasic course, associated with a good outcome at last follow-up (Rankin Score ≤2: 89%), despite a severe clinical presentation. Adult and pediatric features are similar. Thirty-two patients were treated with immunotherapy. A total of 11/22 patients showed negative conversion of GFAP antibodies.DiscussionGFAP autoimmunity is mainly associated with acute/subacute meningoencephalomyelitis with prodromal symptoms, for which tumors and T-cell dysfunction are frequent triggers. The majority of patients followed a monophasic course with a good outcome.
Published: 2021

16. Myelin-oligodendrocyte glycoprotein antibody-associated disease

Author: Axel Petzold, Tobias Derfuss, Bruno Stankoff, Aksel Siva, Maria Pia Amato, Tanuja Chitnis, Alvaro Cobo-Calvo, Romain Marignier, Sandra Vukusic, Nasrin Asgari, Christopher Linington, Edgar Meinl, Emmanuelle Waubant, Marco Capobianco, Patrick Waters, Hans Lassmann, Ingo Kleiter, Anu Jacob, Sean J. Pittock, Mar Tintoré, Friedemann Paul, Brenda Banwell, Kumaran Deiva, Kazuo Fujihara, Jeffrey Bennett, Jacqueline Palace, Krzysztof Selmaj, Olga Ciccarelli, Anthony Traboulsee, Brian G. Weinshenker, Fabienne Brilot, Jérôme De Seze, Maria Isabel Leite, Harry Alexopoulos, Ho Jin Kim, Anne-Katrin Pröbstel, Douglas Kazutoshi Sato, Bernhard Hemmer, Markus Reindl, Yael Hacohen, and Orhan Aktas
Subjects: Adult, Adolescent, CNS demyelination, Demyelinating Autoimmune Diseases, CNS, Disease, Myelin oligodendrocyte glycoprotein, Young Adult, medicine, Humans, Immunologic Factors, Spectrum disorder, Child, Pathological, Autoantibodies, Neuromyelitis optica, biology, business.industry, Multiple sclerosis, Middle Aged, medicine.disease, Immunology, biology.protein, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), Antibody, business, Biomarkers
Abstract: Myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently identified autoimmune disorder that presents in both adults and children as CNS demyelination. Although there are clinical phenotypic overlaps between MOGAD, multiple sclerosis, and aquaporin-4 antibody-associated neuromyelitis optica spectrum disorder (NMOSD) cumulative biological, clinical, and pathological evidence discriminates between these conditions. Patients should not be diagnosed with multiple sclerosis or NMOSD if they have anti-MOG antibodies in their serum. However, many questions related to the clinical characterisation of MOGAD and pathogenetic role of MOG antibodies are still unanswered. Furthermore, therapy is mainly based on standard protocols for aquaporin-4 antibody-associated NMOSD and multiple sclerosis, and more evidence is needed regarding how and when to treat patients with MOGAD.
Published: 2021

17. Time to steroids impacts visual outcome of optic neuritis in MOGAD

Author: Julie Rode, Julie Pique, Adil Maarouf, Xavier Ayrignac, Bertrand Bourre, Jonathan Ciron, Mikael Cohen, Nicolas Collongues, Romain Deschamps, Elisabeth Maillart, Alexis Montcuquet, Caroline Papeix, Aurelie Ruet, Sandrine Wiertlewski, Helene Zephir, Romain Marignier, Bertrand Audoin, Pôle de Neurosciences Cliniques, Service de Neurologie, APHM, Hôpital de la Timone, Aix-Marseille University, Marseille, France, Centre de résonance magnétique biologique et médicale (CRMBM), and Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Psychiatry and Mental health, Surgery, Neurology (clinical), [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: BackgroundTo characterise the response to treatment of inaugural optic neuritis (ON) in patients with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).MethodsWe searched the French MOGAD database for adults with inaugural ON with a detailed report of acute treatment modalities and measures of high-contrast best-corrected visual acuity (BCVA) at nadir and after 3 months. Predictors of visual outcomes were assessed by multivariable analysis.ResultsAmong 245 patients with at least one episode of ON, 82 fulfilled all criteria, and data on the peripapillary retinal nerve fibre layer (pRNFL) were available for 44. All patients received methylprednisolone (MP), combined with plasma exchange in 18. After 3 months, 75 of 82 (91%) patients retained full BCVA recovery, and median (range) pRNFL of the affected eye was 72 µm (40–102). Failure to regain 0.0 logarithmic minimum angle of resolution vision (Snellen 20/20) at 3 months was associated with time to first MP treatment ≥10 days (OR 16, 95% CI 1.14 to 213, p=0.01). pRNFL thickness after 3 months was related to better BCVA at nadir and time to first MP treatment ConclusionsTime to MP affects functional but also structural visual outcomes of ON in MOGAD.
Published: 2022

18. Longitudinal Retinal Changes in MOGAD

Author: Frederike Cosima, Oertel, Elias S, Sotirchos, Hanna G, Zimmermann, Seyedamirhosein, Motamedi, Svenja, Specovius, Eva Susanna, Asseyer, Claudia, Chien, Lawrence, Cook, Eleni, Vasileiou, Angeliki, Filippatou, Peter A, Calabresi, Shiv, Saidha, Lekha, Pandit, Anitha, D'Cunha, Olivier, Outteryck, Hélène, Zéphir, Sean, Pittock, Eoin P, Flanagan, M Tariq, Bhatti, Paulus S, Rommer, Gabriel, Bsteh, Tobias, Zrzavy, Tania, Kuempfel, Orhan, Aktas, Marius, Ringelstein, Philipp, Albrecht, Ilya, Ayzenberg, Thivya, Pakeerathan, Benjamin, Knier, Lilian, Aly, Nasrin, Asgari, Kerstin, Soelberg, Romain, Marignier, Caroline Froment, Tilikete, Alvaro, Cobo Calvo, Pablo, Villoslada, Bernardo, Sanchez-Dalmau, Elena H, Martinez-Lapiscina, Sara, Llufriu, Ari J, Green, Michael R, Yeaman, Terry J, Smith, Alexander U, Brandt, John, Chen, Friedemann, Paul, Joachim, Havla, and Caryl, Tongco
Subjects: Optic Neuritis, Retinal Degeneration, Immunologic Deficiency Syndromes, Retina, ddc, Cohort Studies, Research Article, Research Articles, Neurology, Case-Control Studies, Humans, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), Longitudinal Studies, Tomography, Optical Coherence, Retinal Neurons
Abstract: Patients with myelin oligodendrocyte glycoprotein antibody (MOG-IgG)-associated disease (MOGAD) suffer from severe optic neuritis (ON) leading to retinal neuro-axonal loss, which can be quantified by optical coherence tomography (OCT). We assessed whether ON-independent retinal atrophy can be detected in MOGAD.Eighty patients with MOGAD and 139 healthy controls (HCs) were included. OCT data was acquired with (1) Spectralis spectral domain OCT (MOGAD: N = 66 and HCs: N = 103) and (2) Cirrus high-definition OCT (MOGAD: N = 14 and HCs: N = 36). Macular combined ganglion cell and inner plexiform layer (GCIPL) and peripapillary retinal nerve fiber layer (pRNFL) were quantified.At baseline, GCIPL and pRNFL were lower in MOGAD eyes with a history of ON (MOGAD-ON) compared with MOGAD eyes without a history of ON (MOGAD-NON) and HCs (p 0.001). MOGAD-NON eyes had lower GCIPL volume compared to HCs (p 0.001) in the Spectralis, but not in the Cirrus cohort. Longitudinally (follow-up up to 3 years), MOGAD-ON with ON within the last 6-12 months before baseline exhibited greater pRNFL thinning than MOGAD-ON with an ON greater than 12 months ago (p 0.001). The overall MOGAD cohort did not exhibit faster GCIPL thinning compared with the HC cohort.Our study suggests the absence of attack-independent retinal damage in patients with MOGAD. Yet, ongoing neuroaxonal damage or edema resolution seems to occur for up to 12 months after ON, which is longer than what has been reported with other ON forms. These findings support that the pathomechanisms underlying optic nerve involvement and the evolution of OCT retinal changes after ON is distinct in patients with MOGAD. ANN NEUROL 2022;92:476-485.
Published: 2022

19. MRI to detect and localize the area postrema in multiple sclerosis: The role of 3D‐DIR and 3D‐FLAIR

Author: Romain Marignier, Valentine Farges, Salem Hannoun, François Cotton, and Théo Benini
Subjects: Multiple Sclerosis, Inversion recovery, Fluid-attenuated inversion recovery, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, Imaging, Three-Dimensional, 0302 clinical medicine, medicine, Humans, Radiology, Nuclear Medicine and imaging, Neuromyelitis optica, medicine.diagnostic_test, business.industry, Multiple sclerosis, Neuromyelitis Optica, Area postrema, Magnetic resonance imaging, medicine.disease, Magnetic Resonance Imaging, Area Postrema, Medulla oblongata, Neurology (clinical), No detection, Nuclear medicine, business, 030217 neurology & neurosurgery
Abstract: Background and purpose Area postrema (AP) is a highly vascularized paired 2 mm-long anatomical structure, localized on the dorsal inferior surface of the medulla oblongata, at the caudal end of the fourth-ventricle. AP is principally affected in AP syndrome, which is commonly associated with autoimmune inflammatory diseases, including essentially neuromyelitis optica spectrum disorder (NMOSD). The aim of this study is to assess the best cerebral MRI sequences and planes for AP detection in order to assist or aid in the diagnosis of difficult NMOSD cases. Methods 3DT1, 2DT2, 3D-fluid-attenuated inversion recovery (3DFLAIR), and 3D-double inversion recuperation (3DDIR), routinely used in inflammatory diseases, were analyzed and scored based on quality (0-2), and ability to detect AP in each plane (0 = no detection, 1 = probable detection, 2 = obvious detection). Based on image availability, subjects were divided into three groups: Group-1, including 100 randomly selected subjects with 3DT1 and 3DFLAIR, Group-2, including 30 multiple sclerosis (MS) patients from the "Observatoire Francais de la Sclerose En Plaques" (OFSEP) with 3DT1, 3DFLAIR, and 3DDIR, and Group-3, including 164 OFSEP MS patients with 3DFLAIR and 2DT2. Results AP was undetectable on 3DT1 and 2DT2. AP was detected in 87% of 3DFLAIR in Group-1, 90% in Group-2, and 90% in Group-3. AP was also detected in 100% of 3DDIR images in the axial plane. Conclusions As evidenced, AP was easily assessed on 3DDIR and 3DFLAIR emphasizing the importance of adding these sequences to NMOSD MRI-protocols. Moreover, the most effective imaging plane in identifying AP was the axial plane.
Published: 2021

20. International Delphi Consensus on the Management of AQP4-IgG+ NMOSD

Author: Friedemann Paul, Romain Marignier, Jacqueline Palace, Georgina Arrambide, Nasrin Asgari, Jeffrey L. Bennett, Bruce Anthony Campbell Cree, Jérôme De Sèze, Kazuo Fujihara, Ho Jin Kim, Rebecca Hornby, Saif Huda, Najib Kissani, Ingo Kleiter, Satoshi Kuwabara, Marco Lana-Peixoto, Lisa Law, M. Isabel Leite, Lekha Pandit, Sean J. Pittock, Chao Quan, Sudarshini Ramanathan, Dalia Rotstein, Albert Saiz, Douglas Kazutoshi Sato, and Adi Vaknin-Dembinsky
Subjects: Aquaporin 4, Consensus, Good Health and Well Being, Delphi Technique, Neurology, Clinical Research, Immunoglobulin G, Neuromyelitis Optica, Humans, Neurology (clinical), Function and Dysfunction of the Nervous System
Abstract: Background and ObjectivesNeuromyelitis optica spectrum disorder (NMOSD) is a rare debilitating autoimmune disease of the CNS. Three monoclonal antibodies were recently approved as maintenance therapies for aquaporin-4 immunoglobulin G (AQP4-IgG)–seropositive NMOSD (eculizumab, inebilizumab, and satralizumab), prompting the need to consider best practice therapeutic decision-making for this indication. Our objective was to develop validated statements for the management of AQP4-IgG–seropositive NMOSD, through an evidence-based Delphi consensus process, with a focus on recommendations for eculizumab, inebilizumab, and satralizumab.MethodsWe recruited an international panel of clinical experts in NMOSD and asked them to complete a questionnaire on NMOSD management. Panel members received a summary of evidence identified through a targeted literature review and provided free-text responses to the questionnaire based on both the data provided and their clinical experience. Responses were used to generate draft statements on NMOSD-related themes. Statements were voted on over a maximum of 3 rounds; participation in at least 1 of the first 2 rounds was mandatory. Panel members anonymously provided their level of agreement (6-point Likert scale) on each statement. Statements that failed to reach a predefined consensus threshold (≥67%) were revised based on feedback and then voted on in the next round. Final statements were those that met the consensus threshold (≥67%).ResultsThe Delphi panel comprised 24 experts, who completed the Delphi process in November 2021 after 2 voting rounds. In round 1, 23/25 statements reached consensus and were accepted as final. The 2 statements that failed to reach consensus were revised. In round 2, both revised statements reached consensus. Twenty-five statements were agreed in total: 11 on initiation of or switching between eculizumab, inebilizumab, and satralizumab; 3 on monotherapy/combination therapy; 7 on safety and patient population considerations; 3 on biomarkers/patient-reported outcomes; and 1 on research gaps.DiscussionAn established consensus method was used to develop statements relevant to the management of AQP4-IgG–seropositive NMOSD. These international statements will be valuable for informing individualized therapeutic decision-making and could form the basis for standardized practice guidelines.
Published: 2023

21. Serum neurofilament light chain levels at attack predict post-attack disability worsening and are mitigated by inebilizumab: analysis of four potential biomarkers in neuromyelitis optica spectrum disorder

Author: Orhan Aktas, Hans-Peter Hartung, Michael A Smith, William A Rees, Kazuo Fujihara, Friedemann Paul, Romain Marignier, Jeffrey L Bennett, Ho Jin Kim, Brian G Weinshenker, Sean J Pittock, Dean M Wingerchuk, Gary Cutter, Dewei She, Michele Gunsior, Daniel Cimbora, Eliezer Katz, and Bruce A Cree
Subjects: Psychiatry and Mental health, Surgery, Neurology (clinical)
Abstract: ObjectiveTo investigate relationships between serum neurofilament light chain (sNfL), ubiquitin C-terminal hydrolase L1 (sUCHL1), tau (sTau) and glial fibrillary acidic protein (sGFAP) levels and disease activity/disability in neuromyelitis optica spectrum disorder (NMOSD), and the effects of inebilizumab on these biomarkers in N-MOmentum.MethodsN-MOmentum randomised participants to receive inebilizumab or placebo with a randomised controlled period (RCP) of 28 weeks and an open-label follow-up period of ≥2 years. The sNfL, sUCHL1, sTau and sGFAP were measured using single-molecule arrays in 1260 scheduled and attack-related samples from N-MOmentum participants (immunoglobulin G (IgG) autoantibodies to aquaporin-4-positive, myelin oligodendrocyte glycoprotein-IgG-positive or double autoantibody-negative) and two control groups (healthy donors and patients with relapsing–remitting multiple sclerosis).ResultsThe concentration of all four biomarkers increased during NMOSD attacks. At attack, sNfL had the strongest correlation with disability worsening during attacks (Spearman R2=0.40; p=0.01) and prediction of disability worsening after attacks (sNfL cut-off 32 pg/mL; area under the curve 0.71 (95% CI 0.51 to 0.89); p=0.02), but only sGFAP predicted upcoming attacks. At RCP end, fewer inebilizumab-treated than placebo-treated participants had sNfL>16 pg/mL (22% vs 45%; OR 0.36 (95% CI 0.17 to 0.76); p=0.004).ConclusionsCompared with sGFAP, sTau and sUCHL1, sNfL at attack was the strongest predictor of disability worsening at attack and follow-up, suggesting a role for identifying participants with NMOSD at risk of limited post-relapse recovery. Treatment with inebilizumab was associated with lower levels of sGFAP and sNfL than placebo.Trial registration numberNCT02200770.
Published: 2023

22. Acute Optic Neuritis: what are the clues to the aetiological diagnosis in real life?

Author: Romain Deschamps, Natalia Shor, Catherine Vignal, Jessica Guillaume, Caroline Bensa, Augustin Lecler, Romain Marignier, Vivien Vasseur, Caroline Papeix, Marine Boudot de la Motte, and Cedric Lamirel
Subjects: Neurology, Neurology (clinical), General Medicine
Published: 2023

23. Maladie liée aux anticorps anti-MOG à début tardif : description d’une cohorte française

Author: Lionel Meens, Julie Pique, Jonathan Ciron, Elisabeth Maillart, Mikael Cohen, Romain Deschamps, and Romain Marignier
Subjects: Neurology, Neurology (clinical)
Published: 2023

24. Méningo-radiculite à Borrelia miyamotoi sous anti-CD20, identifiée par analyse méta-transcriptomique du LCR

Author: Philippe Nicolas, Gregory Destras, Antonin Bal, Sandra Vukusic, Romain Marignier, Sophie Jarraud, and Laurence Josset
Subjects: Neurology, Neurology (clinical)
Published: 2023

25. Immunité cellulaire et humorale anti-SARS-CoV-2 chez les patients SEP traités par anti-CD20

Author: Philippe Nicolas, Hugo Marion-Moffet, Morgane Gossez, Sandra Vukusic, Guillaume Monneret, Romain Marignier, and Fabienne Venet
Subjects: Neurology, Neurology (clinical)
Published: 2023

26. Grossesse et post-partum chez les patientes présentant une MOGAD

Author: Clarisse Carra-Dallière, Fabien Rollot, Romain Deschamps, Jonathan Ciron, Sandra Vukusic, Bertrand Audoin, Bertrand Bourre, Illiasse El-Bahi, Pierre Labauge, Aurélie Ruet, Elisabeth Maillart, Caroline Papeix, Hélène Zéphir, David Laplaud, Mikael Cohen, Romain Casey, Xavier Ayrignac, and Romain Marignier
Subjects: Neurology, Neurology (clinical)
Published: 2023

27. Maladies associées aux anticorps anti-MOG en pédiatrie : devenir scolaire et cognitif

Author: Audrey Mittelman, Anne Lise Poulat, Vincent des Portes, Julie Pique, Kumaran Deiva, and Romain Marignier
Subjects: Neurology, Neurology (clinical)
Published: 2023

28. Consensus Delphi d’experts internationaux pour la prise en charge des troubles du spectre de la neuromyélite optique positifs aux anticorps anti-aquaporine-4 – focus sur les recommandations de traitement avec eculizumab, inebilizumab et satralizumab

Author: Friedemann Paul, Romain Marignier, and Jacqueline Palace
Subjects: Neurology, Neurology (clinical)
Published: 2023

29. Pregnancy in Patients With AQP4-Ab, MOG-Ab, or Double-Negative Neuromyelitis Optica Disorder

Author: C. Papeix, Nicolas Collongues, Jacqueline Palace, Cécilia Alves Do Rego, Jérôme De Seze, Maria Isabel Leite, Romain Marignier, Ana Martins da Silva, Ernestina Santos, Elisabeth Maillart, Bertrand Bourre, and Damien Biotti
Subjects: Adult, medicine.medical_specialty, Abortion, Autoantigens, Preeclampsia, Myelin oligodendrocyte glycoprotein, Miscarriage, 03 medical and health sciences, 0302 clinical medicine, Pregnancy, Recurrence, medicine, Humans, Spectrum disorder, 030212 general & internal medicine, Autoantibodies, Retrospective Studies, Aquaporin 4, Neuromyelitis optica, biology, Obstetrics, business.industry, Neuromyelitis Optica, Postpartum Period, medicine.disease, Pregnancy Complications, biology.protein, Female, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), Serostatus, business, Immunosuppressive Agents, 030217 neurology & neurosurgery
Abstract: ObjectiveTo analyze the effects of pregnancy on neuromyelitis optica spectrum disorder (NMOSD) according to patients' serostatus and immunosuppressive therapy (IST).MethodsWe performed a retrospective multicenter international study on patients with NMOSD. Patients were tested for aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibodies (Ab). Informative pregnancies were reported when NMOSD onset occurred before or during pregnancy or up to 12 months postpartum. The mean annualized relapse rate (ARR) was calculated for the 12 months before conception, for each trimester of pregnancy, and postpartum. Events such as miscarriage, abortion, and preeclampsia were reported. IST was considered if taken in the 3 months before or during pregnancy.ResultsWe included 89 pregnancies (46 with AQP4-Ab, 30 with MOG-Ab, and 13 without either Ab) in 58 patients with NMOSD. Compared to the prepregnancy period, the ARR was lower during pregnancy in each serostatus group and higher during the postpartum period in patients with AQP4-Ab (p < 0.01). Forty-eight percent (n = 31) of pregnancies occurred during IST and these patients presented fewer relapses during pregnancy and the 12 months postpartum than untreated patients (26% vs 53%, p = 0.04). Miscarriages occurred in 10 (11%) pregnancies, and were mainly in patients with AQP4-Ab (with or without IST) and a previous history of miscarriage. Preeclampsia was reported in 2 (2%) patients who were AQP4-Ab-positive.ConclusionWe found a rebound in the ARR during the first postpartum trimester that was higher than the prepregnancy period only in AQP4-Ab-positive patients. Taking IST just before or during pregnancy reduces the risk of relapses in these conditions.
Published: 2021

30. The long‐term outcome of MOGAD: An observational national cohort study of 61 patients

Author: Valerie Touitou, Bertrand Audoin, Nicolas Collongues, Mikael Cohen, Romain Deschamps, David Laplaud, Nathalie Derache, Jonathan Ciron, Eric Thouvenot, Françoise Durand-Dubief, Caroline Papeix, Jennifer Aboab, Guillaume Mathey, Julie Pique, C. Vignal-Clermont, Aurélie Ruet, Elisabeth Maillart, Romain Marignier, Hélène Zéphir, Bertrand Bourre, Olivier Gout, and Xavier Ayrignac
Subjects: Pediatrics, medicine.medical_specialty, Optic Neuritis, Visual acuity, Bilateral blindness, medicine.medical_treatment, Urinary catheterization, National cohort, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, Optic neuritis, 030212 general & internal medicine, Autoantibodies, Retrospective Studies, Aquaporin 4, First episode, Expanded Disability Status Scale, business.industry, medicine.disease, Neurology, Myelin-Oligodendrocyte Glycoprotein, Observational study, Neurology (clinical), medicine.symptom, business, 030217 neurology & neurosurgery
Abstract: BACKGROUND AND OBJECTIVE The prognosis in myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a matter of debate. Our aim was to assess the long-term outcomes of patients with MOGAD. METHODS We retrospectively analysed the clinical and paraclinical data of patients from the French nationwide observatory study NOMADMUS who tested positive for MOG antibodies (MOG-IgG) and who had clinical follow-up of at least 8 years from their first episode. RESULTS Sixty-one patients (median [range] age at onset 27 [3-69] years), with a median (mean; range) follow-up of 177 (212.8; 98-657) months, were included. Among 58 patients with a relapsing course, 26.3% relapsed in the first year after onset. Of the 61 patients, 90.2% experienced at least one episode of optic neuritis. At last visit, the median (mean; range) Expanded Disability Status Scale (EDSS) score was 1 (2.12; 0-7.5), 12.5% had an EDSS score ≥6 and 37.5% had an EDSS score ≥3. Of 51 patients with final visual acuity (VA) data available, 15.7% had VA ≤0.1 in at least one eye and 25.5% had VA ≤0.5 in at least one eye. Bilateral blindness (VA ≤0.1) was present in 5.9% of patients. Finally, 12.5% of patients presented bladder dysfunction requiring long-term urinary catheterization. No factor associated significantly with a final EDSS score ≥3 or with final VA ≤0.1 was found. CONCLUSION Overall long-term favourable outcomes were achieved in a majority of our patients, but severe impairment, in particular visual damage, was not uncommon.
Published: 2021

31. MRI characteristics of MOG-Ab associated disease in adults: An update

Author: F. Durand-Dubief, Mikael Cohen, François Cotton, Elisabeth Maillart, Natalia Shor, Romain Marignier, Bertrand Bourre, Xavier Ayrignac, A Cobo Calvo, David-Axel Laplaud, C. Papeix, Kumaran Deiva, Jonathan Ciron, Nicolas Collongues, Bertrand Audoin, Hélène Zéphir, Romain Deschamps, Sandra Vukusic, Aurélie Ruet, Sorbonne Université - Faculté de Médecine (SU FM), Sorbonne Université (SU), Hôpital de la Fondation Ophtalmologique Adolphe de Rothschild [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Référence des Maladies Inflammatoires Rares du Cerveau et de la Moelle (MIRCEM), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), i-SEP (i-SEP), Hôpital neurologique et neurochirurgical Pierre Wertheimer [CHU - HCL], Hospices Civils de Lyon (HCL), RMN et optique : De la mesure au biomarqueur, Centre de Recherche en Acquisition et Traitement de l'Image pour la Santé (CREATIS), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale (U1215 Inserm - UB), Université de Bordeaux (UB)-Institut François Magendie-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Bordeaux [Bordeaux], Département de neurologie [Montpellier], Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [CHU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université de Montpellier (UM), Harvard Medical School [Boston] (HMS), Rutgers New Jersey Medical School (NJMS), Rutgers University System (Rutgers), Centre hospitalier universitaire de Nantes (CHU Nantes), Infectious Diseases Models for Innovative Therapies (IDMIT), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay, CHU Rouen, Normandie Université (NU), Assistance Publique - Hôpitaux de Marseille (APHM), CHU Strasbourg, Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université de Lyon, the NOMADMUS study group, and Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [Montpellier]-Université de Montpellier (UM)
Subjects: Adult, Pathology, medicine.medical_specialty, [SDV.IB.IMA]Life Sciences [q-bio]/Bioengineering/Imaging, Myelitis, Optic neuritis, Disease, Imaging, Myelin oligodendrocyte glycoprotein, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, Myelin Oligodendrocyte Glycoprotein, 030212 general & internal medicine, Autoantibodies, Aquaporin 4, Neuromyelitis optica, medicine.diagnostic_test, biology, business.industry, Multiple sclerosis, Neuromyelitis Optica, Magnetic resonance imaging, medicine.disease, Magnetic Resonance Imaging, Neurology, biology.protein, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), Differential diagnosis, business, Aquaporin-4, 030217 neurology & neurosurgery
Abstract: International audience; Our knowledge of the radiological spectrum of myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) is growing rapidly. An update on the radiological features of the disease, and its evolution is thus necessary. Magnetic resonance imaging (MRI) has an increasingly important role in the differential diagnosis of MOGAD particularly from aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD), and multiple sclerosis (MS). Differentiating these conditions is of prime importance because the management is different between the three inflammatory diseases, and thus could prevent further attack-related disability. Therefore, identifying the MRI features suggestive of MOGAD has diagnostic and prognostic implications. We herein review optic nerve, spinal cord and the brain MRI findings from MOGAD adult patients, and compare them to AQP4-NMOSD and MS.
Published: 2021

32. Clinical significance of a single cerebrospinal fluid immunoglobulin band: A retrospective study

Author: Guillaume Monneret, Morgane Gossez, Romain Marignier, Françoise Poitevin, Sandra Vukusic, Maud Tusseau, Julien Bancel, Estelle Cheli, and Christophe Malcus
Subjects: Central Nervous System, 030213 general clinical medicine, Pathology, medicine.medical_specialty, Central nervous system, Immunoglobulins, 03 medical and health sciences, 0302 clinical medicine, Cerebrospinal fluid, medicine, Humans, Clinical significance, Retrospective Studies, biology, business.industry, Multiple sclerosis, Retrospective cohort study, medicine.disease, medicine.anatomical_structure, Neurology, biology.protein, Neurology (clinical), Nervous System Diseases, Antibody, business, 030217 neurology & neurosurgery
Abstract: Background: To demonstrate an inflammatory process in the central nervous system, the presence of at least two immunoglobulin (Ig) bands in the cerebrospinal fluid (CSF) is required. So far, the presence of a single abnormal Ig band is considered as negative. Objective: The objective was to assess retrospectively the significance of a single CSF Ig band in clinical practice. Methods and results: Out of 10,286 CSF analyses, we retained 214 results with single Ig. An inflammatory neurological disorder was diagnosed in 41% of patients. Conclusion: Despite a modest sensitivity, the presence of a single CSF Ig band may be a biomarker of an inflammatory mechanism and, as such, may prompt the clinician to repeat the analysis when the clinical context remains suggestive.
Published: 2020

33. The frequent sign of a rare disease or the rare sign of a frequent disease?

Author: Romain Deschamps, Jérome Lambert, and Romain Marignier
Subjects: Optic Neuritis, Rare Diseases, Neurology, Humans, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), General Medicine, Tomography, Optical Coherence, Autoantibodies
Published: 2022

34. Risk factors for academic difficulties in children with myelin oligodendrocyte glycoprotein antibody‐associated acute demyelinating syndromes

Author: Alvaro Cobo-Calvo, Sandra Vukusic, Béatrice Husson, Kidbiosep Cohort, Aliénor de Chalus, Che Serguerra, Philippe Horellou, Romain Marignier, Hélène Maurey, Elise Yazbeck, and Kumaran Deiva
Subjects: Male, 030506 rehabilitation, Pediatrics, medicine.medical_specialty, Adolescent, Grey matter, Antibodies, Myelin oligodendrocyte glycoprotein, 03 medical and health sciences, 0302 clinical medicine, Developmental Neuroscience, Risk Factors, Interquartile range, Humans, Medicine, Child, Retrospective Studies, Academic Success, biology, business.industry, Encephalomyelitis, Acute Disseminated, Brain, Retrospective cohort study, Odds ratio, medicine.disease, Magnetic Resonance Imaging, Confidence interval, medicine.anatomical_structure, Pediatrics, Perinatology and Child Health, Cohort, Acute disseminated encephalomyelitis, biology.protein, Female, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), 0305 other medical science, business, 030217 neurology & neurosurgery, Demyelinating Diseases
Abstract: AIM To describe cognitive abilities through the evaluation of academic difficulties in children with acute demyelinating syndromes (ADS) and myelin oligodendrocyte glycoprotein (MOG) antibodies. METHOD This was an observational, retrospective study of a French paediatric cohort that included children aged 18 years and younger. Clinical, biological, and imaging data were collected and academic outcome was measured. RESULTS Seventy-six children were included in the study with a mean (SD) follow-up of 4 years 7 months (6y 4mo). Median age at disease onset was 9 years 1 months (interquartile range=4y 7mo-13y 11mo; 36 females, 40 males). Thirty-six children relapsed and 20 had academic difficulties at the last follow-up. Academic difficulties, as well as deep grey matter and putaminal lesions (p=0.047 and p=0.006 respectively), were significantly more prevalent in children aged 10 years and younger (p=0.02). Using univariate binary regression analysis, we found that age at disease onset of 10 years and younger (odds ratio [OR] 3.72 [95% confidence interval {CI} 1.19-11.64]; p=0.024), acute disseminated encephalomyelitis at disease onset (OR 52.5 [95% CI 5.97-461.4]; p
Published: 2020

35. Diagnosis and classification of optic neuritis

Author: Axel Petzold, Clare L Fraser, Mathias Abegg, Raed Alroughani, Daniah Alshowaeir, Regina Alvarenga, Cécile Andris, Nasrin Asgari, Yael Barnett, Roberto Battistella, Raed Behbehani, Thomas Berger, Mukharram M Bikbov, Damien Biotti, Valerie Biousse, Antonella Boschi, Milan Brazdil, Andrei Brezhnev, Peter A Calabresi, Monique Cordonnier, Fiona Costello, Franz M Cruz, Leonardo Provetti Cunha, Smail Daoudi, Romain Deschamps, Jerome de Seze, Ricarda Diem, Masoud Etemadifar, Jose Flores-Rivera, Pedro Fonseca, Jette Frederiksen, Elliot Frohman, Teresa Frohman, Caroline Froment Tilikete, Kazuo Fujihara, Alberto Gálvez, Riadh Gouider, Fernando Gracia, Nikolaos Grigoriadis, José M Guajardo, Mario Habek, Marko Hawlina, Elena H Martínez-Lapiscina, Juzar Hooker, Jyh Yung Hor, William Howlett, Yumin Huang-Link, Zhannat Idrissova, Zsolt Illes, Jasna Jancic, Panitha Jindahra, Dimitrios Karussis, Emilia Kerty, Ho Jin Kim, Wolf Lagrèze, Letizia Leocani, Netta Levin, Petra Liskova, Yaou Liu, Youssoufa Maiga, Romain Marignier, Chris McGuigan, Dália Meira, Harold Merle, Mário L R Monteiro, Anand Moodley, Frederico Moura, Silvia Muñoz, Sharik Mustafa, Ichiro Nakashima, Susana Noval, Carlos Oehninger, Olufunmilola Ogun, Afekhide Omoti, Lekha Pandit, Friedemann Paul, Gema Rebolleda, Stephen Reddel, Konrad Rejdak, Robert Rejdak, Alfonso J Rodriguez-Morales, Marie-Bénédicte Rougier, Maria Jose Sa, Bernardo Sanchez-Dalmau, Deanna Saylor, Ismail Shatriah, Aksel Siva, Hadas Stiebel-Kalish, Gabriella Szatmary, Linh Ta, Silvia Tenembaum, Huy Tran, Yevgen Trufanov, Vincent van Pesch, An-Guor Wang, Mike P Wattjes, Ernest Willoughby, Magd Zakaria, Jasmin Zvornicanin, Laura Balcer, Gordon T Plant, and UCL - SSS/IONS/CEMO - Pôle Cellulaire et moléculaire
Subjects: Aquaporin 4, Optic Neuritis, Multiple Sclerosis, Neuromyelitis Optica, Humans, Neurology (clinical), 610 Medicine & health, Retrospective Studies, Autoantibodies
Abstract: There is no consensus regarding the classification of optic neuritis, and precise diagnostic criteria are not available. This reality means that the diagnosis of disorders that have optic neuritis as the first manifestation can be challenging. Accurate diagnosis of optic neuritis at presentation can facilitate the timely treatment of individuals with multiple sclerosis, neuromyelitis optica spectrum disorder, or myelin oligodendrocyte glycoprotein antibody-associated disease. Epidemiological data show that, cumulatively, optic neuritis is most frequently caused by many conditions other than multiple sclerosis. Worldwide, the cause and management of optic neuritis varies with geographical location, treatment availability, and ethnic background. We have developed diagnostic criteria for optic neuritis and a classification of optic neuritis subgroups. Our diagnostic criteria are based on clinical features that permit a diagnosis of possible optic neuritis; further paraclinical tests, utilising brain, orbital, and retinal imaging, together with antibody and other protein biomarker data, can lead to a diagnosis of definite optic neuritis. Paraclinical tests can also be applied retrospectively on stored samples and historical brain or retinal scans, which will be useful for future validation studies. Our criteria have the potential to reduce the risk of misdiagnosis, provide information on optic neuritis disease course that can guide future treatment trial design, and enable physicians to judge the likelihood of a need for long-term pharmacological management, which might differ according to optic neuritis subgroups.
Published: 2022

36. Association of Maintenance Intravenous Immunoglobulin With Prevention of Relapse in Adult Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

Author: John J. Chen, Saif Huda, Yael Hacohen, Michael Levy, Itay Lotan, Adi Wilf-Yarkoni, Hadas Stiebel-Kalish, Mark A. Hellmann, Elias S. Sotirchos, Amanda D. Henderson, Sean J. Pittock, M. Tariq Bhatti, Eric R. Eggenberger, Marie Di Nome, Ho Jin Kim, Su-Hyun Kim, Albert Saiz, Friedemann Paul, Russell C. Dale, Sudarshini Ramanathan, Jacqueline Palace, Valentina Camera, Maria Isabel Leite, Byron L. Lam, Jeffrey L. Bennett, Sara Mariotto, Dave Hodge, Bertrand Audoin, Elisabeth Maillart, Romain Deschamps, Julie Pique, Eoin P. Flanagan, and Romain Marignier
Subjects: Male, maintenance intravenous immunoglobulin (IVIG), Immunoglobulins, Intravenous, adult patients, Cohort Studies, reduction in relapse frequency, Recurrence, Chronic Disease, Humans, Immunologic Factors, Female, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), Child, myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD), Autoantibodies, Retrospective Studies, Original Investigation
Abstract: IMPORTANCE: Recent studies suggest that maintenance intravenous immunoglobulin (IVIG) may be an effective treatment to prevent relapses in myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD); however, most of these studies had pediatric cohorts, and few studies have evaluated IVIG in adult patients. OBJECTIVE: To determine the association of maintenance IVIG with the prevention of disease relapse in a large adult cohort of patients with MOGAD. DESIGN, SETTING, AND PARTICIPANTS: This was a retrospective cohort study conducted from January 1, 2010, to October 31, 2021. Patients were recruited from 14 hospitals in 9 countries and were included in the analysis if they (1) had a history of 1 or more central nervous system demyelinating attacks consistent with MOGAD, (2) had MOG-IgG seropositivity tested by cell-based assay, and (3) were age 18 years or older when starting IVIG treatment. These patients were retrospectively evaluated for a history of maintenance IVIG treatment. EXPOSURES: Maintenance IVIG. MAIN OUTCOMES AND MEASURES: Relapse rates while receiving maintenance IVIG compared with before initiation of therapy. RESULTS: Of the 876 adult patients initially identified with MOGAD, 59 (median [range] age, 36 [18-69] years; 33 women [56%]) were treated with maintenance IVIG. IVIG was initiated as first-line immunotherapy in 15 patients (25%) and as second-line therapy in 37 patients (63%) owing to failure of prior immunotherapy and in 7 patients (12%) owing to intolerance to prior immunotherapy. The median (range) annualized relapse rate before IVIG treatment was 1.4 (0-6.1), compared with a median (range) annualized relapse rate while receiving IVIG of 0 (0-3) (t(108) = 7.14; P
Published: 2022

37. It is imperative to reconsider the concept of seronegative NMOSD

Author: Romain Marignier
Subjects: Neurology, Neurology (clinical)
Published: 2022

38. Interleukin-6 Receptor Blockade in Treatment-Refractory MOG-IgG–Associated Disease and Neuromyelitis Optica Spectrum Disorders

Author: Joachim Röther, Giovanni Novi, Friedemann Paul, Bertrand Audoin, Robert Berger, Nadja Siebert, Matthias Grothe, Antoine Gueguen, Katrin Giglhuber, Michael Deppe, Helen K Hayward-Könnecke, Jan-Patrick Stellmann, Markus Kraemer, Romain Deschamps, Axel Haarmann, Romain Marignier, Hélène Zéphir, Sven G. Meuth, Ingo Kleiter, Paulus S. Rommer, Damien Biotti, Klemens Ruprecht, Ilya Ayzenberg, Philipp Albrecht, Kerstin Hellwig, Anna Gahlen, Achim Berthele, Sven Jarius, Brigitte Wildemann, Corinna Trebst, Hans-Peter Hartung, Tania Kümpfel, Martin W. Hümmert, Tobias Zrzavy, Michael J. Levy, Daniel Whittam, Nicolas Collongues, Michael Karenfort, Gero Lindenblatt, Antonios Bayas, Orhan Aktas, Anu Jacob, Barbara Kornek, Jonathan Ciron, Ralf Gold, Katinka Fischer, Vivien Häußler, Marius Ringelstein, Sven Schippling, Heinrich Heine Universität Düsseldorf = Heinrich Heine University [Düsseldorf], Department of Neurology St. Josef-Hospital, Ruhr University Bochum, Germany, Department of Neurology Sechenov First Moscow State Medical University, Russia, Department of Neurology Johanna Etienne Hospital, Neuss, Germany, Department of Neurology San Martino Hospital, Genova, Italy, Neuroimmunology and MS Research Department of Neurology, University Hospital Zürich, Switzerland, Medizinische Universität Wien = Medical University of Vienna, Département Neurologie [CHU Toulouse], Pôle Neurosciences [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Institut Toulousain des Maladies Infectieuses et Inflammatoires (Infinity), Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Pôle de Neurosciences Cliniques, Service de Neurologie, APHM, Hôpital de la Timone, Aix-Marseille University, Marseille, France, Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), Centre d'Exploration Métabolique par Résonance Magnétique [Hôpital de la Timone - APHM] (CEMEREM), Hôpital de la Timone [CHU - APHM] (TIMONE)-Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS)-Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), Technical University of Munich, School of Medicine, Department of Neurology, Klinikum rechts der Isar, Germany, Université de Lille, Institute of Clinical Neuroimmunology, Faculty of Medicine, Ludwig Maximilian University, Munich, Department of Neurology, Asklepios Klinik Altona, Hamburg, Department of Neurology and Institute of Neuroimmunology and MS (INIMS), University Medical Center Hamburg-Eppendorf, Germany, Department of Neurology, The Walton Centre, Liverpool, United Kingdom, the Cleveland Clinic Abu Dhabi, UAE, Department of Neurology, Alfried Krupp Hospital, Essen, Germany, Department of Neurology, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France, Department of Neurology, Universitätsklinikum Augsburg, Department of Neurology, Hannover Medical School, Department of Neurology, University of Würzburg, Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Department of Neurology, University hospital Greifswald, NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Max Delbrueck Center for Molecular Medicine and Charité Universitätsmedizin Berlin, Freie Universität Berlin, Department of Neurology, University Hospital Strasbourg, Service de neurologie, sclérose en plaques, pathologies de la myéline et neuro-inflammation - Hôpital Neurologique Pierre Wertheimer Hospices Civils de Lyon, France, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, Department of Neurology, University Hospital, Münster, Department of Neurology Medical University of Vienna, Austria, Department of Neurology B4 unit, CRC-SEP, Toulouse Purpan University Hospital, France, Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre d'Exploration Métabolique par Résonance Magnétique [Hôpital de la Timone - AP-HM] (CEMEREM), and RANJEVA, Jean-Philippe
Subjects: Adult, Male, medicine.medical_specialty, Demyelinating Autoimmune Diseases, CNS, Antibodies, Monoclonal, Humanized, Gastroenterology, Article, chemistry.chemical_compound, Young Adult, Tocilizumab, Interquartile range, Internal medicine, Outcome Assessment, Health Care, medicine, Secondary Prevention, Humans, ddc:610, Adverse effect, Aquaporin 4, Expanded Disability Status Scale, business.industry, Neuromyelitis Optica, Chronic pain, [SDV.IMM.IMM]Life Sciences [q-bio]/Immunology/Immunotherapy, Middle Aged, medicine.disease, Receptors, Interleukin-6, Blockade, Neurology, chemistry, Interleukin-6 receptor, Rituximab, Female, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), [SDV.IMM.IMM] Life Sciences [q-bio]/Immunology/Immunotherapy, Function and Dysfunction of the Nervous System, business, medicine.drug
Abstract: Background and ObjectivesTo evaluate the long-term safety and efficacy of tocilizumab (TCZ), a humanized anti–interleukin-6 receptor antibody in myelin oligodendrocyte glycoprotein–IgG–associated disease (MOGAD) and neuromyelitis optica spectrum disorders (NMOSD).MethodsAnnualized relapse rate (ARR), Expanded Disability Status Scale score, MRI, autoantibody titers, pain, and adverse events were retrospectively evaluated in 57 patients with MOGAD (n = 14), aquaporin-4 (AQP4)-IgG seropositive (n = 36), and seronegative NMOSD (n = 7; 12%), switched to TCZ from previous immunotherapies, particularly rituximab.ResultsPatients received TCZ for 23.8 months (median; interquartile range 13.0–51.1 months), with an IV dose of 8.0 mg/kg (median; range 6–12 mg/kg) every 31.6 days (mean; range 26–44 days). For MOGAD, the median ARR decreased from 1.75 (range 0.5–5) to 0 (range 0–0.9; p = 0.0011) under TCZ. A similar effect was seen for AQP4-IgG+ (ARR reduction from 1.5 [range 0–5] to 0 [range 0–4.2]; p < 0.001) and for seronegative NMOSD (from 3.0 [range 1.0–3.0] to 0.2 [range 0–2.0]; p = 0.031). During TCZ, 60% of all patients were relapse free (79% for MOGAD, 56% for AQP4-IgG+, and 43% for seronegative NMOSD). Disability follow-up indicated stabilization. MRI inflammatory activity decreased in MOGAD (p = 0.04; for the brain) and in AQP4-IgG+ NMOSD (p < 0.001; for the spinal cord). Chronic pain was unchanged. Regarding only patients treated with TCZ for at least 12 months (n = 44), ARR reductions were confirmed, including the subgroups of MOGAD (n = 11) and AQP4-IgG+ patients (n = 28). Similarly, in the group of patients treated with TCZ for at least 12 months, 59% of them were relapse free, with 73% for MOGAD, 57% for AQP4-IgG+, and 40% for patients with seronegative NMOSD. No severe or unexpected safety signals were observed. Add-on therapy showed no advantage compared with TCZ monotherapy.DiscussionThis study provides Class III evidence that long-term TCZ therapy is safe and reduces relapse probability in MOGAD and AQP4-IgG+ NMOSD.
Published: 2021

39. Lésions inflammatoires démyélinisantes pseudo-kystiques dans la sclérose en plaques : description clinique, radiologique et anatomopathologique

Author: Florent Cluse, François Cotton, Tanguy Fenouil, Sandra Vukusic, Romain Marignier, and Françoise Durand-Dubief
Subjects: Neurology, Neurology (clinical)
Published: 2022

40. Diagnostic value of bright spotty lesions on MRI after a first episode of acute myelopathy

Author: Sylvain Rabasté, Alvaro Cobo-Calvo, Veronica Nistiriuc-Muntean, Sandra Vukusic, Romain Marignier, François Cotton, Audoin Bertrand, Ayrignac Xavier, Bertrand Bourre, Jonathan Ciron, Mikael Cohen, Nicolas Collongues, Romain Deschamps, Françoise Durand-Dubief, Julien Savatovsky, David Laplaud, Elisabeth Maillart, Caroline Papeix, Aurelie Ruet, Stéphane Kremer, Thomas Tourdias, Helene Zephir, Hôpital neurologique et neurochirurgical Pierre Wertheimer [CHU - HCL], Hospices Civils de Lyon (HCL), Service de neurologie [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU), CHU Toulouse [Toulouse], Hôpital Pasteur [Nice] (CHU), Service de Neurologie [Strasbourg], CHU Strasbourg-Hopital Civil, Service de Radiologie [Hôpital de la Croix-Rousse - HCL], Hôpital de la Croix-Rousse [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), RMN et optique : De la mesure au biomarqueur, Centre de Recherche en Acquisition et Traitement de l'Image pour la Santé (CREATIS), Université Jean Monnet [Saint-Étienne] (UJM)-Hospices Civils de Lyon (HCL)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Jean Monnet [Saint-Étienne] (UJM)-Hospices Civils de Lyon (HCL)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Fondation Ophtalmologique Adolphe de Rothschild [Paris], Centre de Recherche en Transplantation et Immunologie (U1064 Inserm - CRTI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Pierre Wertheimer, Département de Neurologie, CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Neuroinflammation: imagerie et thérapie de la sclérose en plaques, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de radiologie [Strasbourg], CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Service de Neurologie [Lyon], CHU Lyon, Université de Lille, Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), OFSEP, NOMADMUS Study Group: Audoin Bertrand , Ayrignac Xavier , Bertrand Bourre , Jonathan Ciron , Mikael Cohen , Nicolas Collongues , François Cotton , Romain Deschamps , Françoise Durand-Dubief , Julien Savatovsky , David Laplaud , Elisabeth Maillart , Romain Marignier , Caroline Papeix , Aurelie Ruet , Stéphane Kremer , Thomas Tourdias , Sandra Vukusic , Helene Zephir, CCSD, Accord Elsevier, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), and Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université Claude Bernard Lyon 1 (UCBL)
Subjects: medicine.medical_specialty, Cord, Myelitis, Bright, Spinal Cord Diseases, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, Myelopathy, 0302 clinical medicine, Medicine, Humans, Radiology, Nuclear Medicine and imaging, ComputingMilieux_MISCELLANEOUS, Retrospective Studies, First episode, Aquaporin 4, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, Neuromyelitis optica, Radiological and Ultrasound Technology, business.industry, Neuromyelitis Optica, Spotty, Retrospective cohort study, medicine.disease, Spinal cord, Magnetic Resonance Imaging, Sagittal plane, medicine.anatomical_structure, Neurology (clinical), Radiology, business, 030217 neurology & neurosurgery, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, MRI
Abstract: Background and purpose To determine the diagnostic value of bright spotty lesions (BSLs) for aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (NMOSDAQP4+), the predictive value of axial-BSLs for AQP4-IgG seropositivity, and the radio-clinical differences in NMOSDAQP4+ patients with and without axial-BSLs. Materials and methods Retrospective study that included patients aged ≥ 16 years, with a first acute spinal cord syndrome between 2005 and 2018 and abnormal spinal cord MRI with axial and sagittal T2 sequences. Patients with MRI findings consistent with compressive myelopathy were excluded. All spinal cord MRI were retrospectively evaluated for the presence of BSLs by 2 radiologists blinded to the diagnosis of acute myelopathy. Results A total of 82 patients were included; 15 aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder patients (NMOSDAQP4+), and 67 other patients, considered as the other causes of myelopathy (OM) group. The specificity of axial-BSLs for NMOSDAQP4+ patients was 94.0% (95% CI [85.6 to 97.7]). The sensitivity was 40.0% (95% CI [19.8 to 64.3]). In the multivariable analysis, the only MRI characteristic associated with AQP4-IgG positivity was the presence of axial-BSLs (OR: 9.2, 95% CI [1.2 to 72.9]; P = 0.022). In NMOSDAQP4+ patients, the median of cord expansion ratio was higher with axial-BSL (1.2, IQR [1.1–1.3]) than without axial-BSL (1.1, IQR [1.0–1.2]; P = 0.046). Conclusion After a first acute spinal cord syndrome, the presence of axial-BSLs on spinal cord MRI seems very specific for NMOSDAQP4+ and seems to be a predictor radiological marker of AQP4-IgG positivity.
Published: 2020

41. A meta-analysis comparing first-line immunosuppressants in neuromyelitis optica

Author: Elisabeth Maillart, Morgan McCreary, Bertrand Bourre, Su-Hyun Kim, Romain Deschamps, Xavier Ayrignac, Hélène Zéphir, Sandra Vukusic, David Laplaud, Luc Dauchet, Caroline Papeix, Benjamin Greenberg, Mikael Cohen, Romain Marignier, Aurélie Ruet, Ho Jin Kim, Jonathan Giovannelli, Nicolas Collongues, Nemos investigators, Bertrand Audoin, Jan‐Patrik Stellmann, Jonathan Ciron, Françoise Durand-Dubief, Ingo Kleiter, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Unité de Recherche Clinique de la Côte d’Azur (URRIS UR2CA), Centre Hospitalier Universitaire de Nice (CHU Nice)-Université Côte d'Azur (UCA), Hôpital Pasteur [Nice] (CHU), Hospital of National Cancer Center [Goyang], Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), Hôpital de la Timone [CHU - APHM] (TIMONE), Ruhr University Bochum (RUB), University of Texas Southwestern Medical Center [Dallas], Hôpital de la Fondation Ophtalmologique Adolphe de Rothschild [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de Référence des Maladies Inflammatoires Rares du Cerveau et de la Moelle (MIRCEM), Biopathologie de la Myéline, Neuroprotection et Stratégies Thérapeutiques (BMNST), Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg (UNISTRA), CHU Rouen, Normandie Université (NU), Centre hospitalier universitaire de Nantes (CHU Nantes), Centre de Recherche en Transplantation et Immunologie - Center for Research in Transplantation and Translational Immunology (U1064 Inserm - CR2TI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Nantes Université - UFR de Médecine et des Techniques Médicales (Nantes Univ - UFR MEDECINE), Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ), CHU Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Institut des Neurosciences de Montpellier (INM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Hôpital neurologique et neurochirurgical Pierre Wertheimer [CHU - HCL], Hospices Civils de Lyon (HCL), Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale (U1215 Inserm - UB), Université de Bordeaux (UB)-Institut François Magendie-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Bordeaux [Bordeaux], Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), CHU Lille, Facteurs de Risque et Déterminants Moléculaires des Maladies liées au Vieillissement - U 1167 (RID-AGE), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Lille Neurosciences & Cognition - U 1172 (LilNCog), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Universitaire Purpan, Unité de Recherche Clinique de la Côte d’Azur [Nice] (URRIS UR2CA), Université Côte d'Azur (UCA), and Université de Lyon-Université de Lyon-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Oncology, medicine.medical_specialty, First line, medicine.medical_treatment, MEDLINE, Neurosciences. Biological psychiatry. Neuropsychiatry, Azathioprine, Internal medicine, Outcome Assessment, Health Care, medicine, Humans, RC346-429, Research Articles, Neuromyelitis optica, business.industry, General Neuroscience, Hazard ratio, Neuromyelitis Optica, Immunosuppression, Mycophenolic Acid, medicine.disease, Meta-analysis, Rituximab, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], Neurology. Diseases of the nervous system, Neurology (clinical), business, Immunosuppressive Agents, RC321-571, medicine.drug, Research Article
Abstract: International audience; Objective : As phase III trials have shown interest in innovative but expensive drugs in the treatment of neuromyelitis optica spectrum disorder (NMOSD), data are needed to clarify strategies in the treatment of neuromyelitis optica (NMO). This meta-analysis compares the efficacy of first-line strategies using rituximab (RTX), mycophenolate mofetil (MMF), or azathioprine (AZA), which are still widely used. Methods : Studies identified by the systematic review of Huang et al. (2019) were selected if they considered at least two first-line immunosuppressants among RTX, MMF, and AZA. We updated this review. The Medline, Cochrane Central Register of Controlled Trials, Embase, and ClinicalTrials databases were queried between November 2018 and April 2020. To be included, the hazard ratio (HR) [95% CI] for the time to first relapse after first-line immunosuppression had to be available, calculable, or provided by the authors. Results : We gathered data from 919 NMO patients (232 RTX-, 294 MMF-, and 393 AZA-treated patients). The risk of first relapse after first-line immunosuppression was 1.55 [1.04, 2.31] (p = 0.03) for MMF compared with RTX, 1.42 [0.87, 2.30] (p = 0.16) for AZA compared with RTX, and 0.94 [0.58, 1.54] (p = 0.08) for MMF compared with AZA. Interpretation : The findings suggest that RTX is more efficient than MMF as a first-line therapy. Even if the results of our meta-analysis cannot conclude that RTX has a better efficacy in delaying the first relapse than AZA, the observed effect difference between both treatments combined with the results of previous studies using as outcome the annualized relapse rate may be in favor of RTX.
Published: 2021

42. Case Report: In Situ Expression of a Proliferation-Inducing Ligand in Neuromyelitis Optica

Author: Romain Marignier, Hans Lassmann, Bertrand Huard, and Laurie Baert
Subjects: 0301 basic medicine, Pathology, medicine.medical_specialty, APRIL (TNFSF13), neuromyelitis optica, Case Report, Lesion, 03 medical and health sciences, 0302 clinical medicine, medicine, Optic neuritis, RC346-429, B cells, Neuromyelitis optica, business.industry, TACI, Multiple sclerosis, astrocytes, Meninges, medicine.disease, Spinal cord, 030104 developmental biology, medicine.anatomical_structure, Neurology, Immunohistochemistry, Neurology. Diseases of the nervous system, Neurology (clinical), medicine.symptom, business, Infiltration (medical), 030217 neurology & neurosurgery
Abstract: A proliferation inducing ligand (APRIL) mediates a key role in the generation and survival of antibody-inducing plasmocytes. Based on this, APRIL has been targeted in autoimmune diseases including multiple sclerosis (MS) and optic neuritis (ON). In MS lesions, APRIL has a new cellular target, the reactive astrocyte and mediates an immunosuppressive activity. Here, we analyzed APRIL expression in a case of neuromyelitis optica (NMO), another autoimmune neurodegenerative disease, showing selective aquaporin-4 depletion in the spinal cord, complement deposition and infiltration of polymorphonuclear cells. We analyzed by immunohistochemistry the presence of APRIL-producing cells, plasmocytes, astrocytes and the localization of secreted APRIL in a lesion from NMO. Plasmocytes were present close to APRIL-producing cells in meninges. However, our main observation was that APRIL targets reactive astrocytes in this lesion of NMO similarly to MS.
Published: 2021

43. Oral nomegestrol acetate and transdermal 17-beta-estradiol for preventing post-partum relapses in multiple sclerosis: The POPARTMUS study

Author: Martine El-Etr, Jérôme De Seze, Patrick Hautecoeur, Françoise Durand-Dubief, Jean Pelletier, Etienne-Emile Baulieu, Francesco Patti, David-Axel Laplaud, L. Gignoux, Maria Trojano, Emmanuelle Le Page, Sandra Vukusic, Pierre Clavelou, Luca Durelli, Iuliana Ionescu, David Brassat, Eric Berger, Catherine Cornu, Laurent Remontet, Nadine Bossard, Giovanni Castelnovo, Marc Debouverie, François Cotton, Olivier Heinzlef, Romain Marignier, Thibault Moreau, Christine Lebrun-Frenay, Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Hôpital neurologique et neurochirurgical Pierre Wertheimer [CHU - HCL], Hospices Civils de Lyon (HCL), Observatoire Français de la Sclérose En Plaques [Lyon] (OFSEP), Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), Service Hospitalo-Universitaire de Pharmacologie et Toxicologie - Lyon (SHUPT Lyon), Service de Biostatistiques [Lyon], Centre de Recherche en Acquisition et Traitement de l'Image pour la Santé (CREATIS), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Département de Neuroradiologie [Centre Hospitalier Lyon Sud - HCL], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Università degli studi di Torino = University of Turin (UNITO), Centre hospitalier universitaire de Nantes (CHU Nantes), Centre de Recherche en Transplantation et Immunologie (U1064 Inserm - CRTI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Service de Neurologie générale, vasculaire et dégénérative (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Neuro-Dol (Neuro-Dol), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020]), CHU Strasbourg, Maladies chroniques, santé perçue, et processus d'adaptation (APEMAC), Université de Lorraine (UL), Centre de Physiopathologie Toulouse Purpan (CPTP), Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Pôle Neurosciences [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Assistance Publique - Hôpitaux de Marseille (APHM), Unité de Recherche Clinique de la Côte d’Azur (URRIS UR2CA), Centre Hospitalier Universitaire de Nice (CHU Nice)-Université Côte d'Azur (UCA), Centre d'Investigation Clinique [Rennes] (CIC), Université de Rennes (UR)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], Service de Neurologie [CHU Nimes] (Pôle NIRR), Hôpital Universitaire Carémeau [Nîmes] (CHU Nîmes), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes)-Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Service de Neurologie [CHRU Besançon], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Département de neurologie [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHI Poissy-Saint-Germain, Università degli studi di Bari Aldo Moro = University of Bari Aldo Moro (UNIBA), University of Catania [Italy], Petites Molécules de neuroprotection, neurorégénération et remyélinisation, Université Paris-Sud - Paris 11 (UP11)-Institut National de la Santé et de la Recherche Médicale (INSERM), Association Européenne contre les Leucodystrophies, Myelin Project, Fondation pour l'Aide à la Recherche sur la Sclérose en Plaques, Programme Hospitalier de Recherche Clinique 2005, Ministère des Affaires Sociales et de la Santé, Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre de recherche en neurosciences de Lyon (CRNL), Université de Lyon-Université de Lyon-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université Jean Monnet [Saint-Étienne] (UJM)-Hospices Civils de Lyon (HCL)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Università degli studi di Torino (UNITO), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Pôle neurosciences [Hôpital de Purpan - Toulouse], CHU Toulouse [Toulouse], Unité de Recherche Clinique de la Côte d’Azur [Nice] (URRIS UR2CA), Université Côte d'Azur (UCA), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Département de neurologie[Lille], Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Università degli studi di Bari Aldo Moro (UNIBA), and Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Université Claude Bernard Lyon 1 (UCBL)
Subjects: Nomegestrol acetate, medicine.medical_specialty, relapses, Norpregnadienes, [SDV]Life Sciences [q-bio], Relapse rate, progesterone, Gastroenterology, sex hormones, Multiple sclerosis, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Pregnancy, Recurrence, Internal medicine, estradiol, Medicine, Humans, Post partum, Transdermal, business.industry, Postpartum Period, post-partum, Megestrol, medicine.disease, 17 beta estradiol, 3. Good health, Neurology, chemistry, Female, Neurology (clinical), business, 030217 neurology & neurosurgery, 030215 immunology
Abstract: Background: Sex steroids could explain the course of multiple sclerosis (MS) in pregnancy. Objective: To compare the annualized relapse rate (ARR) 12 weeks post-partum in women treated with nomegestrol acetate (NOMAc) and 17-beta-estradiol (E2) versus placebo. Methods: POPARTMUS is a randomized, proof-of-concept trial in women with MS, receiving oral NOMAc 10 mg/day and transdermal estradiol 75 µg/week, or placebo. Results: Recruitment was stopped prematurely due to slow inclusions ( n = 202). No treatment effect was observed on ARR after 12 weeks (sex steroids = 0.90 (0.58–1.39), placebo = 0.97 (0.63–1.50) ( p = 0.79)). Conclusion: POPARTMUS failed showing efficacy of a NOMAc–E2 combination in preventing post-partum relapses.
Published: 2021

44. Prognostic Factors and Treatment Efficacy in Spinal Cord Sarcoidosis: An Observational Cohort With Long-term Follow-up

Author: Antoine Gavoille, Anne-Claire Desbois, Bastien Joubert, Cécile-Audrey Durel, Clément Auvens, Emilie Berthoux, Thierry Delboy, Jean François Dufour, Alin Turcu, Bernard Bonnotte, Thibault Moreau, Guillaume Le Guenno, Marc André, Marc Ruivard, Jean-Philippe Camdessanche, Jean-Christophe G. Antoine, Romain Marignier, Catherine Chapelon-Abric, David Saadoun, and Pascal Sève
Subjects: Treatment Outcome, Sarcoidosis, Spinal Cord, Contrast Media, Humans, Gadolinium, Neurology (clinical), Prognosis, Immunosuppressive Agents, Follow-Up Studies, Retrospective Studies
Abstract: Background and ObjectivesSpinal cord sarcoidosis is a rare manifestation of sarcoidosis with a consequent risk of neurologic sequelae for the patient. We investigated prognostic factors and efficacy of immunosuppressive treatments in a longitudinal cohort.MethodsWe retrospectively studied patients with spinal cord sarcoidosis followed between 1995 and 2021 in 7 centers in France. Patients with definite, probable, or possible spinal cord sarcoidosis according to the Neurosarcoidosis Consortium Consensus Group criteria and with spinal cord involvement confirmed by MRI were included. We analyzed relapse or progression rate with a Poisson model, initial Rankin score with a linear model, and change in the Rankin score during follow-up with a logistic model.ResultsA total of 97 patients were followed for a median of 7.8 years. Overall mean relapse or progression rate was 0.17 per person-year and decreased over time. At last visit, 46 (47.4%) patients had a loss of autonomy (Rankin score ≥2). The main prognostic factors significantly associated with relapse or progression rate were gadolinium enhancement (relative rate [95% CI] 0.61 [0.4, 0.95]) or meningeal involvement (relative rate [95% CI] 2.05 [1.31, 3.19]) on spinal cord MRI and cell count (relative rate [95% CI] per 1 log increase 1.16 [1.01, 1.33]) on CSF analysis. Relapse or progression rate was not significantly associated with initial Rankin score or Expanded Disability Status Scale. Tumor necrosis factor–α (TNF-α) antagonists significantly decreased relapse or progression rate compared with corticosteroids alone (relative rate [95% CI] 0.33 [0.11, 0.98]). Azathioprine was significantly less effective than methotrexate on relapse or progression rate (relative rate [95% CI] 2.83 [1.04, 7.75]) and change in Rankin score (mean difference [95% CI] 0.65 [0.23, 1.08]).DiscussionRegarding the relapse or progression rate, meningeal localization of sarcoidosis was associated with a worse prognosis, TNF-α antagonists resulted in a significant decrease compared to corticosteroids alone, and methotrexate was more effective than azathioprine.Classification of EvidenceThis study provides Class IV evidence that in individuals with spinal cord neurosarcoidosis, TNF-α antagonists were associated with decreased relapse or progression rate compared to corticosteroids alone, but other therapies showed no significant benefit.
Published: 2021

45. TNF-α inhibitors used as steroid-sparing maintenance monotherapy in parenchymal CNS sarcoidosis

Author: Romain Marignier, Thomas Sené, Sarah Demortiere, Jean Pelletier, Jean Capron, Bertrand Audoin, Mathieu Vaillant, Frédéric Hilezian, Claire Giannesini, Géraldine Androdias, Philippe Kerschen, Olivier Casez, Adil Maarouf, Jonathan Ciron, Guillaume Mathey, Jean-Philippe Camdessanché, Arsène Mekinian, Catalina Coclitu, Nicolas Collongues, Caroline Bensa-Koscher, Audrey Rico, Clemence Boutiere, Neurologie, maladies neuro-musculaires [Hôpital de la Timone - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), Médecine légale [Hôpital de la Timone - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), Service de neurologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier de Luxembourg [Luxembourg] (CHL), CHU Rothschild [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CHU Saint-Antoine [AP-HP], Service de Neurologie [CHU de Saint-Étienne], Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E)-Université Jean Monnet - Saint-Étienne (UJM), Hospices Civils de Lyon, Departement de Neurologie (HCL), Hôpital neurologique et neurochirurgical Pierre Wertheimer [CHU - HCL], Hospices Civils de Lyon (HCL), Service de Neurologie [Strasbourg], CHU Strasbourg-Hopital Civil, Pôle Psychiatrie et Neurologie [Grenoble], Centre Hospitalier Universitaire [Grenoble] (CHU), Neurologie générale et maladies inflammatoires du système nerveux [Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Assistance Publique - Hôpitaux de Marseille (APHM)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Hôpital Rothschild [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Neurologie [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Service de médecine interne [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de neurologie [CHU de Saint-Étienne], Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Hôpital Purpan [Toulouse], and CHU Toulouse [Toulouse]
Subjects: Neuro-Inflammation, medicine.medical_specialty, Necrosis, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Refractory, Internal medicine, medicine, Adalimumab, 030212 general & internal medicine, Dosing, business.industry, Multiple sclerosis, medicine.disease, Infliximab, 3. Good health, Psychiatry and Mental health, Surgery, Tumor necrosis factor alpha, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], Neurology (clinical), Sarcoidosis, medicine.symptom, business, 030217 neurology & neurosurgery, medicine.drug
Abstract: International audience; Objective To assess the efficacy of tumour necrosis factor-α (TNF-α) inhibitors used as steroid-sparing monotherapy in central nervous system (CNS) parenchymal sarcoidosis. Methods The French Multiple Sclerosis and Neuroinflammation Centers retrospectively identified patients with definite or probable CNS sarcoidosis treated with TNF-α inhibitors as steroid-sparing monotherapy. Only patients with CNS parenchymal involvement demonstrated by MRI and imaging follow-up were included. The primary outcome was the minimum dose of steroids reached that was not associated with clinical or imaging worsening during a minimum of 3 months after dosing change. Results Of the identified 38 patients with CNS sarcoidosis treated with TNF-α inhibitors, 23 fulfilled all criteria (13 females). Treatments were infliximab (n=22) or adalimumab (n=1) for a median (IQR) of 24 (17–40) months. At treatment initiation, the mean (SD) age was 41.5 (10.5) years and median (IQR) disease duration 22 (14–49.5) months. Overall, 60% of patients received other immunosuppressive agents before a TNF-α inhibitor. The mean (SD) minimum dose of steroids was 31.5 (33) mg before TNF-α inhibitor initiation and 6.5 (5.5) mg after (p=0.001). In all, 65% of patients achieved steroids dosing
Published: 2021

46. Myelin oligodendrocyte glycoprotein antibody-associated diffuse orbital inflammation

Author: Thomas Sené, Romain Deschamps, Olivier Gout, Romain Marignier, Guillaume Poillon, and Alexandre Marill
Subjects: Male, Chemosis, Pathology, medicine.medical_specialty, Optic Neuritis, Visual acuity, genetic structures, Exophthalmos, Visual Acuity, Myelin oligodendrocyte glycoprotein, 03 medical and health sciences, 0302 clinical medicine, Edema, medicine, Humans, Optic neuritis, 030212 general & internal medicine, Autoantibodies, Aged, 80 and over, Inflammation, biology, medicine.diagnostic_test, business.industry, Multiple sclerosis, Magnetic resonance imaging, medicine.disease, eye diseases, Neurology, biology.protein, Myelin-Oligodendrocyte Glycoprotein, sense organs, Neurology (clinical), medicine.symptom, business, 030217 neurology & neurosurgery
Abstract: An 87-year-old man presented with a 2-day history of painful bilateral visual loss. On examination, exophthalmos, lid edema, chemosis, and optic disc edema, on the left side only, were found. Visual acuity was 4/10 OD and no light perception OS. Magnetic resonance imaging (MRI) revealed bilateral optic neuritis and a diffuse and severe infiltration of the intra- and extraconal fat on the left. Laboratory testing was negative except for serum myelin oligodendrocyte glycoprotein (MOG) antibodies. This presentation adds a new variant to the MOG-associated disease spectrum. Testing for MOG antibodies should be considered in patients presenting with diffuse orbital inflammation and optic neuritis.
Published: 2020

47. Clinical spectrum of central nervous system myelin oligodendrocyte glycoprotein autoimmunity in adults

Author: Alvaro Cobo-Calvo, Sandra Vukusic, and Romain Marignier
Subjects: Adult, 0301 basic medicine, biology, business.industry, Specific detection, Treatment outcome, Central nervous system, Demyelinating Autoimmune Diseases, CNS, medicine.disease_cause, Myelin oligodendrocyte glycoprotein, Autoimmunity, 03 medical and health sciences, Treatment Outcome, 030104 developmental biology, 0302 clinical medicine, medicine.anatomical_structure, Neurology, biology.protein, Humans, Medicine, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), business, Neuroscience, 030217 neurology & neurosurgery
Abstract: The clinical interest for auto-antibodies against myelin oligodendrocyte glycoprotein (MOG) has recently reemerged, with the use of more specific detection methods. Large national cohorts have allowed characterizing a more precise clinical spectrum delineated by the presence of human MOG-antibodies.In adults with MOG-antibodies, optic neuritis is the most frequent clinical presentation, with features different from multiple sclerosis (MS), including bilateral involvement and predilection for the anterior part of the optic nerve. Myelitis and brainstem syndrome are also frequent, and may clinically mimic neuromyelitis optica spectrum disorders (NMOSD). Despite the frequently severe clinical presentation, most of patients recover quickly after steroids initiation. Other less typical presentations include encephalitis with seizures, cranial nerve involvement, and chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids-like. Although the majority of adult patients follow a relapsing course, long-term prognosis differs from aquaporin-4-antibodies NMOSD, with only a small proportion of patients with a poor outcome.MOG-antibodies-associated disease is a new entity in the spectrum of inflammatory demyelinating diseases, distinct from both MS and NMOSD. There is a crucial need to identify factors associated to the risk of relapse or poor outcome, to seek patient subgroups in which immunoactive treatments could be beneficial.
Published: 2019

48. Frequency and characteristics of short versus longitudinally extensive myelitis in adults with MOG antibodies: A retrospective multicentric study

Author: Carole Henry, Sandra Vukusic, Alexis Montcuquet, Clarisse Gagou-Scherer, Mikael Cohen, Bruno Brochet, Nathalie Derache, Alvaro Cobo-Calvo, David Brassat, Caroline Papeix, Philippe Kerschen, Françoise Durand-Dubief, Jonathan Ciron, Nicolas Collongues, Hélène Zéphir, Nicolas Maubeuge, Thibault Moreau, Romain Deschamps, Aude Maurousset, Romain Marignier, Matthieu Bereau, Fabien Rollot, Eric Thouvenot, Clarisse Carra-Dalliere, Christophe Prat, David Laplaud, Frederic Taithe, Ayman Tourbah, Elisabeth Maillart, Bertrand Bourre, Guillaume Mathey, Bertrand Audoin, Département de Neurologie [Toulouse], Institut des Neurosciences [Toulouse], Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT), Hôpital neurologique et neurochirurgical Pierre Wertheimer [CHU - HCL], Hospices Civils de Lyon (HCL), Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Neurologie, maladies neuro-musculaires [Hôpital de la Timone - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Service de neurologie [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Neurologie générale et maladies inflammatoires du système nerveux [Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de Neurologie [CHU Nice], Hôpital Pasteur [Nice] (CHU)-Centre Hospitalier Universitaire de Nice (CHU Nice), Biopathologie de la Myéline, Neuroprotection et Stratégies Thérapeutiques (BMNST), Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital de la Fondation Ophtalmologique Adolphe de Rothschild [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de neurologie [Nantes], Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Hôpital Guillaume-et-René-Laennec [Saint-Herblain], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Département de neurologie [Lille], Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Service de neurologie [Bordeaux], CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de Neurologie [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Service de neurologie [Angers], Université d'Angers (UA)-Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Centre Hospitalier de Saint-Denis [Ile-de-France], Centre Hospitalier de Luxembourg [Luxembourg] (CHL), Service de neurologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service de neurologie [Poitiers], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Service de Neurologie [CHU Limoges], CHU Limoges, Service de Neurologie générale, vasculaire et dégénérative (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier d'Angoulême (CH Angoulême), CHU Clermont-Ferrand, Hôpital Universitaire Carémeau [Nîmes] (CHU Nîmes), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Service de neurologie [Reims], Centre Hospitalier Universitaire de Reims (CHU Reims), Laboratoire de Psychopathologie et Neuropsychologie (LPN), Université Paris 8 Vincennes-Saint-Denis (UP8), Université de Lyon, Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées, Centre de recherche en neurosciences de Lyon (CRNL), Université de Lyon-Université de Lyon-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse], Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Département de neurologie[Lille], Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: Adult, Male, Pathology, medicine.medical_specialty, Myelitis, MESH: Myelitis / physiopathology, Prognosis, Registries, Retrospective Studies, Severity of Illness Index, Young Adult, Transverse myelitis, Myelin oligodendrocyte glycoprotein, 03 medical and health sciences, Myelin, 0302 clinical medicine, MESH: Adult, Aged, Autoantibodies, Disease Progression, Female, Follow-Up Studies, Humans, Magnetic Resonance Imaging, Medicine, 10. No inequality, 030304 developmental biology, 0303 health sciences, biology, business.industry, Multiple sclerosis, Middle Aged, medicine.disease, 3. Good health, medicine.anatomical_structure, Neurology, Cohort, biology.protein, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), MESH: Male, Myelin-Oligodendrocyte Glycoprotein / immunology, Myelitis / diagnosis, Myelitis / immunology, Myelitis / pathology, Antibody, business, 030217 neurology & neurosurgery
Abstract: Objectives: We aim to (1) determine the frequency and distinctive features of short myelitis (SM) and longitudinally extensive transverse myelitis (LETM) in a cohort of adults with myelin oligodendrocyte glycoprotein (MOG)-antibody (Ab)-associated myelitis and (2) determine baseline prognostic factors among MOG-Ab-positive patients whose disease started with myelitis. Material and Methods: We retrospectively analyzed clinical and paraclinical variables from a multicentric French cohort of adults with MOG-Ab-associated myelitis. At last follow-up, patients were classified into two groups according to the severity of the Expanded Disability Status Scale (EDSS) as ⩽2.5 or ⩾3.0. Results: Seventy-three patients with at least one episode of myelitis over disease course were included; among them, 28 (38.4%) presented with SM at the time of the first myelitis. Motor and sphincter involvement was less frequently observed in SM (51.9% and 48.2%, respectively) than in LETM patients (83.3% and 78.6%, respectively), p = 0.007 and p = 0.017; 61% of LETM patients displayed brain lesions compared to 28.6% in the SM group, p = 0.008, and the thoracic segment was more frequently involved in the LETM (82.2%) than in the SM group (39.3%), p Conclusion: SM in MOG-Ab-associated disease is not rare. The severity at onset was the only independent factor related to the final prognosis in MOG-Ab-associated myelitis.
Published: 2019

49. Pathologic and MRI analysis in acute atypical inflammatory demyelinating lesions

Author: Thierry Vincent, A.-M. Guennoc, Pierre Labauge, Anne Kerbrat, François Cotton, Stéphane Kremer, Béatrice Carsin-Nicol, Mahmoud Charif, Sandra Vukusic, Thibaut Allou, Jérôme De Seze, Aurélie Ruet, Laurent Magy, Nicolas Menjot de Champfleur, Clarisse Carra-Dalliere, Guido Ahle, Eric Thouvenot, Françoise Durand-Dubief, Hélène Oesterle, Valérie Rigau, Denis Sablot, Romain Marignier, Nicolas Collongues, Gilles Edan, Mikael Cohen, Sonia Hebbadj, Benoit Lhermitte, Frederic Taithe, M. P. Boncoeur-Martel, Xavier Ayrignac, Grelier, Elisabeth, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Institut des Neurosciences de Montpellier (INM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Service d'Anatomie Pathologique Générale, CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Département d’Immunologie, Département de neurologie [Montpellier], Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [Montpellier]-Université de Montpellier (UM), Service de Neurologie [Strasbourg], CHU Strasbourg-Hopital Civil, Service de radiologie [Strasbourg], Service de Neurologie [Hôpitaux Civils de Colmar], Hôpitaux Civils Colmar, Hôpitaux Civils de Colmar, Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), RMN et optique : De la mesure au biomarqueur, Centre de Recherche en Acquisition et Traitement de l'Image pour la Santé (CREATIS), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Pierre Wertheimer, Département de Neurologie, Service de Neurologie [Lyon], CHU Lyon, Service de Neurologie [CHU Clermont-Ferrand], CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand-CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand, Hôpital Pasteur [Nice] (CHU), Service de Neurologie [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)-Hôpital Bretonneau, Service de Neurologie [Rennes] = Neurology [Rennes], CHU Pontchaillou [Rennes], Service de neurologie [Rennes], Université de Rennes (UR), Service de neurochirurgie [Rennes] = Neurosurgery [Rennes], Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Service de Neurologie [CHU Nimes] (Pôle NIRR), Hôpital Universitaire Carémeau [Nîmes] (CHU Nîmes), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes)-Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Neuroinflammation: imagerie et thérapie de la sclérose en plaques, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Neurologie [CHU Limoges], CHU Limoges, Neuroépidémiologie Tropicale (NET), CHU Limoges-Institut d'Epidémiologie Neurologique et de Neurologie Tropicale-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST), Université de Limoges (UNILIM)-Université de Limoges (UNILIM), Service de Radiologie et Imagerie Médicale [CHU Limoges], Institut des Neurosciences de Montpellier - Déficits sensoriels et moteurs (INM), Hôpital Gui de Chauliac [Montpellier]-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université Montpellier 1 (UM1)-Université de Montpellier (UM), Université Jean Monnet [Saint-Étienne] (UJM)-Hospices Civils de Lyon (HCL)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Jean Monnet [Saint-Étienne] (UJM)-Hospices Civils de Lyon (HCL)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université Claude Bernard Lyon 1 (UCBL), Hôpital Bretonneau-Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [CHU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université de Montpellier (UM), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), and Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Adult, Male, Atypical inflammatory demyelinating syndrome, medicine.medical_specialty, Pathology, Multiple Sclerosis, Histopathology, Context (language use), Immunopathology, Cohort Studies, Lesion, Young Adult, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, 030212 general & internal medicine, Pathological, Aged, Retrospective Studies, Neuroradiology, Aquaporin 4, medicine.diagnostic_test, business.industry, Multiple sclerosis, Brain biopsy, Neuromyelitis Optica, food and beverages, Magnetic resonance imaging, Middle Aged, medicine.disease, Atypical demyelinating lesions, Magnetic Resonance Imaging, 3. Good health, Neurology, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, Acute Disease, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Female, Neurology (clinical), medicine.symptom, business, Aquaporin-4, 030217 neurology & neurosurgery, Demyelinating Diseases
Abstract: The diagnosis of atypical inflammatory demyelinating lesions can be difficult. Brain biopsy is often required to exclude neoplasms. Moreover, the relationship between these lesions and multiple sclerosis and NMOSD is not clear. Our objectives were to describe radiological and pathological characteristics of patients with acute inflammatory demyelinating lesions. We retrospectively identified patients with brain biopsy performed for diagnostic uncertainty revealing a demyelinating lesion. A complete clinical, biological, radiological and pathological analysis was performed. Twenty patients (15 with a single lesion) were included. MRI disclosed a wide range of lesions including infiltrative lesions (40%), ring-like lesion (15%) Balo-like lesion (15%) and acute haemorrhagic leukoencephalitis (20%). In spite of a marked heterogeneity, some findings were common: a peripheral B1000 hyperintense rim (70%), a slight oedema with mild mass effect (75%) and an open-rim peripheral enhancement (75%). Histopathology revealed that all cases featured macrophages distributed throughout, extensive demyelination, axonal preservation and absence of haemorrhagic changes. In the majority of cases, macrophages were the predominant inflammatory infiltrate and astrocytes were reactive and dystrophic. Aquaporin-4 staining was systematically preserved. After a mean follow-up of 5 years (1–12), 16/20 patients had a diagnosis of monophasic acute atypical inflammatory demyelinating lesion. One patient was diagnosed with MS and 3 with AQP4 negative NMOSD. Although imaging findings in patients with atypical inflammatory demyelinating lesions are heterogeneous, some common features such as peripheral DWI hyperintense rim with open-rim enhancement and absence of oedema argue in favour of a demyelinating lesion and should preclude a brain biopsy. In this context, AQP4 staining is systematically preserved and argues against an AQP4-positive NMOSD. Moreover, long-term follow-up is characterized by low recurrence rate.
Published: 2019

50. Usefulness of MOG-antibody titres at first episode to predict the future clinical course in adults

Author: Nuria Sola-Valls, Sandra Vukusic, Françoise Durand-Dubief, Eric Thouvenot, Damien Biotti, Hélène Zéphir, Sara Llufriu, Caroline Papeix, Elisabeth Maillart, Romain Deschamps, Nicolas Collongues, Albert Saiz, Pierre Labauge, Alexis Montcuquet, Anne Ruiz, Thaís Armangue, Alvaro Cobo-Calvo, Maria S. Sepúlveda, Xavier Ayrignac, Bertrand Audoin, Jérôme De Seze, Hyacintha d'Indy, Yolanda Blanco, Romain Marignier, Jonathan Ciron, Hôpital neurologique et neurochirurgical Pierre Wertheimer [CHU - HCL], Hospices Civils de Lyon (HCL), Centre de recherche en neurosciences de Lyon (CRNL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Universitat de Barcelona (UB), Sant Joan de Déu-Barcelona Children's Hospital's, Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Neurologie, maladies neuro-musculaires [Hôpital de la Timone - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Service de Neurologie [Hôpital Roger Salengro], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Département de Neurologie [Toulouse], Institut des Neurosciences [Toulouse], Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées, Service de Neurologie [Strasbourg], CHU Strasbourg-Hopital Civil, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre référent Sclérose Latérale Amyotrophique [CHRU Montpellier] (SLA CHRU Montpellier), Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de Neurologie [CHU Nimes] (Pôle NIRR), Hôpital Universitaire Carémeau [Nîmes] (CHU Nîmes), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes)-Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Service de Neurologie [CHU Limoges], CHU Limoges, Fondation Ophtalmologique Adolphe de Rotschild, Clinical and experimental neuroimmunology [IDIBAPS], Institut d'Investigacions Biomèdiques August Pi i Sunyer, Hospices Civils de Lyon, Departement de Neurologie (HCL), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université de Toulouse (UT)-Université de Toulouse (UT)-Université Toulouse III - Paul Sabatier (UT3), and Université de Toulouse (UT)-Université de Toulouse (UT)
Subjects: Male, Visual acuity, Optic neuritis, Logistic regression, Gastroenterology, 0302 clinical medicine, Recurrence, immune system diseases, Medicine, 030212 general & internal medicine, 10. No inequality, First episode, hemic and immune systems, Middle Aged, Myelitis, Prognosis, Neuromyelitis optica, 3. Good health, Titer, MESH: Autoantibodies / blood, Demyelinating diseases / immunology, HEK293 Cells, Young Adults, Neurology, Titre, Cohort, Disease Progression, [SDV.IMM]Life Sciences [q-bio]/Immunology, Biological Assay, Female, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], medicine.symptom, Adult, medicine.medical_specialty, Adolescent, MOG antibodies, Young Adult, 03 medical and health sciences, Internal medicine, Humans, [SDV.MHEP.OS]Life Sciences [q-bio]/Human health and pathology/Sensory Organs, Autoantibodies, Retrospective Studies, business.industry, Proportional hazards model, Retrospective cohort study, medicine.disease, nervous system diseases, nervous system, Myelin-Oligodendrocyte Glycoprotein, Neurology (clinical), business, 030217 neurology & neurosurgery, Demyelinating Diseases, Follow-Up Studies
Abstract: OBJECTIVE: To analyze whether myelin oligodendrocyte glycoprotein antibody (MOG-Ab) titres at onset of the disease were different according to the clinical phenotype at presentation, and to investigate whether the titres were associated with risk of further relapses or predicted clinical outcome in adult patients. Finally, we assessed an alternative method to the classical measurement of MOG-Ab levels by serial dilutions. METHODS: This is a retrospective study including 79 MOG-Ab-positive adult patients, whose samples were obtained at first episode. MOG-Ab were tested by cell-based assay. HEK293 cells were transfected (tHEK293) with human-MOG plasmid. Non-tHEK293 cells were used as negative controls. Assessment of antibody titres was performed by serial dilution, and delta mean fluorescence intensity ratio signal (MOG-ratio MFI) by flow cytometry. MOG-ratio MFI was calculated as follows: (MFI tHEK293cells- MFI non-tHEK293cells)/MFI non-tHEK293cells. MOG-ratio MFI was calculated from the first serum dilution at 1:320. The association between MOG-Ab titres and risk of relapse was analyzed by Cox regression. The association between MOG-Ab titres and visual or motor disability at last follow-up was performed by binary logistic regression. Poor visual outcome was defined when patients displayed some degree of visual disability (visual acuity [VA] < 20/20) and poor motor outcome when patients displayed some degree of motor disability (Disability Status Scale [DSS] > 1). We also investigated correlations between MOG-Ab titres and MOG-ratio MFI. RESULTS: MOG-Ab titres were higher in Caucasians than in those with other ethnicities, and in patients with a more severe VA (VA = 20/100) or motor disability (DSS = 3.0) at onset (p = 0.006, 0.034, and 0.058, respectively). MOG-Ab titres were not associated with risk of relapses or with the final clinical outcome. MOG-ratio MFI correlated with MOG-Ab titres in the whole cohort (? = 0.90; p < 0.001), and when stratified by initial clinical phenotype. CONCLUSION: High MOG-Ab titres at onset are associated with a more severe presentation, but do not predict the future disease course. MOG-ratio MFI is an alternative and straightforward method to determine MOG-Ab levels.
Published: 2019

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