, 8% (95%CI 48.7-96.6; Log-rank p = 0.458) for optic neuritis and transverse myelitis, and 44.4% (95%CI 19.6-79.6; Log-rank p = 0.617) for encephalopathy/brainstem syndrome. (B) Time to NMOSD-like phenotype conversion according to clinical phenotype at the onset. The 2-year risk to NMOSD-like phenotype conversion was 17, Figure S1. (A) Kaplan-Meier estimation of time to first relapse, according to clinical phenotype at the onset. The 2-year risk of the first relapse was 70.7% (95%CI 60.7-80.1) for optic neuritis (reference), vol.2, pp.64-95

, AQP4: Aquaporin-4; ARR: Annualised relapse rates

, CBA: Cell-based assay; CNS: Central nervous system

, CYC: Cyclophosphamide; EDSS: Expanded Disability Status Scale; IS: Immunosuppressants; IVIG: Intravenous immunoglobulins; MADEM: Multiphasic acute disseminated encephalomyelitis; MiTX: Mitoxantrone; MMF: Mycophenolate mophetil, CSF: Cerebrospinal fluid

, MOG: Myelin oligodendrocyte glycoprotein; MRI: Magnetic resonance imaging; MS: Multiple sclerosis; MS-DMD: MS disease-modifying drugs

, MTX: Methotrexate; NMOSD: Neuromyelitis optica spectrum disorders

, OCB: Oligoclonal bands; ON: Optic neuritis; PS: Propensity score

, RTX: Rituximab; SD: Standard deviation; TM: Transverse myelitis; VA: Visual acuity Faculté de Médecine Lyon-Est

, Observatoire Francais de la Sclérose En Plaques (OFSEP), Hôpital Pierre-Wertheimer

, APHP, issue.7

, Hôpital Pasteur, vol.2

, Centre de référence des maladies inflammatoires rares du cerveau et de la moelle (MIRCEM)

J. Kitley, P. Waters, M. Woodhall, M. I. Leite, A. Murchison et al., Neuromyelitis optica spectrum disorders with aquaporin-4 and myelinoligodendrocyte glycoprotein antibodies: a comparative study, JAMA Neurol, vol.71, pp.276-83, 2014.

D. K. Sato, D. Callegaro, L. , M. A. Waters, P. J. De-haidar-jorge et al., Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders, Neurology, vol.82, pp.474-81, 2014.

Y. Hacohen, M. Absoud, K. Deiva, C. Hemingway, P. Nytrova et al., Myelin oligodendrocyte glycoprotein antibodies are associated with a non-MS course in children, Neurol Neuroimmunol Neuroinflammation, vol.2, p.81, 2015.

R. Höftberger, M. Sepulveda, T. Armangue, Y. Blanco, K. Rostásy et al., Antibodies to MOG and AQP4 in adults with neuromyelitis optica and suspected limited forms of the disease, Mult Scler, vol.21, pp.866-74, 2015.

E. Hennes, M. Baumann, K. Schanda, B. Anlar, B. Bajer-kornek et al., Prognostic relevance of MOG antibodies in children with an acquired demyelinating syndrome, Neurology, vol.89, pp.900-908, 2017.

M. Jurynczyk, R. Geraldes, F. Probert, M. R. Woodhall, P. Waters et al., Distinct brain imaging characteristics of autoantibody-mediated CNS conditions and multiple sclerosis, Brain, vol.140, pp.617-644, 2017.

S. Jarius, F. Paul, O. Aktas, N. Asgari, R. C. Dale et al., MOG encephalomyelitis: international recommendations on diagnosis and antibody testing, J Neuroinflammation, vol.15, p.134, 2018.

M. Sepúlveda, T. Armangue, E. Martinez-hernandez, G. Arrambide, N. Solavalls et al., Clinical spectrum associated with MOG autoimmunity in adults: significance of sharing rodent MOG epitopes, J Neurol, vol.263, pp.1349-60, 2016.

M. Jurynczyk, S. Messina, M. R. Woodhall, N. Raza, R. Everett et al., Clinical presentation and prognosis in MOG-antibody disease: a UK study, Brain, vol.140, pp.3128-3166, 2017.

S. Kim, M. R. Woodhall, J. Kim, S. Kim, K. S. Park et al., Antibodies to MOG in adults with inflammatory demyelinating disease of the CNS, Neurol Neuroimmunol Neuroinflammation, vol.2, p.163, 2015.

S. Hamid, D. Whittam, M. Saviour, A. Alorainy, K. Mutch et al., Seizures and encephalitis in myelin oligodendrocyte glycoprotein IgG disease vs aquaporin 4 IgG disease, JAMA Neurol, vol.75, pp.65-71, 2017.

S. Jarius, K. Ruprecht, I. Kleiter, N. Borisow, N. Asgari et al., MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 1: frequency, syndrome specificity, influence of disease activity, long-term course, association with AQP4-IgG, and origin, J Neuroinflammation, vol.13, p.279, 2016.

A. Cobo-calvo, A. Ruiz, E. Maillart, B. Audoin, H. Zephir et al., Clinical spectrum and prognostic value of CNS MOG autoimmunity in adults: the MOGADOR study, Neurology, vol.90, pp.1858-69, 2018.
URL : https://hal.archives-ouvertes.fr/hal-01919569

S. Mader, V. Gredler, K. Schanda, K. Rostasy, I. Dujmovic et al., Complement activating antibodies to myelin oligodendrocyte glycoprotein in neuromyelitis optica and related disorders, J Neuroinflammation, vol.8, p.184, 2011.

S. Jarius, I. Kleiter, K. Ruprecht, N. Asgari, K. Pitarokoili et al., MOGIgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: brainstem involvement -frequency, presentation and outcome, J Neuroinflammation, vol.13, p.281, 2016.

S. Jarius, K. Ruprecht, I. Kleiter, N. Borisow, N. Asgari et al., MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome, J Neuroinflammation, vol.13, p.280, 2016.

Y. Hacohen, Y. Y. Wong, C. Lechner, M. Jurynczyk, S. Wright et al., Disease course and treatment responses in children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease, JAMA Neurol, vol.75, pp.478-87, 2018.

S. Ramanathan, S. Mohammad, E. Tantsis, T. K. Nguyen, V. Merheb et al., Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination, J Neurol Neurosurg Psychiatry, vol.89, pp.127-164, 2017.

M. Sepúlveda, T. Armangué, N. Sola-valls, G. Arrambide, J. E. Meca-lallana et al., Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus, Neurol Neuroimmunol Neuroinflammation, vol.3, p.225, 2016.

J. Sellner, M. Boggild, M. Clanet, R. Q. Hintzen, Z. Illes et al., EFNS guidelines on diagnosis and management of neuromyelitis optica, Eur J Neurol, vol.17, pp.1019-1051, 2010.

C. Trebst, S. Jarius, A. Berthele, F. Paul, S. Schippling et al., Update on the diagnosis and treatment of neuromyelitis optica: recommendations of the Neuromyelitis Optica Study Group (NEMOS), J Neurol, vol.261, pp.1-16, 2014.

P. C. Austin, Variance estimation when using inverse probability of treatment weighting (IPTW) with survival analysis, Stat Med, vol.35, pp.5642-55, 2016.

P. C. Austin, Balance diagnostics for comparing the distribution of baseline covariates between treatment groups in propensity-score matched samples, Stat Med, vol.28, pp.3083-107, 2009.

D. M. Wingerchuk, B. Banwell, J. L. Bennett, P. Cabre, W. Carroll et al., International consensus diagnostic criteria for neuromyelitis optica spectrum disorders, Neurology, vol.85, pp.177-89, 2015.

C. H. Polman, S. C. Reingold, B. Banwell, M. Clanet, J. A. Cohen et al., Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria, Ann Neurol, vol.69, pp.292-302, 2011.

L. B. Krupp, M. Tardieu, M. P. Amato, B. Banwell, T. Chitnis et al., International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions, Mult Scler, vol.19, pp.1261-1268, 2013.

R. Marignier, C. Calvo, A. Vukusic, and S. , Neuromyelitis optica and neuromyelitis optica spectrum disorders, Curr Opin Neurol, vol.30, pp.208-223, 2017.
URL : https://hal.archives-ouvertes.fr/hal-01798528

M. C. Papadopoulos, J. L. Bennett, and A. S. Verkman, Treatment of neuromyelitis optica: state-of-the-art and emerging therapies, Nat Rev Neurol, vol.10, pp.493-506, 2014.

Á. Cobo-calvo, A. Ruiz, D. 'indy, H. Poulat, A. Carneiro et al., MOGantibody-related disorders: common features and uncommon presentations, J Neurol, vol.264, pp.1945-55, 2017.

C. Costanzi, M. Matiello, C. F. Lucchinetti, B. G. Weinshenker, S. J. Pittock et al., Azathioprine: tolerability, efficacy, and predictors of benefit in neuromyelitis optica, Neurology, vol.77, pp.659-66, 2011.

S. Huh, S. Kim, J. Hyun, A. Joung, M. S. Park et al., Mycophenolate mofetil in the treatment of neuromyelitis optica spectrum disorder, JAMA Neurol, vol.71, p.1372, 2014.

A. Montcuquet, N. Collongues, C. Papeix, H. Zephir, B. Audoin et al., Effectiveness of mycophenolate mofetil as first-line therapy in AQP4-IgG, MOG-IgG, and seronegative neuromyelitis optica spectrum disorders, Mult Scler, vol.23, pp.1377-84, 2016.
URL : https://hal.archives-ouvertes.fr/hal-01798528

H. L. Pellkofer, M. Krumbholz, A. Berthele, B. Hemmer, L. A. Gerdes et al., Long-term follow-up of patients with neuromyelitis optica after repeated therapy with rituximab, Neurology, vol.76, pp.1310-1315, 2011.

H. Zéphir, R. Bernard-valnet, C. Lebrun, O. Outteryck, B. Audoin et al., Rituximab as first-line therapy in neuromyelitis optica: efficiency and tolerability, J Neurol, vol.262, pp.2329-2364, 2015.

N. Collongues, D. Brassat, E. Maillart, P. Labauge, J. C. Ouallet et al., Efficacy of rituximab in refractory neuromyelitis optica, Mult Scler, vol.22, pp.955-964, 2015.
URL : https://hal.archives-ouvertes.fr/hal-01889926

V. Damato, A. Evoli, and R. Iorio, Efficacy and safety of rituximab therapy in neuromyelitis optica spectrum disorders, JAMA Neurol, vol.73, p.1342, 2016.

M. Spadaro, L. A. Gerdes, M. Krumbholz, B. Ertl-wagner, F. S. Thaler et al., Autoantibodies to MOG in a distinct subgroup of adult multiple sclerosis, Neurol Neuroimmunol Neuroinflammation, vol.3, p.257, 2016.

J. Palace, M. I. Leite, A. Nairne, and A. Vincent, Interferon Beta treatment in neuromyelitis optica: increase in relapses and aquaporin 4 antibody titers, Arch Neurol, vol.67, pp.1016-1023, 2010.

K. C. Wang, K. H. Lin, T. C. Lee, C. L. Lee, S. Y. Chen et al., Poor responses to interferon-beta treatment in patients with neuromyelitis optica and multiple sclerosis with long spinal cord lesions, PLoS One, vol.9, p.98192, 2014.

J. Kitley, N. Evangelou, W. Küker, A. Jacob, M. I. Leite et al., Catastrophic brain relapse in seronegative NMO after a single dose of natalizumab, J Neurol Sci, vol.339, pp.223-228, 2014.

M. Trojano, F. Pellegrini, A. Fuiani, D. Paolicelli, and V. Zipoli, New natural history of interferon-?-treated relapsing multiple sclerosis, Ann Neurol, vol.61, pp.300-306, 2007.

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