Update on diagnosis and treatment of neuromyelitis optica spectrum disorders (NMOSD) — recommendations of Section of Multiple Sclerosis and Neuroimmunology of Polish Neurological Society
Abstract
Introduction. An expert panel of the Section of Multiple Sclerosis and Neuroimmunology of the Polish Neurological Society has developed principles for the management of neuromyelitis optica spectrum disorders (NMOSD). These principles are based on expert opinion and data from the literature published up to May 2023. Recommendations were developed based on the results of the most recent clinical trials, guidelines of foreign and international scientific societies, and the authors’ clinical experience.
Clinical implications. The principles for diagnosing NMOSD are discussed, with particular emphasis on serological and neuroimaging diagnosis. Recommendations for the treatment of relapses and chronic immunosuppressive treatment, including the most recent methods of immunotherapy, are also presented. Additionally, the principles of monitoring treatment efficacy and safety are included. Therapy regimens are completed with recommendations for symptomatic treatment. The paper also includes an algorithm for vaccination in patients with NMOSD. Therapeutic management in pregnant women with NMOSD is discussed.
Keywords: recommendationsdiagnosis and treatmentneuromyelitis optica spectrum disordersNMOSD
References
- Devic E. Myelite aigue dorso-lombaire avec nevrite optique. Autopsie. Congres francais de Medecine. ; 1895: 434–439.
- Fujihara K. Neuromyelitis optica spectrum disorders: still evolving and broadening. Curr Opin Neurol. 2019; 32(3): 385–394.
- Jarius S, Wildemann B. An early case of neuromyelitis optica: on a forgotten report by Jacob Lockhart Clarke, FRS. Mult Scler. 2011; 17(11): 1384–1386.
- Lucchinetti CF, Guo Y, Popescu BF, et al. The pathology of an autoimmune astrocytopathy: lessons learned from neuromyelitis optica. Brain Pathol. 2014; 24(1): 83–97.
- Wingerchuk DM, Lennon VA, Lucchinetti CF, et al. The spectrum of neuromyelitis optica. Lancet Neurol. 2007; 6(9): 805–815.
- Wingerchuk DM, Hogancamp WF, O'Brien PC, et al. The clinical course of neuromyelitis optica (Devic's syndrome). Neurology. 1999; 53(5): 1107–1114.
- Graber DJ, Levy M, Kerr D, et al. Neuromyelitis optica pathogenesis and aquaporin 4. J Neuroinflammation. 2008; 5: 22.
- Jarius S, Aktas O, Ayzenberg I, et al. Neuromyelitis Optica Study Group (NEMOS). Update on the diagnosis and treatment of neuromyelits optica spectrum disorders (NMOSD) - revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part I: Diagnosis and differential diagnosis. J Neurol. 2023; 270(7): 3341–3368.
- Lennon VA, Wingerchuk DM, Kryzer TJ, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004; 364(9451): 2106–2112.
- Wingerchuk DM, Banwell B, Bennett JL, et al. International Panel for NMO Diagnosis. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015; 85(2): 177–189.
- Mader S, Gredler V, Schanda K, et al. Complement activating antibodies to myelin oligodendrocyte glycoprotein in neuromyelitis optica and related disorders. J Neuroinflammation. 2011; 8: 184.
- Ramanathan S, Reddel SW, Henderson A, et al. Antibodies to myelin oligodendrocyte glycoprotein in bilateral and recurrent optic neuritis. Neurol Neuroimmunol Neuroinflamm. 2014; 1(4): e40.
- Jarius S, Ruprecht K, Kleiter I, et al. in cooperation with the Neuromyelitis Optica Study Group (NEMOS). 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. 2016; 13(1): 280.
- Waters P, Woodhall M, O'Connor KC, et al. MOG cell-based assay detects non-MS patients with inflammatory neurologic disease. Neurol Neuroimmunol Neuroinflamm. 2015; 2(3): e89.
- Sato DK, Callegaro D, Lana-Peixoto MA, et al. Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders. Neurology. 2014; 82(6): 474–481.
- Kitley J, Woodhall M, Waters P, et al. Myelin-oligodendrocyte glycoprotein antibodies in adults with a neuromyelitis optica phenotype. Neurology. 2012; 79(12): 1273–1277.
- Jarius S, Kleiter I, Ruprecht K, et al. in cooperation with the Neuromyelitis Optica Study Group (NEMOS). MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: Brainstem involvement - frequency, presentation and outcome. J Neuroinflammation. 2016; 13(1): 281.
- Marignier R, Hacohen Y, Cobo-Calvo A, et al. Myelin-oligodendrocyte glycoprotein antibody-associated disease. Lancet Neurol. 2021; 20(9): 762–772.
- Ciotti JR, Eby NS, Wu GF, et al. Clinical and laboratory features distinguishing MOG antibody disease from multiple sclerosis and AQP4 antibody-positive neuromyelitis optica. Mult Scler Relat Disord. 2020; 45: 102399.
- Hor JY, Asgari N, Nakashima I, et al. Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide. Front Neurol. 2020; 11: 501.
- Jarius S, Paul F, Aktas O, et al. MOG encephalomyelitis: international recommendations on diagnosis and antibody testing. J Neuroinflammation. 2018; 15(1): 134.
- Banwell B, Bennett JL, Marignier R, et al. Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria. Lancet Neurol. 2023; 22(3): 268–282.
- Carnero Contentti E, Okuda DT, Rojas JI, et al. MRI to differentiate multiple sclerosis, neuromyelitis optica, and myelin oligodendrocyte glycoprotein antibody disease. J Neuroimaging. 2023; 33(5): 688–702.
- Selmaj K, Selmaj I. Novel emerging treatments for NMOSD. Neurol Neurochir Pol. 2019; 53(5): 317–326.
- Ayzenberg I, Kleiter I, Schröder A, et al. Interleukin 6 receptor blockade in patients with neuromyelitis optica nonresponsive to anti-CD20 therapy. JAMA Neurol. 2013; 70(3): 394–397.
- Kleiter I, Traboulsee A, Palace J, et al. Trial of Satralizumab in Neuromyelitis Optica Spectrum Disorder. N Engl J Med. 2019; 381(22): 2114–2124.
- Ringelstein M, Ayzenberg I, Harmel J, et al. Long-term Therapy With Interleukin 6 Receptor Blockade in Highly Active Neuromyelitis Optica Spectrum Disorder. JAMA Neurol. 2015; 72(7): 756–763.
- Zhang C, Zhang M, Qiu W, et al. TANGO Study Investigators. Safety and efficacy of tocilizumab versus azathioprine in highly relapsing neuromyelitis optica spectrum disorder (TANGO): an open-label, multicentre, randomised, phase 2 trial. Lancet Neurol. 2020; 19(5): 391–401.
- Borisow N, Mori M, Kuwabara S, et al. Diagnosis and Treatment of NMO Spectrum Disorder and MOG-Encephalomyelitis. Front Neurol. 2018; 9: 888.
- Abboud H, Salazar-Camelo A, George N, et al. On-behalf of the Guthy-Jackson Foundation NMO International Clinical Consortium. Symptomatic and restorative therapies in neuromyelitis optica spectrum disorders. J Neurol. 2022; 269(4): 1786–1801.
- Jarius S, Paul F, Weinshenker BG, et al. Neuromyelitis optica. Nat Rev Dis Primers. 2020; 6(1): 85.
- Tugizova M, Vlahovic L, Tomczak A, et al. New Therapeutic Landscape in Neuromyelitis Optica. Curr Treat Options Neurol. 2021; 23(4): 13.
- Costello F. Neuromyelitis Optica Spectrum Disorders. Continuum (Minneap Minn). 2022; 28(4): 1131–1170.
- Carnero Contentti E, Rojas JI, Cristiano E, et al. Latin American consensus recommendations for management and treatment of neuromyelitis optica spectrum disorders in clinical practice. Mult Scler Relat Disord. 2020; 45: 102428.
- Sellner J, Boggild M, Clanet M, et al. EFNS guidelines on diagnosis and management of neuromyelitis optica. Eur J Neurol. 2010; 17(8): 1019–1032.
- Juryńczyk M, Weinshenker B, Akman-Demir G, et al. Status of diagnostic approaches to AQP4-IgG seronegative NMO and NMO/MS overlap syndromes. J Neurol. 2016; 263(1): 140–149.
- Wingerchuk DM, Weinshenker BG. Neuromyelitis optica: clinical predictors of a relapsing course and survival. Neurology. 2003; 60(5): 848–853.
- Kim SM, Kim SJ, Lee HJ, et al. Differential diagnosis of neuromyelitis optica spectrum disorders. Ther Adv Neurol Disord. 2017; 10(7): 265–289.
- Jasiak-Zatońska M, Michalak S, Kozubski W, et al. Diagnostic pitfalls in neuromyelitis optica spectrum disorders. Aktualności Neurologiczne. 2021; 21(3): 143–154.
- Waters PJ, McKeon A, Leite MI, et al. Serologic diagnosis of NMO: a multicenter comparison of aquaporin-4-IgG assays. Neurology. 2012; 78(9): 665–71; discussion 669.
- Waters P, Reindl M, Saiz A, et al. Multicentre comparison of a diagnostic assay: aquaporin-4 antibodies in neuromyelitis optica. J Neurol Neurosurg Psychiatry. 2016; 87(9): 1005–1015.
- Melamed E, Levy M, Waters PJ, et al. Update on biomarkers in neuromyelitis optica. Neurol Neuroimmunol Neuroinflamm. 2015; 2(4): e134.
- Jarius S, Franciotta D, Bergamaschi R, et al. NMO-IgG in the diagnosis of neuromyelitis optica. Neurology. 2007; 68(13): 1076–1077.
- Scott TF, Kassab SL, Pittock SJ. Neuromyelitis optica IgG status in acute partial transverse myelitis. Arch Neurol. 2006; 63(10): 1398–1400.
- Sąsiadek M, Hartel M, Siger M, et al. Recommendations of the Polish Medical Society of Radiology and the Polish Society of Neurology for a protocol concerning routinely used magnetic resonance imaging in patients with multiple sclerosis. Neurol Neurochir Pol. 2020; 54(5): 410–415.
- Flanagan EP, Weinshenker BG, Krecke KN, et al. Short myelitis lesions in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorders. JAMA Neurol. 2015; 72(1): 81–87.
- Kim W, Park MSu, Lee SH, et al. Characteristic brain magnetic resonance imaging abnormalities in central nervous system aquaporin-4 autoimmunity. Mult Scler. 2010; 16(10): 1229–1236.
- Huh SY, Min JH, Kim W, et al. The usefulness of brain MRI at onset in the differentiation of multiple sclerosis and seropositive neuromyelitis optica spectrum disorders. Mult Scler. 2014; 20(6): 695–704.
- Tillema JM, Pirko I. Neuroradiological evaluation of demyelinating disease. Ther Adv Neurol Disord. 2013; 6(4): 249–268.
- Solomon JM, Paul F, Chien C, et al. A window into the future? MRI for evaluation of neuromyelitis optica spectrum disorder throughout the disease course. Ther Adv Neurol Disord. 2021; 14: 17562864211014389.
- Bonnan M, Valentino R, Debeugny S, et al. Short delay to initiate plasma exchange is the strongest predictor of outcome in severe attacks of NMO spectrum disorders. J Neurol Neurosurg Psychiatry. 2018; 89(4): 346–351.
- Jiao Y, Cui L, Zhang W, et al. Plasma Exchange for Neuromyelitis Optica Spectrum Disorders in Chinese Patients and Factors Predictive of Short-term Outcome. Clin Ther. 2018; 40(4): 603–612.
- Reeves HM, Winters JL. The mechanisms of action of plasma exchange. Br J Haematol. 2014; 164(3): 342–351.
- Keegan M, Pineda AA, McClelland RL, et al. Plasma exchange for severe attacks of CNS demyelination: predictors of response. Neurology. 2002; 58(1): 143–146.
- Elsone L, Panicker J, Mutch K, et al. Role of intravenous immunoglobulin in the treatment of acute relapses of neuromyelitis optica: experience in 10 patients. Mult Scler. 2014; 20(4): 501–504.
- Galati A, Brown ES, Bove R, et al. Glucocorticoids for therapeutic immunosuppression: Clinical pearls for the practicing neurologist. J Neurol Sci. 2021; 430: 120004.
- Costanzi C, Matiello M, Lucchinetti CF, et al. Azathioprine: tolerability, efficacy, and predictors of benefit in neuromyelitis optica. Neurology. 2011; 77(7): 659–666.
- Wei K, Nie Q, Zhu Y, et al. Different doses of Rituximab for the therapy of Neuromyelitis optica spectrum disorder: A systematic review and meta-analysis. Mult Scler Relat Disord. 2022; 68: 104127.
- Jacob A, Matiello M, Weinshenker BG, et al. Treatment of neuro- myelitis optica with mycophenolate mofetil: retrospective analysis of 24 patients. Arch Neurol. 2009; 66(9): 1128–1133, doi: 10.1001/ archneurol.2009. 175, indexed in Pubmed. ; 19752302.
- Montcuquet A, Collongues N, Papeix C, et al. NOMADMUS study group and the Observatoire Français de la Sclérose en Plaques (OFSEP). Effectiveness of mycophenolate mofetil as first-line therapy in AQP4-IgG, MOG-IgG, and seronegative neuromyelitis optica spectrum disorders. Mult Scler. 2017; 23(10): 1377–1384.
- Zakrzewska-Pniewska B, Adamczyk-Sowa M, Brola W, et al. Leczenie i postępowanie objawowe w stwardnieniu rozsianym Rekomendacje Sekcji SM i Neuroimmunologii Polskiego Towarzystwa Neurologicznego. Polski Przegląd Neurologiczny. 2020; 15(4): 191–217.
- Bove RM, Houtchens MK. Pregnancy Management in Multiple Sclerosis and Other Demyelinating Diseases. Continuum (Minneap Minn). 2022; 28(1): 12–33.
- Nour MM, Nakashima I, Coutinho E, et al. Pregnancy outcomes in aquaporin-4-positive neuromyelitis optica spectrum disorder. Neurology. 2016; 86(1): 79–87.
- D'Souza R, Wuebbolt D, Andrejevic K, et al. Pregnancy and Neuromyelitis Optica Spectrum Disorder - Reciprocal Effects and Practical Recommendations: A Systematic Review. Front Neurol. 2020; 11: 544434.
- Kim W, Kim SH, Nakashima I, et al. Influence of pregnancy on neuromyelitis optica spectrum disorder. Neurology. 2012; 78(16): 1264–1267.
- Anderson A, Singh J, Bove R. Neuroimaging and radiation exposure in pregnancy. Handb Clin Neurol. 2020; 171: 179–191.
- Gur C, Diav-Citrin O, Shechtman S, et al. Pregnancy outcome after first trimester exposure to corticosteroids: a prospective controlled study. Reprod Toxicol. 2004; 18(1): 93–101.
- Padmanabhan A, Connelly-Smith L, Aqui N, et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice - Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019; 34(3): 171–354.
- Wind M, Gaasbeek AGA, Oosten LEM, et al. Therapeutic plasma exchange in pregnancy: A literature review. Eur J Obstet Gynecol Reprod Biol. 2021; 260: 29–36.
- Wallach AI, Tremblay M, Kister I. Advances in the Treatment of Neuromyelitis Optica Spectrum Disorder. Neurol Clin. 2021; 39(1): 35–49.
- Mao-Draayer Y, Thiel S, Mills EA, et al. Neuromyelitis optica spectrum disorders and pregnancy: therapeutic considerations. Nat Rev Neurol. 2020; 16(3): 154–170.
- Winkelmann A, Loebermann M, Barnett M, et al. Vaccination and immunotherapies in neuroimmunological diseases. Nat Rev Neurol. 2022; 18(5): 289–306.
- Bühler S, Eperon G, Ribi C, et al. Vaccination recommendations for adult patients with autoimmune inflammatory rheumatic diseases. Swiss Med Wkly. 2015; 145: w14159.
- Guptill JT, Juel VC, Massey JM, et al. Effect of therapeutic plasma exchange on immunoglobulins in myasthenia gravis. Autoimmunity. 2016; 49(7): 472–479.
- Furer V, Rondaan C, Heijstek M, et al. 2019 update of EULAR recommendations for vaccination in adult patients with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis. 2020; 79(1): 39–52.
- Huber F, Ehrensperger B, Hatz C, et al. Safety of live vaccines on immunosuppressive or immunomodulatory therapy-a retrospective study in three Swiss Travel Clinics. J Travel Med. 2018; 25(1).