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Optic Neuritis as the Presenting Feature of Neuromyelitis Optica (NMO): Diagnosis and Management

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Abstract

Since its description in 1894 by Eugene Devic, many had considered neuromyelitis optica (NMO) to be a “variant” of multiple sclerosis (MS). Clinical and MRI differences between MS and NMO along with the landmark discovery of highly specific anti-aquaporin 4 (AQP4) antibodies in 2004 indicated that NMO is a distinct disease marked by severe demyelination of the central nervous system (CNS) with particular predilection for the optic nerves, spinal cord, and area postrema in the medulla. Over the last 13 years, there has been a plethora of research and clinical interest in NMO facilitating improved awareness among physicians. Despite this, controversies on how to best diagnosis and treat NMO persist. This chapter will discuss the current evidence-based understanding of optic neuritis as the presenting feature of NMO while highlighting a few actively debated differences in its clinical care throughout the world.

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References

  1. Etemadifar M, Nasr Z, Khalili B, Taherioun M, Vosoughi R. Epidemiology of neuromyelitis optica in the world: a systematic review and meta-analysis. Mult Scler Int. 2015;2015:174720. https://doi.org/10.1155/2015/174720.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Weinshenker BG, Wingerchuk DM. Neuromyelitis spectrum disorders. Mayo Clin Proc. 2017;92(4):663–79. https://doi.org/10.1016/j.mayocp.2016.12.014.

    Article  PubMed  Google Scholar 

  3. Flanagan EP, Cabre P, Weinshenker BG, St Sauver J, Jacobson DJ, Majed M, et al. Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum. Ann Neurol. 2016;79:775–83. https://doi.org/10.1002/ana.24617.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Wingerchuk DM, Banwell B, Bennett JL, Cabre P, Carroll W, Chitnis T, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177–89. https://doi.org/10.1212/WNL.0000000000001729.

    Article  PubMed  PubMed Central  Google Scholar 

  5. The clinical profile of optic neuritis. Experience of the Optic Neuritis Treatment Trial. Optic Neuritis Study Group. Arch Ophthalmol. 1991;109(12):1673–8.

    Google Scholar 

  6. Visual function 5 years after optic neuritis: experience of the Optic Neuritis Treatment Trial. The Optic Neuritis Study Group. Arch Ophthalmol. 1997;115(12):1545–52.

    Google Scholar 

  7. Beck RW, Cleary PA, Backlund JC. The course of visual recovery after optic neuritis. Experience of the Optic Neuritis Treatment Trial. Ophthalmology. 1994;101(11):1771–8.

    Article  CAS  Google Scholar 

  8. Merle H, Olindo S, Bonnan M, Donnio A, Richer R, Smadja D, et al. Natural history of the visual impairment of relapsing neuromyelitis optica. Ophthalmology. 2007;114(4):810–5. https://doi.org/10.1016/j.ophtha.2006.06.060.

    Article  PubMed  Google Scholar 

  9. Akaishi T, Nakashima I, Sato DK, Takahashi T, Fujihara K. Neuromyelitis optica spectrum disorders. Neuroimaging Clin N Am. 2017;27(2):251–65. https://doi.org/10.1016/j.nic.2016.12.010.

    Article  PubMed  Google Scholar 

  10. Khanna S, Sharma A, Huecker J, Gordon M, Naismith RT, Van Stavern GP. Magnetic resonance imaging of optic neuritis in patients with neuromyelitis optica versus multiple sclerosis. J Neuroophthalmol. 2012;32(3):216–20. https://doi.org/10.1097/WNO.0b013e318254c62d.

    Article  PubMed  Google Scholar 

  11. Huh SY, Min JH, Kim W, Kim SH, Kim HJ, Kim BJ, 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. https://doi.org/10.1177/1352458513506953.

    Article  PubMed  Google Scholar 

  12. Pittock SJ, Lennon VA, Krecke K, Wingerchuk DM, Lucchinetti CF, Weinshenker BG. Brain abnormalities in neuromyelitis optica. Arch Neurol. 2006;63(3):390–6. https://doi.org/10.1001/archneur.63.3.390.

    Article  PubMed  Google Scholar 

  13. Morrow MJ, Wingerchuk D. Neuromyelitis optica. J Neuroophthalmol. 2012;32(2):154–66. https://doi.org/10.1097/WNO.0b013e31825662f1.

    Article  PubMed  Google Scholar 

  14. Naismith RT, Tutlam NT, Xu J, Klawiter EC, Shepherd J, Trinkaus K, et al. Optical coherence tomography differs in neuromyelitis optica compared with multiple sclerosis. Neurology. 2009;72(12):1077–82. https://doi.org/10.1212/01.wnl.0000345042.53843.d5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Waters PJ, McKeon A, Leite MI, Rajasekharan S, Lennon VA, Villalobos A, et al. Serologic diagnosis of NMO: a multicenter comparison of aquaporin-4-IgG assays. Neurology. 2012;78(9):665–71; discussion 9. https://doi.org/10.1212/WNL.0b013e318248dec1.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Levin MH. Advances in neuromyelitis optica: take them to the clinic. J Neuroophthalmol. 2017;37(3):300–2. https://doi.org/10.1097/WNO.0000000000000509.

    Article  PubMed  Google Scholar 

  17. Peschl P, Bradl M, Hoftberger R, Berger T, Reindl M. Myelin oligodendrocyte glycoprotein: deciphering a target in inflammatory demyelinating diseases. Front Immunol. 2017;8:529. https://doi.org/10.3389/fimmu.2017.00529.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Ramanthan S, Dale RC, Brilot F. Anti-MOG antibody: the history, clinical phenotype, and pathogenicity of a serum biomarker for demyelination. Autoimmun Rev. 2016;15:307–24.

    Article  Google Scholar 

  19. Weber MS, Derfuss T, Metz I, Bruck W. Defining distinct features of anti-MOG antibody associated central nervous system demyelination. Ther Adv Neurol Disord. 2018;11:1–15.

    Article  Google Scholar 

  20. Fan S, Xu Y, Ren H, Guan H, Feng F, Gao X, Ding D, Fang F, Shan G, Zhang Y, Dai Y, Yao M, Peng B, Zhu Y, Cui L. Comparison of myelin oligodendrocyte glycoprotein (MOG)-antibody disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) when they co-exist with anti-NMDA (N-methyl-d-aspartate) receptor encephalitis. Mult Scler Relat Dis. 2018;20:144–52.

    Article  Google Scholar 

  21. Kim SM, Kim SJ, Lee HJ, Kuroda H, Palace J, Fujihara K. Differential diagnosis of neuromyelitis optica spectrum disorders. Ther Adv Neurol Disord. 2017;10(7):265–89. https://doi.org/10.1177/1756285617709723.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. McClelland CM, Van Stavern GP, Tselis AC. Leber hereditary optic neuropathy mimicking neuromyelitis optica. J Neuroophthalmol. 2011;31(3):265–8. https://doi.org/10.1097/WNO.0b013e318225247b.

    Article  PubMed  Google Scholar 

  23. Jarius S, Wildemann B. The history of neuromyelitis optica. J Neuroinflammation. 2013;10:8. https://doi.org/10.1186/1742-2094-10-8.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Lennon VA, Wingerchuk DM, Kryzer TJ, Pittock SJ, Lucchinetti CF, Fujihara K, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004;364(9451):2106–12. https://doi.org/10.1016/S0140-6736(04)17551-X.

    Article  CAS  PubMed  Google Scholar 

  25. Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for neuromyelitis optica. Neurology. 2006;66(10):1485–9. https://doi.org/10.1212/01.wnl.0000216139.44259.74.

    Article  CAS  PubMed  Google Scholar 

  26. Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol. 2007;6(9):805–15. https://doi.org/10.1016/S1474-4422(07)70216-8.

    Article  CAS  PubMed  Google Scholar 

  27. Sato DK, Callegaro D, Lana-Peixoto MA, Waters PJ, de Haidar Jorge FM, Takahashi T, et al. Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders. Neurology. 2014;82(6):474–81. https://doi.org/10.1212/WNL.0000000000000101.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Lucchinetti CF, Mandler RN, McGavern D, Bruck W, Gleich G, Ransohoff RM, et al. A role for humoral mechanisms in the pathogenesis of Devic’s neuromyelitis optica. Brain. 2002;125(Pt 7):1450–61.

    Article  Google Scholar 

  29. Abboud H, Petrak A, Mealy M, Sasidharan S, Siddique L, Levy M. Treatment of acute relapses in neuromyelitis optica: steroids alone versus steroids plus plasma exchange. Mult Scler. 2016;22(2):185–92. https://doi.org/10.1177/1352458515581438.

    Article  CAS  PubMed  Google Scholar 

  30. Pula JH, Glisson CC. Should plasma exchange be offered to patients with multiple sclerosis-associated optic neuritis? J Neuroophthalmol. 2015;35(1):86–9. https://doi.org/10.1097/WNO.0000000000000197.

    Article  PubMed  Google Scholar 

  31. Ayzenberg I, Schollhammer J, Hoepner R, Hellwig K, Ringelstein M, Aktas O, et al. Efficacy of glatiramer acetate in neuromyelitis optica spectrum disorder: a multicenter retrospective study. J Neurol. 2016;263(3):575–82. https://doi.org/10.1007/s00415-015-7991-1.

    Article  CAS  PubMed  Google Scholar 

  32. Gahlen A, Trampe AK, Haupeltshofer S, Ringelstein M, Aktas O, Berthele A, et al. Aquaporin-4 antibodies in patients treated with natalizumab for suspected MS. Neurol Neuroimmunol Neuroinflamm. 2017;4(4):e363. https://doi.org/10.1212/NXI.0000000000000363.

    Article  PubMed  PubMed Central  Google Scholar 

  33. Gelfand JM, Cotter J, Klingman J, Huang EJ, Cree BA. Massive CNS monocytic infiltration at autopsy in an alemtuzumab-treated patient with NMO. Neurol Neuroimmunol Neuroinflamm. 2014;1(3):e34. https://doi.org/10.1212/NXI.0000000000000034.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Kleiter I, Hellwig K, Berthele A, Kumpfel T, Linker RA, Hartung HP, et al. Failure of natalizumab to prevent relapses in neuromyelitis optica. Arch Neurol. 2012;69(2):239–45. https://doi.org/10.1001/archneurol.2011.216.

    Article  PubMed  Google Scholar 

  35. Min JH, Kim BJ, Lee KH. Development of extensive brain lesions following fingolimod (FTY720) treatment in a patient with neuromyelitis optica spectrum disorder. Mult Scler. 2012;18(1):113–5. https://doi.org/10.1177/1352458511431973.

    Article  CAS  PubMed  Google Scholar 

  36. Palace J, Leite MI, Nairne A, Vincent A. Interferon Beta treatment in neuromyelitis optica: increase in relapses and aquaporin 4 antibody titers. Arch Neurol. 2010;67(8):1016–7. https://doi.org/10.1001/archneurol.2010.188.

    Article  PubMed  Google Scholar 

  37. Kleiter I, Gahlen A, Borisow N, Fischer K, Wernecke KD, Wegner B, et al. Neuromyelitis optica: Evaluation of 871 attacks and 1,153 treatment courses. Ann Neurol. 2016;79(2):206–16. https://doi.org/10.1002/ana.24554.

    Article  CAS  PubMed  Google Scholar 

  38. Stellmann JP, Krumbholz M, Friede T, Gahlen A, Borisow N, Fischer K, et al. Immunotherapies in neuromyelitis optica spectrum disorder: efficacy and predictors of response. J Neurol Neurosurg Psychiatry. 2017;88(8):639–47. https://doi.org/10.1136/jnnp-2017-315603.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Xu Y, Wang Q, Ren HT, Qiao L, Zhang Y, Fei YY, et al. Comparison of efficacy and tolerability of azathioprine, mycophenolate mofetil, and cyclophosphamide among patients with neuromyelitis optica spectrum disorder: a prospective cohort study. J Neurol Sci. 2016;370:224–8. https://doi.org/10.1016/j.jns.2016.09.035.

    Article  CAS  PubMed  Google Scholar 

  40. Bennett JL, Owens GP. Neuromyelitis optica: deciphering a complex immune-mediated astrocytopathy. J Neuroophthalmol. 2017;37(3):291–9. https://doi.org/10.1097/WNO.0000000000000508.

    Article  PubMed  PubMed Central  Google Scholar 

  41. Ohkubo A, Okado T. Selective plasma exchange. Transfus Apher Sci. 2017;56(5):657–60. https://doi.org/10.1016/j.transci.2017.08.010.

    Article  PubMed  Google Scholar 

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Acknowledgments

The authors would like to thank Dr. Claire Sheldon for contributing materials helpful to the creation of this chapter.

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Correspondence to Mark S. Gans .

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McClelland, C.M., Lee, M.S., Gans, M.S. (2019). Optic Neuritis as the Presenting Feature of Neuromyelitis Optica (NMO): Diagnosis and Management. In: Lee, A., Sinclair, A., Sadaka, A., Berry, S., Mollan, S. (eds) Neuro-Ophthalmology. Springer, Cham. https://doi.org/10.1007/978-3-319-98455-1_2

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  • DOI: https://doi.org/10.1007/978-3-319-98455-1_2

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