Skip to main content
SpringerLink
Log in
Book cover

Neurorheumatology pp 187–195Cite as

Neuromyelitis Optica Spectrum Disorders

  • Chapter
  • First Online:

  • 965 Accesses

Abstract

Neuromyelitis optica (NMO) is an autoimmune disease that is characterized by antibody-mediated inflammation, demyelination, neuronal loss, and necrosis of the central nervous system (CNS) – most commonly of optic nerves, spinal cord, and brainstem. In 2006, Wingerchuk et al. proposed that the diagnosis of NMO requires a clinical episode of either optic neuritis (ON) or acute myelitis and at least two out of the three following supportive criteria for the diagnosis of NMO: continuous spinal cord lesion encompassing more than three vertebral segments on magnetic resonance imaging (MRI), brain MRI not fulfilling diagnostic criteria for multiple sclerosis (MS), and aquaporin-4 (AQP4) immunoglobulin G seropositivity. In 2015, the diagnostic criteria were once again revised to incorporate other characteristic findings of the disorder including an area postrema syndrome, tumefactive presentations, as well as brainstem, thalamic, and hypothalamic manifestations.

More recently, researchers have expanded the breadth of NMO spectrum disorders, and the identification of the aquaporin-4 (AQP4) antibody has also allowed for B-cell targeting treatments. In this chapter, we will discuss current knowledge about the epidemiology, pathogenesis, diagnosis, treatments, and important ongoing research.

This is a preview of subscription content, log in via an institution.

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   159.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  1. Devic E. Myélite aiguë compliquée de névrite optique. Bull Med (Paris). 1894;8:1033.

    Google Scholar 

  2. Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology. 1999;53:1107–14.

    Article  CAS  PubMed  Google Scholar 

  3. Lennon V, Wingerchuk D, Kryzer T, Pittock S, Lucchinetti C, Fujihara K, Nakashima I, Weinshenker B. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004;364:2106–12.

    Article  CAS  PubMed  Google Scholar 

  4. Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med. 2005;202(4):473–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Wingerchuk D, Lennon V, Pittock S, Lucchinetti C, Weinshenker B. Revised diagnostic criteria for neuromyelitis optica. Neurology. 2006;66:1485–9.

    Article  CAS  PubMed  Google Scholar 

  6. Wingerchuk DM, Banwell B, Bennett JL, Cabre P, Carroll W, Chitnis T, et al; International Panel for NMO Diagnosis. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177–89.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Krumbholz M, Meinl E. B cells in MS and NMO: pathogenesis and therapy. Semin Immunopathol. 2014;36(3):339–50.

    Article  CAS  PubMed  Google Scholar 

  8. Jarius S, Wildemann B. AQP4 antibodies in neuromyelitis optica: diagnostic and pathogenetic relevance. Nat Rev Neurol. 2010;6:383–92.

    Article  CAS  PubMed  Google Scholar 

  9. Waters P, Reindl M, Saiz A, Schanda K, Tuller F, Kral V, et al. Multicentre comparison of a diagnostic assay: aquaporin-4 antibodies in neuromyelitis optica. J Neurol Neurosurg Psychiatry. 2016;87:1005–15.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Jarius S, Franciotta D, Paul F, Ruprecht K, Bergamaschi R, Rommer PS, et al. Cerebrospinal fluid antibodies to aquaporin-4 in neuromyelitis optica and related disorders: frequency, origin, and diagnostic relevance. J Neuroinflammation. 2010;7:52.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  11. Nielsen S, Nagelhus EA, Amiry-Moghaddam M, Bourque C, Agre P, Ottersen OP. Specialized membrane domains for water transport in glial cells: high-resolution immunogold cytochemistry of aquaporin-4 in rat brain. J Neurosci. 1997;17:171–80.

    Article  CAS  PubMed  Google Scholar 

  12. Rash JE, Yasumura T, Hudson CS, Agre P, Nielsen S. Direct immunogold labeling of aquaporin-4 in square arrays of astrocyte and ependymocyte plasma membranes in rat brain and spinal cord. Proc Natl Acad Sci U S A. 1998;95(20):11981–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Kinoshita M, Nakatsuji Y, Kimura T, Moriya M, Takata K, Okuno T, et al. Neuromyelitis optica: passive transfer to rats by human immunoglobulin. Biochem Biophys Res Commun. 2009;386(4):623–7.

    Article  CAS  PubMed  Google Scholar 

  14. Saadoun S, Waters P, Owens GP, Bennett JL, Vincent A, Papadopoulos MC. Neuromyelitis optica MOG-IgG causes reversible lesions in mouse brain. Acta Neuropathol Commun. 2014;2:35.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Roemer SF, Parisi JE, Lennon VA, Benarroch EE, Lassmann H, Bruck W, et al. Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis. Brain. 2007;130(Pt 5):1194–205.

    Article  PubMed  Google Scholar 

  16. 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(7):1450–61.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Misu T, Fujihara K, Kakita A, Konno H, Nakamura M, Watanabe S, et al. Loss of aquaporin 4 in lesions of neuromyelitis optica: distinction from multiple sclerosis. Brain. 2007;130(5):1224–34.

    Article  CAS  PubMed  Google Scholar 

  18. Matiello M, Kim HJ, Kim W, Brum DG, Barreira AA, Kingsbury DJ, et al. Familial neuromyelitis optica. Neurology. 2010;75(4):310–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Wang H, Dai Y, Qiu W, Zhong X, Wu A, Wang Y, Lu Z, et al. HLA-DPB1 0501 is associated with susceptibility to anti-aquaporin-4 antibodies positive neuromyelitis optica in southern Han Chinese. J Neuroimmunol. 2011;233(1–2):181–4.

    Article  CAS  PubMed  Google Scholar 

  20. Kim HJ, Park HY, Kim E, Lee KS, Kim KK, Choi BO, et al. Common CYP7A1 promoter polymorphism associated with risk of neuromyelitis optica. Neurobiol Dis. 2010;37(2):349–55.

    Article  CAS  PubMed  Google Scholar 

  21. Matiello M, Schaefer-Klein JL, Hebrink DD, Kingsbury DJ, Atkinson EJ, Weinshenker BG; NMO Genetics Collaborators. Genetic analysis of aquaporin-4 in neuromyelitis optica. Neurology. 2011;77(12):1149–55.

    Article  CAS  PubMed  Google Scholar 

  22. Vaishnav RA, Liu R, Chapman J, Roberts AM, Ye H, Rebolledo-Mendez JD, et al. Aquaporin 4 molecular mimicry and implications for neuromyelitis optica. J Neuroimmunol. 2013;260(1–2):92–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. El-Otmani H, Rafai MA, Moutaouakil F, El-Moutawakkil B, Gam I, El Meziane A, et al. Devic’s optic neuromyelitis and pulmonary tuberculosis. Rev Mal Respir. 2005;22:143–6.

    Article  CAS  PubMed  Google Scholar 

  24. Hawley RJ, Madrid R. Post-infectious central and peripheral nervous system diseases in patient with Devic’s disease and Guillain-Barre syndrome. Eur J Neurol. 2003;10:600.

    Article  CAS  PubMed  Google Scholar 

  25. Al Deeb SM, Yaqub BA, Khoja WO. Devic’s neuromyelitis optica Kaiser R, Lucking CH. GFAP-specific oligoclonal bands in the CSF and varicella [letter]. J Neurol. 1993;240:450–1.

    Google Scholar 

  26. Wilcox RA, Burrow J, Slee M, Craig J, Thyagarajan D. Neuromyelitis optica (Devic’s disease) in a patient with syphilis. Mult Scler. 2008;14(2):268–71.

    Article  PubMed  Google Scholar 

  27. Blanche P, Diaz E, Gombert B, Sicard D. Devic’s neuromyelitis optica and HIV-1 infection. J Neurol Neurosurg Psychiatry. 2000;68(6):795–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Feyissa AM, Singh P, Smith RG. Neuromyelitis optica in patients with coexisting human immunodeficiency virus infections. Mult Scler. 2013;19(10):1363–6.

    Article  PubMed  Google Scholar 

  29. Graber D, Levy M, Kerr D. Neuromyelitis optica pathogenesis and aquaporin 4. J Neuroinflammation. 2008;5:22.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  30. Graves J, Grandhe S, Weinfurtner K, Krupp L, Belman A, Chitnis T, et al; US Network of Pediatric Multiple Sclerosis Centers. Protective environmental factors for neuromyelitis optica. Neurology. 2014;83(21):1923–9.

    Article  PubMed  PubMed Central  Google Scholar 

  31. Cabrera-Gómez JA, Kurtzke JF, González-Quevedo A, Lara-Rodríguez R. An epidemiological study of neuromyelitis optica in Cuba. J Neurol. 2009;256(1):35–44.

    Article  PubMed  Google Scholar 

  32. Asgari N, Lillevang ST, Skejoe HPB, Falah M, Stenager E, Kyvik KO. A population-based study of neuromyelitis optica in Caucasians. Neurology. 2011;76(18):1589–95.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. 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(5):775–83.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Wingerchuk DM. Neuromyelitis optica: effect of gender. J Neurol Sci. 2009 Nov 15;286(1–2):18–23.

    Article  CAS  PubMed  Google Scholar 

  35. Sellner J, Boggild M, Clanet M, Hintzen RQ, Illes Z, Montalban X, et al. EFNS guidelines on diagnosis and management of neuromyelitis optica. Eur J Neurol. 2010;17(8):1019–32.

    Article  CAS  PubMed  Google Scholar 

  36. 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.

    PubMed  PubMed Central  Google Scholar 

  37. Cabre P, Heinzlef O, Merle H, Buisson GG, Bera O, Bellance R, et al. MS and neuromyelitis optica in Martinique (French West Indies). Neurology. 2001;56(4):507–14.

    Article  CAS  PubMed  Google Scholar 

  38. Wu JS, Zhang MN, Carroll WM, Kermode AG. Characterisation of the spectrum of demyelinating disease in Western Australia. J Neurol Neurosurg Psychiatry. 2008;79:1022–6.

    Article  PubMed  Google Scholar 

  39. Kira J. Multiple sclerosis in the Japanese population. Lancet Neurol. 2003;2(2):117–27.

    Article  PubMed  Google Scholar 

  40. Jarius S, Ruprecht K, Wildemann B, Kuempfel T, Ringelstein M, Geis C, et al. Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: a multicentre study of 175 patients. J Neuroinflammation. 2012;9:14.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Jarius S, Ruprecht K, Kleiter I, Borisow N, Asgari N, Pitarokoili 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 2: epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome. J Neuroinflammation. 2016;13(1):280.

    Google Scholar 

  42. Juryńczyk M, Weinshenker B, Akman-Demir G, Asgari N, Barnes D, Boggild M, et al. Status of diagnostic approaches to AQP4-IgG seronegative NMO and NMO/MS overlap syndromes. J Neurol. 2016;263(1):140–9.

    Article  PubMed  CAS  Google Scholar 

  43. Pandit L, Asgari N, Apiwattanakul M, Palace J, Paul F, Leite MI, et al; the GJCF International Clinical Consortium & Biorepository for Neuromyelitis Optica. Demographic and clinical features of neuromyelitis optica: a review. Mult Scler. 2015;21(7):845–53.

    Google Scholar 

  44. Iorio R, Lucchinetti CF, Lennon VA, Costanzi C, Hinson S, Weinshenker BG, et al. Syndrome of inappropriate antidiuresis may herald or accompany neuromyelitis optica. Neurology. 2011;77(17):1644–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. 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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Weinshenker BG, Wingerchuk DM, Pittock SJ, Lucchinetti CF, Lennon VA. NMO-IgG: a specific biomarker for neuromyelitis optica. Dis Markers. 2006;22(4):197–206.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  47. Wingerchuk DM, Weinshenker BG. The emerging relationship between neuromyelitis optica and systemic rheumatologic autoimmune disease. Mult Scler. 2012;18:5–10.

    Article  CAS  PubMed  Google Scholar 

  48. 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;78:665–71.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Jarius S, Wildemann B. Aquaporin-4 antibodies (NMO-IgG) as a serological marker of neuromyelitis optica: a critical review of the literature. Brain Pathol. 2013;23(6):661–83.

    Article  CAS  PubMed  Google Scholar 

  50. Waters PJ, Pittock SJ, Bennett JL, Jarius S, Weinshenker BG, Wingerchuk DM. Evaluation of aquaporin-4 antibody assays. Clin Exp Neuroimmunol. 2014;5:290–303.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  51. Matiello M, Lennon VA, Jacob A, Pittock SJ, Lucchinetti CF, Wingerchuk DM, et al. NMO-IgG predicts the outcome of recurrent optic neuritis. Neurology. 2008;70:2197–200.

    Article  CAS  PubMed  Google Scholar 

  52. Weinshenker BG, Wingerchuk DM, Vukusic S, Linbo L, Pittock SJ, Lucchinetti CF, et al. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol. 2006;59(3):566–9.

    Article  CAS  PubMed  Google Scholar 

  53. Jarius S, Kleiter I, Ruprecht K, Asgari N, Pitarokoili K, Borisow N, et al. MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: brainstem involvement—frequency, presentation and outcome. In cooperation with the Neuromyelitis Optica Study Group (NEMOS). J Neuroinflammation. 2016;13(1):281.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  54. Dos Passos GR, Oliveira LM, da Costa BK, Apostolos-Pereira SL, Callegaro D, Fujihara K, et al. MOG-IgG-associated optic neuritis, encephalitis, and myelitis: lessons learned from neuromyelitis optica spectrum disorder. Front Neurol. 2018;9:217.

    Article  PubMed  PubMed Central  Google Scholar 

  55. Reindl M, Rostasy K. MOG antibody-associated diseases. Neurol Neuroimmunol Neuroinflamm. 2015;2(1):e60.

    Article  PubMed  PubMed Central  Google Scholar 

  56. Waters P, Woodhall M, O’Connor KC, Reindl M, Lang B, Sato DK, et al. MOG cell-based assay detects non-MS patients with inflammatory neurologic disease. Neurol Neuroimmunol Neuroinflamm. 2015;2(3):e89.

    Article  PubMed  PubMed Central  Google Scholar 

  57. Melamed E, Levy M, Waters PJ, Sato DK, Bennett JL, John GR, et al. Update on biomarkers in neuromyelitis optica. Neurol Neuroimmunol Neuroinflamm. 2015;2(4):e134.

    Article  PubMed  PubMed Central  Google Scholar 

  58. Misu T, Takano R, Fujihara K, Takahashi T, Sato S, Itoyama Y. Marked increase in cerebrospinal fluid glial fibrillar acidic protein in neuromyelitis optica: an astrocytic damage marker. J Neurol Neurosurg Psychiatry. 2009;80(5):575–7.

    Article  CAS  PubMed  Google Scholar 

  59. Uzawa A, Mori M, Arai K, Sato Y, Hayakawa S, Masuda S, et al. Cytokine and chemokine profiles in neuromyelitis optica: significance of interleukin-6. Mult Scler. 2010;16(12):1443–52.

    Article  CAS  PubMed  Google Scholar 

  60. Bergamaschi R, Tonietti S, Franciotta D, Candeloro E, Tavazzi E, Piccolo G, et al. Oligoclonal bands in Devic’s neuromyelitis optica and multiple sclerosis: differences in repeated cerebrospinal fluid examinations. Mult Scler. 2004;10(1):2–4.

    Article  CAS  PubMed  Google Scholar 

  61. Pittock SJ, Lucchinetti CF. Neuromyelitis optica and the evolving spectrum of autoimmune aquaporin-4 channelopathies: a decade later. Ann N Y Acad Sci. 2016;1366(1):20–39.

    Article  PubMed  Google Scholar 

  62. Nakamura M, Miyazawa I, Fujihara K, Nakashima I, Misu T, Watanabe S, et al. Preferential spinal central gray matter involvement in neuromyelitis optica. An MRI study. J Neurol. 2008;255(2):163–70.

    Article  CAS  PubMed  Google Scholar 

  63. Kim HJ, Paul F, Lana-Peixoto MA, Tenembaum S, Asgari N, Palace J, et al; Guthy-Jackson Charitable Foundation NMO International Clinical Consortium & Biorepository. MRI characteristics of neuromyelitis optica spectrum disorder: an international update. Neurology. 2015;84(11):1165–73.

    Google Scholar 

  64. Storoni M, Davagnanam I, Radon M, Siddiqui A, Plant GT. Distinguishing optic neuritis in neuromyelitis optica spectrum disease from multiple sclerosis: a novel magnetic resonance imaging scoring system. J Neuroophthalmol. 2013;33(2):123–7.

    Article  PubMed  Google Scholar 

  65. 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.

    Article  PubMed  Google Scholar 

  66. Pittock SJ, Lennon VA, Krecke K, Wingerchuk DM, Lucchinetti CF, Weinshenker BG. Brain abnormalities in neuromyelitis optica. Arch Neurol. 2006;63(3):390–6.

    Article  PubMed  Google Scholar 

  67. Barnett Y, Sutton IJ, Ghadiri M, Masters L, Zivadinov R, Barnett MH. Conventional and advanced imaging in neuromyelitis optica. AJNR Am J Neuroradiol. 2014;35(8):1458–66.

    Article  CAS  PubMed  Google Scholar 

  68. Vodopivec I, Matiello M, Prasad S. Treatment of neuromyelitis optica. Curr Opin Ophthalmol. 2015;26(6):476–83.

    Article  PubMed  Google Scholar 

  69. Weinshenker BG, O’Brien PC, Petterson TM, Noseworthy JH, Lucchinetti CF, Dodick DW, et al. A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol. 1999;46(6):878–86.

    Article  CAS  PubMed  Google Scholar 

  70. Bonnan M, Valentino R, Debeugny S, Merle H, Fergé JL, Mehdaoui H, 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):346–51.

    Article  PubMed  Google Scholar 

  71. Elsone L, Panicker J, Mutch K, Boggild M, Appleton R, Jacob A. Role of intravenous immunoglobulin in the treatment of acute relapses of neuromyelitis optica: experience in 10 patients. Mult Scler. 2014;20(4):501–4.

    Article  CAS  PubMed  Google Scholar 

  72. Costanzi C, Matiello M, Lucchinetti CF, Weinshenker BG, Pittock SJ, Mandrekar J, Thapa P, McKeon A. Azathioprine: tolerability, efficacy, and predictors of benefit in neuromyelitis optica. Neurology. 2011;77(7):659–66.

    Article  CAS  PubMed  Google Scholar 

  73. Jacob A, Matiello M, Weinshenker BG, Wingerchuk DM, Lucchinetti C, Shuster E, et al. Treatment of neuromyelitis optica with mycophenolate mofetil: retrospective analysis of 24 patients. Arch Neurol. 2009;66(9):1128–33.

    Article  PubMed  Google Scholar 

  74. Mealy MA, Wingerchuk DM, Palace J, Greenberg BM, Levy M. Comparison of relapse and treatment failure rates among patients with neuromyelitis optica: multicenter study of treatment efficacy. JAMA Neurol. 2014;71(3):324–30.

    Article  PubMed  Google Scholar 

  75. Berger JR, Malik V, Lacey S, Brunetta P, Lehane PB. Progressive multifocal leukoencephalopathy in rituximab-treated rheumatic diseases: a rare event. J Neurovirol. 2018;24(3):323–31.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  76. Kitley J, Elsone L, George J, Waters P, Woodhall M, Vincent A, et al. Methotrexate is an alternative to azathioprine in neuromyelitis optica spectrum disorders with aquaporin-4 antibodies. J Neurol Neurosurg Psychiatry. 2013;84(8):918–21.

    Article  PubMed  Google Scholar 

  77. Khatri BO, Kramer J, Dukic M, Palencia M, Verre W. Maintenance plasma exchange therapy for steroid-refractory neuromyelitis optica. J Clin Apher. 2012;27(4):183–92.

    Article  PubMed  Google Scholar 

  78. Pittock SJ, Lennon VA, McKeon A, Mandrekar J, Weinshenker BG, Lucchinetti CF, et al. Eculizumab in AQP4-IgG-positive relapsing neuromyelitis optica spectrum disorders: an open-label pilot study. Lancet Neurol. 2013;12(6):554–62.

    Article  CAS  PubMed  Google Scholar 

  79. Yamamura T, Araki M. Use of tocilizumab, an antibody against interleukin-6 receptor, for the treatment of neuromyelitis optica. Brain Nerve. 2014;66(10):1159–65.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Brigham and Women’s Hospital, and Massachusetts General Hospital, Boston, MA, USA

    Kristin M. Galetta

  2. Department of Neurology, Harvard Medical School, Boston, MA, USA

    Marcelo Matiello

  3. Massachusetts General Hospital Neurology Inpatient Services, Boston, MA, USA

    Marcelo Matiello

  4. Massachusetts General Hospital Routine and Urgent Teleneurology Program, Boston, MA, USA

    Marcelo Matiello

  5. Department of Neurology, Neurology Associates, Boston, MA, USA

    Marcelo Matiello

Authors
  1. Kristin M. Galetta
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Marcelo Matiello
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Kristin M. Galetta .

Editor information

Editors and Affiliations

  1. Division of Neuroimmunology, University of Iowa, Iowa City, IA, USA

    Tracey A. Cho

  2. Department of Neurology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Shamik Bhattacharyya

  3. Division of Rheumatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Simon Helfgott

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Galetta, K.M., Matiello, M. (2019). Neuromyelitis Optica Spectrum Disorders. In: Cho, T., Bhattacharyya, S., Helfgott, S. (eds) Neurorheumatology. Springer, Cham. https://doi.org/10.1007/978-3-030-16928-2_19

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-16928-2_19

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-16927-5

  • Online ISBN: 978-3-030-16928-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation