Skip to main content

Advertisement

SpringerLink
Log in

Neuromyelitis Optica

  • Neuroimmunology
  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Opinion statement

Neuromyelitis optica (NMO) or Devic’s disease typically involves the optic nerves and the spinal cord and is most often relapsing. The pathogenesis is one of an acute inflammatory process targeting astrocytes and resulting in demyelination, as well as axonal injury. In a high proportion of recognized cases of NMO, there is a highly specific autoantibody (NMO-IgG), which is directed to the common central nervous system water channel, aquaporin-4. NMO attacks usually result in severe residual visual impairment or myelopathy. Despite the publication of new diagnostic criteria for NMO, uncertainty at the time of the index event as to whether the attack is due to multiple sclerosis or NMO can cause therapeutic hesitancy. Nevertheless, whenever a reasonable degree of suspicion exists, therapies directed to limiting acute injury and to preventing subsequent further injury mediated by humoral mechanisms should be instituted immediately. Investigations can then be completed and the therapeutic direction confirmed.

For an acute attack, high-dose methylprednisolone and plasma exchange (generally given sequentially) are most useful.

For the prevention of further attacks, selective or nonselective immunosuppressive therapy directed to humoral mechanisms is preferred. Agents recommended are oral azathioprine or mycophenolate mofetil with or without low-dose prednisolone or rituximab. Therapy should be planned to continue for up to 5 years in all patients, including those with a single attack who are at high risk of further relapse.

Regrettably, there are no controlled trials for the treatment of either the classic manifestations of NMO or the so-called limited manifestations known as NMO spectrum disorders. The therapeutic opinions expressed in this article are therefore based on the current understanding of the pathogenesis of this disorder, reports of small series of patients receiving a range of treatments, and expert opinions.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Aoyama T: A case of myelitis and blindness. J Tokyo Med Assoc 1891, 5:827–830.

    Google Scholar 

  2. Allbutt TC: On the ophthalmoscopic signs of spinal disease. Lancet 1870, 1:76–78.

    Google Scholar 

  3. Dévic E: Myélite aiguë dorso-lombaire avec névrite optique. Autopsie. Congrès Français de Médecine (Lyon) 1894, 1:434–439.

    Google Scholar 

  4. Gault F: De la neuromyélite optique aiguë [thesis]. Lyon, 1894.

  5. Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG: The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology 1999, 53:1107–1114.

    CAS  PubMed  Google Scholar 

  6. Lotze TE, Northrop JL, Hutton GJ, et al.: Spectrum of pediatric neuromyelitis optica. Pediatrics 2008, 122:e1039–e1047.

    Article  PubMed  Google Scholar 

  7. Pittock SJ, Lennon VA, Krecke K, et al.: Brain abnormalities in neuromyelitis optica. Arch Neurol 2006, 63:390–396.

    Article  PubMed  Google Scholar 

  8. Matsushita T, Isobe N, Piao H, et al.: Reappraisal of brain MRI features in patients with multiple sclerosis and neuromyelitis optica according to anti-aquaporin-4 antibody status. J Neurol Sci 2010 Jan 30 (Epub ahead of print). This article describes the MRI features in AQP4-positive Japanese NMO patients and shows that ovoid lesions of MS are common and that those with prominent brain lesions have high risk of recurrent attacks.

  9. Scolding N: Devic’s disease and autoantibodies. Lancet Neurol 2005, 4:136–137.

    PubMed  Google Scholar 

  10. Wingerchuk DM, Lennon VA, Pittock SJ, et al.: Revised diagnostic criteria for neuromyelitis optica. Neurology 2006, 66:1485–1489.

    Article  CAS  PubMed  Google Scholar 

  11. Lennon VA, Kryzer TJ, Pittock SJ, et al.: IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med 2005, 202:473–477.

    Article  CAS  PubMed  Google Scholar 

  12. Matiello M, Lennon VA, Jacob A, et al.: NMO-IgG predicts the outcome of recurrent optic neuritis. Neurology 2008, 70:2197–2200.

    Article  CAS  PubMed  Google Scholar 

  13. Misu T, Fujihara K, Kakita A, et al.: Loss of aquaporin-4 in lesions in neuromyelitis optica: distinction from multiple sclerosis. Brain 2007, 130:1224–1234.

    Article  CAS  PubMed  Google Scholar 

  14. Roemer SF, Parisi JE, Lennon VA, et al.: Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis. Brain 2007, 130:1194–1205.

    Article  PubMed  Google Scholar 

  15. Jarius S, Paul F, Franciotta D, et al.: Mechanisms of disease: aquaporin-4 antibodies in neuromyelitis optica. Nat Clin Pract Neurol 2008, 4:202–214.

    CAS  PubMed  Google Scholar 

  16. Cree B: Neuromyelitis optica: diagnosis, pathogenesis, and treatment. Curr Neurol Neurosci Rep 2008, 8:427–433.

    Article  PubMed  Google Scholar 

  17. Bradl M, Misu T, Takahashi T, et al: Neuromyelitis optica: pathogenicity of patient immunoglobulin in vivo. Ann Neurol 2009, 66:630–643.

    Article  CAS  PubMed  Google Scholar 

  18. Nicchia GP, Mastrototaro M, Rossi A, et al.: Aquaporin-4 orthogonal arrays of particles are the target for neuromyelitis optica autoantibodies. Glia 2009, 57:1363–1373.

    Article  PubMed  Google Scholar 

  19. Bennett JL, Lam C, Kalluri SR, et al.: Intrathecal pathogenic anti-aquaporin-4 antibodies in early neuromyelitis optica. Ann Neurol 2009, 66:617–629.

    Article  CAS  PubMed  Google Scholar 

  20. Sloka JS, Steffanelli M: The mechanism of action of methylprednisolone in the treatment of multiple sclerosis. Mult Scler 2005, 11(4):425–432.

    Article  CAS  PubMed  Google Scholar 

  21. Braitch M, Harikrishnan S, Robins RA, et al.: Glucocorticoids increase CD4 + CD25 high cell percentage and Foxp3 expression in patients with multiple sclerosis. Acta Neurol Scand 2009, 119(4):239–245.

    Article  CAS  PubMed  Google Scholar 

  22. Goto H, Matsuo H, Nakane S, et al.: Plasmapheresis affects T helper type-1/T helper type-2 balance of circulating peripheral lymphocytes. Ther Apher 2001, 5:494–496.

    Article  CAS  PubMed  Google Scholar 

  23. Lehmann HC, Hartung HP, Hetzel GR, et al.: Plasma exchange in neuroimmunological disorders. Part 1: Rationale and treatment of inflammatory central nervous system disorders. Arch Neurol 2006, 63:930–935.

    Article  PubMed  Google Scholar 

  24. Weinshenker BG, O'Brien PC, Petterson TM, et al.: A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol 1999, 46:878–886.

    Article  CAS  PubMed  Google Scholar 

  25. Keegan M, Pineda AA, McClelland RL, et al.: Plasma exchange for severe attacks of CNS demyelination: predictors of response. Neurology 2002, 58:143–146.

    CAS  PubMed  Google Scholar 

  26. Watanabe S, Nashima I, Misu T, et al.: Therapeutic efficacy of plasma exchange in NMO-IgG–positive patients with neuromyelitis optica. Mult Scler 2007, 13:128–132.

    Article  CAS  PubMed  Google Scholar 

  27. Bonnan M, Valentino R, Olindo S: Plasma exchange in severe spinal attacks associated with neuromyelitis optica spectrum disorder. Mult Scler 2009, 15:487–492.

    Article  CAS  PubMed  Google Scholar 

  28. Llufriu S, Castillo J, Blanco Y, et al.: Plasma exchange for acute attacks of CNS demyelination: predictors of improvement. Neurology 2009, 73:949–953.

    Article  CAS  PubMed  Google Scholar 

  29. Aguilera AJ, Carlow TJ, Smith KJ, et al.: Lymphocytaplasmapheresis in Devic’s syndrome. Transfusion 1985, 25:54–56.

    Article  CAS  PubMed  Google Scholar 

  30. Nozaki I, Hamaguchi T, Komai K, et al.: Fulminant Devic disease successfully treated by lymphocytapheresis. J Neurol Neurosurg Psychiatry 2006, 77:1094–1095.

    Article  CAS  PubMed  Google Scholar 

  31. Papeix C, Vidal JS, De Seze J, et al.: Immunosuppressive therapy is more effective than interferon in neuromyelitis optica. Mult Scler 2007, 13:256–259.

    Article  CAS  PubMed  Google Scholar 

  32. Tanaka M, Tanaka K, Komori M: Interferonβ1b treatment in neuromyelitis optica. Eur Neurol 2009, 62:167–170.

    Article  CAS  PubMed  Google Scholar 

  33. Warabi Y, Matsumoto Y, Hayashi H: Interferon beta-1b exacerbates multiple sclerosis with severe optic nerve and spinal cord demyelination. J Neurol Sci 2007, 252:57–61.

    Article  CAS  PubMed  Google Scholar 

  34. Shimizu Y, Yokoyama K, Misu T, et al.: Development of extensive brain lesions following interferon beta therapy in relapsing neuromyelitis optica and longitudinally extensive myelitis. J Neurol 2008, 255:305–307.

    Article  PubMed  Google Scholar 

  35. Mandler RN, Ahmed W, Dencoff JE: Devic’s neuromyelitis optica: a prospective study of seven patients treated with prednisone and azathioprine. Neurology 1998, 51:1219–1220.

    CAS  PubMed  Google Scholar 

  36. Jacob A, Matiello M, Weinshenker BG, Wingerchuk DM: Treatment of neuromyelitis optica with mycophenolate mofetil. Retrospective analysis of 24 patients. Arch Neurol 2009, 66:1128–1133.

    Article  PubMed  Google Scholar 

  37. Jacob A, Weinshenker B, Violich I, et al.: Treatment of neuromyelitis optica with rituximab: retrospective analysis of 25 patients. Arch Neurol 2008, 65:1443–1448.

    Article  PubMed  Google Scholar 

  38. Kim HJ, Kim W, Park MS, et al.: B cell depletion therapy using rituximab in neuromyelitis optica spectrum disorders. Mult Scler 2009, 15:S253.

    Article  Google Scholar 

  39. Cree BA, Lamb S, Morgan K, et al.: An open label study of the effects of rituximab in neuromyelitis optica. Neurology 2005, 64:1270–1272.

    CAS  PubMed  Google Scholar 

  40. Weinstock-Guttman B, Ramanathan M, Lincoff N, et al.: Study of mitoxantrone for the treatment of neuromyelitis optica (Devic disease). Arch Neurol 2006, 63:957–963.

    Article  PubMed  Google Scholar 

  41. Watanabe S, Misu T, Miyazama I, et al.: Low-dose corticosteroids reduce relapses in neuromyelitis optica: a retrospective analysis. Mult Scler 2007, 13:968–974.

    Article  CAS  PubMed  Google Scholar 

  42. Okada K, Tsuji S, Takana K: Intermittent intravenous immunoglobulin successfully prevents relapses of neuromyelitis optica. Intern Med 2007, 46:1671–1672.

    Article  PubMed  Google Scholar 

  43. Bakker J, Metz L: Devic’s neuromyelitis optica treated with intravenous gamma globulin (IVIG). Can J Neurol Sci 2004, 31:265–267.

    PubMed  Google Scholar 

  44. Mok CC, To CH, Mak A, Poon WL: Immunoablative cyclophosphamide for refractory lupus-related neuromyelitis optica. J Rheumatol 2008, 35:172–174.

    PubMed  Google Scholar 

  45. Nasir S, Kerr D, Birnbaum J: Nineteen episodes of recurrent myelitis in a woman with neuromyelitis optica and systemic lupus erythematosus. Arch Neurol 2009, 66:1160–1163.

    Article  PubMed  Google Scholar 

  46. Petelin Gadze Z, Hajnsek S, Basic S, et al.: Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report. BMC Neurol 2009, 9:56.

    Article  PubMed  Google Scholar 

  47. An open label study of the effects of eculizumab in neuromyelitis optica. (http://clinicaltrials.gov/ct2/show/NCT00904826). Accessed March 8, 2010.

  48. Meinl E, Krumbholz M, Hohlfeld R: B lineage cells in the inflammatory central nervous system environment: migration, maintenance, local antibody production and therapeutic modulation. Ann Neurol 2006, 59:880–892.

    Article  CAS  PubMed  Google Scholar 

  49. Hematopoietic stem cell transplant in Devic's disease. (http://clinicaltrials.gov/ct2/show/NCT00787722). Accessed March 3, 2010.

  50. Warth A: Prevention of orthogonal array of particles formation as a treatment approach for neuromyelitis optica. Med Hypotheses 2009, 73:361–362.

    Article  CAS  PubMed  Google Scholar 

  51. Dale RC, Brilot F, Banwell B: Pediatric central nervous system inflammatory demyelination: acute disseminated encephalomyelitis, clinically isolated syndromes, neuromyelitis optica, and multiple sclerosis. Curr Opin Neurol 2009, 22:233–240.

    Article  PubMed  Google Scholar 

Download references

Disclosure

Dr. Fujihara has received honoraria for speaking or consulting from Asahi Kasei Kuraray Medical, a manufacturer of devices for apheresis. No other potential conflicts of interest relevant to this article were reported.

Author information

Authors and Affiliations

  1. Department of Neurology, Sir Charles Gairdner Hospital and the Centre for Neuromuscular and Neurological Disorders Australian Neuromuscular Research Institute, University of Western Australia, Hospital Avenue, Nedlands, Perth, Western Australia, 6009, Australia

    William M. Carroll MBBS, MD

  2. Department of Multiple Sclerosis Therapeutics, Tohoku University Graduate School of Medicine, 1-1 Seiryomachi, Aobaku, Sendai, 980-8574, Japan

    Kazuo Fujihara MD

Authors
  1. William M. Carroll MBBS, MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kazuo Fujihara MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to William M. Carroll MBBS, MD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Carroll, W.M., Fujihara, K. Neuromyelitis Optica. Curr Treat Options Neurol 12, 244–255 (2010). https://doi.org/10.1007/s11940-010-0071-z

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11940-010-0071-z

Keywords

Search

Navigation