Skip to main content

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 318))

Neuromyelitis optica (NMO) is a severe demyelinating disease of the CNS that preferentially affects the optic nerves and spinal cord, tends to relapse, and results in early permanent disability for most affected patients. A new autoantibody marker called neuromyelitis optica immunoglobulin G (NMO-IgG), which targets the water channel protein aquaporin-4, is highly specific for NMO. The marker has demonstrated that the NMO spectrum of disorders is wider than previously known and includes some patients with single-episode or recurrent longitudinally extensive myelitis, recurrent isolated optic neuritis, Asian optic—spinal multiple sclerosis, and patients with co-existing systemic autoimmune diseases such as lupus erythematosus or Sjögren's syndrome. We review the place of NMO within the nosology of CNS demyelinating diseases, the discovery of NMO-IgG and its impact on the definition of NMO and its spectrum, implications for understanding NMO pathogenesis, and informing treatment decisions.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
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

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Agre P, Kozono D (2003) Aquaporin water channels: molecular mechanisms for human diseases. FEBS Lett 555:72-78

    Article  CAS  PubMed  Google Scholar 

  2. Albutt TC (1870) On the ophthalmoscopic signs of spinal disease. Lancet 1:76-78

    Google Scholar 

  3. Amiry-Moghaddam M, Ottersen OP (2003) The molecular basis of water transport in the brain. Nat Rev Neurosci 4:991-1001

    Article  CAS  PubMed  Google Scholar 

  4. Cree BA, Lamb S, Morgan K, Chen A, Waubant E, Genain C (2005) An open label study of the effects of rituximab in neuromyelitis optica. Neurology 64:1270-1272

    CAS  PubMed  Google Scholar 

  5. de Seze J, Lebrun C, Stojkovic T, Ferriby D, Chatel M, Vermersch P (2003) Is Devic’s neuromyelitis optica a separate disease? A comparative study with multiple sclerosis. Mult Scler 9:521-525

    Article  CAS  PubMed  Google Scholar 

  6. de Seze J, Stojkovic T, Ferriby D, Gauvrit JY, Montagne C, Mounier-Vehier F, Verier A, Pruvo JP, Hache JC, Vermersch P (2002) Devic’s neuromyelitis optica: clinical, laboratory, MRI and outcome profile. J Neurol Sci 197:57-61

    Article  CAS  PubMed  Google Scholar 

  7. Devic E (1894) Myélite aigue compliquée de névrite optique. Bull Med (Paris) 8:1033-1034

    Google Scholar 

  8. Ghezzi A, Bergamaschi R, Martinelli V, Trojano M, Tola MR, Merelli E, Mancardi L, Gallo P, Filippi M, Zaffaroni M, Comi G (2004) Clinical characteristics, course and prognosis of relapsing Devic’s neuromyelitis optica. J Neurol 251:47-52

    Article  PubMed  Google Scholar 

  9. Jarius S, Franciotta D, Bergamaschi R, Wright H, Littleton E, Palace J, Hohlfeld R, Vincent A (2007) NMO-IgG in the diagnosis of neuromyelitis optica. Neurology 68:1076-1077

    Article  CAS  PubMed  Google Scholar 

  10. Keegan M, Konig F, McClelland R, Bruck W, Morales Y, Bitsch A, Panitch H, Lassmann H, Weinshenker B, Rodriguez M, Parisi J, Lucchinetti CF (2005) Relation between humoral pathological changes in multiple sclerosis and response to therapeutic plasma exchange. Lancet 366:579-582

    Article  PubMed  Google Scholar 

  11. Keegan M, Pineda AA, McClelland RL, Darby CH, Rodriguez M, Weinshenker BG (2002) Plasma exchange for severe attacks of CNS demyelination: predictors of response. Neurology 58:143-146

    CAS  PubMed  Google Scholar 

  12. Kira J (2003) Multiple sclerosis in the Japanese population. Lancet Neurol 2:117-127

    Article  PubMed  Google Scholar 

  13. Kister I, Gulati S, Boz C, Bergamaschi R, Piccolo G, Oger J, Swerdlow ML (2006) Neuromyelitis optica in patients with myasthenia gravis who underwent thymectomy. Arch Neurol 63:851-856

    Article  PubMed  Google Scholar 

  14. Lassmann H, Bruck W, Lucchinetti C (2001) Heterogeneity of multiple sclerosis pathogenesis: implications for diagnosis and therapy. Trends Mol Med 7:115-121

    Article  CAS  PubMed  Google Scholar 

  15. Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR (2005) IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med 202:473-477

    Article  CAS  PubMed  Google Scholar 

  16. Lennon VA, Wingerchuk DM, Kryzer TJ, Pittock SJ, Lucchinetti CF, Fujihara K, Nakashima I, Weinshenker BG (2004) A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 364:2106-2112

    Article  CAS  PubMed  Google Scholar 

  17. Lucchinetti CF, Mandler RN, McGavern D, Bruck W, Gleich G, Ransohoff RM, Trebst C, Weinshenker B, Wingerchuk D, Parisi JE, Lassmann H (2002) A role for humoral mechanisms in the pathogenesis of Devic’s neuromyelitis optica. Brain 125:1450-1461

    Article  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  19. Misu T, Fujihara K, Nakamura M, Murakami K, Endo M, Konno H, Itoyama Y (2006) Loss of aquaporin-4 in active perivascular lesions in neuromyelitis optica: a case report. Tohoku J Exp Med 209:269-275

    Article  PubMed  Google Scholar 

  20. Misu T, Kakita A, Fujihara K et al (2005) A comparative neuropathological analysis of Japanese cases of neuromyelitis optica and multiple sclerosis. Neurology 64:A39

    Google Scholar 

  21. Nakashima I, Fujihara K, Miyazawa I, Misu T, Narikawa K, Nakamura M, Watanabe S, Takahashi T, Nishiyama S, Shiga Y, Sato S, Weinshenker BG, Itoyama Y (2006) Clinical and MRI features of Japanese patients with multiple sclerosis positive for NMO-IgG. J Neurol Neurosurg Psychiatry 77:1073-1075

    Article  CAS  PubMed  Google Scholar 

  22. Nielsen S, Nagelhus EA, Amiry-Moghaddam M, Bourque C, Agre P, Ottersen OP (1997) Specialized membrane domains for water transport in glial cells: high-resolution immunogold cytochemistry of aquaporin-4 in rat brain. J Neurosci 17:171-180

    CAS  PubMed  Google Scholar 

  23. O’Riordan JI, Gallagher HL, Thompson AJ, Howard RS, Kingsley DP, Thompson EJ, McDonald WI, Miller DH (1996) Clinical, CSF, and MRI findings in Devic’s neuromyelitis optica. J Neurol Neurosurg Psychiatry 60:382-387

    Article  PubMed  Google Scholar 

  24. Papais-Alvarenga RM, Miranda-Santos CM, Puccioni-Sohler M, de Almeida AM, Oliveira S, Basilio De Oliveira CA, Alvarenga H, Poser CM (2002) Optic neuromyelitis syndrome in Brazilian patients. J Neurol Neurosurg Psychiatry 73:429-435

    Article  CAS  PubMed  Google Scholar 

  25. Papeix C, de Seze J (2005) French therapeutic experience of Devic’s disease: a retrospective study of 33 cases. Neurology 64 [Suppl1]:A328-A329

    Google Scholar 

  26. Pittock SJ, Mayr WT, McClelland RL, Jorgensen NW, Weigand SD, Noseworthy JH, Rodriguez M (2004) Disability profile of MS did not change over 10 years in a populationbased prevalence cohort. Neurology 62:601-606

    CAS  PubMed  Google Scholar 

  27. Pittock SJ, Lennon VA, Krecke K, Wingerchuk DM, Lucchinetti CF, Weinshenker BG (2006) Brain abnormalities in neuromyelitis optica. Arch Neurol 63:390-396

    Article  PubMed  Google Scholar 

  28. Pittock SJ, Weinshenker BG, Lucchinetti CF, Wingerchuk DM, Corboy JR, Lennon VA (2006) Neuromyelitis optica brain lesions localized at sites of high aquaporin 4 expression. Arch Neurol 63:964-968

    Article  PubMed  Google Scholar 

  29. Poppe AY, Lapierre Y, Melancon D, Lowden D, Wardell L, Fullerton LM, Bar-Or A (2005) Neuromyelitis optica with hypothalamic involvement. Mult Scler 11:617-621

    Article  PubMed  Google Scholar 

  30. Roemer SF, Parisi JE, Lennon VA, Benarroch EE, Lassmann H, Bruck W, Mandler RN, Weinshenker BG, Pittock SJ, Wingerchuk DM, Lucchinetti CF (2007) Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis. Brain 130:1194-1205

    Article  PubMed  Google Scholar 

  31. Warabi Y, Matsumoto Y, Hayashi H (2006) Interferon beta-1b exacerbates multiple sclerosis with severe optic nerve and spinal cord demyelination. J Neurol Sci 252:57-61

    Article  PubMed  Google Scholar 

  32. Weinshenker B, Pittock S, de Seze J, Vermersch P, Wingerchuk D, Zephir H, Homberger H, Lucchinetti C, Lennon V (2006) The relationship between neuromyelitis optica and systemic autoimmune disease. Mult Scler 12:S16

    Google Scholar 

  33. Weinshenker BG, O’Brien PC, Petterson TM, Noseworthy JH, Lucchinetti CF, Dodick DW, Pineda AA, Stevens LN, Rodriguez M (1999) A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol 46:878-886

    Article  CAS  PubMed  Google Scholar 

  34. Weinshenker BG, Wingerchuk DM, Vukusic S, Linbo L, Pittock SJ, Lucchinetti CF, Lennon VA (2006) Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol 59:566-569

    Article  CAS  PubMed  Google Scholar 

  35. Weinstock-Guttman B, Ramanathan M, Lincoff N, Napoli SQ, Sharma J, Feichter J, Bakshi R (2006) Study of mitoxantrone for the treatment of recurrent neuromyelitis optica (Devic disease). Arch Neurol 63:957-963

    Article  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  37. Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG (2006) Revised diagnostic criteria for neuromyelitis optica. Neurology 66:1485-1489

    Article  CAS  PubMed  Google Scholar 

  38. Wingerchuk DM, Weinshenker BG (2005) Neuromyelitis optica. Curr Treat Options Neurol 7:173-182

    Article  PubMed  Google Scholar 

  39. Zuliani L, Lopez de Munain A, Ruiz Martinez J, Olascoaga J, Graus F, Saiz A (2006) Anticuerpos IgG-NMO en la neuromyelitis optica: proposito de 2 casos. Neurologia 21:314-317

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Weinshenker, B.G., Wingerchuk, D.M. (2008). Neuromyelitis Optica: Clinical Syndrome and the NMO-IgG Autoantibody Marker. In: Rodriguez, M. (eds) Advances in multiple Sclerosis and Experimental Demyelinating Diseases. Current Topics in Microbiology and Immunology, vol 318. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-73677-6_14

Download citation

Publish with us

Policies and ethics