20. Diagnosis and Management of Neuromyelitis Optica

  1. Nils Erik Gilhus MD, PHD10,11,
  2. Michael P. Barnes MD, FRCP12,13 and
  3. Michael Brainin MD14,15,16
  1. J. Sellner1,
  2. M. Boggild2,
  3. M. Clanet3,
  4. R. Q. Hintzen4,
  5. Z. Illes5,
  6. X. Montalban6,
  7. R. A. Du Pasquier7,
  8. C. H. Polman8,
  9. P. Soelberg Sørensen9 and
  10. B. Hemmer1

Published Online: 21 SEP 2011

DOI: 10.1002/9781444346268.ch20

European Handbook of Neurological Management, Volume 2, Second Edition

European Handbook of Neurological Management, Volume 2, Second Edition

How to Cite

Sellner, J., Boggild, M., Clanet, M., Hintzen, R. Q., Illes, Z., Montalban, X., Du Pasquier, R. A., Polman, C. H., Soelberg Sørensen, P. and Hemmer, B. (2011) Diagnosis and Management of Neuromyelitis Optica, in European Handbook of Neurological Management, Volume 2, Second Edition (eds N. E. Gilhus, M. P. Barnes and M. Brainin), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444346268.ch20

Editor Information

  1. 10

    Department of Clinical Medicine, University of Bergen, Norway

  2. 11

    Department of Neurology, Haukeland University Hospital, Bergen, Norway

  3. 12

    University of Newcastle, Newcastle upon Tyne, UK

  4. 13

    Hunters Moor Neurorehabilitation Ltd, Newcastle upon Tyne, UK

  5. 14

    Department of Clinical Medicine and Prevention, Austria

  6. 15

    Center for Clinical Neurosciences, Donau-Universität Krems, Austria

  7. 16

    Department of Neurology, Landesklinikum Donauregion Tulln, Tulln, Austria

Author Information

  1. 1

    Klinikum rechts der Isar, Technische Universität München, Munich, Germany

  2. 2

    The Walton Centre for Neurology and Neurosurgery, Liverpool, United Kingdom

  3. 3

    Purpan University Hospital, Toulouse, France

  4. 4

    Erasmus MC, University Medical Centre, Rotterdam, The Netherlands

  5. 5

    University of Pecs, Pecs, Hungary

  6. 6

    Hospital Universitari Vall d'Hebron (HUVH), Barcelona, Spain

  7. 7

    Centre Hospitalier Universitaire Vaudois and University of Lausanne, Lausanne, Switzerland

  8. 8

    VU University Medical Centre, MS Center, Amsterdam, The Netherlands

  9. 9

    Copenhagen University and Rigshospitalet, Copenhagen, Denmark

Publication History

  1. Published Online: 21 SEP 2011
  2. Published Print: 30 SEP 2011

ISBN Information

Print ISBN: 9781405185349

Online ISBN: 9781444346268



  • diagnosis and management of neuromyelitis optica;
  • guidelines, for best practice diagnosis and management of adult neuromyelitis optica;
  • neuromyelitis optica (NMO, as Devic's disease)-severe idiopathic immune-mediated demyelinating and necrotizing disease;
  • NMO, recurrent autoimmune CNS disorder-clinical, neuroimaging and laboratory findings, distinct from MS;
  • NMO, different clinical entity from MS-worldwide occurrence, with ethnic variations;
  • myelitis, spinal cord involvement-complete transverse myelitis with para-or tetraparesis;
  • NMO-IgG/AQP4 antibodies-detected years, before onset of NMO;
  • NMO spectrum disorders-spatially limited syndromes, brain lesions, comorbidities and OSMS;
  • therapeutic efficacy of plasmapheresis-for relapse prevention;
  • panel recommendations-for immunosuppressive treatment of NMO


Background and purpose: Neuromyelitis optica (NMO) or Devic′s disease is a rare inflammatory and demyelinating autoimmune disorder of the central nervous system (CNS) characterized by recurrent attacks of optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM), which is distinct from multiple sclerosis (MS). The guidelines are designed to provide guidance for best clinical practice based on the current state of clinical and scientific knowledge.

Search strategy: Evidence was collected from searches of original articles, case reports and meta-analyses in the Medline and Cochrane databases. In addition, clinical practice guidelines of professional neurological and rheumatological organizations were studied.

Results: Different diagnostic criteria for NMO diagnosis (Wingerchuk et al. Revised NMO criteria, 2006; Miller et al. NMSS task force criteria, 2008) and features potentially indicative of NMO facilitate the diagnosis. In addition, guidance for the work-up and diagnosis of spatially limited NMO spectrum disorders is provided by the Task Force. Owing to a lack of studies fulfilling the requirement for the highest levels of evidence the Task Force suggests treatment of acute exacerbations and attack prevention based on expert opinion.

Conclusions: Studies of diagnosis and management of NMO fulfilling requirements for the highest levels of evidence (class I–III rating) are limited and diagnostic and therapeutic concepts based on expert opinion and consensus of the Task Force members were drawn up for this guideline.