Cited by 595Multiple Sclerosis Research StudiesOcular Diseases and Behçet’s SyndromePeripheral Neuropathies and Disorders

Update on the diagnosis and treatment of neuromyelitis optica: Recommendations of the Neuromyelitis Optica Study Group (NEMOS)

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Neuromyelitis Optica Study Group (NEMOS), Trebst, Corinna, Jarius, Sven, Berthele, Achim, Paul, Friedemann, Schippling, Sven, Wildemann, Brigitte, Borisow, Nadja +3 more·2014·Journal of Neurology

Abstract

Neuromyelitis optica (NMO, Devic's syndrome), long considered a clinical variant of multiple sclerosis, is now regarded as a distinct disease entity. Major progress has been made in the diagnosis and treatment of NMO since aquaporin-4 antibodies (AQP4-Ab; also termed NMO-IgG) were first described in 2004. In this review, the Neuromyelitis Optica Study Group (NEMOS) summarizes recently obtained knowledge on NMO and highlights new developments in its diagnosis and treatment, based on current guidelines, the published literature and expert discussion at regular NEMOS meetings. Testing of AQP4-Ab is essential and is the most important test in the diagnostic work-up of suspected NMO, and helps to distinguish NMO from other autoimmune diseases. Furthermore, AQP4-Ab testing has expanded our knowledge of the clinical presentation of NMO spectrum disorders (NMOSD). In addition, imaging techniques, particularly magnetic resonance imaging of the brain and spinal cord, are obligatory in the diagnostic workup. It is important to note that brain lesions in NMO and NMOSD are not uncommon, do not rule out the diagnosis, and show characteristic patterns. Other imaging modalities such as optical coherence tomography are proposed as useful tools in the assessment of retinal damage. Therapy of NMO should be initiated early. Azathioprine and rituximab are suggested as first-line treatments, the latter being increasingly regarded as an established therapy with long-term efficacy and an acceptable safety profile in NMO patients. Other immunosuppressive drugs, such as methotrexate, mycophenolate mofetil and mitoxantrone, are recommended as second-line treatments. Promising new therapies are emerging in the form of anti-IL6 receptor, anti-complement or anti-AQP4-Ab biologicals.

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  • An accurate diagnosis of NMOSD is essential since prompt treatment of acute attacks and long-term immunosuppression appear to reduce disability, while conventional MS treatments may aggravate NMOSD.

    “An accurate diagnosis of NMOSD is essential since prompt treatment of acute attacks and long-term immunosuppression appear to reduce disability, while conventional MS treatments may aggravate NMOSD.44,48”

    From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [44]

  • The identification of a pathogenic IgG antibody directed against the aquaporin-4 (AQP4-IgG) water channel has established NMOSD as a specific disease entity that needs to be differentiated from MS because of important differences in prognosis and treatment.

    “The identification of a pathogenic IgG antibody directed against the aquaporin-4 (AQP4-IgG) water channel has established NMOSD as a specific disease entity that needs to be differentiated from MS because of important differences in prognosis and treatment.44,45”

    From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [44]

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Authors
Neuromyelitis Optica Study Group (NEMOS), Trebst, C., Jarius, S., Berthele, A., Paul, F., Schippling, S., Wildemann, B., Borisow, N., Kleiter, I., Aktas, O., & Kümpfel, T.
Affiliations
  • Medizinische Hochschule Hannover (DE)
  • Heidelberg University (DE)
  • Max Delbrück Center (DE)
  • St. Josef-Hospital (DE)
  • Institut für Klinische Neuroimmunologie (DE)
Corresponding author
Kümpfel, Tania
Venue
Journal of Neurology
Published
2013-11-23
Volume
261
Issue
1
Pages
1-16
ISSN
  • 0012-1037 (ISSN-L)
  • 0340-5354
  • 1432-1459
OpenAlex
W2038941725
Cited by
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FWCI
6.38
Type
journal article
Language
en
Open access
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  • MDC Repository (Max-Delbrueck-Center for Molecular Medicine) (submittedVersion)
  • Zurich Open Repository and Archive (University of Zurich) (submittedVersion, other-oa)
  • mediaTUM – the media and publications repository of the Technical University Munich (Technical University Munich) (submittedVersion)
  • Zurich Open Repository and Archive (University of Zurich)
Topics
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
  • Health Sciences › Medicine › Ophthalmology › Ocular Diseases and Behçet’s Syndrome
  • Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
Keywords
neuromyelitis optica, Devic's syndrome, aquaporin-4 antibodies, neuromyelitis optica spectrum disorder, NMO spectrum disorders, diagnosis, treatment, rituximab, azathioprine, immunosuppressive therapy, brain lesions, optical coherence tomography, retinal damage, anti-complement therapy, magnetic resonance imaging
MeSH terms
Diagnosis-Related Groups, Humans, Neuromyelitis Optica, Medication Reconciliation

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

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