Diagnosis will incorporate at least 1 of the following 6 core clinical
characteristics, all evident on MRI1:

Optic neuritis

Optic neuritis

Acute myelitis icon

Acute myelitis

Area postrema syndrome

Area postrema syndrome

Acute Brain Stem

Acute brain stem syndrome (with associated periependymal brain stem lesions on MRI in AQP4-IgG negative patients)

Symptomatic narcolepsy icon

Symptomatic narcolepsy or acute diencephalic clinical syndrome with diencephalic lesions on MRI that are characteristic of NMOSD

Symptomatic cerebral syndrome icon

Symptomatic cerebral syndrome with brain lesions that are characteristic of NMOSD

Monitoring treatment efficacy should be part of any
long-term treatment plan2

Clinical markers and patient symptoms provide important
information about disease activity3,4

MRI scan icon

May be a predictive tool of disease severity and the potential for attack recovery5,6

  • Longer lesion length, presence of Gd enhancement, and central cord involvement are associated with higher EDSS scores5,6
  • MRI may help discriminate relapses from pseudorelapses and determine if a change in therapy is warranted6

Serum cell-based assay test icon

Levels at onset may help indicate future disease course and disease severity3

  • Higher levels of sNfL have been correlated with higher EDSS scores at disease onset3
  • Increased levels of sGFAP are associated with an increased risk and intensity of NMOSD attack7
  • B-cell counts may be helpful in assessing a patient’s risk for major attack8
  • AQP4-IgG titers may indicate disease severity during the attack phase9

Sleep disorders icon

May be a factor of predictive value in NMOSD attack4

  • Sleep disturbance is common among the NMOSD population4
  • The frequency of sleep disturbance increases around the time of acute NMOSD attack4

Acute myelitis icon

Common and associated with reduced QOL10

  • In a survey of 166 patients with NMOSD, 75% suffered from chronic pain10
  • NMOSD-associated pain severity has been strongly correlated with reduction of activities of daily living10

Any new or worsening symptom (eg, tingling, numbness, vision loss, sleep
disturbance) should be investigated as a potential sign of attack recurrence
and loss of therapeutic efficacy2,11

EDSS, Expanded Disability Status Scale; Gd, gadolinium; sGFAP, serum glial fibrillary acidic protein; sNfL, serum neurofilament light chain.

  1. Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85:177-189.
  2. Paul F, Marignier R, Palace J; on behalf of the NMOSD Delphi Panel. International, evidence-based Delphi consensus on the management of AQP4-IgG positive NMOSD, with a focus on treatment recommendations for eculizumab, inebilizumab and satralizumab. Poster P008 presented at: ECTRIMS 2022 (Amsterdam): October 25-28, 2022.
  3. Wang J, Cui C, Lu Y, et al. Therapeutic response and possible biomarkers in acute attacks of neuromyelitis optica spectrum disorders: a prospective observational study. Front Immunol. 2021;12:1-11. doi:10.3389/fimmu.2021.720907
  4. Rabi SHM, Shahmirzaei S, Sahraian MA, et al. Sleep disorders as a possible predisposing attack factor in neuromyelitis optica spectrum disorder (NMOSD): a case control study. Clin Neurol Neurosurg. 2021;204:1-5. doi:10.1016/j.clineuro.2021.106606
  5. Xu Y, Ren Y, Li X, et al. Persistently gadolinium-enhancing lesion is a predictor of poor prognosis in NMOSD attack: a clinical trial. Neurotherapeutics. 2021;18:868-877.
  6. Solomon JM, Paul F, Chien C. A window into the future? MRI for evaluation of neuromyelitis optica spectrum disorder throughout the disease course. Ther Adv Neurol Disord. 2021;14:1-18. doi:10.1177/17562864211014389
  7. Aktas O, Smith MA, Rees WA, et al. Serum glial fibrillary acidic protein: a neuromyelitis optica spectrum disorder biomarker. Ann Neurol. 2021;89:895-910.
  8. Traub J, Häusser-Kinzel S, Weber MS. Differential effects of MS therapeutics on B cells—implications for their use and failure in AQP4-positive NMOSD patients. Int J Mol Sci. 2020;21:1-30. doi:10.3390/ijms21145021
  9. Liu J, Tan G, Gao Y, et al. Serum aquaporin 4-immunoglobulin G titer and neuromyelitis optica spectrum disorder activity and severity: a systematic review and meta-analysis. Front Neurol. 2021:12:1-10. doi:10.3389/fneur.2021.746959
  10. Ayzenberg I, Richter D, Henke E, et al. Pain, depression, and quality of life in neuromyelitis optica spectrum disorder: a cross-sectional study of 166 AQP4 antibody–seropositive patients. Neurol Neuroimmunol Neuroinflamm. 2021;8:1-14. doi:10.1212/NXI.0000000000000985
  11. Wingerchuk DM, Lucchinetti CF. Neuromyelitis optica spectrum disorder. N Engl J Med. 2022;387:631-639. doi:10.1056/NEJMra1904655