May be a predictive tool of disease severity and the potential for attack recovery5,6
Diagnosis will incorporate at least 1 of the following 6 core clinical
characteristics, all evident on MRI1:
Optic neuritis
Acute myelitis
Area postrema syndrome
Acute brain stem syndrome (with associated periependymal brain stem lesions on MRI in AQP4-IgG negative patients)
Symptomatic narcolepsy or acute diencephalic clinical syndrome with diencephalic lesions on MRI that are characteristic of NMOSD
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
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.
References
- Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85:177-189.
- 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.
- 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
- 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
- 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.
- 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
- 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.
- 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
- 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
- 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
- Wingerchuk DM, Lucchinetti CF. Neuromyelitis optica spectrum disorder. N Engl J Med. 2022;387:631-639. doi:10.1056/NEJMra1904655