Frequently Asked Questions
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NMOSD (historically known as Devic’s disease) is a rare autoimmune disease characterized by repeat attacks and accumulating, often permanent disability. NMOSD is characterized by attacks of optic neuritis, longitudinally extensive transverse myelitis, and area postrema syndrome.1-5

Review common symptoms of NMOSD

NMOSD is considered a rare disease. In the United States, it affects ~16,000 to ~17,000 people. NMOSD is most prevalent in women and African and Asian populations.6-9

Explore NMOSD demographics

B cells play multiple roles in the immunopathogenesis of NMOSD, including stimulating T cells and secreting cytokines. A pivotal role is played by plasmablasts and plasma B cells, which produce aquaporin-4 immunoglobulin (AQP4-IgG), the primary driver of NMOSD activity.10,11

Read more about the role of B cells in NMOSD

The root cause of NMOSD is unknown. The immunopathogenesis of NMOSD is characterized by the development of AQP4-IgG in B cells (predominantly in plasmablasts and plasma cells). These antibodies can cross the blood-brain barrier and target AQP4, an important water channel protein present on astrocytes in the central nervous system. Astrocytic damage leads to secondary injury to oligodendrocytes and results in demyelination.10-13

Explore the mechanism of disease of NMOSD

While NMOSD and multiple sclerosis (MS) do share certain clinical presentations and symptoms, NMOSD can be differentiated from MS through MRI distinction and AQP4-IgG cell-based antibody testing.14-16

See how to differentiate MS from NMOSD

Outcomes in patients with NMOSD can be devastating. While multiple sclerosis (MS) is characterized by relapses with near complete recovery in typical cases, the rate of recovery from NMOSD attack is low, and any single NMOSD attack can result in permanent disability.17,18

Compare the clinical courses of NMOSD and MS

The most common symptoms of NMOSD involve optic neuritis (eg, vision loss, ocular pain) or transverse myelitis (eg, limb weakness, numbness, sensory loss, or bladder and bowel problems). A smaller proportion of patients experience symptoms associated with area postrema syndrome, including vomiting, nausea, and intractable hiccups.4,5,19,20

See other symptoms of NMOSD

Optic neuritis is 1 of the 3 hallmark symptoms of NMOSD. In NMOSD, optic neuritis can present with blurred vision, severe vision loss, or even blindness. MRI typically reveals extensive optic nerve involvement (either unilateral or bilateral) that can extend into the optic chiasm.15,19

Review the initial clinical presentation of NMOSD

Transverse myelitis is 1 of the 3 hallmark symptoms of NMOSD. In NMOSD, myelitis is longitudinally extensive over multiple spinal cord segments and will typically involve >50% of the central spinal cord.4,15,16

Review the initial clinical presentation of NMOSD

In NMOSD, hiccups, nausea, and vomiting are caused by periventricular or circumventricular lesions in the brain or brain stem (also known as area postrema syndrome).15

Review the initial clinical presentation of NMOSD

Yes, though rarely. These symptoms are more indicative of area postrema syndrome, one of the core characteristics of NMOSD.5,20,21

Learn more about area postrema syndrome

Devastating attacks in NMOSD often lead to permanent disability, including blindness and paralysis. In one review of 163 patients with NMOSD, 40% of patients were legally blind in at least 1 eye at 5 years from first attack. In another review of 106 patients with NMOSD, 34% of patients had a motor disability and 23% were wheelchair-dependent at 6 years.1,2,22,23

See how disability can accumulate in NMOSD over time

If left untreated, 60% of patients will suffer a repeat NMOSD attack within a year, and 90% of patients will have a repeat attack within 3 years.21

Explore a timeline of the NMOSD patient experience

Without treatment, NMOSD has a mortality rate of 22% to 30% at 5 years from onset. However, with recent advancements in treatment, the 5-year mortality rate for patients with NMOSD has declined to ~5%.24

Learn what can happen without proper NMOSD treatment

NMOSD diagnosis requires the patient’s AQP4-IgG serostatus and a combination of 6 core characteristics: optic neuritis, transverse myelitis, area postrema syndrome, acute brain stem syndrome, symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions, and symptomatic cerebral syndrome with NMOSD-typical brain lesions.16

View all NMOSD diagnostic criteria

Area postrema syndrome is 1 of the 3 hallmark symptoms of NMOSD. The symptoms of area postrema syndrome include intractable hiccups, vomiting, and nausea. Approximately 30% of patients with NMOSD will experience area postrema symptoms during their illness.4,5,20

Review all 6 core clinical characteristics of NMOSD

MRI findings for NMOSD can include unilateral or bilateral optic neuritis, longitudinally extensive transverse myelitis, and periventricular or circumventricular lesions of the brain or brain stem.16

View MRI characteristics of NMOSD

AQP4-IgG antibodies are present in up to 90% of patients with NMOSD. Serum cell-based assays are the most sensitive method of detecting AQP4-IgG antibodies.6,16,22,25

Read more about cell-based assays in NMOSD

Currently, there are 3 FDA-approved treatments for NMOSD in adult patients who are AQP4-IgG+. Treatment choice should account for efficacy, safety profile, dosing frequency, administration, potential for adherence, and patient preference.26,27

Read about recent developments in NMOSD treatment

In 2022, the European Committee for Treatment and Research in Multiple Sclerosis (ECTRIMS) invited a panel of international NMOSD experts to develop validated statements on the management of AQP4-IgG+ NMOSD through the Delphi consensus process.27

View NMOSD treatment recommendations

The 3 currently FDA-approved NMOSD treatments differ in mechanism of action, dosing frequency, and method of administration. There have been no head-to-head trials among any of these 3 approved agents to date.28-30

See an NMOSD treatment comparison

Monitoring treatment efficacy should be part of any long-term treatment plan for patients with NMOSD. MRI may be a predictive tool of disease severity and the potential for attack recovery and may help you determine if a change in therapy is warranted. While certain serum and/or cerebral spinal fluid biomarkers have been shown to be of potential use for NMOSD diagnostic and treatment purposes, no guidelines currently exist for their use in a clinical setting.27,31,32

Explore methods of monitoring NMOSD treatment efficacy

Advocacy groups like The Guthy-Jackson Charitable Foundation, The Siegel Rare Neuroimmune Association, and The Sumaira Foundation can help support your patient throughout their treatment journey.

Visit an NMOSD resource library

Find an NMOSD Specialist

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  2. Borisow N, Mori M, Kuwabara S, Scheel M, Paul F. Diagnosis and treatment of NMO spectrum disorder and MOG-encephalomyelitis. Front Neurol. 2018;9:1-15. doi:10.3389/fneur.2018.00888
  3. Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology. 1999;53:1107-1114.
  4. Mealy MA, Wingerchuk DM, Greenberg BM, Levy M. Epidemiology of neuromyelitis optica in the United States: a multicenter analysis. Arch Neurol. 2012;69(9):1176-1180.
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  8. Bukhari W, Prain KM, Waters P, et al. Incidence and prevalence of NMOSD in Australia and New Zealand. J Neurol Neurosurg Psychiatry. 2017;0:1-7. doi:10.1136/jnnp-2016-314839
  9. Kim S-H, Mealy MA, Levy M, et al. Racial differences in neuromyelitis optica spectrum disorder. Neurology. 2018;91:e2089-e2099. doi:10.1212/WNL.0000000000006574
  10. Bennett JL, O’Connor KC, Bar-or A, et al. B lymphocytes in neuromyelitis optica. Neurology. 2015;2:1-11. doi:10.1212/NXI.0000000000000104
  11. Forsthuber TG, Cimbora TM, Ratchford JN, et al. B cell-based therapies in CNS autoimmunity: differentiating CD19 and CD20 as therapeutic targets. Ther Adv Neurol Disord. 2018;11:1-13. doi:10.1177/1756286418761697
  12. Carnero Contentti E, Correale J. Neuromyelitis optica spectrum disorders: from pathophysiology to therapeutic strategies. J Neuroinflamm. 2021;18(208):1-18. doi:10.1186/s12974-021-02249-1
  13. Papadopolous MC, Verkman AS. Aquaporin 4 and neuromyelitis optica. Lancet Neurol. 2012;11(6):535-544. doi:10.1016/S1474-4422(12)70133-3
  14. Beekman J, Keisler A, Pedraza O, et al. Neuromyelitis optica spectrum disorder: patient experience and quality of life. Neurol Neuroimmunol Neuroinflamm. 2019;6:1-14. doi:10.1212/NXI.0000000000000580
  15. Dutra BG, da Rocha AJ, Nunes RH, Martins Maia Júnior AC. Neuromyelitis optica spectrum disorders: spectrum of MR imaging findings and their differential diagnosis. RadioGraphics. 2018;38:169-193.
  16. Wingerchuk DM, Banwell B, Bennett J, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85:177-189.
  17. Huda S, Whittam D, Bhojak M. Neuromyelitis optica spectrum disorders. Clin Med. 2019;19(2):169-176.
  18. Kawachi I, Lassmann H. Neurodegeneration in multiple sclerosis and neuromyelitis optica. J Neurol Neurosurg Psychiatry. 2016;88:137-145.
  19. NORD rare disease database. Accessed on November 21, 2022. https://rarediseases.org/rare-diseases/neuromyelitis-optica/
  20. Shosha E, Dubey D, Palace J, et al. Area postrema syndrome: frequency, criteria, and severity in AQP4-IgG–positive NMOSD. Neurology. 2018;19:e1642-e1651.
  21. Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol. 2007;6:805-815
  22. Jiao Y, Fryer JP, Lennon VA, et al. Updated estimate of AQP4-IgG serostatus and disability outcome in neuromyelitis optica. Neurology. 2013;81:1197-1204.
  23. Kitley J, Leite MI, Nakashima I, et al. Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan. Brain. 2012;135:1834-1849.
  24. Wallach AI, Tremblay M, Kister I. Advances in the treatment of neuromyelitis optica spectrum disorder. Neurol Clin. 2021;39:35-49.
  25. Hamid SHM, Whittam D, Mutch K, et al. What proportion of AQP4‐IgG‐negative NMO spectrum disorder patients are MOG‐IgG positive? A cross sectional study of 132 patients. J Neurol. 2017;264:2088-2094.
  26. Wingerchuk DM, Lucchinetti CF. Neuromyelitis optica spectrum disorder. N Engl J Med. 2022;387:631-639. doi:10.1056/NEJMra1904655
  27. 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: 38th Congress of ECTRIMS (Amsterdam); October 26-28, 2022.
  28. Cree BAC, Greenberg B, Cameron C, Weinshenker BG. Letter to the editor regarding ‘‘Network meta-analysis of Food and Drug Administration-approved treatment options for adults with aquaporin-4 immunoglobulin G-positive neuromyelitis optica spectrum disorder.” Neurol Ther. 2022;11:1439-1443.
  29. SOLIRIS® (eculizumab) [prescribing information]. Alexion Pharmaceuticals, Inc.
  30. ENSPRYNG® (satralizumab-mwge) [prescribing information]. Genentech, Inc.
  31. 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
  32. 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