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NMOSD is a rare autoimmune disease characterized by repeat attacks and accumulating, often permanent disability1,2

Predominantly involves attacks of optic neuritis or longitudinally extensive transverse myelitis, but can also involve brain or brain stem inflammation3-5

optic-neuritis-icon
Optic neuritis
longitudinally-extensive-transverse-myelitis-icon
Longitudinally extensive transverse myelitis
brain-stem-inflammation-icon
Brain or brain stem inflammation

NMOSD is most prevalent in women and disproportionately affects African American and Asian populations6-9

Differences exist in disease presentation and attack severity, and genetic variations can complicate treatment8-11

  • Patient
    Population

  • Race

  • Gene Mutation
    Affecting
    Treatment

US population of NMOSD patients icon

Estimated US population: 16,000 to 17,0007

AQP4-IgG+ NMOSD is more prevalent in women icon

AQP4-IgG+ NMOSD is 9 times more prevalent in
women vs men6

Calendar icon

Median onset: ∼40 years (Range = ∼20 to 70 years)1,4

Race icon

Prevalence is ∼2- to 3-fold higher in African American and Asian patients than in Caucasian patients7,8

African American and Asian patients

  • Are an average of ∼10 years younger at diagnosis than Caucasian patients9
  • Are more likely to have brain or brain stem involvement than Caucasian patients9

African American patients

  • Have a more aggressive course of disease than Caucasian patients, resulting in a greater risk of early and severe disability9
  • Are more likely to suffer a severe attack at onset than Caucasian patients9
  • Have a 15% mortality rate, more than twice the rate of the total NMOSD population12

Gene mutation affecting treatment icon

>40% of patients have a genetic risk factor that results in reduced IgG binding and diminished efficacy of some monoclonal antibodies, including rituximab10,11

  • This mutation has been associated with a risk of at least 1 relapse while receiving rituximab treatment, a risk of insufficient memory B-cell depletion, and a short retreatment interval during the initial 2 years of treatment11

Explore the mechanism of disease of NMOSD Mechanism of Disease

NMOSD, neuromyelitis optica spectrum disorder.

  • References

    1. Ajmera MR, Boscoe A, Mauskopf J, Candrilli SD, Levy M. Evaluation of comorbidities and health care resource use among patients with highly active neuromyelitis optica. J Neurol Sci. 2018;384:96-103.
    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.
    5. Sellner J, Boggild M, Clanet M, et al. EFNS guidelines on diagnosis and management of neuromyelitis optica. Eur Neurol. 2010;17:1019-1032.
    6. Jarius S, Ruprecht K, Wildemann B, et al. Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: a multicentre study of 175 patients. J Neuroinflammation. 2012;9(14):1-17.
    7. Flanagan EP, Cabre P, Weinshenker BG, et al. Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum. Ann Neurol. 2016;79(5):775-783. doi:10.1002/ana.24617
    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. Mahaweni NM, Olieslagers TI, Rivas IO, et al. A comprehensive overview of FCGR3A gene variability by full-length gene sequencing including the identification of V158F polymorphism. Nature. 2018;8:1-11. doi:10.1038/s41598-018-34258-1
    11. Kim S-H, Jeong IH, Hyun J-W, et al. Treatment outcomes with rituximab in 100 patients with neuromyelitis optica: influence of FCGR3A polymorphisms on the therapeutic response to rituximab. JAMA Neurol. 2015;72(9):989-995. doi:10.1001/jamaneurol.2015.1276
    12. Mealy MA, Kessler RA, Rimler Z, et al. Mortality in neuromyelitis optica is strongly associated with African ancestry. Neurol Neuroimmunol Neuroinflamm. 2018;5:1-4. doi:10.1212/NXI.0000000000000468