Clinical features and prognosis of late-onset neuromyelitis optica spectrum disorders in a Latin American cohort

  • 17 Accesses



We aimed to assess the clinical, paraclinical, imaging and prognostic features of patients with late-onset neuromyelitis optica spectrum disorder (LO-NMOSD; ≥ 50 years at disease onset) LO-NMOSD, compared with early onset-NMOSD (EO-NMOSD, ≤ 49 years at disease onset), in Latin American (LATAM).


We retrospectively reviewed the medical records of patients with NMOSD, as defined using the 2015 validated diagnostic criteria. We included patients from Argentina, Brazil and Venezuela. They were divided into: LO-NMOSD and EO-NMOSD and comparison among the groups were performed.


Among these 140 NMOSD patients, 24 (17.1%) were LO-NMOSD; 64% were positive for aquaporin-4 antibodies; and 41.5% of this population cohort was non-Caucasian. Severe disability [expanded disability status scale (EDSS) ≥ 6] at the last follow-up and presence of comorbidities were significantly associated with LO-NMOSD, compared with EO-NMOSD. LO-NMOSD patients had a shorter median time to EDSS ≥ 4 than EO-NMOSD patients (46 vs. 60 months; log-rank test p = 0.0006). Furthermore, we observed a positive correlation between age at onset and EDSS score at the last follow-up (Spearman r = 0.34, p < 0.0001).


LO-NMOSD patients from LATAM developed early severe disability, compared with EO-NMOSD. Therefore, age at onset could have important implications for the long-term prognosis of NMOSD patients.

This is a preview of subscription content, log in to check access.

Access options

Buy single article

Instant unlimited access to the full article PDF.

US$ 39.95

Price includes VAT for USA

Subscribe to journal

Immediate online access to all issues from 2019. Subscription will auto renew annually.

US$ 199

This is the net price. Taxes to be calculated in checkout.

Fig. 1
Fig. 2


  1. 1.

    Wingerchuk DM, Lennon VA, Lucchinetti CF et al (2007) The spectrum of neuromyelitis optica. Lancet Neurol 6:805–815

  2. 2.

    Carnero Contentti E, De Virgiliis M, Hryb JP et al (2019) Aquaporin-4 serostatus and visual outcomes in clinically isolated acute optic neuritis. J Neuroophthalmol 39(2):165–169

  3. 3.

    Akman-Demir G, Tüzün E, Waters P et al (2011) Prognostic implications of aquaporin-4 antibody status in neuromyelitis optica patients. J Neurol 258(3):464–470

  4. 4.

    Wingerchuk D, Banwell B, Bennett J et al (2015) International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology 85:177–189

  5. 5.

    Lennon V, Wingerchuk D, Kryzer T et al (2004) A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 364:2106–2112

  6. 6.

    Sepulveda M, Armangue T, Sola-Valls N et al (2016) Neuromyelitis optica spectrum disorders. Comparison according to the phenotype and serostatus. Neurol Neuroimmunol Neuroinflamm 3:e225

  7. 7.

    Waters P, McKeon A, Leite MI et al (2012) Serological diagnosis of NMO: a multicenter comparison of aquaporin-4-IgG assays. Neurology 78:665–671

  8. 8.

    Kitley J, Woodhall M, Waters P et al (2012) Myelin-oligodendrocyte glycoprotein antibodies in adults with a neuromyelitis optica phenotype. Neurology 79:1273–1277

  9. 9.

    Hamid SHM, Whittam D, Mutch K et al (2017) What proportion of AQP4-IgG-negative NMO spectrum disorder patients are MOG-IgG positive? A cross sectional study of 132 patients. J Neurol 264:2088–2094

  10. 10.

    Palace J, Lin DY, Zeng D et al (2019) Outcome prediction models in AQP4-IgG positive neuromyelitis optica spectrum disorders. Brain 142:1310–1323

  11. 11.

    Kitley J, Leite MI, Nakashima I et al (2012) Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan. Brain 135:1834–1849

  12. 12.

    Collongues N, Marignier R, Zephir H et al (2010) Neuromyelitis optica in France: a multicenter study of 125 patients. Neurology 74:736–742

  13. 13.

    Nagaishi A, Takagi M, Umemura A et al (2011) Clinical features of neuromyelitis optica in a large Japanese cohort: comparison between phenotypes. J Neurol Neurosurg Psychiatry 82:1360–1364

  14. 14.

    Collongues N, Marignier R, Jacob A et al (2014) Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset. Mult Scler 20:1086–1094

  15. 15.

    Fragoso YD, Ruocco HH, Dias RM et al (2019) Late onset of neuromyelitis optica spectrum disorders. Neurol Ther. ahead of print)

  16. 16.

    Zhang LJ, Yang LN, Li T et al (2017) Distinctive characteristics of early-onset and late-onset neuromyelitis optica spectrum disorders. Int J Neurosci 127(4):334–338

  17. 17.

    Mao Z, Yin J, Zhong X et al (2015) Late-onset neuromyelitis optica spectrum disorder in AQP4-seropositive patients in a Chinese population. BMC Neurol 15:160

  18. 18.

    Seok JM, Cho HJ, Ahn SW et al (2017) Clinical characteristics of late-onset neuromyelitis optica spectrum. Mult Scler 23(13):1748–1756

  19. 19.

    Sepulveda M, Delgado-García G, Blanco Y et al (2019) Late-onset neuromyelitis optica spectrum disorder: the importance of autoantibody serostatus. Neurol Neuroimmunol Neuroinflamm 6(6):e607

  20. 20.

    Kurtzke JF (1983) Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology 33:1444–1452

  21. 21.

    Bedoya G, Montoya P, García J et al (2006) Admixture dynamics in Hispanics: a shift in the nuclear genetic ancestry of a South American population isolate. Proc Natl Acad Sci USA 103:7234–7239

  22. 22.

    Carnero Contentti E, Daccach Marques V, Soto de Castillo I et al (2018) Frequency of brain MRI abnormalities in neuromyelitis optica spectrum disorder at presentation: a cohort of Latin American patients. Mult Scler Relat Disord 19:73–78

  23. 23.

    Carnero Contentti E, Soto de Castillo I, Daccach Marques V et al (2018) Application of the 2015 diagnostic criteria for neuromyelitis optica spectrum disorders in a cohort of Latin American patients. Mult Scler Relat Disord 20:109–114

  24. 24.

    Mealy MA, Mossburg SE, Kim SH et al (2018) Long-term disability in neuromyelitis optica spectrum disorder with a history of myelitis is associated with age at onset, delay in diagnosis/preventive treatment, MRI lesion length and presence of symptomatic brain lesions. Mult Scler Relat Disord 28:64–68

  25. 25.

    Wingerchuk DM, Pittock SJ, Lucchinetti CF et al (2007) A secondary progressive clinical course is uncommon in neuromyelitis optica. Neurology 68:603–605

  26. 26.

    Krumbholz M, Hofstadt-van Oy U, Angstwurm K et al (2015) Very late-onset neuromyelitis optica spectrum disorder beyond the age of 75. J Neurol 262:1379–1384

  27. 27.

    Marrie RA (2019) Comorbidity in multiple sclerosis: past, present and future. Clin Investig Med 42(1):E5–E12

Download references

Author information

Correspondence to Edgar Carnero Contentti.

Ethics declarations

Conflicts of interest

None of the authors have any potential financial conflict of interest relating to this manuscript.

Ethical standards

This study was approved by the local ethics committee of each participating center.

Informed consent

Informed consent was obtained from all participants.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary file1 (DOCX 12 kb)

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Carnero Contentti, E., Daccach Marques, V., Soto de Castillo, I. et al. Clinical features and prognosis of late-onset neuromyelitis optica spectrum disorders in a Latin American cohort. J Neurol (2020) doi:10.1007/s00415-020-09699-2

Download citation


  • Late-onset NMOSD
  • Neuromyelitis optica spectrum disorder
  • Prognosis
  • Disability
  • Latin america