Skip to main content
SpringerLink
Log in

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

  • Original Communication
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

Background

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).

Methods

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.

Results

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).

Conclusion

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 via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

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

    Article  CAS  Google Scholar 

  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

    Article  Google Scholar 

  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

    Article  CAS  Google Scholar 

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

    Article  Google Scholar 

  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

    Article  CAS  Google Scholar 

  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

    Article  Google Scholar 

  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

    Article  CAS  Google Scholar 

  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

    Article  CAS  Google Scholar 

  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

    Article  CAS  Google Scholar 

  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

    Article  Google Scholar 

  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

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

  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

    Article  Google Scholar 

  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

    Article  CAS  Google Scholar 

  15. Fragoso YD, Ruocco HH, Dias RM et al (2019) Late onset of neuromyelitis optica spectrum disorders. Neurol Ther. https://doi.org/10.1007/s40120-019-0143-2(Epub ahead of print)

    Article  PubMed  PubMed Central  Google Scholar 

  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

    Article  Google Scholar 

  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

    Article  Google Scholar 

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

    Article  Google Scholar 

  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

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

  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

    Article  CAS  Google Scholar 

  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

    Article  Google Scholar 

  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

    Article  CAS  Google Scholar 

  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

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

  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

    Article  Google Scholar 

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

    Article  Google Scholar 

Download references

Funding

This research did not receive any specific grant.

Author information

Authors and Affiliations

  1. Neuroimmunology Unit, Department of Neuroscience, Hospital Alemán, Av Pueyrredón 1640, C1118AAT, Buenos Aires, Argentina

    Edgar Carnero Contentti, Pablo A. López, Juan Pablo Pettinicchi & Alejandro Caride

  2. Department of Neurosciences and Behavioral Sciences, Hospital das Clínicas, Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil

    Vanessa Daccach Marques, Gabriel Braga Diégues Serva & Antonio Carlos dos Santos

  3. Neurology Department, Hospital Universitario de Maracaibo, Maracaibo, Bolivarian Republic of Venezuela

    Ibis Soto de Castillo, Maria C. Castillo & Omaira Molina

  4. Seccion de Neuroinmunologia y Enfermedades Desmielinizantes, Servicio de Neurología, Hospital de Clínicas José de San Martin, Buenos Aires, Argentina

    Verónica Tkachuk, Bustos Ariel, Ana Mariel Finkelsteyn & Vanesa Toneguzzo

  5. Centro de Esclerosis Múltiple de Buenos Aires, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina

    Edgardo Cristiano, Liliana Patrucco & Juan Ignacio Rojas

Authors
  1. Edgar Carnero Contentti
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Vanessa Daccach Marques
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ibis Soto de Castillo
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Verónica Tkachuk
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Bustos Ariel
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Maria C. Castillo
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Edgardo Cristiano
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Gabriel Braga Diégues Serva
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Antonio Carlos dos Santos
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Ana Mariel Finkelsteyn
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Pablo A. López
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Liliana Patrucco
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Omaira Molina
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Juan Pablo Pettinicchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Vanesa Toneguzzo
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. Alejandro Caride
    View author publications

    You can also search for this author in PubMed Google Scholar

  17. Juan Ignacio Rojas
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

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

Check for updates. 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 267, 1260–1268 (2020). https://doi.org/10.1007/s00415-020-09699-2

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-020-09699-2

Keywords

Search

Navigation