Late onset neuromyelitis optica spectrum disorders (LONMOSD) from a nationwide Portuguese study: Anti-AQP4 positive, anti-MOG positive and seronegative subgroups

Data de publicação: Data Ahead of Print:

Autores da FMUP

  • Pedro Miguel Paredes De Abreu

    Autor

  • Joana Da Cruz Guimarães Ferreira De Almeida

    Autor

  • João Pedro Melo Marques Pinho Ferreira

    Autor

Participantes de fora da FMUP

  • Santos, E
  • Moura, J
  • Samoes, R
  • Sousa, AP
  • Mendonça, T
  • Correia, I
  • Duraes, J
  • Sousa, L
  • de Sá, J
  • Sousa, F
  • Sequeira, M
  • Correia, AS
  • André, AL
  • Basílio, C
  • Arenga, M
  • Marques, IB
  • Perdigao, S
  • Alves, I
  • Santos, M
  • Salgado, V
  • Palos, A
  • Guerreiro, R
  • Isidoro, L
  • Boleixa, D
  • Carneiro, P
  • Neves, E
  • Silva, AM
  • Gonçalves, G
  • Sá, MJ

Unidades de investigação

Abstract

Introduction: Several neuroimmunological disorders have distinct phenotypes according to the age of onset, as in multiple sclerosis or myasthenia gravis. It is also described that late onset NMOSD (LONMOSD) has a different phenotype. Objective: To describe the clinical/demographic characteristics of the LONMOSD and distinguish them from those with early onset (EONMOSD). Methods: From a nationwide Portuguese NMOSD study we analyzed the clinical/demographic characteristics of the LONMOSD. Results: From the 180 Portuguese patients 45 had disease onset after 50 years old, 80% were female. 23 had anti-AQP4 antibodies (51.1%), 13 anti-MOG antibodies (28.9%) and 9 were double seronegative (20.0%). The most common presenting phenotypes in LONMOSD were transverse myelitis (53.3%) and optic neuritis (26.7%), without difference from EONMOSD (p = 0.074). The mean EDSS for LONMOSD was 6.0 (SD=2.8), after a mean follow-up time of 4.58 (SD=4.47) years, which was significantly greater than the mean EDSS of EONMOSD (3.25, SD=1.80)(p = 0.022). Anti-AQP4 antibodies positive LONMOSD patients had increased disability compared to anti-MOG antibodies positive LONMOSD (p = 0.022). The survival analysis showed a reduced time to use a cane for LONMOSD, irrespective of serostatus (p<0.001). Conclusions: LONMOSD has increased disability and faster progression, despite no differences in the presenting clinical phenotype were seen in our cohort.

Dados da publicação

ISSN/ISSNe:
2211-0356, 2211-0348

Multiple Sclerosis and Related Disorders  Elsevier

Tipo:
Article
Páginas:
-
Link para outro recurso:
www.scopus.com

Citações Recebidas na Web of Science: 3

Citações Recebidas na Scopus: 7

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Keywords

  • Aquaporin 4; Autoantibodies; Female; Humans; Male; Myelitis, Transverse; Neuromyelitis Optica; Portugal; aquaporin 4 antibody; azathioprine; immunoglobulin; methotrexate; mycophenolate mofetil; myelin oligodendrocyte glycoprotein antibody; protein antibody; rituximab; steroid; unclassified drug; aquaporin 4; autoantibody; adult; aged; Article; clinical feature; cohort analysis; controlled study; demographics; disability; disease exacerbation; female; follow up; human; major clinical study; male; middle aged; myelooptic neuropathy; optic neuritis; phenotype; plasma exchange; Portuguese (citizen); retrospective study; survival analysis; transverse myelitis; very elderly; myelitis; myelooptic neuropathy; Portugal

Financiamento

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