Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study

Data de publicação: Data Ahead of Print:

Autores da FMUP

  • Pedro Miguel Paredes De Abreu

    Autor

  • Joana Da Cruz Guimarães Ferreira De Almeida

    Autor

  • Cláudia Raquel Ferrão De Melo

    Autor

  • João Pedro Melo Marques Pinho Ferreira

    Autor

Participantes de fora da FMUP

  • Santos, E
  • Rocha, AL
  • Oliveira, V
  • Ferro, D
  • Samo, R
  • Sousa, AP
  • Figueiroa, S
  • Mendonça, T
  • Sousa, R
  • Correia, I
  • Duraes, J
  • Sousa, L
  • de Sá, J
  • Sousa, F
  • Sequeira, M
  • Correia, AS
  • André, AL
  • Basilio, C
  • Arenga, M
  • Mendes, I
  • Marques, IB
  • Perdigao, S
  • Felgueiras, H
  • Alves, I
  • Correia, F
  • Barroso, C
  • Morganho, A
  • Carmona, C
  • Palavra, F
  • Santos, M
  • Salgado, V
  • Palos, A
  • Nzwalo, H
  • Timóteo, A
  • Guerreiro, R
  • Isidoro, L
  • Boleixa, D
  • Carneiro, P
  • Neves, E
  • Silva, AM
  • Gonçalves, G
  • Leite, MI
  • Sá, MJ

Unidades de investigação

Abstract

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is a rare disorder in which astrocyte damage and/or demyelination often cause severe neurological deficits. Objective: To identify Portuguese patients with NMOSD and assess their epidemiological/clinical characteristics. Methods: This was a nationwide multicenter study. Twenty-four Portuguese adult and 3 neuropediatric centers following NMOSD patients were included. Results: A total of 180 patients met the 2015 Wingerchuk NMOSD criteria, 77 were AQP4-antibody positive (Abs+), 67 MOG-Abs+, and 36 seronegative. Point prevalence on December 31, 2018 was 1.71/100,000 for NMOSD, 0.71/100,000 for AQP4-Abs+, 0.65/100,000 for MOG-Abs+, and 0.35/100,000 for seronegative NMOSD. A total of 44 new NMOSD cases were identified during the two-year study period (11 AQP4-Abs+, 27 MOG-Abs+, and 6 seronegative). The annual incidence rate in that period was 0.21/100,000 person-years for NMOSD, 0.05/100,000 for AQP4-Abs+, 0.13/100,000 for MOG-Abs+, and 0.03/100,000 for seronegative NMOSD. AQP4-Abs+ predominated in females and was associated with autoimmune disorders. Frequently presented with myelitis. Area postrema syndrome was exclusive of this subtype, and associated with higher morbidity/mortality than other forms of NMOSD. MOG-Ab+ more often presented with optic neuritis, required less immunosuppression, and had better outcome. Conclusion: Epidemiological/clinical NMOSD profiles in the Portuguese population are similar to other European countries

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: 6

Citações Recebidas na Scopus: 13

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Keywords

  • Clinical; Epidemiological; Neuromyelitis optica spectrum disorders; Portugal

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