Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study

Autores da FMUP
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
- Tipo:
- Article
- Páginas:
- -
- PubMed:
- 34583213
- Link para outro recurso:
- www.scopus.com
Multiple Sclerosis and Related Disorders Elsevier
Citações Recebidas na Web of Science: 6
Citações Recebidas na Scopus: 13
Documentos
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Filiações
Keywords
- Clinical; Epidemiological; Neuromyelitis optica spectrum disorders; Portugal
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