Author(s):
Santos, E ; Rocha, AL ; Oliveira, V ; Ferro, D ; Samões, R ; Sousa, P ; Figueiroa, S ; Mendonça, T ; Abreu, P ; Guimarães, J ; Sousa, R ; Melo, C ; Correia, I ; Durães, J ; Sousa, L ; Ferreira, J ; de Sá, J ; Sousa, F ; Sequeira, M ; Correia, AS ; André, AL ; Basílio, C ; Arenga, M ; Mendes, I ; Marques, IB ; Perdigão, 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
Date: 2021
Persistent ID: http://hdl.handle.net/10400.26/46536
Origin: Hospital de Cascais
Subject(s): Neuromielite Óptica/diagnóstico; Neuromielite Óptica/epidemiologia; Neuromyelitis Optica /diagnosis; Neuromyelitis Optica/epidemiology
Description
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.
