Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study

• Main Author: Santos, E
• Publication Date: 2021
• Other Authors: Rocha, AL, Oliveira, V, Ferro, D, Samões, R, Sousa, AP, 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
• Format: Article
• Language: eng
• Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
• Download full: http://hdl.handle.net/10400.26/37588
• topic: Neuromielite Óptica/diagnóstico; Neuromielite Óptica/epidemiology; Neuromyelitis Optica /diagnosis; Neuromyelitis Optica/epidemiology

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