Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative Subgroups

• Autor(a) principal: Santos, E
• Data de Publicação: 2022
• Outros Autores: Moura, J, Samões, R, Sousa, AP, Mendonça, T, Abreu, P, Guimarães, J, Correia, I, Durães, J, Sousa, L, Ferreira, J, de Sá, J, Sousa, F, Sequeira, M, Correia, AS, André, AL, Basílio, C, Arenga, M, Brás Marques, I, Perdigão, S, Alves, I, Santos, M, Salgado, V, Palos, A, Guerreiro, R, Isidoro, L, Boleixa, D, Carneiro, P, Neves, E, Martins Silva, A, Gonçalves, G, Sá, MJ
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://hdl.handle.net/10400.17/4413
• topic: CHLC NEU; Aquaporin 4; Humans; Male; Female; Autoantibodies; Myelitis, Transverse*; Neuromyelitis Optica* / epidemiology; Portugal / epidemiology

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.