Author(s):
Santos, E ; 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
Date: 2022
Persistent ID: http://hdl.handle.net/10400.17/4413
Origin: Repositório do Centro Hospitalar de Lisboa Central, EPE
Subject(s): CHLC NEU; Aquaporin 4; Humans; Male; Female; Autoantibodies; Myelitis, Transverse*; Neuromyelitis Optica* / epidemiology; Portugal / epidemiology
Description
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.
