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Late onset neuromyelitis optica spectrum disorders (LONMOSD) from a nationwide Portuguese study: Anti-AQP4 positive, anti-MOG positive and seronegative subgroups

2022; Elsevier BV; Volume: 63; Linguagem: Inglês

10.1016/j.msard.2022.103845

2211-0356

Ernestina Santos, João Moura, Raquel Samões, Ana Paula Sousa, Teresa Mendonça, Pedro Abreu, Joana Guimarães, Inês Correia, João Durães, Lívia Sousa, João Ferreira, João de Sá, Filipa Sousa, Marta Sequeira, Ana Sofia Correia, Ana André, Carlos Basílio, Marta Arenga, Inês Brás Marques, Sandra Perdigão, Ivânia Alves, Mariana Santos, Vasco Salgado, Adelaide Palos, Rui Guerreiro, Luís Isidoro, Daniela Boleixa, Paula Carneiro, Esmeralda Neves, Ana Silva, Guilherme Gonçalves, María José Sá,

Protein Tyrosine Phosphatases

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.To describe the clinical/demographic characteristics of the LONMOSD and distinguish them from those with early onset (EONMOSD).From a nationwide Portuguese NMOSD study we analyzed the clinical/demographic characteristics of the LONMOSD.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).LONMOSD has increased disability and faster progression, despite no differences in the presenting clinical phenotype were seen in our cohort.