Neuromyelitis optica in Portugal (NEMIPORT) – A multicentre study

• Observational, retrospective, multicenter study in 5 Hospital Centers in Portugal.
• Inclusion criteria Wingerchuk 2006: 67 patients included (21 definite NMO; 46 NMOSD).
• Demographic features were similar to other studies.
• Concomitant autoimmune disease was significantly associated with AQP4-IgG positivity.

BackgroundNeuromyelitis Optica (NMO) is an inflammatory demyelinating disease of the CNS. There have been few epidemiologic studies on NMO, none in Portugal.ObjectiveTo analyze the clinical, biological and MRI characteristics from a cohort of Portuguese patients who fulfilled the Wingerchuk 2006 NMO/NMOSD criteria. To identify and characterize those who had concomitant autoimmune disease or circulating autoantibodies.MethodsWe performed an observational, retrospective, multicenter study in 5 Hospital Centers in Portugal.ResultsSixty-seven patients fulfilled the inclusion criteria. They were mainly Caucasian, 55 female. Median age at onset was 32.0 years and mean follow-up 7.4 ± 6.0 years. Twenty-one patients were definite NMO and optic neuritis (ON) the most frequent initial presentation. Forty-six were classified as NMO spectrum disorders. The main subtypes were recurrent ON and single longitudinally extensive transverse myelitis. Twenty-four patients had positive AQP4-IgG. Twenty-three had other circulating autoantibodies. Fifteen out of 67 patients had concomitant autoimmune disease. There was a significant correlation between the presence of autoimmune disease and the positivity for AQP4-IgG. Five patients died, all definite NMO.ConclusionThis is the first study about this rare disease in Portugal. Demographic features were similar to other studies. The existence of concomitant autoimmune disease was significantly associated with seropositivity for AQP4-IgG.