title: Neuromyelitis optica in Portugal (NEMIPORT) - A multicentre study.
abstract: BACKGROUND
  Neuromyelitis Optica (NMO) is an inflammatory demyelinating disease of the CNS. There have been few epidemiologic studies on NMO, none in Portugal.
  
  
  OBJECTIVE
  To 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.
  
  
  METHODS
  We performed an observational, retrospective, multicenter study in 5 Hospital Centers in Portugal.
  
  
  RESULTS
  Sixty-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.
  
  
  CONCLUSIONS
  This 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.
authors.affiliation: Centro Hospitalar do Porto - Hospital de Santo António, Department of Neurology, Largo Prof. Abel Salazar, 4099-001 Porto, Portugal. Electronic address: joanadomingos@hotmail.com.
authors.forename: Joana
authors.initials: J
authors.lastname: Domingos
authors.affiliation: Centro Hospitalar e Universitário de Coimbra, EPE, Department of Neurology, Avenida Bissaya Barreto, Praçeta Prof. Mota Pinto, 3000-075 Coimbra, Portugal. Electronic address: luis_isidoro@yahoo.com.
authors.forename: Luís
authors.initials: L
authors.lastname: Isidoro
authors.affiliation: Centro Hospitalar de São João, EPE, Department of Neuroradiology, Alameda Prof. Hernâni Monteiro, 4200-319 Porto, Portugal. Electronic address: ritafig7@gmail.com.
authors.forename: Rita
authors.initials: R
authors.lastname: Figueiredo
authors.affiliation: Centro Hospitalar de Setúbal, EPE, Department of Neurology, Rua Camilo Castelo Branco, 2910-446 Setúbal, Portugal. Electronic address: marisatbrum@gmail.com.
authors.forename: Marisa
authors.initials: M
authors.lastname: Brum
authors.affiliation: Centro Hospitalar de Lisboa Central, EPE, Department of Neurology, Alameda Santo António dos Capuchos, 1169-050 Lisboa, Portugal. Electronic address: cmc120@gmail.com.
authors.forename: Carlos
authors.initials: C
authors.lastname: Capela
authors.affiliation: Centro Hospitalar de Vila Nova de Gaia, EPE, Department of Neurology, Rua Conceição Fernandes, 4434-502 Vila Nova de Gaia, Portugal. Electronic address: pedrojgbarros@gmail.com.
authors.forename: Pedro
authors.initials: P
authors.lastname: Barros
authors.affiliation: Centro Hospitalar do Porto - Hospital de Santo António, Department of Neurology, Largo Prof. Abel Salazar, 4099-001 Porto, Portugal; UMIB, Instituto de Ciências Biomédicas Abel Salazar, Universidade do Porto, Porto, Portugal. Electronic address: ernestina.santos@gmail.com.
authors.forename: Ernestina
authors.initials: E
authors.lastname: Santos
authors.affiliation: Centro Hospitalar e Universitário de Coimbra, EPE, Department of Neurology, Avenida Bissaya Barreto, Praçeta Prof. Mota Pinto, 3000-075 Coimbra, Portugal. Electronic address: carmo.macario@gmail.com.
authors.forename: Maria do Carmo
authors.initials: Mdo C
authors.lastname: Macário
authors.affiliation: Centro Hospitalar de Setúbal, EPE, Department of Neurology, Rua Camilo Castelo Branco, 2910-446 Setúbal, Portugal. Electronic address: josefernandopintomarques@gmail.com.
authors.forename: José
authors.initials: J
authors.lastname: Pinto Marques
authors.affiliation: Centro Hospitalar de Lisboa Central, EPE, Department of Neurology, Alameda Santo António dos Capuchos, 1169-050 Lisboa, Portugal. Electronic address: rui.pedrosa@chlc.min-saude.pt.
authors.forename: Rui
authors.initials: R
authors.lastname: Pedrosa
authors.affiliation: Hospital Beatriz Ângelo, Department of Neurology, Avenida Carlos Teixeira, 3, 2674-514 Loures, Portugal. Electronic address: Josevale.neuro@gmail.com.
authors.forename: José
authors.initials: J
authors.lastname: Vale
authors.affiliation: Centro Hospitalar de São João, Department of Neurology, Alameda Prof. Hernâni Monteiro, 4200-319 Porto, Portugal; Faculty of Health Sciences, University Fernando Pessoa, Porto, Portugal. Electronic address: mjsa@med.up.pt.
authors.forename: Maria José
authors.initials: MJ
authors.lastname: Sá
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000453:epidemiology* / Q000276:immunology; D001921:Brain / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015897:Comorbidity; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009471:Neuromyelitis Optica / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology; D009902:Optic Neuritis / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology; D011174:Portugal / Q000453:epidemiology; D012189:Retrospective Studies; D017678:Sex Distribution; D055815:Young Adult
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D007074:Immunoglobulin G
keywords: AQP4; Aquaporin 4; Autoimmune disorders; NMO; Neuromyelitis optica