Clinical neurology and neurosurgery · 2015
Neuromyelitis optica in Portugal (NEMIPORT) - A multicentre study.
СтатьяМногоцентровое исследованиеНаблюдательное исследованиеПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Domingos Joana, Isidoro Luís, Figueiredo Rita, Brum Marisa, Capela Carlos, Barros Pedro, Santos Ernestina, Macário Maria do Carmo, Pinto Marques José, Pedrosa Rui, Vale José, Sá Maria José
Аннотация
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.
CONCLUSION: 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.
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.
CONCLUSION: 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.
Предметные рубрики MeSH
AdolescentAdultAge of OnsetAgedAquaporin 4AutoantibodiesAutoimmune DiseasesBrainChildChild, PreschoolCohort StudiesComorbidityFemaleFollow-Up StudiesHumansImmunoglobulin GMagnetic Resonance ImagingMaleMiddle AgedNeuromyelitis OpticaOptic NeuritisPortugalRetrospective StudiesSex DistributionYoung Adult