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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.
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.
摘要：
背景：视神经脊髓炎（NMO）是CNS的炎性脱髓鞘疾病。关于NMO的流行病学研究很少，在葡萄牙没有。
目的：分析临床，一组符合Wingerchuk2006NMO/NMOSD标准的葡萄牙患者的生物学和MRI特征.鉴定和表征那些患有伴随自身免疫性疾病或循环自身抗体的人。
方法：我们进行了观察，回顾性,葡萄牙5个医院中心的多中心研究。
结果：67例患者符合纳入标准。他们主要是白种人,55女发病年龄中位数为32.0岁，平均随访7.4±6.0年。21例患者以明确的NMO和视神经炎（ON）为最常见的初始表现。46例被归类为NMO谱系障碍。主要亚型为复发性ON和单发纵向广泛性横贯性脊髓炎。24例患者AQP4-IgG阳性。23人具有其他循环自身抗体。67例患者中有15例合并有自身免疫性疾病。自身免疫性疾病的存在与AQP4-IgG的阳性之间存在显着相关性。五名病人死亡,所有明确的NMO。
结论：这是葡萄牙关于这种罕见疾病的第一项研究。人口统计学特征与其他研究相似。伴随自身免疫性疾病的存在与AQP4-IgG的血清阳性显着相关。
目的：分析临床，一组符合Wingerchuk2006NMO/NMOSD标准的葡萄牙患者的生物学和MRI特征.鉴定和表征那些患有伴随自身免疫性疾病或循环自身抗体的人。
方法：我们进行了观察，回顾性,葡萄牙5个医院中心的多中心研究。
结果：67例患者符合纳入标准。他们主要是白种人,55女发病年龄中位数为32.0岁，平均随访7.4±6.0年。21例患者以明确的NMO和视神经炎（ON）为最常见的初始表现。46例被归类为NMO谱系障碍。主要亚型为复发性ON和单发纵向广泛性横贯性脊髓炎。24例患者AQP4-IgG阳性。23人具有其他循环自身抗体。67例患者中有15例合并有自身免疫性疾病。自身免疫性疾病的存在与AQP4-IgG的阳性之间存在显着相关性。五名病人死亡,所有明确的NMO。
结论：这是葡萄牙关于这种罕见疾病的第一项研究。人口统计学特征与其他研究相似。伴随自身免疫性疾病的存在与AQP4-IgG的血清阳性显着相关。
