From the Department of Neurology (S.-H.K., H.-J.S., J.-W.H., H.J.K.) and Biometric Research Branch (M.H.), Research Institute and Hospital of National Cancer Center, Goyang, South Korea; Department of Neurology (M.A.M., M.L.), Johns Hopkins University School of Medicine, Baltimore, MD; NeuroCure Clinical Research Center (F.S., F.P.) and Department of Neurology (F.S., K.R., F.P.), Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health; Experimental and Clinical Research Center (F.S., F.P.), Max Delbrück Center for Molecular Medicine and Charité-Universitätsmedizin Berlin; Department of Neurology (M.R., O.A., H.-P.H.), Medical Faculty, Heinrich Heine University, Düsseldorf, Germany; Department of Neurology (N.A.), Slagelse Hospital and Institute of Regional Health Research & Molecular Medicine, University of Southern Denmark, Odense; Department of Neurology (J.L.T.-C.), Queen Elizabeth Hospital, Hong Kong, China; Department of Medicine (S.S., N.P.), Siriraj Hospital, Mahidol University, Bangkok, Thailand; and Nuffield Department of Clinical Neurosciences (M.I.L., J.P.), John Radcliffe Hospital, University of Oxford, UK.
Neurology. 2018 Nov 27;91(22):e2089-e2099. doi: 10.1212/WNL.0000000000006574. Epub 2018 Oct 26.
We aimed to evaluate racial differences in the clinical features of neuromyelitis optica spectrum disorder.
This retrospective review included 603 patients (304 Asian, 207 Caucasian, and 92 Afro-American/Afro-European), who were seropositive for anti-aquaporin-4 antibody, from 6 centers in Denmark, Germany, South Korea, United Kingdom, United States, and Thailand.
Median disease duration at last follow-up was 8 years (range 0.3-38.4 years). Asian and Afro-American/Afro-European patients had a younger onset age than Caucasian patients (mean 36, 33, and 44 years, respectively; < 0.001). During the disease course, Caucasian patients (23%) had a lower incidence of brain/brainstem involvement than Asian (42%) and Afro-American/Afro-European patients (38%) ( < 0.001). Severe attacks (visual acuity ≤0.1 in at least one eye or Expanded Disability Status Scale score ≥6.0 at nadir) at onset occurred more frequently in Afro-American/Afro-European (58%) than in Asian (46%) and Caucasian (38%) patients ( = 0.005). In the multivariable analysis, older age at onset, higher number of attacks before and after immunosuppressive treatment, but not race, were independent predictors of severe motor disabilities at last follow-up.
A review of a large international cohort revealed that race affected the clinical phenotype, age at onset, and severity of attacks, but the overall outcome was most dependent on early and effective immunosuppressive treatment.
评估视神经脊髓炎谱系疾病的临床特征中的种族差异。
本回顾性研究纳入了 6 个中心（丹麦、德国、韩国、英国、美国和泰国）的 603 例抗水通道蛋白 4 抗体阳性的患者（304 例亚洲人、207 例白种人、92 例非裔/非裔欧洲人）。
末次随访时的中位疾病病程为 8 年（范围 0.3-38.4 年）。亚洲和非裔/非裔欧洲患者的发病年龄均小于白种人（平均 36、33 和 44 岁；<0.001）。在疾病过程中，白种人（23%）脑/脑干受累的发生率低于亚洲人（42%）和非裔/非裔欧洲人（38%）（<0.001）。首发时即出现严重发作（至少一眼视力≤0.1 或扩展残疾状况量表评分≥6.0 时的最低值）的患者中非裔/非裔欧洲人（58%）多于亚洲人（46%）和白种人（38%）（=0.005）。多变量分析显示，发病年龄较大、免疫抑制治疗前后发作次数较多，但种族不是导致末次随访时严重运动残疾的独立预测因素。
对大型国际队列的回顾显示，种族影响疾病表型、发病年龄和发作严重程度，但总体结局主要取决于早期有效的免疫抑制治疗。
