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Neurology. 2018 Nov 27;91(22):e2089-e2099. doi: 10.1212/WNL.0000000000006574. Epub 2018 Oct 26.

Racial differences in neuromyelitis optica spectrum disorder.

Author information

1
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.
2
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. hojinkim@ncc.re.kr.

Abstract

OBJECTIVE:

We aimed to evaluate racial differences in the clinical features of neuromyelitis optica spectrum disorder.

METHODS:

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.

RESULTS:

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; p < 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%) (p < 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 (p = 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.

CONCLUSION:

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.

PMID:
30366977
PMCID:
PMC6282238
[Available on 2019-11-27]
DOI:
10.1212/WNL.0000000000006574

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