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Induction of Paralysis and Visual System Injury in Mice by T Cells Specific for Neuromyelitis Optica Autoantigen Aquaporin-4
Published on: August 21, 2017
Racial differences in neuromyelitis optica spectrum disorder
Su-Hyun Kim1, Maureen A Mealy1, Michael Levy1
1From 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.
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.
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