Advertisement
Research Article| Volume 75, 104736, July 2023

The prevalence and clinical phenotype of dual seropositive neuromyelitis optica spectrum disorders at a national reference center in South Asia

  • Chirag Sunil Lalwani
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author
  • Fida Faisal
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author
  • Anjali Yadav
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author
  • Sudheeran Kannoth
    Correspondence
    Corresponding author at: Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India.
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India

    NeuroImmunology Laboratory, Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author
  • Vivek Nambiar
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author
  • Sibi Gopinath
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author
  • Anand Kumar
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author
  • Saraf Udit Umesh
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author
  • Jino Vincent
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author
  • Sruthi Anoop
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author
  • Annamma Mathai
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India

    NeuroImmunology Laboratory, Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author
  • Suprabha Panicker
    Affiliations
    Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India

    NeuroImmunology Laboratory, Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India
    Search for articles by this author

      Highlights

      • Exceptionally, NMO and MOG-IgGs do co-exist.
      • In our study population of 1935 patients, dual positivity was recorded in 3.
      • The prevalence of MOG-IgG was substantially higher than NMO-IgG in our study.

      Abstract

      Background

      Neuromyelitis Optica Spectrum Disorders (NMOSD) is an autoimmune syndrome that is frequently positive for Aquaporin 4 (AQP4) IgG or Myelin Oligodendrocyte Glycoproteins (MOG) IgG. However, dual positivity to both is rare.

      Objective

      To assess the prevalence of dual-positive NMOSD and outline its clinical phenotype.

      Design/Methods

      This is a retrospective cross-sectional study conducted at a tertiary healthcare center in South Asia between August 2018 and November 2021. The serum and/or CSF samples of suspected cases of NMOSD were tested for both AQP4-IgG and MOG-IgG using an Indirect immunofluorescence test on transfected cells.

      Results

      During the study period, 1935 cases of NMOSD were tested for both antibodies- 65 patients (3.35%; 57 females and 8 males) tested positive for AQP4-IgG, 217 patients (11.21%; 122 females and 95 males) tested positive for MOG-IgG and 3 patients (0.15%; 2 females and 1 male) showed dual positivity. There was a strong female preponderance in all three groups (87.69%, 56.22%, and 66.66% respectively).
      This study identified 3 patients with dual positivity. The first patient (42 years, Male) presented with area postrema syndrome initially and subsequently relapsed by developing right-sided numbness of the temporal area and limbs during which he tested dual positive. The second patient (27 years, Female) presented with bilateral optic neuritis (left>right) initially and subsequently relapsed following an episode of a seizure with left-sided hemiplegia. The third patient (25 years, Female) initially presented with acute bilateral optic neuritis and later developed left-sided hemiplegia post-recovery at which point she tested dual positive.
      Management using methylprednisolone was ineffective for all three patients, however, plasmapheresis and/or periodic rituximab injections produced an excellent response.

      Conclusions

      Our study reports that the prevalence of dual-positive NMOSD is 0.15% and its clinical phenotype is more similar to NMO rather than MOG- associated disease.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Multiple Sclerosis and Related Disorders
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Ghezzi A.
        • Bergamaschi R.
        • Martinelli V.
        • Trojano M.
        • Tola M.R.
        • Merelli E.
        • et al.
        Clinical characteristics, course and prognosis of relapsing Devic's Neuromyelitis Optica.
        J. Neurol. 2004; 251: 47-52
        • Gospe S.M.
        • Chen J.J.
        • Bhatti MT.
        Neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein associated disorder-optic neuritis: a comprehensive review of diagnosis and treatment.
        Eye. 2021; 35: 753-768
        • Hor J.Y.
        • Asgari N.
        • Nakashima I.
        • Broadley S.A.
        • Leite M.I.
        • Kissani N.
        • et al.
        Epidemiology of neuromyelitis optica spectrum disorder and its prevalence and incidence worldwide.
        Front. Neurol. 2020; 11: 501
        • Jurynczyk M.
        • Messina S.
        • Woodhall M.R.
        • Raza N.
        • Everett R.
        • Roca-Fernandez A.
        • et al.
        Clinical presentation and prognosis in MOG-antibody disease: a UK study.
        Brain. 2017; 140: 3128-3138
        • Kunchok A.
        • Chen J.J.
        • McKeon A.
        • Mills J.R.
        • Flanagan E.P.
        • Pittock SJ.
        Coexistence of myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies in adult and pediatric patients.
        JAMA Neurol. 2020; 77 (Feb 1): 257-259
        • Marchionatti A.
        • Hansel G.
        • Avila G.U.
        • Sato DK.
        Detection of MOG-IgG in clinical samples by live cell-based assays: performance of immunofluorescence microscopy and flow cytometry.
        Front. Immunol. 2021; : 1599
        • O'Connell K.
        • Hamilton-Shield A.
        • Woodhall M.
        • Messina S.
        • Mariano R.
        • Waters P.
        • et al.
        Prevalence and incidence of neuromyelitis optica spectrum disorder, aquaporin-4 antibody-positive NMOSD and MOG antibody-positive disease in Oxfordshire, UK.
        J. Neurol. Neurosurg. Psychiatry. 2020; 91: 1126-1128
        • Pandit L.
        • Kundapur R.
        Prevalence and patterns of demyelinating central nervous system disorders in urban Mangalore, South India.
        Mult. Scler. J. 2014; 20: 1651-1653
        • Pittock S.J.
        • Lucchinetti CF.
        Neuromyelitis optica and the evolving spectrum of autoimmune aquaporin-4 channelopathies: a decade later.
        Ann. N. Y. Acad. Sci. 2016; 1366: 20-39
        • Quek A.M.L.
        • McKeon A.
        • Lennon V.A.
        • Mandrekar J.N.
        • Iorio R.
        • Jiao Y.
        • et al.
        Effects of age and sex on aquaporin-4 autoimmunity.
        Arch Neurol. 2012 Aug; 69: 1039-1043