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The Prevalence, Demographics, Clinical Features, Neuroimaging, and Inter-ethnic Differences of MOGAD in Malaysia with Global Perspectives

Published:September 08, 2022DOI:https://doi.org/10.1016/j.msard.2022.104168

      Highlights

      • A proportion of CNS IIDD patients fulfilling 2015 NMOSD panel criteria for Anti AQP4 antibody seronegative NMOSD or limited forms of relapsing/monophasic optic neuritis,transverse myelitis, isolated brain syndromes or ADEM seronegative for Anti AQP4 antibody are MOG positive.
      • Malaysian MOGAD has shown an ethnic predisposition towards the Indian population.
      • Malaysian Chinese MOGAD patients have a more severe disease course.
      • Malaysian MOGAD has a similar disease profile to international MOGAD positive patients highlighting the role of shared immunopathogenetic mechanisms.
      • Prolonged steroid maintenance is essential in preventing relapses in a proportion of MOG positive patients.
      • Long lag exists between index event in MOGAD and the second attack in some patients.
      • There is an urgent need for biomarker investment and accessibility within the Southeast Asian region to identify and characterize the disease.

      Abstract

      Introduction

      CNS IIDDs
      Central Nervous System Idiopathic Inflammatory Demyelinating Diseases
      1Central Nervous System Idiopathic Inflammatory Demyelinating Diseases
      tested positive for anti-MOG
      Myelin Oligodendrocyte Glycoprotein
      2Myelin Oligodendrocyte Glycoprotein
      are known to have a distinct clinical profile with a better overall prognosis.

      Objectives

      We aim to determine the prevalence, demographic and clinical characteristics of MOG antibody disease (MOGAD) specifically identifying any ethnic variations unique to our local population, with global perspectives.

      Methods

      This is a cross-sectional study conducted at the Neurology Department, Kuala Lumpur Hospital from January 2018 to January 2021. Out of 750 CNS IIDDs, seventy-eight consecutive anti-AQP4 antibody negative NMOSD/high risk undifferentiated relapsing or monophasic CNSIIDD subjects were tested for anti-MOG.

      Results

      Anti-MOG was positive in thirty six out of seventy-eight (%)(46.1%) seronegative patients. The prevalence of MOGAD in our Malaysian population is 0.12 per 100,000 persons with less marked female preponderance of 2:1 and younger age at onset of 23.8 ± 14.4 years. Despite a predominantly ethnic Malay population, a high proportion of our MOGAD patients were Indian (Proportion of Malay:Chinese:Indian:others; 16:9:10:1, prevalence 0.5 per 100,000 population for Indians) with favourable disease course in the most with minor exceptions. Monophasic and relapsing disease course was seen in 11.2% and 88.8% of patients respectively. However, fulminant aggressive disease can occur especially amongst the Chinese and paediatric cohorts. Optic neuritis, NMOSD and ADEM were the commonest presentations at onset and first relapse. EDSS at diagnosis, first relapse, and last follow-up were 4.5±2.5, 3±2.0, and 1.75(range 1-3). Neuroimaging showed large, fluffy, PRES- like supratentorial cortical, periventricular deep white matter ,diencephalon lesions,enhancing anterior optic nerve with or without chiasmal sparring lesions and cervical/cervicothoracic involvement. Area post rema lesions were rare. Threshold steroid levels exist relapsing on withdrawal some fulminantly requiring Immunosuppressants(rituximab) and intravenous immunoglobulins to maintain remission.

      Conclusion

      Malaysian MOGAD profile was similar to its international descriptions of the disease with ethnic selectivity for Indians. Prolonged steroid maintenance is essential to prevent relapses. Fulminant aggressive cases of MOGAD especially amongst Paediatric patients and the Chinese cohort have been reported.

      Keywords

      Abbreviations:

      NMOSD (Neuromyelitis optica spectrum disorder), CNSIIDDs (Central nervous system idiopathic inflammatory demyelinating diseases), MOGAD (Myelin Oligodendrocyte glycoprotein associated disorders), CSF (cerebrospinal fluids), EDSS (Expanded disability status scale), PRES (Posterior reversible encephalopathy syndrome), IVIG (Intravenous immunoglobulins), ADEM (Acute disseminated encephalomyelitis)
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