The Prevalence and Clinical Phenotype of Dual Positive Neuromyelitis Optica Spectrum Disorders (NMOSD) at a National Reference Centre. (5th December 2022)

• Record Type: Journal Article
• Authors: Lalwani, Chirag; Faisal, Fida; Yadav, Anjali; Kannoth, Sudheeran; Nambiar, Vivek; Gopinath, Sibi; Kumar, Anand; Saraf, Udit; Vincent, Jino; Anoop, Sruthi; Mathai, Annamma; Panicker, Suprabha
• Is Part Of: Neurology. Volume 99:Number 23(2022)Supplement 2
• Page Start: S1
• Page End: S1
• Publication Date: 2022-12-05
• Subjects: Neurology -- Periodicals; Neurology -- Periodicals; Neurologie -- Périodiques; 616.8
• DOI: 10.1212/01.wnl.0000903752.47975.59
• Languages: English
• ISSNs: 0028-3878

Abstract

Abstract : Objective: To assess the prevalence of dual positive NMOSD and outline its clinical phenotype. 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. 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 (3.36%; 57 females and 8 males) tested positive for AQP4-IgG, 217 patients (11.23%; 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 and right-sided facial deviation. 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.