Seroprevalence of MOG and AQP4 Antibodies and Outcomes in an Indian Cohort of Pediatric Acquired Demyelinating Syndromes

Abstract

This study aimed to determine the seropositivity of myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) and aquaporin-4 antibodies (AQP4-Ab) and outcomes in children with acquired demyelinating syndromes (ADS). Children (6months-15years) with suspected ADS were enrolled and tested for MOG-Ab and AQP4-Ab prospectively over 18 months at a tertiary-care hospital in North India. Children with proven non-immune mediated neurological disorders were enrolled as controls. Of 79 children with suspected ADS, 66 were enrolled. Among the enrolled children with ADS, ADEM (25) was the commonest first clinical event followed by ON (20) and TM [19; one child had ON and TM simultaneously (NMOSD)], while two children had CIS apart from ON and TM. Fourteen (21.2%, CI 11.3-31.1) tested positive for one antibody [12 (18.1%; 95% CI 10.5-25.5%) for MOG-Ab and two (3%; 95% CI 0-7.2%) for AQP4-Ab]. None of the 62 controls tested positive for any antibody. The final diagnosis in those with the monophasic ADS was ADEM (21), ON (13), TM (16), and other CIS (1) while that in children with recurrent events was MDEM (2), NMOSD (3), ADEM-ON (4), recurrent ON (4), and MS (2). Among those with the first event, 4/51 (7.8%; 95%CI 0.5-15.2%) were MOG-Ab positive and two AQP4-Ab positive, whereas, 8/15 [53.3%, (95%CI: 28.1-78.6%)] with recurrent events [MDEM (2), ADEM-ON (4), recurrent ON (1), and recurrent TM (1)] were MOG-Ab positive. Hence, MOG-Ab are the most common antibodies detected in one in five children with pediatric ADS, especially in relapsing disease. AQP4-Ab are rare in children with ADS.

Clinico-radiologic Spectrum and Outcome of Pediatric Acquired Demyelinating Disorders of Central Nervous System: A Retrospective Indian Tertiary Care Hospital Cohort

“…10 But other studies have reported optic neuritis to be common. 16,24,31 We observed female predominance as noted earlier. 12,17,23 However, EU consortium reported equal gender distribution in MOGAD with a slight female preponderance in older children.…”

“…We found MOG-IgG seropositivity of 62% which is comparable to paediatric cohorts 3,10,23 but higher compared to a study by Sankhyan et al from North India. 16 Most of the children with NMOSD had seronegative/unknown status for AQP4 IgG but neuroimaging features helped in the diagnosis. However, most of these children were diagnosed before testing for serum MOG antibodies became available hence, we cannot rule out the possibility of few of these being MOGAD.…”

“…Proportion with abnormal CSF (46.7%) was comparable to other studies reporting CSF abnormalities in ADS. 15,16,24 Neuroimaging plays a crucial role in diagnosing ADS and further phenotyping them. Brain MRI showing T2 and FLAIR hyperintense lesions involving bilateral subcortical white matter, optic nerve, middle cerebellar peduncle, basal ganglia/thalamus were observed in MOGAD and ADEM, which has been reported.…”

“…Majority showed good recovery (91.4%) at discharge as comparable to other studies. 15,16,25 Twelve (17.1%) relapsed after first episode of NMOSD, MOGAD and MS. Recent studies have suggested second line immunotherapy with agents like rituximab and steroid sparing agents like azathioprine are being used in those who are refractory to treatment and in relapses.…”

“…1,3,10,12 However, studies from the Indian subcontinent describing the spectrum and outcome of ADS subtypes are scarce. [15][16][17] Despite the variable presentation and radiologic lesions, treatment outcomes were generally observed to be favorable with few relapses. This prompted the retrospective chart review in the backdrop of evolving biomarkers and classification of ADS categories.…”