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

Abstract

Paediatric acquired demyelinating syndrome(ADS) constitute group of treatable disorders with acute neurologic dysfunction. Neuroimaging has played significant role in diagnosis of ADS. We describe clinico-radiologic spectrum, outcome and compare the groups Acute disseminated encephalomyelitis (ADEM), Neuromyelitis-optica-spectrum disorder (NMOSD), clinically-isolated-syndrome (CIS), Multiple sclerosis (MS) and Myelin-oligodendrocyte-glycoprotein antibody associated disorders (MOGAD). Methods: Retrospective review of 70 children with ADS at tertiary-care hospital over 15 years (2008 to 2023) done. Diagnosis assigned as per International Paediatric Multiple Sclerosis Study Group criteria (IPMSSG)2016. Fisher-exact, chi-square test was applied. Results: 39 boys,31 girls aged 8.2±4.0 years with CIS (n=27), ADEM (n=16), NMOSD (n=13), MS (n=1) and MOGAD (n=13) were included. Clinical syndromes with positive significant association included polyfocal symptoms, encephalopathy in ADEM, optic neuritis in MOGAD, brainstem, area postrema syndrome in NMOSD. MOGAD presented with atypical presentations like prolonged fever (76.9%) and aseptic meningitis (23%). Seropositivity for MOG-IgG was 62%, NMO-IgG 2.6%. Neuroimaging of MOGAD showed lesions predominantly in basal ganglia/thalami (69.2%), optic nerve (46.2%) and cerebellum (46.2%). Imaging patterns between ADEM and MOGAD were comparable except for more ON (p=0.004), spinal cord (p=0.01) and cerebellar lesions (p=0.03) in MOGAD. Area postrema lesion was unique to NMOSD. All received immunotherapy, 91.4% (n=64) had good recovery, 8.6% (n=6) had functional limitation on modified Rankin scale (mRS) at discharge. Twelve (17.1%) relapsed. Conclusions: Largest group was CIS. Seropositivity of MOG was high with atypical presentations like prolonged fever, aseptic meningitis. Specific neuroimaging patterns correlated with ADS categories. Short-term outcome with immunotherapy was favourable inspite of relapses.