ICNC Abstracts, ICNC 2018

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Clinico-radiological correlation of Acquired Demyelination Syndromes(ADS) of central nervous system: A pediatric cohort
ANSHITA ARORA, SONAM KOTHARI, SHILPA KULKARNI, ANAITA HEGDE, KRISHANKUMAR SHAH

Last modified: 2018-09-09

Abstract


Introduction: Acquired demyelinating syndromes (ADS) are defined as acute-onset neurologic deficits with evidence of central nervous system (CNS) demyelination. Clinico-radiological heterogeneity in ADS pose a diagnostic & prognostic dilemma. Methodology: A retrospective clinical, etiological, treatment and follow up analysis was carried out of all children diagnosed with CNS ADS in last five years (2013-2018). Results: Total of 30 children (M:F ratio:1.1:1) were evaluated. Age of onset was from 10 months to 13 years (median: 5.6 years). They presented with fever(n=16,53%), altered sensorium(n=13,43%), seizures(n=12,40%), headache(n=7,23%), optic neuritis(n=11,36%), pyramidal(n=12,40%), extra pyramidal (ataxia-2, movement disorders-2), sensory loss(n=2,7%). Neuroimaging showed T2 hyperintensities in spinal cord (n=12, 40%), cerebrum (n=8,26%), optic nerve (n=7,23%), cerebellum (n=6,20%), thalamus (n=5,17%), basal ganglia and brain stem(n=3, 10%). 11/30 children presented with recurrent episodes, having female preponderance (M:F-1:3). Seropositive status was seen in seven children: Aquaporin antibodies (n=6) and anti-myelin oligodendrocyte glycoprotein (MOG) (n=1). Depending upon clinic imaging features, children were classified under following groups: Acute disseminated encephalomyelitis (ADEM) (single/multiple) (n=14, 47%), clinically isolated syndromes (CIS) (n=6, 20%), neuromyelitis optica (NMO) (n=3, 10%), NMO spectrum disorder (n=6, 20%) and multiple sclerosis (n=1, 3.3%). Response to steroids alone was seen in73% (n=20, 67%), steroid+ Intravenous immunoglobulin in 16% (n=5) and 13% (n=4) required disease modifying drugs. Conclusion: Age of onset>5 years, female sex, presentation without fever, diffuse MRI involvement (supra, infra tentorium and long segment of spinal cord) and seropositive status are indicators of poor prognosis and high recurrence risk. Clinico imaging and etiological pattern recognition is of utmost importance.


Keywords


Demyelination, Neuromyelitis optica, Multiple sclerosis, Aquaporin antibodies, Intravenous immunoglobulin

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