ICNC Abstracts, ICNC 2018

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Acquired Central Nervous System demyelinating syndromes in children: experience of a tertiary centre from North India
Priyanka Madaan, Biswaroop Chakrabarty, Prateek Kumar Panda, Juhi Gupta, Vishal Sondhi, Rachana Dubey, Atin Kumar, Manisha Jana, Sheffali Gulati

Last modified: 2018-09-09

Abstract


Introduction

Acute disseminated encephalomyelitis (ADEM), clinically isolated syndrome (CIS), Multiple sclerosis (MS) and Neuromyelitis optica spectrum disorders (NMOSD), collectively known as acquired demyelination syndromes (ADS), are being increasingly recognized in children.

Methods

Retrospective chart review of children with acquired demyelinating disorders presenting to a north Indian tertiary care hospital over 9 years (2009-2017) was done.

Results

Out of 95 cases, 66 were monophasic (19 ADEM and 46 CIS). Median age at presentation was 7years (Range:1-12years) with 41 boys. Among CIS, 19 had isolated acute transverse myelitis, 5 had NMOSD like presentation and rest were isolated optic neuritis, hemispheric and extrapyramidal syndromes. Most of the children had intact recovery with steroids.

Amongst the 29 patients with recurrent demyelination, majority were MS (18/29) followed by NMOSD (9/29; 2 were NMO ab positive), and multiphasic ADEM (2/29). Median age at presentation was 7.5years (range:3-12years) with 21 boys. Around 1/4th had disability (PCPS score 3-6 in 7 children). Recurrent demyelinating disorders require long-term immunomodulation and Azathioprine is the preferred agent used in resource-limited settings. Mean relapses in MS and NMOSD was approximately 1.5 episode per patient. One child with NMO failed to respond to azathioprine and required rituximab.

Conclusion: Acquired demyelinating syndromes need early identification and institution of appropriate therapy to minimize morbidity, avoid mortality and optimize outcome. Azathioprine appears to be an effective immunomodulator in recurrent demyelination, particularly in resource limited settings.


Keywords


demyelinating syndrome; central nervous syndrome;acquired;children

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