Acquired Demyelination Syndrome in Children and Adolescents: 10 Years Experience from a Tertiary Care Centre in North India.

Abstract

The childhood central nervous system (CNS) acquired demyelinating syndromes (ADS) can be monophasic or recurrent, with both having considerable overlap in the first decade of life. The objective of the study was to describe clinical and radiological features, immunological characteristics, response to therapy and difference between monophasic and first episode of recurrent disorders of pediatric-onset CNS ADS. Case records of all patients presenting with CNS ADS to the Department of Pediatrics between January 2009 to December 2018 were retrospectively reviewed. Those with complete records and at least 12 months follow up were included for analysis. Overall 95 case records were reviewed (66 monophasic: 20 ADEM and 46 CIS, 29 recurrent: 18 MS, 9 NMOSD, and 2 multiphasic ADEM). The median age of the cohort was 7 years (range: 1-12) and nearly two-thirds (62/95) were males. All acute cases were treated with intravenous pulse followed by tapering oral steroid therapy. All the recurrent entities received azathioprine with rituximab in few. Certain clinical and radiological features of CIS and immune and inflammatory characteristics in CSF were found to be significantly different in monophasic cases compared to first episode of recurrent cases. The CNS ADS show favourable response to immunotherapy. Azathioprine may be an effective long term immunomodulator, particularly in resource limited settings. Certain clinical, radiological and immunological features may differentiate monophasic illness from first episode of recurrent disorder.