Clinical Spectrum of Acquired Demyelinating Syndromes in Children: A Tertiary Hospital Experience.

INTRODUCTION

Although acquired demyelinating syndromes (ADS) are rare in children, the incidence and prevalence of ADS vary internationally. As data on pediatric ADS in Saudi Arabia is limited, the aim of this study was to describe the clinical spectrum of pediatric ADS, its clinical characteristics, and management options at a tertiary hospital in Saudi Arabia.

METHODS

A retrospective observational study was conducted at King Abdulaziz Medical City (KAMC) and King Abdullah Specialized Children Hospital (KASCH) in Riyadh, Saudi Arabia between January 2016 and December 2022. All patients with ADS fulfilling criteria of each subtype (multiple sclerosis (MS), clinically isolated syndrome (CIS), acute disseminated encephalomyelitis (ADEM), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD)) were included in this study.

RESULTS

Forty-five pediatric patients with ADS were analyzed, with the majority diagnosed with MS. The median age of onset was higher in the MS group compared to monophasic CIS and MOGAD, with statistically significant differences in age at onset between the MS group and both the CIS and MOGAD groups (p = 0.0002). Significant differences were also observed in the type of initial central nervous system (CNS) attack, with optic neuritis being more common in MS and transverse myelitis in CIS (p < 0.0001). Laboratory results revealed a higher incidence of cerebrospinal fluid (CSF) oligoclonal bands in patients with MS, which was statistically significant (p =  0.04), and MOG antibodies were found in all patients with MOGAD. Intravenous pulse steroids were administered in most patients, while disease-modifying drugs (DMTs) were employed most frequently in patients with MS. The Expanded Disability Status Scale scores indicated little disability in most patients with MS and CIS, with more disability noted in a subgroup of ADEM. Overall, the study underscores the clinical heterogeneity of pediatric ADS and points out the statistically significant difference in age at onset, presenting features, and laboratory findings among ADS subtypes.

CONCLUSIONS

This study provides a thorough overview of pediatric ADS, including important distinctions between MS, ADEM, CIS, and MOGAD. Marked differences in age at onset, presentation, and imaging among these subtypes are informative for maximizing diagnosis and treatment. The key findings are the subsequent development of MS from CIS and MOGAD, varying patterns of first attack, and imaging characteristics like callososeptal interface lesions in MS and posterior fossa hyperintensities in MOGAD. All these indicate the need for individualized diagnostic and therapeutic approaches for improved outcomes.