First demyelinating attack in children: A twelve year single center cohort

Area of Science:

• Pediatric Neurology
• Neuroimmunology
• Demyelinating Diseases

Background:

• Acquired demyelinating syndromes (ADS) in children present diagnostic challenges due to overlapping symptoms.
• Accurate differentiation between monophasic and recurrent conditions like pediatric-onset multiple sclerosis (POMS), myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and seronegative ADS is crucial for effective treatment and prognosis.

Purpose of the Study:

• To characterize the initial presentation of pediatric ADS.
• To evaluate diagnostic evolution over time to guide treatment decisions.

Main Methods:

• Retrospective analysis of 59 children with ADS (2012-2024) at a tertiary pediatric center.
• Initial diagnoses included MS, ADEM, CIS, ON, NMOSD, or 'Indeterminate'. Final diagnoses were MS, MOGAD, or 'Other'.
• Analysis of demographic, clinical, MRI, CSF data, and outcomes, with statistical comparisons.

Main Results:

• Older age at onset, absence of preceding infection, and specific MRI findings (periventricular/callosal lesions, cerebellar involvement) were more common in the MS group.
• Younger age, preceding infection, fever, irritability, and cortical involvement were associated with MOGAD.
• 29% of final MS cases were initially diagnosed as CIS or ON; no ADEM cases converted to MS. 'Other' ADS patients exhibited more severe initial disability.

Conclusions:

• Pediatric ADS exhibit heterogeneity at onset.
• Accurate acute management and longitudinal follow-up are essential for refining diagnosis and guiding treatment in pediatric demyelinating diseases.