Abstract
Background
Autoimmune diseases of the central nervous system in children present a significant
diagnostic challenge due to their heterogeneous nature. The identification of anti-myelin
oligodendrocyte glycoprotein (MOG) antibodies has contributed to our understanding
and classification of these disorders.
Objective
This study aims to characterize the clinical, paraclinical, and neuroimaging findings
in pediatric patients diagnosed with acquired demyelinating syndromes (ADS) and autoimmune
encephalitis (AE).
Methods
Conducted as a descriptive case series, this study retrospectively analyzed medical
records of children under 18 years diagnosed with ADS or AE from January 2017 to February
2022. We employed basic descriptive statistics to analyze patient demographics, clinical
presentations, laboratory findings, and neuroimaging results.
Results
A total of 71 cases were evaluated, with 80% classified as ADS and 20% as AE. Within
ADS, acute disseminated encephalomyelitis and optic neuritis were the most prevalent
at 19%, followed by pediatric-onset multiple sclerosis at 17%, and transverse myelitis
at 16%. AE cases were predominantly seropositive (93%), with a vast majority (92%)
showing anti-NMDAR antibodies compared with 8% with anti-MOG antibodies. Since MOG
antibody measurement became available at our institution in 2020, many new cases have
been diagnosed as MOGAD (MOG antibody-associated disease), a new entity with different
clinical manifestations.
Conclusion
In children demyelinating disorders are more frequent than autoimmune encephalitis.
New biomarkers, such as anti-MOG antibodies, have led to a better understanding of
pediatric autoimmune CNS diseases. The study underscores the necessity of continuous
reevaluation of diagnostic criteria and treatment protocols considering emerging biomarkers
in neuroimmunology.
Keywords
autoimmune encephalitis - acquired demyelinating syndrome - multiple sclerosis
