Childhood Acquired Demyelinating Syndromes: Diagnostic Challenges From First Attack To Final Diagnosis

Objective: To investigate the diagnostic journey from first attack to reach a final diagnosis in acquired demyelinating syndrome (ADS) of childhood.

Methods: A total of 110 children (56 females, mean age at first attack: 10.6±4.1 years) with a diagnosis of childhood ADS were included from Ege University Pediatric Neurology Patient Dataset between 2010 to 2023. The final diagnosis of each patient was established based on the international consensus criteria of ADS neuromyelitis optica spectrum disorder (NMOSD)-2015, multiple sclerosis (MS)-2016, and myelin oligodendrocyte glycoprotein antibody-associated disorder (MOGAD)-2023 by two experienced pediatric neurologists (SK and SY).

Results: The first attack diagnosis and the final diagnosis of ADS cohort is presented in figure 1. The mean follow-up duration of the cohort was 34.7 months with a relapse rate of 27.3% (n=30/110). The 66.7% (n=20) of clinical relapses occurred within the first-year follow-up. Sixteen (34.7%) patients with clinically isolated syndrome (CIS) had relapse, and 87.5% of final diagnosis of MS. Among the patients with acute disseminated encephalomyelitis (ADEM), the relapse rate was 21.4% (n=6/28) with following new ADS diagnosis: MS (n=2), NMOSD (n=2), MOGAD (n=1), and multiphasic disseminated encephalomyelitis (n=1). However, isolated acute transverse myelitis exhibited a monophasic trajectory.

Conclusions: The observed temporal progression of diagnostic alterations in instances of pediatric ADS is noteworthy. Specifically, the relapse rates of CIS and ADEM were 34.7% and 21.4%, respectively. In the antibody era, the rational use of neuroimaging modalities might promptly provide a final correct diagnosis of childhood ADS for the disease-spesific treatment protocols.