Epidemiology, diagnosis and outcomes of acquired demyelinating syndromes in children: A Czech perspective.

BACKGROUND

Acquired demyelinating syndromes (ADS) include rare diagnoses of acute disseminated encephalomyelitis (ADEM), clinically isolated syndrome (CIS), multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Their characteristics in the Czech children are currently unknown.

OBJECTIVES

To identify the epidemiology, diagnosis, care management, and outcomes of Czech children with ADS.

METHODS

Data from a two-year prospective national multicenter study were analyzed. The incidence was calculated for the Czech population (< 18 years of age) per 100,000 per year sourcing data from the Czech Statistical Office.

RESULTS

Our study covered 84.1% of the population and included 81 patients. The overall incidence of pediatric ADS was 2.24/100,000 (95% confidence interval [CI] 1.76-2.79). In particular, POMS, CIS, ADEM, and NMOSD had an incidence of 1.19 (0.85-1.62); 0.7 (0.35-1.33); 0.32 (0.16-0.57); and 0.03 (0.0007-0.16), respectively. Myelin oligodendrocyte glycoprotein (MOG) antibodies were present in 19.8% of all patients. Age was associated with diagnoses, MOG antibodies and preceding infections. After the first ADS episode, 6.3% of the children remained severely affected.

CONCLUSION

This first comprehensive analysis of pediatric ADS in the Czech Republic provided unique data on ADS incidence, identified a significant proportion of POMS, and highlighted challenges in diagnosis and care.