AUTHOR=Simone Marta , Palazzo Claudia , Mastrapasqua Mariangela , Bollo Luca , Pompamea Francesco , Gabellone Alessandra , Marzulli Lucia , Giordano Paola , De Giacomo Andrea , Frigeri Antonio , Ruggieri Maddalena , Margari Lucia 

TITLE=Serum Neurofilament Light Chain Levels and Myelin Oligodendrocyte Glycoprotein Antibodies in Pediatric Acquired Demyelinating Syndromes

JOURNAL=Frontiers in Neurology

VOLUME=Volume 12 - 2021

YEAR=2021

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2021.754518

DOI=10.3389/fneur.2021.754518

ISSN=1664-2295

ABSTRACT=Introduction: The relationship between neurofilament light chain serum levels (sNFL) and myelin oligodendrocyte glycoprotein antibody (MOG-Ab) status has not been yet investigated in children with acquired demyelinating syndrome (ADS) 

Objective and Methods: sNFl levels and MOG-Abs were measured by ultra-sensitive single-molecule array and cell-based assay in a cohort of 37 children with ADS onset and negativity for serum anti-aquaporin 4 (AQP4) antibodies. sNFL levels were compared in MOG-Ab+/MOG-Ab, and in two subgroups MOG Ab + with /without encephalopathy. 

Results: 40% ADS resulted MOG-Ab+. MOG-Ab+ were younger at sampling (Median=9.8; range=2.17-17.5 vs 14.7/ 9-17; p=0.002) with lower frequency of cerebrospinal fluid oligoclonal bands positivity (27% vs 70%; p=0.013) compared to MOG-Ab.53 % of MOG+Ab presented encephalopathy at onset, 1/22 of MOG-Ab (p=0.0006).  Higher sNF-L levels (p= 0.0001) were found in MOG-Ab+ (Median / Range =11.11 /6.8-1129) and MOG-Ab- (Median / Range =11.6 /4.3-788) compared to age-matched controls (Median / Range =2.98 (1-4.53), without significant difference. MOG-Ab+ with encephalopathy resulted significantly younger at sampling (Median / Range: 4.5/2.17-11.17 vs 14.16/9.8-17.5 p= 0.004), had higher sNfL levels (Median/Range:75.24/9.1-1129 vs 10.22/6./6.83-50.53; p=0.04) and showed a trend for higher MOG-Ab titer (0.28/0.04-0.69 vs 0.05/0.04-0.28; p=0.1) in comparison to those without encephalopathy.

Discussion: we confirmed high sNfL levels in pediatric ADS independently from the MOG-Ab status. Encephalopathy at onset is associated more frequently with MOG-Ab+ children with higher sNFl levels and MOG titer. These findings suggest a role of acute demyelination in association with axonal damage in the pathogenesis of encephalopathy in pediatric ADS.