CSF neurofilament light chain is elevated in OMS (decreasing with immunotherapy) and other pediatric neuroinflammatory disorders

Abstract

Using a panel of seven brain cell-specific biomarkers in cerebrospinal fluid (CSF), pediatric opsoclonus-myoclonus syndrome (OMS) (n=234) was compared to pediatric non-inflammatory neurological controls (n=84) and other inflammatory neurological disorders (OIND) (n=44). Only CSF NFL was elevated in untreated OMS versus controls (+83%). It was 87% higher in OIND than in OMS. On combination treatment with front-loaded ACTH, IVIg, rituximab, median CSF NFL decreased by 60% to control levels. These biochemical data suggest neuronal/axonal injury in some children with OMS without indicators of astrogliosis, and reduction on sufficient immunotherapy.

Keywords: ACTH; CSF; ELISA; GFAP; IVIg; NFL; NIND; NND; NSE; Neuroblastoma; Neurofilament pediatric ranges; OIND; Opsoclonus–myoclonus syndrome; Paraneoplastic syndrome; Pediatric neuroinflammation; S-100-beta protein; S100B; adrenocorticotropic hormone; cerebrospinal fluid; enzyme-linked immunosorbent assay; glia fibrillary acidic protein; intravenous immunoglobulins; neurofilament light chain protein or low molecular weight subunit; neuron specific enolase; non-inflammatory neurological disorders; non-inflammatory non-neurological disorders; other inflammatory neurological disorders; pNFH; phosphorylated neurofilament heavy chain protein or high molecular weight subunit.