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

J Neuroimmunol. 2014 Jan 15;266(1-2):75-81. doi: 10.1016/j.jneuroim.2013.11.004. Epub 2013 Nov 16.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adrenocorticotropic Hormone / therapeutic use
  • Adult
  • Antibodies, Monoclonal, Murine-Derived / therapeutic use
  • Child
  • Child, Preschool
  • Cross-Sectional Studies
  • Cytokines / metabolism
  • Encephalitis / cerebrospinal fluid*
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Factors / therapeutic use
  • Male
  • Neurofilament Proteins / cerebrospinal fluid*
  • Observation
  • Opsoclonus-Myoclonus Syndrome / cerebrospinal fluid*
  • Retrospective Studies
  • Rituximab
  • Severity of Illness Index
  • Young Adult


  • Antibodies, Monoclonal, Murine-Derived
  • Cytokines
  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Neurofilament Proteins
  • neurofilament protein L
  • Rituximab
  • Adrenocorticotropic Hormone