title: 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.
authors.affiliation: National Pediatric Myoclonus Center and Neuroimmunology Laboratory, Southern Illinois University School of Medicine, PO Box 19643, Springfield, IL 62794-9643, USA; Department of Neurology, Southern Illinois University School of Medicine, PO Box 19643, Springfield, IL 62794-9643, USA. Electronic address: mpranzatelli@siumed.edu.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Pranzatelli
authors.affiliation: National Pediatric Myoclonus Center and Neuroimmunology Laboratory, Southern Illinois University School of Medicine, PO Box 19643, Springfield, IL 62794-9643, USA; Department of Neurology, Southern Illinois University School of Medicine, PO Box 19643, Springfield, IL 62794-9643, USA.
authors.forename: Elizabeth D
authors.initials: ED
authors.lastname: Tate
authors.affiliation: National Pediatric Myoclonus Center and Neuroimmunology Laboratory, Southern Illinois University School of Medicine, PO Box 19643, Springfield, IL 62794-9643, USA; Department of Neurology, Southern Illinois University School of Medicine, PO Box 19643, Springfield, IL 62794-9643, USA.
authors.forename: Nathan R
authors.initials: NR
authors.lastname: McGee
authors.affiliation: Department of Statistics and Research Consulting, Southern Illinois University School of Medicine, PO Box 19664, Springfield, IL 62794-9664, USA.
authors.forename: Steven J
authors.initials: SJ
authors.lastname: Verhulst
mesh_terms: D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D016207:Cytokines / Q000378:metabolism; D004660:Encephalitis / Q000134:cerebrospinal fluid*; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D016900:Neurofilament Proteins / Q000134:cerebrospinal fluid*; D019370:Observation; D053578:Opsoclonus-Myoclonus Syndrome / Q000134:cerebrospinal fluid*; D012189:Retrospective Studies; D000069283:Rituximab; D012720:Severity of Illness Index; D055815:Young Adult
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D016207:Cytokines; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D016900:Neurofilament Proteins; C044882:neurofilament protein L; D000069283:Rituximab; D000324:Adrenocorticotropic Hormone
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