Research Article| Volume 24, P175-183, August 2018

Increased cerebrospinal fluid chitinase 3-like 1 and neurofilament light chain in pediatric acquired demyelinating syndromes


      • Chitinase 3-like 1 and neurofilament light chain are increased in pediatric acquired demyelinating syndromes (ADS).
      • Chitinase 3-like 1 and neurofilament light chain better distinguish between ADS and non-ADS than oligoclonal bands alone.
      • Chitinase 3-like 1 is increased in ADEM compared with MS.



      Chitinase 3-like 1 (CHI3L1), neurofilament light chain (NFL) and oligoclonal bands (OCB) in cerebrospinal fluid are associated with central nervous system demyelination in adults. CHI3L1 and OCB are markers of central nervous system inflammation, whereas NFL is a marker of white-matter axonal injury. The aim was to examine whether CHI3L1 and NFL in cerebrospinal fluid are associated with acquired demyelinating syndromes at disease onset in a pediatric population.


      Children (<18 years) referred to hospital for possible neuroinflammatory disease were retrospectively included from 2010 to 2016. Case ascertainment was by review of medical records. NFL and CHI3L1 were measured by enzyme-linked immunosorbent assays. Endpoints were differences in concentrations of cerebrospinal fluid NFL and CHI3L1.


      We included 193 children who all underwent cerebrospinal fluid OCB examination as part of their diagnostic work-up and classified these children into 5 groups: acquired demyelinating syndromes (n = 33), normal diagnostic work-up (n = 36), inflammatory neurological disease (n = 50), other neurological disease (n = 55), and systemic inflammatory diseases (n = 19). NFL and CHI3L1 in cerebrospinal fluid differed significantly between the five groups (p = 0.0001). CHI3L1 was significantly higher in acquired demyelinating syndromes than in all other groups, and NFL was significantly higher in acquired demyelinating syndromes than in the other groups except systemic inflammatory disease. Children with acute disseminated encephalomyelitis had significantly higher concentrations of CHI3L1 than did children with multiple sclerosis.


      We provide class II evidence that CHI3L1 and NFL are associated with pediatric acquired demyelinating syndromes. CHI3L1 may help distinguishing between acute disseminated encephalomyelitis and multiple sclerosis, but this needs further confirmation.



      ADEM (Acute disseminated encephalomyelitis), ADS (Acquired demyelinating syndromes), AUC (Area under the curve), CHI3L1 (Chitinase 3-like 1), CIS (Clinically isolated syndrome), CNS (Central nervous system), CSF (Cerebrospinal fluid), ELISA (Enzyme-linked immunosorbent assay), IPMSSG (International Pediatric MS Study Group), MOG (Myelin oligodendrocyte glycoprotein), MRI (Magnetic resonance imagining), MS (Multiple sclerosis), NFL (Neurofilament light chain), NMOSD (Neuromyelitis optica spectrum disorder), OCB (oligoclonal bands), ON (Optic neuritis), SIMOA (Single molecule array), ROC (Receiver operating characteristic), TM (Transverse myelitis)
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