European Journal of Pediatrics

, Volume 157, Supplement 2, pp S118–S121

Demyelination and inborn errors of the single carbon transfer pathway

  • R. Surtees

DOI: 10.1007/PL00014296

Cite this article as:
Surtees, R. Eur J Pediatr (1998) 157(Suppl 2): S118. doi:10.1007/PL00014296


Inborn errors of the single-carbon transfer pathway are rare disorders of folate and cobalamin metabolism. They may be complicated by demyelination resembling subacute combined degeneration of the cord and brain. The study of CSF metabolites in children with serial errors affecting the single-carbon transfer pathway has suggested that S-adenosylmethionine deficiency is a cause of the demyelination. This deficiency is corrected by treatment that causes clinical improvement and remyelination. Some treatments can only have an indirect effect on the brain and this is discussed with other evidence that the liver may produce factors that are necessary for the maintenance of central myelin.

Key words Demyelination Metabolism Inborn errors Cerebrospinal fluid Adenosylmethionine 

Copyright information

© Springer-Verlag Berlin Heidelberg 1998

Authors and Affiliations

  • R. Surtees
    • 1
  1. 1.Institute of Child Health (UCLMS), 30 Guilford Street, London WC1N 1EH, UKGB
  2. 2.Neuroscience Unit, The Wolfson Centre, Mecklenburgh Square, London WC1N 2AP, UK, Fax: +44 171-883 9469GB

Personalised recommendations