Iron status in children with recurrent episodes of tumefactive cerebral demyelination

J Child Neurol. 2010 Nov;25(11):1401-7. doi: 10.1177/0883073810366179. Epub 2010 Apr 15.

Abstract

Iron is a vital element in the multifactorial initiation of myelination. It is required for cholesterol and lipid biosynthesis, both key components of myelin. Iron also plays an important role in energy production by mitochondrial oxidative metabolism which occurs in myelin-producing oligodentrocytes at a higher rate than in any other cell. Iron deficiency can, therefore, result in decreased oligodendrocyte survival and defective myelination. This led us to investigate iron status in 2 consecutive children with multiple sclerosis who presented with recurrent episodes of tumefactive demyelination. Testing revealed nonanemic iron deficiency in both patients. Discontinuation of iron supplementation in both children resulted in recurrent decreased iron parameters which can indicate mutations in proteins responsible for regulation of iron uptake. Further studies are warranted to explore the association of low iron in children presenting with recurrent episodes of tumefactive demyelination.

Publication types

  • Case Reports

MeSH terms

  • Cerebral Cortex / pathology*
  • Child, Preschool
  • Demyelinating Diseases / blood*
  • Demyelinating Diseases / pathology
  • Hemoglobins / metabolism
  • Humans
  • Infant
  • Iron / blood*
  • Magnetic Resonance Imaging
  • Male
  • Recurrence

Substances

  • Hemoglobins
  • Iron