Therapeutic modification of membrane lipid abnormalities in juvenile neuronal ceroid-lipofuscinosis (Batten disease)

Am J Med Genet Suppl. 1988:5:275-81. doi: 10.1002/ajmg.1320310630.

Abstract

Five patients with juvenile neuronal ceroid-lipofuscinosis, who were shown to have absent or reduced serum prebetalipoprotein and a deficiency of polyunsaturated fatty acids in erythrocyte membrane lipids, were treated for a period of one year with supplements of fish oil extract (rich in omega-3 fatty acids) and encouraged to increase dietary intake of corn oil lipid (rich in omega-6 fatty acids) in an attempt to promote incorporation of these fatty acids into membranes. After one year there was a significant increase in omega-3 fatty acids (P less than 0.05) and in the total polyunsaturated fatty acids (P less than 0.02) in erythrocyte membranes but no change in the incorporation of omega-6 fatty acids. The patients were assessed clinically, psychometrically and neurophysiologically before and after the supplementation. No significant changes were noted after one year of treatment.

MeSH terms

  • Adolescent
  • Corn Oil / therapeutic use
  • Dietary Fats*
  • Fish Oils / therapeutic use*
  • Follow-Up Studies
  • Humans
  • Lipid Metabolism
  • Lipoproteins / blood
  • Neuronal Ceroid-Lipofuscinoses / diet therapy
  • Neuronal Ceroid-Lipofuscinoses / drug therapy
  • Neuronal Ceroid-Lipofuscinoses / therapy*

Substances

  • Dietary Fats
  • Fish Oils
  • Lipoproteins
  • Corn Oil