title: Therapeutic modification of membrane lipid abnormalities in juvenile neuronal ceroid-lipofuscinosis (Batten disease).
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.
authors.affiliation: Department of Chemical Pathology, Sheffield Children's Hospital, United Kingdom.
authors.forename: M J
authors.initials: MJ
authors.lastname: Bennett
authors.forename: G P
authors.initials: GP
authors.lastname: Hosking
authors.forename: R
authors.initials: R
authors.lastname: Gayton
authors.forename: G
authors.initials: G
authors.lastname: Thompson
authors.forename: J H
authors.initials: JH
authors.lastname: Galloway
authors.forename: I J
authors.initials: IJ
authors.lastname: Cartwright
mesh_terms: D000293:Adolescent; D003314:Corn Oil / Q000627:therapeutic use; D004041:Dietary Fats; D005395:Fish Oils / Q000627:therapeutic use*; D005500:Follow-Up Studies; D006801:Humans; D050356:Lipid Metabolism; D008074:Lipoproteins / Q000097:blood; D009472:Neuronal Ceroid-Lipofuscinoses / Q000178:diet therapy / Q000188:drug therapy / Q000628:therapy*
chemical_list: D004041:Dietary Fats; D005395:Fish Oils; D008074:Lipoproteins; D003314:Corn Oil