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

✍ Scribed by Bennett, Michael J. ;Hosking, Gwilym P. ;Gayton, Romayne ;Thompson, Georgina ;Galloway, John H. ;Cartwright, Ian J. ;Opitz, John M. ;Reynolds, James F. ;Pullarkat, Raju K.

• Publisher: John Wiley and Sons
• Year: 1988
• Tongue: English
• Weight: 372 KB
• Volume: 31
• Category: Article
• ISSN: 0148-7299
• DOI: 10.1002/ajmg.1320310630

✦ Synopsis

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.