Bennett M J, Hosking G P, Gayton R, Thompson G, Galloway J H, Cartwright I J
Department of Chemical Pathology, Sheffield Children's Hospital, United Kingdom.
Am J Med Genet Suppl. 1988;5:275-81. doi: 10.1002/ajmg.1320310630.
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.
五名患有青少年神经元蜡样脂褐质沉积症的患者，其血清前β脂蛋白缺失或减少，红细胞膜脂质中多不饱和脂肪酸缺乏，接受了为期一年的鱼油提取物（富含ω-3脂肪酸）补充治疗，并被鼓励增加玉米油脂质（富含ω-6脂肪酸）的饮食摄入量，试图促进这些脂肪酸掺入膜中。一年后，红细胞膜中的ω-3脂肪酸（P<0.05）和总多不饱和脂肪酸（P<0.02）显著增加，但ω-6脂肪酸的掺入没有变化。在补充治疗前后，对患者进行了临床、心理测量和神经生理学评估。治疗一年后未发现显著变化。
