American Journal of Clinical Nutrition, Vol 27, 1152-1157, Copyright © 1974 by The American Society for Clinical Nutrition, Inc.

Blood levels of -tocopherol in a disorder of lipid peroxidation: Batten's disease

¹ From the Department of Pathology, Indiana University School of Medicine, Indianapolis, Indiana 46202

Batten's disease is characterized by the onset of progressive mental regression and seizures. The brain cells of patients with this disease show the accumulation of large masses of a lipopigment, ceroid. Similar accumulations have been observed in the brain of a genetic strain of English setters. Previous studies by our laboratory have established that the accumulated lipopigment, ceroid, is clearly different from "age" pigment, or lipofuscin, in a number of parameters, although some similarities exist between the two lipopigments. Zeman and others have proposed that this group of disorders is the result of a massive peroxidation of essential fatty acids with the accumulation of large masses of lipid polymers in the form of lipopigments. Since the proposed biological role of -tocopherol is that of an antioxidant, this compound has been utilized in experimental therapeutic approaches with patients affected with Batten's disease. At this time, the standard treatment for this group of disorders is the administration of a mixture of vitamin E, ascorbic acid, methionine, and butylated hydroxylated toluene as originally proposed by Tappel. Patients on this therapeutic regimen have shown some improvement. Our approach to this problem was to investigate the blood and tissue levels of -tocopherol in this disorder. Early studies suggested that blood -tocopherol levels were below normal values. However, a study on a large number of samples from both human patients and dogs with a similar disease clearly established that blood and tissue levels of -tocopherol are significantly higher than normal age-matched controls. Obviously this "peroxidative disorder" progresses in spite of an ample supply of the biological antioxidant, -tocopherol. If -tocopherol is a significant factor in the inhibition of peroxidative damage, then -tocopherol may not act exclusively as an antioxidant as has been proposed by a number of investigators.