Alterations in the composition of brain lipids in patients with Creutzfeldt-Jakob disease

Abstract

Brain lipids were isolated from 3 patients with Creutzfeldt-Jakob (C-J) disease and their chemical constituents were investigated. The total lipid content increased slightly in diseased gray matter. Ganglioside was reduced to less than 20 and 50% of that of control values in gray and white matter of the Creutzfeldt-Jakob brains, respectively, on both a wet and dry weight basis. Abnormal thin-layer chromatography patterns were observed in the gangliosides from the diseased brains. The C 20-sphingosine in the gangliosides from the Creutzfeldt-Jakob brains was markedly reduced. The ratio of C 20 to C 18-sphingosine was 0.03 to 0.23 in the diseased gray matter. An alteration in the fatty acid composition of ganglioside was observed in 1 case of C-J disease. The total cholesterol content increased both in gray and white matter of the Creutzfeldt-Jakob brain, and one-third of the total cholesterol was in the esterified form in gray matter. Major fatty acids of cholesterol ester were palmitic and oleic acids. The total phospholipids were severely decreased in the diseased brains, but the relative proportions of the individual phospholipids were constant. Polyunsaturated fatty acids in phosphatidyl ethanolamine from the diseased gray matter decreased with a moderate increase in oleic acid. Galactolipid levels and types were essentially unchanged. From the above described abnormalities of brain lipids, the pathogenesis of this disease is discussed.