Chemical pathology of Krabbe's disease. I. Lipid composition and fatty acid patterns of phosphoglycerides in brain.

Abstract

ABSTRACT. Vanier, M.‐Th. and Svennerhohn, L. (Department of Neurochemistry, Psychiatric Research Centre, University of Göteborg, Göteborg, Sweden). Chemical pathology of Krabbe's Disease. I. Lipid composition and fatty acid patterns of phosphoglycerides in brain. Acta Paediatr Scand, 63: 494, 1974.—Lipid biochemical determinations were performed on autopsy material from 18 cases of Krabbe's disease, aged 7–23 months, collected in Sweden between 1960 and 1972.Cerebral white matter. The lipid concentration of the cerebral white matter was found substantially reduced. It was the same in 17 cases, independent of age at death, and rather similar to that of cerebral cortex. All major lipids were diminished, but the myelin lipids, cholesterol and particularly galactolipids (cerebrosides and sulfatides) were reduced more than the phospholipids. The ratio cerebroside/sulfatide was significantly increased, but this. was not a constant finding. The relative percentage of ethanolamine phosphoglycerides was decreased and that of choline phosphoglycerides increased. The fatty acid composition of the phosphoglycerides shifted towards values found in the white matter of 2 to 4 month‐old infants. In addition, a characteristic significant increase of arachidonic acid occurred.Cerebral cortex. Only minor changes were observed, but they were of the same type as some of the changes of the white matter: a decrease in the relative percentage of ethanolamine phosphoglycerides and an increase of arachidonic acid.The lipid changes in Krabbe's disease are suggested to be the result of the combined effect of a serious myelin deficiency, and the replacement of the normal brain cytoarchi‐tecture by proliferated astroglial cells, ephitheliod cells and globoid bodies.