Metachromatic Sulfatides in Cerebral White Matter and Kidney.

Abstract

Metachromatic sulfuric acid esters (SAE) were measured in sulfatide fractions extracted from normal and abnormal white matter and kidney. Slightly lower than average SAE values for white matter were found in early childhood, in old age, and in disease interfering with myelin metabolism. The average SAE value in normal mature white matter was 58 mg/g. This was 10 times the value for normal kidney and corresponds with the much greater lipid metachromasia in white matter histologically. Statistically significant elevations in sulfatide values were found in white matter and kidney from 4 patients with one disease (metachromatic leucoencephalopathy). This accumulation may be related to an error in sulfatide metabolism, and appears, descriptively, to constitute a "sulfatide lipidosis".