Subacute Sclerosing Leukoencephalitis*†‡

Abstract

Chemical studies were done on cerebral white matter, gray matter, and myelin of a case of subacute sclerosing leukoencephalitis. Myelin was obtained in one-third the normal yield and was found to have an abnormal lipid composition but a normal lipid-protein ratio, normal solubility properties in CHCl₃-CH₃OH and normal morphology. The myelin cholesterol was increased by 50 per cent with a concomitant decrease of cerebrosides, sulfatides and ethanolamine phosphatides. The white matter showed a pattern typical of severe demyelination, whereas the gray matter was relatively normal. Total solids of white matter were 50 per cent of normal; the cholesterol ester was 27 per cent of total lipid with large decreases in proteolipid protein, cerebrosides and ethanolamine phosphatides and smaller decreases in other lipids. During the myelin isolation a light, abnormal fraction was found which was 57 per cent cholesterol ester and presumably represents the sudanophilic material. A ganglioside abnormality was found only in the white matter. The total amount of gangliosides were twice normal on a dry weight basis and were found to have greatly increased amounts of the usually minor gangliosides, G_2A, G_3A, G₅ and G₆. These 4 gangliosides accounted for 57 per cent of the total lipid neuraminic acid.