The cerebral lipid composition of the brain in six cases of Krabbe's disease

Abstract

A case of Krabbe''s globoid cell leukodystrophy is presented. Chemical analysis of cerebral lipids was performed in the same case, as well as in 5 other histologically verified cases, and the results are reported. It can be seen that in every case there is a very considerable loss of the myelin lipids and especially of total phospho lipids, of cholesterol, and of cerebroside in the cerebral white matter. The cerebral cortex was only significantly abnormal in the patient described here and the one from Japan. These 2 patients showed a marked loss of total phospho lipids and a much smaller reduction in cholesterol and cerebrosides. None of the other lipids such as total hexosamine and ganglioside showed any significant abnormality.