Pathology of the central nervous system with special reference to the lipids.
The historical background of lipid research with particular reference to the nervous system is outlined. Attention is called to the early studies of Swedish investigators on the morphology and physical properties of the lipid rich myelin sheath. The pioneering research of the late Gunnar Brante on quantitative topical lipid chemistry of the human nervous system in health and disease is pointed out. A selection of lipid histochemical methods for various spingolipids is presented. Results are reported of their application to cases of four genetically determined sphingolipidoses. The morphological changes characterizing these diseases are interpreted in terms of histochemistry and biochemistry. The recently developed method by Dowson for the characterization of autofluorescence emission spectra from neuronal lipopigment in various ceroidoses (ceroid-lipofuscinosis) has been applied to cases of Sanfilippo's syndrome and Salla disease. The results are briefly commented upon.
Sourander
P
P
Department of Pathology I, University of Göteborg, Sweden.
D001928:Brain Diseases, Metabolic / Q000378:metabolism*; D006801:Humans; D050356:Lipid Metabolism; D008052:Lipid Metabolism, Inborn Errors / Q000378:metabolism; D013050:Spectrometry, Fluorescence