Neuropathologie Des Encéphalopathies Par Erreurs Innées Du Métabolisme Des Lipides, Des Glucides Et Des Acides Aminés

Summary Morphological features of the central nervous system, by light and electron microscopy, are reported in various encephalopathies by inborn enzymatic defect in lipid, glucidic and aminoacid metabolisms. The authors stress on the caracteristics of the abnormal storage material in Neurolipidoses, Ceroid-Lipofuscinoses, Glycogenosis type II and Lafora disease. On the other hand, they insist on the non specific lesions of the central nervous system in Aminoacidopathies by enzymatic defect.