Abstract
Ultrastructural and biochemical studies were performed on a biopsy from the cerebral cortex of a child with late infantile amaurotic idiocy. The disease began clinically at 26 months of age with intellectual and behavioral deterioration and progressive blinijness resulting from pigmentary degeneration of the retina. The cytoplasm olf neurons was distended by material that stained intensely in periodic acid-Schiff preparations, and ultrastructural studies showed many abnormal membrane-bound cytosomes. Although concentrations of lipid-bound sialic acid were normal, showing only minor changes in the ganglioside pattern, riondialyzable sialoglycoproteins were strikingly increased. Biochemical characterization of some forms of amaurotic familial idiocy may be dependent on increasing knowledge of metabolic interrelations between sialoglycopiroteins and gangliosides.
- © 1974 by the American Academy of Neurology
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