To sum up. Ihc following can he said with regard to the amaurotic idiocies: (a) In infantile amaurotic idiocy (Tay-Sachs) the histology of the retina is characterized exclusively by degeneration of ganglion cells and atrophy of their axons; (b) In juvenile amaurotic idiocy (Spielmeyer-Vogt). on the other hand, degeneration of ganglion cells and atrophy of their axons is accompanied by the histological picture of tapetoretinal degeneration. Our histological study endorses the view that infantile amau rotic idiocy (Tay-Sachs’ disease) and juvenile amaurotic idiocy (Spielmeyer-Vogts disease) are separate entities. © 1968 S. Karger AG, Basel.