INCLUSION BODIES AND LATE FATE OF GANGLION CELLS IN INFANTILE AMAUROTIC FAMILY IDIOCY

Abstract

The changes in the ganglion cells in infantile amaurotic family idiocy are well known through the studies of Sachs,¹ Schaffer,² Bielschowsky³ and Spielmeyer,⁴ to mention only the leading investigators in this field. There are lacking, however, investigations concerned with the later fate of the altered ganglion cells in cases of infantile amaurotic family idiocy of long duration, as are studies of inclusion bodies, which are obviously related to the late changes in the ganglion cells. Finally it would be desirable to establish the relationship between the pathologic changes and the clinical signs, since these signs cannot be explained exclusively by the alterations in the ganglion cells, which are affected throughout the entire neuraxis. For all these reasons the following cases were studied. REPORT OF CASES CHEMICAL STUDY Case 1. —L. S., a 3 year old boy, was admitted to the Jewish Hospital of Brooklyn, service of