JUVENILE AMAUROTIC IDIOCY

Abstract

Amaurotic idiocy assumed a new significance when Bielschowsky 1 demonstrated its occurrence with Niemann-Pick's disease. He expressed the belief that both diseases were the result of the same pathologic factor, that is, a disturbance of the metabolism. On the other hand, the name given by Bloom, 2 lipoid histiocytosis, signifies that Niemann-Pick's disease is a primary pathologic condition of the mesoderm, while amaurotic idiocy has been considered a primary disease of the neuroderm. In 1928, Abt and Bloom 3 were able to collect reports of fifteen cases of Niemann-Pick's disease, but in only two was there record of an examination of the brain. Since that time the association of amaurotic idiocy with Niemann-Pick's disease has been described by Wascowitz, 4 Hassin, 5 Poncher 6 and others. Kufs 7 concurred with Bielschowsky 1 in the point of view that amaurotic idiocy is part of Niemann-Pick's disease, but he believed that varying