AMAUROTIC FAMILIAL IDIOCY IN IDENTICAL TWINS

Abstract

Since the original descriptions by Tay in 1881 and Sachs in 1887 of amaurotic familial idiocy in infants, a great deal of knowledge has been added to the subject, and it is now recognized that this condition exists not only in the form of (<i>a</i>) infantile amaurotic familial idiocy as originally described but in three additional forms, namely (<i>b</i>) the late infantile type, (<i>c</i>) the juvenile type and (<i>d</i>) the adult type.¹As the cases reported in this paper are instances of the classic form of amaurotic familial idiocy, to which the name Tay-Sachs disease has been given, a brief description of the disease will be limited to this form only and the others will be omitted as being beyond the scope of this presentation. Tay-Sachs disease is uncommon, though not rare; and while cases have been reported for the past fifty years, a search of the literature available