Typical ophthalmoscopic picture of ''cherry-red spot'' in an adult with the myoclonic syndrome

Abstract

A case is reported of a myoclonic syndrome in an adult male with the typical fundus picture of Tay-Sachs' disease. Although there were no psychic disorders the authors believe that the case may be included in the group of amaurotic idiocies. The rarity of this finding is stressed by the fact that 7 cases only of typical ''cherry-red spot'' have previously been described in young subjects. It is suggested that such cases represent a particular variant in which amaurotic idiocy is characterized by a late onset.