JUVENILE FAMILIAL AMAUROTIC IDIOCY (VOGTSPIELMEYER DISEASE)

Abstract

The juvenile type of familial amaurotic idiocy (Vogt-Spielmeyer disease) is a rare disorder, there having been only a few cases reported up to the present time. Particularly few case reports have been published in the United States. In the majority of these the diagnosis was made on the basis of pathologic observations, and this aspect of the disease was especially emphasized. In view of the fact that the literature has not been reviewed in English and clinical observations are rare, the present study was undertaken. In the case now reported for the first time the clinical, psychologic and electroencephalographic aspects have been emphasized. HISTORICAL REVIEW OF THE LITERATURE Since the concept of juvenile familial amaurotic idiocy was originally based on what is known of the infantile form of the disorder (Tay-Sachs disease), it seems best to start this review with a discussion of the latter disorder. Observations on the eyegrounds