Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address Please contact the publisher to request reinstatement.
April 22, 1933

Die juvenile amaurotische Idiotie: Klinische und erblichkeitsmedizinische Untersuchungen.

Author Affiliations

Von Torsten Sjögren, Statens Institut för Rasbiologi, Uppsala und S:t Lars Sjukhus, Lund. Paper. Pp. 197-426, with illustrations. Lund: Berlingska Boktryckeriet, 1931.

JAMA. 1933;100(16):1281. doi:10.1001/jama.1933.02740160065035

This article is only available in the PDF format. Download the PDF to view the article, as well as its associated figures and tables.


The term amaurotic family idiocy usually recalls to one's mind the well known morbid entity described by Tay in 1881 and by Sachs in 1887 and known as the infantile form of this disease. The most remarkable features are the exclusive and early occurrence (at about 6 to 8 months) in Jewish infants of blindness, of dementia and of a cherry-red spot in the region of the macula. Amaurotic family idiocy occurs also in non-Jewish children but much later in life, at the age of 4 years or after, lasts much longer, and, instead of a cherry-red spot, it exhibits other retinal changes, mainly retinitis pigmentosa. While the infantile form of Tay-Sachs disease is easy to diagnose, the juvenile form always remains undiagnosed and is considered a very rare disease. Sjögren showed that this is not the case. By studies of fifty cases of blind children in various stages of

First Page Preview View Large
First page PDF preview
First page PDF preview