[Skip to Content]
[Skip to Content Landing]
Other
December 1924

A STUDY OF THE HISTOPATHOLOGY OF AMAUROTIC FAMILY IDIOCY (INFANTILE TYPE OF TAY-SACHS)

Author Affiliations

Associate Professor of Neurology, College of Medicine, University of Illinois CHICAGO

From the Division of Neurology of the College of Medicine, University of Illinois, and the pathologic laboratories of the Illinois State Psychopathic Institute and Cook County Hospital.

Arch NeurPsych. 1924;12(6):640-662. doi:10.1001/archneurpsyc.1924.02200060041004
Abstract

The term "amaurotic family idiocy" comprises a large group of disorders in which there are more or less definite pathologic changes. Here belong the infantile type of Tay1-Sachs,2 the late infantile variety described by Jansky,3 Schob,4 but especially well studied by Bielschowsky5 and the juvenile form of Vogt6 and Spielmeyer.7 Ichikawa8 accepts six varieties in which, he states, the changes, especially the retinal, as well as the clinical features are practically alike. Vogt, Spielmeyer and Bielschowsky also class with amaurotic family idiocy cases of exquisitely hereditary and familial cerebral disorders recorded by Pelizaeus,9 Higier,10 Merzbacher11 and one lately reported by Wolpert.12 However, like many other cases of blind idiocy, such as Winkler's,13 these have little in common, either clinically or anatomically, with the three well studied types mentioned above. Possessing common clinical features—blindness and progressive mental

×