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Juvenile Amaurotic Idiocy: A Case Report

Published online by Cambridge University Press:  08 February 2018

John Johnson
John Johnson*
Affiliation:
The Maudsley Hospital, London
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Extract

The first clinical histories of this condition were reported in Norway by Stengel (1826). Batten (1903) described changes in the macula in two members of a family who had undergone progressive mental deterioration with loss of visual and motor functions. Vogt (1906) published similar cases and attempted to delimit them as a juvenile form of Tay Sachs disease. It differed from the infantile form in that it occurred in childhood after a period of normal development; the rate of progression was slower; it was not limited to Jewish children and the ophthalmological appearances were those of optic atrophy. The following year Spielmeyer described the pathological changes in juvenile Tay Sachs disease as essentially similar to the infantile form. Further detailed pathological studies were made by Greenfield and Holmes (1925).

Type
Original Articles
Information
Journal of Mental Science , Volume 107 , Issue 450 , September 1961 , pp. 931 - 935
Copyright
Copyright © Royal College of Psychiatrists, 1961 

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