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July 1935

LATE INFANTILE AMAUROTIC IDIOCY WITH MARKED CEREBRAL ATROPHY: CLINICAL AND ANATOMIC REPORT OF A CASE

Author Affiliations

CHICAGO
From the Departments of Neurology and Pediatrics of Rush Medical College.

Am J Dis Child. 1935;50(1):111-131. doi:10.1001/archpedi.1935.01970070120010
Abstract

As recently as 1909, Schaffer1 made the statement, which he has since greatly modified, that infantile amaurotic family idiocy was characterized by no macroscopic anatomic changes. Since that time a number of brains of persons with this disease showing gross changes of greater or lesser magnitude have been reported on, but these are not so numerous that observations on an additional one are without interest. Moreover, any unusual data in the realm of amaurotic idiocy are of value, since this condition occupies so important a position as the paradigm of heredodegeneration of the nervous system and as a definite anatomic species within the great group of the idiocies. This, we think, justifies adding the present report to a literature already abundant and not a little repetitious.

REPORT OF CASE  History.—The patient, a boy 5 years old not of Jewish parentage, was admitted to the service of one of

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