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July 1930


Author Affiliations

Professor of Neurology, College of Medicine, University of Illinois; Histologist to Illinois State Psychopathic Institute CHICAGO

From the Pathologic Laboratories of the Research and Educational Hospitals of the University of Illinois and the State Psychopathic Institute.

Arch NeurPsych. 1930;24(1):61-70. doi:10.1001/archneurpsyc.1930.02220130064006

Some clinical manifestations of Niemann-Pick's disease, particularly those pertaining to the central nervous system, so much resemble the infantile form (type Tay-Sachs) of amaurotic family idiocy that the question naturally arises whether these two morbid conditions are not one disease. In a very few instances1 it was possible to show their identity by pathologic studies, while in rare instances it was possible to prove the presence in Niemann-Pick's disease of a cherry-red spot which is pathognomonic of the infantile form of amaurotic family idiocy. Though in the case recorded here such a spot was absent, the histologic as well as the gross changes were decidedly those of amaurotic family idiocy. There were some differences, which will be pointed out.

The clinical and general pathologic features of this case were typical of Niemann-Pick's disease. They will be recorded by Dr. Poncher and Dr. Boikan, while Mr. MacFate will report his