JUVENILE AMAUROTIC IDIOCY
- 1 July 1934
- journal article
- other
- Published by American Medical Association (AMA) in Archives of Neurology & Psychiatry
- Vol. 32 (1) , 198-209
- https://doi.org/10.1001/archneurpsyc.1934.02250070204013
Abstract
Amaurotic idiocy assumed a new significance when Bielschowsky1demonstrated its occurrence with Niemann-Pick's disease. He expressed the belief that both diseases were the result of the same pathologic factor, that is, a disturbance of the lipoid metabolism. On the other hand, the name given by Bloom,2"lipoid histiocytosis," signifies that Niemann-Pick's disease is a primary pathologic condition of the mesoderm, while amaurotic idiocy has been considered a primary disease of the neuroderm. In 1928, Abt and Bloom3were able to collect reports of fifteen cases of Niemann-Pick's disease, but in only two was there record of an examination of the brain. Since that time the association of amaurotic idiocy with Niemann-Pick's disease has been described by Wascowitz,4Hassin,5Poncher6and others. Kufs7concurred with Bielschowsky1in the point of view that amaurotic idiocy is part of Niemann-Pick's disease, but he believed that varyingKeywords
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