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The Subacute Form of Niemann-Pick Disease
J. A. Lowden, MD, PhD;
M. A. LaRamée, BS;
P. Wentworth, MD, MB, BCh
Arch Neurol. 1967;17(3):230-237.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings. |
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NIEMANN-PICK disease is a progressive, familial disorder of childhood and is characterized by hepatosplenomegaly and cerebral degeneration. Increased amounts of sphingolipids are found in lungs, liver, spleen, brain, and most other organs of the body. These organs are filled with large, pale, foamy cells which stain positively with lipid stains. About 150 cases have now been reported in clinical, pathological, and, rarely, in chemical studies. The largest group is a series of 18 cases described by Crocker and Farber.1 Crocker2 has divided this series into four distinct clinical types: the classic infantile; the infantile without neurological involvement; the more slowly progressive or juvenile type; and a less clearly defined group of adolescent, Roman Catholic, Nova Scotians. However, Terry et al3 have described a rapidly progressive adult form of Niemann-Pick disease, and Pfändler4 also lists a group of adults who probably have this illness. Thus, Crocker's
. . . [Full Text PDF of this Article]
Author Affiliations
Toronto
From the Department of Paediatrics, University of Toronto and the Research Institute, the Hospital for Sick Children, Toronto.
Footnotes
Submitted for publication March 23, 1967; accepted April 19.
Read in part at the annual meeting of the Canadian Society for Clinical Investigation, Ottawa, Jan 19, 1967.
Reprint requests to the Research Institute, the Hospital for Sick Children, 555 University Ave, Toronto, Ontario (Dr. Lowden).
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