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September 1967

The Subacute Form of Niemann-Pick Disease

Author Affiliations

From the Department of Paediatrics, University of Toronto and the Research Institute, the Hospital for Sick Children, Toronto.

Arch Neurol. 1967;17(3):230-237. doi:10.1001/archneur.1967.00470270008002

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

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