GARGOYLISM (LIPOCHONDRODYSTROPHY)

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Abstract
Gargoylism is . an heredofamilial disease characterized by chondrody-strophic skeletal changes, corneal opacities, mental deficiency and hepatosplenomegaly. The essential pathologic change in the nervous system is a swelling of the nerve cells with intracellular accumulation of lipid material. Similar alterations are found in the extra-axial nerve cells and the wall of the intestinal tract. The similarity of the changes in this disease to those in amaurotic family idiocy suggests that alterations in the nervous system in gargoylism, as in amaurotic idiocy, are due to an inherent disturbance of lipid metabolism.

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