Any disorder of LIPID metabolism in body cells. Some hereditary disorders cause deposition of lipids within the brain.
(lipoidosis) n. (pl. lipidoses) any disorder of lipid metabolism within the cells of the body. The brain lipidoses (see Gaucher’s disease; Hunter’s syndrome; Hurler’s syndrome; Tay–Sachs disease) are inborn defects causing the accumulation of lipids within the brain.
(amaurotic familial idiocy) an inherited disorder of lipid metabolism (see lipidosis) in which abnormal accumulation of lipid in the brain leads to blindness, learning disabilities, and death in infancy. The gene responsible for the disorder is *recessive, and the disease can now be largely prevented by genetic counselling in communities known to be affected (primarily Ashkenazi Jewish communities). [W. Tay (1843–1927), British physician; B. Sachs (1858–1944), US neurologist]... tay–sachs disease
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