(n) a hereditary disorder of lipid metabolism occurring most frequently in individuals of Jewish descent in eastern Europe; accumulation of lipids in nervous tissue results in death in early childhood, Syn.infantile amaurotic idiocy, Tay-Sachs, Sachs disease
The Collaborative International Dictionary of English (GCIDE) v.0.53
n. (Med.) A rare hereditary disease affecting lipid metabolism in humans, due to a deficiency of hexosaminidase. It occurs in infants and children, and causes death before the onset of adulthood. It occurs most commonly of people of Jewish origin from easter Europe. It is characterized by accumulation of lipids in nervous tissue, causes a red spot on the retina, and eventual blindness and paralysis before death. [ PJC ]
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