Tay-Sachs disease

Tay-Sachs disease  (tsks)

n.

A hereditary disease that affects young children almost exclusively of eastern European Jewish descent, in which an enzyme deficiency leads to the accumulation of gangliosides in the brain and nerve tissue, resulting in mental retardation, convulsions, blindness, and, ultimately, death.

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[After Warren Tay (1843-1927), British physician, and Bernard Sachs (1858-1944), American neurologist.]

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Tay-Sachs disease [ˌteɪˈsæks]

n

(Medicine / Pathology) an inherited disorder, caused by a faulty recessive gene, in which lipids accumulate in the brain, leading to mental retardation and blindness. It occurs mostly in Ashkenazi Jews

[named after W. Tay (1843-1927), British physician, and B. Sachs (1858-1944), US neurologist]

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Tay-Sachs disease  (tsks)

A genetic disease in which the products of fat metabolism accumulate in the nervous system, causing retardation, paralysis, and death by preschool age. Individuals of eastern European Jewish descent have a higher risk of inheriting Tay-Sachs disease. The disease is named after its describers, British ophthalmologist Warren Tay (1843-1927) and American neurologist Bernard Sachs (1858-1944).

Thesaurus

Noun

1.

Tay-Sachs disease - 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 infantile amaurotic idiocy, Sachs disease, Tay-Sachs monogenic disease, monogenic disorder - an inherited disease controlled by a single pair of genes autosomal recessive defect, autosomal recessive disease - a disease caused by the presence of two recessive mutant genes on an autosome lipidosis - a disorder of lipid metabolism; abnormal levels of certain fats accumulate in the body