n : a hereditary disorder of lipid metabolism that typically affects individuals of eastern European Jewish ancestry, that is marked by the accumulation of lipids esp. in nervous tissue due to a deficiency of hexosaminidase A, that is characterized by weakness, macrocephaly, red retinal spots, hyperacusis, retarded development, blindness, convulsions, paralysis, and death in early childhood, and that is inherited as an autosomal recessive trait - called also infantile amaurotic idiocy , ay-Sachs - see SANDHOFF'S DISEASE - compare GAUCHER'S DISEASE NIEMANN-PICK DISEASE SPIELMEYER-VOGT DISEASE
Tay, Warren (1843-1927), British physician. Tay specialized in ophthalmology, dermatology, and pediatrics. In 1881 he described a degenerative condition of the choroid found in a type of amaurotic idiocy.
Sachs, Bernard (1858-1944), American neurologist. Sachs was a neurologist associated with several New York City hospitals. In 1887 he published a comprehensive description of a form of amaurotic idiocy. His observations were made independently of Tay. The condition is known as Tay-Sachs disease as an acknowledgment of Sachs' later but more comprehensive account.
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