1. 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
   "The genetic counsellor discussed the risks of infantile amaurotic idiocy with the expecting parents";
   - Tay-Sachs disease, Tay-Sachs, Sachs disease

Derived forms: infantile amaurotic idiocies

Type of: autosomal recessive defect, autosomal recessive disease, lipidosis, monogenic disease, monogenic disorder