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Tay-Sachs disease An inherited condition characterized by the absence of a single lysosomal enzyme called Hex A, which normally breaks down a membrane glycolipid called ganglioside G_M2 that is especially prevalent in nerve cells; usually occurs in children of Ashkenazi (eastern European Jewish) descent and is characterized by seizures and muscle rigidity.
(wÄ­lâ²sÉn) or WilÂ·son's disease (-sÉnz) n. A rare genetic disease caused by a defect in copper metabolism, resulting in the accumulation of copper in the liver, brain, kidneys, and cornea and causing neurological symptoms and liver disease. [ 1 After Samuel Alexander Kinnier Wilson (1877-1937), British neurologist.]