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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.
Individuals with Tay-Sachs Disease lack an enzyme called hexosaminidase (Hex A). The child loses all motor skills and becomes blind, deaf and unresponsive. Death usually occurs by the age of four. The carrier rate in the Ashkenazi Jewish population is approximately 1 in 25. More rare than the infantile type is Late Onset Tay-Sachs Disease, where the progression of symptoms is slower and milder.