An inherited genetic disorder involving an autosomal recessive condition that causes progressive destruction of the central nervous system through the accumulation of lipids due to the absence of a vital enzyme called hexosaminidase that is important in the metabolism of gangliosides (fatty substances necessary for brain development). It is acquired early in gestation, but does not manifest itself until the child is several months old. A fatal disease, with death usually occurring by 5 years of age, it is most frequent among individuals of Eastern European Jewish (Ashkenazi) descent, with 1 in 25 of them carrying the gene
. See Autosomal dominant condition/disease