What syndrome result in a eaaaaarly child hood death?

One syndrome that can result in early childhood death is Tay-Sachs disease, a genetic disorder caused by a deficiency of the enzyme hexosaminidase A. This leads to the accumulation of GM2 gangliOSides in the nerve cells, resulting in severe neurological deterioration. Affected children typically show symptoms by six months of age and often do not survive beyond early childhood, usually succumbing to complications of the disease by age four or five. Other similar syndromes include Sandhoff disease and infantile spinal muscular atrophy, which also lead to early mortality.