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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.
Niemann-Pick Disease Type A (NPA) is an extremely rare lysosomal storage disorder, which causes neurodegeneration. The science of it is that children produce less than 1% of the enzyme acid sphingomyelinase (ASM) required to metabolize a lipid (fat) called sphingomyelin.