Tay-Sachs Disease and Sandhoff Disease By Matt Demczko, MD, Mitochondrial Medicine, Children's Hospital of Philadelphia Reviewed / Revised Oct 2021 | Modified Sep 2022 View Patient Education More Information Topic Resources Tay-Sachs Disease (Cherry-Red.. Tay-Sachs disease and Sandhoff disease are sphingolipidoses, inherited disorders of metabolism, caused by hexosaminidase deficiency that causes severe neurologic symptoms and early death.