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Symptoms associated with Tay-Sachs disease may include an exaggerated startle response to sudden noises, listlessness, loss of previously acquired skills (i.e., psychomotor regression), and severely diminished muscle tone (hypotonia).ay Sachs Disease. Important. It is possible that the main title of the report Tay Sachs Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Tay-Sachs disease (TSD) is a fatal genetic disorder, most commonly occurring in children, that results in progressive destruction of the nervous system. Tay-Sachs is caused by the absence of a vital enzyme called hexosaminidase-A (Hex-A).s for the childhood form of Tay-Sachs, there is no cure. Treatment involves managing the symptoms of the disease. Defect in Hex-A Gene Causes Tay-Sachs: Tay-Sachs disease results from defects in a gene on chromosome 15 that codes for production of the enzyme Hex-A.