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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. Tay-Sachs disease (the most severe form of Hexosaminidase A deficiency) is a progressive, fatal genetic condition that affects the nerve cells in the brain.eople with Tay-Sachs lack a specific protein (enzyme) called hexosaminidase A. This enzyme deficiency causes a fatty substance, GM2 ganglioside, to build up in the brain. It is this accumulation that causes the symptoms of Tay-Sachs.