Related Terms

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Background

• Tay-Sachs disease (TSD) is a rare inherited disorder that progressively destroys the brain and nervous system. The body progressively loses basic functions, leading to deafness, blindness, and paralysis. Individuals with TSD usually do not live beyond five years of age. Infection is a common cause of death in TSD patients.
• TSD is caused by mutations in the HEXA gene. This gene contains instructions for making the hexosaminidase A enzyme, which plays an important role in maintaining the central nervous system. The central nervous system, which controls many of the bodily functions, is eventually destroyed by TSD. Because it affects the nervous system, TSD is classified as a neurological disease.
• TSD is named after British ophthalmologist Warren Tay and American physician Bernard Sachs, who independently described the symptoms of what would later be known as infantile TSD. In 1881, Tay described eye defects, which are characteristic of TSD, in an infant with a progressive neurological disease. In 1887, Sachs presented his observations of a disease that was prevalent among German immigrants of Jewish heritage.
• TSD is a recessive genetic condition, which means that the mutation must be inherited from both parents for the disease to occur. People who have inherited the mutation from only one parent do not have TSD, but are called "carriers" because they may pass the disease on to their children. Historically, certain populations (such as French Canadians and those of Ashkenazi Jewish descent) had a higher proportion of carriers and thus a higher incidence of TSD.
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Risk Factors

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Causes

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Signs and Symptoms

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Types of the Disease

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Diagnosis

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Complications

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Treatment

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Integrative Therapies

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Prevention

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Author Information

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References

Natural Standard developed the above evidence-based information based on a thorough systematic review of the available scientific articles. For comprehensive information about alternative and complementary therapies on the professional level, go to www.naturalstandard.com. Selected references are listed below.

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