Late-onset Tay-Sachs disease
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Late-onset Tay-Sachs disease
Late-onset Tay-Sachs (LOTS) is a rare genetic disease in which fatty compounds, called gangliosides, do not break down normally because the body produces too little of the enzyme hexosaminidase A (or hex A). Gradually, gangliosides accumulate and damage brain and nerve cells, which affects a person's mental functioning.
This condition is a recently discovered form of Tay-Sachs disease and occurs most often in people of Ashkenazi Jewish descent. People of French-Canadian or Cajun descent are also at a higher risk than the general population.
Symptoms of LOTS vary but usually include clumsiness or mood changes that begin between adolescence and the mid-30s. At first, symptoms are subtle and may go unnoticed. Other symptoms that may develop include:
- Personality changes.
- Muscle weakness or twitching.
- Slurred speech.
- Impaired thinking and reasoning ability, such as memory problems, difficulty with comprehension, and short attention span.
- Inability to distinguish between what is real and unreal (psychotic episodes) or depression.
Treatment for late-onset Tay-Sachs disease focuses on controlling symptoms. There is no cure. The life expectancy for a person with LOTS is unknown. Depending on the severity of the symptoms, the person may live as long as someone who does not have the disease.
Credits
| Author | Jeannette Curtis |
| Author | Caroline Rea, RN, BS, MS |
| Editor | Kathleen M. Ariss, MS |
| Editor | Susan Van Houten, RN, BSN, MBA |
| Associate Editor | Tracy Landauer |
| Associate Editor | Pat Truman, MATC |
| Primary Medical Reviewer | Patrice Burgess, MD - Family Medicine |
| Primary Medical Reviewer | Adam Husney, MD - Family Medicine |
| Primary Medical Reviewer | Kathleen Romito, MD - Family Medicine |
| Last Updated | May 25, 2007 |
| Last updated: | May 25, 2007 |
|---|---|
| Author: | Caroline Rea, RN, BS, MS |
| Reviewed By: | Kathleen Romito, MD - Family Medicine |
| Editors: | Susan Van Houten, RN, BSN, MBA, Pat Truman, MATC |
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