Late-onset Tay-Sachs disease (LOTS)
Late-onset Tay-Sachs (LOTS) is a very rare genetic disease in which fatty compounds, called gangliosides, do not break down fully because the body produces too little of the enzyme hexosaminidase A (Hex A). Over time, gangliosides build up in the brain and damage brain nerve cells. This affects a person's mental functioning.
This condition 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 occur 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.
Current as of: July 31, 2024