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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 build up in the body 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 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.

By Healthwise Staff
Primary Medical Reviewer Sarah Marshall, MD - Family Medicine
Specialist Medical Reviewer Siobhan M. Dolan, MD, MPH - Reproductive Genetics
Last Revised September 6, 2011

WebMD Medical Reference from Healthwise

Last Updated: September 06, 2011
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