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Late-onset Tay-Sachs (LOTS) is a very 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). Over time, gangliosides build up in the brain and damage brain nerve cells. This 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:
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.
Last Revised: June 20, 2013
Author: Healthwise Staff
Medical Review: Sarah Marshall, MD - Family Medicine & Siobhan M. Dolan, MD, MPH - Reproductive Genetics
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