« Previous Page

Late-Onset Tay-Sachs Disease

Late-Onset Tay-Sachs Disease

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:

  • 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.

Last Revised: June 20, 2013

Author: Healthwise Staff

Medical Review: Sarah Marshall, MD - Family Medicine & Siobhan M. Dolan, MD, MPH - Reproductive Genetics

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use. How this information was developed to help you make better health decisions.

© 1995-2014 Healthwise, Incorporated. Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.

× Alternate Text