Notice: Function _load_textdomain_just_in_time was called incorrectly. Translation loading for the mypractis domain was triggered too early. This is usually an indicator for some code in the plugin or theme running too early. Translations should be loaded at the init action or later. Please see Debugging in WordPress for more information. (This message was added in version 6.7.0.) in /var/www/vhosts/obgynofakron.com/httpdocs/wp-includes/functions.php on line 6114

Notice: Function _load_textdomain_just_in_time was called incorrectly. Translation loading for the uael domain was triggered too early. This is usually an indicator for some code in the plugin or theme running too early. Translations should be loaded at the init action or later. Please see Debugging in WordPress for more information. (This message was added in version 6.7.0.) in /var/www/vhosts/obgynofakron.com/httpdocs/wp-includes/functions.php on line 6114

Notice: Function _load_textdomain_just_in_time was called incorrectly. Translation loading for the astra-addon domain was triggered too early. This is usually an indicator for some code in the plugin or theme running too early. Translations should be loaded at the init action or later. Please see Debugging in WordPress for more information. (This message was added in version 6.7.0.) in /var/www/vhosts/obgynofakron.com/httpdocs/wp-includes/functions.php on line 6114
Late-onset Tay-Sachs disease (LOTS) - OBGYN Associates

Our Health Library information does not replace the advice of a doctor. Please be advised that this information is made available to assist our patients to learn more about their health. Our providers may not see and/or treat all topics found herein.

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

Author: Ignite Healthwise, LLC Staff

Clinical Review Board
All Healthwise education is reviewed by a team that includes physicians, nurses, advanced practitioners, registered dieticians, and other healthcare professionals.