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
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:
weakness or twitching.
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.
Sarah Marshall, MD - Family Medicine & Siobhan M. Dolan, MD, MPH - Reproductive Genetics
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