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 who have family from the quebec bas-st-laurent or gaspésie regions or new brunswick territories 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.

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late-onset tay-sachs disease (lots)

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