Tay-Sachs disease – causes, side effects and treatments at NaturalPedia.com

Monday, July 02, 2018 by

People with Taye-Sachs disease do not possess hexosaminidase A, an enzyme which helps break down a group of chemicals called gangliosides. In particular, this will result in ganglioside build up in nerve endings, often occurring in nerves in the brain. The condition is potentially life-threatening, and it is passed down through families.

The condition is prevalent among the Ashkenazi Jewish population, where one in 27 people carries the Tay-Sachs gene. If both parents have the Taye-Sachs gene, their offspring will have a 25 percent chance of getting the condition. However, if only one parent has the gene, the disease will not manifest in their offspring; rather, the child will be a carrier of the gene, which will remain dormant until he sires children from a Taye-Sachs carrier.

The symptoms of Taye-Sachs disease are usually seen in infants three to six months old; however, the onset of nerve damage can occur even earlier during the fetal development stage. An infant with Taye-Sachs will usually die by the age of four or five. It’s rare to see the condition develop during adulthood.

Known risk factors and symptoms of Tay-Sachs disease

Certain ethnic groups have an increased risk of Tay-Sachs disease, including Ashkenazi Jews (as well as those from Eastern and Central Europe), those from certain French-Canadian communities in Quebec, Old Order Amish groups in Pennsylvania, and Cajuns from Louisiana.

Taye-Sachs disease is categorized into three forms, which represent the age group affected by the condition.

  • Infantile Tay-Sachs disease is the most common, and the most severe, form of hexosaminidase A deficiency. In this form, Taye-Sachs disease progresses rapidly, and it can result in severe physical and mental impairment. Typical symptoms of infantile Taye-Sachs disease include mild muscle weakness, twitching or jerking of muscles (myoclonic jerks), and an exaggerated response to being startled, such as when there is a loud or unexpected noise. As the infant grows, he will not gain new motor skill and may experience diminished muscle weakness, as well as diminished muscle tone (hypotonia) and reduce mental function. Cherry red spots will also start to form in the eyes, a defining characteristic of  Taye-Sachs disease in infants. The body will continue to deteriorate, and life-threatening complications occur by age three.
  • Juvenile Taye-Sachs disease is a condition which develops in children between two to 10 years old. Symptoms in this age include difficulty controlling body movement (ataxia), behavioral problems, and issues with their intellectual ability. Severe complications will occur by the age of 15.
  • Late-onset Taye-Sachs disease is a rare occurrence. People with this condition experience mood alterations, clumsiness, and even dystonia, a condition where the body may involuntarily move into unusual and painful movements and positions. As the disease progresses, this can result in the person requiring assistive devices to function.

Body systems affected by Tay-Sachs disease

Tay-Sachs disease is a progressive neurodegenerative disorder that severely impairs a person’s movement.

Food items or nutrients that may prevent Tay-Sachs disease

No information is available on food items that may treat or prevent Tay-Sachs disease. However, ensuring that a healthy nervous system can help boost resistance against neurodegenerative disorders such as Tay-Sachs disease. Some food items to include fish, which are high in omega-3 fatty acids, green leafy vegetables, and sea vegetables like seaweed.

Treatment and management options for Tay-Sachs disease

No treatment exists for Tay-Sachs disease, and most healthcare professionals will aim for palliative care when dealing with the condition.

Where to learn more

Summary

Taye-Sachs disease is a condition where a person does not possess the enzyme hexosaminidase A, leading to ganglioside build up in nerve endings.

Taye-Sachs disease is seen in infants three to six months old. An infant with Taye-Sachs will usually die by the age of four or five.

No treatment exists for Tay-Sachs disease, and most healthcare professionals will aim for palliative care when dealing with the condition.

Sources include:

MedLinePlus.gov

MayClinic.org

RareDiseases.org

Patient.info

Livestrong.com

NHSDirect.Wales.NHS.uk



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