Alphabetic Guide to Child Care - Tay-sachs disease

Tay-Sachs Disease

Tay-Sachs disease is an inherited disorder of the nervous system. It causes degeneration of the nerves, starting after six months of age. Infant development through pregnancy and the first six months is normal, but the disease can be detected through genetic testing. The first symptoms to appear are subtle, becoming more obvious with motor deterioration. They include a slowing of development, loss of vision, and eventually convulsions. A cherry-red spot develops on the retina of the eye. The child will lose the motor coordination that he already had, and will deteriorate to the point where he will be unable to lift his head. The child will not normally survive past the age of four.

Tay-Sachs is found with higher prevalence among the following populations: Ashkenazi Jews, Eastern European Jews, and French Canadians. Testing is recommended to anyone from these populations, and testing is also recommended for all Jews to determine if one is a carrier of the disease.

Tay-Sachs is a form of cerebral sphingolipidosis. It is the infantile onset of the disorder. The others are Jansky-Bielschowsky disease (early juvenile), Spielmeyer-Vogt and Batten-Mayou disease (late juvenile), and Kufs’ disease (adult onset). Symptoms are similar and include blindness within two years of the onset of the disorder.

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