Whereas the content standards will provide the topic of the lesson and what exactly the students should be doing with that topic (e.g., solving problems, creating models, ranking ideas), the English language proficiency or development standards help to identify language skills and functions that students should be working on to achieve academic language fluency.
Tay-Sachs disease is a gangliosidosis characterised by: An exaggerated startle response. Delay in psychomotor development. Hypotonia (followed by spasticity) Visual loss. A macular cherry-red spot. Pathogenesis. Tay-Sachs disease (and its variants) are caused by absence or defects of the alpha subunit of hexosaminidase A.
Tay-Sachs disease is a rare disorder passed from parents to child. In the most common form, a baby about 6 months old will begin to show symptoms. Tay-Sachs disease results when an enzyme that helps break down fatty substances is absent. These fatty substances build up to toxic levels in the child's brain and affect the nerve cells. As the disease progresses, the child's body loses function, leading to blindness, deafness, paralysis and death. There is no cure for Tay-Sachs disease. Gene therapy or enzyme replacement therapy research may eventually lead to a cure or treatment to slow the progression of Tay-Sachs disease.