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.
Individuals with Tay-Sachs Disease lack an enzyme called hexosaminidase (Hex A). The child loses all motor skills and becomes blind, deaf and unresponsive. Death usually occurs by the age of four. The carrier rate in the Ashkenazi Jewish population is approximately 1 in 25. More rare than the infantile type is Late Onset Tay-Sachs Disease, where the progression of symptoms is slower and milder.