Tay Sach's disease?

Tay Sach鈥檚 disease is a genetic disorder where fat deposits build up in nerve cells leading to a slow loss of function. Since this usually strikes very young children (under 1 year old) the symptoms are not immediately apparent. Think about what this condition would cause in terms of symptoms. List some that you think are possible. What would be the inevitable end result as the disease progresses?

Tay-Sachs disease is a fatal genetic lipid storage disorder in which harmful quantities of a fatty substance called ganglioside GM2 build up in tissues and nerve cells in the brain.

The condition is caused by insufficient activity of an enzyme called beta-hexosaminidase A that catalyzes the biodegradation of acidic fatty materials known as gangliosides. Gangliosides are made and biodegraded rapidly in early life as the brain develops.

Infants with Tay-Sachs disease appear to develop normally for the first few months of life. Then, as nerve cells become distended with fatty material, a relentless deterioration of mental and physical abilities occurs.
The child becomes blind, deaf, and unable to swallow. Muscles begin to atrophy and paralysis sets in.
Other neurological symptoms include dementia, seizures, and an increased startle reflex to noise.

Even with the best of care, children with Tay-Sachs disease usually die by age 4, from recurring infection.

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