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Infantile amaurotic idiocy $(Tay-Sachs disease)$ is
The result of severe protein deficiency in diet and damages the muscle development in growing children
An inherited disorder in which the spinal cord and brain are severely damaged within a few months of birth leading to paralysis and mental retardation, and finally the infant's death in $3$ or $4$ years
A genetic abnormality which appears at the age of $25-30$ years and damages the bones
A chromosomal disorder in young children
Solution
(b) Tay-Sachs disease is the most common $G{M_2}$ gangliosidosis, occuring almost exclusively among north-east European Jews and specifically characterized by infantile onset ($3-6$ months), doll-like facies, cherry red macular spot ($90 +$ percent of the infants) early blindness, hyperacusis, macrocephaly, siezures and hypotonia; the children die between $2$ and $5$ years of age. It is caused by the deficiency of hexosaminidase A enzyme in tissue.
