Which condition is associated with the progressive loss of vision in children due to lipid accumulation?

Tay-Sachs disease is a genetically inherited disorder that is characterized by the progressive accumulation of GM2 gangliosides, a type of lipid, particularly in the nerve cells of the brain and spinal cord. This accumulation occurs due to a deficiency of the enzyme hexosaminidase A, which normally breaks down these lipids. As a result, affected individuals experience severe neurological decline, including progressive vision loss, beginning in infancy.

In Tay-Sachs disease, the optic nerves can become affected due to neuronal damage, leading to vision problems that often worsen over time. Children with this condition typically show symptoms such as decreased visual acuity, an abnormal retina, and over time may also face complete blindness.

In contrast, other conditions listed do not primarily involve lipid accumulation leading to progressive vision loss in children. For example, retinopathy of prematurity is related to abnormal blood vessel development in the retina of premature infants. Diabetic retinopathy occurs as a complication of diabetes through vascular damage in adults, and Leber's congenital amaurosis is a hereditary retinal dystrophy that primarily affects the photoreceptors and is not related to lipid accumulation. Thus, Tay-Sachs disease is distinctly marked by its association with lipid metabolism and subsequent vision loss in