Which condition is specifically associated with progressive cognitive decline and visual problems starting in childhood?

Batten Disease is a neurodegenerative disorder that specifically manifests in childhood and is characterized by progressive cognitive decline and visual problems. This condition is classified under neuronal ceroid lipofuscinoses and typically begins between the ages of 5 and 10 years. One of the hallmark features of Batten Disease is the gradual loss of vision due to retinal degeneration, coupled with cognitive impairments that worsen over time, leading to significant developmental delays and eventual loss of motor abilities.

The other conditions listed, while they may involve neurological or metabolic issues, do not begin specifically in childhood with the same pattern of cognitive decline and visual problems as Batten Disease. For example, Fabry Disease primarily affects the kidneys and heart and has other systemic issues rather than early childhood cognitive deterioration. Ornithine Transcarbamylase Deficiency is a urea cycle disorder resulting in ammonia accumulation but typically presents with metabolic crises rather than specific cognitive and visual decline in early childhood. Methylmalonic Acidemia primarily impacts metabolic processes and can lead to developmental delays but does not present in a manner specifically associated with cognitive decline and visual problems starting in childhood.