Which condition is associated with progressive psychomotor retardation and large globoid bodies in white matter?

The condition associated with progressive psychomotor retardation and the presence of large globoid bodies in white matter is indeed Krabbe's Disease. This rare genetic disorder is a type of leukodystrophy caused by a deficiency of the enzyme galactocerebrosidase (GALC). This deficiency leads to an accumulation of psychosine, which is toxic to oligodendrocytes (the cells responsible for myelin production in the central nervous system).

As the disease progresses, children with Krabbe's Disease exhibit severe developmental delays, loss of milestones, and eventually profound neurological impairment, which is characterized by psychomotor retardation. The presence of globoid bodies, which are aggregates of demyelinated cellular debris, is a hallmark finding in the brain tissue of affected individuals, particularly visible at postmortem examination. These globoid bodies are formed as a result of the breakdown of myelin due to the ongoing neurodegeneration and are indicative of the underlying pathology related to oligodendrocyte dysfunction.

The other conditions listed do not present with this specific combination of symptoms and pathological findings. Fabry's Disease primarily affects the kidneys and heart, Gaucher's Disease typically involves splenomegaly, bone pain, and various