Progressive Brain Polydystrophy

Progressive brain polydystrophia (polydystrophia encephali progressiva) is a rare hereditary disease of the central nervous system, characterized by progressive brain atrophy.

The main signs of the disease: delayed psychomotor and speech development, muscle hypotonia, convulsions. With age, regression of acquired skills, mental retardation, paralysis, blindness and deafness are observed.

The cause of the disease is mutations in the POLR3A and POLR3B genes, leading to disruption of RNA and protein synthesis in brain cells. This causes progressive neuronal degeneration.

Treatment of progressive brain polydystrophy is symptomatic. The prognosis is unfavorable, death occurs in early childhood.

**Polydystrophy of the brain** (Greek poly - a lot and other Greek δυσ- τροφία - malnutrition) is a very rare hereditary disease, usually developing in childhood and characterized by progressive brain depletion in combination with dementia. In parallel with intelligence, there is a significant deterioration in cognitive

Progressive brain polydystrophy is also known as Foote's disease, a poor condition associated with a head-hydraulic cancer called basiccentric hydrocephalus. In this disease, a tumor in the brain secretes a viscous fluid that accumulates in the space between neurons and forms an area called a “brain tumor.” This makes it very difficult for the brain to function as the tissues cannot function as normal. Affected people may experience changes in memory, thinking and behavior due to deterioration in their brain function.