Papova virus infection of oligodendroglia. Peripheral and central white matter disorder with age of onset between 12 and 18 months. Autosomal recessive inherited dysmyelinating disorder characterized by an intralysosomal storage of sulfatides. Extensive involvement of the entire white matter. Inclusions in macrophages, neurons, Schwann cells and other organ systems.
Inclusions are accumulations of sulfatides.
Deficiency of Arylsulfatase A, the enzyme that catabolizes sulfatides. Newly formed myelin unstable due to the increased amounts of sulfatides present. Mutant enzyme present but no activity. Metachromatic bodies found in the urine. Degenerative white matter disease presenting between 3 and 6 months of age.