This results in the synthesis of oligoclonal IgG by plasma cells. Rare, but becoming more common. Progressive with profound dementia and coma within 6 months of symptoms. Diverse but reflect disease in cerebral hemispheres. Multiple foci of demyelination that progressively increase in size and number.
Lesions are noninflammatory.
Oligodendroglia and myelin absent from lesions. 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.