Spongy degeneration of white matter in brain with relative axonal preservation and Alzheimer type II astrocytes in gray matter; lso with spasticity, seizures, psychomotor degeneration and macrocephaly. Lesions are located in central white matter and occasionally in cerebellum and basal ganglia; associated with aspartoacyclase deficiency. Autosomal recessive condition that usu. presents in mid- to late infancy; occurs more commonly in Jews and tends to be associated with lighter complexion and hair. Disease may progress to death in several months to years.