WHITE MATTER DYSFUNCTION AND ITS NEUROPSYCHOLOGICAL CORRELATES - A LONGITUDINAL-STUDY OF A CASE OF METACHROMATIC LEUKODYSTROPHY TREATED WITH BONE-MARROW TRANSPLANT

A 10-year-old white female who had received a bone marrow transplant (BMT) at 57 months of age as treatment for late infantile onset metachromatic leukodystrophy (MLD), a neurodegenerative autosomal recessive storage disease, showed stabilization of the cognitive degenerative process and demonstrated a partial pattern of cognitive deficits and behavioral abnormalities that has been called NLD (nonverbal learning disabilities) associated with white matter disease. A pattern of good rote memory, reading skills, and concrete language contrasted with poor visual spatial skills, mathematics, and abstract problem solving. She did not show the usual speech prosody and social deficits associated with NLD.