NEUROPSYCHOLOGICAL PROFILE OF CHILDREN WITH NEUROFIBROMATOSIS, BRAIN-TUMOR, OR BOTH

Neurofibromatosis type 1, a common autosomal dominant genetic disorder , is associated with numerous physical and medical anomalies as well a s an increased incidence of learning disability. Tumors of the central nervous system have been estimated to occur in 15%, but their contrib ution to neuropsychological status is unknown. This study examines the relative contribution of neurofibromatosis and brain tumor to the cog nitive profile of children with neurofibromatosis. A comprehensive bat tery of neuropsychological and behavioral tests was administered to a group of 65 children with neurofibromatosis type 1. Fourteen were then matched on demographic variables with two other groups of children wh o had either a brain tumor in addition to neurofibromatosis or a brain tumor alone. The two brain tumor groups were also matched on tumor ty pe, location, and therapy. Mean scores of the neurofibromatosis-brain tumor group were generally the lowest of the three groups; those of th e brain tumor group were highest, and performance of the neurofibromat osis group was generally between the other two groups. These results s uggest that neurofibromatosis is, by itself, associated with significa nt cognitive morbidity, but that the severity of the problems is incre ased somewhat if a brain tumor is also present.