MRI AND NONVERBAL COGNITIVE DEFICITS IN CHILDREN WITH NEUROFIBROMATOSIS-1

Magnetic resonance imaging brain scans and neuropsychological assessme nts of 17 children who met the NIH consensus diagnostic criteria for n eurofibromatosis Type 1 were carried out in order to determine if ther e is a relationship between presence of high intensity signal abnormal ities on MRI scans and nonverbal cognitive deficits. Cranial MRI scans in 10 patients (55.8%) demonstrated high intensity signal abnormaliti es, most frequently in the cerebral peduncles. Fifteen patients had no nverbal cognitive deficits (88.2%), including difficulty judging the o rientation of lines, matching complex visual stimulus configurations, recalling pictures of faces, as well as copying and drawing from memor y a complex geometric figure. There was not a significant association between nonverbal neuropsychological deficits and presence of high int ensity signal abnormalities on MRI scans, possibly because the locatio n of these hyperintense abnormalities was typically below the level of the basal ganglia. These findings suggest that the high intensity sig nal lesions seen on the MRI scans of children with neurofibromatosis T ype 1 do not predict or explain their nonverbal cognitive deficits.