Relationship of Cognitive Functioning,Whole Brain Volumes,and T2-Weighted Hyperintensities in Neurofibromatosis-1

Abstract

Using quantitative magnetic resonance imaging morphometry, we report that the whole brain volumes of patients with neurofibromatosis-1 are significantly larger than normal, confirm the prevalence of macrocephaly as about 50%, and report that macrocephaly in patients with neurofibromatosis-1 does not appear to be related to the familial or sporadic origin of the neurofibromatosis-1 or to the presence or absence of T₂-weighted hyperintensities. No strong relationship emerged between the extent of neurofibromatosis-1-associated impairment of cognitive functions and degree of macrocephaly; however, the macrocephalic neurofibromatosis-1 group did have a significant verbal impairment relative to the non-macrocephalic neurofibromatosis-1 group in vocabulary (P < .009). (J Child Neurol 2000;15:157-160).