title: 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 nonmacrocephalic neurofibromatosis-1 group in vocabulary (P < .009).
authors.affiliation: Developmental Cognitive Neurology, Kennedy Krieger Institute, Baltimore, MD 21205, USA. cutting@kennedykrieger.org
authors.forename: L E
authors.initials: LE
authors.lastname: Cutting
authors.forename: C W
authors.initials: CW
authors.lastname: Koth
authors.forename: C P
authors.initials: CP
authors.lastname: Burnette
authors.forename: M T
authors.initials: MT
authors.lastname: Abrams
authors.forename: W E
authors.initials: WE
authors.lastname: Kaufmann
authors.forename: M B
authors.initials: MB
authors.lastname: Denckla
mesh_terms: D000293:Adolescent; D001921:Brain / Q000473:pathology*; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000503:physiopathology / Q000523:psychology; D002508:Cephalometry; D002648:Child; D006801:Humans; D007360:Intelligence / Q000502:physiology*; D007805:Language Development Disorders / Q000175:diagnosis* / Q000503:physiopathology / Q000523:psychology; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000503:physiopathology / Q000523:psychology; D012016:Reference Values; D014888:Wechsler Scales