Relationship of cognitive functioning, whole brain volumes, and T2-weighted hyperintensities in neurofibromatosis-1.
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).
Cutting
L E
LE
Developmental Cognitive Neurology, Kennedy Krieger Institute, Baltimore, MD 21205, USA. cutting@kennedykrieger.org
Koth
C W
CW
Burnette
C P
CP
Abrams
M T
MT
Kaufmann
W E
WE
Denckla
M B
MB
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