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

Developmental Cognitive Neurology, Johns Hopkins School of Medicine, Baltimore, MD, cutting{at}kennedykrieger.org, Department of Neurology, Johns Hopkins School of Medicine, Baltimore, MD

Christine W. Koth, MS

Developmental Cognitive Neurology, Johns Hopkins School of Medicine, Baltimore, MD

Courtney P. Burnette, BA

Neuroimaging Laboratory Kennedy Krieger Institute, Johns Hopkins School of Medicine, Baltimore, MD

Michael T. Abrams, BA

Neuroimaging Laboratory Kennedy Krieger Institute, Johns Hopkins School of Medicine, Baltimore, MD, Department of Psychiatry and Behavioral Sciences, Johns Hopkins School of Medicine, Baltimore, MD

Walter E. Kaufmann, MD

Department of Neurology, Johns Hopkins School of Medicine, Baltimore, MD, Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, MD, Department of Psychiatry and Behavioral Sciences, Johns Hopkins School of Medicine, Baltimore, MD, Department of Pathology Johns Hopkins School of Medicine, Baltimore, MD

Martha Bridge Denckla, MD

Developmental Cognitive Neurology, Johns Hopkins School of Medicine, Baltimore, MD, Department of Neurology, Johns Hopkins School of Medicine, Baltimore, MD, Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, MD, Department of Psychiatry and Behavioral Sciences, Johns Hopkins School of Medicine, Baltimore, MD

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).

Journal of Child Neurology, Vol. 15, No. 3, 157-160 (2000)
DOI: 10.1177/088307380001500303


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