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Robert S. Greenwood, MD; Larry A. Tupler, PhD; J. Kenneth Whitt, PhD; Anne Buu, PhD; Carrie B. Dombeck, BS; Amanda G. Harp; Martha E. Payne, MPH, RD; James D. Eastwood, MD; K. Ranga R. Krishnan, MD; James R. MacFall, PhD

Arch Neurol. 2005;62:1904-1908.

Background  Larger gray matter (GM) volume in healthy children is correlated with higher IQ. Children with neurofibromatosis type 1 (NF1) have larger brains, their magnetic resonance images frequently show T2-weighted hyperintensities, and their IQs are lower.

Objectives  To confirm the hypotheses that (1) children with NF1 have larger GM and white matter volumes, (2) the greatest volume differences are in the frontal and parietal regions and in children with NF1 with hyperintensities, and (3) GM volume is inversely related to IQ in children with NF1.

Design  Wechsler Intelligence Scale for Children–Third Edition IQ testing and measurement of cerebral volumes and hyperintensities in brain magnetic resonance images were performed on 36 children with NF1 and on 36 matched relatives who served as control subjects.

Results  Gray matter and white matter volumes were significantly larger in children with NF1. The greatest difference was observed in cerebral white matter volume, predominantly in the frontal lobes, whereas the greatest difference in GM volume was in the temporal, parietal, and occipital regions. In controls, IQ was significantly related to GM volume, but in children with NF1, IQ was not inversely associated with GM volume, although IQs of children with NF1 were significantly lower.

Conclusions  Children with NF1 do not have the normal relationship between GM volume and IQ. Larger GM volume in the posterior brain regions and larger white matter volumes in the frontal brain regions contribute to the larger brain volume in children with NF1.

Author Affiliations: Departments of Neurology (Dr Greenwood and Ms Harp), Pediatrics (Drs Greenwood and Whitt and Ms Harp), and Psychiatry (Dr Whitt), University of North Carolina School of Medicine, and Curriculum of Neurobiology and Neurodevelopmental Disorders Center (Dr Greenwood), The University of North Carolina at Chapel Hill; Departments of Psychiatry and Behavioral Sciences (Drs Tupler and Krishnan and Mss Dombeck and Payne) and Radiology (Drs Eastwood and MacFall), Duke University Medical Center, Durham, NC; and Department of Statistics, University of Michigan at Ann Arbor (Dr Buu).

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