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Francis J. DiMario, Jr, MD; Gale R. Ramsby, MD; Joseph A. Burleson, PhD

Arch Neurol. 1999;56:1343-1346.

Rationale and Objectives  To investigate the relationships between brain and skull base growth in patients with neurofibromatosis 1 (NF1) compared with healthy control subjects using brain magnetic resonance imaging (MRI) for morphometric analysis.

Methods  Evaluated patients included children who underwent T₁- and T₂-weighted or dual-echo proton density axial and T₁-weighted sagittal brain MRI from January 1, 1988, to December 31, 1995. Study subjects (n=27) received a diagnosis of NF1 by accepted National Institutes of Health clinical criteria and were compared with an age- and sex-matched control group (n=43). Twenty-four predetermined ventricular and brain parenchymal dimensions and area calculations were evaluated. Data were analyzed using 2-tailed t tests, ² analysis, analysis of variance, and analysis of covariance adjusted for age and sex. Correlational analyses with respect to subject type and age were performed separately.

Results  There were 27 patients (20 boys, aged 1.0-17.7 years; mean age, 8.8 years) and 43 controls (22 boys, aged 0.1-17.7 years; mean age, 5.9 years). The mean ages between groups (boys, girls, and totals) were not statistically different. Significant differences were appreciated for 6 of 24 measures. Patients with NF1 had a significantly larger bicaudate width (P=.002), biatrial width (P<.001), and biparietal diameter (P=.003), but not hemispheric length. They also had significantly increased iter measures (P=.004), descending sigmoid sinus (P<.001), and an age-specific increase in brainstem height (P=.03) not seen in controls.

Conclusions  Patients with NF1 experience dynamic changes in brain morphometry, resulting in a predominant lateral volume expansion of the supratentorial compartment and an increasing velocity of brainstem growth as they age. These data underscore brain-region–specific parenchymal overgrowth potential.

From the Departments of Pediatrics (Dr DiMario), Radiology (Drs DiMario and Ramsby), and Behavioral Sciences and Community Health (Dr Burleson), University of Connecticut, Farmington; Connecticut Children's Medical Center, Hartford (Dr DiMario); and the University of Connecticut Health Center, Farmington (Drs DiMario, Ramsby, and Burleson).

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