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J. Eric Schmitt, AB; Stephan Eliez, MD; Ursula Bellugi, EdD; Allan L. Reiss, MD

Arch Neurol. 2001;58:283-287.

Background  As a neurobehavioral disorder with a specific neurocognitive profile and a well-defined genetic etiology, Williams syndrome (WMS) provides an exceptional opportunity to examine associations among measures of behavior, neuroanatomy, and genetics. This study was designed to determine how cerebral shape differs between the brains of subjects with WMS and those of normal controls.

Subjects  Twenty adults with clinically and genetically diagnosed WMS (mean ± SD age, 28.5 ± 8.3 years) and 20 healthy, age- and sex-matched controls (mean ± SD age, 28.5 ± 8.2 years).

Design  High-resolution structural magnetic resonance imaging data were used for shape-based morphological analysis of the right and left cerebral hemispheres and the corpus callosum. Statistical analyses were performed to examine group differences.

Results  Both right and left cerebral hemispheres of subjects with WMS bend to a lesser degree in the sagittal plane than normal controls (P<.001). The corpus callosum also bends less in subjects with WMS (P = .05). In addition, subjects with WMS have decreased cerebral (P<.001) and corpus callosum (P<.001) midline lengths.

Conclusions  Subjects with WMS have significantly different cerebral shape from normal controls, perhaps due to decreased parieto-occipital lobe volumes relative to frontal regions. The similar observation in the corpus callosum may be associated with a decreased size of the splenium in WMS. These findings may provide important clues to the effect of genes in the WMS-deleted region on brain development.

From the Stanford Psychiatry Neuroimaging Laboratory, Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Stanford, Calif (Mr Schmitt and Drs Eliez and Reiss); and the Laboratory for Cognitive Neuroscience, the Salk Institute for Biological Studies, La Jolla, Calif (Dr Bellugi).

Reprints: Allan L. Reiss, MD, Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, 401 Quarry Rd, Stanford, CA 94305-5719 (e-mail: reiss{at}stanford.edu).

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