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Albert M. Galaburda, MD; J. Eric Schmitt, BS; Scott W. Atlas, MD; Stephan Eliez, MD; Ursula Bellugi, EdD; Allan L. Reiss, MD

Arch Neurol. 2001;58:1865-1869.

Background  Williams syndrome (WMS) is a rare neurogenetic condition with a behavioral phenotype that suggests a dorsal and/or ventral developmental dissociation, with deficits in dorsal but not the ventral hemispheric visual stream. A shortened extent of the dorsal central sulcus has been observed in autopsy specimens.

Objective  To compare gross anatomical features between the dorsal and ventral portions of the cerebral hemispheres by examining the dorsal extent of the central sulcus in brain magnetic resonance images from a sample of subjects with WMS and age- and sex-matched control subjects.

Subjects  Twenty-one subjects having clinically and genetically diagnosed WMS (mean ± SD age, 28.9 ± 7.9 years) were compared with 21 age- and sex-matched typically developing controls (mean ± SD age, 28.8 ± 7.9 years).

Design  High-resolution structural magnetic resonance images were acquired. The extent of the central sulcus was qualitatively assessed via surface projections of the cerebral cortex.

Results  The dorsal central sulcus is less likely to reach the interhemispheric fissure in subjects with WMS than in controls for both left (P<.001, ² = 15.79) and right (P<.001, ² = 12.95) hemispheres. No differences between the groups were found in the ventral extent of the central sulcus.

Conclusions  Anomalies in the dorsal region in patients with WMS are indicative of early neurodevelopmental problems affecting the development of the dorsal forebrain and are most likely related to the deficits in visuospatial ability and behavioral timing often observed in this condition.

From the Division of Behavioral Neurology, Department of Neurology, Beth Israel–Deaconess Medical Center and Harvard Medical School, Boston, Mass (Dr Galaburda); Stanford Psychiatry Neuroimaging Laboratory, Department of Psychiatry and Behavioral Sciences (Mr Schmitt and Drs Eliez and Reiss) and the Division of Neuroradiology, Department of Radiology (Dr Atlas), Stanford University School of Medicine, Stanford, Calif; and the Laboratory for Cognitive Neuroscience, Salk Institute for Biological Studies, La Jolla, Calif (Dr Bellugi).

Corresponding author and reprints: Albert M. Galaburda, MD, Beth Israel–Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215 (e-mail: agalabur{at}caregroup.harvard.edu).

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