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Dorsal Forebrain Anomaly in Williams Syndrome
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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