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The Journal of Neuroscience, October 31, 2007, 27(44):11960-11965; doi:10.1523/JNEUROSCI.3591-07.2007

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Neurobiology of Disease
More Is Not Always Better: Increased Fractional Anisotropy of Superior Longitudinal Fasciculus Associated with Poor Visuospatial Abilities in Williams Syndrome

Fumiko Hoeft,¹ Naama Barnea-Goraly,¹ Brian W. Haas,¹ Golijeh Golarai,¹ Derek Ng,¹ Debra Mills,² Julie Korenberg,^3,4 Ursula Bellugi,⁵ Albert Galaburda,⁶ and Allan L. Reiss¹

¹Center for Interdisciplinary Brain Sciences Research, Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Palo Alto, California 94305-5795, ²Department of Psychology, Emory University, Atlanta, Georgia 30322, ³Department of Pediatrics, Cedars Sinai Medical Center, Los Angeles, California 90048, ⁴Department of Human Genetics, David Geffen School of Medicine at the University of California, Los Angeles, Los Angeles, California 90095-7088, ⁵Laboratory for Cognitive Neuroscience, Salk Institute for Biological Studies, La Jolla, California 92037, and ⁶Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02215

Correspondence should be addressed to Fumiko Hoeft, Center for Interdisciplinary Brain Sciences Research, Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, 401 Quarry Road, Palo Alto, CA 94305-5795. Email: fumiko{at}stanford.edu

We used diffusion tensor imaging to examine white matter integrity in the dorsal and ventral streams among individuals with Williams syndrome (WS) compared with two control groups (typically developing and developmentally delayed) and using three separate analysis methods (whole brain, region of interest, and fiber tractography). All analysis methods consistently showed that fractional anisotropy (FA; a measure of microstructural integrity) was higher in the right superior longitudinal fasciculus (SLF) in WS compared with both control groups. There was a significant association with deficits in visuospatial construction and higher FA in WS individuals. Comparable increases in FA across analytic methods were not observed in the left SLF or the bilateral inferior longitudinal fasciculus in WS subjects. Together, these findings suggest a specific role of right SLF abnormality in visuospatial construction deficits in WS.

Key words: Williams syndrome; visuospatial construction; superior longitudinal fasciculus; diffusion tensor imaging; developmental disabilities; genetics

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Received Aug. 8, 2007; revised Sept. 11, 2007; accepted Sept. 11, 2007.

Correspondence should be addressed to Fumiko Hoeft, Center for Interdisciplinary Brain Sciences Research, Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, 401 Quarry Road, Palo Alto, CA 94305-5795. Email: fumiko{at}stanford.edu

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