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Laboratory for Cognitive Neuroscience (Drs. Wang, Doherty, and Bellugi), Salk Institute for Biological Studies, La Jolla, CA; the Departments of Radiology (Dr. Hesselink) and Psychiatry (Dr. Jernigan), University of California, San Diego School of Medicine, San Diego, CA; and the Veterans Affairs Medical Center (Dr. Jernigan), San Diego, CA.
Previous work demonstrated enlargement of the neocerebellar vermis in Williams' syndrome (WS), despite diminished volumes in the cerebral hemispheres. We present the first in vivo volumetric study of any structure within the cerebellar hemispheres. Using MRI, we identified and reliably measured the neocerebellar tonsils in WS subjects; Down's syndrome (DS) subjects matched for age, IQ, and cerebral volume; and age-matched normal controls. WS tonsils were equal in size to control tonsils and larger than DS tonsils. In proportion to the cerebrum, WS tonsils were larger than controls'. These results coincide with the remarkable neuropsychological preservation of language and affect in WS, despite general cognitive impairment. They contrast with the neocerebellar vermal hypoplasia seen in autism, with its communicative and affective deficits. Additionally, two WS subjects showed Chiari type I malformations, but the average tonsillar position in WS was not found to be different than in controls.
Address correspondence and reprint requests to Dr. Paul P. Wang, The Salk Institute - LCN, 10010 North Torrey Pines Road, La Jolla, CA 92037
Supported by NIH grants #HD 26022 and #DC 00146 to Dr. Bellugi, and #P50 NS22343 for the Center for the Study of the Neurological Basis of Language, and by the John D. and Cathenne T. MacArthur Foundation Research Network. Dr. Wang is a William T. Grant Foundation fellow of the Pediatric Scientist Development Program.
Received January 14, 1992. Accepted for publication in final form March 25, 1992.
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