Williams syndrome: recent developments

Williams syndrome is a rare neurogenetic condition caused by a hemizygous microdeletion on chromosome 7. The resultant behavioral phenotype includes hypersociality, anxiety, and impaired visual-spatial ability. Recent work has generated increasing knowledge about Williams syndrome on several levels. Geneticists are coming ever closer to understanding the etiology of this condition; meanwhile, the known pattern of cognitive and behavioral features in Williams syndrome is being revised as more detailed information is acquired. Advances in the neuroanatomic characterization of Williams syndrome is providing a bridge between genetics and cognition. In this paper, the most prominent features of Williams syndrome are reviewed, with a focus on the most recent findings in the neuropsychological, genetic, psychiatric, and neuroanatomical fields. (C) 2001 Lippincott Williams & Wilkins.