ABSTRACT
Williams syndrome (WS) is a rare developmental disorder characterized by mental retardation (Jones & Smith, 1975; Preus, 1984; von Arnim & Engel, 1964; Williams, Barrett-Boyes, & Lowe, 1961). Several reports have appeared describing some of the neuropsychological deficits observed in WS subjects (Crisco, Dobbs, & Mulhern, 1988; MacDonald & Roy, 1988; Udwin & Yule, 1991). Although these studies present conflicting results, most note their particularly poor visuospatial and visuomotor abilities. These studies have generally contrasted the performance of WS subjects on standardized tests to those of normal children or IQ-matched controls with mixed or nonspecific developmental disorders. In a series of recent neurobehavioral investigations, the unusual profile of higher cognitive functions in WS subjects has been illuminated (Bellugi, Bihrle, Jernigan, Trauner, & Doherty, 1990; Bellugi, Bihrle, Neville, Jernigan, & Doherty, 1992; Bellugi, Sabo, & Vaid, 1988; see also chapter 2 of this volume).
