LINGUISTIC ABILITIES IN ITALIAN CHILDREN WITH WILLIAMS SYNDROME Virginia Volterra 1 , Olga CapircP, Grazia PezzinF, Letizia SabbadinP, Stefano Vicari 3 eInstitute of Psychology, C.N.R., Department of Neuropsychology of Language and Deafness, Rome; 2Children's Hospital Bambino Gesu, Department of Neuropsychology and Clinical Psychology, Rome; 3Children's Hospital Bambino Gesu, Department of Neurology and Rehabilitation, Santa Marinella, Rome) ABSTRACT Recent studies on subjects with Williams syndrome (WS) have revealed a particular facility for language, rarely observed in other mental retarded populations, inspiring much belief in the independence of language from cognition. Lexical and morphosyntactic abilities of 17 Italian WS individuals, between 4.10 and 15.3 years of age, were evaluated both in comprehension and production and compared with those of normally developing Italian children. WS subjects look similar to normal controls in lexical comprehension, but they appear to perform more poorly in grammatical comprehension. Furthermore they look deviant from normals in some morphosyntactic aspects of their production. They perform better than normal controls only with respect to phonological fluency, when semantic aspects are not involved. Our data show very little evidence for a dissociation between language and cognition. INTRODUCTION , Williams syndrome is a rare genetic condition, first identified in 1961 by Williams and his colleagues in New Zealand (Williams, Barratt-Boyes and Lowe, 1961). The syndrome is sometimes known in Europe as Beuren's syndrome, because in 1962 Beuren and colleagues have described a similar profile (Beuren, Apitz and Harmjanz, 1962). WS is typified by a number of common characteristics: mental retardation, a peculiar facial appearance, and severe medical anomalies (renal and cardiovascular, in particular supra-valvular aortic stenosis). Mental handicap falls in the range of mild to severe retardation (Arnold, Yule and Martin, 1985; Morris, Demsey, Leonard et aI., 1988; Udwin, Yule and Martin, 1987). More recently some researchers have suggested that WS may result from a microdeletion of the long arm of the chromosome 7 and in particular the deletion of one allele of the elastin gene (Morris, Thomas and Greenberg, 1993; Mari, Amati, Mingarelli et aI., 1995). At birth WS infants are below average weight, often show no spontaneous sucking with frequent vomiting during the first few months. WS children suffer from hyperacusis, an unusual sensitivity to certain environmental sounds. As children, they are extremely social and show marked friendliness in their social behaviour towards strangers (Gosch, Stading and Pankau, 1994). As adults they appear very variable in clinical presentation and Cortex, (1996) 32, 663-677