“I never knew talent was a birth defect,” Jonas Salk has been quoted as saying about Williams Syndrome (cf. Bellugi & St. George 2001:xii), referring to the discrepancy between the extraordinary language abilities of persons with WS and their overall intellectual functioning, which usually falls within the bounds of mental retardation. The first of its kind, Semel & Rosner's very interesting sourcebook addresses the question: “How is it possible to conceptualize a group of children who test as though retarded, speak as though gifted, behave sometimes as though emotionally disturbed, and function like the learning disabled?” (p. 1). The insights into the origins and manifestations of this intriguing disorder discussed in this book have numerous theoretical and practical implications for understanding the molecular genetic and neurophysiological underpinnings of cognition, language, and sociability.

Highly readable and well organized, the book offers a wealth of information on the behavioral patterns of individuals with WS in four major areas: language, perceptual-motor functioning, specific aptitudes, and behavioral problems. The authors combine their own original research (the Utah Survey, a parent questionnaire study) with their extensive expertise in learning disabilities, speech pathology, special education (Semel) and developmental, cognitive, and clinical psychology (Rosner) to review, summarize, and organize the research literature on WS that has accumulated within several disciplines since the 1980s. Semel & Rosner draw upon both quantitative and qualitative research and effectively illustrate findings with real-life examples from personal accounts. Taking a lifespan developmental approach, the authors provide information on milestones of acquisition and change in main domains of functioning, and thus accomplish a comprehensive portrayal not only of the disorder but also of the children, adolescents, and adults who live with it.

Semel & Rosner succeed in making their book useful as well as informative. As often is the case with source books, however, it is unlikely that many readers will find it useful in its entirety. As their potential audience, the authors list parents and family members, teachers, practitioners of speech and language, physical, occupational and other therapies, psychologists, medical specialists in pediatrics, cardiology, psychiatry, genetics and internal medicine, and researchers in neurobiology, psycholinguistics, and cognitive psychology. Those working in the fields of linguistic and sociocultural anthropology, and especially those conducting interdisciplinary research in narrative analysis, pragmatics, and the neurobiology of language, will find parts of this book thought-provoking, shedding new light on such notions as “communicative competence,” “perspective-taking,” “narrative,” and “self”. Those interested in autism will find it valuable because of the dramatic contrast (as well as some similarities, and, in rare cases, coexistence) between these two disorders. Additionally, many readers will find informative Semel & Rosner's comparative approach to the similarities and differences between features of WS and other conditions such as Down, Prader-Willie, and Fragile X syndromes.

Chap. 1, “Introduction” (1–14), provides an overview of WS. People affected by this rare genetic neuro-developmental disorder have a distinctive pixie-like appearance, and they look much more like others with WS than like members of their own families. Subject from birth to serious cardiac, digestive, metabolic, and other medical problems, children with WS show delays in most areas of development. A brief historical overview (2) describes the circumstances of discovery of the disorder. Named after a New Zealand cardiologist, J. C. R. Williams, and independently recognized as a syndrome by German cardiologist Alois J. Beuren (2) and Swiss pediatrician Guido Fanconi (Bellugi & St. George 2001: xiv), it was initially presented as an organic, primarily cardiac condition involving supravalvular aortic stenosis (narrowing of the aorta), accompanied by mental retardation and certain facial characteristics. In spite of the atypical, extremely strong impulse toward social interaction and affective expression shown by individuals with WS, it was never seen as a disorder of “affective contact,” as was the case for autism (Kanner 1943).

Chap. 2, “Language skills and problems” (15–63), delineates the unusual mixture of abilities and impairments across different domains of language, such as the pattern of language development, voice quality, articulation and prosody, syntax, semantics, pragmatics, and discourse competence. Early language development of most children with WS is characterized by significant delays, including a delay in prelinguistic nonverbal behaviors such as pointing, showing, and giving objects. Children with WS begin to speak before they are able to point, a reversal of the developmental sequence in most populations. The language delay is followed by a developmental spurt and a continuous language growth. Children with WS eventually grow up fluent and articulate and use grammatically complex, generally correct language. They also have extensive vocabulary and are avid storytellers. These abilities are especially remarkable when compared to their presumed low intellectual functioning. Especially notable is the discussion of pragmatics of language use and narrative discourse (48–62). Persons with WS show an impressive ability to use language creatively in conversation, especially in narrative discourse. Their grasp of the narrative genre, whether spontaneous imaginary tales or the retelling of a picture-book story, is in fact superior in some areas (e.g., affective expressions, elaboration, and evaluative devices) to the performance of unaffected children at the same age. Apparently adept at using their sociocultural background knowledge in narrative discourse, persons with WS are not able to put it to use when they are not the protagonists but the participants in everyday social situations. There is no specific discussion, however, about the differences between their competence in telling everyday narratives of personal experience versus their retelling stories from picture books in laboratory settings. Their recognition of social group membership appears extremely limited, something that can be quite dangerous because individuals with WS approach strangers and family members alike with the same charming enthusiasm. Like people with autism, individuals with WS are often oblivious to social conventions as they engage in incessant greeting behavior or use overly familiar forms of address. Semel & Rosner convincingly argue that language studies of individuals with WS, with its unique constellation of abilities and impairments, illuminate such theoretical issues as the unity or fractionalization of language and the modularity and dissociation of distinct language domains, contributing new information to established theories of child language acquisition and developmental psychology.

Chap. 3, “Intervention approaches for language problems” (64–107), describes interventions for each of the areas discussed in chap. 1. This chapter relies heavily on the reader's familiarity with numerous assessment tests and measures. Significant effort in intervention is directed to developing control of undesirable behaviors, and clinicians are advised to use socially based rewards (e.g., an opportunity to have a conversation with a favorite teacher) rather than material ones. A section on improving language pragmatics (88–105) is an informative accompaniment to the corresponding section in chap. 2. For example, a mediating strategy for inappropriate greeting behavior is to identify and become aware of the social-role differences of various membership groups encountered in everyday life.

Chap. 4, “Perceptual and motor performance” (108–86), addresses visuomotor performance, tactile defensiveness and sensitivity, auditory hypersensitivity, and psycho-educational interventions in these areas. There is a dramatic discrepancy between language abilities of individuals with WS and their ability to separate perceptually a coherent whole into constituent parts, as well as to integrate parts into a coherent whole. Such tasks as drawing and block constructions are especially telling because of the fragmented and disorganized images and patterns they produce. The difficulty appears to be with figure-ground relations, or being able to perceive a target figure against a background, as well as to infer a global figure from constitutive parts. Although there are some parallels in this information-processing style with the one present in autism (Weak Central Coherence theory, Frith 1989), no discussion of similarities or differences between the two is provided in this section. The remarkable face recognition and memory abilities of individuals with WS are attributed in part to the local processing of visual information, as well as to the special status of human faces for persons with WS.

Chap. 5, “Specific aptitudes” (187–251), discusses sociability, curiosity, memory, and musicality. Exceptionally friendly, outgoing, and sensitive, persons with WS have been characterized as “loving, joyful, with a great sense of humor,” “always happy,” as well as “hypersocial” and “socially disinhibited” (188). They may not know how to tie their shoes because of severe motor impairments, but they have a common love and talent for music, both in playing musical instruments and in appreciating performances of others. Additionally, the incidence of perfect pitch seems to be higher in individuals with WS than in the general population. Empathy is considered to be an area of remarkable strength for individuals with WS. Social cognition as measured by theory of mind and false belief tasks is discussed; the results of these studies, however, are evaluated by Semel & Rosner as inconclusive.

Chap. 6, “Maladaptive behaviors” (252–96), describes behavior problems and compares them with behaviors characteristic of other disorders. A comparative account of autism and WS (281–86) gives a detailed overview of similarities and differences between the two disorders, although the authors admit that it “necessarily glosses over certain findings and individual differences” (284). Chap. 7, “Intervention approaches for maladaptive behaviors” (297–358), covers evaluation and diagnosis of the behaviors discussed in chap. 6, as well as clinical interventions appropriate to each type.

Chap. 8, “Summary and conclusions” (359–401), integrates the information covered in the book into an overview of a prototypical profile of WS, and discusses the genetic and neurophysiological processes associated with cardinal features of the disorder. Similarly, the educational and clinical guidelines are integrated into a broader framework, which provides a general intervention approach to education and treatment of persons with WS. A special contribution of this chapter is the discussion of Semel & Rosner's original research, the Utah Survey, in which parents of children and young adults with WS answered questions about four major themes of the book: language, perceptual-motor functioning, specific aptitudes, and behavioral problems. The results of the study indicate interconnectedness among behavioral categories. For example, there is an inverse relation between the domain of Maladaptive Problems and Social Skills and Empathy, and between Language Problems and Social Skills. The authors call for further research on the interconnectedness of behavioral domains and for further work on language difficulties, empathy, and narrative abilities. “The best predictor of future success is present behavior,” the authors conclude optimistically (391), predicting an informative and productive future for WS research. Besides its significant contribution to the research on WS, the book exemplifies the value of the study of atypical populations to the study of language.