Williams syndrome: cognition, personality, and adaptive behavior

Williams syndrome is caused by a microdeletion of at least 16 genes on chromosome 7q11.23. The syndrome results in mild to moderate mental retardation or learning disability. The behavioral phenotype for Williams syndrome is characterized by a distinctive cognitive profile and an unusual personality profile. Relative to overall level of intellectual ability, individuals with Williams syndrome typically show a clear strength in auditory rote memory, a strength in language, and an extreme weakness in visuospatial construction. The personality of individuals with Williams syndrome involves high sociability, overfriendliness, and empathy, with an undercurrent of anxiety related to social situations. The adaptive behavior profile for Williams syndrome involves clear strength in socialization skills (especially interpersonal skills related to initiating social interaction), strength in communication, and clear weakness in daily living skills and motor skills, relative to overall level of adaptive behavior functioning. Literature relevant to each of the components of the Williams syndrome behavioral phenotype is reviewed, including operationalizations of the Williams syndrome cognitive profile and the Williams syndrome personality profile. The sensitivity and specificity of these profiles for Williams syndrome, relative to individuals with other syndromes or mental retardation or borderline normal intelligence of unknown etiology, is considered. The adaptive behavior profile is discussed in relation to the cognitive and personality profiles. The importance of operationalizations of crucial components of the behavioral phenotype for the study of genotype/phenotype correlations in Williams syndrome is stressed. MRDD Research Reviews 2000;6:148-158.     

Dyskinesias differentiate autistic disorder from catatonia

Autistic disorder and catatonia are neuropsychiatric syndromes defined by impairments in social interaction, communication, and restricted, stereotypical motor routines. Assessments of children with these disorders are typically restricted in scope by the patients' limited ability to comprehend directions. The authors performed systematic assessments of dyskinesias on six prepubertal boys with autistic disorder and mental retardation and on one adolescent male with catatonia to determine if this type of information could be routinely obtained. The boys with autistic disorder had more stereotypies and tics, a greater degree of akathisia and hyperactivity, and more compulsions than the adolescent with catatonia. Catatonia was associated with catalepsy and dystonic postures. The authors conclude that the diagnostic accuracy and specificity of neuropsychiatric syndromes may be enhanced by the systematic assessment of the dyskinesias associated with each condition.     

Subjective science: Kenneth Spence's human learning research program

This article examines Kenneth Spence's human learning research from 1949 to 1967. His works show the conditioning field to be in turmoil over basic issues during this time. These issues included whether determinism should be accepted, how to interpret a psychological test score, what psychometric properties a test should have, and how to use theoretical constructs. Turmoil occurred because behaviors and events had different meanings and degrees of importance to different investigators. Thus, what appeared as "objective" scientific change in the conditioning field was really the adaptation of personal preferences in word meanings, research goals, and research methods. These issues also indicate disputes about definitions and purposes of scientific activity during Spence's era.     

Triarchic theories of intelligence: Aristotle and Sternberg

Aristotle's triarchic theory of intelligence prefigures, but apparently does not serve directly as a source for, Sternberg's comparable contemporary theory. The indepently derived similarities in their gross structure lend weight to some triarchic view. Sternberg's analytical, practical, and creative intelligences closely resemble Aristotle's theoretical, practical, and productive excellences of intelligence. Each comparable component incorporates significantly related ideas. Disparities may be attributed to a variety of differences in the aims, methods, and resources of Aristotle's and Sternberg's inquiries. This is not the first time in the history of psychology that roughly parallel investigations have yielded comparable theories, but it is surely among the most temporally disjoined.     

Effects of the beholder's age on the perception of facial attractiveness

Children have been shown to prefer faces rated as highly attractive by adults over faces rated as quite unattractive. We investigated to what extent this agreement holds not only for the general direction of preferences but for preference strengths as well. In a choice experiment, we presented 40 nine-year-old girls and their mothers and 40 twelve-year-old girls and their mothers with pairs of women's and girls' faces and asked the subjects to pick the face that appeared prettier to them. Preference frequencies and simple attractiveness scales derived from these preference frequencies by fitting the Bradley-Terry-Luce rule (Luce, D. R. (1959). Individual choice behavior: a theoretical analysis. New York: Wiley) were compared across subject groups. For the women's faces, we found no difference in preferences between nine-year-olds, twelve-year-olds, and adults, neither in direction nor in strength. For the girls' faces, we also found no major differences in preference direction, however, we did find reliable differences in preference strengths. To a considerable part these differences were due to the fact that the children showed less pronounced preferences between face stimuli than the adults. These results suggest a role of developmental factors in the perception of facial attractiveness.     

Reflections on receiving an award

Reflecting on the events that culminated in her receiving the Award for Distinguished Contributions to Applied Psychology as a Professional Practice, Canter, an independent practitioner, discusses the road she traveled to become a clinical psychologist and to become involved in professional organizational activities. She believes that this award was given to her because of her contributions to psychology over her lifetime as an effective and hardworking leader, mentor, and role model in her home state of Arizona and nationally. She addresses some of her ideas about effective leadership and mentoring in the American Psychological Association (APA), providing many examples from which she has learned. Canter also shares some thoughts about APA's position as a leader in the development and enforcement of professional ethics.     

Promoting parental compliance with home infant apnea monitor use

OBJECTIVE: To promote greater compliance in using prescribed infant apnea monitors among families with a medically at-risk infant. Eight consecutively referred non-compliant families were treated by a medical social worker who provided educational, behavioral prompting, and case management services. In six families results were evaluated with A-B single case research designs, and in two families A-B-A designs were used. Enhanced compliance followed intervention with all eight families. Clinically satisfactory compliance permitting the pediatrician to safely discontinue monitor use occurred in five of the eight families. Simple behavioral and case management interventions show great promise in promoting caregiver compliance with using home-based infant apnea monitors.     

Attention and joint attention in preschool children with autism

Three experiments examined the role of attention in explaining dyadic (child-adult) and triadic (child-adult-object) joint attention difficulties in autism. Experiments 1 and 2 investigated children's ability to orient to an adult's attention bid and to follow the direction of a human or nonhuman cue. Experiment 3 tested ability to disengage and shift attention to objects. Results showed autism-specific difficulties at both dyadic and triadic levels. Children with autism were less responsive than developmentally delayed controls in orienting to attention bids and in following a human head-turn cue yet had no difficulty in shifting attention and were faster overall in orienting to targets. Results suggest a specific developmental delay in which children with autism rely on the presence of objects in the visual field to guide action. The relation between this problem and autistic children's difficulties with human communicative signals is discussed.