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.     

Assaultive behavior personality and personal space

Eysenck's Psychoticism, Extraversion and Neuroticism (PEN) Inventory was used to explore the role of personality variables in augmented personal space required by assaultive offenders. For both violent (n = 20) and nonviolent (n = 20) subjects personal space was greatest from a rear approach. Neither personality scores nor nature of offense alone predicted personal space needs; however personal space was significantly elevated in all directions for assaultive offenders with high scores on the psychoticism scale of the PEN Inventory.     

Type A behavior, personality hardiness, and cardiovascular responses to stress

Type A behavior and hardiness were examined as predictors of cardiovascular responses to stress in 68 male undergraduates. Systolic and diastolic blood pressure (SBP and DBP) and heart rate were monitored while subjects performed a difficult mirror-tracing task. Type A assessments based on the Structured Interview, but not those based on the Jenkins Activity Survey, were associated with significantly enhanced SBP and DBP elevations. Hardiness was associated with significantly reduced DBP responsiveness. In addition, a significant interaction indicated that the Type B-high hardiness group showed the least DBP reactivity. A near-significant interaction (p = .06) suggested that Type B-high hardiness subjects also reported the least anger. Further exploration of the data indicated that the challenge component of hardiness accounted for its relationship to DBP reactivity. These results have implications both for the psychophysiologic study of Type A behavior and for understanding the health-promoting effects of hardiness.     

Involuntary subordination and its relation to personality, mood, and submissive behavior

According to social rank theory, involuntary subordination may be adaptive in species that compete for resources as a mechanism to switch off fighting behaviors when loss is imminent (thus saving an organism from injury). In humans, major depression is thought to occur when involuntary subordination becomes prolonged. The present study sought to operationalize involuntary subordination. Study 1 involved a reanalysis of a Gilbert and Allan (1998) study, with the hypothesis that social comparison (i.e., perceived status), submissive behavior, feelings of defeat, and entrapment would load on a common factor (interpreted as involuntary subordination). Exploratory and confirmatory factor analysis supported this model. In Study 2 measures of these same variables were administered to a group of undergraduate students. Eight items were selected from each measure (on the basis of item-total correlations) to form the Involuntary Subordination Questionnaire (ISQ). In Study 3 scores on the ISQ showed high levels of internal consistency and test-retest reliability in a sample of undergraduate students. Scores on the ISQ were significantly positively correlated with various neurotic personality styles and negatively correlated with variables indicating dominance or mastery. Involuntary subordination scores also significantly predicted change in social anxiety symptoms over 9 weeks. In Study 4 scores on the ISQ were examined in relation to nonverbal behaviors. In men, the ISQ was correlated with behaviors indicating a lack of confidence and submissiveness. Involuntary subordination appears to be a relatively stable trait with implications for personality, mood, and real-world behavior.     

Subliminal stimulation, choice behavior and some personality correlates of subliminal sensitivity

The aim of this study was to investigate the possible relations between specific personality variables and the effect of subliminal stimulation on choice behavior. It was hypothesized that subjects with low anxiety, less neuroticism, and external locus of control and the trait of extraversion would be more susceptible to subliminal stimulation. 38 undergraduate students were exposed to subliminal messages urging them to choose symbols instead of numbers or letters. Analysis showed that subliminal stimulation did not generate a preference for symbols over numbers or letters. No support was observed for the hypothesis of an association between susceptibility and personality variables. These results may be explained in terms of symbol choice being a neutral message that did not relate to the subjects' needs and motivation.     

Altruism and antisocial behavior: independent tendencies, unique personality correlates, distinct etiologies

The relationship between altruism and antisocial behavior has received limited attention because altruism and antisocial behavior tend to be studied and discussed in distinct literatures. Our research bridges these literatures by focusing on three fundamental questions. First, are altruism and antisocial behavior opposite ends of a single dimension, or can they coexist in the same individual? Second, do altruism and antisocial behavior have the same or distinct etiologies? Third, do they stem from the same or from distinct aspects of a person's personality? Our findings indicate that altruism and antisocial behavior are uncorrelated tendencies stemming from different sources. Whereas altruism was linked primarily to shared (i.e., familial) environments, unique (i.e., nonfamilial) environments, and personality traits reflecting positive emotionality, antisocial behavior was linked primarily to genes, unique environments, and personality traits reflecting negative emotionality and a lack of constraint.     

Predicting social behavior from personality traits

The classic view of traits as dispositions was examined and a number of ambiguities noted. When clarified, implications for predicting social behaviors from personality variables were derived. Two types of behavioral criterion were delineated, and it was argued that personality measures should be systematically related to multiple act criteria, but not necessarily to single act criteria. It was suggested that behaviors could be treated as items on a behavioral measure of personality and that standard scaling criteria be applied in identifying behaviors that should be related to a given trait dimension. In attempting to identify behaviors which are linearly related to a personality trait, the application of Bayes theorem was shown to be of theoretical interest. Two indices, a validity index and a linearity index, were derived from Bayes theorem and it was shown that validity is a necessary but not sufficient condition for linearity.     

Comparing personality constructs of risk-taking behavior

Canadian subjects (N = 120) completed four psychological scales. Three of the scales were directly related to risk-taking behavior. These were the Sensation Seeking Scale (SSS V; Zuckerman, 1979), the Tension Risk Adventure Inventory (TRAI; Keinan et al., 1984) and the Telic Dominance Scale (TDS; Murgatroyd et al., 1978). In addition, subjects completed the Desire for Control Scale (DCS; Burger and Cooper, 1979) to investigate the role of desire for control in risk-taking. The results indicated (1) significant differences between age and sensation seeking (2) a four-factor structure for the TRAI following factor analysis with this Canadian sample (3) numerous intra- and inter-scale relationships obtained from correlation analyses of and (4) four major dimensions arising from PCA of the four inventory scales.     

Expressive control,expressive consistency,and the correspondence between expressive behavior and personality1

Does expressive behavior reflect personality? This paper proposes that expressive control is an important “moderating variable” affecting expressive behavior, expressive consistency, and the correspondence between expressive behavior and personality. To demonstrate this, a study was carried out in which 68 subjects were selected, according to a 2×2×2 factorial design, who were low and high on assessed extraversion, neuroticism, and self-monitoring (a measure of expressive control). Subjects were then videotaped as they role-played being teachers. A number of specific expressive behaviors were measured (subjects' stride length, graphic expansiveness, percent of forward eye-contact, and percent of time talking), and also groups of naive judges rated how “extraverted” and “anxious” subjects appeared. The following results supported our hypothesis: (1) Self-monitoring was significantly related to subjects' expressive behaviors and judged personalities, while assessed extraversion and anxiety were not. (2) Expressive control was used to suppress the “accurate” display of anxiety but not extraversion. (3) Bodily expression was less controlled than facial or vocal expression. And (4), persons low and high on self-monitoring showed different patterns of cross-situational and cross-channel (face, body, voice) expressive consistency.     

Suicidal behavior in borderline personality disorder: prevalence, risk factors, prediction, and prevention

Suicidal behavior is frequent in patients with borderline personality disorder (BPD); at least three-quarters of these patients attempt suicide and approximately 10% eventually complete suicide. Borderline patients at greatest risk for suicidal behavior include those with prior attempts, comorbid major depressive disorder, or a substance use disorder. Comorbidity with major depression serves to increase both the number and seriousness of the suicide attempts. Hopelessness and impulsivity independently increase the risk of suicidal behavior, as does a turbulent early life and the presence of antisocial traits. In summary, because BPD is frequently complicated by suicidal behavior, clinicians must avoid the mistake of thinking that a pattern of repeated attempts indicates little desire to die. Clinicians have an important role in preventing suicide attempts and completed suicides by understanding the risk factors.     

Subtyping borderline personality disorder by suicidal behavior

Course and outcome of Borderline Personality Disorder (BPD) are favorable for the vast majority of patients; however, up to 10% die by suicide. This discrepancy begs the question of whether there is a high lethality subtype in BPD, defined by recurrent suicidal behavior and increasing attempt lethality over time. In a prospective, longitudinal study, we sought predictors of high lethality among repeat attempters, and defined clinical subtypes by applying trajectory analysis to consecutive lethality scores. Criteria-defined subjects with BPD were assessed using standardized instruments and followed longitudinally. Suicidal behavior was assessed on the Columbia Suicide History, Lethality Rating Scale, and Suicide Intent Scale. Variables discriminating single and repeat attempters were entered into logistic regression models to define predictors of high and low lethality attempts. Trajectory analysis using three attempt and five attempt models identified discrete patterns of Lethality Rating Scale scores. A high lethality trajectory was associated with inpatient recruitment, and poor psychosocial function, a low lethality trajectory with greater Negativism, Substance Use Disorders, Histrionic and/or Narcissistic PD co-morbidity. Illness severity, older age, and poor psychosocial function are characteristics of a poor prognosis subtype related to suicidal behavior.     

Etiologic connections among substance dependence,antisocial behavior,and personality: modeling the externalizing spectrum

A hierarchical biometric model is presented of the origins of comorbidity among substance dependence, antisocial behavior, and a disinhibited personality style. The model posits a spectrum of personality and psychopathology, united by an externalizing factor linked to each phenotype within the spectrum, as well as specific factors that account for distinctions among phenotypes within the spectrum. This model fit self-report and mother-report data from 1,048 male and female 17-year-old twins. The variance of the externalizing factor was mostly genetic, but both genetic and environmental factors accounted for distinctions among phenotypes within the spectrum. These results reconcile evidence for general and specific causal factors within the externalizing spectrum and offer the externalizing factor as a novel target for future research.     

Criterion-related validity of the three-factor model of psychopathy: personality, behavior, and adaptive functioning

The Psychopathy Checklist-Revised (PCL-R) has been conceptualized as indexing two distinct but correlated factors. Previous research has established that these factors demonstrate distinct patterns of relations with external criteria. However, more recent findings suggest that the PCL-R psychopathy construct may encompass three distinguishable factors, reflecting affective, interpersonal, and behavioral symptoms. Here, we evaluated the validity of this newer three-factor model of the PCL-R factors with reference to external criteria from the domains of personality, antisocial behavior; and adaptive functioning in a sample of 310 incarcerated offenders. The interpersonal factor was related to social dominance, low stress reactivity, and higher adaptive functioning; the affective factor was correlated with low social closeness and violent offending; and the behavioral factor was associated with negative emotionality, disinhibition, reactive aggression, and poor adaptive functioning. These findings provide support for the convergent and discriminant validity of these psychopathy facets.     

Externalizing behavior trajectories: The role of parenting, sibling relationships and child personality

Based on longitudinal multilevel modeling and using a multi-informant strategy, this study examines trajectories of externalizing problem behavior (EPB) in childhood as predicted by parental behavior (absolute level of parenting [ALP] and parental differential treatment [PDT]), parental self-efficacy (PSE), child personality and sibling relationships. Besides main effects, several interactions were tested. The effects of changes in parenting on changes in EPB were also examined. A total of 119 families from the French-speaking area of Belgium rearing a child (3- to 5-year-olds at the onset of the study) referred for EPB were studied. The effects of both level-of and changes-in mothers' ALP were partially confirmed, but for fathers, only ALP was predictive. There were some significant interactions between ALP and PDT for both parents. Mothers' PSE explained some variance in EPB, additional to that explained by the parenting measures. Some parenting by personality and by sibling relationship interactions were found.