Attentional disengagement in adults with Williams syndrome

Williams syndrome (WS) is a neurodevelopmental disorder characterized by a distinctive behavioral and cognitive profile, including widespread problems with attention. However, the specific nature of their attentional difficulties, such as inappropriate attentional allocation and/or poor attentional disengagement abilities, has yet to be elucidated. Furthermore, it is unknown if there is an underlying difficulty with the temporal dynamics of attention in WS or if their attentional difficulties are task-dependent, because previous studies have examined attention in established areas of deficit and atypicality (specifically, visuospatial and face processing). In this study, we examined attentional processing in 14 adults with WS (20–59 years) and 17 typically developing controls (19–39 years) using an attentional blink (AB) paradigm. The AB is the decreased ability to detect a second target when it is presented in close proximity to an initial target. Overall, adults with WS had an AB that was prolonged in duration, but no different in magnitude, compared with typically developing control participants. AB performance was not explained by IQ, working memory, or processing speed in either group. Thus, results suggest that the attention problems in WS are primarily due to general attentional disengagement difficulties rather than inappropriate attentional allocation.     

The role of the cingulate cortex in the generation of motor tics and the experience of the premonitory urge-to-tic in Tourette syndrome

Tourette syndrome (TS) is a neurological disorder of childhood onset that is characterized by the occurrence of motor and vocal tics. TS is associated with cortical-striatal-thalamic-cortical circuit [CSTC] dysfunction and hyper-excitability of cortical limbic and motor regions that are thought to lead to the occurrence of tics. Individuals with TS often report that their tics are preceded by ‘premonitory sensory/urge phenomena’ (PU) that are described as uncomfortable bodily sensations that precede the execution of a tic and are experienced as a strong urge for motor discharge. While the precise role played by PU in the occurrence of tics is largely unknown, they are nonetheless of considerable theoretical and clinical importance as they form a core component of many behavioural therapies used in the treatment of tic disorders. Recent evidence indicates that the cingulate cortex may play an important role in the generation of PU in TS, and in ‘urges-for-action’ more generally. In the current study, we utilized voxel-based morphometry (VBM) techniques, together with ‘seed-to-voxel’ structural covariance network (SCN) mapping, to investigate the putative role played by the cingulate cortex in the generation of motor tics and the experience of PU in a relatively large group of young people with TS. Whole-brain VBM analysis revealed that TS was associated with clusters of significantly reduced grey matter volumes bilaterally within: the orbito-frontal cortex; the cerebellum; and the anterior and mid-cingulate cortex. Similarly, analysis of SCNs associated with bilateral mid- and anterior cingulate ‘seed’ regions demonstrated that TS is associated with increased structural covariance primarily with the bilateral motor cerebellum; the inferior frontal cortex; and the posterior cingulate cortex.     

How parents introduce new words to young children: The influence of development and developmental disorders

This study documents how parents weave new words into on-going interactions with children who are just beginning to speak. Dyads with typically developing toddlers and with young children with autism spectrum disorder and Down syndrome (n = 56, 23, and 29) were observed using a Communication Play Protocol during which parents could use novel words to refer to novel objects. Parents readily introduced both labels and sound words even when their child did not respond expressively or produce the words. Results highlight both how parents act in ways that may facilitate their child's appreciation of the relation between a new word and its referent and how they subtly adjust their actions to suit their child's level of word learning and specific learning challenges.     

The Role for Occupational Therapists in Community Mobility Training for People With Autism Spectrum Disorders

Symptoms and behaviors of Autism Spectrum Disorders can challenge community mobility, thus hindering independence and participation in occupation. Community mobility, also called travel training, helps people make journeys safely using public transportation, enabling engagement in activities/services otherwise unavailable. Yet, few articles have been written about this topic for people with Autism Spectrum Disorders. The purpose of the authors in this article is to: (1) explain the need for travel training with people diagnosed with Autism Spectrum Disorders; (2) describe specific travel training assessments; (3) review literature on travel training programs; and (4) define a role for occupational therapy in travel training with individuals diagnosed with Autism Spectrum Disorders.     

Facial emotion recognition in Williams syndrome and Down syndrome: A matching and developmental study

In this study both the matching and developmental trajectories approaches were used to clarify questions that remain open in the literature on facial emotion recognition in Williams syndrome (WS) and Down syndrome (DS). The matching approach showed that individuals with WS or DS exhibit neither proficiency for the expression of happiness nor specific impairments for negative emotions. Instead, they present the same pattern of emotion recognition as typically developing (TD) individuals. Thus, the better performance on the recognition of positive compared to negative emotions usually reported in WS and DS is not specific of these populations but seems to represent a typical pattern. Prior studies based on the matching approach suggested that the development of facial emotion recognition is delayed in WS and atypical in DS. Nevertheless, and even though performance levels were lower in DS than in WS, the developmental trajectories approach used in this study evidenced that not only individuals with DS but also those with WS present atypical development in facial emotion recognition. Unlike in the TD participants, where developmental changes were observed along with age, in the WS and DS groups, the development of facial emotion recognition was static. Both individuals with WS and those with DS reached an early maximum developmental level due to cognitive constraints.     

Visual attention skills of premature infants with and without intraventricular hemorrhage

The relation between two medical complications associated with prematurity (intraventricular hemorrhage (IVH) and respiratory distress syndrome (RDS)) and visual attention skills of very low birth-weight (VLBW) infants were examined. Fourteen preterm VLBW infants with IVH and RDS, 9 preterms with RDS and no IVH, and 10 full-term infants matched on SES, sex, and race were assessed at 7 months of age (after correction for prematurity) for their ability to show a decremental response to a familiar visual stimulus and an increment in response to a novel visual stimulus. Measures of attention getting (turning toward a stimulus) and attention holding (sustained fixation on the stimulus after turning) were taken. On the attention-getting measures, IVH-RDS preterms had longer latencies than both the RDS-no-IVH preterm group and the full-term group. There were no group differences on attention-holding measures. The three groups did not differ in habituation to the familiar visual stimulus or discrimination of the novel stimulus. This study indicates that the early visual attention skills of preterm infants are differentially affected by specific types of prenatal complications.     

The long-term outcome of a personal network-oriented HIV prevention intervention for injection drug users: The SAFE study

Social influence processes have been found to affect numerous drug and health-related behaviors. We postulated that by using a network-oriented intervention it may be possible to capitalize on social influence processes to reduce human immunodeficiency virus (HIV) risk behaviors. The present study used an experimental study design for delivering a psychoeducational acquired immunodeficiency syndrome (AIDS) preventive intervention to injection drug sharing networks. Participants were recruited from the ALIVE study, an epidemiological study in Baltimore. In the present paper we examine the self-reported behavioral outcomes of 117 injection drug users 18 months after the baseline interview. HIV seronegative experimental participants reported significantly less frequent needle sharing and less injecting of heroin and cocaine than controls. In multiple logistic regression models of HIV seronegative participants, there was a significant negative association between assignment to the experimental group and the HIV-related behaviors of needle sharing and sharing of cookers in the prior 6 months; controls were 2.8 times more likely than experimentals to report needle sharing and were 2.7 times more likely to report sharing cookers. The results of this 18-month follow-up suggest that among injection drug users network-oriented interventions may be a promising approach to HIV prevention.     

Patterns of adolescent involvement in problem behaviors: Relationship to self-efficacy,social competence,and life events

Using a sample of 556 adolescents from a suburban community, patterns of various adolescent problem behaviors (e.g., delinquent behavior, smoking, use of alcohol or drugs) and their links to self-efficacy, social competence, and life events were examined. Cluster analysis was conducted to identify four subgroups of adolescents who showed distinct patterns of problem behaviors. These clusters were compared on the measures of self-efficacy, social competence, and life events. Overall results suggest there are meaningful links between adolescents’ problem behavior patterns and self-efficacy, the amount and quality of participation in various after school activities, and life events. For example, a subgroup of adolescents who showed uniformly low prevalence of all problem behaviors reported more positive academic self-efficacy, more active participation in sports and nonsports activities, more positive life events, and fewer negative events than adolescents who were involved in multiple problem behaviors. Implications for prevention and future research on adolescent problem behaviors are discussed.     

Cry analysis detects subclinical effects of prenatal alcohol exposure in newborn infants

The threshold, latency, and peak fundamental frequency (basic pitch) of crying were sensitive to the subclinical effects of prenatal alcohol exposure through the first month of postnatal life. Whereas infants with prenatal alcohol exposure showed a lower cry pitch and higher cry threshold at 2 days of age, higher pitched cries, typical of nervous system insult, were evident at 14 and 28 days. A longer latency was also evident at 14 days.     

Impaired tactual perception in children with Down's syndrome

Using standardized neuropsychological tests, tactual finger discrimination, graphaesthesia and stereognosis were studied in a group of 11 children aged 7–11 years with Down's syndrome (DS). All tasks were solved without visual control. The performance was subnormal in each test and the results of the stereognosis test were particularly poor. It was ascertained that the DS children could understand test instructions. Moreover, under visual control all objects used in the stereognosis test were handled correctly. The results suggest that tactual perception is subnormal in children with DS. The pathogenesis of the somatosensory abnormalities should be explored.