Visually guided step descent in children with Williams syndrome

Individuals with Williams syndrome (WS) have impairments in visuospatial tasks and in manual visuomotor control, consistent with parietal and cerebellar abnormalities. Here we examined whether individuals with WS also have difficulties in visually controlling whole-body movements. We investigated visual control of stepping down at a change of level in children with WS (5-16-year-olds), who descended a single step while their movement was kinematically recorded. On each trial step height was set unpredictably, so that visual information was necessary to perceive the step depth and position the legs appropriately before landing. Kinematic measures established that children with WS did not use visual information to slow the leg at an appropriate point during the step. This pattern contrasts with that observed in typically developing 3- and 4-year-old children, implying severe impairment in whole-body visuomotor control in WS. For children with WS, performance was not significantly predicted by low-level visual or balance problems, but improved significantly with verbal age. The results suggest some plasticity and development in WS whole-body control. These data clearly show that visuospatial and visuomotor deficits in WS extend to the locomotor domain. Taken together with evidence for parietal and cerebellar abnormalities in WS, these results also provide new evidence for the role of these circuits in the visual control of whole-body movement.     

The extent of working memory deficits associated with Williams syndrome: exploration of verbal and spatial domains and executively controlled processes

The present study investigated verbal and spatial working memory (WM) functioning in individuals with the neuro-developmental disorder Williams syndrome (WS) using WM component tasks. While there is strong evidence of WM impairments in WS, previous research has focused on short-term memory and has neglected assessment of executive components of WM. There is a particular lack of consensus concerning the profile of verbal WM functioning in WS. Here, WS participants were compared to typically developing participants matched for (1) verbal ability and (2) spatial ability (N = 14 in each of the 3 groups). Individuals with WS were impaired on verbal WM tasks, both those involving short-term maintenance of information and executive manipulation, in comparison to verbal-matched controls. Surprisingly, individuals with WS were not impaired on a spatial task assessing short-term maintenance of information in memory (remembering spatial locations) compared to spatial-matched controls. They were, however, impaired on a spatial executive WM task requiring the manipulation of spatial information in memory. The present study suggests that individuals with WS show WM impairments that extend to both verbal and spatial domains, although spatial deficits are selective to executive aspects of WM function.     

Activation of brain attention systems in individuals with symptoms of ADHD

Previous research investigating attention and impulse control in individuals with Attention-Deficit/Hyperactivity Disorder (ADHD) has largely ignored the symptomatic differences among the three subtypes of ADHD: ADHD-Inattentive Type, ADHD-Hyperactive/Impulsive Type, and ADHD-Combined Type. The present study examined attention and impulse control by focusing on these subtypes. Based on their self-reported symptoms of ADHD, participants belonged to one of four groups: ADHD-Inattentive, ADHD-Hyperactive/Impulsive, ADHD-Combined, and control. Cortical activity was recorded from participants during performance of a Go/NoGo task. The event-related potentials (ERP) measured at frontal and posterior sites discriminated between the control group and participants with symptoms of ADHD. The control group consistently exhibited a higher P3 amplitude than all the ADHD groups. The main difference occurred at the frontal site, indicating that individuals with ADHD symptoms have deficits in the anterior attentional system, which mediates signal detection. Behavioral measures of signal sensitivity revealed that the ADHD-Inattentive and the ADHD-Hyperactive/Impulsive groups had more difficulty with the attention-demanding Go/NoGo respond-to-target task, while behavioral measures of response bias indicated that the ADHD-Hyperactive/Impulsive and the ADHD-Combined groups responded more liberally in the inhibition-demanding Go/NoGo suppress-to-target task.     

How semantic categorization influences inhibitory processing in middle-childhood: an Event Related Potentials study

Throughout middle-childhood, inhibitory processes, which underlie many higher order cognitive tasks, are developing. Little is known about how inhibitory processes change as a task becomes conceptually more difficult during these important years. In adults, as Go/NoGo tasks become more difficult there is a systematic decrease in the P3NoGo response, indicating the use of effective inhibitory strategies (Maguire et al., 2009). This paper investigates the age at which children employ similar inhibitory strategies by studying behavioral and Event Related Potential (ERP) measures of response inhibition for three Go/NoGo tasks. Seventeen 7-8 year-olds and twenty 10-11-year-olds completed three Go/NoGo tasks that differed in the level of categorization necessary to respond. Both age groups displayed slower reaction times as the tasks became more difficult. Further, both groups displayed the predicted Go vs. NoGo P3 amplitude differences in the two simplest tasks, but no significant P3 differences for the most complex task. The reason for this pattern of responses was different in the different age groups. Similar to adults in previous work, the oldest children showed an attenuation of the P3 NoGo response with task difficulty, and no corresponding changes in the Go amplitude. The younger children displayed the opposite pattern, a significant increase in the Go amplitude with task difficulty, and no changes in the NoGo response. These response patterns indicate that efficient inhibitory strategies are developing throughout middle-childhood.     

How do individuals with Williams syndrome learn a route in a real‐world environment?

Individuals with Williams syndrome (WS) show a specific deficit in visuo‐spatial abilities. This finding, however, derives mainly from performance on small‐scale laboratory‐based tasks. This study investigated large‐scale route learning in individuals with WS and two matched control groups (moderate learning difficulty group [MLD], typically developing group [TD]). In a non‐labelling and a labelling (verbal information provided along the route) condition, participants were guided along one of two unfamiliar 1‐km routes with 20 junctions, and then retraced the route themselves (two trials). The WS participants performed less well than the other groups, but given verbal information and repeated experience they learnt nearly all of the turns along the route. The extent of improvement in route knowledge (correct turns) in WS was comparable to that of the control groups. Relational knowledge (correctly identifying spatial relationships between landmarks), compared with the TD group, remained poor for both the WS and the MLD group. Assessment of the relationship between performance on the large‐scale route‐learning task and that on three small‐scale tasks (maze learning, perspective taking, map use) showed no relationship for the TD controls, and only a few non‐specific associations in the MLD and WS groups.     

Previous reward decreases errors of commission on later ‘No‐Go’ trials in children 4 to 12 years of age: evidence for a context monitoring account

Inhibitory control is widely hypothesized to be the cornerstone of executive function in childhood and the central deficit in a number of developmental disorders, including attention‐deficit/hyperactivity disorder (ADHD). However, recent evidence from adults indicates that performance on response inhibition tasks may primarily reflect non‐inhibitory attentional control (context monitoring) processes. Yet it may be that inhibition plays a more central role in childhood – a time when the architecture of cognitive processes might be more transparent due to wide variability in skill level. Here we directly test inhibitory and context monitoring explanations of task performance on a Go/No‐Go task in a large group of children 4–12 years of age. We conclude that traditional inhibitory conceptualizations of task performance on the Go/No‐Go task cannot account for our findings, calling into question evidence supporting a central role for inhibitory control in cognitive development or developmental psychopathology.     

Sport-specific decision-making in a Go/NoGo reaction task: difference among nonathletes and baseball and basketball players

The present study examined whether Go/Nogo reaction time (RT) is a relevant index of the sport expertise relating to sport-specific decision-making. 57 male university students, 20 basketball players, 24 baseball players, and 13 sedentary students as a control group, performed a Simple RT task and Go/NoGo RT task which had baseball specific stimulus-response relations. Participants in baseball and basketball differed further in having high, medium, and low experience in the sports. For comparisons across sports, the basketball and the baseball players had significantly shorter reaction times than the nonathletes in both tasks. In contrast, reaction times varied significantly across experience for the baseball players in the Go/NoGo RT task but not for basketball players. These results suggested that Go/NoGo RT could be used as an index of expertise for sport-specific decision-making, if stimulus-response relation in Go/NoGo RT task has a natural relation for a particular sport-domain.     

Motor excitability during movement preparation in Tourette syndrome

Tourette syndrome (TS) is a neurodevelopmental disorder characterized by the occurrence of motor and vocal tics. TS has been linked to the impaired operation of cortical‐striatal‐thalamic‐cortical circuits that give rise to hyper‐excitability of cortical motor areas, which may be exacerbated by dysfunctional intra‐cortical inhibitory mechanisms. That said, many individuals gain control over their tics during adolescence and it has been suggested that this increased control arises as a result of the development of mechanisms that operate to suppress corticospinal excitability (CSE) ahead of volitional movements. Here we used single‐pulse transcranial magnetic stimulation (TMS) in conjunction with a manual Go/NoGo task to investigate alterations in CSE ahead of volitional movements in a group of adolescents with TS (N = 10). Our study demonstrated that CSE, as measured by TMS‐induced motor‐evoked potentials (MEPs), was significantly reduced in the TS group in the period immediately preceding a finger movement. More specifically, we show that individuals with TS, unlike their age‐matched controls, do not exhibit the predicted increase in mean MEP amplitude and decrease in MEP variability that immediately precede the execution of volitional movements in typically developing young adults. Finally, we report that the magnitude of the rise in MEP amplitude across the movement preparation period in TS is significantly negatively correlated with clinical measures of motor tic severity, suggesting that individuals with severe motor tics are least able to modulate motor cortical excitability.     

Emotions in Go/NoGo conflicts

On the basis of current emotion theories and functional and neurophysiological ties between the processing of conflicts and errors on the one hand and errors and emotions on the other hand we predicted that conflicts between prepotent Go responses and occasional NoGo trials in the Go/NoGo task would induce emotions. Skin conductance responses (SCRs), corrugator muscle activity, and startle blink responses were measured in three experiments requiring speeded Go responses intermixed with NoGo trials of different relative probability and in a choice reaction experiment serving as a control. NoGo trials affected several of these emotion-sensitive indicators as SCRs and startle blinks were reduced whereas corrugator activity was prolonged as compared to Go trials. From the pattern of findings we suggest that NoGo conflicts are not aversive. Instead, they appear to be appraised as obstructive for the response goal and as less action relevant than Go trials.     

Brain and behavioral inhibitory control of kindergartners facing negative emotions

Inhibitory control (IC) – one of the most critical functions underlying a child's ability to self‐regulate – develops significantly throughout the kindergarten years. Experiencing negative emotions imposes challenges on executive functioning and may specifically affect IC. In this study, we examined kindergartners' IC and its related brain activity during a negative emotional situation: 58 children (aged 5.5–6.5 years) performed an emotion‐induction Go/NoGo task. During this task, we recorded children's performance and brain activity, focusing on the fronto‐central N2 component in the event‐related potential (ERP) and the power of its underlying theta frequency. Compared to Go trials, inhibition of NoGo trials was associated with larger N2 amplitudes and theta power. The negative emotional experience resulted in better IC performance and, at the brain level, in larger theta power. Source localization of this effect showed that the brain activity related to IC during the negative emotional experience was principally generated in the posterior frontal regions. Furthermore, the band power measure was found to be a more sensitive index for children's inhibitory processes than N2 amplitudes. This is the first study to focus on kindergartners' IC while manipulating their emotional experience to induce negative emotions. Our findings suggest that a kindergartner's experience of negative emotion can result in improved IC and increases in associated aspects of brain activity. Our results also suggest the utility of time‐frequency analyses in the study of brain processes associated with response inhibition in young children.     

The Swedish Hayling task,and its relation to working memory,verbal ability,and speech‐recognition‐in‐noise

Cognitive functions and speech‐recognition‐in‐noise were evaluated with a cognitive test battery, assessing response inhibition using the Hayling task, working memory capacity (WMC) and verbal information processing, and an auditory test of speech recognition. The cognitive tests were performed in silence whereas the speech recognition task was presented in noise. Thirty young normally‐hearing individuals participated in the study. The aim of the study was to investigate one executive function, response inhibition, and whether it is related to individual working memory capacity (WMC), and how speech‐recognition‐in‐noise relates to WMC and inhibitory control. The results showed a significant difference between initiation and response inhibition, suggesting that the Hayling task taps cognitive activity responsible for executive control. Our findings also suggest that high verbal ability was associated with better performance in the Hayling task. We also present findings suggesting that individuals who perform well on tasks involving response inhibition, and WMC, also perform well on a speech‐in‐noise task. Our findings indicate that capacity to resist semantic interference can be used to predict performance on speech‐in‐noise tasks.     

Face-to-face interference in typical and atypical development

Visual communication cues facilitate interpersonal communication. It is important that we look at faces to retrieve and subsequently process such cues. It is also important that we sometimes look away from faces as they increase cognitive load that may interfere with online processing. Indeed, when typically developing individuals hold face gaze it interferes with task completion. In this novel study we quantify face interference for the first time in Williams syndrome (WS) and Autism Spectrum Disorder (ASD). These disorders of development impact on cognition and social attention, but how do faces interfere with cognitive processing? Individuals developing typically as well as those with ASD (n = 19) and WS (n = 16) were recorded during a question and answer session that involved mathematics questions. In phase 1 gaze behaviour was not manipulated, but in phase 2 participants were required to maintain eye contact with the experimenter at all times. Looking at faces decreased task accuracy for individuals who were developing typically. Critically, the same pattern was seen in WS and ASD, whereby task performance decreased when participants were required to hold face gaze. The results show that looking at faces interferes with task performance in all groups. This finding requires the caveat that individuals with WS and ASD found it harder than individuals who were developing typically to maintain eye contact throughout the interaction. Individuals with ASD struggled to hold eye contact at all points of the interaction while those with WS found it especially difficult when thinking.     

Unpleasant stimuli differentially modulate inhibitory processes in an emotional Go/NoGo task: an event-related potential study

Threat stimuli typically elicit a psychophysiological response pattern supporting the organism's preparation for active defence. Differently, blood stimuli prompt a distinctive autonomic response pattern and sustained processing, which do not call for clear-cut mobilisation for action. However, the contribution of motor disposition in these response patterns remains unclear. One way to address this issue is to investigate whether threat and blood stimuli differentially affect the active suppression of an ongoing motor activity. Thirty-two undergraduates were presented with threat, mutilation, pleasant, and neutral pictures in an emotional Go/NoGo task. The amplitudes of the NoGo-N2 and NoGo-P3 components of the event-related potentials were analysed as indices of conflict monitoring and inhibition of motor response, respectively. Reaction times to Go trials were significantly faster for threat than for mutilations. The NoGo-N2 was significantly larger to threat than to mutilations, whereas the NoGo-P3amplitude did not differ between the two conditions. These findings suggest that threat stimuli facilitated the execution of a prepotent response and enhanced conflict monitoring when action must be withheld. In contrast, blood stimuli did not either promote action in the Go trials or increase conflict in the NoGo condition, suggesting a response pattern compatible with defensive immobility.     

Everyday executive function in standard-risk acute lymphoblastic leukemia survivors

Although absolute pitch (AP) is a rare skill in typical development, individuals with Williams syndrome (WS) are often referred to as possessing this musical ability. However, there is paucity of research on the topic. In this article, 2 studies were conducted to evaluate AP in WS. In Study 1, seven musically trained individuals with WS, 14 musically trained typically developing controls matched for chronological age, and 2 experienced musicians with AP completed a pitch-identification task. Although the task was a classical assessment of AP, it required participants to have musical knowledge, and the availability and accessibility of musically trained individuals with WS is very low. In Study 2, a paradigm suitable for evaluating AP in individuals without musical training was used, which made it possible to evaluate a larger group of participants with WS. A pitch memory test for isolated tones was presented to 27 individuals with WS, 54 typically developing peers matched for chronological age, and the 2 musicians with AP. Both individuals with WS and their controls obtained low results in the two studies. They showed an arbitrary pattern of response, and their performance was far from that of musicians with AP. Therefore, participants with WS did not appear to possess AP. Unlike what is usually claimed, results suggest that AP is not a remarkable ability in WS and that, as in the typically developing population, this musical ability is also rare in individuals with WS.     

Do children with autism use inner speech and visuospatial resources for the service of executive control? Evidence from suppression in dual tasks

Three experiments used dual‐task suppression methodology to study the use of inner speech and visuospatial resources for mediating central executive performance by children with autism (CWA) and group‐matched typically developing (TD) controls. Expt 1 revealed that CWA did not recruit inner speech to facilitate arithmetic task‐switching performance: there was no effect of articulatory suppression (AS) on completion time for CWA compared to the TD group. Expt 2 revealed that suppression of visuospatial resources disrupted the task‐switching performance of both CWA and TD groups. It also confirmed that the task‐switching performance of CWA was significantly slowed by visuospatial compared to AS. Expt 3 showed that CWA also did not employ inner speech, compared to visuospatial resources, for implementing planning movements. Overall, compared to the mixture of representations used by the TD group for problem solving, CWA seemed to use visuospatial working memory resources but not inner speech to service executive control.     

Assessing inattention and impulsivity in children during the Go/NoGo task

Behavioural performance in the Go/NoGo task was compared with caregiver and teacher reports of inattention and hyperactivity‐impulsivity in 1,151 children (N=557 boys; N=594 girls) age 9–10 years old. Errors of commission (NoGo errors) were significantly correlated with symptom counts of hyperactivity‐impulsivity, while errors of omission (Go errors) were significantly correlated with symptom counts for inattention in both caregiver and teacher reports. Cross‐correlations were also evident, however, such that errors of commission were related to inattention symptoms, and errors of omission were related to hyperactivity‐impulsivity. Moreover, hyperactivity‐impulsivity and inattention symptoms were highly intercorrelated in both caregiver (r=.52) and teacher reports (r=.70), while errors of commission and omission were virtually uncorrelated in the Go/NoGo task (r=.06). The results highlight the difficulty in disentangling hyperactivity‐impulsivity and inattention in questionnaires, and suggest that these constructs may be more clearly distinguished in laboratory measures such as the Go/NoGo task.     

Executive cognitive functioning and aggression: Is it an issue of impulsivity?

A large body of research has documented a relation between the executive cognitive functions (ECFs) and interpersonal aggressive behavior. A predominant theory proposes that individuals with poor ECFs are more aggressive because they are unable to inhibit impulsive behaviors. However, evidence for this relationship is typically indirect. In this study, 46 healthy men and women completed measures of ECF, the Taylor Aggression Paradigm, and the Go/No‐Go discrimination task, a behavioral measure of impulsivity. Also, impulsiveness of participant responses during the aggression task was directly assessed by measuring latency of responses to provocation (“set‐time”). It was hypothesized that low‐quartile–scoring ECF men and women would perform more aggressively and more impulsively than high‐quartile peers. Consistent with expectations, results indicated that ECF was related to aggression and to impulsivity on the Go/No‐Go task. However, low‐ECF men and women did not have shorter set‐times; in fact, on this task, low‐ECF participants' behavioral decisions seemed slightly slower than those of high‐ECF participants. In light of these results, the authors speculate that a social information‐processing problem may mediate the ECF aggression relationship rather than altered impulsivity per se. Aggr. Behav. 29:15–30, 2003. © 2003 Wiley‐Liss, Inc.     

Working memory functioning in developmental dyslexia

Working memory impairments in dyslexia are well documented. However, research has mostly been limited to the phonological domain, a modality in which people with dyslexia have a range of problems. In this paper, 22 adult students with dyslexia and 22 age- and IQ-matched controls were presented with both verbal and visuospatial working memory tasks. Performance was compared on measures of simple span, complex span (requiring both storage and processing), and dynamic memory updating in the two domains. The dyslexic group had significantly lower spans than the controls on all the verbal tasks, both simple and complex, and also on the spatial complex span measure. Impairments remained on the complex span measures after controlling statistically for simple span performance, suggesting a central executive impairment in dyslexia. The novelty of task demands on the initial trials of the spatial updating task also proved more problematic for the dyslexic than control participants. The results are interpreted in terms of extant theories of dyslexia. The possibility of a supervisory attentional system deficit in dyslexia is also raised. It seems clear that working memory difficulties in dyslexia extend into adulthood, can affect performance in both the phonological and visuospatial modalities, and implicate central executive dysfunction, in addition to problems with storage.     

Impaired executive functions in subjects with frequent nightmares as reflected by performance in different neuropsychological tasks

Nightmare disorder is a prevalent parasomnia characterized by vivid and highly unpleasant dream experiences during night time sleep. The neural background of disturbed dreaming was proposed to be associated with impaired prefrontal and fronto-limbic functioning during REM sleep. We hypothesized that the impaired prefrontal and fronto-limbic functioning in subjects with frequent nightmares would be reflected at the behavioral level during waking tasks as well. 35-35 Subjects with frequent nightmares and matched controls participated in Study 1, involving an Emotional Go/NoGo, an Emotional Stroop task, and a Verbal Fluency task. Nightmare subjects exhibited longer reaction times in the Emotional Go/NoGo and Emotional Stroop tasks. Moreover, they committed more perseveration errors and showed less fluent word generation in the Verbal Fluency task. Nightmare subjects showed an overall slowing irrespective of the valence of the stimuli. While the effects of sleep quality and waking anxiety were associated to these deficits in some cases, these factors could not solely explain the difference between the two groups. In Study 2, 17 subjects with frequent nightmares and 18 controls were compared by a Color-word and an Emotional, block design Stroop task in order to avoid the slow effects of emotional interference potentially caused by previous items. Nightmare subjects were characterized by an overall slowing in the Emotional Stroop task, irrespective of the valence of the stimuli. In the Color-word Stroop task, nightmare subjects were not significantly slower in comparison with controls. Our results suggest that individuals with frequent nightmares are impaired in executive tasks involving the suppression of task-irrelevant semantic representations.     

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.