A three-years follow-up of extra intensive support for individuals with intellectual disability and severe challenging behaviour in the Netherlands.

Adults with Intellectual Disability who show severe challenging behaviour need intensive individual support. If intensive support proves to be insufficient, extra intensive support can be provided in the Netherlands, which is characterized by more time for individual care. The present study evaluates the impact of extra intensive support over time. Client characteristics of adults receiving intensive support (IS, N=70) or extra intensive support (IS+, N=35) are compared and the impact of provided support on challenging behaviour (Developmental Behaviour Checklist-Adults), adaptive behaviour (Vineland II), and Quality of Life (San Martin Scale) is evaluated over a three years period. Compared to adults receiving intensive support, those receiving extra intensive support initially showed higher intensity of challenging behaviour, higher number of mental health diagnoses and stronger focus on goals to reduce challenging behaviour. Over time, intensity of challenging behaviour decreased in adults receiving extra intensive support, although Quality of Life and adaptive functioning did not improve. Results show that the indications for receiving extra intensive support are clear and that the extra support is effective over time. It is concluded that extra individual support is serving those who need this support.

Recognition of Facial Emotion and Affective Prosody in Children at High Risk of Criminal Behavior.

OBJECTIVE: Emotion recognition is an important aspect of emotion processing, which is needed for appropriate social behavior and normal socialization. Previous studies in adults with antisocial personality disorder or psychopathy, in those convicted of criminal behavior, or in children with conduct disorder show impairments in negative emotion recognition. The present study investigated affective facial and prosody recognition in a sample of children at high risk of developing future criminal behavior. METHODS: Participants were 8- to 12-year-old children at high risk of developing criminal behavior (N=219, 83.1% boys) and typically developing controls (N=43, 72.1% boys). The high-risk children were recruited through an ongoing early intervention project of the city of Amsterdam, that focuses on the underage siblings or children of delinquents, and those failing to attend school. Facial and vocal recognition of happy, sad, angry, and fear was measured with the Facial Emotion Recognition (FER) test and the prosody test of the Amsterdam Neuropsychological Tasks (ANT), respectively. RESULTS: The high-risk group was significantly worse in facial affect recognition and had particular problems with fear and sadness recognition. No hostile attribution bias was found. The high-risk group did not differ from controls in affective prosody recognition but needed significantly more time to recognize emotions. CONCLUSIONS: The emotion-specific deficits found in forensic and clinical populations are already present in a sample of children at high risk of developing future criminal behavior. These findings help us understand a possible underlying mechanism of antisocial behavior that could provide directions for tailored interventions. (JINS, 2019, 25, 57-64).

The impact of metabolic control and tetrahydrobiopterin treatment on health related quality of life of patients with early-treated phenylketonuria: A PKU-COBESO study.

The aim of this study was to examine Health-Related Quality of Life (HRQoL) of patients with Phenylketonuria (PKU) in three different age groups and to investigate the impact of metabolic control and tetrahydrobiopterin (BH4) treatment on HRQoL of these patients. Participants were 90 early-treated patients aged 7 to 40 years (M = 21.0, SD = 10.1) and 109 controls aged 7 to 40.8 years (M = 19.4, SD = 8.6). HRQoL was assessed with the (generic) TNO-AZL questionnaires. Overall, good HRQoL was reported for children below 12 years of age, although they were judged to be less autonomic than their healthy counterparts. Adolescents aged 12-15 years showed poorer HRQoL in the domain "cognitive functioning" compared to controls. For adults ≥16 years, poorer age-controlled HRQoL was found for the domains cognition, depressive moods, and anger, with a further trend for the domain "pain". With respect to metabolic control, only for adult PKU-patients robust associations were observed, indicating poorer functioning, most notably in the domains cognition, sleep, pain, sexuality and anger, with higher historical and concurrent Phe-levels. With respect to BH4-use, effects on HRQoL were again only observed for adult PKU-patients. After controlling for age and historical Phe-levels, small but significant differences in favor of adult BH4-users compared to non-users were observed for HRQoL-categories happiness, anger, and social functioning. Together, these results show that, particularly for adult PKU-patients, HRQoL-problems are evident and that many of these problems are related to (history of) metabolic control. Beneficial effects of BH4-use appear to be limited to those associated with relief from the practical burdens related to the strict dietary treatment regimen, i.e. general mood and sociability, whereas metabolic control is more strongly related to basic physical and cognitive functioning.

International investigation of neurocognitive and behavioral phenotype in 47,XXY (Klinefelter syndrome): Predicting individual differences.

47,XXY (KS) occurs in 1:650 male births, though less than 25% are ever identified. We assessed stability of neurocognitive features across diverse populations and quantified factors mediating outcome. Forty-four boys from the Netherlands (NL) and 54 boys from the United States (US) participated. The Wechsler Intelligence Scales assessed intellectual functioning; the ANT program evaluated cognitive function; and the CBCL assessed behavioral functioning. ANOVA was used for group comparisons. Hierarchical regressions assessed variance explained by each independent variable: parental education, timing of diagnosis, testosterone, age, and nationality. Parental education, timing of diagnosis, and hormonal treatment all played an important role in neurocognitive performance. The observed higher IQ and better attention regulation in the US group as compared to the NL group was observed with decreased levels of behavioral problems in the US group. Cognitive measures that were different between the NL and US groups, i.e., attention regulation and IQ scores, were also significantly influenced by external factors including timing of diagnosis, testosterone treatment, and parental education. On the ANT, a cognitive phenotype of 47,XXY was observed, with similar scores on 9 out of the 10 ANT subtests for the NL and US groups. This study lays additional features to the foundation for an algorithm linking external variables to outcome on various neurodevelopmental measures.

Late, but not early, arriving younger siblings foster firstborns' understanding of second-order false belief.

This study examined the influence of younger siblings on children's understanding of second-order false belief. In a representative community sample of firstborn children (N=229) with a mean age of 7years (SD=4.58), false belief was assessed during a home visit using an adaptation of a well-established second-order false belief narrative enacted with Playmobil figures. Children's responses were coded to establish performance on second-order false belief questions. When controlling for verbal IQ and age, the existence of a younger sibling predicted a twofold advantage in children's second-order false belief performance, yet this was the case only for firstborns who experienced the arrival of a sibling after their second birthday. These findings provide a foundation for future research on family influences on social cognition.

Long-Term Follow-Up of Cognition and Mental Health in Adult Phenylketonuria: A PKU-COBESO Study.

Cognitive and mental health problems in individuals with the inherited metabolic disorder phenylketonuria (PKU) have often been associated with metabolic control and its history. For the present study executive functioning (EF) was assessed in 21 PKU patients during childhood (T1, mean age 10.4 years, SD = 2.0) and again in adulthood (T2, mean age 25.8 years, SD = 2.3). At T2 additional assessments of EF in daily life and mental health were performed. Childhood (i.e. 0-12 years) blood phenylalanine was significantly related to cognitive flexibility, executive motor control, EF in daily life and mental health in adulthood (i.e. at T2). Patients with a greater increase in phenylalanine levels after the age of 12 performed more poorly on EF-tasks at T2. Group-based analyses showed that patients with phenylalanine <360 µmol/L in childhood and phenylalanine ≥360 µmol/L from age 13 onwards (n = 11) had better cognitive flexibility and executive motor control than those who had phenylalanine ≥360 µmol/L throughout life (n = 7), supporting the notion that phenylalanine should be below the recommended upper treatment target of 360 µmol/L during childhood for better outcome in adulthood. Despite some results indicating additional influence of phenylalanine levels between 13 and 17 years of age, evidence for a continued influence of phenylalanine levels after childhood on adult outcomes was largely lacking. This may be explained by the fact that the patients in the present study had relatively low phenylalanine levels during childhood (mean: 330 µmol/L, range: 219-581 µmol/L) and thereafter (mean Index of Dietary Control at T2: 464 µmol/L, range: 276-743 µmol/L), which may have buffered against transitory periods of poor metabolic control during adolescence and early adulthood.

Affective empathy, cognitive empathy and social attention in children at high risk of criminal behaviour.

BACKGROUND: Empathy deficits are hypothesized to underlie impairments in social interaction exhibited by those who engage in antisocial behaviour. Social attention is an essential precursor to empathy; however, no studies have yet examined social attention in relation to cognitive and affective empathy in those exhibiting antisocial behaviour. METHODS: Participants were 8- to 12-year-old children at high risk of developing criminal behaviour (N = 114, 80.7% boys) and typically developing controls (N = 43, 72.1% boys). The high-risk children were recruited through an ongoing early identification and intervention project of the city of Amsterdam, focusing on the underage siblings or children of delinquents and those failing primary school. Video clips with neutral and emotional content (fear, happiness and pain) were shown, while heart rate (HR), skin conductance level (SCL) and skin conductance responses (SCRs) were recorded to measure affective empathy. Answers to questions about emotions in the clips were coded to measure cognitive empathy. Eye-tracking was used to evaluate visual scanning patterns towards social relevant cues (eyes and face) in the clips. RESULTS: The high-risk group did not differ from the control group in social attention and cognitive empathy, but showed reduced HR to pain and fear, and reduced SCL and SCRs to pain. CONCLUSIONS: Children at high risk of developing criminal behaviour show impaired affective empathy but unimpaired social attention and cognitive empathy. The implications for early identification and intervention studies with antisocial children are discussed.

Social-cognitive functioning and social skills in patients with early treated phenylketonuria: a PKU-COBESO study.

OBJECTIVE: Early treatment of phenylketonuria (ET-PKU) prevents mental retardation, but many patients still show cognitive and mood problems. In this study, it was investigated whether ET-PKU-patients have specific phenylalanine (Phe-)related problems with respect to social-cognitive functioning and social skills. METHODS: Ninety five PKU-patients (mean age 21.6 ± 10.2 years) and 95 healthy controls (mean age 19.6 ± 8.7 years) were compared on performance of computerized and paper-and-pencil tasks measuring social-cognitive abilities and on parent- and self-reported social skills, using multivariate analyses of variance, and controlling for general cognitive ability (IQ-estimate). Further comparisons were made between patients using tetrahydrobiopterin (BH4, N = 30) and patients not using BH4. Associations with Phe-levels on the day of testing, during childhood, during adolescence and throughout life were examined. RESULTS: PKU-patients showed poorer social-cognitive functioning and reportedly had poorer social skills than controls (regardless of general cognitive abilities). Quality of social-cognitive functioning was negatively related to recent Phe-levels and Phe-levels between 8 and 12 years for adolescents with PKU. Quality of social skills was negatively related to lifetime phenylalanine levels in adult patients, and specifically to Phe-levels between 0 and 7, and between 8 and 12 years. There were no differences with respect to social outcome measures between the BH4 and non-BH4 groups. CONCLUSION: PKU-patients have Phe-related difficulties with social-cognitive functioning and social skills. Problems seem to be more evident among adolescents and adults with PKU. High Phe-levels during childhood and early adolescence seem to be of greater influence than current and recent Phe-levels for these patients.

Parental Perceptions of Aggressive Behavior in Preschoolers: Inhibitory Control Moderates the Association With Negative Emotionality.

Inhibitory control (IC) and negative emotionality (NE) are both linked to aggressive behavior, but their interplay has not yet been clarified. This study examines different NE × IC interaction models in relation to aggressive behavior in 855 preschoolers (aged 2-5 years) using parental questionnaires. Hierarchical regression analyses revealed that NE and IC predict aggression both directly and interactively. The highest aggression levels were reported in children with high NE and low IC. Interestingly, the protective effect of IC for aggressive behavior increases with rising levels of NE. Analyses focusing on physical aggression revealed a significant NE × IC interaction in boys aged 4-5 years only. These findings shed new light on potential compensatory mechanisms for aggressive behavior in developing children.

Is BRIEF a useful instrument in day to day care of patients with phenylketonuria?

OBJECTIVES: Despite early and continuous treatment many patients with phenylketonuria (PKU) still experience neurocognitive problems. Most problems have been observed in the domain of executive functioning (EF). For regular monitoring of EF, the use of the Behavior Rating Inventory of Executive Function (BRIEF) has been proposed. The aim of this study was to investigate whether the BRIEF is indeed a useful screening instrument in monitoring of adults with PKU. STUDY DESIGN: Adult PKU patients (n = 55; mean age 28.3 ± 6.2 years) filled out the BRIEF-A (higher scores=poorer EF) and performed computerized tasks measuring executive functions (inhibition, cognitive flexibility, and working memory). The outcome of the BRIEF-A questionnaire was compared with the neurocognitive outcome as measured by three tasks from the Amsterdam Neuropsychological Tasks (ANT). RESULTS: Forty-two percent of the PKU patients scored in the borderline/clinical range of the BRIEF-A. Six of the 55 patients (11%) scored >1 SD above the normative mean, mostly on the Metacognition Index. With respect to ANT measurements, patients mainly showed deficits in inhibitory control (34-36%) and cognitive flexibility (31-40%) as compared to the general Dutch population. No significant correlations between the two methods were found, which was confirmed with the Bland-Altman approach where no agreement between the two methods was observed. Only with respect to inhibitory control, patients scored significantly worse on both BRIEF-A and ANT classifications. No other associations between classification according to the BRIEF-A and classifications according to the ANT tasks were found. CONCLUSIONS: Patients reporting EF problems in daily life are not necessarily those that present with core EF deficits. The results of this study suggest that regular self-administration of the BRIEF-A is not a sufficient way to monitor EF in adult PKU patients.

Executive Dysfunction 25 Years after Treatment with Cranial Radiotherapy for Pediatric Lymphoid Malignancies.

The first cohorts to survive childhood lymphoid malignancies treated with cranial irradiation are now aging into adulthood, and concerns are growing about the development of radiotherapy-induced cognitive deficits in the aging brain. These deficits are hypothesized to increase over time. Their impact on daily functioning of older survivors, and the accompanying need for interventions, should be anticipated. By describing a detailed profile of executive function deficits and their associations with age, specific targets for neuropsychological intervention can be identified. Fifty survivors of childhood lymphoid malignancies and 58 related controls were assessed with the Amsterdam Neuropsychological Tasks program. The survivors were on average 31.1 (4.9) years old, treated with 22.5 (6.8) Gy cranial irradiation, and examined on average 25.5 (3.1) years after diagnosis. The survivors showed significantly decreased response speed, irrespective of the task at hand. Furthermore, we found deficits in working memory capacity, inhibition, cognitive flexibility, executive visuomotor control, attentional fluctuations, and sustained attention. Older age was associated with poorer performance on executive visuomotor control and inhibition. On executive visuomotor control, 50% of female survivors performed more than 1.5 SD below average, versus 15.4% of male survivors. The combination of visuospatial working memory problems and decreasing executive visuomotor control could result in difficulty with learning new motor skills at older ages, like walking with a cane. Deterioration of executive control and inhibition may result in decreased behavioral and emotional regulation in aging survivors. Especially the deficiency in executive visuomotor control in female survivors should be considered for (prophylactic) intervention.

Processing of facial and nonsocial information is differentially associated with severity of symptoms in patients with multiepisode schizophrenia.

Patients with schizophrenia show impairments in social cognitive abilities, such as recognizing facial emotions. However, the relation to symptoms remains unclear. The goal of this study was to explore whether facial emotion recognition and face identity recognition are associated with severity of symptoms and to which extent associations with symptoms differ for processing of social versus nonsocial information. Facial emotion recognition, face recognition, and abstract pattern recognition were evaluated in 98 patients with multiepisode schizophrenia. Severity of symptoms was measured using a five-factor model of the Positive and Negative Syndrome Scale. Results show that facial emotion recognition and, to a lesser extent, face recognition were predominantly associated with severity of disorganization symptoms. In contrast, recognition of nonsocial patterns was associated with negative symptoms, excitement, and emotional distress. Reaction time rather than accuracy of social cognition explained variance in symptomatology. These results lead to the conclusion that facial emotion processing in schizophrenia appears to be associated with severity of symptoms, especially disorganization.

Neurocognitive evidence for revision of treatment targets and guidelines for phenylketonuria.

OBJECTIVES: To compare the neurocognitive outcomes of patients with phenylketonuria (PKU) to determine whether decreasing phenylalanine (Phe) levels to <240 is preferable to the use of 360 µmol/L as an upper-target Phe level. An additional aim was to establish the influence of biochemical indices other than Phe on neurocognitive outcomes. STUDY DESIGN: Patients with PKU (n = 63; mean age 10.8 ± 2.3 years) and healthy controls (n = 73; mean age 10.9 ± 2.2 years) performed computerized tasks measuring neurocognitive functions (inhibitory control, cognitive flexibility, and motor control). Lifetime and concurrent blood Phe levels, Phe-to-tyrosine ratio (Phe:Tyr), and Phe variations were examined in relation to neurocognitive outcomes using nonparametric tests and regression analyses. RESULTS: Patients with PKU with Phe levels ≤240 µmol/L and healthy controls performed equally well. Patients with Phe levels between 240 and 360 µmol/L and ≥360 µmol/L performed more poorly than did controls across tasks. Patients with Phe levels ≤240 µmol/L performed significantly better than patients with levels between 240 and 360 µmol/L on tasks measuring inhibitory control and cognitive flexibility. Absolute Phe levels and Phe variation were the best predictors of motor control, whereas Phe:Tyr were the best predictors of inhibitory control. CONCLUSIONS: The results of this study suggest that upper Phe targets should be lowered to optimize neurocognitive outcomes. Moreover, Phe variation and Phe:Tyr appear to be of additional value in treatment monitoring.

Quality of life: a case-controlled long-term follow-up study, comparing young high-functioning adults with autism spectrum disorders with adults with other psychiatric disorders diagnosed in childhood.

BACKGROUND: Long term outcome in childhood autism spectrum disorders (ASD) was evaluated by studying quality of life (QoL) in young adulthood in comparison to the outcome of other child psychiatric disorders. METHODS: In this follow-up study, objective and subjective QoL of 169 high-functioning (IQ>70) adults with ASD (19 to 30 years) was contrasted with QoL data of age matched adults diagnosed with attention deficit/hyperactivity disorder (N=85), disruptive behaviour disorders (N=83), and affective disorders (N=85) during childhood. The mean follow-up period of the ASD patients was 13.9 years. Objective QoL included marital status, living arrangements, level of education, employment, and usage of mental health care. Subjective QoL included satisfaction concerning living arrangements, work or education, physical condition, partner relationship, social relationships, state of mind, and future perspective. RESULTS: QoL was more compromised in adults diagnosed with ASD in childhood than in adults with other psychiatric disorders in childhood. A relatively large proportion of the adults with ASD were single, few lived with a partner or a family and many of them were institutionalized. Adults with ASD had lower educational levels, relatively few had paid employment and many were social security recipients, as compared to the other psychiatric patients. In case the adults with ASD used medication, 47% used anti-psychotics. Regarding the subjective QoL, the adults with ASD were less satisfied about their work or education, partner relationship, and future perspective than the other groups. Even when highly educated adults with ASD were compared to highly educated adults diagnosed with other childhood disorders, the QoL appeared to be more disadvantageous in adults with ASD. CONCLUSION: Many studies have shown that QoL is threatened in psychiatric patients, but findings of this study indicate that young high-functioning adults diagnosed with ASD in childhood are at relatively high risk for poor QoL compared to other childhood psychiatric disorders.

Recognition of facial emotion and affective prosody in children with ASD (+ADHD) and their unaffected siblings.

Autism is a highly heritable and clinically heterogeneous neuropsychiatric disorder that frequently co-occurs with other psychopathologies, such as attention-deficit/hyperactivity disorder (ADHD). An approach to parse heterogeneity is by forming more homogeneous subgroups of autism spectrum disorder (ASD) patients based on their underlying, heritable cognitive vulnerabilities (endophenotypes). Emotion recognition is a likely endophenotypic candidate for ASD and possibly for ADHD. Therefore, this study aimed to examine whether emotion recognition is a viable endophenotypic candidate for ASD and to assess the impact of comorbid ADHD in this context. A total of 90 children with ASD (43 with and 47 without ADHD), 79 ASD unaffected siblings, and 139 controls aged 6-13 years, were included to test recognition of facial emotion and affective prosody. Our results revealed that the recognition of both facial emotion and affective prosody was impaired in children with ASD and aggravated by the presence of ADHD. The latter could only be partly explained by typical ADHD cognitive deficits, such as inhibitory and attentional problems. The performance of unaffected siblings could overall be considered at an intermediate level, performing somewhat worse than the controls and better than the ASD probands. Our findings suggest that emotion recognition might be a viable endophenotype in ASD and a fruitful target in future family studies of the genetic contribution to ASD and comorbid ADHD. Furthermore, our results suggest that children with comorbid ASD and ADHD are at highest risk for emotion recognition problems.

Inattention symptom severity and cognitive processes in children at risk of ADHD: the moderating role of separation anxiety.

Impairments in cognitive processes and their associations with dimensional measures of inattention, hyperactivity-impulsivity and anxiety were examined in children at risk of Attention-Deficit Hyperactivity Disorder. Children referred by teachers for exhibiting ADHD-type problems (n = 116; 43 meeting full diagnostic criteria for ADHD; 4-8 years) completed computerized tasks measuring episodic memory, response inhibition, visuomotor control and sustained attention, while parents were interviewed (DAWBA) to assess ADHD and anxiety symptoms. Of the 116 children assessed, 72% exhibited impaired cognitive processes; 47% had impaired visuomotor control, 37% impaired response inhibition, and 35% had impaired episodic memory. Correlational and hierarchical regression analyses using our final analytic sample (i.e., children who completed all cognitive tasks and a vocabulary assessment, n = 114) showed that poorer task performance and greater within-subject variability were significantly associated with more severe inattention symptoms but not with hyperactivity-impulsivity severity. Symptoms of separation anxiety, which were reported in over half of the sample, moderated associations between inattention and episodic memory, and between inattention and inhibition. Only children without separation anxiety showed significant correlations between ADHD symptoms and poor performance. However, separation anxiety had no moderating effect on associations between inattention and visuomotor control or sustaining attention. Children exhibiting signs of ADHD show impairments across a range of cognitive tasks. Further research to improve our understanding of these processes may be useful in the development of early interventions. Our results suggest that separation anxiety should be taken into account when considering interventions to address emerging neuropsychological deficits associated with this disorder.

Neuropsychological task outcomes among survivors of childhood acute lymphoblastic leukemia in Malaysia.

This study intended to explore the neuropsychological ramifications in childhood acute lymphoblastic leukemia (ALL) survivors in Malaysia and to examine treatment-related sequelae. A case-control study was conducted over a 2-year period. Seventy-one survivors of childhood ALL who had completed treatment for a minimum of 1 year and were in remission, and 71 healthy volunteers were enlisted. To assess alertness (processing speed) and essential executive functioning skills such as working memory capacity, inhibition, cognitive flexibility, and sustained attention, seven measures from the Amsterdam Neuropsychological Tasks (ANT) program were chosen. Main outcome measures were speed, stability and accuracy of responses. Mean age at diagnosis was 4.50 years (SD ± 2.40) while mean age at study entry was 12.18 years (SD ± 3.14). Survivors of childhood ALL underperformed on 6 out of 7 ANT tasks, indicating poorer sustained attention, working memory capacity, executive visuomotor control, and cognitive flexibility. Duration of treatment, age at diagnosis, gender, and cumulative doses of chemotherapy were not found to correlate with any of the neuropsychological outcome measures. Childhood ALL survivors in our center demonstrated significantly poorer neuropsychological status compared to healthy controls.

Mental health and social functioning in early treated Phenylketonuria: the PKU-COBESO study.

This article presents a new Dutch multicenter study ("PKU-COBESO") into cognitive and behavioral sequelae of early and continuously treated Phenylketonuria (PKU) patients. Part of the study sample will consist of young adult PKU patients who have participated in a large neuropsychological study approximately 10 years ago, when they were 7-to-15-year-olds (Huijbregts et al., 2002 [1]). Their neurocognitive development will be mapped in association with their earlier and continued metabolic history, taking into account possible changes in, for instance, medication. A second part of the sample will consist of PKU patients between the ages of 7 and approximately 40 years (i.e., born in or after 1974, when neonatal screening was introduced in The Netherlands), who have not participated in the earlier neuropsychological study. Again, their cognitive functioning will be related to their metabolic history. With respect to aspects of cognition, there will be an emphasis on executive functioning. The concept of executive functioning will however be extended with further emphasis on the impact of cognitive deficits on the daily lives of PKU patients, aspects of social cognition, social functioning, and behavior/mental health (i.e., COgnition, BEhavior, SOcial functioning: COBESO). In addition to a description of the PKU-COBESO study, some preliminary results with respect to mental health and social functioning will be presented in this article. Thirty adult PKU patients (mean age 27.8, SD 6.4) and 23 PKU patients under the age of 18 years (mean age 11.0, SD 3.3) were compared to 14 (mean age 26.9 years, SD 5.9) and 7 matched controls (mean age 10.5, SD 2.6) respectively, with respect to their scores on the Adult Self-Report or Child Behavior Checklist (measuring mental health problems) and the Social Skills Checklist or Social Skills Rating System (measuring social skills). Whereas there were very few significant group differences (except for mental health problems in the internalizing spectrum for adult PKU patients), possibly due to the small control groups, several significant associations between mental health problems and Phe levels were observed for the PKU patients. Childhood Phe levels and internalizing problems for adult PKU patients were related; concurrent Phe was associated with both internalizing and externalizing behavioral problems for those under the age of 18. These preliminary results underline the importance of early dietary adherence.

Impaired response inhibition in autism spectrum disorders, a marker of vulnerability to schizophrenia spectrum disorders?

In this study, we addressed the relation between specific deficits in cognitive control and schizotypal symptomatology in adolescents with autism spectrum disorders (ASD) diagnosed in childhood. We aimed to identify cognitive control deficits as markers of vulnerability to the development of schizophrenia spectrum pathology in ASD. Symptoms of autism and the risk for schizotypal symptomatology were assessed in 29 high-functioning adolescents with ASD, and compared with 40 typically developing adolescents. Cognitive control (response inhibition, mental flexibility, visuo-motor control, interference control, and perseveration) was evaluated for specific association with schizotypal symptomatology. Impaired response inhibition appeared to be strongly and specifically associated with schizotypal symptomatology in adolescents with ASD, especially those with positive and disorganized symptoms. Response inhibition problems could indicate vulnerability to the development of schizotypal symptomatology in ASD.

Cognitive control of feature bindings: evidence from children with autistic spectrum disorder.

Understanding how the brain integrates features from different domains that are processed in distinct cortical regions calls for the examination of integration processes. Recent studies of feature-repetition effects demonstrated interactions across perceptual features and action-related features: repeating only some features of the perception-action episode hinders performance. These partial-repetition costs point to the existence of temporary memory traces (event files). However, the principles and the constraints that govern the management of such traces are still unclear. Here, we investigated whether children with autistic spectrum disorder (ASD) differ from typically developing children in managing episodic memory traces. The results show that both groups integrate stimulus features along with action features, but children with ASD exhibit larger partial-repetition costs, suggesting lesser control and flexibility in updating episodic memory traces. The findings are discussed in the light of evidence for a central role of the dopaminergic system in cognitive integration, ASD, and cognitive control.