Measurement of restricted and repetitive behaviour in children with autism spectrum disorder: Selecting a questionnaire or interview

Assessment of children's restricted and repetitive behaviours offers potential opportunities to improve early diagnosis of autism spectrum disorder (ASD) and timely access to interventions and support. To facilitate this requires understanding of the phenomenology of repetitive behaviours in ASD, including differentiating behaviours seen in ASD from other populations such as young typically developing and developmentally delayed children. A key factor in achieving understanding is valid and reliable measurement.This review considers the issues in conceptualisation of repetitive behaviours which should guide the choice of measurement tool, including definition of subtypes of repetitive behaviours, associations with age and ability, and categorisation of frequency and severity. The resulting conclusions about the requirements for measurement are applied within a systematic review of quantitative interview and questionnaire measures of repetitive behaviour used with children with ASD. The conclusions of the review lead to recommendations for existing and future research on restricted and repetitive behaviours in ASD.     

Distinctiveness and correlates of maladaptive behaviour in children and adolescents with Smith–Magenis syndrome

This two-part study examines the distinctiveness and correlates of maladaptive behaviour in 35 children and adolescents with Smith–Magenis syndrome, a developmental disorder caused by an interstitial deletion of chromosome 17 (p11.2). Study I compares Child Behavior Checklist scores in 35 children with Smith–Magenis syndrome to age- and gender-matched subjects with Prader–Willi syndrome and mixed intellectual disability. Subjects with Smith–Magenis syndrome had significantly higher levels of maladaptive behaviour than the other groups. Although some problems were shared across groups, 12 behaviours differentiated the three groups with 100% accuracy. Study 2 assessed the frequency and correlates of self-injurious and stereotypical behaviours, including unusual features such as nail-yanking, inserting objects into bodily orifices, self-hugging and a ‘lick-and flip’ behaviour. Nail-yanking and bodily insertions were less common than other types of self-injury, and self-hugs and the ‘lick-and flip’ stereotypies were seen in about half the sample. Although age and degree of delay were correlated with problem behaviours, sleep disturbance emerged as the strongest predictor of maladaptive behaviour. The implications are discussed for clinical diagnostic ambiguities between the Smith–Magenis and Prader–Willi syndromes, and for intervention.     

One-year Change in Repetitive Behaviours in Young Children with Communication Disorders Including Autism

Repetitive behaviours are a relatively neglected area of study in autism. Previous research has concluded that repetitive behaviour is inversely related to ability and that it tends to increase over the preschool years. One-hundred and four children ages 24-48 months, with autism, autism spectrum disorder (ASD) or other disorders, were followed for 13 months. Twelve items from the Autism Diagnostic Interview (ADI-R) were analysed, as well as diagnostic algorithm scores. Ability was related to degree of repetitive behaviours, except for one cluster of relatively able children. ADI-R repetitive behaviour algorithm scores increased over time; however, when all 12 behaviours were considered, there was a general decrease in impact upon the child's and family's activities. Reasons for this decrease are discussed.     

The relationship between a deficit in attention switching and specific behaviours in Prader‐Willi syndrome

Background : We aimed to investigate the causes of phenotypic behaviours in Prader‐Willi syndrome (PWS). Interviews with carers of 46 children with PWS suggested that frequent temper outbursts and repetitive questions were related to a resistance to change. In a group comparison with FraX and typically developing children, PWS children showed a specific deficit in attention switching (direct assessment), which was correlated to informant scores of resistance to change and repetitive questions. We aimed to investigate neural correlates and hypothesized behavioural effects of the PWS deficit in attention switching. Method : Multiple single case experimental designs were employed with four individuals with PWS. Cognitive challenges placed demands on attention switching or controlled for these demands. Changes to routines or expectations were presented during controlled games, or imposed on participant’s natural environments, and compared to corresponding sessions when no changes were imposed. Behaviour was recorded and physiological arousal was estimated using heart rate measurements. Eight participants with PWS were compared to age and gender matched controls. Structural T1, T2 and diffusion weighted images were acquired in a Philips T3 MRI scanner. T2* weighted functional images were acquired in a block design (eight presentations of each block per participant) with alternating switching and non‐switching blocks. Results : In the single case experiments, significantly increased temper outburst related behaviours (p < 0.05) and arousal (decreased heart rate variability: p = 0.042) were shown in switching verses control challenges. Significantly increased temper outburst related behaviours were also shown when changes were imposed versus when no changes occurred (p < 0.03). Structural images suggest abnormalities in specific frontal regions in individuals with PWS and functional images revealed significant differences in switch‐related activation in individuals with PWS compared to controls. Conclusion : We propose a directional relationship between specific neural abnormalities, cognitive deficit and behaviour in PWS via particular environmental and physiological interactions.     

ADHD symptoms and insistence on sameness in Prader-Willi syndrome

Background Apart from a pervasive eating disorder, the Prader-Willi (PWS) syndrome is characterized by a distinct behavioural profile comprising maladaptive behaviours, obsessive-compulsive traits and skin picking, all included in the PWS behavioural phenotype. In this study, we present a further delineation of this characteristic behavioural profile by screening for indices of executive dysfunctions related to attention-deficit/hyperactivity disorder (ADHD), immature compulsive-like adherence to sameness and skin picking, and how these features aggregate into symptom constellations in children and adolescents with PWS. Method Parents of 58 individuals with PWS (aged 5–18 years) participated by completing Childhood Routines Inventory (CRI) and Conners’ Parent Rating Scale (CPRS-48). Results Results showed that indices of ADHD and excessive insistence on sameness were common, comorbid and of early onset. They were both associated with conduct problems. Skin picking, appearing as a single and comorbid symptom, was less associated with childlike compulsions and ADHD-related problems. Conclusions Findings are discussed in terms of further research in executive dysfunctions in PWS.     

Children with Prader–Willi syndrome vs. Williams syndrome: indirect effects on parents during a jigsaw puzzle task

Background Genetic disorders predispose individuals to exhibit characteristic behaviours, which in turn elicit particular behaviours from others. In response to the strength of Prader–Willi syndrome (PWS) and weakness of Williams syndrome (WS) in visual-spatial tasks such as jigsaw puzzles, parents’ behaviours can be affected by their child's level of puzzle ability or syndrome diagnosis. Methods Children were asked to complete two jigsaw puzzles (one with the experimenter and the other with the parent) for 5 min each. Frequencies of parental helping and reinforcement behaviours, along with ratings of parental directiveness, were examined as parents interacted with their children on a jigsaw puzzle task. Within each aetiological group, correlates of parental behaviours with child characteristics were also examined. Results Compared to parents of children with PWS, parents of children with WS engaged in a more directive style of interaction, and provided more help and reinforcement. Relative to parents of children with higher puzzle abilities (from both aetiologies), parents of children with lower abilities also showed the same pattern. Both the child's aetiology and puzzle abilities were important in predicting parents’ directiveness and helping and reinforcement behaviours. Within the PWS group, parents’ behaviours correlated negatively with the child's puzzle abilities and general cognitive functioning; no such relations occurred in the WS group. Conclusions Parents’ behaviours were affected by both the child's diagnosis and actual puzzle abilities, suggesting important implications for understanding and intervening with parents and children with different genetic syndromes.     

Stereotyped behaviours in blind children

Stereotyped behaviours occur frequently in blind children. Most authors attribute stereotyped mannerisms to factors such as hospitalisation, motor limitations, and reduced capacity for exploration. There seems to be a specific association between blindness and behavioural mannerisms, such as eye pressing and eye poking, which have been observed in children with peripheral blindness. We studied the prevalence of stereotyped motor behaviours in a sample of congenitally blind children with and without other neurodevelopmental disabilities in order to assess the types and features of such stereotyped behavioural traits. Twenty-six congenitally blind children (11 male and 15 female) were assessed through videotape recording and through a questionnaire focusing on the type, frequency, form of manifestation and duration of the children's stereotyped behaviours. Stereotyped behavioural traits were observed in 19 (73%) of the patients. Stereotyped behaviours most frequently observed were body rocking (8; 30.7%), repetitive handling of objects (8; 30.7%), hand and finger movements (7; 26.9%), eye pressing and eye poking (8; 30.7%), and lying face downwards (6; 22.8%) and jumping (3; 11.4%). We found that a reduction in stereotyped behavioural traits could be obtained by stimulating appropriate adaptive behaviour in children, while these behaviours were increased by restricted environmental conditions, reduced sensory stimulation and reduced motility.     

Parenting behaviour among parents of children with autism spectrum disorder

Contrary to the extensive amount of empirical findings about parental perceptions, parenting cognitions, and coping in families with a child with autism spectrum disorder (ASD), research about parenting itself is very scarce. A first goal of this study was to examine the factor structure and internal consistency of two scales to measure parenting behaviour: the Parental Behaviour Scale-short version (PBS, Van Leeuwen, 2002, Van Leeuwen and Vermulst, 2004, Van Leeuwen and Vermulst, 2010) and a new scale to measure parenting behaviours more specifically relevant to children with ASD. A second goal was to compare general and more specific parenting behaviour among parents of children with and without ASD. The participants were 305 parents of a child with ASD between 8 and 18 years old and 325 parents of a typically developing child between 8 and 18 years old. Parents completed both scales. Exploratory factor analysis of the new scale resulted in two factors: Stimulating the Development and Adapting the Environment. Multisample confirmatory factor analyses showed good fit indices for the noninvariant model of both the PBS and the new scale. Mean level analyses revealed significant main effects of group with higher mean scores for the control group in comparison with the experimental group for the subscale Harsh Punishment and the reversed effect for the subscale Stimulating the Development. We concluded that it is possible to use the parenting constructs of the PBS-short version to investigate parenting behaviour of parents with and without children with ASD. Furthermore, the new scale showed a relatively stable two factor structure for the control group as well as for the experimental group. The two factors ‘Stimulating the child's Development’ and ‘Adapting the child's Environment’ were also convincing as regards content. A first indication was found that parents of children with ASD indeed use more specifically relevant parenting behaviours. It is self-evident that more research is needed to gain further insight into the nature of these behaviours.     

An intervention to reduce disruptive behaviours in children with brain injury

This study investigated the effectiveness of a behavioural intervention programme in reducing disruptive behaviours in children with brain injury. The behavioural package included programme rules, a token economy with response cost and mystery motivators. Participants were three male patients in an after-school programme at a rehabilitation hospital who were identified as having both a brain injury and disruptive behaviours in the classroom setting. Two control composites were formed, one with children who behaved appropriately and one with children who behaved in a disruptive manner. This study employed a multiple baseline design across individuals. The participants’ disruptive behaviour decreased during the intervention phase by an average of 69%; the effect size of each participant's improvement was ‘large’. The comparisons’ disruptive behaviour was unchanged. This pronounced decrease in disruptive behaviours for the three participants was maintained in the follow-up phase. This short-term, easily implemented package altered important programme and social behaviours positively, were well received by children and staff and resulted in long-term improvements to behavioural deficits secondary to brain injury. These results are discussed in terms of theoretical disagreements, methodological issues and practical community-based interventions in brain-injured children.     

Development and behaviour in Marshall-Smith syndrome: an exploratory study of cognition, phenotype and autism

Background Marshall–Smith syndrome (MSS) is an infrequently described entity characterised by failure to thrive, developmental delay, abnormal bone maturation and a characteristic face. In studying the physical features of a group of patients, we noticed unusual behavioural traits. This urged us to study cognition, behavioural phenotype and autism in six patients. Methods Information on development, behavioural characteristics, autism symptoms, and adaptive and psychological functioning of six MSS children was collected through in‐person examinations, questionnaires, semi‐structured interviews of parents and neuropsychological assessments. Results Participants showed moderate to severe delays in mental age, motor development and adaptive functioning, with several similarities in communication, social interactions and behaviour. There was severe delay of speech and motor milestones, a friendly or happy demeanour and enjoyment of social interactions with familiar others. They exhibited minimal maladaptive behaviours. Deficits in communication and social interactions, lack of reciprocal social communication skills, limited imaginary play and the occurrence of stereotyped, repetitive behaviours were noted during assessments. Conclusions Systematic collection of developmental and behavioural data in very rare entities such as MSS allows recognition of specific patterns in these qualities. Clinical recognition of physical,developmental and behavioural features is important not only for diagnosis, prognosis and counselling of families, but also increases our understanding of the biological basis of the human physical and behavioural phenotype.     

Distinguishing features of autism in boys with fragile X syndrome

Background Males with fragile X syndrome and autism (FXS/autism) represent a distinct subgroup of males with FXS at risk for markedly poorer outcomes. Early identification and intervention can improve outcomes for males with autism spectrum disorder. Method To advance the development of a specialised autism screening tool for young males with FXS that could assist in early identification, backward regression was used to identify the combination of parent‐report questionnaire items that best predicted autism symptoms in a sample of 60 males with FXS, ages 4–18 years old. Results Both social and repetitive behaviours distinguished males with FXS/autism, with repetitive behaviours playing a more prominent role than previously documented in the literature. Conclusions Healthcare workers and early interventionists may be able to interview parents about a few key behaviours to determine if young child with FXS should be formally evaluated for autism. Evidence‐based practices identified for children with autism spectrum disorder can be implemented as early as possible.     

Stereotypies and repetitive/restrictive behaviours in infants with autism and pervasive developmental disorder

Purpose: Autism Spectrum Disorders (ASD) are characterized by severe and debilitating symptoms including stereotyped and repetitive behaviours. Stereotypies and repetitive behaviours constitute core features of ASD and markedly impede attempts to remediate the disorder. Little previous research has examined characteristics of the core features of ASD in infants.

Method: In the present study, 760 infants with autism, PDD-NOS or no diagnosis of ASD but at risk for other developmental delays or physical disabilities were evaluated with respect to the nature and extent of their stereotyped and ritualistic behaviour using the Baby and Infant Screen for Children with aUtIsm Traits (BISCUIT). A Kruskal-Wallis test with follow-up Mann-Whitney tests were employed to test for significant differences.

Results: Infants with autism evinced the highest amount of stereotypic behaviour, followed by those with PDD-NOS and atypical development. A sub-set of BISCUIT items could accurately predict diagnostic group membership.

Conclusions: These data suggest that many core features of ASD are distinct and can be reliably identified early in life. The potential early identification of these behavioural challenges could lead to earlier intervention practices and symptom alleviation for children in this population.     

Factor Analytic Study of Repetitive Behaviours in Young Children with Pervasive Developmental Disorders

The aim of the current study was to investigate the manifestation of repetitive behaviour profiles in young children with a Pervasive Developmental Disorder. The sample consisted of 137 developmentally delayed children with a DSM-IV-TR Pervasive Developmental Disorder (PDD) and 61 developmentally delayed children without a PDD. An exploratory factor analytic investigation using 12 ADI-R repetitive behaviour items from parent report of children with a PDD reported the emergence of two factors. The first factor consisted of higher-level, “insistence on sameness” behaviours, and the second of lower-level, repetitive “sensory-motor” behaviours. This factor structure was also applicable to a more general group of young children with developmental delay, regardless of their diagnosis. Correlational analyses highlighted contrasting relationships between developmental variables and the different repetitive behaviour factors. These relationships were different for children with a PDD and those without a PDD. The findings have potential implications for the early assessment and diagnosis of PDDs in young children.     

Stereotyped behaviours in children with autism spectrum disorders and atypical development as measured by the BPI-01

Objective: As repetitive behaviours in children with autism spectrum disorders (ASDs) can significantly interfere with one's daily functioning, continued research on these behaviours is needed.

Methods: This study examined the occurrence of repetitive motor movements in toddlers (17–37 months) with ASD and atypical development and the relationship between stereotypies and sensory impairment.

Results: Children with ASD (n?=?13) exhibited significantly more repetitive behaviours than the non-ASD group (n?=?12). Greater percentages of endorsement were evident for the ASD group on nearly all items of the Behaviour Problems Inventory-01 (BPI-01) Stereotypy subscale. More repetitive behaviours tended to co-occur with other stereotypies for the ASD group. A moderate correlation was found between stereotypy severity and sensory deficits.

Conclusion: These findings suggest that stereotyped behaviours can be identified at very young ages, negatively affect the behavioural presentation of those with ASDs and should be considered when developing treatment plans.     

Self-injurious behaviour in Cornelia de Lange syndrome: 1. Prevalence and phenomenology

Background   Self-injurious behaviour is frequently identified as part of the behavioural phenotype of Cornelia de Lange syndrome (CdLS). We conducted a case-control study of the prevalence and phenomenology of self-injurious behaviour (SIB) in CdLS.
Methods   A total of 54 participants with CdLS were compared with 46 individuals who were comparable on key variables including age, degree of intellectual disability and wheelchair use, using questionnaire and observational measures.
Results   Clinically significant self-injury was not more prevalent in the CdLS group (55.6%), nor was it different in presentation from that seen in the comparison group. Hyperactivity, stereotyped and compulsive behaviours predicted clinically significant self-injury in all participants. Hand directed, mild self-injury was more prevalent in CdLS.
Conclusions   The results show that clinically significant self-injury may not be part of the behavioural phenotype of CdLS but a specific body target for proto-SIB is more common.     

Behavioural problems and autism in children with hydrocephalus

Objective To investigate the prevalence of behavioural problems and autism in a population-based group of children with hydrocephalus and to see whether learning disabilities, cerebral palsy (CP), epilepsy, myelomeningocele (MMC) or preterm birth increase the risk of these problems. Method In the 107 children with hydrocephalus born in western Sweden in 1989–1993, behaviour was assessed using the Conners’ parent rating scales in 66 and the teacher’s rating scales in 57. Autism was investigated using the Childhood Autism Rating Scale. Results Parents rated 67% of the children and teachers 39% of the children as having behavioural problems (>1.5 SD, or T score >65). Learning disabilities increased the risk significantly and almost all the children with CP and/or epilepsy had behavioural problems. Autism was present in nine children (13%), in 20% of those without MMC and in one of 26 with MMC. Autism was significantly more frequent in children with learning disabilities (27% vs. 7%) and in children with CP and/or epilepsy (33% vs. 6%). Conclusion The majority of children with hydrocephalus have behavioural problems and many have autism. It is therefore important to assess and understand all the aspects of cognition and behaviour in these children in order to minimise disability and enhance participation for the child.     

Early play behaviour in children with 5p- (Cri-du-Chat) syndrome

Background 5p‐ (Cri‐du‐Chat) syndrome (5p‐S) is a well defined chromosomal condition. While the physical symptoms have frequently been documented, the developmental and behavioural aspects of the syndrome have not been explored adequately, especially in young children. Method Mental level, and complexity and style of play were analysed in 10 children who were homogeneously selected with respect to their chronological age (range = 2–7 years). Results A high rate of distractability and alow level of object‐directed behaviours were observed in the play sessions. The findings were compared to two comparison groups (subjects with Down's syndrome or Cornelia‐de‐Lange syndrome) matched for mental age in order to analyse the specificity of these behavioural features for young children with 5p‐S. Conclusions A low level of object‐directed behaviours may be an early precursor of hyperactivity, distractability and stereotypy, which have been reported to be the characteristic features of the behavioural phenotype of older individuals with 5p‐S.