Change in Autism Symptoms and Maladaptive Behaviors in Adolescents and Adults with an Autism Spectrum Disorder

This study examined change prospectively in autism symptoms and maladaptive behaviors during a 4.5 year period in 241 adolescents and adults with an autism spectrum disorder who were 10–52 years old (mean = 22.0) when the study began. Although many individuals’ symptoms remained stable, a greater proportion of the sample experienced declines than increases in their level of autism symptoms and maladaptive behaviors, and there were significant improvements in mean levels of symptoms. Individuals with mental retardation had more autism symptoms and maladaptive behaviors than those without mental retardation, and they improved less over time. Compared to adolescents, older sample members (31 and older) had fewer maladaptive behaviors and experienced more improvement in these behaviors over time.     

Rating problem behaviors in outpatients with mental retardation: use of the Aberrant Behavior Checklist.

Parent and teacher ratings of behavior problems of an outpatient sample of 110 children, adolescents, and young adults with IQs ranging from severe mental retardation to borderline were obtained using a modified version of the Aberrant Behavior Checklist (ABC). Using factor analytic techniques, the five-factor structure of the parent data corresponded extremely well with the five factors originally obtained from staff ratings of mentally retarded inpatients (i.e., Irritability, Withdrawal, Hyperactivity, Stereotypies, and Inappropriate Speech). Factor content was virtually identical between the parent and original ABC data with differences involving only one or two items per scale. The teacher data also revealed a factor structure that corresponded to the same five factors as the parent and original data. Although the teacher and parent factors showed a high degree of similarity, the teacher data suggested that the Stereotypies and Inappropriate Speech factors of the parent and original analyses were not the same constructs for teacher respondents. Age was related to the withdrawal factor for parent data; level of intellectual functioning was the only subject characteristic related to factor scale scores in both parent and teacher data. Test-retest reliabilities were adequate to excellent for all factors for both parent and teacher data. Parent-teacher cross-informant reliabilities were adequate for at least four of the factors. The results of the report indicate that the ABC is a useful, reliable instrument for assessing maladaptive behaviors in young, developmentally disabled outpatients.     

The Aberrant Behavior Checklist and the Behavior Problems Inventory: convergent and divergent validity

This study was designed to compare and cross-validate two rating instruments [the Aberrant Behavior Checklist (ABC) and the Behavior Problems Inventory (BPI)] for assessing maladaptive behavior. The BPI assesses three types of behavior problems: Self-Injurious Behavior (SIB), Stereotyped Behavior and Aggressive/Destructive Behavior. The ABC assesses five domains including these three. We collected data on 226 adults, mostly with severe or profound mental retardation, from a medium-sized developmental center. Individuals with elevated BPI scores generally had higher ABC scores; however, the extent of covariation differed across subscales. Similarly, multiple regression analyses showed that BPI subscales significantly but selectively predicted ABC subscale scores. Measures of differential diagnostic value (positive and negative predictive power, sensitivity, specificity and overall correct diagnostic efficiency) confirmed the anticipated partial overlap between instruments. Both instruments were used to rate participants with and without a Diagnosis of Stereotyped Movement Disorder. BPI, SIB and Stereotypy subscale composite had stronger positive predictive power than the ABC Stereotypy scale, while the ABC had higher negative predictive power and greater overall diagnostic efficiency. Thus, the ABC and the BPI cross-validated one another where expected, and they diverged for subscales thought to have little relationship.     

The Behavior Problems Inventory: An Instrument for the Assessment of Self-Injury, Stereotyped Behavior, and Aggression/Destruction in Individuals with Developmental Disabilities

The Behavior Problems Inventory (BPI-01) is a 52-item respondent-based behavior rating instrument for self-injurious, stereotypic, and aggressive/destructive behavior in mental retardation and other developmental disabilities. Items are rated on a frequency scale and a severity scale. The BPI-01 was administered by interviewing direct care staff of 432 randomly selected residents from a developmental center between the ages of 14 to 91 years. For 73% of those selected, at least one problem was endorsed on the BPI-01. A total of 43% showed self-injury, 54% stereotyped behavior, and 38% aggressive/destructive behavior. Confirmatory factor analysis and item-total correlations supported the three a priori factors. Analyses of variance (ANOVA) showed that of the variables age, sex, and level of mental retardation, only the latter had a significant effect on the BPI-01 total score, the SIB subscale score, and the Stereotyped Behavior subscale score. Aggression/destruction was not significantly related to any of the three variables. Individuals with a diagnosis of pervasive developmental disorder had higher scores on all three subscales than those without, whereas residents with a diagnosis of stereotyped movement disorder had higher Stereotyped Behavior scale scores than those without. The BPI-01 was found to be a reliable (retest reliability, internal consistency, and between-interviewer-agreement) and valid (factor and criterion validity) behavior rating instrument for problem behaviors in mental retardation and developmental disabilities with a variety of potentially useful applications. Strengths and limitations of the instrument are discussed.     

Behavioral and Emotional Symptoms of Children and Adolescents with Prader-Willi Syndrome

To examine the behavioral and emotional difficulties of 73 children and adolescents with Prader-Willi Syndrome (PWS), mental retardation-only, and dual diagnosis (i.e., mental retardation and psychiatrically disordered) on the Devereux Scales of Mental Disorders (DSMD: Naglieri, LeBuffe, & Pfeiffer, Devereux Scales of Mental Disorders (DSMD) San Antonio, TX: PsychCorp 1994). Multivariate analyses and "Italic">d-ratios were computed to assess the statistical and clinically meaningful differences between pairs of samples. The PWS sample exhibited statistically significant higher levels of psychopathology than the mentally-retarded-only sample on the Total, Externalizing, Internalizing, Attention/Delinquency, Conduct, Anxiety, and Acute Problems Scales. When compared to the dually-diagnosed sample, children with PWS Syndrome had comparable levels of psychopathology, but lower levels of depression. Results revealed that PWS represents a highly unique and complex psychological disorder with multiple areas of disturbances.     

The psychometric properties of the Multi-Dimensional Observation Scale for Elderly Subjects (MOSES) in middle aged and older populations of people with mental retardation

OBJECTIVE: To assess the internal consistency, inter-scale correlations and factor structure of the MOSES with older adults with mental retardation. METHOD: A series of outpatients with mental retardation were assessed with the MOSES. 163 middle aged and older adults with mental retardation living in community settings participated. RESULTS: The subscales and total scale of the Multi-Dimension Observation Scale for Elderly Subjects (MOSES) were highly internally consistent. The pattern of correlations between the five scales was very similar to that reported by Dalton et al. (1999). The results of a factor analysis using varimax rotation indicated a three-factor structure corresponding to adaptive behavior, externalizing and internalizing maladaptive behaviors. CONCLUSIONS: The MOSES appears to be was a psychometrically adequate instrument to screen older adults with mental retardation.     

Behavioral and psychiatric disorders in Prader-Willi syndrome: a population study in Japan

Prader-Willi syndrome (PWS) is a genetically determined neurodevelopmental disorder characterized by mental retardation and distinct physical, behavioral, and psychiatric features. Based on parents' questionnaires, we examined the prevalence of behavioral and psychiatric disorders of 165 persons with PWS aged 2-31 years in Japan. The data were analyzed comparing four different age groups with PWS: group 1, 2-5 years (n=34); group 2, 6-11 years (n=57); group 3, 12-17 years (n=45); and group 4, 18-31 years (n=29). Further, we compared the results of our PWS group 4 with those of 42 age-, gender-, and intelligence level-matched intellectual disability (ID) individuals without PWS. Our results showed that repetitive speech and stubbornness were prominent from early childhood and other behavioral problems such as hyperphagia, stealing food, temper tantrums, lying, and emotional lability tended to be more frequent with age among persons with PWS. Moreover, young adults with PWS have significantly higher rates of behavioral and psychiatric disorders than IDs without PWS, such as stubbornness, hyperphagia, temper tantrums, self-injurious behavior (skin picking), hypersomnia, inactivity, and delusion. Degree of obesity was not necessarily related to behavioral and psychiatric features associated with PWS. Our findings revealed that persons with PWS are more vulnerable to behavioral and psychiatric disorders particularly in young adulthood compared to those with ID from other etiologies in Japan.     

Repetitive behavior profiles: Consistency across autism spectrum disorder cohorts and divergence from Prader�CWilli syndrome

Restricted and repetitive behavior (RRB) is a group of heterogeneous maladaptive behaviors. RRB is one of the key diagnostic features of autism spectrum disorders (ASDs) and also commonly observed in Prader–Willi syndrome (PWS). In this study, we assessed RRB using the Repetitive Behavior Scale-Revised (RBS-R) in two ASD samples (University of Illinois at Chicago [UIC] and University of Florida [UF]) and one PWS sample. We compared the RBS-R item endorsements across three ASD cohorts (UIC, UF and an ASD sample from Lam, The Repetitive Behavior Scale-Revised: independent validation and the effect of subject variables, PhD thesis, 2004), and a PWS sample. We also compared the mean RBS-R subscale/sum scores across the UIC, UF and PWS samples; across the combined ASD (UIC + UF), PWS-deletion and PWS-disomy groups; and across the combined ASD sample, PWS subgroup with a Social Communication Questionnaire (SCQ) score ≥15, and PWS subgroup with a SCQ score <15. Despite the highly heterogeneous nature, the three ASD samples (UIC, UF and Lam’s) showed a similar pattern of the RBS-R endorsements, and the mean RBS-R scores were not different between the UIC and UF samples. However, higher RRB was noted in the ASD sample compared with the PWS sample, as well as in the PWS subgroup with a SCQ score ≥15 compared with the PWS subgroup with a SCQ score <15. Study limitations include a small sample size, a wide age range of our participants, and not controlling for potential covariates. A future replication study using a larger sample and further investigation into the genetic bases of overlapping ASD and RRB phenomenology are needed, given the higher RRB in the PWS subgroup with a SCQ score ≥15.     

Individuals with Smith-Magenis syndrome display profound neurodevelopmental behavioral deficiencies and exhibit food-related behaviors equivalent to Prader-Willi syndrome

Smith-Magenis syndrome (SMS) is a neurodevelopmental disorder associated with intellectual disability, sleep disturbances, early onset obesity and vast behavioral deficits. We used the Behavior Problems Inventory-01 to categorize the frequency and severity of behavioral abnormalities in a SMS cohort relative to individuals with intellectual disability of heterogeneous etiology. Self-injurious, stereotyped, and aggressive/destructive behavioral scores indicated that both frequency and severity were significantly higher among individuals with SMS relative to those with intellectual disability. Next, we categorized food behaviors in our SMS cohort across age using the Food Related Problems Questionnaire (FRPQ) and found that problems began to occur in SMS children as early as 5–11 years old, but children 12–18 years old and adults manifested the most severe problems. Furthermore, we evaluated the similarities of SMS adult food-related behaviors to those with intellectual disability and found that SMS adults had more severe behavioral problems. Many neurodevelopmental disorders exhibit syndromic obesity including SMS. Prader-Willi syndrome (PWS) is the most frequent neurodevelopmental disorder with syndromic obesity and has a well-established management and treatment plan. Using the FRPQ we found that SMS adults had similar scores relative to PWS adults. Both syndromes manifest weight gain early in development, and the FRPQ scores highlight specific areas in which behavioral similarities exist, including preoccupation with food, impaired satiety, and negative behavioral responses. SMS food-related behavior treatment paradigms are not as refined as PWS, suggesting that current PWS treatments for prevention of obesity may be beneficial for individuals with SMS.     

Behavior and personality characteristics of children and young adults with Prader-Willi syndrome: a controlled study.

OBJECTIVE: To analyze (1) which behavior and personality characteristics in Prader-Willi syndrome (PWS) are primarily linked to the syndrome and not to mental retardation or being overweight, (2) how early in life such traits appear, and (3) whether current therapies affect behavior. METHOD: Parents of a group of 44 individuals with PWS and of a comparison group were interviewed and completed questionnaires about their children's behavior and personality. RESULTS: Individuals with PWS had more behavior problems than those in the comparison group. Some behaviors were specific to PWS. Younger PWS cases had fewer behavior problems than older PWS cases. Treated individuals had approximately the same degree of behavior problems as those untreated, even though a few symptoms occurred at lower rates. CONCLUSIONS: PWS is associated with behavior correlates that are not related to weight or IQ. In the first few years of life, children with PWS do not demonstrate the characteristic profile of preoccupation with food, ritualism, irritability, temper tantrums, and skin-picking which is typical of older individuals with PWS. Current therapies (including treatment with growth hormone) do not seem to radically affect the behavioral expression of the disorder, even though some problems tended to abate with treatment.     

Factor validity and norms for the Aberrant Behavior Checklist in a community sample of children with mental retardation

The Aberrant Behavior Checklist (ABC) is a 58-item rating scale that was developed primarily to measure the effects of pharmacological intervention in individuals living in residential facilities. This study investigated the use of the ABC in a sample of community children with mental retardation. Teacher ratings on the ABC were collected on 666 students attending special classes. The data were factor analyzed and compared with other studies using the ABC. In addition, subscales were analyzed as a function of age, sex, and classroom placement, and preliminary norms were derived. A four-factor solution of the ABC was obtained. Congruence between the four derived factors and corresponding factors from the original ABC was high (congruence coefficients ranged between .87 and .96). Classroom placement and age had significant effects on subscale scores, whereas sex failed to affect ratings. The current results are sufficiently close to the original factor solution that the original scoring method can be used with community samples, although further studies are needed to look at this in more detail.Work on this paper was supported by a research project grant from the National Institute of Mental Health (Grant MH 44122) to M. G. Aman. The authors thank the departments of special education within public school districts, the county boards of MR/DD, and the numerous teachers in central and northern Ohio who participated in this study.     

Descriptive assessment of sleep patterns among community-living adults with mental retardation

There is little information about the sleep patterns of adults who have mental retardation and are supported in the community. In the present study, direct-care staff recorded sleep behaviors of 59 adults residing in 16 suburban group homes. Based on direct observation and measurement procedures, the adults averaged 7.9 hours of sleep each evening and had low incidence of sleep problems. Sleep duration was not influenced by age, gender, degree of mental retardation, or psychiatric status. Adults taking antidepressant medication (SSRI) had fewer hours of sleep. We discuss implications of these findings and factors contributing to healthy sleep hygiene among community-living adults with mental retardation.     

Sleep disturbances and behavioural problems in adults with Prader–Willi syndrome

Background Individuals with Prader–Willi syndrome (PWS) are at risk of sleep disturbances, such as excessive daytime sleepiness (EDS) and sleep apnoea, and behavioural problems. Sleep disturbances and their relationship with other variables had not been researched extensively in adults with PWS. Method Sleep disturbances and behavioural problems were investigated in adults with genetically confirmed PWS using standardised questionnaires. Results of adults with paternal deletion (n = 45) were compared with those of adults with maternal uniparental disomy (n = 33). Results Eleven adults with PWS (i.e. 15%) had a current sleep problem, mostly night waking problems. Twenty‐six adults with PWS (i.e. 33%) suffered from severe EDS. No differences in prevalence of sleep disturbances between genetic subtypes were found. Seventeen adults with deletion (i.e. 38%) and 17 adults with maternal uniparental disomy (i.e. 52%) had behavioural problems. No significant relationships were found between sleep disturbances and behavioural problems. Conclusions In adults with PWS, EDS is the most common type of sleep disturbance. Men and individuals with relative high body mass index are at increased risk for EDS. More research, aimed at developing a suitable screening instrument for sleep apnoea in adults with PWS, is necessary. Clinical implications of the findings are discussed.     

Staff perceptions of reinforcer responsiveness and aberrant behaviors in people with mental retardation

Perceptions by staff of the classes of reinforcers and aberrant behaviors of a sample of 470 people with predominantly severe or profound mental retardation were explored. Principal components analysis of a 45-item survey suggested eight classes of reinforcers: consumable, verbal-speaker, visual-motor, social, physical-contact, passive-observer, play, and academic reinforcers. Stepwise multiple regression was used to predict five classes of maladaptive behaviors as measured by the Aberrant Behavior Checklist (irritability, lethargy, stereotypy, hyperactivity, and inappropriate speech) from the eight classes of reinforcers. Each class of psychopathology was related to a unique set of predictors. All classes of psychopathology could be predicted by staff perceptions of underresponsiveness to social reinforcers and overresponsiveness to consumable reinforcers. The findings of organized structures of reinforcers and their covariation with pathological behaviors have implications for research and intervention as well as theoretical value in defining aberrant behaviors in people with mental retardation.     

A cross-sectional test of the similar-trajectory hypothesis among adults with mental retardation

The similar-sequence and the similar-structure hypotheses are the two mainstays of the developmental approach to mental retardation. In the present study, a third way, the similar-trajectory hypothesis, is described and illustrated using the WAIS-R results of adults with and without mental retardation aged from 20 to 54 years. The whole sample (N=633) comprised 306 participants with mental retardation and 327 without mental retardation. Hierarchical regression analyses comparing the two groups showed similar evolutions of scores with increasing age for verbal and performance scales. These results seem to validate the similar-trajectory hypothesis, at least for the present samples and for the aspects of cognitive development considered here. Some weaknesses and implications of the study are considered in the discussion.     

Assessing independent and interactive effects of behavioral and pharmacologic interventions for a client with dual diagnoses

Behavior analytic methods were applied to the assessment and treatment of the problem behaviors of an 8-year-old male classified as having moderate mental retardation and atypical psychosis. Functional assessment procedures demonstrated that verbal behaviors diagnosed as hallucinatory were affected by environmental contingencies. Next, a multi-element/multiple baseline design was used to evaluate the independent and interactive effects of contingency management and pharmacologic interventions on three dependent variables: (1) psychotic speech; (2) aggressive-disruptive behavior; and (3) appropriate speech. Results indicated that contingency management without haloperidol was the most effective treatment for all inappropriate and appropriate behaviors. These results are important in that behavior analytic methods were shown to have utility for: (1) assessing the functional relationship between environmental contingencies and behaviors related to differential diagnosis; and (2) evaluating the independent and interactive effects of behavioral and pharmacologic treatments.     

Normal ageing in adults with Down's syndrome: a longitudinal study

The ubiquitous presence of the neuropathoiogy of Alzheimer disease (AD) in individuals with Down's syndrome (DS) over 40 years of age suggests that this group of people will exhibit a high prevalence of dementia of the Alzheimer type (DAT) as they age. The present study indicates that there is a clear discrepancy between the presumed presence of AD neuropathoiogy and the clinical expression of DAT among older people with DS. In the first 6 years of a longitudinal study, the present authors compared 91 adults (31–63 years of age) with DS and mild or moderate mental retardation to 64 adults (3 l –76 years of age) with other forms of mental retardation (MR) on yearly measures of mental status, short-and long-term memory, speeded psychomotor function, and visuospatial organization. The results indicated that, over repeated testing on the verbal long–term memory test, younger participants with DS showed small increases in their scores, while older participants with DS showed very slight decreases. Overall performance scores on this test and a speeded psychomotor task were poorer for both diagnostic groups in individuals aged SO years and older. The magnitude and type of these selective changes in performance were consistent with performance profiles observed in older healthy adults without mental retardation on tests measuring similar cognitive functions. Only four out of the 91 people with DS in the present sample showed changes in funaioning that have led to a diagnosis of possible DAT. and in these individuals, alternative causes of performance declines were concurrently present (e.g, thyroid dysfunction). These findings indicate that some age–associated changes in funaioning are related to ‘normal’ but probably precocious ageing among adults with DS. Furthermore, these findings suggest that adults with DS and mild or moderate mental retardation may be at lower risk for dementia during their fourth and fifth decades of life than previous studies have suggested.     

A neuropsychological assessment of frontal cognitive functions in Prader–Willi syndrome

Background Prader–Willi syndrome (PWS) is associated with a characteristic behavioural phenotype whose main features are, alongside compulsive hyperphagia, deficits in social behaviour: social withdrawal, temper tantrums, perseverative speech and behaviour, mental rigidity, stereotyped behaviour, impulsiveness, etc. Similar symptoms may also be found in autistic spectrum disorders and lesional pathologies of the frontal lobe. In both cases, such symptoms have been related to dysfunctions in frontal cognitive processes such as attention, working memory and executive functions. This study uses standardized neuropsychological instruments to analyse the degree to which these processes are affected in PWS. Methods The sample comprised 16 individuals with a genetically confirmed PWS diagnosis. Subjects’ IQ (Wechsler Adult Intelligence Scale), academic level, laterality and body mass index (BMI) were calculated. Attention, memory and executive functions were analysed using standard, widely employed neuropsychological tests. We compared the results of the sample group with the general population. Correlation analyses were carried out with IQ, academic level and BMI. Results In all the neuropsychological measures focusing on attention, executive functions and visuoperceptual organization, the study sample scored significantly lower than the normative reference population. The scores of the tests used for measuring immediate memory were also significantly lower when trials required sequential processing, although not when they required simultaneous processing. In the memorization of a list of words, subjects showed an initial deficit which disappeared with repetition, enabling them to obtain scores similar to the reference population. No significant correlations were found with BMI, and a higher IQ or academic level did not improve scores in the majority of tests. Conclusions The study shows a deficit in elementary frontal cognitive processes in PWS patients. This deficit may be involved in the social behaviour disorders that characterize such patients, as described in other development or frontal syndrome pathologies. However, we cannot affirm that the deficits found are specific to PWS; they could also occur in other causes of intellectual disability. Although in the study sample IQ did not correlate with frontal deficits, further research is needed to establish whether the neuropsychological alterations described form part of a cognitive phenotype for PWS. We believe that our understanding of the social behaviours typical of PWS may be improved by taking into consideration the cognitive functioning models of the prefrontal lobe, particularly those applied to pervasive developmental disorders.     

An investigation of executive function abilities in adults with Prader–Willi syndrome

Background Prader–Willi syndrome (PWS) is a genetic disorder caused by the absence of expression of maternally imprinted genes on the long arm of chromosome 15 (15q 11-13). There are two main genetic sub-types: (1) deletion, caused by the absence of paternally derived genetic material; and (2) uniparental disomy (UPD), where two copies of maternally derived chromosome 15 are present. In addition to generally mild/borderline intellectual disability (ID) and the almost universal feature of hyperphagia, PWS is associated with high rates of behaviour problems including temper tantrums, compulsive behaviour, perseverative speech, skin picking and rigid thinking. The present study seeks to explore whether these behaviours are associated with relative deficits in executive function (EF), which comprises the set of non-automatic processes utilized by an individual when faced with a novel situation. Methods Eighteen adult participants with a clinical diagnosis of PWS (12 with deletion sub-type, 6 with UPD) were recruited from a UK Health Service PWS clinic, and compared with 15 participants of similar age and verbal ability on a series of EF tasks and also Digit Span Forwards. An informant completed two ratings of behaviour, the Aberrant Behavior Checklist (ABC) and the Dysexecutive Questionnaire (DEX). Results The PWS group had significantly higher scores on the ABC but not on the DEX. There were no significant differences between the whole PWS group and the comparison group on any of the EF tasks. The deletion sub-type group was significantly poorer at a non-executive task, Digit Span Forwards. There was an unexpected trend for the deletion sub-type group to show more efficient performance on a visuospatial planning task, the Tower of London (TOL), but this trend did not reach significance. Conclusions The lack of relative deficits in EF task performance does not support the hypothesis that EF differences could account for the high levels of behaviour problems found in PWS. Applying the Baddeley and Hitch model of working memory it is suggested that the PWS group have a relatively intact central executive and visuospatial sketchpad but a relative impairment in the phonological loop, perhaps relating to the capacity of the phonological store. This latter finding seems to be particularly salient for those with a deletion. As differences in EF ability were not found, it is suggested that a region of the brain involved in the modulation of emotion but not particularly with EF, the orbitofrontal cortex (OFC), may be implicated in the behaviour problems reported in PWS.