Measures Of Adaptive Behavior Research Articles

Global development and adaptive behaviour in children with early-onset epilepsy: a population-based case-control study.

There are limited population-based data on global development and adaptive behaviour in children with early-onset epilepsy. The aims of this study were: (1) to identify the prevalence of deficits in global development and adaptive behaviour experienced by children with early-onset epilepsy; (2) to identify factors associated with such deficits; and (3) to compare the relationship between measures of neurodevelopment in the group with epilepsy to a group without epilepsy who had other neurological or neurodevelopmental difficulties. The Sussex Early Epilepsy and Neurobehaviour study is a prospective, community-based study involving children (1-7y) with epilepsy. We undertook comprehensive psychological assessment with participants, including measures of global development and adaptive behaviour. We compared the children with epilepsy with a sex, age, and developmentally-matched group of children without epilepsy who had neurodevelopmental or neurological difficulties using correlation matrices. Forty-eight children (91% of the eligible population) with epilepsy underwent assessment. Seventy-one per cent of children displayed delayed global development (<2SD) and 56% showed significant deficits (<2SD) in adaptive behaviour. Our analysis revealed that non-white ethnicity and use of polytherapy were independently associated with decreased scores on measures of global development and adaptive behaviour. The correlations between measures of developmental functioning were higher in children with epilepsy than in those without. Children with early-onset epilepsy frequently have difficulties with global development and adaptive behaviour. The higher correlations between neurodevelopmental measures in children with epilepsy suggest that the profile in children with epilepsy is different. This may have significant implications for both neuropathology and interventions. Children with early-onset epilepsy are at significant risk of intellectual disability. Developmental impairment is associated with use of polytherapy but not with any seizure parameters. Developmental profiles in young children with epilepsy differ from other conditions.

Differential outcome subgroups in children with autism spectrum disorder attending early intervention.

The finding of positive outcomes at the group level for children with autism spectrum disorder (ASD) who complete comprehensive early intervention programmes often masks considerable individual variability. We therefore aimed to identify subgroups of children based on their response to intervention and to compare outcome variables between groups at two points in time. We used model-based cluster analysis to explore response to intervention using a longitudinal design for 210 children with ASD who had completed an early intervention programme. Children were assessed on entry at time 1 and again at time 2, which was after 12months or when they exited the programme (whichever came first) using measures of ASD symptoms (Social Communication Questionnaire), cognition (Mullen Scales of Early Learning) and adaptive behaviour (Vineland Adaptive Behaviour Scales-II). A two-cluster solution was identified, including a high change group who improved consistently more than the low change group across measures, and showed significantly fewer autism symptoms, higher non-verbal and verbal cognition and adaptive behaviour composite scores at time 1. The findings indicated that children's response to early intervention is not uniform but instead included subgroups characterised by patterns of high and low change. Further research is needed to identify clinically relevant mediators of differential response group membership.

Observational Prospective Natural History of Patients with Sanfilippo Syndrome Type B

To evaluate the natural course of disease progression in patients with Sanfilippo syndrome type B (mucopolysaccharidosis type IIIB), identify potential end points for future therapy trials, and characterize biomarkers related to the disease. A prospective, multicenter study was conducted. Baseline, 6-month, and 12-month assessments included neurodevelopmental status (Bayley Scales of Infant Development, Third edition), adaptive status (Vineland Adaptive Behavior Scales, Second Edition), volumetric brain magnetic resonance imaging, cerebrospinal fluid heparan sulfate, and urine glycosaminoglycan (GAG) measurements. Nineteen patients aged 1.6-31.7 years were enrolled. Over 12 months, cognition, adaptive behavior, and cortical gray matter volume (GMV) declined in most patients. For patients diagnosed at <6 years, although there was no overall mean change over 12 months, there were 10%-48%, 3%-66%, and 1%-14% decreases in cognitive development quotient score, Vineland Adaptive Behavior Scales, Second Edition development quotient score, and cortical GMV in 8/12, 9/11, and 10/11 patients, respectively. Mean urine GAG and cerebrospinal fluid heparan sulfate levels were stable, but patients diagnosed at <6 years (n = 14) had higher levels than those ≥6 years at diagnosis (n = 4), which was likely associated with age as they also were generally younger. Cognition, adaptive behavior, and cortical GMV measures sensitively tracked deterioration in patients with mucopolysaccharidosis type IIIB aged ≤8.6 years. Biomarkers may have prognostic value, but their sensitivity to disease progression requires further investigation. These findings should help evaluate enzyme replacement and gene therapy agents for this rare, devastating, neurodegenerative disease. ClinicalTrials.gov: NCT01509768.

Validity, interrater reliability, and measures of adaptive behavior: Concerns regarding the probative versus prejudicial value.

Validity, interrater reliability, and measures of adaptive behavior: Concerns regarding the probative versus prejudicial value.

The Application of Adaptive Behaviour Models: A Systematic Review.

Adaptive behaviour has been viewed broadly as an individual’s ability to meet the standards of social responsibilities and independence; however, this definition has been a source of debate amongst researchers and clinicians. Based on the rich history and the importance of the construct of adaptive behaviour, the current study aimed to provide a comprehensive overview of the application of adaptive behaviour models to assessment tools, through a systematic review. A plethora of assessment measures for adaptive behaviour have been developed in order to adequately assess the construct; however, it appears that the only definition on which authors seem to agree is that adaptive behaviour is what adaptive behaviour scales measure. The importance of the construct for diagnosis, intervention and planning has been highlighted throughout the literature. It is recommended that researchers and clinicians critically review what measures of adaptive behaviour they are utilising and it is suggested that the definition and theory is revisited.

Prospective investigation of FOXP1 syndrome

BackgroundHaploinsufficiency of the forkhead-box protein P1 (FOXP1) gene leads to a neurodevelopmental disorder termed FOXP1 syndrome. Previous studies in individuals carrying FOXP1 mutations and deletions have described the presence of autism spectrum disorder (ASD) traits, intellectual disability, language impairment, and psychiatric features. The goal of the present study was to comprehensively characterize the genetic and clinical spectrum of FOXP1 syndrome. This is the first study to prospectively examine the genotype-phenotype relationship in multiple individuals with FOXP1 syndrome, using a battery of standardized clinical assessments.MethodsGenetic and clinical data was obtained and analyzed from nine children and adolescents between the ages of 5–17 with mutations in FOXP1. Phenotypic characterization included gold standard ASD testing and norm-referenced measures of cognition, adaptive behavior, language, motor, and visual-motor integration skills. In addition, psychiatric, medical, neurological, and dysmorphology examinations were completed by a multidisciplinary team of clinicians. A comprehensive review of reported cases was also performed. All missense and in-frame mutations were mapped onto the three-dimensional structure of DNA-bound FOXP1.ResultsWe have identified nine de novo mutations, including three frameshift, one nonsense, one mutation in an essential splice site resulting in frameshift and insertion of a premature stop codon, three missense, and one in-frame deletion. Reviewing prior literature, we found seven instances of recurrent mutations and another 34 private mutations. The majority of pathogenic missense and in-frame mutations, including all four missense mutations in our cohort, lie in the DNA-binding domain. Through structural analyses, we show that the mutations perturb amino acids necessary for binding to the DNA or interfere with the domain swapping that mediates FOXP1 dimerization. Individuals with FOXP1 syndrome presented with delays in early motor and language milestones, language impairment (expressive language > receptive language), ASD symptoms, visual-motor integration deficits, and complex psychiatric presentations characterized by anxiety, obsessive-compulsive traits, attention deficits, and externalizing symptoms. Medical features included non-specific structural brain abnormalities and dysmorphic features, endocrine and gastrointestinal problems, sleep disturbances, and sinopulmonary infections.ConclusionsThis study identifies novel FOXP1 mutations associated with FOXP1 syndrome, identifies recurrent mutations, and demonstrates significant clustering of missense mutations in the DNA-binding domain. Clinical findings confirm the role FOXP1 plays in development across multiple domains of functioning. The genetic findings can be incorporated into clinical genetics practice to improve accurate genetic diagnosis of FOXP1 syndrome and the clinical findings can inform monitoring and treatment of individuals with FOXP1 syndrome.

Gender comparisons in children with ASD entering early intervention

BackgroundMales are diagnosed with Autism Spectrum Disorder (ASD) approximately four times as often as females. This has led to interest in recent years of potential under-diagnosis of females, as well as negative consequences for females with ASD due to under-identification. A number of potential explanations for gender bias in diagnosis are discussed including that females and males may present differently despite showing the same core symptoms. Previous research has shown inconsistent findings in comparisons between genders in young children with ASD for whom early intervention is vital. Thus, the aim of the present study was to investigate the social, communication, and cognitive functioning, as well as level of ASD symptoms, in a cohort of children who presented for early intervention to inform understanding of gender differences in this population, as well as to inform understanding of the mechanisms by which gender bias may occur. MethodParticipants included 254 children (42 females) aged 29–74 months who completed measures of cognition, communication skills, adaptive behaviour, and ASD symptoms on entry to early intervention. ResultsConsistent with hypotheses, no significant gender differences were found both overall, and when split by functioning level. However, a similar ratio of males and females was found in both high- and low-functioning groups contrary to predictions. ConclusionsThese results are consistent with some of the previous research that suggests gender differences may not be apparent in clinical samples at this young age. We highlight a need for further research that may use universal screening or longitudinal methods to understand the trajectory of development for females with ASD specifically. Such research could better inform timely and tailored intervention from the preschool years onwards.

The relationship between sound-shape matching and cognitive ability in adults with Down syndrome.

Down syndrome (DS), the most common genetic cause of intellectual disability, is characterised by a pattern of cognitive deficits hypothesised as relating to later developing neural systems. Multisensory integration (MSI) has been shown to benefit cognitive performance on numerous tasks in the typically developing population and is implicated in the early development of various cognitive processes. Given these developmental links of both MSI and DS it is important to determine the relationship between MSI and DS. This study aimed to characterise sound-shape matching performance in young adults with DS as an indicator of MSI (correct response rate around 90% in typically developing individuals). We further investigated the relationship between task performance and estimated cognitive ability (verbal and non-verbal) in addition to everyday adaptive behavior skills. Those answering correctly (72.5%) scored significantly higher across cognitive and adaptive behavior measures compared to those answering incorrectly. Furthermore, 57.1% of individuals with estimated cognitive ability scores below the median value answered correctly compared to 89.5% of individuals scoring above the median, with similar values found for adaptive behavior skills (57.9% vs 94.4%). This preliminary finding suggests sound-shape matching deficits are relatively common in DS but may be restricted to individuals of lower ability as opposed to being a general characteristic of DS. Further studies investigating aspects of MSI across a range of modalities are necessary to fully characterise the nature of MSI in DS and to explore underlying neural correlates and mechanisms.

Author response: CSF concentrations of 5-methyltetrahydrofolate in a cohort of young children with autism.

On behalf of my coauthors, I thank Dr. Barrett for the comment on our article.1 Dr. Barrett correctly recognizes that one of the central aspects of our study was to investigate whether a relationship of CSF 5-MTHF levels to symptoms in young children with autism exists. As addressed in the article, a major difficulty in diagnosing cerebral folate deficiency using CSF 5-MTHF levels was the lack of reference intervals from a normal population that would allow defining clinical decision limits.1 To gain insight into the relationship between CSF 5-MTHF levels and clinical decision limits for young children with autism, we assessed CSF 5-MTHF over time and correlated these levels with detailed clinical assessments. The CSF 5-MTHF levels varied significantly and were not associated with any measures of adaptive behavior, cognitive ability, or autism spectrum disorder symptom severity. As discussed in the article, pediatric CSF normative data that consider natural CSF 5-MTHF variability over time are essential to defining reference intervals as well as critical decision limits.1 In our cohort of young children with autism, a critical decision limit for CSF 5-MTHF levels was not identified. Further research in patients with autism is needed before CSF 5-MTHF levels can be used to justify lumbar puncture to diagnose cerebral folate deficiency or treatment of patients with compounds such as folinic acid or 5-MTHF.

Developmental profiles of infants with an FMR1 premutation.

BackgroundEmerging evidence suggests that a subset of FMR1 premutation carriers is at an increased risk for cognitive, emotional, and medical conditions. However, because the premutation is rarely diagnosed at birth, the early developmental trajectories of children with a premutation are not known.MethodsThis exploratory study examined the cognitive, communication, and social-behavioral profiles of 26 infants with a premutation who were identified through participation in a newborn screening for fragile X syndrome pilot study. In this study, families whose newborn screened positive for an FMR1 premutation were invited to participate in a longitudinal study of early development. Twenty-six infants with the premutation and 21 matched, screen-negative comparison babies were assessed using validated standardized measures at 6-month intervals starting as young as 3 months of age. The babies were assessed up to seven times over a 4-year period.ResultsThe premutation group was not statistically different from the comparison group on measures of cognitive development, adaptive behavior, temperament, or overall communication. However, the babies with the premutation had a significantly different developmental trajectory on measures of nonverbal communication and hyperresponsivity to sensory experiences. They also were significantly more hyporesponsive at all ages than the comparison group. Cytosine-guanine-guanine repeat length was linearly associated with overall cognitive development.ConclusionsThese results suggest that infants with a premutation may present with subtle developmental differences as young as 12 months of age that may be early markers of later anxiety, social deficits, or other challenges thought to be experienced by a subset of carriers.

Impact of maladaptive behavior on school function in Down syndrome

Background/aimsIndividuals with Down syndrome (DS) are at an increased risk for certain maladaptive behaviors. This study characterized maladaptive behavior in school-aged children with DS and examined the extent to which maladaptive behaviors are associated with school function. Methods and proceduresParticipants were 24 students with DS [mean nonverbal mental age (NVMA)=43.83months; mean chronological age (CA)=77.58months] who completed the Leiter Scales of Performance- Revised (Leiter-R; Roid & Miller, 1997). Their teachers completed the Behavior Assessment Scales for Children Version 2 (Reynolds & Kamphaus, 2004), and the School Function Assessment (Coster et al., 1998), measures of maladaptive and adaptive behavior as observed in the school setting. ResultsFindings reveal a maladaptive behavior profile of elevated areas including Aggression, Attention Problems, and Somatization. When examining the association between maladaptive behavior and school function, multivariate regression results indicated a significant association between Aggression and Compliance, and Attention Problems and Task Completion. Conclusions and implicationsResults underscore the importance of developing training for educators regarding the potential impact of maladaptive behavior on school function for students with Down syndrome.

The NIH Toolbox Cognitive Battery for intellectual disabilities: three preliminary studies and future directions.

BackgroundRecent advances in understanding molecular and synaptic mechanisms of intellectual disabilities (ID) in fragile X syndrome (FXS) and Down syndrome (DS) through animal models have led to targeted controlled trials with pharmacological agents designed to normalize these underlying mechanisms and improve clinical outcomes. However, several human clinical trials have failed to demonstrate efficacy of these targeted treatments to improve surrogate behavioral endpoints. Because the ultimate index of disease modification in these disorders is amelioration of ID, the validation of cognitive measures for tracking treatment response is essential. Here, we present preliminary research to validate the National Institutes of Health Toolbox Cognitive Battery (NIH-TCB) for ID.MethodsWe completed three pilot studies of patients with FXS (total n = 63; mean age 19.3 ± 8.3 years, mean mental age 5.3 ± 1.6 years), DS (n = 47; mean age 16.1 ± 6.2, mean mental age 5.4 ± 2.0), and idiopathic ID (IID; n = 16; mean age 16.1 ± 5.0, mean mental age 6.6 ± 2.3) measuring processing speed, executive function, episodic memory, word/letter reading, receptive vocabulary, and working memory using the web-based NIH-TB-CB, addressing feasibility, test-retest reliability, construct validity, ecological validity, and syndrome differences and profiles.ResultsFeasibility was good to excellent (≥80 % of participants with valid scores) for above mental age 4 years for all tests except list sorting (working memory). Test-retest stability was good to excellent, and convergent validity was similar to or better than results obtained from typically developing children in the normal sample for executive function and language measures. Examination of ecological validity revealed moderate to very strong correlations between the NIH-TCB composite and adaptive behavior and full-scale IQ measures. Syndrome/group comparisons demonstrated significant deficits for the FXS and DS groups relative to IID on attention and inhibitory control, a significant reading weakness for FXS, and a receptive vocabulary weakness for DS.ConclusionsThe NIH-TCB has potential for assessing important dimensions of cognition in persons with ID, and several tests may be useful for tracking response to intervention. However, more extensive psychometric studies, evaluation of the NIH-TCB’s sensitivity to change, both developmentally and in the context of treatment, and perhaps establishing links to brain function in these populations, are required to determine the true utility of the battery as a set of outcome measures.

PBDE exposures during pregnancy and risk of Autism Spectrum Disorder at 3 years: Results from the prospective MARBLES study.

Background: Exposure to polybrominated diphenyl ether (PBDE) flame retardants remains widespread in the general population. Rodent experiments show that PBDEs interact with pathways involved in normal brain development. Objectives: To study associations between PBDE exposures during pregnancy and the risk of having a child with Autism Spectrum Disorder (ASD) or other developmental concerns (ODC). Method: We analyzed a subsample of the ongoing MARBLES (Markers of Autism Risk in Babies – Learning Early Signs) study that enrolls mothers of a child diagnosed with ASD who are either pregnant or planning a pregnancy. At 36 months the child was assessed with measures of cognitive function and adaptive behaviors, and two gold-standard diagnostic instruments for ASD. From this evaluation, the child was categorized as: ASD (n = 26), ODC (n = 29) or typically developed (TD, n = 77) based on a consensus clinical best estimate from two independent clinicians reviewing all psychometric results. PBDE (47, 99, 100, 153) and lipid concentrations were measured in repeated plasma samples collected during pregnancy. Adjusted ORs are reported for a 2 fold increase in the mean of the PBDE concentrations (ng/g lipids). Results: PBDE 47, 99, 100 and 153 were detected, respectively in 99%, 74%, 99% and 82% of maternal pregnancy plasma samples. In logistic regression models adjusted for confounders, we observed a modest increased risk of ASD (OR = 1.35, 90%CI: 1.00; 1.82) in association with PBDE 153 concentration. This association was stronger in analyses limited to boys (OR = 1.52, 90%CI: 1.07; 2.18). No other PBDE was associated with risk of ASD or ODC. Conclusion: Our results indicate that PBDE 153 was associated with increased risk of ASD. More studies with larger sample size, prospective design and exposure assessments during both the pre- and early post-natal periods are needed to confirm our results and clarify the role of timing of exposures.

Neurobehavioral Deficits Consistent Across Age and Sex in Youth with Prenatal Alcohol Exposure.

Neurobehavioral consequences of heavy prenatal alcohol exposure are well documented; however, the role of age or sex in these effects has not been studied. The current study examined the effects of prenatal alcohol exposure, sex, and age on neurobehavioral functioning in children. Subjects were 407 youth with prenatal alcohol exposure (n=192) and controls (n=215). Two age groups (child [5 to 7years] or adolescent [10 to 16years]) and both sexes were included. All subjects completed standardized neuropsychological testing, and caregivers completed parent-report measures of psychopathology and adaptive behavior. Neuropsychological functioning, psychopathology, and adaptive behavior were analyzed with separate 2 (exposure history)×2 (sex)×2 (age) multivariate analyses of variance (MANOVAs). Significant effects were followed by univariate analyses. No 3-way or 2-way interactions were significant. The main effect of group was significant in all 3 MANOVAs, with the control group performing better than the alcohol-exposed group on all measures. The main effect of age was significant for neuropsychological performance and adaptive functioning across exposure groups with younger children performing better than older children on 3 measures (language, communication, socialization). Older children performed better than younger children on a different language measure. The main effect of sex was significant for neuropsychological performance and psychopathology; across exposure groups, males had stronger language and visual spatial scores and fewer somatic complaints than females. Prenatal alcohol exposure resulted in impaired neuropsychological and behavioral functioning. Although adolescents with prenatal alcohol exposure may perform more poorly than younger exposed children, the same was true for nonexposed children. Thus, these cross-sectional data indicate that the developmental trajectory for neuropsychological and behavioral performance is not altered by prenatal alcohol exposure, but rather, deficits are consistent across the 2 age groups tested. Similarly, observed sex differences on specific measures were consistent across the groups and do not support sexually dimorphic effects in these domains.

Commentary: Visual Fixation in Human Newborns Correlates with Extensive White Matter Networks and Predicts Long-Term Neurocognitive Development.

Commentary: Visual Fixation in Human Newborns Correlates with Extensive White Matter Networks and Predicts Long-Term Neurocognitive Development.

Neuropsychological presentation and adaptive skills in high-functioning adolescents with visual impairment: A preliminary investigation

ABSTRACTStudies in infants and young children with congenital visual impairment (VI) have indicated early developmental vulnerabilities, conversely research with older children and adults have highlighted areas of cognitive strength. A minimal amount is known, however, about the possible combination of strengths and weaknesses in adolescence, and this present study therefore aims to explore the neuropsychological presentation and adaptive behavior profile in high-functioning adolescents with congenital VI. Participants completed a battery of commonly used neuropsychological measures assessing memory, executive function, and attention. The measures utilized focused on auditory neuropsychological function, because only subtests that could be completed with auditory administration were suitable for this sample. Parents completed standardized measures of adaptive behavior, executive function, and social communication. Compared to aged-based norms for normal sight, adolescents with VI demonstrated strengths in aspects of working memory and verbal memory. Furthermore, performance across the neuropsychological battery was within or above the average range for the majority of the sample. In contrast, parent-report measures indicated areas of weakness in adaptive functioning, social communication, and behavioral executive functioning. Overall, this study provides preliminary evidence that relative to fully sighted peers, high-functioning adolescents with VI present with an uneven profile of cognitive and adaptive skills, which has important implications for assessment and intervention.

Abstract 14337: Preschool Neurodevelopmental Outcomes in Children With Congenital Heart Disease After Surgical Palliation

Introduction: Children with congenital heart disease (CHD) are at risk for neurodevelopmental (ND) delays. The American Heart Association (AHA) recommends serial ND evaluation for children with CHD. This study reports preschool ND testing results of children with CHD. Methods: Children with CHD meeting the AHA high-risk criteria for ND delay were referred for evaluation at 4-5 years of age. Testing included standardized neuropsychological measures. Parents completed measures of adaptive behavior, emotional functioning, social skills, attention, language, family functioning and child quality of life (QOL). Patient scores and parent ratings were compared to test norms and were classified as: average (within one SD of test mean); at risk (1-2 SDs from the test mean); and impaired (&gt;2SD from test mean). T-tests were used to compare scores based on cardiac anatomy (1V vs. 2V). Results: Data on 102 consecutively referred patients were analyzed. Median age at first cardiac operation was 13 days. Mean age at testing was 4.5 years (range 4 to 5.9). Patients had the following characteristics: 1V anatomy 33%; male 63%; prenatal diagnosis 54%; premature 16%; non-white/Hispanic race 16%; and comorbidities: medical 26%, genetic 17%. ND test scores were lower than norms for IQ, pre-spelling, visual-motor, fine motor, adaptive behavior, and social skills, and higher than norms for attention problems; however, means were within one SD of norms for all measures. ND scores did not differ based on cardiac anatomy (1V vs. 2V); however, parents of children with 1V reported lower child QOL scores and worse family functioning than parents of children with 2V (p&lt;0.05). The % of children within the normal, at risk, and impaired range on ND measures is presented in the figure. Conclusion: ND outcome for preschool aged children identified more delays than expected in some areas. Most deficits were mild and may not be detected without formal testing. ND outcome was not related to cardiac anatomy.

Does a Measure of Support Needs Predict Funding Need Better Than a Measure of Adaptive and Maladaptive Behavior?

Internationally, various approaches are used for the allocation of individualized funding. When using a databased approach, a key question is the predictive validity of adaptive behavior versus support needs assessment. This article reports on a subset of data from a larger project that allowed for a comparison of support needs and adaptive behavior assessments when predicting person-centered funding allocation. The first phase of the project involved a trial of the Inventory for Client and Agency Planning (ICAP) adaptive behavior and Instrument for the Classification and Assessment of Support Needs (I-CAN)-Brief Research version support needs assessments. Participants were in receipt of an individual support package allocated using a person-centered planning process, and were stable in their support arrangements. Regression analysis showed that the most useful items in predicting funding allocation came from the I-CAN-Brief Research. No additional variance could be explained by adding the ICAP, or using the ICAP alone. A further unique approach of including only items from the I-CAN-Brief Research marked as funded supports showed high predictive validity. It appears support need is more effective at determining resource need than adaptive behavior.

Tetrasomy 18p: report of cognitive and behavioral characteristics.

Our purpose was to describe intellectual and behavioral characteristics of persons with tetrasomy 18p. This is a more detailed investigation into the cognitive and behavioral characteristics of our previously reported tetrasomy 18p cohort of 43 plus six additional participants. We evaluated the intellectual functioning using standard measures of cognitive ability, measures of executive functioning, adaptive and maladaptive behaviors. Intellectual abilities ranged from mild impairment/borderline normal to severe/profound impairment calling into question the assumption that severe cognitive limitation is always a feature of tetrasomy 18p. For persons with tetrasomy 18p with mild cognitive deficits, the main barriers to successful functioning stems from limited social and metacognitive skill development and behavior regulation problems rather than being solely determined by cognitive deficits alone.

Adaptive Behavior Functioning in Children with Autism

To investigate the relationship between intellectual functioning, symptom severity, and adaptive behavior functioning of children with autism spectrum disorders (ASD). Retrospective case records (1999 to 2013) of 523 children [Mean age 4.79y (SD 2.37)] maintained by the Pediatric Psychology Unit at the Department of Pediatrics of a tertiary care teaching hospital in North India were examined. The adaptive behavior functioning was measured by the Indian adaptation of the Vineland Social Maturity Scale. Symptom severity was assessed using the Childhood Autism Rating Scale (CARS). The mean Social Quotient (SQ) of the sample was 62.40 (SD = 20.41). Nearly two-third (63.3%) of the ASD had SQs less than 70 and only 15% of the ASD children had SQs above 85. Adaptive behavior scores in the lower functioning ASD children were significantly higher than their Intelligence Quotient (IQ) scores while for the high functioning ASD group, the SQs were significantly lower than their IQs. Multiple regression analysis revealed that IQ, age of the child, CARS score, and education of the mother accounted for 62.5% of the variance in the SQ of children with ASD (F = 198.01, P 0.000). Adaptive behavior measures must constitute a crucial component of not only diagnostic assessment of ASD children but also as an important goal of treatment.