Brief Report: Imitation of Meaningless Gestures in Individuals with Asperger Syndrome and High-functioning Autism

Nineteen people with Asperger syndrome (AS)/High-Functioning Autism (HFA) (ages 7–15) were tested on imitation of two types of meaningless gesture: hand postures and finger positions. The individuals with AS/HFA achieved lower scores in the imitation of both hand and finger positions relative to a matched neurotypical group. The between-group difference was primarily accounted for by performance on a test of visual motor integration, together with a hand imitation deficit which was specifically due to errors in body part orientation. Our findings implicate both visuomotor processes (Damasio and Maurer, 1978) and self-other mapping (Rogers and Pennington, 1991) in ASD imitation deficits. Following Goldenberg (1999), we propose that difficulties with body part orientation may underlie problems in meaningless gesture imitation.     

Brief Report: Social and Communication Abilities and Disabilities in Higher Functioning Individuals with Autism and Asperger Syndrome

Individuals with higher functioning autism (HFA) fail to translate their cognitive potential into real-life adaptation, and the severity of their symptoms is considerable despite their intellectual ability. This paper reports on a subsample from a larger study (A. Klin et al., in press) analyzed here by autism spectrum subtypes. It focuses on the nature of ability and disability in HFA and Asperger syndrome (AS) in relation to age and IQ. Participants included 32 individuals with autism and 35 with AS. Individuals with AS had significantly higher Verbal IQ scores and less symptomatology than individuals with autism, but their Vineland scores were equally impaired, highlighting the adaptive deficits in ASD regardless of classification. No relationship was found between adaptive functioning and symptom severity.     

Brief Report: Acrocallosal Syndrome and Autism

The authors describe a boy presenting with acrocallosal syndrome and autism. Clinical features included craniofacial dysmorphisms, polydactyly, and mental retardation, besides behavioral symptoms compatible with autism. Neuroimaging revealed hypoplasia of the corpus callosum and cerebellar abnormalities. The role of this entity and other associated conditions in autism may be coincidental or reveal new clues to the understanding of autism as a behavioral syndrome.     

Self-Regulation and Affective Expression During Play in Children with Autism or Down Syndrome: A Short-Term Longitudinal Study

Our study examined stability of self-regulation and affective expression in children with autism or Down syndrome over a 2 year period. A behaviorally-anchored rating scale was used to assess a self-regulation factor (attention, adaptability, object orientation, and persistence), negative affect factor (hostility, irritability, and compliance), and positive affect factor (positive affect, affective sharing, and dull affect) from videotapes of play sessions involving each child and his or her mother. The patterns of ratings within each group were similar from time 1 to time 2, with the autism group showing more deviant ratings on measures of self-regulation and affective sharing. From time 1 to time 2, children with autism showed relatively high stability for the self-regulation factor, but less stability than children with Down syndrome for all three factors.     

Comparison Groups in Autism Family Research: Down Syndrome, Fragile X Syndrome, and Schizophrenia

This paper examines methodological challenges inherent in conducting research on families of children with autism and in comparing these families with others who are coping with different types of disabilities or who have nondisabled children. Although most comparative research has contrasted families whose child has autism with those whose child has Down syndrome, the range of comparison groups can be expanded to offer additional points of contrast and control. We discuss both matching and statistical control procedures and point to next steps in this line of comparative autism family research.     

Pre-Linguistic Autism Diagnostic Observation Schedule Adapted for Older Individuals with Severe to Profound Mental Retardation: A Pilot Study

The Autism Diagnostic Observational Schedule (ADOS) is a semi-structured observational scale developed to assess social interaction, communication and play in individuals who are suspected to have autism. Since the ADOS is not suitable to be used with severely or profoundly mentally retarded adolescents and adults with very limited language skills, materials and some of the tasks of the PL-ADOS and the original ADOS (the former versions of the current ADOS) were adapted. Results indicated that almost all of the overall ratings showed good reliability and discriminative diagnostic validity. Furthermore, the combination of codings into an overall algorithm score on social/communicative behavior resulted in a sensitivity of .82 and a specificity of .85 when using a cut-off score of 15.     

Joint Engagement and the Emergence of Language in Children with Autism and Down Syndrome

Systematic longitudinal observations were made as typically developing toddlers and young children with autism and with Down syndrome interacted with their caregivers in order to document how joint engagement developed over a year-long period and how variations in joint engagement experiences predicted language outcome. Children with autism displayed a persistent deficit in coordinated joint attention; children with Down syndrome were significantly less able to infuse symbols into joint engagement. For all groups, variations in amount of symbol-infused supported joint engagement, a state in which the child attended to a shared object and to language but not actively to the partner, contributed to differences in expressive and receptive language outcome, over and above initial language capacity.     

Phonotactic patterns in the speech of children with Down syndrome

Phonotactic patterns of seven 11–15‐year‐old Kannada speaking children with Down syndrome (DS), mental age matched children with mental retardation (MR) without DS and six 4–5‐year‐old typically developing (TD) children were investigated. Conversational speech analyses and target analyses of conversational speech were carried out in all three groups of participants. Imitated speech samples from both groups of children with disorders were also analysed with respect to phonotactic patterns. Both conversational and imitated speech analyses revealed that children with DS showed a higher percentage of occurrences of simpler phonotactic patterns than the later acquired complex ones. Target analyses revealed certain similarities in all three groups indicating that while persons with Down syndrome attempted certain complex phonotactic shapes, errors such as consonant deletions, syllable deletions and cluster reductions led to the use of simpler phonotactic patterns. Based on these analyses, the study explores the possible presence of Childhood Apraxia of Speech (CAS) in children with DS and stresses the importance of assessing phonotactic deficits in these children.     

Assessing Need for Social Support in Parents of Children with Autism and Down Syndrome

Parents of children with autism frequently turn to the service delivery system to access supports designed to help adapt to the challenges of having a child with a life-long impairment. Although studies have suggested various supports and coping strategies that are effective for adapting, few studies have examined parents’ own perceptions of needs, and whether parents felt their needs were being met. In the present study the Family Needs Questionnaire (FNQ; Waaland et al., 1993) was modified to address needs for children with developmental disorders. A sample of fifty-six parents of children with autism and a comparison group of thirty-two parents of children with Down syndrome completed the FNQ. The groups did not differ significantly on the number of important needs reported nor the number of important needs being met. However, the two groups differed in the types of supports they most frequently endorsed as Important or Unmet.     

Behavioral Characteristics of Individuals with Down Syndrome

Introduction: Children and young adults with Down syndrome can demonstrate increased behavior problems compared to their typically developing peers through childhood and adolescence. Though current tools measure behavior problems in persons with intellectual disabilities, they do not capture all the behavioral problems that can occur in individuals with Down syndrome. We: (1) identify new behavioral problems observed by parents of persons with Down syndrome that are not included on standard measures of behavior, but observed by parents; (2) examine the degree to which these behaviors may be impacted by expressive language, gender, and age; and (3) suggest the need to create a new measure. Methods: This investigation examines the identified behaviors and level of parental concern of 274 children and young adults with Down syndrome receiving care at a single medical center. Results: Ninety-four percent of children with Down syndrome engaged in behavioral problems, which was significantly correlated with age and expressive language abilities. Conclusions: Early detection of problem behaviors provides an opportunity for parent resources and professional support to reduce long-term adverse effects and prevent the occurrence of additional problematic behaviors. Results from this study indicate that a measure to more effectively capture and differentiate problem behaviors in children and adolescents with Down syndrome is greatly needed.     

Parental Perspectives on Inclusion: Effects of Autism and Down Syndrome

This study examined the effects of the child's diagnosis (autism vs. Down syndrome), age, and current educational placement on parental perceptions toward inclusion for their child with disabilities. Parents of children with autism and with Down syndrome completed surveys regarding their opinions on their child's current educational placement, their desire for changing the current placement, and their views on inclusive education. Results indicated that diagnosis, age, and current placement influenced parental opinion on the ideal educational placement for their child. Parents of children with Down syndrome were significantly more likely to endorse inclusion (full-time placement in general education) as the ideal educational program for their child whereas parents of children with autism were more likely to endorse mainstreaming (consistent part-time placement with general education students). Parents of younger children and parents whose children were already placed in general education programs were more positive towards inclusion than parents of older children or students currently in special education. Findings are discussed in terms of child characteristics and prevailing educational practices.     

A Family Genetic Study of Autism Associated with Profound Mental Retardation

We sought to determine if the family loading for either the broader autism phenotype or for cognitive impairment differed according to whether or not autism was accompanied by severe mental retardation. The sample comprised 47 probands with autism meeting ICD-10 criteria, as assessed by the Autism Diagnostic Interview and the Autism Diagnostic Observation Schedule. Family history interview and findings were compared with those for the higher IQ autism and Down syndrome samples in the Bolton et al. (1994) study. The familial loading for autism and for the broader phenotype was closely comparable to that in the study of higher IQ autism, and different from that for Down syndrome. The family loading for scholastic achievement difficulties was slightly, but significantly, higher when autism was accompanied by severe retardation.     

Varieties of Repetitive Behavior in Autism: Comparisons to Mental Retardation

Systematic study of abnormal repetitive behaviors in autism has been lacking despite the diagnostic significance of such behavior. The occurrence of specific topographies of repetitive behaviors as well as their severity was assessed in individuals with mental retardation with and without autism. The occurrence of each behavior category, except dyskinesias, was higher in the autism group and autistic subjects exhibited a significantly greater number of topographies of stereotypy and compulsions. Both groups had significant patterns of repetitive behavior co-occurrence. Autistic subjects had significantly greater severity ratings for compulsions, stereotypy, and self-injury. Repetitive behavior severity also predicted severity of autism. Although abnormal repetition is not specific to autism, an elevated pattern of occurrence and severity appears to characterize the disorder.     

Behavioural flexibility in individuals with Angelman syndrome, Down syndrome, non-specific intellectual disability and Autism spectrum disorder

Background   Little is known about behavioural flexibility in children and adults with Angelman syndrome and whether people with this syndrome have more or less problems in being behaviourally flexible as compared with other people.
Method   Behavioural flexibility scores were assessed in 129 individuals with Angelman syndrome using 11 items from the Behavioural Flexibility Rating Scale-Revised ( Green et al. 2007 ). Level of behavioural flexibility scores in individuals with Angelman syndrome ( N  = 129) was compared with that of people with non-specific intellectual disability (ID) ( N  = 90), Down syndrome ( N  = 398) and Autism spectrum disorder ( N  = 235).
Results   Comparative analyses show that individuals with Angelman syndrome were more flexible than those with non-specific ID ( P  < 0.001) and those with Autism spectrum disorder ( P  < 0.01). There were no differences in behavioural flexibility scores between individuals with Angelman syndrome and those with Down syndrome ( P  = 0.94).
Conclusion   It is concluded that individuals with Angelman syndrome are comparatively flexible in their behaviour.     

The PDD-MRS: An Instrument for Identification of Autism Spectrum Disorders in Persons with Mental Retardation

The Scale of Pervasive Developmental Disorder in Mentally Retarded Persons (PDD-MRS) is described. The PDD-MRS is a simple classification and screening instrument devised for identification of autistic disorders (of the entire spectrum) in persons with mental retardation from mild to profound levels, age-range 2–55 years. The norms of the scale are based on the research protocols of 1230 Dutch persons with mental retardation. The scale’s sensitivity for the entire normative sample was found to be 92.4%; calculated separately for persons at all levels of mentally retarded functioning, male and female persons, speaking and non-speaking persons and five age categories, the sensitivity figures range between 87.0 and 100.0%. The specificity of the scale is also 92.4%; for the aforementioned subgroups separately, the specificity figures range between 84.6 and 95.5%. Roughly similar values for sensitivity and specificity were found when using the scale with severely visually impaired/blind persons; severely hearing-impaired/deaf persons; persons with Down syndrome; male persons with fragile X syndrome. The original version of the PDD-MRS dates from 1990; since then the scale has been widely used in the Netherlands and Belgium. The PDD-MRS should be regarded as a useful instrument for identifying PDD in persons with mental retardation.     

Multiple Cerebral Hemorrhagic Lesions Depicted by Susceptibility-Weighted Imaging in a Patient with Down Syndrome: Case Report

Our objective is to study a 53-year-old woman with Down syndrome presented with massive lobar hematoma in the left fronto-parietal lobe, and who underwent craniotomy and hematoma evacuation. Histopathological diagnosis of surgical specimen was amyloid angiopathy. Postoperative magnetic resonance studies were performed. The lesion this time showed mixed intensity on susceptibility-weighted imaging. In addition, multiple hypointense lesions were evident. An old previously unidentified hemorrhage in the right temporo-parietal lobe was accompanied by superficial cortical siderosis. Old bleeds were apparent in subcortical areas. These various kinds of hemorrhagic lesion were consistent with findings of amyloid angiopathy reported in the elderly. Most reported cases of Down syndrome associated with intracerebral hemorrhage have involved middle-aged patients. Magnetic resonance studies for Down syndrome patients before old age may disclose the degree to which amyloid angiopathy progresses in the brain of these patients.     

Brief Report: Perception of Genuine and Posed Smiles by Individuals with Autism

Individuals with autism are impaired in the recognition of fear, which may be due to their reduced tendency to look at the eyes. Here we investigated another potential perceptual and social consequence of reduced eye fixation. The eye region of the face is critical for identifying genuine, or sincere, smiles. We therefore investigated this ability in adults with autism. We used eye-tracking to measure gaze behaviour to faces displaying posed and genuine smiles. Adults with autism were impaired on the posed/genuine smile task and looked at the eyes significantly less than did controls. Also, within the autism group, task performance correlated with social interaction ability. We conclude that reduced eye contact in autism leads to reduced ability to discriminate genuine from posed smiles with downstream effects on social interaction.     

Brief Report: Eye Direction Detection Improves with Development in Autism

Eye direction detection has been claimed to be intact in autism, but the development of this skill has not been investigated. Eleven children with autism and 11 typically developing children performed a demanding face-to-face eye direction detection task. Younger children with autism demonstrated a deficit in this skill, relative to younger control participants. Older children with autism were as accurate as older control participants on this task. In autism, eye direction detection is deficient in late childhood but is typically accurate by adolescence. The implications of this finding for models of social cognitive development in autism are considered.