Stephen von Tetzchner

VISION, COGNITION AND DEVELOPMENTAL CHARACTERISTICS OF GIRLS AND WOMEN WITH RETT SYNDROME

Forty‐two females with Rett syndrome, aged 2.5 to 47 years, were assessed with the Teller Acuity Cards and a new version of the Fagan test for age 2 years and above, and their parents were interviewed about the childrens communication skills. The visual function of the subjects indicated arrested development, and they scored significantly lower on the Fagan test than a normal comparison group. Their visual processing and memory deteriorated somewhat with age, while those of the comparison group showed a slight increase. Both age at onset of Rett syndrome symptomatology and speech measures were inversely correlated with visual processing and memory, indicating that age at recession may have differential consequences for different functions. Among the subjects, persistent looking was associated with low cognitive function. The results have implications for intervention, and demonstrate that the paradigm of preferential looking may be useful in cognitive assessment of females with Rett syndrome.

Inclusão de crianças em educação pré-escolar regular utilizando comunicação suplementar e alternativa

Many children who use alternative means of communication are educationally segregated even if there is no evidence that segregated educational settings will promote their communication and language development better than nonsegregated settings. The potential positive effects of being part of inclusive setting are not yet fully described but recent theoretical explanations of language development suggest that the communication and language development of children who use manual and graphic communication may be better scaffolded in inclusive than in segregated preschool settings provided the communicative practices of the adults and the peers are sufficiently adapted to the abilities and limitations of the children developing and facilitate child-child interactions and enculturation. The examples presented illustrate how inclusive practices may be used to promote alternative language development in children with different abilities and limitations, suggest several ways in which peers may interact with children who develop alternative means of communication, including situations involving ordinary child conflicts, and indicate reasons why inclusive settings may be beneficial for many children developing alternative communication.

Terminology and notation in written representations of conversations with augmentative and alternative communication.

There is a need for a continuous discussion about what terms one should use within the field of augmentative and alternative communication. When talking and thinking about people in their role as users of alternative communication forms, the terms should reflect their communicative ways and means, their achievements and what they are doing, rather than focus on what they cannot do. There are rather few articles analyzing utterance construction and dialogue processes involving children and adults using manual and graphic communication systems. The aim of this paper was to contribute to reviving the discussion of terminology and to more analyses of signing and aided communication and an increase in the use of conversation excerpts in the AAC Journal and elsewhere.

Constructing Preschool Communities of Learners that Afford Alternative Language Development

The present paper discusses the theoretical bases of language development and inclusion, and describes some strategies for scaffolding the communication and language development of children who use manual and graphic communication in ordinary preschool settings. The potential positive effects of being part of an inclusive setting are not yet fully described, but they will depend on whether the language environment is sufficiently adapted to the abilities and limitations of the children. The examples presented focus on child – child interactions and illustrate that alternative language development and enculturation may be supported by the communicative practices of adults and children in ordinary preschools. The examples also illustrate how inclusive practices may be used to promote alternative language development in children with different abilities and limitations; suggest several ways in which peers may interact with children who develop alternative means of communication, including situations involving ordinary child conflicts; and indicate reasons why inclusive settings may be beneficial for many children developing alternative communication.

Rett syndrome: geographic variation in prevalence in Norway

The prevalence of Rett syndrome is reported for three Norwegian counties (Rogaland, Ostfold and Nordland). The total number of females between 3 and 19 years of age in these counties was 96,920, and among these 21 females with Rett syndrome were identified, yielding a prevalence rate for Rett syndrome of 2.17 per 10,000 girls. One reason for this comparatively high prevalence rate might be that the full spectrum of Rett syndrome variants was included. The quality of the health care system and the awareness of Rett syndrome and its variants among Norwegians physicians also make it unlikely that many case were missed. However, the high total prevalence was caused by a statistically significant larger number of girls with Rett syndrome in Rogaland than in the other two counties. Sixteen of the girls were identified in Rogaland county, which gives a prevalence rate for this county of 3.77 per 10,000 girls. The prevalence rates in the two other counties were 1.05 and 0.77 per 10,000 girls. The geographical distribution of girls with Rett syndrome in Rogaland is probably due to genetic clustering. Geographical mobility in Norway is limited and many families have lived in the same geographical area for generations. An explanation based on genetic clustering is also supported by the fact that several of the girls with Rett syndrome in Rogaland county are known to be related.

Screening Individuals with Intellectual Disability for Psychiatric Disorders: Comparison of Four Measures.

Convergences and divergences between four of the most commonly used checklists for psychiatric disorders (the Reiss Screen, The Mini PAS-ADD, the DASH-II, and the ADD) were examined. We screened 181 individuals with intellectual disability for psychiatric disorders with the four checklists and compared the results on the checklists. The concordance of the overall scores on the four checklists was high; but the agreement on specific psychiatric disorders was limited. The results indicate that the checklists are useful as general indicators of psychiatric disorders, but are of less value for specifying the nature of the disorders in individuals with intellectual disability.

Social Participation of School-aged Children Who Use Communication Aids: The Views of Children and Parents

Abstract Social participation is crucial for childrens development and well-being; however, little is known about the social participation of children who use communication aids. This article presents findings from interviews with eight 5- to 14-year-old children who used communication aids and their parents about social participation, communicative interactions, and peer relationships. Video- and audio-recordings were transcribed and analyzed using thematic content analysis, and five themes were identified. Two themes reflect parents’ views: Communication partners and strategies and Access to aided communication. Three themes reflect perceptions expressed both by children and parents: Participation in society, Interaction opportunities, and Social relationships. The findings provide insights into both the achievements and the challenges experienced by young people who use aided communication.

Evaluation of the International Child Development Programme (ICDP) as a community-wide parenting programme.

Background: Many parenting programmes lack proper evaluation, especially under community-wide implementation. Objective: Examining the effectiveness of the eight-week International Child Development Programme (ICDP), implemented as a general programme. Methodology: Non-clinical caregivers attending ICDP (N = 141) and a non-attending community comparison group (N = 79) completed questionnaires on parenting, psychosocial functioning, and child difficulties before and after ICDP course. Analyses compare changes in scores for both groups over time. Results: The ICDP group showed more positive attitudes towards child management and reported better child management, improved parental strategies and less impact of child difficulties. Caregivers with low initial scores benefited most. The comparison group showed little change with a significant decrease in scores on the caregiver–child activity scale. Discussion: The results suggest that caregivers in the community who do not show clinical signs or have children with behaviour or other disorders, may benefit from participating in parent training based on ICDP.

Utility of the Child Behavior Checklist as a Screener for Autism Spectrum Disorder

The Child Behavior Checklist (CBCL) has been proposed for screening of autism spectrum disorders (ASD) in clinical settings. Given the already widespread use of the CBCL, this could have great implications for clinical practice. This study examined the utility of CBCL profiles in differentiating children with ASD from children with other clinical disorders. Participants were 226 children with ASD and 163 children with attention‐deficit/hyperactivity disorder, intellectual disability, language disorders, or emotional disorders, aged 2–13 years. Diagnosis was based on comprehensive clinical evaluation including well‐validated diagnostic instruments for ASD and cognitive testing. Discriminative validity of CBCL profiles proposed for ASD screening was examined with area under the curve (AUC) scores, sensitivity, and specificity. The CBCL profiles showed low discriminative accuracy for ASD (AUC 0.59–0.70). Meeting cutoffs proposed for ASD was associated with general emotional/behavioral problems (EBP; mood problems/aggressive behavior), both in children with and without ASD. Cutoff adjustment depending on EBP‐level was associated with improved discriminative accuracy for school‐age children. However, the rate of false positives remained high in children with clinical levels of EBP. The results indicate that use of the CBCL profiles for ASD‐specific screening would likely result in a large number of misclassifications. Although taking EBP‐level into account was associated with improved discriminative accuracy for ASD, acceptable specificity could only be achieved for school‐age children with below clinical levels of EBP. Further research should explore the potential of using the EBP adjustment strategy to improve the screening efficiency of other more ASD‐specific instruments. Autism Res 2015.

The Prevalence of Behavior Problems Among People with Intellectual Disability Living in Community Settings

With the desegregation processes of services for people with intellectual disability (ID) that is taking place in most Western countries there is a need for more knowledge related to the prevalence of behavior problems among people living in community settings. This study investigates the prevalence of behavior problems among 140 adolescents and adults with ID living in 5 municipalities in Norway using the Aberrant Behavior Checklist (Aman & Singh, 1986, 1994). Twenty percent of the sample were reported to show severe behavior disorder or “challenging behavior,” and this gives a prevalence for challenging behavior of 63.6 people per 100,000 base population. On the average, people showing challenging behavior had 7 behaviors rated as severe problems. A total of 60 participants were reported to have moderate or severe behavior problems, giving a prevalence for moderate and severe behavior problems of 136.3 per 100,000 base population. There were more people with profound ID and more people with autism in the group with challenging behavior and more people with Down syndrome in the group with mild or no problems. Those with severe behavior problems were perceived to be significantly less satisfied with their life situation than people without severe behavior problems. Challenging behavior seems to have a significant negative impact on the quality of life of people with ID. The results of this and other studies of challenging behavior among individuals with ID who live in areas with different service structures, using base population information, are important for analyzing the impact of reforms and different service systems on the life quality and well-being of people with ID.