Karl Jacobsen

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.

Rett syndrome and ageing: a case study

PURPOSE This case study of an elderly women with Rett syndrome is used to consider whether observed changes may be related to physiological processes involved in ageing or environmental adaptations, which is important for delivery of rehabilitation. METHOD The life story of a woman with Rett syndrome who lived to the age of 60 is presented. It is based on medical records, older and more recent videotapes, and interviews with her sister and care staff. RESULTS After 21 years without walking, following intensive physiotherapy, she regained the ability to walk without support. She also showed improvement in hand use a few years before she died. During the early regression she appeared to lose social interest. The interest improved after some time, but she remained wary of people she did not know. CONCLUSION The walking and hand use indicate that these functions may have been present to a greater extent than assumed by people in the environment and that her poor function reflects dyspraxia and lack of opportunity and training rather than lack of ability. Although more studies of elderly women with Rett syndrome is needed to answer whether the observed changes were due to physiological processes involved in ageing or environmental adaptations, the present case story demonstrates that identification of females with Rett syndrome is important at all age levels.Purpose : This case study of an elderly women with Rett syndrome is used to consider whether observed changes may be related to physiological processes involved in ageing or environmental adaptations, which is important for delivery of rehabilitation. Method : The life story of a woman with Rett syndrome who lived to the age of 60 is presented. It is based on medical records, older and more recent videotapes, and interviews with her sister and care staff. Results : After 21 years without walking, following intensive physiotherapy, she regained the ability to walk without support. She also showed improvement in hand use a few years before she died. During the early regression she appeared to lose social interest. The interest improved after some time, but she remained wary of people she did not know. Conclusion : The walking and hand use indicate that these functions may have been present to a greater extent than assumed by people in the environment and that her poor function reflects dyspraxia and lack of opportunity and training rather than lack of ability. Although more studies of elderly women with Rett syndrome is needed to answer whether the observed changes were due to physiological processes involved in ageing or environmental adaptations, the present case story demonstrates that identification of females with Rett syndrome is important at all age levels.

Outdoor education gives fewer demands for action regulation and an increased variability of affordances

In childrens lives there are a lot of instigators for actions in every milieu and situation. When children grow older, the cortical activity starts to regulate the action instigation from the limbic system. The school system makes demands of action regulation for children. In outdoor education the many instigators for actions are not under the same demands for regulation as inside the classroom. In this study we have interviewed nine children about their experience with outdoor education. The interviews shows a wide variability of affordances grasped from outdoor education, as well as a tendency toward gender differences in what kind of action possibilities they grasp. These activities give the children useful experiences that are directly and indirectly relevant for their curriculum.

Predictors of Psychosocial Outcomes in Hard of Hearing Preschool Children

Children with hearing loss are at risk for developing psychosocial problems. Children with mild to severe hearing loss are less frequently subject to research, in particular in preschool, and we therefore know less about the risk in this particular group. To address this, we compared psychosocial functioning in thirty-five 4-5-year olds with hearing aids to that of 180 typically hearing children. Parent ratings of psychosocial functioning and social skills, as well as scores of receptive vocabulary, were obtained. Children with hearing loss evidenced more psychosocial problems than hearing agemates. Female gender and early detection of hearing loss predicted better psychosocial functioning among children with hearing loss, whereas vocabulary and degree of hearing loss did not. Early intervention addressing psychosocial functioning is warranted for children with all degrees of hearing loss, including mild and moderate. Gender differences should be investigated in future research.

Relation Between the School Environment and the Children's Behaviour

The aim of this study was to investigate the behavioural outcomes in outdoor education compared with the traditional indoor school condition. 12 children were observed intensively during three days with outdoor education and four days with the indoor condition. Results showed that different organization of the educational environment affected the childrens behaviour in various ways, such as levels of physical activity, concentration, being at ease in the situation, verbal and motor agitation, emotional expressions and communication. Outdoor education influenced behavioural changes in a positive direction. During outdoor education, there were only minor differences between leisure time and pedagogical time, with the most desired result in leisure time. In classroom, the difference was enormous, with the most desired results in leisure time.

Challenging behaviour in an adult male with congenital deaf-blindness: analysis and intervention

People with severe congenital disabilities have been assessed on negatives, on what they do not have. Skill training and education of these missing abilities have been the major focus for the habilitation since emergence of the normalization ideology in the 1960s. Developmental theories and movements like quality of life and positive psychology have changed focus from training and education to well-being and other internal states in people with disabilities. This article describes how challenging behaviour vanished in a deaf-blind man when developmental theory was applied as the framework for his habilitation. Emotional processing and initiatives increased and the man became easier to understand for the staff. The special case of a deaf-blind man illustrates how simple a focus on internal states may slip and be exchanged for intervention dominated by demands and training. The article discusses whether the framework employed in the present intervention should be present in all kind of habilitation.

Emotion Understanding in Preschool Children with Mild-to-Severe Hearing Loss

Abstract Deaf and hard of hearing school-aged children are at risk for delayed development of emotion understanding; however, little is known about this during the preschool years. We compared the level of emotion understanding in a group of 35 4–5-year-old children who use hearing aids to that of 130 children with typical hearing. Moreover, we investigated the parents’ perception of their childs level of emotion understanding. Children were assessed with the Test of Emotion Comprehension. Parents were presented with the same test and asked to guess what their child answered on each item. The results showed that children with hearing loss performed at the same level as typically hearing children, despite having lower vocabulary scores. Parents of children with hearing loss were more accurate in their estimations of their childs competence, and higher accuracy was associated with better emotion understanding. These findings may have implications for early intervention planning.

Difficulties in understanding the impact and functions of visual attention patterns of persons with autism

Abstract Twenty persons with autism were assessed with two visual acuity tests with different demands to language competence, sustained attention and direction of the subjects attention. For only one of the persons with autism did the two tests reveal equal visual acuity. For 5 of the remaining 19 persons with autism did the two tests reveal quite different visual acuity, and 14 of the persons with autism did only respond to one of the two visual acuity tests and not to the other. Visual attention pattern was also assessed. Results suggested that two forms of rigid visual attention pattern among the persons with autism may form the basis for differences in their test performance. A relation between visual attention pattern and reduced accuracy in eye movements in persons with autism is suggested. Dependent on developmental pathways, the visual attention pattern may either be adaptive for information pickup, or maladaptive and protective from a chaotic visual world.

Social skills in preschool children with unilateral and mild bilateral hearing loss

ABSTRACT Hearing loss may represent a risk for developing social skills difficulties; however, little is known about the potential risk resulting from unilateral or mild bilateral hearing loss (UMHL). We compared the social skills of 14 children with UMHL and 21 children with moderate to severe hearing loss (MSHL) with those of 123 children with typical hearing (TH). All the children were 4–5 years old, and all the children with hearing loss used hearing aids. The study was carried out in Norway. Associations between social skills and age at amplification and vocabulary skills were examined. The children with UMHL had lower social skills than the TH children, whereas the children with MSHL received scores similar to those of the TH children. The children with UMHL were detected and amplified later than the children with MSHL. Early amplification was associated with better social skills but not with better vocabulary. The results suggest that despite a limited effect on vocabulary development, early intervention is likely to promote social skills development in children with UMHL.

Mapping inclusion of a child with autism in a mainstream kindergarten: how can we move towards more inclusive practices?

ABSTRACT This study identify and reflect on barriers to inclusion that children with autism can meet in kindergarten. We use a single case study with participant- and video observation to map inclusion for a single 5-year-old boy with autism, in a mainstream kindergarten in Norway. Analysis identified three modes of inclusion; distance-keeping, maintaining proximity and interacting. The mapping procedure demonstrated that barriers to inclusion continue to operate. The extent of the childs participation seemed to relate to what he was doing and who he was with; overall, limited social inclusion amongst peers being achieved. Results indicated that predictable frameworks and teacher support increased participation. We discuss how participation for children with autism can be promoted. Our study points toward the need to extend current adaptations and support to children with autism within the educational settings, to enable a more inclusive practice.