Acquisition of graphic communication by a young girl without comprehension of spoken language.

PURPOSE: To describe a graphic-mode communication intervention involving a girl with intellectual impairment and autism who did not develop comprehension of spoken language. The aim was to teach graphic-mode vocabulary that reflected her interests, preferences, and the activities and routines of her daily life, by providing sufficient cues to the meanings of the graphic representations so that she would not need to comprehend spoken instructions. METHOD: An individual case study design was selected, including the use of written records, participant observation, and registration of the girl's graphic vocabulary and use of graphic signs and other communicative expressions. RESULTS: While the girl's comprehension (and hence use) of spoken language remained lacking over a 3-year period, she acquired an active use of over 80 photographs and pictograms. CONCLUSIONS: The girl was able to cope better with the cognitive and attentional requirements of graphic communication than those of spoken language and manual signs, which had been focused in earlier interventions. Her achievements demonstrate that it is possible for communication-impaired children to learn to use an augmentative and alternative communication system without speech comprehension, provided the intervention utilizes functional strategies and non-language cues to the meaning of the graphic representations that are taught.

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.

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.

The use of graphic language intervention among young children in Norway.

Knowledge about graphic communication intervention and use among young disabled children has been sparse. The present study is based on a thorough search, and provides reliable information about children who are 10 years old or younger who have graphic communication (for example, photographs, drawings, Blissymbols, PIC, traditional orthography) as their main form of communication. The study covers all such children in three Norwegian counties, comprising a population of 1.1 million people. Detailed descriptions of the children, the systems they use, and their educational settings are presented, and implications for the organisation of habilitation services are discussed.

Historical issues in intervention research: hidden knowledge and facilitating techniques in Denmark.

Facilitating techniques have recently both gained wider popularity and stirred considerable controversy. They are usually assumed to originate in Australia but the present paper documents that similar techniques created a major public event in Denmark and appeared occasionally in the USA in the 1960s and 1970s. However, in spite of the dramatic claims made by proponents of the techniques, their use did not spread widely and the international network has remained tenuous. It is argued that the "discovery' of facilitating techniques as a means to disclose unexpected literacy depends on the use of hand guidance and a context of professional beliefs where responses of unclear origin may be attributed to the person whose hand is being guided.

Communication skills among females with Rett syndrome.

The present study describes communicative behaviors of 42 Norwegian girls and women with Rett syndrome (RS) aged 2 1/2-47 years. Most of them did not seem to show behaviors which may be interpreted as indicative of an intention to communicate. Many did not use gaze shifts and the shifts of those who did were usually limited to 1-3 turns. The implication for intervention is a need to apply structured overinterpretation where significant people in the environment systematic interpret behaviors indicative of interests, needs and preferences as communicative, as well as structured total communication to provide non-speech means of communication. It is argued that although the use of these strategies often will not lead to intentional communicative behaviors in females with RS, they may contribute to a responsive and predictable environment for the females and to the establishment of a set of strategies which may be used by significant people in their environment.

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 children's 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.

Communicative, sensorimotor, and language skills of young children with Down syndrome.

The transition from preverbal to verbal communication of retarded and nonretarded children was explored in a prospective, longitudinal study. Mental development was tested at ages 13 months and at 2 and 3 years. At age 2 years, the MA of the retarded subjects matched the MA of the nonretarded at 13 months. Sensorimotor development of the retarded children at 2 and 3 years of age was assessed by the Uzgiris-Hunt scales. Splitting the sample according to whether or not the retarded subjects had reached Sensorimotor Stage 6 at age 3 yielded differential results on concurrent language skills. Pragmatic skills measured at age 2 for the retarded subjects were deficient as compared with such skills assessed at 13 months for the nonretarded subjects. Results seem to support the postulate of a skill-specific homology in the transition from preverbal to verbal communication.

A longitudinal study of low birthweight children: reproductive, perinatal, and environmental precursors of developmental status at three years of age.

The study includes all children (N = 62) with birthweight below 2000 grams, born within a 15 months period at a municipal hospital in Oslo, Norway. Since there are no private hospitals, and home births are almost non-existent, children born at the municipal hospital constitute a cross-section of the population. Low birthweight children made up 2.3% of all births in the hospital during the 15-month period. A wide range of medical data concerning pregnancy, delivery, and the perinatal period was collected. On the basis of these data an optimality index was determined for each child. Intellectual assessments were made at 3, 6, 9, 12, 18, and 36 months. Observation of the home environment was made at 6 months. Parents were interviewed about their children's behavioral problems, the language skills were assessed, at 36 months. Children who at 3 years of age had poor performance on the Stanford-Binet and on the Reynell receptive scale, were among those who had had a low optimality index. The optimality index did not discriminate children with low scores on the Reynell expressive scale, spontaneous to MLU, and the behavioral questionnaire. Children with a low optimality index, but who performed well on the Stanford-Binet and on the Reynell receptive scale had higher SES, as compared with children with a low optimality index who had poor performance on these measures at 3 years. Therefore environmental factors may modate the influences of reproductive and perinatal risk.