An analogue assessment of repetitive hand behaviours in girls and young women with Rett syndrome
tone and motor problems; Rett syndrome; psychological intervention; continuous adult attention; no stimulation; demands vs stimulation; repetitive hand movements; hand movements
BACKGROUND: Rett syndrome is a neurodevelopmental disorder that almost exclusively affects females. In addition to neurodevelopmental regression and loss of hand skills, apraxia, deceleration of head growth, and increasing spasticity and scoliosis, a number of behavioural features are also seen, including stereotypic hand movements, hyperventilation and breath holding. The aim of the study was to investigate the extent to which analogue environmental conditions affected the frequency of repetitive hand behaviour in eight girls and young women with Rett syndrome. METHOD: The frequency of repetitive hand movements was observed every 10 s for four 4-min sessions under the following conditions: Continuous Adult Attention, Adult Demands, Stimulation and No Stimulation. RESULTS: The frequency of repetitive hand movements was high -- they occurred in above 60% of all intervals in all conditions for all participants and at nearly 100% for some participants in some conditions. For one participant the frequency of repetitive hand movements was somewhat reduced in the Stimulation condition; for another it was relatively increased in the No Stimulation condition. CONCLUSIONS: Overall, environmental manipulations had relatively limited effects on repetitive hand behaviours. Repetitive hand behaviour in Rett syndrome may be maintained by automatic reinforcement or neurochemical processes and may not be primarily influenced by contingent reinforcement.
Wales L; Charman T; Mount R H
Journal of Intellectual Disability Research
2004
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1365-2788.2003.00590.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2003.00590.x</a>
Self-injurious behaviour in Cornelia de Lange syndrome: 2. association with environmental events
intervention; symptoms; children; Rehabilitation; Neurology; Education & Educational Research; prevalence; Genetics & Heredity; behavioural phenotype; brachmann-delange syndrome; Cornelia de Lange syndrome; descriptive analysis; functional-analysis; individuals; Neurosciences &; phenomenology; phenotype; prader-willi syndrome; Psychiatry; self-injurious behaviour; social reinforcement; behavioral problems; De Lange syndrome; psychological intervention; environmental conditions; demand denial no contact; environment; self-injury
Background Self-injurious behaviour is commonly seen in Cornelia de Lange syndrome (CdLS). However, there has been limited research into the aetiology of self-injury in CdLS and whether environmental factors influence the behaviour. Methods We observed the self-injury of 27 individuals with CdLS and 17 participants who did not have CdLS matched for age, gender, level of intellectual disability and mobility. Descriptive analyses were used to determine the extent to which environmental events were associated with self-injury. Results Lag sequential analysis of the association between self-injurious behaviour and environmental events revealed no differences between the two groups in terms of either the number or degree of environmental associations. Conclusions The results suggest that the associations between the environment and self-injury in CdLS do not differ from those seen in the broader population of people with intellectual disability. By implication the social reinforcement hypothesis is equally applicable to both groups.
Sloneem J; Arron K; Hall S; Oliver C
Journal of Intellectual Disability Research
2009
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1365-2788.2009.01183.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2009.01183.x</a>
Guided eating or feeding: three girls with Rett syndrome
children; Rehabilitation; occupational therapy; skills; Activity; dyspraxia; engagement; hand function; hand splints; hand use; involvement; movements; participation; stereotyped hand movements; tone and motor problems; Rett syndrome; psychological intervention; guided eating; being fed
Rett syndrome (RTT) considerably limits participation in daily activities but food and mealtimes are most often motivating activities for persons with RTT. The aim of this study was to investigate whether there is a difference in participation during meals when the persons eating do so through guided eating compared with being fed. Three girls with classic RTT participated in a study inspired by single-subject design. Investigation was performed during two meals at which the girls were fed and during a seven- to eight-week period when guided eating took place. Video analysis and registration forms were used, investigating (1) coordination between opening of the mouth and spoon movement, (2) signs of involvement during the meal, and (3) cooperation in arm movements during guided eating. Guided eating led to improved coordination between opening of the mouth and spoon movement, resulting in opening of the mouth before the spoon arrived, for all of the girls. Signs of involvement changed in two of the girls. According to the guiders, they were able to feel cooperation in arm movements during the different food intake sequences in all three girls. These results indicate that guided eating improved involvement and participation in the eating process in these girls.
Qvarfordt I; Engerstrom I W; Eliasson A C
Scandinavian Journal of Occupational Therapy
2009
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1080/11038120802326214" target="_blank" rel="noreferrer noopener">10.1080/11038120802326214</a>
TEACHING SELF-FEEDING SKILLS TO PATIENTS WITH RETT-SYNDROME
Pediatrics; Neurosciences & Neurology; girls; splints; feeding difficulties; tone and motor problems; Rett syndrome; psychological intervention
Few studies have examined the extent to which functional hand-use may be taught to girls with Rett syndrome after those skills have been lost. In this investigation, five females with classic Rett syndrome taught to feed themselves using a method of prompting and reinforcement. All patients demonstrated improvements in their self-feeding skills. The results are discussed in terms of the factors that may influence the acquisition of skills in girls with Rett syndrome, and directions for future research and proposed.
Piazza C C; Anderson C; Fisher W
Developmental Medicine and Child Neurology
1993
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1469-8749.1993.tb11581.x" target="_blank" rel="noreferrer noopener">10.1111/j.1469-8749.1993.tb11581.x</a>
Improving functional skills and physical fitness in children with Rett syndrome
Treatment Outcome; Child; Humans; Female; Motor Skills; Statistics as Topic; Feasibility Studies; Social Behavior; Physical Fitness; Activities of Daily Living/psychology; Education of Intellectually Disabled; Exercise/psychology; Rett Syndrome/psychology/rehabilitation; tone and motor problems; Rett syndrome; psychological intervention; daily conductive educational program
BACKGROUND: To investigate the feasibility of a physical exercise programme with treadmill for persons with Rett syndrome (RS) in order to promote fitness and health. METHODS: A daily training programme on a treadmill was designed for four females with RS over a period of 2 months with tests performed in three intervals, at time 1, 2 and 3, 2 months apart with intervention taking place between tests 2 and 3. Participants were four girls with RS aged 8.5-11 years (mean: 10 years) attending the educational facility Beit Issie Shapiro, Raanana, Israel, all with independent mobility and with typical characteristics of RS stage III. The training took place at the educational facility, on a 1400 model treadmill (Trimline, capable of very low speeds < 0.5 k/h), with very long side rails. Special low side rails were adapted to the treadmill in order to fit the height of the children and velcro straps were added to assist in safely placing the hands. Pulse was monitored constantly during exercise by an A3 polar pulse belt. Pulse measurements at rest during training were considered as evaluators of aerobic physical condition. Functional measurement was based on a scale specially established for the present study. The scale was a 31-item motor-functioning tool that measures the ability of participants to knee walk and knee stand, to get up to a standing position, duration of walking different paths, and to go up and down stairs and slopes. RESULTS: The study showed that physical fitness of the children at the end of the training programme had improved considerably (P < 0.05). Tests showed that general functional abilities had improved considerably (P < 0.0001). Although all items of the functional ability measure showed impressive positive change, some of the 31 items on it showed statistically significant improvement (knee walking, going up and down stairs and speed of walking for 25 m. Pearson correlation showed high linkage (r = -0.76) between functional improvement and change in physical fitness. CONCLUSIONS: Physical fitness programme executed on a daily basis is capable of improving functional ability of children with RS. Nonprofessional personnel can execute such a programme under supervision of a qualified physical therapist.
Lotan M; Isakov E; Merrick J
Journal of Intellectual Disability Research
2004
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1365-2788.2003.00589.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2003.00589.x</a>
Behavior management of feeding disturbances in urea cycle and organic acid disorders
Pediatrics; feeding difficulties; argininosuccinate kyase deficiency; MCM deficiency; ornithine carbamoyltransferase deficiency disease; propionic acidemia; psychological intervention; positive reinforcement; guidance techniques
Hyman S L; Porter C A; Page T J; Iwata B A; Kissel R; Batshaw M L
Journal of Pediatrics
1987
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1016/s0022-3476(87)80121-x" target="_blank" rel="noreferrer noopener">10.1016/s0022-3476(87)80121-x</a>
Sleep problems in children with Sanfilippo syndrome.
Humans; Longitudinal Studies; Sleep Disorders/etiology; Questionnaires; Time Factors; Sleep Disorders/therapy; Child Preschool; Mucopolysaccharidosis III/classification; Mucopolysaccharidosis III/complications; child; female; male; sleep disturbance/disorders; MPS III; psychological intervention
Sanfilippo syndrome is a rare degenerative disorder which has severe intellectual and behavioural sequelae, commonly including sleep problems. A parental questionnaire was used to gather information on the sleep patterns of 80 children with Sanfilippo syndrome (mean age 10 years 2 months). The majority were found to have sleep problems (78%). Many also exhibited other distressing and unusual night time behaviours (staying up all night, chewing the bedclothes or crying out suddenly), and a few laughed or sang. Such problems may have been more severe in those with Sanfilippo syndrome type B. In four of the families offered individually tailored behaviour-management advice there was immediate improvement, which was maintained at followup in two cases. These results demonstrate the usefulness of even such a minimal intervention, even in a very difficult population such as this.
Colville G A; Watters J P; Yule W; Bax M
Developmental Medicine and Child Neurology
1996
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1469-8749.1996.tb12114.x" target="_blank" rel="noreferrer noopener">10.1111/j.1469-8749.1996.tb12114.x</a>
Punishment learning and self mutilation in Lesch Nyhan disease
automutilation; aversion; child; etiology; learning; Lesch Nyhan syndrome; punishment; reinforcement therapy; behavioral problems; psychological intervention; aversive stimulation electric shock; positive reinforcement; time-out; self-injury; self-mutilation
The self injurious behavior of five male children with Lesch Nyhan disease was studied during three learning paradigms: punishment, positive reinforcement and time out from reinforcement following an attempt at self injury. Punishment of self injury by contingent electric finger shock failed to suppress this behavior but positive reinforcement of non self injury and time out procedures were effective. The results stress the importance of environmental factors in the development and control of self injurious behavior, and suggest the presence of an unusual harmful defect which may be related to the specific enzyme deficiency in Lesch Nyhan disease.
Anderson L; Dancis J; Alpert M; Herrmann L
Nature
1977
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1038/265461a0" target="_blank" rel="noreferrer noopener">10.1038/265461a0</a>
Behavioral contingencies and self-mutilation in Lesch-Nyhan disease
Adolescent; Behavior Therapy/mt [Methods]; Child; Preschool; Humans; Lesch-Nyhan Syndrome/px [Psychology]; Lesch-Nyhan Syndrome/th [Therapy]; Male Punishment; Self Mutilation/th [Therapy]; behavioral problems; Lesch-Nyhan syndrome; psychological intervention; aversive stimulation electric shock; positive reinforcement; time-out; self-injury; self-mutilation
Lesch-Nyhan syndrome is a rare, sex-linked, recessive disease that is accompanied by severe self-mutilation, especially finger biting. Evidence is presented suggesting that parental response patterns may contribute to the genesis of the self-injurious behavior (SIB). The therapeutic effectiveness of punishment, positive reinforcement of either SIB or non-SIB, and time-out learning paradigms were evaluated in 5 Ss aged 3-13 yrs. Electric skin shock failed to suppress the behavior. Positive reinforcement of non-self-injury and time-out from social reinforcement were consistently and rapidly effective, indicating a complex interaction of genetic and environmental factors in the production of SIB. Elimination or major reductions in incidence of SIB was maintained during follow-up periods of 2 yrs. (19 ref)
Anderson L; Dancis J; Alpert M
Journal of Consulting and Clinical Psychology
1978
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1037/0022-006X.46.3.529" target="_blank" rel="noreferrer noopener">10.1037/0022-006X.46.3.529</a>