Self-injury and aggression in tuberous sclerosis complex: cross syndrome comparison and associated risk markers
Pain; Aggression; problem behavior; Neurosciences & Neurology; Tuberous sclerosis complex; functional-analysis; autism spectrum disorders; intellectual disabilities; ASD; challenging behavior; cognitive impairments; de-lange-syndrome; developmental-disabilities; early-childhood; Impulsivity; psychometric properties; Repetitive/stereotyped behaviour; Self-injury; behavioral problems; De Lange syndrome; tuberous sclerosis; trajectory; characteristics; pain behaviors; mood; low mood; hyperactivity; repetitive language use
Background: Research reporting prevalence rates of self-injurious and aggressive behaviour in people with tuberous sclerosis complex (TSC) is limited. No studies have compared rates of these behaviours in TSC with those in other syndrome groups matched for degree of disability or investigated risk markers for these behaviours in TSC. Methods: Data from the Challenging Behaviour Questionnaire were collected for 37 children, aged 4 to 15 years, with TSC. Odds ratios were used to compare rates of self-injury and aggression in children with TSC with children with idiopathic autism spectrum disorder (ASD), fragile X, Cornelia de Lange and Down syndromes. Characteristics were measured using the Mood Interest and Pleasure Questionnaire, the Activity Questionnaire, the Social Communication Questionnaire, the Repetitive Behaviour Questionnaire, the Wessex Behaviour Schedule and the revised Non-communicating Children Pain Checklist. Mann-Whitney U analyses were used to compare characteristics between individuals with self-injury and aggression and those not showing these behaviours. Results: Rates of self-injury and aggression in TSC were 27% and 50%, respectively. These are high but not significantly different from rates in children with Down syndrome or other syndrome groups. Both self-injury and aggression were associated with stereotyped and pain-related behaviours, low mood, hyperactivity, impulsivity and repetitive use of language. Children who engaged in self-injury also had lower levels of interest and pleasure and showed a greater degree of `insistence on sameness' than children who did not self-injure. Aggression was associated with repetitive behaviour. The majority of these associations remained significant when the association with level of adaptive functioning was controlled for. Conclusions: Behavioural profiles can be used to identify those most at risk of developing self-injury and aggression. Further research is warranted to understand the influence of such internal factors as mood, ASD symptomatology and pain on challenging behaviour in people with intellectual disability.
Eden K E; de Vries P J; Moss J; Richards C; Oliver C
Journal of Neurodevelopmental Disorders
2014
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/10.1186/1866-1955-6-10" target="_blank" rel="noreferrer noopener">10.1186/1866-1955-6-10</a>
Snake: the development and validation of a questionnaire on sleep disturbances in children with severe psychomotor impairment
Validity; Screening; Neurosciences & Neurology; psychometric properties; behaviors; scale; disabilities; Children with severe psychomotor impairment; Daytime restlessness; instrument; Parent questionnaire; school-aged children; Sleep disturbance/disorders; sleep disturbance; Q3 conditions; tool development; scale development; SNAKE
Objective: Sleep disturbance and daytime restlessness are present in 50% to 80% of children with severe psychomotor impairment due to neurologic or other complex diseases. Although these issues severely impair the quality of life of the children and their families, they are not well addressed or managed by professionals. The lack of validated assessment tools prevents further research and the development of adequate therapies. Our aim is to develop and validate a sleep questionnaire for these children that could be used both for clinical management and systematic research. Methods: The sleep questionnaire for children with severe psychomotor impairment (Schlaffragebogen fur Kinder mit Neurologischen und Anderen Komplexen Erkrankungen, SNAKE) is based on expert opinion and consultation with parents. The psychometric quality of the questionnaire was assessed in a sample of 224 children with severe psychomotor impairment. Results: Confirmative factor analysis showed that SNAKE comprises of five factors (based on ICSD-2). Fit indices, analysis of item characteristics and convergent validity (coherence with measures of sleep [i.e., sleep efficiency]) and correlation with selected subscales of the Sleep Disturbance Scale for Children (SDSC) were good. Re-test analysis (n = 62) depicted high stability and good replication of validity. Conclusions: SNAKE is a reliable and valid tool for the diagnosis of sleep disturbances in children with severe psychomotor impairment. The SNAKE questionnaire is the first tool that addresses the specific relationship between sleep disturbance and severe disability in children. (c) 2013 Elsevier B.V. All rights reserved.
Blankenburg M; Tietze A L; Hechler T; Hirschfeld G; Michel E; Koh M; Zernikow B
Sleep Medicine
2013
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/j.sleep.2012.12.008" target="_blank" rel="noreferrer noopener">10.1016/j.sleep.2012.12.008</a>