The Prevalence and Phenomenology of Repetitive Behavior in Genetic Syndromes

Signalling changes to individuals who show resistance to change can reduce challenging behaviour

“…Magenis, and Lowe syndromes compared to multiple neurodevelopmental disorder comparison groups (Moss, Oliver, Arron, Burbidge & Berg, 2009). The increased risk of challenging behaviour following changes to expectations has been demonstrated in research with individuals with Prader-Willi and fragile X syndromes (Tunnicliffe, Woodcock, Oliver, Bull & Penhallow, 2014;Woodcock, Oliver & Humphreys 2009a) and is reported anecdotally by families with these other genetic syndromes.…”

“…PWS is caused by a mutation affecting the paternally derived q11-q13 region of chromosome 15. Most prevalence estimates for clinically elevated preference for predictability are upwards of 60% of individuals with the syndrome (Holland et al, 2003;Moss et al, 2009). The profile of challenging behaviour precipitated by changes in people with PWS most commonly takes the 1 Prader-Willi syndrome is abbreviated as PWS form of temper outbursts, which are shown by at least 80% of individuals and are an important priority for intervention (Holland et al, 2003;Tunnicliffe et al, 2014;Woodcock et al, 2009a).…”

Experimental functional analysis of aggression in children with Angelman syndrome

“…Between 5-10% of people who show the clinical features of Angelman syndrome have no demonstrable cytogenetic or molecular abnormality of chromosome 15q11-13 (Clayton-Smith & Laan, 2003;Laan, Halley, den Boer, Hennekam, Renier & Brouwer, 1998;Lossie, Whitney, Amidon, Dong, Chen, Theriaque, Hutson, Nicholls, Zori, Williams & Driscoll, 2001). The syndrome is associated with specific physical characteristics, developmental delay and distinctive cognitive and behavioral phenotypes (Williams, Beaudet, Clayton-Smith, Knoll, Kyllerman, Laan, Magenis, Moncla, Schinzel, Summers & Wagstaff, 2006;Horsler & Oliver, 2006a;Moss, Oliver, Arron, Burbidge & Berg, 2009). One of the most salient behavioral features of Angelman syndrome is the presence of pro-social behaviors, such as excessive laughing and smiling, noted in a detailed review of 64 studies documenting 842 cases in the literature (Horsler and Oliver, 2006a).…”

Cornelia De Lange, Cri Du Chat, and Rubinstein-Taybi Syndromes

“…However, Moss, Oliver, Arron, Burbidge and Berg (2009) report that an attachment to specific objects is a marked characteristic of the syndrome. Occurring at a clinically significant level in over 67% of individuals, the frequency of this behaviour is significantly higher in CdCS than in four other genetic syndromes including Angelman, Cornelia de Lange, Fragile X and Prader Willi syndromes and in comparison to individuals with intellectual disability of heterogeneous cause.…”