Angelman
Food related issues in Angelman and Prader-Willi syndromes
Angelman (AS) and Prader-Willi (PWS) syndromes are caused by missing genetic information on the same area of chromosome 15 (15q11-13). In AS this disruption occurs on the maternal chromosome and in PWS the paternal chromosome. Although AS and PWS are often described as “sister” syndromes, there are very few behavioural characteristics which are associated with both syndromes.
A behavioural characteristic more commonly associated with PWS than AS is hyperphagia (excessive hunger or increased appetite), which can lead to excessive eating and never feeling full. However, research conducted at the Cerebra Centre suggests that children with AS may also show difficulties with feeding. In a questionnaire study conducted at the Centre, food related problems were assessed in 156 children and adults with PWS, AS and three other genetic syndromes (1p36, Cornelia de Lange and fragile X syndrome). Supporting previous research, children with PWS showed a high frequency of food related problems compared to other groups. However, many children with AS also displayed specific food related problems including taking/storing food and negative behaviours (for example eating inedible items). The results begin to suggest that food related problems may be present in a similar proportion of children and adults with AS and PWS, and warrant further research to describe these behaviours in a larger sample of children and adults.