Cognition in people with Prader-Willi syndrome: Insights into genetic influences on cognitive and social development

- PWS is caused by loss of maternally imprinted genes on chromosome 15.
- There is Impaired intellectual and social cognition in PWS.
- Impaired cognition underlies some behavioural problems in PWS.
- Subtle differences in cognition exist between genetic subtypes of PWS.

We present a mini-review of cognition in Prader-Willi syndrome. Studies cited include findings on general ability (IQ), IQ correlates with family members, strengths and weaknesses in cognitive profiles in genetic subtypes, attainment in literacy and numeracy, language, comprehension, modality preferences, executive functions, and social cognition. The latter includes investigations of theory of mind, emotion recognition, face processing and knowledge of social norms. Results from research on mouse models and brain imaging studies relevant to cognition are briefly discussed.The importance of these studies to understanding and managing education and behaviour in PWS and the limitations of the studies in terms of small numbers, non-representativeness, and lack of replication is also touched upon.