Background: Prader-Willi syndrome (PWS) is a neurodevelopmental genetic disorder involving executive deficits notably with planning. The main objective of the study is to assess the effectiveness of cognitive training on daily life planning difficulties in PWS patients. Methods/design: The study is a double-blind randomized controlled trial which will compare the effectiveness of a metacognitive strategy intervention designed to improve planning difficulties for PWS patients to usual occupational therapy. Sixty adults will be included over 20 months. The main outcome measure will be the performance on the Modified Six Elements Test from the BADS; secondary outcome measures will be computerized executive tasks and questionnaires. Daily life planning difficulties will be identified and transformed into measurable goals using Goal Attainment Scaling. Discussion: The project will provide knowledge on the difficulties experienced by PWS patients, in relation to their executive functioning in order to implement effective intervention for planning in daily life.
Keywords: Cognitive rehabilitation; Prader-Willi syndrome; executive functions; metacognitive strategy; planning; randomised controlled trial.