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Tijdschrift voor Psychiatrie 63 (2021) 6, 432 - 440

Review article

Psychiatric disorders in adults with Prader-Willi syndrome: a systematic literature review

A. Tarsimi, S. Van Den Ameele, C.L. Crunelle, J. De schepper, N. Vanderbruggen


Background Prader-Willi syndrome (PWS) is a genetic syndrome characterized by dysmorphic features and endocrine, cognitive and psychiatric problems. Psychiatric problems interfere with the transition from pediatric to adult care. Psychiatric expertise is needed to facilitate this transition.
Aim To provide a literature review on the prevalence and clinical presentation of psychiatric disorders in adults with PWS.
Method A systematic literature review following the PRISMA-guidelines.
Results Thirty-three articles were included. Most adults with PWS had a specific behavioral profile with disruptive, autistic and compulsive characteristics. Psychotic symptoms occured in one third of adults with PWS, mostly in patients with maternal uniparental disomy. Mood disorders were present in 10 to 20% of adults with PWS and often accompanied by psychotic features. Studies were limited and heterogeneous in samples and methods.
Conclusion There is a broad spectrum of psychiatric symptoms in adults with PWS. The clinical presentation does not fully fit within the DSM categories and shows differences between genetic subgroups. Longitudinal studies assessing the psychiatric symptoms with standardized methods are needed to improve practices on diagnosing, prevention, and treatment.