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Prader–Willi syndrome: cycloid psychosis in a genetic subtype?

Published online by Cambridge University Press:  24 June 2014

W. M. A. Verhoeven ,
S. Tuinier  and
L. M. G. Curfs
W. M. A. Verhoeven*
Affiliation:
Vincent van Gogh Institute for Psychiatry, Venray Faculty of Medicine and Medical Sciences, Erasmus University, Rotterdam
S. Tuinier
Affiliation:
Vincent van Gogh Institute for Psychiatry, Venray
L. M. G. Curfs
Affiliation:
Clinical Genetics Center South-East Netherlands, Maastricht, the Netherlands
*
Prof. Dr W.M.A. Verhoeven MD, PhD, Vincent van Gogh Institute for Psychiatry, Stationsweg 46, 5803 AC Venray, the Netherlands. Tel: 31 478 52733; Fax: 31 478 527110
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Abstract

Prader–Willi syndrome (PWS) is a genetically determined disorder associated with the loss of the paternal contribution to the proximal part of the long arm of chromosome 15. Its pathophysiology is dominated by hypothalamic dysfunctions. The psychopathological phenotype comprises affective and psychotic symptoms as well as an increase of pre-existent obsessive-compulsive behaviors. The present study comprises 19 PWS patients who were referred for neuropsychiatric evaluation because of psychotic deterioration. Patients were assessed by using the elements of semistructured symptom checklists. In the majority a genetic analysis was performed to detect the underlying chromosomal defect. In 16 of the 19 patients a diagnosis of cycloid psychosis could be established. The other three showed a bipolar affective disorder. Of the psychotic patients, 11 were diagnosed as UPD and one as del 15q11–13. The remaining four patients were diagnosed clinically. For various reasons the genetic etiology could not be established. In PWS patients with a psychotic disorder (cycloid psychosis) a disproportional number of UPD is found.

Keywords

cycloid psychosisPrader–Willi syndromeuniparental disomy
Type
Research Article
Information
Acta Neuropsychiatrica , Volume 15 , Issue 1 , February 2003 , pp. 32 - 37
Copyright
Copyright © 2003 Blackwell Munksgaard

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