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Prader-Willi syndrome and psychotic symptoms: report of a further case

Published online by Cambridge University Press:  13 June 2014

David J Clarke ,
Tessa Webb  and
Joyce P Bachmann-Clarke
David J Clarke
Affiliation:
Centre for Research and Information in Mental Disability, Department of Psychiatry, University of Birmingham, Queen Elizabeth Psychiatric Hospital, Edgbaston, Birmingham, B15 2QZ, England
Tessa Webb
Affiliation:
Department of Paediatrics and Child Health, University of Birmingham, Maternity Hospital, Birmingham, B15 2TG, England
Joyce P Bachmann-Clarke
Affiliation:
University of Basel, Basel, Switzerland
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Abstract

A 21 year-old woman with Prader-Willi syndrome (PWS) and a delusional disorder (paranoid psychosis) is described. Other reports of psychoses associated with PWS are reviewed. Reasons for the co-existence of the two disorders are considered, with discussion of the possible role of auditory information processing deficits in the genesis of psychotic symptoms. Such symptoms must be differentiated from maladaptive behaviours not arising from psychosis, the latter being relatively common in PWS. Treatment with a small dose of flupenthixol greatly reduced the impact of the patient's psychotic symptoms, with a corresponding improvement in her quality of life.

Keywords

Prader-Willi syndromePsychiatryPsychosesGeneticsbehavioural aspectsMental retardation
Type
Brief Reports
Information
Irish Journal of Psychological Medicine , Volume 12 , Issue 1 , March 1995 , pp. 27 - 29
Copyright
Copyright © Cambridge University Press 1995

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References

1.Prader, A, Labhart, A, Willi, H. Ein Syndrom von Adipositas, Kleinwuchs, Kryptorchismus und Oligophrenie nach myatonieartigem Zustand im Neugeborenalter. Schweiz Med Wochenschr 1956; 86: 1260–1.Google Scholar
2.Butler, MG. Prader-Willi syndrome: current understanding of cause and diagnosis. Am J Med Gen 1990; 35: 319–32.CrossRefGoogle ScholarPubMed
3.Knoll, JHN, Wagstaff, J, Lalande, M. Cytogenetic and molecular studies in the Prader-Willi and Angelman syndromes. Am J Med Gen 1993; 46: 2–6.CrossRefGoogle ScholarPubMed
4.Nicholls, RD. Genomic imprinting and uniparental disomy in Angelman and Prader-Willi syndromes: a review. Am J Med Gen 1993; 46: 16–35.CrossRefGoogle ScholarPubMed
5.Dunn, HG. The Prader-Labhart-Willi syndrome: review of the literature and report of nine cases. ActaPaed Scand 1968; Suppl 186: 1–38.Google Scholar
6.Greenswag, LR. Adults with Prader-Willi syndrome: a survey of 232 cases. Dev Med Child Neurol 1987; 29: 145–52.CrossRefGoogle ScholarPubMed
7.Whitman, BY, Accardo, P. Emotional symptoms in Prader-Willi syndrome adolescents. Am J Med Gen 1987; 28: 897–905.CrossRefGoogle ScholarPubMed
8.Clarke, DJ, Waters, J, Corbett, JA. Adults with Prader-Willi syndrome: abnormalities of sleep and behaviour. J Roy Soc Med 1989; 82: 21–4.CrossRefGoogle ScholarPubMed
9.Borghgraef, M, Fryns, JP, Van den, Berghe H. Psychological profile and behavioural characteristics in 12 patients with Prader-Willi syndrome. Gen Counseling 1990; 1: 141–50.Google ScholarPubMed
10.Curfs, LMG, Verhulst, FC, Fryns, JP. Behavioural and emotional problems in youngsters with Prader-Willi syndrome. Gen Counseling 1991; 2: 33–41.Google ScholarPubMed
11.Holm, VA, Cassidy, SB, Butler, MG, Hanchett, JM, Greenberg, F, Whitman, BY, Greenswag, LR. Diagnostic criteria for Prader-Willi syndrome. In: Cassidy, SB, editor. Prader-Willi Syndrome and other chromosome 15q deletion disorders. NATO ASI Series H: Cell Biology Vol 61. Berlin: Springer-Verlag, 1992; 105–17.CrossRefGoogle Scholar
12.Clarke, DJ. Prader-Willi syndrome and psychoses. Br J Psychiatry 1993; 163: 680–4.CrossRefGoogle ScholarPubMed
13.World Health Organisation. The ICD-10 Classification of Mental and Behavioural Disorders: diagnostic criteria for research. Geneva: World Health Organisation, 1993.Google Scholar
14.Nicholls, RD. Genomic imprinting and candidate genes in the Prader-Willi and Angelman syndromes. Curr Opin Genet Dev 1993; 3: 445–56.CrossRefGoogle ScholarPubMed
15.Kollrack, HW, Wolff, D. Paranoid-halluzinatorische Psychose bei Prdaer-Labhart-Willi-Fanconi syndrome. Acta Paedopsychiatrica 1966; 33: 309–14.Google ScholarPubMed
16.Bray, GA, Dahms, WT, Swerdloff, RS, Fisher, RH, Atkinson, RL, Carrel, RE. The Prader-Willi syndrome: a study of 40 patients and a review of the literature. Medicine 1982; 62: 59–80.CrossRefGoogle Scholar
17.Bhate, MS, Robertson, PE, Davison, EV, Brummitt, JA. Prader-Willi syndrome with hypothyroidism. J Ment Def Res 1989; 33: 235–44.Google ScholarPubMed
18.Jerome, L. Prader-Willi syndrome and bipolar illness. J Am Acad Child Adolesc Psychiatry 1993; 32: 876–7.CrossRefGoogle ScholarPubMed
19.Tu, J-B, Hartridge, C, Izawa, J. Psychopharmacogenetic aspects of Prader-Willi syndrome. J Am Acad Child Adolesc Psychiatry 1992; 31: 1137–40.CrossRefGoogle ScholarPubMed
20.Verhoeven, WMA, Tuinier, S. Pharmacological advances in mental retardation; towards a specific phenotypical approach?Paper presented at the Society for the Study of Behavioural Phenotypes Third International Symposium; 11 1994, Congrescentrum Rolduc, Maastricht.Google Scholar
21.Åkefeldt, A, Gillberg, C, Larsson, C. Prader-Willi syndrome in a Swedish rural county: epidemiological aspects. Dev Med Child Neurol 1991; 33: 715–21.CrossRefGoogle Scholar
22.Corbett, JA. Psychiatric morbidity and mental retardation. In: Snaith, P, James, FE, editors. Psychiatric illness and mental handicap. London: Gaskell, 1979.Google Scholar
23.Curfs, LMG. Psychological profile and behavioral characteristics in the Prader-Willi syndrome. In: Cassidy, SB, editor. Prader-Willi syndrome and other chromosome 15q deletion disorders. NATO ASI Series H: Cell Biology Vol 61. Berlin: Springer-Verlag, 1992; 211–21.CrossRefGoogle Scholar
24.Whitman, BY, Greenswag, LR. The use of psychotropic medications in persons with Prader-Willi syndrome. In: Cassidy, SB, editor. Prader-Willi syndrome and other chromosome 15q deletion disorders. NATO ASI Series H: Cell Biology Vol 61. Berlin: Springer-Verlag, 1992; 223–31.CrossRefGoogle Scholar